medical study

CAROTID SINUS SYNDROME

2009-01-24T06:05:00.001-08:00

CAROTID SINUS SYNDROME - Jeremy Golding, MD
BASICS
DESCRIPTION
• In carotid sinus syndrome, stimulation of one or both of the hypersensitive carotid sinuses at the bifurcation of the common carotid arteries produces brief episodes of faintness or loss of consciousness. 4 types are described
- Cardioinhibitory: Vagally mediated, causing bradycardia, sinus arrest, or atrioventricular block for >3 seconds
- Vasodepressor: A sudden drop of peripheral vascular resistance leads to a >50 mm Hg decrease in systolic blood pressure (BP) without change in heart rate or to a >30 mm Hg symptomatic drop in systolic BP.
- Mixed: Combined cardioinhibitory and vasodepressor changes
- Cerebral: Extremely rare; carotid sinus hypersensitivity occurs without bradycardia or hypotension.
• System(s) Affected: Cardiovascular; Nervous
• Synonym(s): Hypersensitive carotid sinus syndrome; Carotid sinus syncope; Carotid sinus hypersensitivity
ALERT
Geriatric Considerations
• More likely to occur in elderly
• Associated with atheromata secondary to coronary artery disease. Should be considered in elderly patients with frequent falls.
GENERAL PREVENTION
• Avoidance of pressure on the neck
• Support hose may be helpful for some patients with vasodepressor type.
EPIDEMIOLOGY
• Predominant age: Elderly
• Predominant sex: Male > Female
Incidence
64 of 132 consecutive patients (48.5%) >65 evaluated for dizziness, falls, or syncope were found to have carotid sinus-type sensitivity.
RISK FACTORS
• Diffuse atherosclerosis
• Wearing tight collars
• Shaving over region of carotid sinus
• Emotional upheaval
• Head movement
ETIOLOGY
• Unknown etiology
• Stimulation of the hypersensitive baroreceptors in the carotid sinus affects vagus and sympathetic nerve outflow.
• Carotid body tumors
• Inflammatory and malignant lymph nodes in the neck
• Metastatic cancer
• Coronary artery disease
ASSOCIATED CONDITIONS
• Sick sinus syndrome
• Atrioventricular block
• Coronary artery disease

DIAGNOSIS
SIGNS AND SYMPTOMS
Paroxysmal
Dizziness
Syncope
Falls
Blurred vision
Vertigo
Tinnitus
Bradycardia
Hypotension
Pallor
Sweating
Tachypnea
No postictal symptoms
TESTS
Special tests
• With the patient in the supine position and while the ECG is monitored, manual pressure ("massage") of the carotid sinus causes asystole of >3 seconds (cardioinhibitory) and/or a drop in systolic BP as described in Description. Diagnostic yield may be increased by combining with tilt-table testing.
Diagnostic Procedures/Surgery
• Unilateral carotid sinus pressure ("massage") (check for potential contraindications before performing massage, including carotid bruits, known carotid hypersensitivity, and demonstrated carotid artery disease). Direct steady pressure is applied over the carotid sinus for 10 seconds.
• Orthostatic vital signs (exclude orthostatic hypotension)
• Electrophysiologic studies
• ECG
• Carotid duplex scan
DIFFERENTIAL DIAGNOSIS
• Vasovagal syncope
• Postural hypotension
• Primary autonomic insufficiency
• Hypovolemia
• Arrhythmias
• Sick sinus syndrome
• Syncope secondary to reduced cardiac output (e.g., aortic stenosis)
• Cerebrovascular insufficiency
• Emotional disturbances
• Other causes of syncope
TREATMENT
Outpatient. No treatment is required for asymptomatic individuals.
GENERAL MEASURES
Cardiac pacing (dual chamber) is the treatment of choice for recurrent episodes.
Diet
No special diet
Activity
No restrictions
MEDICATION (DRUGS)
First Line
• Anticholinergics: Atropine (acutely) for the cardioinhibitory type
• Sympathomimetics: Ephedrine
• Theophylline
• In one study, selective serotonin reuptake inhibitors were successful in controlling symptoms.
• Contraindications: Refer to manufacturer's instructions.
• Precautions: Concomitant usage of digitalis, -blockers, clonidine, and -methyldopa may accentuate response to carotid sinus massage.
• Significant possible interactions: Refer to manufacturer's instructions.
Second Line
Fludrocortisone has been used in clinical trials for patients with vasodepressor carotid sinus syndrome.
SURGERY
• Carotid sinus denervation by surgery or radiation therapy for selected patients
• Implantation of a permanent pacemaker helps prevent recurrent symptoms in patients with cardioinhibitory component.
• Surgery for selected patients with atheromata
FOLLOW-UP
DISPOSITION
Admission Criteria
Syncope of uncertain etiology
PROGNOSIS
May be serious if syncope is associated with atheromatous narrowing of sinus artery or basilar artery
COMPLICATIONS
• Prolonged confusion
• Frequent falls, leading to injuries and fractures
PATIENT MONITORING
Follow as an outpatient.
REFERENCES
1. ACC/AHA/NASPE 2002 Guideline update for implantation of cardiac pacemakers and antiarrhythmia devices: Summary article. A report of the American College of Cardiology/American Heart Association task force on practice guidelines (ACC/AHA/NASPE committee to update the 1998 pacemaker guidelines). Circulation. 2002;106:2145.
2. Braunwald E, ed. Heart Disease: A Textbook of Cardiovascular Medicine, 6th ed. Philadelphia: WB Saunders, 2001.
3. Isselbacher KJ, et al., eds. Harrison's Principles of Internal Medicine, 14th ed. New York: McGraw-Hill, 1998.
4. McIntoch SJ, Lawson J, Kenny RA. Clinical characteristics of vasopressor, cardioinhibitory, and mixed carotid sinus syndrome in the elderly. Am J Med. 1993;95:203-208.
MISCELLANEOUS
• See also: Atherosclerosis
• It is clinically important to distinguish carotid sinus syndrome from sick sinus syndrome.

CAROTID SINUS SYNDROME

2009-01-24T06:05:00.000-08:00

CARDIOMYOPATHY, END-STAGE

2009-01-24T05:59:00.000-08:00

CARDIOMYOPATHY, END-STAGE - Tim Fitzgibbons, MD; Theo E. Meyer, MD, DPhil
BASICS
DESCRIPTION
In 1995, the WHO defined cardiomyopathy as a "disease of the myocardium associated with cardiac dysfunction." They proposed a classification system based on pathophysiology. Each class may be caused by many disorders, and some disorders may overlap classes.
• Classification of cardiomyopathy
- Dilated (systolic)
 Characterized by dilation and reduced systolic function of one or both ventricles
- Hypertrophic (diastolic)
 Left and or right ventricular hypertrophy with normal to reduced end diastolic volumes
 May include asymmetric septal hypertrophy
 Cause of SCD in young athletes
- Restrictive (diastolic)
 Restrictive filling and reduced diastolic volume of either or both ventricles
 Systolic function may be near normal
 Etiology: Idiopathic, amyloidosis, etc.
- Arrhythmogenic right ventricular (RV) dysplasia
 Fibrofatty replacement of the RV
 May present with arrhythmia or SCD in the young
- Unclassified
 Cases that do not fit easily into 1 group (i.e., non compacted myocardium)
- Specific: Includes patients with cardiomyopathy in association with a known systemic disorder, for example
 Ischemic
 Valvular
 Hypertensive
 Inflammatory
 Metabolic
 Peripartum
• "End-stage" cardiomyopathy patients have "Stage D" heart failure, or severe symptoms at rest refractory to standard medical therapy.
• System(s) Affected: Cardiovascular; Renal
ALERT
Pediatric Considerations
Etiology: Idiopathic, viral, congenital heart disease, and familial.
Pregnancy Considerations
May occur in women postpartum
GENERAL PREVENTION
Reduce salt and water intake; home blood pressure measurement and a daily weight
EPIDEMIOLOGY
Predominant age: Ischemic cardiomyopathy is the most common etiology; predominantly patients >50 years. Consider uncommon causes in young.
Incidence
• 60,000 patients 65 die each year from end-stage heart disease.
• From 35,000-70,000 of the population might benefit from cardiac transplant or chronic support.
Prevalence
Most rapidly growing form of heart disease
RISK FACTORS
• Hypertension
• Hyperlipidemia
• Obesity
• Diabetes mellitus
• Smoking
• Physical inactivity
• Excessive alcohol intake
• Dietary sodium
Genetics
Hypertrophic, dilated cardiomyopathy, and arrhythmogenic RV dysplasia may present as familial syndromes with autosomal dominant inheritance.
ETIOLOGY
• Ischemic heart disease: Most common etiology; up to 66% of patients
• Hypertension
• Familial cardiomyopathies
• Congenital heart disease
• Peripartum/postpartum
• Toxic/metabolic causes
- Alcoholism
- Radiation
- Beriberi
- Kwashiorkor
- Cobalt
- Selenium deficiency
- Hemosiderosis
- Thyrotoxicosis
• Infectious causes
- Viral (e.g., HIV, coxsackievirus)
- Diphtheria
- Toxoplasmosis
- Trichinosis
- Trypanosomiasis
- Acute rheumatic fever
• Inherited disorders of metabolism
- Glycogen storage disease
- Pompe disease
- Hurler syndrome
- Hunter syndrome
- Fabry disease
• Inherited neuromuscular disorders
- Duchenne muscular dystrophy
- Friedreich ataxia
• Drugs
- Chemotherapy: Anthracyclines, cyclophosphamide, Herceptin
• Inflammatory causes
- Giant cell myocarditis
- Loeffler eosinophilia
- Sarcoidosis
• Idiopathic
• Other causes
- Tachycardia-mediated cardiomyopathy
- Amyloidosis
- Valvular heart disease
- Endomyocardial fibrosis

DIAGNOSIS
SIGNS AND SYMPTOMS
• Dyspnea at rest or with exertion
• Paroxysmal nocturnal dyspnea
• Orthopnea
• Postprandial dyspnea
• Fatigue
• Syncope
• Tachypnea
Physical Exam
• Low pulse pressure
• Cool extremities
• Jugular venous distention
• Bibasilar rales
• Tachycardia
• S3 gallop
• Hepatosplenomegaly
• Ascites
• Edema
TESTS
• ECG: LV hypertrophy, interventricular conduction delay, atrial fibrillation, evidence of prior Q-wave infarction.
• Cardiopulmonary exercise testing: Maximal oxygen consumption 10 mL/kg/mm correlates with 50% 1-year mortality, and >18 mL/kg/mm correlates with >90% 1-year survival. Used in stable outpatients to estimate prognosis and prior to cardiac transplant referral.
Lab
• Hyponatremia
• Prerenal azotemia
• Anemia
• Elevated BNP or pro-BNP
• Mild hyperbilirubinemia
• Elevated liver function tests
• Elevated uric acid
Imaging
• Chest radiograph
- Cardiomegaly
- Increased vascular markings to the upper lobes
- Pleural effusions may or may not be present
• ECG
- In dilated cardiomyopathy 4-chamber enlargement and global hypokinesis are present.
- In hypertrophic cardiomyopathy, severe left ventricular (LV) hypertrophy is present.
- Segmental abnormalities in contraction of the LV are indicative of previous localized myocardial infarction.
• Cardiac MRI
- May be useful to characterize particular nonischemic cardiomyopathies
Diagnostic Procedures/Surgery
Cardiac catheterization:
• Helpful to rule out ischemic heart disease
• PA catheters may be reasonable in patients with refractory HF to help guide management (3)[C].
DIFFERENTIAL DIAGNOSIS
• Severe pulmonary disease
• Primary pulmonary hypertension
• Recurrent pulmonary embolism
• Hypothyroidism
• Some advanced forms of malignancy
TREATMENT
GENERAL MEASURES
• Reduction of filling pressures
• Treatment of electrolyte disturbances
Diet
Low fat, low salt, fluid restriction
MEDICATION (DRUGS)
First Line
• Systolic failure syndromes
- ACE inhibitors
 Lisinopril 5-40 mg/d or captopril 6.25-50 mg t.i.d. (3)[A]
- Loop diuretics
 May need to be given IV initially, and then orally as patient stabilizes
 Furosemide 40-120 mg/d or t.i.d.(3)[A]
- -Blockers:
 Use with caution in acutely decompensated or low cardiac output states
 Metoprolol succinate 12.5-200 mg/d, carvedilol 3.125-25 mg b.i.d., or bisoprolol 1.25-10 mg/d (3)[A]
- Aldosterone antagonists
 Patients with NYHA III-IV CHF, EF35%, on standard therapy
 Spironolactone 12.5-25 mg/d (3)[A]
- Digoxin 0.125-0.250 mg/d for symptomatic patients on standard therapy (3)[A]
- BiDil (hydralazine/nitrates)
 BiDil 1 tablet t.i.d is recommended in addition to standard treatment in African American patients with Class III-IV symptoms (3)[A]
• Diastolic failure
- Few evidence-based therapies for diastolic heart failure. Empiric management goals include
 Management of hypertension
 Reduction of congestive states (i.e., diuretics)
 Prevention of progression of LVH (i.e., RAAS blockade)
 Maintenance of sinus rhythm
• Contraindications
- -Blockers: Low cardiac output, 1st- or 2nd-degree heart block
- Aldosterone antagonists: Oliguria, anuria, renal dysfunction
- Loop diuretics: Hypokalemia, hypomagnesemia
- ACE inhibitors: Pregnancy, angioedema
• Precautions
- In patients with CKD, digoxin dosage should be 0.125 mg/d, and drug levels followed carefully.
- Closely monitor electrolytes
- ACE inhibitors: Initiate with care if blood pressure is low. Begin with low-dose captopril, such as 6.25 mg t.i.d.
- -Blockers: Avoid in patients with evidence of poor tissue perfusion; they may further depress systolic function.
- Milrinone, amrinone: Contraindicated for long-term use due to increased mortality
Second Line
• Nesiritide .01 mcg/kg/min IV for 48 hours in HF patients with dyspnea at rest (3)[C]
• Angiotensin receptor blockers as an alternative to, or in addition to ACE inhibitors
SPECIAL THERAPY
• Prophylactic ICD should be considered for patients with an LVEF 30% (3)[A].
• Biventricular pacing should be considered for patients with QRS interval >120 ms, LVEF 35%, and Class III CHF despite medical therapy(3)[A].
• Patients with severe, refractory HF with no reasonable expectation of improvement should not be considered for an ICD (3)[C].
FOLLOW-UP
DISPOSITION
Issues for Referral
• Consider referral to a heart failure center.
• Management by a heart failure team improves outcomes and facilitates early transplant referral.
PROGNOSIS
20-40% of patients in New York functional class IV die within 1 year. With a transplant, a 1-year survival is as high as 94%.
COMPLICATIONS
Worsening congestive heart failure, syncope, arrhythmias, or sudden death
REFERENCES
1. Richardson P. Report of the 1995 WHO on the definition and classification of cardiomyopathies. Circulation. 1996;93:841-842.
2. Noria A, Stevenson LW. Medical management of advanced heart failure. JAMA. 2002;287:628-640.
3. Hunt SA, et al. ACC/AHA 2005 guideline update for the diagnosis and management of chronic heart failure in adults. J Am Coll Cardiol. 2005;46:1116-1143.
MISCELLANEOUS
See also: Alcohol use disorders; Amyloidosis; Diabetes mellitus, type 1; Diabetes mellitus, type 2; Hypertension; Hypothyroidism, adult; Idiopathic hypertrophic subaortic stenosis; Malnutrition, protein-calorie; Rheumatic fever; Sarcoidosis

CARDIAC TAMPONADE

2009-01-24T05:58:00.000-08:00

CARDIAC TAMPONADE - Keith Medeiros, MD
BASICS
DESCRIPTION
• Rapid or slow compression of cardiac chambers by pressure on the heart secondary to an increase in pericardial fluid.
• Tamponade can be acute or subacute depending on the etiology.
• As fluid accumulates, pressure primarily affects the compliant cardiac wall and transmits the pressure transmurally, resulting in increased ventricular pressure. This decreases ventricular filling and reduces cardiac output by reducing stroke volume.
• The compensatory mechanisms for tamponade are increased peripheral resistance, central venous pressure, and heart rate.
• In some patients, pulsus paradoxus (10 mm Hg drop in systolic blood pressure between inspiration and expiration) and equalization of pressures may not occur, the absence of which does not rule out tamponade.
• In patients with elevated left ventricular (LV) diastolic pressures (as with chronic hypertension), resistance to LV filling is constant. Throughout the cardiac cycle, equalization of pressures in these patients may only be noted in the right heart chambers, with LV pressures being higher than right ventricular (RV) pressures.
• Variants include low pressure and regional tamponade.
• System(s) Affected: Cardiovascular
RISK FACTORS
Cardiac tamponade should be suspected in the hemodynamically unstable patient
• With known pericarditis
• Following blunt or penetrating chest trauma
• Following open heart surgery or cardiac catheterization
• With known or suspected intrathoracic neoplasm
• With suspected dissecting aortic aneurysm
• With renal failure on dialysis
PATHOPHYSIOLOGY
Fluid accumulation in the pericardial space leading to compression of cardiac chambers as the heart has to compete with increased pericardial contents for the fixed intrapericardial space. Cardiac filling is reduced, limiting cardiac output and eventually causing hypotension and shock. This can occur rapidly in cases of acute tamponade, usually resulting from trauma or surgery, or over weeks to months with slowly developing effusions that allow the pericardial compliance to increase gradually (1)[A].
ETIOLOGY
• Uremia
• Neoplasm: Breast, lung, lymphoma, leukemia
• Occurs in 1% of fibrinolytic-treated acute MI and is associated with increased 30-day mortality (2)[A].
• Postoperative
• HIV
• Other viruses: Coxsackie group B, influenza, ECHO, herpes
• Bacterial infection: Staphylococcus aureus, Mycobacterium tuberculosis, Streptococcus pneumoniae (rare)
• Fungal infection: Histoplasmosis capsulatum
• Lupus and rheumatologic disease
• Trauma
• Placement of central venous catheter, pacer wires
• Hypothyroidism
• Drug effects
ASSOCIATED CONDITIONS
• Myocardial infarction
• Aortic aneurysm

DIAGNOSIS
SIGNS AND SYMPTOMS
• Acute
- Patients may complain of chest pain or dyspnea
- Markedly elevated JVP
- Signs of cardiogenic shock: Cyanosis, cool extremities, and oligouria
• Subacute
- Most common complaints are intolerance to minimal activity and dyspnea. Agitation, central nervous system depression, coma, and cardiac arrest may develop later.
History
History of renal failure, surgery, neoplasm, or trauma
Physical Exam
• Beck triad: Distant heart sounds, hypotension, distended neck veins
• Narrow pulse pressure
• Pulsus paradoxus: >10 mm Hg drop in systolic blood pressure between inspiration and expiration
• Neck veins may be distended and reveal a rapid systolic (X) descent and attenuated or absent diastolic (Y) descent
• Tachycardia: A compensatory mechanism to maintain output
• Right upper quadrant tenderness due to hepatic engorgement
• Increased area of cardiac dullness outside the apical point of maximum impulse
TESTS
ECG
• May show sinus tachycardia, low-voltage QRS complexes, diffuse ST segment elevation, and PR segment depression of pericarditis
• Electrical alternans (QRS and/or R wave variation from beat to beat) seen in 10-20% of cases of tamponade; 50-60% of these are neoplastic in origin
Lab
• CBC
• Sedimentation rate
• Cardiac enzymes to rule out acute myocardial infarction
• Antinuclear antibodies
• Rheumatoid factor
• BUN/creatinine
• Pericardial fluid for culture of bacteria, fungus, mycobacteria, Gram stain, hematocrit, cell count, cytology, glucose, protein, rheumatoid factors, complement levels
Imaging
• Chest radiograph: May show enlargement of cardiac shadow (if >200 mL fluid present)
• Echocardiogram:
- Diagnostic of cardiac compression
 RA collapse is a sensitive sign of increased intrapericardial pressure but, diastolic RV collapse is more specific for tamponade (3,4)[A].
- Doppler: May show respiratory variation in transvalvular flow velocities, LV ejection, and LV isovolumetric times (3,4)[A].
Diagnostic Procedures/Surgery
Right heart catheterization:
• Equalization (within 2-3 mm) of right atrial, pulmonary artery diastolic, pulmonary capillary wedge, left atrial, and left ventricular diastolic pressures
• The intracardiac diastolic pressure will approximate the intrapericardial pressure.
• The dip and plateau pattern of constriction or restriction pericardial disease is absent.
• Loss of Y descent on atrial waveform
Pathological Findings
Pericardial blood usually does not clot, but occasionally will.
DIFFERENTIAL DIAGNOSIS
• Tension pneumothorax
• Acute RV failure
• COPD
• Constrictive pericarditis
• Acute acceleration of chronic bronchitis
• Acute pulmonary embolus
• Fat embolus
• Excessive or rapid administration of fluids
• Abdominal distention from ascites or ileus
• Increased intrathoracic pressure from pneumothorax, hemothorax, airway obstruction, or mechanical ventilation
• Administration of vasopressors
TREATMENT
STABILIZATION
Inpatient
GENERAL MEASURES
• Maintain hemodynamic stability until definitive correction of the pericardial tamponade
• All patients should have BP, heart rate, and at a minimum, central venous pressure measurement every 15 minutes. Strong consideration should be given to placement of a Swan-Ganz catheter if time allows.
• Fluids may be of temporary benefit, but rising filling pressures may further compromise coronary perfusion
Diet
As tolerated
Activity
Bed rest
MEDICATION (DRUGS)
Inotropic support with or without vasodilators is controversial partly due to maximal endogenous inotropic stimulation present during acute tamponade (1)[A].
SURGERY
Pericardiocentesis:
• Indications
- Rapid deterioration of hemodynamic function
- A delay in operation for traumatic effusion
- Diagnosis
• If rapid reaccumulation is anticipated (as in malignancy), it may be helpful to insert a long-term drainage catheter. Also consider instillation of sclerosing agents.
• Surgery should be performed under the most optimal circumstances available, as the patient's condition allows.
• Blind pericardiocentesis should be performed only in life-threatening emergencies.
• Ideally, echocardiography can be brought to the bedside to assist in needle placement and progress of fluid removal.
• Invasive monitoring is also helpful to follow decrease in pericardial pressures.
• Fluoroscopy may also be used.
• ECG guidance using the "V" lead to avoid contact with the epicardium may be useful.
• 20% of patients with tamponade will have a negative tap because the pericardial sac contains coagulated material. Hemorrhagic pericardial effusions usually do not clot.
FOLLOW-UP
Follow-up echocardiography should be used to evaluate for recurrence of effusions (3,4)[A].
PROGNOSIS
Good results are expected with the appropriate treatment.
COMPLICATIONS
• Cardiac perforation and/or laceration at time of pericardiocentesis
• Pneumothorax at time of pericardiocentesis
• Constriction of pericardium
PATIENT MONITORING
Close monitoring until stable with telemetry to monitor for cardiac arrhythmia
REFERENCES
1. Spodck DH, Acute cardiac tamponade. N Engl J Med 2003 Aug 14;349(7):684-690.
2. Patel MR et al. Cardiac tamponade in the fibrinolytic era: Analysis of 100,000 patients with ST-segment elevation myocardial infarction. Am Heart J 2006 Feb;151(2):316-322.
3. Cheitlin MD, et al. ACC/AHA/ASE 2003 guideline update for the clinical application of echocardiography: Summary article: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines for the Clinical Application of Echocardiography, Circulation. 2003;108:1146.
4. Cheitlin MD, et al, ACC/AHA Guidelines for the Clinical Application of Echocardiography. Circulation 1997;95:1686-1744.

CARDIAC ARREST

2009-01-24T05:56:00.000-08:00

CARDIAC ARREST - Bobby Peters, MD, FAAEM
BASICS
DESCRIPTION
• Absence of effective mechanical cardiac activity
• This section is not a substitute for an American Heart Association-approved Advanced Cardiac Life Support (ACLS) course and is intended only as a quick reference.
• Synonym(s): Code Blue
ALERT
Geriatric Considerations
Poor risk for survival and long-term outcome
Pediatric Considerations
Bradycardia is the most common initial form of cardiac arrest. Most frequently, it is a response to underlying pulmonary disease and hypoxia. Adequate oxygenation and ventilation are especially important.
Pregnancy Considerations
• Displace the uterus either manually or by placing a rolled towel or pad under the right hip. If the patient cannot be resuscitated within 5-15 minutes, consider emergency C-section to relieve uterine obstruction and increase blood return to the heart. This may also be done to save the fetus if at a viable age.
• Consider amniotic fluid embolism or eclampsia-related seizures as precipitating factors.
GENERAL PREVENTION
Treat underlying disease
EPIDEMIOLOGY
• Predominant age: Increases with age
• Predominant sex: Male > Female
Prevalence
In the United States: 200:100,000 (per year)
RISK FACTORS
• Male gender
• Increasing age
• Hypercholesterolemia
• Hypertension
• Cigarette smoking
• Family history of atherosclerosis
• Diabetes
ETIOLOGY
• Asystole (confirm in two leads; 11% actually fine ventricular fibrillation [VF])
• VF
• Pulseless ventricular tachycardia (VT)
• Pulseless electrical activity (PEA, previously known as electrical mechanical dissociation [EMD])
ASSOCIATED CONDITIONS
• Coronary artery disease (cardiac arrest may be first presenting symptom)
• Valvular heart disease
• Hypertension

DIAGNOSIS
SIGNS AND SYMPTOMS
• Loss of consciousness secondary to central nervous system hypoperfusion
• Absence of pulses in large arteries
• Apnea or agonal breathing
• Cyanosis or pallor
History
Find out how patient coded
• Witness or unwitnessed?
• Seizure activity?
• History or risk factors?
Physical Exam
• Check pupils: Dilated may indicate drug overdose
• Check pulse, hydration status, diaphoretic? (i.e., reasons for tachycardia)
• Check lungs (i.e., did person have respiratory decline before cardiac decline?)
TESTS
ECG
Lab
• Arterial blood gases
• Electrolytes
• CBC
• Drug levels (check toxicology screen, Tylenol level, also digoxin level or antiepileptic levels of history of specific medication use, etc.)
• Prothrombin time (international normalized ratio), partial thromboplastin time, type, and cross, if indicated
• Lab results may be altered by
- Digoxin toxicity: May cause hyperkalemia
- Hypo- or hyperventilation: Changes oxygen partial pressure and carbon dioxide partial pressure
- Acidosis: Increases serum potassium
Imaging
Chest radiograph for endotracheal tube (ET) placement, pneumothorax; consider echocardiogram for pericardial effusion
Diagnostic Procedures/Surgery
• If PEA secondary to tamponade, may need paracardiocentesis
• If coding, probably needs airway intubation
• May need central line for IV access
• May need chest tube for pneumothorax
Pathological Findings
Based on underlying cause
DIFFERENTIAL DIAGNOSIS
• Adverse reaction to drugs: Barbiturates, narcotics, calcium channel blockers, beta-blockers, and tricyclic antidepressants
• Shock: Septic or blood-loss induced
• Hypothermia
• Pulmonary embolism
• Cardiac tamponade
• Pneumothorax
• Acidosis
• Electrolyte abnormality
• Carbon monoxide poisoning
TREATMENT
STABILIZATION
• Prehospital emergency medical service personnel, ED, "cardiac arrest team," intensive care setting
• If response time is >5 minutes, improved outcome noted in patients when CPR started before defibrillation in Vfib. (1)[A]
GENERAL MEASURES
• Perform defibrillation 1st
- Adults: 200, 300, or 360 J
- Children: Use largest paddles that will fit on child, even adult size if good contact can be achieved.
- Defibrillate at 2 J/kg once. Increase to 4 J/kg twice.
• Administer 100% oxygen by bag-valve-mask or ET (preferred)
• Start 2 IV lines as close to the heart as possible (central line okay, but do not waste time). Large-bore peripheral lines can deliver fluid more quickly than a central line, especially important in PEA secondary to hypovolemia.
• Perform CPR, including closed-chest compression. Intermittent abdominal compression and active compression/decompression show no survival advantage.
• Keep patient, especially a child, warm if possible.
• Monitor pulse after 3 initial defibrillations. Check monitor between each defibrillation and after any intervention.
• Use an end-tidal CO2 monitor to assess gas exchange, if available. Esophageal intubation will produce a very low end-tidal CO2 and requires proper reintubation.
MEDICATION (DRUGS)
First Line
• Lidocaine, atropine, naloxone, and epinephrine may all be given by ET. Follow with 10 mL of normal saline or sterile water, followed by bagging.
• Epinephrine: 1 mL = 1 mg (1:1,000); 1 mL = 0.1 mg (1:10,000)
• Adults: VT and pulseless VT. Use in order listed below
- Defibrillate (nonsynchronized setting) 3 times at 200, 300, and 360 J
 Check monitor rhythm.
 Follow each drug administration with repeated defibrillation at 360 J.
 Check monitor and pulses after each subsequent intervention.
• Epinephrine: 1 mg IV every 3-5 minutes or a vasopressin 40 U IV single dose, 1 time only; may choose to resume epinephrine if no response after a single dose of vasopressin (high-dose epinephrine is permissible, but discouraged and may actually worsen outcomes).
• Amiodarone: 300 mg IV push may be used prior to lidocaine
• Lidocaine: 1.5 mg/kg IV, repeat in 5 minutes to total dose of 3 mg/kg
• Magnesium sulfate: 1-2 mg IV in suspected torsades de pointes or refractory VF/VT
• Procainamide: 30 mg per minute IV in refractory VF/VT (maximum dose: 17 mg/kg) is permissible. However, because the time to a useful level by infusion is so long, it is discouraged and is unlikely to be of any benefit. No improvement in survival to discharge.
• Bicarbonate: 1 mEq/kg IV only in known preexisting bicarbonate-responsive acidosis or to alkalinize the urine in known tricyclic overdose
Adults: Asystole
• CPR
• Confirm in 2 leads.
• Consider possible causes, including hypoxia, hyperkalemia, hypokalemia, preexisting acidosis, drug overdose, and hypothermia.
• Consider defibrillation, as for VT/VF, since VF may be mistaken for asystole.
• Consider immediate transcutaneous pacing.
• Epinephrine: 1 mg IV push repeated q3-5min; may use intermediate-dose or high-dose epinephrine (2-5 mg IV or 0.1 mg/kg IV) q3-5min
• Atropine: 1 mg IV push q3-5min to total dose of 0.04 mg/kg; shorter atropine dosing intervals acceptable (q1-2min)
• Consider termination of efforts if no reversible underlying cause is found.
For Pulseless Electrical Activity
• Includes EMD, idioventricular rhythms, ventricular escape rhythms, bradycardic-asystolic rhythms, and postdefibrillation idioventricular rhythms
• Assess blood flow by Doppler ultrasound if available.
• Consider possible reversible causes: Cardiogenic shock (weak pump), cardiac tamponade, tension pneumothorax, severe hypovolemia, pulmonary embolism (consider thrombolytics), hypothermia, hypoxia, acidosis, hyperkalemia, or overdose of drugs such as beta-blockers, calcium channel blockers, tricyclics, and digoxin (pnemonic 5H and 5Ts).
• Epinephrine: 1 mg IV push and repeat q3-5min; may use intermediate-dose or high-dose epinephrine (2-5 mg IV or 0.1 mg/kg IV, respectively) q3-5min, but this shows no proven improvement in survival
• Atropine: 1 mg IV q3-5min to total dose of 0.04 mg/kg if absolute bradycardia (60 beats per minute) or relative bradycardia; may decrease interval to 1-2min if desired
Children (drugs listed in alphabetical order):
• Amiodarone for pulseless VF/VT, 5 mg/kg IV or intraosseous (IO) rapid bolus; for perfusing tachyarrhythmias, loading dose of 5 mg/kg IV or IO over 20-60 minutes, maximum dose 15 mg/kg/d
• Atropine: 0.01-0.02 mg/kg per dose; minimum dose is 0.1 mg, maximum single dose is 0.5 mg in child, 1.0 mg in adolescent
• Epinephrine
- For bradycardia: 0.01 mg/kg IV/IO or 0.1 mg/kg ET (1:1,000)
- For asystolic or pulseless arrest: 1st dose is 0.01 to 0.03 mg/kg IV/IO. Doses as high as 0.2 mg/kg may be effective.
- Infusion: 0.1 ug/kg per minute. Titrate to desired effect (0.1-1.0 ug/kg per minute).
• Lidocaine:
- Bolus: 1 mg/kg per dose (maximum 3 mg/kg)
- Infusion: 20-50 ug/kg per minute
• Sodium bicarbonate: 1 mEq/kg per dose or 0.3  kg  base deficit; infuse slowly and only if ventilation is adequate
• Contraindications and precautions
- There are contraindications during an arrest.
- Calcium may be used if known (preexisting) hyperkalemia precipitated arrhythmia; calcium is contraindicated in hyperkalemia secondary to digoxin.
- Magnesium is relatively contraindicated in renal failure, but given the consequences of not terminating rhythm; this is only a relative contraindication in this setting.
Second Line
Asystole: Aminophylline 250 mg IV bolus has been effective in uncontrolled trials, but should be used only when conventional therapy has failed.
SURGERY
If indicated
• Pericardiocentesis to treat cardiac tamponade
• Needle decompression (second intercostal space midclavicular line), then chest tube insertion to treat tension pneumothorax
FOLLOW-UP
PROGNOSIS
• Outcome is related to underlying disease, age, duration of arrest, and other factors.
• Outcome is poor if
- >4 minutes to CPR or >8 minutes to ACLS
- Arrest occurs in field
- Resuscitation effort >30 minutes
• About 14% survive in-hospital arrest, fewer after field arrest
COMPLICATIONS
• Significant neurologic, hepatic, renal, or cardiac ischemic injury
• Rib fractures or pneumothorax from CPR
PATIENT MONITORING
Intensive care setting on continuous monitor to look for precipitating cause, including serial ECGs and enzymes to rule out myocardial infarction
REFERENCES
1. Wik L, Hansen TB, Fylling F, et al. Delaying defibrillation to give basic cardiopulmonary resuscitation to patients with out-of-hospital ventricular fibrillation: A randomized trial. JAMA. 2003;289(11):1389-1395.
2. Graber MA. Emergency medicine. In: Graber MA, Lanternier ML, Graber M, eds. The Family Practice Handbook. St. Louis, MO: Mosby-Yearbook; 1997.
3. The American Heart Association in collaboration with the International Liaison Committee on Resuscitation. Guidelines 2000 for cardiopulmonary resuscitation and emergency cardiovascular care. Circulation. 2000;102(suppl 8):I95-I104.
MISCELLANEOUS
Make sure patient is not listed as Do Not Resuscitate.

CARBON MONOXIDE POISONING

2009-01-24T05:54:00.000-08:00

CARBON MONOXIDE POISONING - Felix B. Chang, MD
BASICS
DESCRIPTION
• Carbon Monoxide (CO) is the leading cause of poisoning death in the us CO is an odorless, tasteless, colorless, gas, produced by combustion of carbon-containing compounds.
• CO inhalation leads to displacement of oxygen from binding sites on hemoglobin.
• CO has about 250 times the affinity for hemoglobin that oxygen has.
• Detrimental effects are related to tissue hypoxia from decreased oxygen content and a shift of the oxyhemoglobin dissociation curve to the left.
• CO binds to cytochrome oxidase, impairing mitochondrial function and to cytochrome oxidase, affecting muscle function.
• CO binding to myoglobin affects muscle activity.
• System(s) Affected: Cardiovascular; Musculoskeletal; Nervous
ALERT
Tissue hypoxia includes the fetus. CO poisoning may cause significant fetal abnormalities, depending on the developmental stage. However, adult hemoglobin holds to oxygen less tightly than does fetal hemoglobin. Therefore, a pregnant mother potentially may be unaffected while the fetus is affected.
EPIDEMIOLOGY
• 40,000 emergency department visits annually
• 5,000 to 6,000 deaths annually in the US
• Inadvertent CO poisoning likely causes 500 deaths annually.
• Unintended poisoning is most common during winter month in cold climates.
• 10,000 individuals miss 1 or more days of work due to CO poisoning.
RISK FACTORS
• Cigarette smoking
• Smoke inhalation
• Being in a closed space with a faulty furnace or stove or running engine
• Employment in coal mine, as an auto mechanic, paint stripper, or in the solvent industry
• Improper vented fuel-burning devices
- Kerosene heaters, charcoal grills, camping stoves, gasoline-power generators, wood stoves
- Open air exposure to motorboat exhaust
- Underground utility electrical cable fires
PATHOPHYSIOLOGY
• CO is rapidly absorbed in lungs.
• CO binds to hemoglobin to form carboxyhemoglobin (COHb), resulting in impaired oxygen carrying capacity, utilization, and delivery.
- Leftward shift of the oxyhemoglobin dissociation curve occurs.
- CO interferes with peripheral oxygen utilization by inactivating cytochrome oxidase.
• Delayed neurologic sequelae, probably involves lipid peroxidation by toxic oxygen species generated by xanthine oxidase.
• The half-life of CO while the patient is breathing room air is ~300 minutes, while breathing high-flow oxygen via a nonrebreathing face mask is ~90 minutes, and with 100 % hyperbaric oxygen is ~30 minutes.
ETIOLOGY
• CO inhalation
• Inhaled or ingested methylene chloride (from paint remover) (dichloromethane) is metabolized to CO by the liver, causing CO toxicity in the absence of ambient CO.
ASSOCIATED CONDITIONS
CO and cyanide poisoning can occur simultaneously following smoke inhalation (synergistic effect).

DIAGNOSIS
• Acute CO poisoning is suggestive by history, physical examination, and an elevated COHb
• Chronic CO intoxication is difficult to diagnose.
• Pulse oximetry cannot screen for CO exposure, because it does not differentiate carboxyhemoglobin from oxyhemoglobin.
PRE HOSPITAL
Patients often present in clusters, with similar symptoms and a common environment.
SIGNS AND SYMPTOMS
• Headaches
• Tinnitus
• Nausea
• Dizziness
• Weakness
• Confusion
• Fatigue
• Flushing
• Vomiting
• Central nervous system depression
• Syncope
• Angina
• Tachycardia
• Tachypnea
• Cardiac dysrhythmias
• Nystagmus
• Ataxia
• Seizures
• Coma
• Diarrhea
• Cardiopulmonary arrest
Physical Exam
• A careful neurologic examination is crucial.
• In absence of trauma or burns, look for altered mental status.
• "Cherry red" appearance of the lips and skin
• Impaired judgment, respiratory depression, arrhythmias, hypotension
• Cyanosis
• Visual field defects, blindness, papilledema
TESTS
Lab
• Measurement of COHb.
• Check CO level via co-oximetry of arterial or venous blood.
• Check acid-base status on blood gas.
• EKG in all patients
• Cardiac enzymes in
-  65 years
- Patient with cardiac risk factors
- Younger patients with chest pain or symptoms suggestive of ischemia
Imaging
Head CT scan is helpful to rule out other causes of neurologic decompensation.
Pathological Findings
Hemorrhagic infarction of the globus pallidus and deep white matter have been reported (rare).
DIFFERENTIAL DIAGNOSIS
• Cyanide toxicity
• Acute viral syndrome
• Other causes of mental status changes: Metabolic, drugs, infectious, trauma
TREATMENT
STABILIZATION
• ED for mild poisoning
• Inpatient treatment for moderate or severe poisoning
GENERAL MEASURES
• Removal from offending source
• Rapid reduction in tissue hypoxia with 100% oxygen to reduce the half-time of elimination of CO to 40 minutes
• Supportive care as necessary
• Intubation and mechanical ventilation may be necessary for severe intoxication.
• Volume resuscitation
Activity
Rest until carboxyhemoglobin reduced and symptoms abate
SPECIAL THERAPY
• 100% oxygen by tight-fitting nonrebreathing mask
• Hyperbaric oxygen for severe poisoning
• For mild poisoning (carboxyhemoglobin levels 30%); no signs or symptoms of cardiovascular or neurologic dysfunction
- Treatment: Admission if carboxyhemoglobin >25%
- Symptomatic medication for headache
- 100% oxygen by nonrebreathing mask until carboxyhemoglobin 5%
- Patients with underlying heart disease should be admitted regardless of level of carboxyhemoglobin.
• For moderate poisoning (carboxyhemoglobin 30-40%); no signs or symptoms of cardiovascular or neurologic dysfunction
- Treatment: Admission
- Cardiovascular status should be followed closely, even in the absence of clear cardiac effects.
- Determination of acid-base status: Corrected by oxygen
- 100% oxygen by nonrebreathing mask until carboxyhemoglobin 5%
• For severe poisoning (carboxyhemoglobin >40%); cardiovascular or neurologic functional impairment at any carboxyhemoglobin level
- Treatment: Admission
- Cardiovascular function monitoring
- Acid-base status monitoring
- 100% oxygen by nonrebreathing mask until carboxyhemoglobin 5%
- Hyperbaric oxygen immediately if available; if unavailable, treat as in moderate poisoning
- If no improvement occurs in cardiovascular or neurologic function within 4 hours, transport the patient to the nearest facility with hyperbaric oxygen, regardless of distance.
MEDICATION (DRUGS)
First Line
• Institution of 100% oxygen by high-flow mask or endotracheal tube
• 100% normobaric oxygen for all suspected victims of CO poisoning, regardless of pulse oximetry or arterial PO2 (1)[B].
FOLLOW-UP
DISPOSITION
Admission Criteria
Patients whose symptoms do not resolve, who demonstrate ECG or laboratory evidence of severe poisoning, or who have other medical or social cause of concern should be hospitalized.
Discharge Criteria
Patient with mild symptoms from accidental poisoning can be managed in the ED and safely discharged.
PROGNOSIS
Most survivors recover completely, with only a minority developing chronic neuropsychiatric impairment.
COMPLICATIONS
• Myocardial infarction
• Pulmonary edema (CHF)
• Pneumonia (aspiration)
• Anoxic encephalopathy
• Long-term neuropsychiatric complications
- Intellectual deterioration
- Memory impairment
• Dysrhythmia
• Shock
• Rhabdomyolysis, personality changes
- Irritability
- Aggressiveness
- Violence
- Moodiness
ALERT
Geriatric Considerations
• Higher incidence of cardiovascular and neurologic disease, increasing complications.
• Atherosclerosis with chronic exposure
PATIENT MONITORING
• Measurement of carboxyhemoglobin levels
• Arterial blood gases
• Psychiatric evaluation and follow-up for intentional exposure
REFERENCES
1. Hampson NB, Scott KL, Zmaeff JL. Carboxyhemoglobin measurement by hospitals: Implications for the diagnosis of carbon monoxide poisoning. J Emerg Med. 2006;31:13.
2. Kao LW, Nanagas KA. Carbon monoxide poisoning. Emerg Med Clin North Am. 2004;22:985.
3. Satran D, Henry CR, Adkinson C, et al. Cardiovascular manifestations of moderate to severe carbon monoxide poisoning. J Am Coll Cardiol. 2005;45:1513.
ADDITIONAL READING
• Internet resources available at: www.cpsc.gov.
• Insufficient evidence to establish usefulness of hyperbaric oxygen for carbon monoxide poisoning. Cochrane Library 2005;1:CD002041.
• Juurlink D, Buckley N, Stanbrook M, et al. Hyperbaric oxygen for carbon monoxide poisoning. Cochrane Database Syst Rev 2005;CD002041.

CANDIDIASIS

2009-01-24T05:53:00.000-08:00

CANDIDIASIS - Brock D. Lutz, MD; Ronald A. Greenfield, MD
BASICS
DESCRIPTION
Candida albicans and related species cause a variety of infections
• Cutaneous syndromes include erosio interdigitalis blastomycetica, folliculitis, balanitis, intertrigo, paronychia, onychomycosis, diaper rash, perianal candidiasis, and the syndromes of chronic mucocutaneous candidiasis.
• Mucous membrane infections include oral candidiasis (thrush), esophagitis, and vaginitis.
• The most serious manifestations of candidiasis are candidemia and hematogenously disseminated invasive candidiasis.
The remainder of this chapter discusses candidemia and hematogenously disseminated candidiasis.
GENERAL PREVENTION
• Polyenes, azoles, and echinocandins reduce the incidence of candidiasis in patients undergoing induction therapy for acute leukemia or bone marrow or stem cell transplantation. (4)[A]
• Fluconazole prophylaxis in high-risk ICU patients reduces the incidence of invasive candidiasis. (6)[A]
EPIDEMIOLOGY
• Predominant age: All ages are susceptible to hematogenously disseminated candidiasis; premature neonates are at particularly high risk.
• Predominant sex: Male = Female (hematogenously disseminated candidiasis)
Incidence
20/100,000 persons per year
RISK FACTORS
• Neutropenia
• Corticosteroid treatment
• HIV infection
• Diabetes mellitus
• Mucocutaneous colonization/infection
• Broad-spectrum antibacterial chemotherapy
• Indwelling intravascular access devices
• Cardiothoracic or abdominal surgery
• Parenteral nutrition
• Prolonged hospital stay
• ICU stay
• Burns
• Premature birth
PATHOPHYSIOLOGY
An acute suppurative infection in which polymorphonuclear host defense is the critical element.
ETIOLOGY
• Candida albicans is the most frequent pathogen. Other important human pathogens include C. tropicalis, C. krusei, C. stellatoidea, C. pseudotropicalis, C. guilliermondi, C. parapsilosis, C. lusitaniae, C. rugosa, C. lambica, and C. glabrata.
• Candida species colonize human mucocutaneous surfaces; most infections are endogenously acquired from this reservoir.
• Human-to-human transmission of Candida occurs in some settings.
ASSOCIATED CONDITIONS
See "Risk Factors."

DIAGNOSIS
SIGNS AND SYMPTOMS
• Fever
• Malaise
• Tachycardia
• Hypotension
• Altered mental status
• Hepatosplenomegaly
• Maculopapular or nodular skin rash
ALERT
Pediatric Considerations
For an infant with thrush, be sure to also check for candidal diaper dermatitis. Also, there is often a concomitant infection.
TESTS
• The diagnosis is established by isolating the causative organism from blood cultures or other normally sterile body sites, or by demonstration of organisms in histopathologic specimens of normally sterile tissues.
• Isolation of Candida from multiple sites should raise the diagnostic suspicion of hematogenously disseminated invasive candidiasis.
• Candida species isolated from a normally sterile site should be identified to the species level. (4)[A]
• Because fluconazole-resistant C. albicans and particularly non-albicans species are reported with increasing frequency, fluconazole susceptibility testing should be performed before treatment with fluconazole. (4)[B]
Imaging
• Generally not specifically useful in diagnosis of hematogenously invasive disseminated candidiasis.
• In the syndrome of hepatosplenic candidiasis (chronic systemic candidiasis) imaging of the liver and spleen by liver scan, ultrasound, CT, or MRI may suggest this syndrome as the cause of persistent fever and liver dysfunction in patients who have recently recovered from neutropenia.
Diagnostic Procedures/Surgery
• If blood cultures remain consistently negative, aspiration or excisional biopsy of sites of focal infection may be useful in diagnosis.
• Aspiration and biopsy of skin lesions occasionally seen with hematogenously disseminated candidiasis are also useful.
Pathological Findings
Characteristic histopathology of lesions of Candida invasion of visceral organs is microabscess formation.
DIFFERENTIAL DIAGNOSIS
Includes a variety of cryptic bacterial infections and, in the neutropenic host, multiple opportunistic infections.
TREATMENT
Inpatient for hematogenously disseminated invasive candidiasis
GENERAL MEASURES
• Fluid and electrolyte therapy are often required.
• Hemodynamic and respiratory support may be required in seriously ill patients.
• The removal of potentially infected intravascular access devices is imperative.
Diet
No special diet
Activity
As tolerated
MEDICATION (DRUGS)
First Line
• Caspofungin
- An initial therapy of choice for any patient with candidemia (4)[A].
- Administer 70 mg IV dose on day 1 followed by 50 mg IV daily for 2 weeks after last positive sterile site culture if no evident metastatic infection
- Modify dose for severe hepatic insufficiency.
- C. parapsilosis has reduced sensitivity to echinocandins.
• Fluconazole
- An initial therapy of choice for some patients (4)[A]
- Because it is fungistatic rather than fungicidal, it should not be used for treatment of patients with severe neutropenia or severe immunosuppression.
- It should only be used after confirmation of in vitro susceptibility in patients with azole therapy in prior 3 months
- Should be used empirically only in institutions with a very low prevalence of resistance
- Useful for switch therapy after demonstration of in vitro susceptibility after initial therapy with amphotericin or an echinocandin.
- For 1st week, administer daily 400-800 mg intravenously, followed by additional IV or oral therapy at the same dose for 2 weeks after the last positive blood culture or last evidence of infection. Higher doses of fluconazole may be required if non-albicans species are known or suspected, because they carry a higher likelihood of drug resistance.
- C. krusei and many C. glabrata are resistant to fluconazole.
• Liposomal amphotericin B
- An initial therapy of choice for any patient with candidemia (4)[A]
- Usual dosage is 3 mg/kg IV daily.
- Substantially more expensive than conventional amphotericin B deoxycholate, but also substantially less toxic
- C. lusitaniae may be resistant.
- Consider higher doses for C. krusei or C. glabrata (5-10 mg/kg/day).
Second Line
• Although caspofungin is the only echinocandin approved by the FDA for this indication, preliminary data suggest that micafungin and anidulafungin have similar efficacy and safety for treatment of hematogenously disseminated invasive candidiasis. (B)
• Other azole antifungals depending on activity and safety (itraconazole and voriconazole). (B)
• Contraindications
- The safety of amphotericin B therapy in pregnant patients has not been established.
- Echinocandins are pregnancy category C.
• Precautions
- Liposomal amphotericin B
 The toxicity is less common than with conventional amphotericin B, but may still be formidable. Acute reactions (fever, rigors, and hypotension) may occur during the initiation of therapy. Ameliorate or eliminate by premedication with acetaminophen or ibuprofen. Use meperidine if needed to abort rigors.
 Azotemia may occur; there may be an indication for reducing dose in some patients (to reduce toxicity). Maintenance of optimal fluid status and prevention of dehydration help minimize the risk of azotemia. "Sodium loading" with 77 mEq (77 mmol) sodium daily (= 1 L half-normal saline) may decrease renal toxicity.
 Significant hypokalemia and renal tubular acidosis) may develop. Significant hypomagnesemia may worsen hypokalemia.
 Anemia commonly develops in patients on protracted therapy, but is almost always reversible.
 Headache and phlebitis are common.
 Leukopenia, thrombocytopenia, and liver function abnormalities are rarely encountered.
• Itraconazole, voriconazole, and caspofungin and other echinocandins do not enter the urinary stream in sufficient concentrations to treat UTIs.
• Significant possible drug-drug interactions
- Caspofungin and other echinocandins
 Potentially important interactions with carbamazepine, phenytoin, cyclosporine, tacrolimus, sirolimus, non-nucleoside reverse transcriptase inhibitors, and rifampin
- Liposomal amphotericin B
 Concomitant therapy with cyclosporine or other nephrotoxic agents, such as aminoglycosides or vancomycin, may increase the risk of amphotericin-induced nephrotoxicity.
- Fluconazole and other azoles
 Potentially important drug-drug interactions may occur in patients receiving oral hypoglycemics, coumarin-type anticoagulants, phenytoin, cyclosporine, rifampin, theophylline, or terfenadine or astemizole.
 These drug-drug interactions are more likely with itraconazole and voriconazole than with fluconazole.
FOLLOW-UP
Patients should receive followup visit approximately 6 weeks after end of therapy and be screened for metastatic infection complications by history and physical exam
PROGNOSIS
Overall mortality for patients with hematogenously disseminated candidiasis is 40-75%, with mortality attributable to candidemia being 15-37%.
COMPLICATIONS
• Systemic inflammatory response syndrome
• Pyelonephritis
• Endophthalmitis
• Endocarditis, myocarditis, pericarditis
• Arthritis, chondritis, osteomyelitis
• Pneumonitis
• Central nervous system infection
PATIENT MONITORING
• Evaluate CBC, serum electrolytes, and serum creatinine at least twice weekly in patients on liposomal amphotericin B therapy.
• If blood cultures are positive, they should be repeated until negative.
REFERENCES
1. Benjamin DK Jr., Stoll BJ, Fanaroff AA, et al. Neonatal candidiasis among extremely low birth weight infants: Risk factors, mortality rates, and neurodevelopmental outcomes at 18 to 22 months. Pediatrics. 2006;117:84-92.
2. Golan Y, Wolf MP, Pauker SG, Wong JB, Hadley S. Empirical anti-Candida therapy among selected patients in the intensive care unit: a cost-effectiveness analysis. Ann Intern Med. 2005;143:857-869.
3. Ostrosky-Zeichner L, Pappas PG. Invasive candidiasis in the intensive care unit. Crit Care Med. 2006;34:857-863.
4. Spellberg BJ, Filler SG, Edwards JE Jr. Current treatment strategies for disseminated candidiasis. Clin Infect Dis. 2006;42:244-251.
5. Uzon D, Anaissie EJ. Predictors of outcome in cancer patients with candidemia. Am Oncol. 2000;11:1517-1521.
6. Varadakas KZ, Samonis G, Michalopoulos A, et al. Antifungal prophylaxis with azole in high-risk, surgical intensive care unit patients: A meta-analysis of randomized, placebo-controlled trials. Crit Care Med. 2006;34:1216-1224.
MISCELLANEOUS
• Other candidal infections
- Intraperitoneal infection in patients with major abdominal surgery
- Biliary tract candidiasis
- Isolated lower UTI
• See also: Candidiasis; Mucocutaneous; Vulvovaginitis; Candidal

CANDIDIASIS, MUCOCUTANEOUS

2009-01-24T05:46:00.000-08:00

CANDIDIASIS, MUCOCUTANEOUS - Susan Louisa Montauk, MD
BASICS
DESCRIPTION
A mucocutaneous disorder caused by infection with various species of Candida. Areas include
• Candida vulvovaginitis: Vaginal mucosa and/or cutaneous aspects of the vulva
• Candidal Balanitis: Glans penis
• Candidal Paronychia: Nail bed of a digit
• Oropharyngeal candidiasis: Oral cavity (thrush) and/or pharynx
• Candida esophagitis: Esophagus (commonly associated with immunosuppression)
• Gastrointestinal candidiasis: Gastritis, sometimes with ulcers, usually associated with thrush; may affect the small and large bowel
• Angular cheilitis: Fissures at mouth corners
• System(s) Affected: Gastrointestinal; Skin/Exocrine; Genitourinary
• Synonym(s): Monilia; Thrush; Yeast
ALERT
Vaginal antifungal creams and suppositories can weaken condoms and diaphragms.
Pregnancy Considerations
• No known fetal complications of maternal Candida
• Miconazole is usually the drug of choice.
GENERAL PREVENTION
• Antibiotics may potentiate candidiasis.
• Candida overgrowth is more likely with pH changes from douching, chemicals (such as spermicides), or other vaginitides.
• Moist environments are conducive to overgrowth of Candida. Cotton underwear may help deter some Candida infections.
EPIDEMIOLOGY
• Common in the United States, very common in with immunodeficiency and/or uncontrolled diabetes
• Predominant age
- Infants and seniors for thrush and cutaneous infections (infant diaper rash)
- Women of childbearing age predominate for vaginitis. It is uncommon to see prepubertal or postmenopausal yeast vaginitis because of atrophic changes in the vaginal wall.
• Predominant sex: Female > Male (because of vaginitis)
Incidence
Not well studied, but some estimate 50/100,000
Prevalence
Candida colonizes more than 1/2 of U.S. population
RISK FACTORS
• Immunosuppression (includes chronic medications such as corticosteroids and immune modulators for transplants or rheumatologic dz
• Antibacterial therapy
• Douches, chemical irritants, and other vaginitides can predispose to yeast vaginitis
• Dentures
• Birth control pills
• Hyperglycemia
Genetics
• Chronic mucocutaneous candidiasis is a heterogeneous, genetic syndrome that usually presents in childhood, but it's mode of inheritance has not been clarified.
• Family analysis has identified an isolated form of mucocutaneous candidiasis, as well as its chromosomal region, which affects nails only.
ETIOLOGY
C. albicans and, less frequently, C. tropicalis
ASSOCIATED CONDITIONS
• HIV and other leukopenias
• Diabetes mellitus
• Cancer and other immunosuppressive disorders
• Disorders that call for steroids (oral or intranasal) (1) and other immunosuppressive chemotherapy

DIAGNOSIS
NOTE: Candida is normally present, in very small amounts, in the oral cavity, gastrointestinal tract, and female genital tract.
SIGNS AND SYMPTOMS
• In children
- Oral: White, raised, painless, distinct patches within the mouth
- Perineal: Erythematous maculopapular rash with white "satellite" pustules
- Angular cheilitispainful fissures in mouth corners
• In adults: Vulvovaginal lesions; thin to thick whitish "cottage cheese-like" discharge; erythematous patches in the vagina or on the perineum; symptoms range from none to intense pruritus with "burning" irritation
• In immunocompromised hosts
- Oral lesions: White, raised, painless, distinct patches; erythematous, slightly raised patches; thick, dark-brownish coating; deep fissures
- Esophagitis: Dysphagia, odynophagia, retrosternal pain; usually associated with thrush
- Gastrointestinal symptoms: Ulcerations, pain
- Balanitis: Erythema, linear erosions, scaling
- Angular cheilitis (see "In children")
TESTS
Lab
• Potassium hydroxide 10% microscopic slide preparation (KOH prep): Breaks down epithelial cell walls; allows yeast forms to be visualized
- Best if heated
- Lack of slide identification does not rule out
- A scant number of fungal forms without symptoms does not imply pathogenesis
• Culture: Blood or Sabouraud agar is present; a positive test may be the result of normal flora.
• Drugs that may alter lab results
- Douches and spermicides
- Inadequately dosed antifungal medications
• Disorders that may alter lab results: Other vaginitides (may obscure vaginal slide findings)
Imaging
Barium swallowesophageal candidiasis may reveal a "cobblestone" appearance, fistulas or esophageal dilatation (from denervation)
Diagnostic Procedures/Surgery
• KOH prepa sample of the discharge or "coating" of the infected area or ulcer is needed.
• Esophagitis may require an endoscopic biopsy.
• HIV seropositivity plus thrush with dysphagia relieved by antifungal treatment is acceptable criteria for the diagnosis of Caplital esophagitis.
Pathological Findings
Slide preparation: Mycelia (hyphae) or pseudomycelia (pseudohyphae) yeast forms; Candida does not induce a heightened polymorphonuclear leukocyte response
DIFFERENTIAL DIAGNOSIS
• Baby formula can mimic thrush.
• Hairy leukoplakiadoes not rub off to erythematous base; usually on lateral tongue.
• Bacterial vaginitis
• Angular Cheilitis from vitamin B or iron deficit, other microbes, or edentulous "over" closure
• Symptoms of Trichomonas vaginalis that are similar to those of Candida vaginalis include
- Initial symptoms appearing postmenstrually
- Marked vulvar irritation
- Labial erythema
- External dysuria
- Vaginal tenderness
• Iron deficiency and staph infections can mimic angular cheilitis
TREATMENT
GENERAL MEASURES
Screen both well infants and patients with severe immunodeficiency at routine visits.
Diet
A few authorities say rectal colonization may be decreased with active-culture yogurt or other live lactobacillus; evidence is not yet strong.
Complementary and Alternative Medicine
Probioticscertain gut bacteria, in particular species of Lactobacillus and Bifidobacterium, may exert beneficial effects in the oral cavity by inhibiting cariogenic streptococci and Candida sp. (2)
MEDICATION (DRUGS) (3,4,5) [A,B]
First Line
Vaginal (choose one):
• Miconazole (Monistat) 2% cream: One applicator or one 100-200 mg suppository, intravaginally q.h.s.  7 days
• Clotrimazole (Gyne-Lotrimin, Mycelex): Intravaginal tablets (100 mg q.h.s.  6-7 days, 200 mg q.h.s.  3 days; 500 mg daily  1), or 1% cream (one applicator q.h.s.  6-7 days)
• Nystatin (Mycostatin, Nilstat): 100,000 U/g cream (one applicator) or 100,000 U tablets (one tablet) intravaginally 1  day  7-14 days
• Fluconazole (Diflucan): 150 mg tablet  1
Oropharangeal
• Clotrimazole (Mycelex): 10 mg troche, suck on over 20 minutes 5  day  7-14 days*
• Nystatin pastilles: 1-2 q.i.d.  7-14 days*

*Two days after disappearance of thrush
Esophagitis
Fluconazole:100 mg/d  14-21 days, load w/200 mg)
Itraconazole (Sporanox)
• Solution: 1-200 mg daily  7-14 days
• Capsules: 200 mg/d (take with food)  2-3 weeks
Gastrointestinal
Therapy not well defined
• Contraindications
- Vaginal antifungal creams and suppositories can decrease protective aspects of condoms and diaphragms.
- Any drug is contraindicated if it causes a severe allergic response or severe adverse reaction.
- Ketoconazole, itraconazole, or nystatin (if swallowed): Severe hepatotoxicity
- Amphotericin B: Renal failure
• Precautions
- Miconazole: Usually pregnancy drug of choice
- Fluconazole: Renal excreted; rare hepatotoxicity; resistance has often been noted
- Itraconazole: Doubling the dosage results in ~3-fold increase in itraconazole plasma concentrations.
• Possible interactions (rarely seen with creams, lotions, or suppositories)
- Fluconazole
 Rifampin: Decreased fluconazole concentrations
 Tolbutamide: Decreased tolbutamide concentrations
 Warfarin, phenytoin, cyclosporine: Altered metabolism; check levels
• Itraconazole: This potent cytochrome P450 3A4 isoenzyme system (CYP3A4) inhibitor may increase plasma concentrations of the many drugs metabolized by that pathway and cause serious cardiovascular events. Carefully assess all co-administered medications.
Second Line
Oropharyngeal
• Nystatin oral suspension (100,000 U/mL): Children: 5-10 mL q.i.d.  10 days directly to oral lesions); Adults: Swish "for as long as reasonable" and swallow 5-10 mL q.i.d.  14 days); prophylaxis = above dosages 2-5  day.
• Fluconazole: 100 mg/d  7-14 days (load immunocompromised patient with 200 mg)
• Itraconazole (Sporanox) Suspension: 200 mg (20 mL) daily swish and swallow  7-14 days* Capsules: 200 mg/d (take with food)  2-4 wks*
• Amphotericin B (Fungizone) oral suspension (100 mg/mL): 1 mL q.i.d., swish "for as long as reasonable" and swallow; use between meals
• Ketoconazole: 200-400 mg PO daily for 14-21 days
Esophagitis: Amphotericin B (variable dosing)
Vaginal:
• Terconazole (Terazol), particularly for recurrent cases that may involve imidazole resistance: 0.4% cream (one applicator intravaginally q.h.s.  7 days); 0.8% cream/80 mg suppositories (1 applicator or 1 suppository intravaginally q.h.s.  3 days)
• Itraconazole 200 mg capsule 1 b.i.d.  1 day
• Any of the antifungal creams or suppositories can be tried every month for a few days near menses to help curb recurrent infections.

*Two days after disappearance of thrush
FOLLOW-UP
DISPOSITION
Issues for Referral
• Patients without obvious reasons for recurrent superficial candidal infections (e.g., HIV, diabetes) may have chronic mucocutaneous candidiasis.
• GI candidiasis
PROGNOSIS
For immunocompetent individuals, a benign course and excellent prognosis are the norm. In immunosuppressed persons, Candida may become an "AIDS-defining illness" by the Centers for Disease Control and Prevention criteria and chronicity may cause much morbidity.
COMPLICATIONS
• Major complications rarely develop in immunocompetent persons.
• In immunosuppressed persons, complications depend on the severity of the immune status. Moderate immunosuppression (e.g., CD4 200-500 cells/mm3) may be associated with chronic candidiasis. In severe immunosuppression (e.g., CD4 100 cells/mm3), thrush may lead to esophagitis, then a full-systemic infection involving every organ system, particularly renal.
PATIENT MONITORING
Immunocompromised persons may benefit from regular symptom evaluation plus "routine" KOH preps during vaginal and oral exams.
REFERENCES
1. Kyrmizakis DE, et al. Acute candidiasis of the oro- and hypopharynx as the result of topical intranasal steroids administration. Rhinology. 2000;38(2):87-89.
2. Strus M. et al. The in vitro activity of vaginal Lactobacillus with probiotic properties against Candida. Infect Dis Obstet Gynecol. 2005;13(2):69-75.
3. Rex JH, et al. Practice guidelines for the treatment of candidiasis. Infectious Diseases Society of America. Clin Infect Dis. 2000;30(4):662-678. Epub 2000 Apr 20.
4. Eggimann P, Garbino J, Pittet D. Management of Candida species infections in critically ill patients. Lancet Infect Dis. 2003;3(12):772-785. Review.
5. Pappas PG, et al. Guidelines for treatment of candidiasis. Clin Infect Dis. 2004;38:161-189.
6. Friedlander SF, Rueda M, Chen BK, Caceres-Rios, HW. Fungal, protozoal, and helminthic infections. In: Schachner LA, Hansen RC, eds. Pediatric Dermatology. Edinburgh: Mosby; 2003:1093.
ADDITIONAL READING
• Betts RF, et al. A Practical Approach to Infectious Diseases. Boston, MA: Little, Brown  Co; 2002.
• Kauffman CL, Barnhill RL, eds. Fungal infections. In: Textbook of Dermatopathology, New York, NY: McGraw-Hill; 2004.
MISCELLANEOUS
• Other notes
- Transmission from person to person is rare.
- Occasionally Candida vaginitis may be sexually transmitted.
- Rarely, oral Candida leukoplakia may be precancerous.
- Skin testing is positive in 70-85% of individuals randomly checked in studies.
• See also: Candidiasis; HIV Infection; AIDS; Vulvovaginitis; Candidal

BURSITIS

2009-01-22T07:12:00.000-08:00

BURSITIS - John Herbert Stevenson, MD; Christopher Lutryzkowski, MD; Peter L. Hoth, MD
BASICS
DESCRIPTION
• A bursa is a sac that is formed or found in areas subject to friction, such as locations where tendons pass over bony landmarks. Most common sites are subdeltoid, olecranon, prepatellar, trochanteric, radiohumeral. They essentially lubricate the region with synovial fluid.
• Large bursae usually communicate with joints and are responsible for retaining the synovial fluid in place.
• Bursae are fluid-filled sacs that serve as a cushion between tendons and bones.
• Bywaters, an English rheumatologist, found at least 78 bursae symmetrically placed on each side of the body.
• System(s) Affected: Musculoskeletal
ALERT
Pediatric Considerations
Bursitis less common in the pediatric population.
GENERAL PREVENTION
• Appropriate warm-up and cool-down maneuvers, avoidance of overuse, or inadequate rest between workouts
• Range-of-motion exercises
• Maintain high level of fitness and general good health.
EPIDEMIOLOGY
Predominant age
• 15-50 years (most common in skeletally mature)
• Traumatic bursitis more likely in patients 35 years of age
Incidence
• Common
• Trochanteric pain: 1.8 per 1000 per year (6)[B]
RISK FACTORS
Individuals who engage in repetitive and vigorous training or others who suddenly increase their level of activity (e.g., "weekend warriors")
ETIOLOGY
• Bursitis may be acute or chronic.
• Many types of bursitis, including infectious, traumatic, inflammatory, and gouty
• Less often rheumatoid disease or tuberculosis as well as gout and pseudogout
ASSOCIATED CONDITIONS
• Tendinitis
• Sprains, strains
• Associated stress fractures

DIAGNOSIS
SIGNS AND SYMPTOMS
• Pain/tenderness
• Decreased range of motion of affected region (rare except at shoulder)
• Erythema if infection present
• Swelling
• Crepitus sometimes found
TESTS
ECG (if shoulder pain mimics cardiac pain)
Lab
• The following may help in differentiating soft-tissue disease from rheumatic and connective tissue disease
- CBC
- ESR
- Serum protein electrophoresis
- Rheumatoid factor
- Serum uric acid
- Phosphorus
- Alkaline phosphatase
- Blood testing for syphilis
- Joint fluid analysis and culture (when indicated)
• Drugs that may alter lab results
- ESR rate may be increased with coexistent use of methyldopa, methysergide, penicillamine, theophylline, vitamin A.
- ESR may be decreased with coexistent use of quinine, salicylates, and drugs that cause a high glucose level.
Imaging
• MRI may prove beneficial if diagnosis is unclear
• Calcific deposits may be seen on plain radiograph.
• Ultrasound (1)[B]
Diagnostic Procedures/Surgery
• Aspiration of swollen bursa and evaluation of synovial fluid
• The clinician must differentiate infected from inflammatory bursitis. Fluid analysis and culture help make the diagnosis. If the Gram stain and culture yield an infective cause, treat with appropriate antibiotics. If the etiology is inflammatory, give local care.
Pathological Findings
• Acute with early inflammation: Bursa is distended with watery or mucoid fluid.
• Infection: Purulent fluid
• Chronic
- Bursal wall is thickened, and inner surface is shaggy and trabeculated.
- The space is filled with granular, brown, inspissated blood admixed with gritty, calcific precipitations.
- Upper extremity tendonitis and bursitis are usually the result of repetitive microtrauma, probably resulting in disruption of fibers leading to pain, spasm, and disability.
DIFFERENTIAL DIAGNOSIS
• Septic arthritis
• Gout, pseudogout
• Rheumatic disorders
• Osteoarthritis
• Tendinitis, strains, and sprains
• Lyme arthritis
TREATMENT
Outpatient; refer only difficult cases.
GENERAL MEASURES
• Conservative therapy consists of rest, ice, and local care; elevation, gentle compression (often referred to as RICE therapy [rest-ice-compression-elevation]).
• Compression with Ace wrap or neoprene sleeve
• Bursa aspiration
• Corticosteroid injection if infectious etiology ruled out
• Treatment of any underlying infection
Diet
Consider changes if bursitis is directly related to obesity/crystalline deposition.
Activity
Rest and elevation of affected extremity
MEDICATION (DRUGS)
First Line
• NSAIDs or aspirin (2,4,5)[C], (9)[C]
• Antibiotic therapy if infection present; cover for staph and strep species (most common) (8)[B]
• Contraindications: Refer to manufacturer's profile of each drug.
• Precautions: Refer to manufacturer's profile of each drug.
• Significant possible interactions: Refer to manufacturer's profile of each drug.
Second Line
• Injectable corticosteroids once infectious etiology ruled out (2,4,5)[C], 3[B], (9)[B]
• Systemic steroids provide limited short-term benefit (7)[B].
SURGERY
Surgical excision in severe cases unresponsive to conservative treatments (8)[B]
FOLLOW-UP
PROGNOSIS
• Most bouts of bursitis heal without sequelae.
• Repetitive acute bouts may lead to chronic bursitis, necessitating repeated joint/bursal aspirations or eventually surgical excision of involved bursa.
COMPLICATIONS
• Septic bursitis may extend to the nearby joint.
• Acute bursitis may progress to chronic.
• Severe long-range limitation of motion
PATIENT MONITORING
• Discontinue NSAIDs as soon as possible to avoid side effects
• Some patients may require repeated injections (usually no more than 3) of a corticosteroid and lidocaine (2,4,5)[C].
REFERENCES
1. Finlay K, Friedman L. Ultrasonography of the lower extremity. Orthop Clin North Am. 2006;37(3):245-75,v.
2. Talia, Alfred H., Cardone, Dennis. Diagnostic and Therapeutic injection of the shoulder region. Am Fam Phys. 2003;67(6): 1271-1278.
3. Buchbinder R, et. al. Corticosteroid injection for shoulder pain. Cochrane Database Sys Rev. Jan. 1, 2003.
4. Cardone D, Tallia AH. Diagnostic and therapeutic injection of the elbow. Am Fam Phys. 2002;66(11):2097-3100.
5. Cardone D, Tallia AH. Diagnostic and therapeutic injection of the hip and knee. Am Fam Phys. 2003;67(10):2147-2153.
6. Lieviense A, et al. Prognosis of trochanteric pain in primary care. Br J Gen Pract. xxx;55(512): 199-204.
7. Buchbinder R, et. al. Short course prednisolone for adhesive capsulitis (frozen shoulder or stiff painful shoulder): A randomized, double blind, placebo controlled trial. Ann Rheum Dis. 2004;63(11):1460-1469.
8. Small LN. Suppurative tenosynovitis and septic bursitis. Infect Dis Clin North Am. 2005;19(4):991-1005, xi.
9. McFarland EG. Miscellaneous conditions about the elbow in athletes. Clin Sports Med. 2004;23(4):743-763, xi-xii.
MISCELLANEOUS
See also: Tendinitis

BURNS

2009-01-22T07:11:00.000-08:00

BURNS - Timothy L. Black, MD; James P. Miller, MD
BASICS
DESCRIPTION
• Tissue injuries caused by application of heat, chemicals, electricity, or irradiation to the tissue
• Extent of injury (depth of burn) is result of intensity of heat (or other exposure) and duration of exposure
- Partial thickness: 1st degree involves superficial layers of epidermis. 2nd degree involves varying degrees of epidermis (with blister formation) and part of the dermis.
- Full thickness: 3rd degree involves destruction of all skin elements with coagulation of subdermal plexus
• System(s) Affected: Endocrine/Metabolic; Skin/Exocrine
ALERT
Geriatric Considerations
• Prognosis poorer for severe burns
• Patients >60 years of age account for 11% of burns. (1)[C]
Pediatric Considerations Consider child abuse or neglect when dealing with hot water burns in children
• Observe distribution of burns.
• Pay attention to straight lines, especially if bilateral.
GENERAL PREVENTION
Skin grafts or newly epithelialized skin is highly sensitive to sun exposure and thermal extremes.
EPIDEMIOLOGY
• Predominant age: All ages
- Average age is 30 years
- 13% are infants, and 11% are >60 years of age
• Predominant sex: Males account for 70%
Incidence
Per year in US
• Total population: 1.2-2 million burns, 700,000 emergency room visits, 45,000-50,000 hospitalizations, 3,900 deaths due to burn-related complications (1,2)[C]
• In children: 250,000 burns, 15,000 hospitalizations, 1,100 deaths
• Estimated total cost of $2 billion annually for burn care in the United States (1)[C]
• 75% of burn related deaths are the result of house fires (1)[C]
RISK FACTORS
• Water heaters set too high
• Workplace exposure to chemicals, electricity, or irradiation
• Young children and elderly adults with thin skin are more susceptible to injury.
• Carelessness with burning cigarettes
• Inadequate or faulty electrical wiring
• Lack of smoke detectors
ETIOLOGY
• Open flame and hot liquid are most common (heat usually 15-45C)
• Caustic chemicals or acids (may show little signs or symptoms for the first few days)
• Electricity (may have significant injury with very little damage to overlying skin)
• Excess sun exposure
ASSOCIATED CONDITIONS
Smoke inhalation syndrome
• Occurs within 72 hours of burn
• Should be suspected in all burns occurring in an enclosed space
• Intubation, ventilation with positive end-expiratory pressure assistance

DIAGNOSIS
SIGNS AND SYMPTOMS
• 1st degree
- Erythema of involved tissue
- Skin blanches with pressure
- Skin may be tender
• 2nd degree
- Skin is red and blistered
- Skin is very tender
• 3rd degree
- Burned skin is tough and leathery
- Skin is not tender
History
• History of source of burn
• In children, check for consistency between the history and the burn's physical characteristics
Physical Exam
• Careful documentation of extent of burn and the estimated depth of burn
• Check for any signs suggestive of potential airway involvement: Singed nasal hair, facial burns, carbonaceous sputum, progressive hoarseness, or tachypnea
TESTS
• Children: Glucose (hypoglycemia may occur in children because of limited glycogen storage)
• Smoke inhalation: Arterial blood gas, carboxyhemoglobin
• Electrical burns: ECG, urine myoglobin, creatine kinase isoenzymes
Lab
• Hematocrit
• Type and cross
• Electrolytes, including blood urea nitrogen and creatinine
• Urinalysis
• Disorders that may alter lab results: Preexisting cardiac disease
Imaging
• Chest radiograph
• Xenon scan may be useful in suspected smoke inhalation.
• Other radiographs if other trauma involved
Diagnostic Procedures/Surgery
Bronchoscopy may be necessary in smoke inhalation to evaluate lower respiratory tract.
Pathological Findings
• 1st degree
- Devitalization of superficial layers of epidermis
- Congestion of intradermal vessels
• 2nd degree
- Coagulation necrosis of varying depths of epidermis
- Clefting of epidermis (blister)
- Coagulation of subdermal plexus
- Skin appendages intact
• 3rd degree
- Necrosis of all skin elements
- Coagulation of subdermal plexus
DIFFERENTIAL DIAGNOSIS
• Toxic epidermal necrolysis
• Scalded skin syndrome
TREATMENT
• Hospitalization for all serious burns
- 2nd-degree burns >10% body surface area, any 3rd-degree burn
- Burns of hands, feet, face, or perineum
- Electrical/lightning burns
- Inhalation injury
- Chemical burns
- Circumferential burn
• Transfer to burn center for (1,2,3)[C]
- 2nd- and 3rd-degree burns >10% body surface area in patients 10 years and >50 years of age
- 2nd-degree burns >20% body surface area and full thickness burns >5% BSA in any age range
- Burns of hands, feet, face, or perineum
- Electrical/lightning burns
- Inhalation injury
- Chemical burns
- Circumferential burn
- Chemical burns with threat of functional impairment
PRE-HOSPITAL (1)[C]
• Remove patient from source of the burn
• Extinguish and remove all burning clothing
• Remove all rings, watches, and jewelry
• Room-temperature water may be poured onto burn but only in the 1st 15 minutes following burn exposure
• Wrap patient to prevent hypothermia
• All patients should receive 100% O2 by face mask
GENERAL MEASURES
• Based on depth of burns and accurate estimate of total body surface area involved (rule of nines)
• Rule of nines (1)[C]
- Each upper extremity: Adult and child 9%
- Each lower extremity: Adult 18%; child 14%
- Anterior trunk: Adult and child 18%
- Posterior trunk: Adult and child 18%
- Head and neck: Adult 10%; child 18%
• Quick estimate (for smaller burns): The surface area of the patient's hand is ~1% of the body surface area.
• Tetanus prophylaxis (if not current)
• Remove all rings, watches, and other items from injured extremities to avoid tourniquet effect.
• Remove clothing and cover all burned areas with dry sheets.
• Flush area of chemical burn (for ~2 hours)
• 100% oxygen administration for all major burns; consider early intubation
• Do not apply ice to burn site.
• Nasogastric tube (high risk of paralytic ileus)
• Foley catheter
• Pain relief
- IV meperidine (Demerol), morphine, or methadone for severe pain
- Oral analgesics, such as acetaminophen (Tylenol) with codeine, acetaminophen with oxycodone (Percocet), or acetaminophen with hydrocodone (Lortab) for moderate pain
• ECG monitoring in 1st 24 hours following electrical burn
• Whirlpool hydrotherapy followed by silver sulfadiazine (Silvadene) occlusive dressings in severe burns
• Once- or twice-a-day cleansing with dressing changes
• Epilock or Elasto-Gel may be used as dressing in selected patients (especially useful for outpatient treatment of minor burns)
• Burn fluid resuscitation (1,2,3)[C]
- Calculate fluid resuscitation from time of burn, not from time treatment begins
- 2-4 mL Ringer's lactate  body weight (kg)  % body surface area burn (1/2 given in 1st 8 hours, 1/4 in 2nd 8 hours, and 1/4 in 3rd 8 hours). In children, this is given in addition to maintenance fluids and is adjusted according to urine output and vital signs.
- Colloid solutions are not recommended during the 1st 12-24 hours of resuscitation (1,2)[C], (4)[A]
• Other: Use of biological membranes or skin substitutes may be indicated for burn coverage.
Diet
• High-protein, high-calorie diet when bowel function resumes
• Nasogastric tube feedings may be required in early postburn period
• Total parenteral nutrition if NPO expected for >5 days
Activity
Early mobilization is the goal.
MEDICATION (DRUGS)
First Line
• Morphine small frequent IV doses (0.1 mg/kg/dose in children; 2.5-20 mg q2-6h in adults)
• Silver sulfadiazine (Silvadene) topically to burn site (can cause leukopenia)
• Electrical burn with myoglobinuria will require alkalinization of urine and mannitol
• No indication for prophylactic antibiotics.
• Consider H2 blockers (cimetidine, ranitidine, famotidine, or nizatidine) for stress ulcer prophylaxis in severely burned patients.
• Contraindications
- Specific drug allergies
• Precautions
- Be alert for respiratory depression with narcotics.
• Significant possible interactions
- Refer to manufacturer's profile for each drug.
Second Line
• Mafenide (Sulfamylon)full-thickness burn (caution: Metabolic acidosis)
• Silver nitrate 0.5% (messy, leaches electrolytes from burn, and causes water toxicity)
• Povidone-iodine (Betadine) may result in iodine absorption from burn, "tan eschar." Makes debridement more difficult.
• Travaseenzymatic debridement
SURGERY
• Escharotomy may be necessary in constricting circumferential burns of extremities or chest.
• Tangential excision with split-thickness skin grafts
FOLLOW-UP
PROGNOSIS
• 1st-degree burn: Complete resolution
• 2nd-degree burn: Epithelialization in 10-14 days (deep 2nd-degree burns will probably require skin graft)
• 3rd-degree burn: No potential for re-epithelialization, skin graft required
• Length of hospital stay and need for ICU care depend on extent of burn, smoke inhalation, and age
• A 50% survival rate can be expected with a 62% burn in ages 0-14 years, 63% burn in ages 15-40 years, 38% burn in age 40-65 years, 25% burn in patients >65 years (1,2,3)[C]
• 90% of survivors can be expected to return to an occupation as remunerative as their preburn employment.
COMPLICATIONS
• Gastroduodenal ulceration (Curling ulcer)
• Marjolin ulcersquamous cell carcinoma developing in old burn site
• Burn wound sepsisusually gram-negative organisms
• Pneumonia
• Decreased mobility with possibility of future flexion contractures
• Hypertrophic scarring common with burns
PATIENT MONITORING
According to extent of burn and treatment
REFERENCES
1. Teague H, Sweneki SA, Tang A. The burned patient: Assessment, diagnosis, and management in the ED. Trauma Reports. 2005;6:1-12.
2. Townsend C, Beauchamp RD, Evers BM, et al. eds. Sabiston Textbook of Surgery 17 ed. Philadelphia, PA: Elsevier Saunders, 2006.
3. Gillespie RW, Dimik AR, Hallberg PW. Advanced Burn Life Support Course Provider's Manual. Lincoln, NE: Nebraska Burn Institute; 1990.
4. Roberts I, Alderson P, Bunn F, et al. Colloids versus crystalloids for fluid resuscitation in critically ill patients (Review). Cochrane Database Sys Rev. 2006; Vol 1.

BRUCELLOSIS

2009-01-22T07:10:00.000-08:00

BRUCELLOSIS - Nancy Snapp, MD, MPH
BASICS
DESCRIPTION
• Systemic bacterial infection caused by Brucella species in infected animal products, or vaccine
• Incubation period usually 5-60 days, but highly variable and may be several months
• Characterized by intermittent or irregular fevers, with symptoms ranging from subclinical disease to infection of almost any organ system
• Bone and joint involvement common
• May be chronic or recurrent
• System(s) Affected: Cardiovascular; Endocrine/Metabolic; Gastrointestinal; Musculoskeletal; Nervous; Pulmonary; Renal/Urologic; Skin/Exocrine
• Synonym(s): Undulant fever; Malta fever
ALERT
Pediatric Considerations
May be mild, subclinical
Pregnancy Considerations
High rates of miscarriage or abortion (can occur in subclinical cases). Early antibiotic treatment is preventive.
GENERAL PREVENTION
• Avoid infected dairy products.
• For occupational exposure, use caution, animal vaccination, protective goggles, protective gloves. There is a possibility of future human vaccine.
• Postexposure prophylaxis same as treatment in large-scale exposure such as bioterrorism
• Susceptible to heat, disinfectant, but can survive in dust, soil, or water for weeks
EPIDEMIOLOGY
• Predominant age: All ages, but especially ages 20-60 years (occupational exposure), sometimes children (milk-related outbreaks)
• Predominant sex:
- Male > Female (occupational exposure)
- Female  Male (milk exposure)
Incidence
~100 per year (0.34/100,000), but probably underreported (1,2)
Prevalence
• Common in developing countries; consider in immigrants
• Highest rates in Hispanic population, along US-Mexico border
• Considered a potential biological terror agent in aerosolized form
• Reportable in all states except Nevada
RISK FACTORS
• In the US, from occupational exposure to infected animals (especially cattle, sheep) veterinarians, meat processors, farm workers who may experience accidental exposure to vaccine.
• Consumer exposure to unpasteurized milk products, cheese, especially in Hispanics along US-Mexico border
• Exposure while traveling in countries where endemic (Mediterranean, Middle East, North and East Africa, Central Asia, India, Mexico, and Central and South America)
• Worse in chronically ill, immunosuppressed, and malnourished
• Iron deficiency increases susceptibility
Genetics
• Some evidence for intrauterine transmission
• Some complications may have genetic predisposition (2)
ETIOLOGY
• Brucella ingestion from tissue or milk
• Worst disease: B. melitensis, B. suis; also B. canis, B. abortus. Enters through mucous membrane or broken skin; occasionally inhaled.
• Facultative intracellular parasite
• Person-to-person transmission rare; sexual, vertical, and possibly breast milk; case report of neonatal brucellosis from a blood transfusion
• Potential air-borne biologic weapon

DIAGNOSIS
SIGNS AND SYMPTOMS
• Fever (may be undulant, increased in afternoon and evening, maximum 101-104F daily); weakness; headache; sweating; chills; generalized aching; arthralgia (90%) (2)[A]
• Also common: Weight loss, depression, irritability, hepatosplenomegaly (20-30%)
• Hepatic dysfunction (abnormal liver function test): 30-60%
• Gastrointestinal symptoms (unusual)
• Lymphadenopathy, especially cervical, inguinal (12-21%)
• Orchitis, epididymitis (normal urinalysis) (2-40%)
• Nephritis, prostatitis (rare)
• Cystitis
• Pulmonarycough or other pulmonary symptoms; radiograph may be normal (15-25%)
• Cutaneousmany transient, nonspecific rashes have been described; also, purpura from thrombopenia (5%)
• Visual disturbances, eye pain
• Chronic fatigue syndrome and various neuropsychiatric symptoms described. Relationship is unclear.
• Also localized supurative infections (see "Complications")
• Malodorous perspiration (2)
History
Exposure
TESTS
Echocardiogram, depending on location
Lab
• Isolation of organism from blood, discharge, bone, or other tissue (3)[C]
- Fastidious and slow growing
- Watch for 3-4 weeks, with periodic subcultures
- Automated systems shorten time, but not all recognize brucellosis.
- Polymerase chain reaction (PCR) accurate, including nonblood samples, but not available in most clinical labs (3)[C]
- Skin tests not standardized, not recommended for diagnosis
• Acute illness: Blood culture positive 70%, bone marrow 90%
• May have thrombocytopenia, disseminated intravascular coagulation; granulopenia, lymphopenia, lymphocytosis. 30-60% with abnormal liver function test. Up to 70% may have normal labs.
• Serology: Use at least 2 tests to confirm (4)[C]
- Brucella standard tube agglutination paired sera, >1:160 or 4 rise (cheapest)
- Easy, accurate, and rapid dipstick for IgM now exists for developing countries
• More effective enzyme-linked immunosorbent assay (ELISA), indirect fluorescent antibody test, Coombs tests, immunocapture-agglutination (Brucellacapt). With ELISA, IgM, IgG, or IgA may be present at low levels >1 year even if treated
• IgM increased initially for several weeks, declines by 3 months
• IgG begins to rise in 2 weeks, may stay up (low levels) >1 year if treated or not treated (though IgM increase may be lower or gone by 6 months if treated, can also persist >1 year at low levels). IgG titer rises again with reinfection or reactivation. IgG and IgA titer >1:160 at 1 year implies ongoing disease. (4)[C]
• New research: Gene cloning and amplification for discriminatory markers detection and strain differences; PCR-ELISA
• Drugs that may alter lab results
- None
• Disorders that may alter lab results
- Serologic cross-reaction with F. tularensis, Yersinia enterocolitica, V. cholerae, or vaccinated patients
- Has been misdiagnosed in culture as Moraxella phenylpyruvica
Imaging
• Bone scan, CT, depending on location
• Chest radiographpleural effusion, lung cavitation
• Joint radiographs frequently normal, requiring scan or MRI
Diagnostic Procedures/Surgery
Biopsy, aspiration, depending on location
Pathological Findings
• Facultative intracellular Gram-negative coccobacillus; can survive inside phagocytic cells, circulate to regional lymph nodes, and into circulation
• Variable tissue reaction depending on site, organisms. Causes local microabscesses; noncaseating granulomas; (1) possibly some immune reaction in arthritis, including elevated C3, C4; antinuclear antibody, and rheumatoid factor.
DIFFERENTIAL DIAGNOSIS
• Many nonspecific systemic febrile illnesses; a great mimic
• Tularemia
• Psittacosis
• Rickettsial disease
• Tuberculosis
• Visceral leishmaniasis
• Other disease of infected organs
• HIV infection
TREATMENT
• Outpatient in mild cases, hospitalization in severe illness
• Cardiac care unit for patients with complicating cardiac disease
GENERAL MEASURES
• Supportive care
• In milk-related or occupational outbreak, look for other cases.
Diet
• No special diet
• May need to provide supplemental foods, such as milk shakes, to counter weight loss
Activity
Bed rest during febrile periods and restricted activity in acute cases
Nursing
Patient comfort, education
IV Fluids
If cardiac complications
MEDICATION (DRUGS)
First Line
• Optimal therapy includes 2 drugs, at least 1 with good intracellular penetration. In some cases, 3 drugs may give a better long-term cure.
• Longer courses (months) may improve relapse rate in complicated disease.
• Rifampin 600-900 mg and doxycycline 200 mg given together every day for at least 6 weeks (possibly for several months with severe complications); 5-10% relapse rate, not related to drug resistanceuse same drugs for relapse. Usual cause is localized sequestration of organisms or noncompliance with medication (5)[C].
• Steroids in Herxheimer reaction, severe illness, and pancytopenia
• Contraindications
- Avoid doxycycline in children and pregnant women (affects bone).
• Precautions:
- May get Herxheimer reaction when therapy initiated
• Significant possible interactions:
- Rifampin is a potent inducer for the hepatic P450 enzyme system, and may increase metabolism of many drugs metabolized by the liver.
- Doxycycline: Antacids, anticoagulants, barbiturates, carbamazepine, hydantoins, cimetidine, digoxin, insulin, iron salts, lithium, methoxyflurane, oral contraceptives, penicillins, sodium bicarbonate
Second Line
• In recent studies, ciprofloxacin 1 g daily and rifampin 600 mg/d for 30 days as effective as rifampin/doxycycline for 4-5 weeks (2,6)[A]
• Doxycycline PO b.i.d. and streptomycin by injectionvery effective (streptomycin currently not available in the US except by special request from Centers for Disease Control and Prevention); slightly more effective than doxycycline/rifampin, especially with spondylitis, but more toxic and less convenient (5)
• In children and pregnant women, rifampin 15 mg/kg for 4-5 weeks plus cotrimoxazole for 6 weeks or gentamicin for 7 days or netilmicin 5-6 mg/kg IM. Significant cotrimoxazole resistance in some countries (1,6)[C]
• Ofloxacin plus rifampin effective in recent study
• Sensitivities frequently don't reflect in vivo action (2)[C]
SURGERY
Specific complications may require surgical drainage or valve replacement (endocarditis).
FOLLOW-UP
PROGNOSIS
• Untreated case fatality 2%
• Most cases resolve with treatment in 2-3 weeks in acute uncomplicated cases, but at least 6 weeks treatment recommended
COMPLICATIONS
• Relapse rate overall: 5-10% (6)[C]
• Complications present 10-15% (4)[C]
• Localized suppurative infectionsosteo-articular (20-85%). Includes arthritis (possibly also immune effect), bursitis, tenosynovitis, osteomyelitis, sacroiliitis, vertebral or paraspinous abscess
• Endocarditisrare, but main cause of death in brucellosis
• Thrombophlebitis
• Neuro-brucellosismost are meningeal. Also peripheral neuritis (usually single; bilateral is possible), encephalitis, myelitis, radiculopathy. Possibly neuropsychiatric symptoms
• Intrinsic ocular lesionsuveitis, retinal thrombophlebitis, nummular keratitis
• Pneumonitis with pleural effusion
• Hepatitis
• Cholecystitis
• Chronic infection. Persistent (>1 year) signs of infection, elevated titers, occasional bacteria in blood or tissue. Chronic fatigue syndrome with everything negative is controversial.
PATIENT MONITORING
• Check serology at 6 months and 1 year for chronic disease (difficult to evaluate if continuing exposure).
• Investigate any evidence of complication or recurrence.
• PCR recently shown to be sensitive and specific for monitoring treatment relapse
REFERENCES
1. Sauret J, Vilissova N. Human brucellosis. J Amer Board Fam Pract. 2002;15:401-406.
2. Pappas, Georgios, et al. Brucellosis. N Eng J Med. 2005;352(22);2325-2336.
3. Al Dahouk S, Tomaso H, et al. Laboratory-based diagnosis of brucellosisa review of the literature. Part I: Techniques for direct detection and identification of Brucella spp. Clin Lab. 2003;49:487-505.
4. Al Dahouk S, Tomaso H, et al. Laboratory-based diagnosis of brucellosisa review of the literature. Part II: Serological tests for brucellosis. Clin Lab. 2003;49:577-589.
5. Montejo JM, et al. Open randomized therapeutic trial of six antimicrobial regimens in brucellosis. Clin Infect Dis. 1993;16:671-676.
6. Pappas, Georgios, et al. New approaches to the antibiotic treatment of brucellosis. Intl J Antimicrob Ag. 2005;26(2);101-105.
MISCELLANEOUS
See also: Abortion, Spontaneous; Chronic Fatigue Syndrome; Thrombosis, Deep Vein (DVT)

BULIMIA NERVOSA

2009-01-22T07:09:00.001-08:00

BULIMIA NERVOSA - Jeffrey L. Goodie, PhD; Pamela Williams, MD
BASICS
DESCRIPTION
• A pattern of uncontrolled eating during discrete periods followed by compensatory behaviors.
• System(s) Affected: Oropharyngeal, Endocrine/Metabolic, Gastrointestinal, Dermatologic, Cardiovascular, Nervous
GENERAL PREVENTION
• Encourage realistic weight management strategies and attitudes
• Moderate overly high self-expectations
• Decrease anxiety/depressive symptoms
• Improve stress management
EPIDEMIOLOGY
• Predominant age: Adolescents and young adults; mean age of onset: 18-19
• Predominant sex: Female > Male (10:1 to 20:1)
Incidence
28.8 women, 0.8 men per 100,000 per year
Prevalence
• 1-2% in women 16-35 years old
• 0.1% in young men
RISK FACTORS
• Female gender
• History of obesity and dieting
• Body dissatisfaction
• Critical comments by family or others about weight, body shape, or eating
• Severe life stressor; achievement pressure; competition stressors
• Low self-esteem
• Perceived pressure to be thin
• Perfectionistic or obsessional thinking
• History of anorexia nervosa
• Environment that stresses thinness or physical fitness (e.g., armed forces, ballet, cheerleaders, gymnastics, or models)
• Family history of substance abuse, affective disorders, eating disorder, or obesity
• Type I diabetes
• Poor impulse control, alcohol misuse
• Sexual abuse is not causally related to bulimia.
ETIOLOGY
Combination of biological, genetic psychological, environmental, and social factors. Unique contribution of any specific factor remains unclear.
ASSOCIATED CONDITIONS
• Major depression and dysthymia
• Anxiety disorders
• Substance abuse/dependence
• Bipolar disorder
• Obsessive-compulsive disorder
• Schizophrenic disorder
• Borderline personality disorder

DIAGNOSIS
DSM IV TR criteria
• Recurrent episodes of binge eating (2 times per week for 3 months)
- Eating in a discrete period of time more than most people would eat during that time
- Perceived lack of control during binge
• Recurrent inappropriate compensatory behavior (2 times per week for 3 months)
• Purging and nonpurging subtypes
- Purging: Often by self-induced vomiting, laxatives, diuretics
- Nonpurging: Binges followed by sharply restricted diet and/or vigorous exercise
• Body shape and weight significantly affect self-evaluation.
SIGNS AND SYMPTOMS
• Unhappiness and/or preoccupation with weight and diet attempts
• Pattern of restricting diet, binge eating, and purging behaviors
- Binge is context specific; amount can vary
 Average binge between 1,000-2,000 kcals2
- Vomiting (often with little effort)
- Vigorous aerobic exercise
- Distress/shame related to loss of control
• Requesting weight loss help
• Menstrual disturbance
• Fatigue and lethargy
• Abdominal pain, bloating, constipation, diarrhea, irritable bowel syndrome, rectal prolapse
• Enamel erosion, parotid swelling, sore throat
• Onset may be stress related
• Mildly underweight to overweight
• Frequent fluctuations in weight
• Diet pill, diuretic, laxative, ipecac, and thyroid medication use/abuse
• Omission/underdosing insulin in diabetes patients
• Depressed mood and self-depreciation following the binges
• Relief and increased ability to concentrate following the purges
History
Corroborate with parent/relative
Physical Exam
• Often normal
• Eroded tooth enamel
• Asymptomatic, non-inflammatory salivary gland (parotid) enlargement
• Calluses, abrasions, bruising on hand, thumb
• Peripheral edema
TESTS
Lab
• All results may be within normal limits and are not necessary for diagnosis.
• Hypokalemia, hypochloremia
• Hypomagnesemia, hyponatraemia, hypocalcaemia, hypophosphataemia
• Alkalosis
• Leukopenia
• Elevated blood urea nitrogen
• Elevated basal serum prolactin
• Mild elevation in serum amylase
Imaging
Not indicated
Diagnostic Procedures/Surgery
Psychological self-report screening
• Eating Attitudes Test
• Eating Disorder Inventory
• Eating Disorder Screen for Primary Care
• Bulimia TestRevised
• Bulimia Investigatory Test Edinburgh
• SCOFF (sick, control, one, fat, food) questionnaire
Pathological Findings
• Esophagitis
• Pseudo-Bartter syndrome
• Acute pancreatitis
• Cardiomyopathy and muscle weakness due to ipecac abuse
DIFFERENTIAL DIAGNOSIS
• Anorexia, binge eating/purging type
• Major depressive disorder
• Psychogenic vomiting
• Hypothalamic brain tumor
• Epileptic equivalent seizures
• Kleine-Levin syndrome
• Body dysmorphic disorder
• Borderline personality disorder
TREATMENT
GENERAL MEASURES
• Most patients can be treated as outpatients.
• Outpatient
- Build trust, increase motivation for change
- Assess psychological and nutritional status
- Consider evidence-based self-help program
- Cognitive behavioral therapy (1-3)[A]
 Involve patient in establishing target goals
 Use self-monitoring techniques of food intake, frequency of binges/purges, related antecedents, consequences, and thoughts
 Self-monitoring weight once per week along with emotional and thought reactions
 Educate about ineffectiveness of purging for weight control and adverse outcomes
 Establish prescribed eating plan to develop regular eating habits; realistic weight goal
 Gradually introduce feared foods into diet
 Problem solve how to cope with triggers
 Address calories, weight, and purging ruminations
 Challenge fear of loss of control
 Establish relapse prevention plan
- Gradual laxative withdrawal
- Interpersonal therapy (1,3)[C]
- Family therapy for adolescents
- Nutritional education, relaxation techniques
- After vomiting, avoid brushing teeth and consider using non-acidic mouthwash (1)
- Limiting acidic foods, beverages to meal time
• Inpatient
- If possible, admit to eating disorders unit
- Supervised meals and bathroom privileges
- Monitor weight and physical activity
- Monitor electrolytes
- See outpatient recommendations
- Gradually shift control to patients as they demonstrate responsibility
Diet
• Balanced diet, normal eating pattern
• Reintroduce feared foods
Activity
• Monitor excess activity
• Encourage enjoyable activities
MEDICATION (DRUGS)
First Line
• Selective serotonin reuptake inhibitors (SSRIs), particularly fluoxetine (Prozac) at 60 mg, are effective in reducing symptoms with relatively few side effects. Higher doses than standard doses for depression are often needed. (1)[B]
• Tricyclic antidepressants (amitriptyline, desipramine, and imipramine) and monoamine oxidase inhibitors: Phenelzine (Nardil) 60-90 mg/d have been shown to decrease binging and vomiting. (1,2)[C] Patients with atypical depression may respond to monoamine oxidase inhibitors and not SSRIs.
• Augment with buspirone (BuSpar) if desired. To prevent relapse, maintain antidepressant medication at full therapeutic dose for at least 1 year.
• Note: Misrepresentation and non-adherence may be more likely in this population.
• Contraindications: Hypersensitivity
• Precautions
- Serious toxicity following overdose is common.
- Patients may vomit medications.
• Significant possible interactions
- Monoamine Oxidase Inhibitor should not be combined with SSRI or tricyclic medication
- Lithium and tricyclic medication can be lethal when administered to hypokalemic patients.
Second Line
• Ondansetron (Zofran) 4-8 mg t.i.d. between meals can help prevent vomiting.
• Psyllium (Metamucil) preparations 1 tbs qhs with glass of water, can prevent constipation during laxative withdrawal.
FOLLOW-UP
DISPOSITION
Admission Criteria
Hospitalize if severe malnutrition, dehydration, electrolyte disturbances, cardiac dysrhythmia, uncontrolled binging and purging, psychiatric emergency, or failed outpatient treatment.
PROGNOSIS
• Following effective treatment
- 50% asymptomatic after 2-10 years, 30% remissions, relapses, or subclinical behaviors; 20% no significant change
• Untreated
- Likely to remain chronic/relapsing problem
• Greater weight fluctuations, other impulsive behaviors, and personality disorder diagnoses may predict poor prognosis.
COMPLICATIONS
• Suicide
• Drug and alcohol abuse
• Infarction and perforation of the stomach
- Gastric dilatation
- Mallory-Weiss tears
- Spontaneous pneumomediastinum
• Potassium depletion; cardiac arrhythmia; cardiac arrest
• Maternal and fetal problems if pregnant
- Binging/purging behaviors may change with pregnancy
PATIENT MONITORING
• Binge-purge activity, including antecedents and consequences
• Level of exercise activity
• Self-esteem, comfort with body and self
• Ruminations and depression
• Repeat any abnormal lab values weekly or monthly until stable
REFERENCES
1. NICE. Eating disorderscore interventions in the treatment of anorexia nervosa, bulimia nervosa, and related eating disorders. NICE Clinical Guideline no 9. London: NICE, 2004: Available at: http://www.nice.org.uk. Accessed January 20, 2006.
2. Bacaltchuk J, Hay P, Trefiglio R. Antidepressants versus psychological treatments and their combination for bulimia nervosa. Cochrane Database Sys Rev. 2001(4):CD003385.
3. Hay PJ, Bacaltchuk J. Psychotherapy for bulimia nervosa and binging. Cochrane Database Syst Rev. 2003(1):CD000562.
4. Fairburn CG, Harrison PJ. Eating disorders. Lancet. 2003;361:407-416.
5. Mehler, PS. Bulmia nervosa. N Engl J Med. 2003;349:875-881.
ADDITIONAL READING
McCabe RE, McFarlane TL, Olmstead MP. Overcoming bulimia: your comprehensive, step-by-step guide to recovery. 2003; Oakland, CA: New Harbinger.
MISCELLANEOUS
See also: Anorexia nervosa; Hyperkalemia; Laxative abuse; Salivary gland tumors

BRONCHITIS, ACUTE

2009-01-22T07:09:00.000-08:00

BRONCHITIS, ACUTE - Alan J. Cropp, MD, FCCP
BASICS
DESCRIPTION
• Inflammation of trachea, bronchi, and bronchioles resulting from a respiratory tract infection or chemical irritant (1,2)
• Cough is the predominant symptom (3,4)
• Generally self-limited with complete healing and full return of function
• Most infections viral if no underlying cardiopulmonary disease is present
• Synonym(s): Tracheobronchitis
ALERT
Geriatric Considerations
Can be a serious illness in this age group, particularly if part of influenza or with underlying chronic obstructive pulmonary disease (COPD)
Pediatric Considerations
• In this age group, usually occurs in association with other conditions of upper and lower respiratory tract (trachea usually involved)
• Some children seem to be more susceptible than others (if repeated attacks, child should be evaluated for anomalies of the respiratory tract including immune deficiencies or for chronic asthma).
• If acute bronchitis is caused by respiratory syncytial virus, it may be fatal.
GENERAL PREVENTION
• Avoid smoking
• Control underlying risk factors (asthma, sinusitis, and reflux)
• Avoid exposure especially daycare
• Vaccinations, specifically pneumovax, influenza
EPIDEMIOLOGY
• Predominant age: All ages
• Predominant sex: Male = Female
Incidence
~5% of adults per year
Prevalence
Results in 10-12 million office visits per year
RISK FACTORS
• Chronic bronchopulmonary diseases
• Chronic sinusitis
• Bronchopulmonary allergy
• Hypertrophied tonsils and adenoids in children
• Immunosuppression
• Air pollutants
• Elderly
• Infants
• Smoking
• 2nd-hand smoke
• Alcoholism
• Gastroesophageal reflux disease (GERD)
• Tracheostomy
• Environmental changes
• Immunoglobulin deficiency
• HIV
Genetics
No known genetic pattern
PATHOPHYSIOLOGY
Acute bronchitis causes an injury to the epithelial surfaces resulting in an increase in mucous production (2)
ETIOLOGY
• Adenovirus
• Influenza A and B
• Parainfluenza
• Chlamydia pneumoniae (TWAR agent)
• Bordetella pertussis
• Respiratory syncytial virus
• Coxsackievirus
• Herpes simplex
• Haemophilus influenzae
• Possibly fungi
• Mycoplasma
• Secondary bacterial infection as part of an acute upper respiratory infection
• Streptococcus pneumoniae
• Moraxella catarrhalis
• Mycobacterium tuberculosis
• Rhinovirus
• Coronavirus (types 1-3)
• Chemical irritants
ASSOCIATED CONDITIONS
• Asthma
• Epiglottitis; rare but can be rapidly fatal
• Coryza
• Pharyngitis
• Croup
• Influenza
• Smoking
• Pneumonia
• Emphysema
• Sinusitis
• Bronchial obstruction
• GERD

DIAGNOSIS
PRE HOSPITAL
Usually treated as outpatient unless comorbidity exists
SIGNS AND SYMPTOMS
• Cough
• Fever
• Fatigue
• Aching (i.e., myalgia)
• Hemoptysis
• Chest burning
• Dyspnea (sometimes)
History
• Preceding respiratory tract infection, such as a common cold with coryza, malaise, chills, slight fever, sore throat, back and muscle pain
• Cough, initially dry and unproductive, then productive; later, mucopurulent sputum, which may indicate secondary infection
Physical Exam
• Rales, rhonchi, wheezing
• No evidence of pulmonary consolidation
• Pharynx injected
• Fever
• Tachypnea
TESTS
• Arterial blood gases: Hypoxemia (rarely)
• Leukocytosis
• Sputum culture/gram stain
• Viral titers
• Mycoplasma titers
• Pulmonary function tests (seldom needed during acute stages): Increased residual volume, decreased maximal expiratory rate (2)
Imaging
Chest radiograph
• Lungs normal if uncomplicated
• Helps rule out other diseases (pneumonia) or complications
Diagnostic Procedures/Surgery
Rarely indicated
DIFFERENTIAL DIAGNOSIS
• Asthma
• Allergy
• Eosinphilic Pneumonitis
• Influenza
• Bronchopneumonia
• Bronchiectasis
• Acute sinusitis
• Aspiration
• Cystic fibrosis
• Reactive airways dysfunction syndrome
• Bacterial tracheitis
• Retained foreign body
• Inhalation injury
• Heart failure
• Bronchogenic carcinoma
• GERD
TREATMENT
PRE-HOSPITAL
Aerosolized bronchodilator may be helpful if patient has bronchospasm
STABILIZATION
• Outpatient unless elderly or complicated by severe underlying disease
• May require supplemental oxygen in selected cases
GENERAL MEASURES
• Rest
• Steam inhalations
• Vaporizers
• Antibiotics if complicated by comorbidity (e.g., COPD, severe asthma, etc.) (overused in United States)
• Adequate hydration
• Stop smoking
• Treat associated illnesses (e.g., GERD)
• Antitussives
Diet
Increased fluids (3-4 L/d) while febrile
Activity
Rest until fever subsides.
Nursing
Ensure patient comfort and moniter for signs of deterioration, especially if underlying lung disease exists.
SPECIAL THERAPY
IV Fluids
May be helpful if patient is dehydrated.
Complementary and Alternative Medicine
Throat lozenges for pharyngitis
MEDICATION (DRUGS)
First Line
• Meta-analysis has demonstrated the lack of efficacy of antibiotics in uncomplicated acute bronchitis. (5)[A]
• Amantadine or rimantadine therapy if influenza A suspected; most effective if started within 24-48 hours of development of symptoms (also consider tamiflu or relenza)
• Decongestants if accompanied by sinus condition (1)[B]
• Antipyretic analgesic such as aspirin or ibuprofen
• Antibiotics (Amoxicillin 500 mg q8h or TMP/SMX DS b.i.d) for more severe symptoms (high fever persists, concomitant COPD, purulent discharge)
• Amoxicillin: 500 mg q8h or trimethoprim sulfamethoxazole DS q12h for routine infection
• Doxycycline: 100 mg/d for 10 days if Moraxella, Chlamydia, or Mycoplasma suspected
• Clarithromycin (Biaxin): 500 mg q12h or azithromycin (Zithromax) Z-pack for PCN or sulfa allergy or mycoplasma infection
• Quinolone for more serious infection or other antibiotic failure or in elderly or multiple comorbidities
• Cough suppressant for troublesome cough (not with COPD); guaifenesin with codeine or dextromethorphan (4)[A]
• Inhaled beta agonist (e.g., albuterol) or in combination with steroids (2)[B]
• Consider steroids for bronchospasm
• Watch for theophylline toxicity with macrolides and quinolones. Macrolides also interfere with oral contraceptives.
• Significant possible interactions: Refer to the manufacturer's literature.
FOLLOW-UP
DISPOSITION
Usually a self-limited disease not requiring follow-up
Admission Criteria
Severe exacerbation of underlying disease
Discharge Criteria
Improvement in symptoms
Issues for Referral
Complications such as pneumonia
PROGNOSIS
• Usual: Complete healing with good return of function
• Can be serious in the elderly or debilitated
• Cough may persist for several weeks after an initial improvement (2)
• Postbronchitic reactive airways disease (rare)
• Bronchiolitis obliterans and organizing pneumonia (rare)
COMPLICATIONS
• Bronchopneumonia
• Acute respiratory failure
• Bronchiectasis
• Chronic cough
• Hemoptysis
• Superinfection
PATIENT MONITORING
• Oximetry until no longer hypoxemic
• Recheck for chronicity
REFERENCES
1. Flaherty K, Saint S, Fenfrick AM, Martinez F. The spectrum of acute bronchitis. Postgrad Med. 2001;109:39-47.
2. Knutson D, Braun C. Diagnosis and management of acute bronchitis. Am Fam Physician. 2002;65:2039-2044.
3. Snow V, Mottur-Pilson C, Gonzales R. Principles of appropriate antibiotic use for treatment of acute bronchitis in adults. Ann Intern Med. 2001;134:518-20.
4. Gonzales R, Bartlett J, Besser R, et al. Principles of appropriate antibiotic use for treatment of uncomplicated acute bronchitis: Background. Ann Intern Med. 2001;134:521-529.
5. MacKay DN: Treatment of acute bronchitis in adults without underlying lung disease. J Gen Intern Med. 1996;11(9):557-562.
MISCELLANEOUS
See also: Asthma; Chronic Obstructive Pulmonary Disease; and Emphysema

BRONCHIOLITIS

2009-01-22T07:08:00.000-08:00

BRONCHIOLITIS - Dennis E. Hughes, DO
BASICS
DESCRIPTION
• Inflammation and obstruction of small airways and reactive airways. May be seasonal (winter and spring) and often occurs in epidemics
• Usual course: Insidious; acute; progressive
ALERT
Pediatric Considerations
Most common in infants
GENERAL PREVENTION
• Hand washing
• Contact isolation of infected babies
• Persons with colds should keep contacts with infants to a minimum
• Palivizumab (Synagis), a monoclonal product, administered monthly, October-May, 15 mg/kg IM; used for respiratory syncytial virus prevention in high-risk patients
- 28-32 week gestation and 6 months old; once begun, continue through end of season regardless of age attained
- 28 weeks gestation and 12 months old
- Moderately severe bronchopulmonary dysplasia and up to 2 years old
- Hemodynamically significant congenital heart disease (until age 6 months)
• Respiratory syncytial virus immune globulin, a human blood product, can also be used in at-risk patients. Monthly infusions of 750 mg/kg, October-May (1).
EPIDEMIOLOGY
• Leading cause of hospitalizations in infants and children.
• Predominant age: Newborn to 2 years (peak age 6 months). Neonates are not protected despite transfer of maternal antibody.
• Predominant sex: Male > Female
Incidence
21% in North America; 3% of children hospitalized with bronchiolitis. Increasing since 1980 (1).
RISK FACTORS
• Smoking
• Low birth weight
• Immunodeficiency
• Formula feeding (not breastfed)
• Contact with infected person
• Children in day care environment
• Heart-lung transplantation patient
• Adults: Exposure to toxic fumes, connective tissue disease
ETIOLOGY
• Respiratory syncytial virus (70%)
• Parainfluenza
• Adenovirus
• Rhinovirus
• Influenza virus
• Chlamydia
• Eye, nose, mouth inoculation
• Necrosis and lysis of epithelial cells and subsequent release of inflammatory mediators. This results in edema, mucus secretion, combined with necrotic debris, and loss of cilia, resulting in luminal obstruction.
ASSOCIATED CONDITIONS
• Common cold
• Conjunctivitis
• Pharyngitis
• Otitis media
• Diarrhea

DIAGNOSIS
SIGNS AND SYMPTOMS
• Anorexia
• Cough
• Cyanosis
• Apnea
• Fever
• Grunting
• Irritability
• Noisy breathing (due to rhinorrhea)
• Vomiting
Physical Exam
• Tachypnea
• Rhinorrhea
• Wheezing
• Retractions
TESTS
• Arterial O2 saturation by pulse oximetry (92% significant)
• Respiratory viral antigens (not usually necessary during RSV season because the disease is managed symptomatically)
• Urine culture is advised as there is a clinically relevant rate of UTI (1).
Sepsis work up not usually necessary if clinical picture is consistent with bronchiolitis
Imaging
Chest radiograph
• Patchy infiltrates
- Focal atelectasisright upper lobe common
- Air trapping
- Flattened diaphragm
- Increased anteroposterior diameter
- Peribronchial cuffing
Pathological Findings
• Abundant mucous exudate
• Mucosal: Hyperemia, edema
• Submucosal lymphocytic infiltrate, monocytic infiltrate, plasmacytic infiltrate
• Small airway debris, fibrin, inflammatory exudate, fibrosis
• Peribronchiolar mononuclear infiltrate
DIFFERENTIAL DIAGNOSIS
• Asthma
• Vascular ring
• Foreign body
• Heart failure
• Bacterial pneumonia
• Gastroesophageal reflux
• Aspiration
• Cystic fibrosis
• Pertussis
• Croup
TREATMENT
• Most patients can be treated at home.
• Inpatient treatment is indicated for a patient with increased respiratory distress, cyanosis, and dehydration or inability to feed.
GENERAL MEASURES
• Most critical phase is the 1st 48-72 hours after onset. Treatment is usually symptomatic.
• Fluid at maintenance (after correcting for any dehydration); add for respiratory fluid loss.
• Mechanical ventilation in respiratory failure
• Isolation: Contact; hand washing most important
• Cardio-respiratory monitoring
Diet
• Frequent small feedings of clear liquids
• If hospitalized, a patient may require intravenous fluids.
Activity
• Avoid exposure to crowds, viral illness for 2 months
• Avoid smoke
MEDICATION (DRUGS)
First Line
• Oxygen
• Nebulized Albuterol (0.15 mg/kg) may be effective for acute symptoms; a trial of therapy is reasonable. No benefit noted in several high quality studies (1, 2)[B].
• Epinephrine aerosols (0.5 mL of 2.25% solution in 3 mL NS) also may be tried. Caution, because a "rebound phenomena" may occur (child is sent home and worsens: monitor for 2 hours). Benefit remains unproven (3)[B].
• Ribavirin
- Controversial (cost, unclear efficacy)
- Inhaled antiviral agent active against respiratory syncytial virus
- May be indicated in patients with underlying cardiopulmonary disease, young age (6 weeks), immunosuppressed (AIDS, organ transplant patients), or premature infants
- Nebulize via small-particle aerosol generator
- Pregnant women should not be exposed (1).
• Corticosteroids
- Oral dexamethasone (1mg/kg loading dose, then 0.6 mg/kg b.i.d. for 5 days) reduced subsequent hospitalization (4)[B].
- Nebulized dexamethasone (2-4 mg in 3 mL NS) may have anecdotal benefit; studies show mixed results (1).
Second Line
• Antibiotics only if secondary bacterial infection present (rare)
• Heliox therapy(70% helium-30% oxygen) maybe of benefit in moderate to severe bronchiolitis (5)[C].
FOLLOW-UP
DISPOSITION
Admission Criteria
• Respiratory rate >70/min with respiratory distress or apnea
• Ill or toxic appearance
• Underlying heart or respiratory condition
• Dehydrated or unable to feed
• Uncertain home care
PROGNOSIS
• In most cases, recovery is complete within 7-14 days.
• Mortality statistics differ, but probably 1%
• High-risk infants (bronchopulmonary dysplasia, congenital heart disease) may have a prolonged course.
COMPLICATIONS
• Bacterial superinfection
• Bronchiolitis obliterans
• Apnea
• Respiratory failure
• Death
• Increased incidence of reactive airway disease
PATIENT MONITORING
• If the patient is receiving home care, follow daily by telephone for 2-4 days; the patient may need frequent office visits.
• For a hospitalized patient, monitor as needed depending on the severity of the infection. Bronchiolitis can be associated with apnea. Hospitalization is usually only required if oxygen is a requirement.
REFERENCES
1. King VJ, et al. Pharmacologic treatment of bronchiolitis in infants and children: A systematic review. Arch Pediatr Adolesc Med 2004;158(2):127-137.
2. Patel H, et al. A randomized, controlled trial of the effectiveness of nebulized therapy with epinephrine compared with albuterol and saline in infants hospitalized for acute viral bronchiolitis. J Ped 2002;141(6):818-824.
3. Mull cc, et al. A randomized trial of nebulized epinephrine vs albuterol in the emergency department of bronchiolitis. Arch Pediatr Adolesc Med 2004;158(2):113-118.
4. Schuh S, et al. Efficacy of oral dexamethasone in outpatients with acute bronchiolitis. J Ped 2002;140(1):27-32.
5. Martinon-Torres F, et al. Heliox therapy in infants with acute bronchiolitis. Pediatrics 2002;109(1):68-73.

BRONCHIOLITIS OBLITERANS AND ORGANIZING PNEUMONIA

2009-01-22T07:07:00.000-08:00

BRONCHIOLITIS OBLITERANS AND ORGANIZING PNEUMONIA - David A. Pope, MD
BASICS
DESCRIPTION
• A primary or secondary process of the lungs characterized by granulation-like tissue involving the distal airways and alveoli
• A specific reaction of lung tissue to a variety of injuries
• It may occur as patchy infiltrates, or it may be nodular or secondary to another lung disease.
• The process may also appear to be a migrating one.
• It may have a gradual or sudden onset.
• Lungs show a pattern of multiple patchy pneumonia, which are seen on the chest radiograph as patchy alveolar or ground glass opacifications with or without interstitial infiltrates; there may be air bronchograms as well.
• Most cases will respond to corticosteroids, which may have to be given for a year or more
• Synonym(s): Intraluminal fibrosis of distal airways; Idiopathic bronchiolitis obliterans and organizing pneumonitis; Cryptogenic organizing pneumonia; Obliterative bronchiolitis
ALERT
Geriatric Considerations
More common than originally thought and may be sudden and very severe
Pediatric Considerations
Rare, but has been reported after viral pneumonia (adenovirus influenza):
- Characteristics include delayed recovery, persistent cough, crackles, or wheezing after pneumonia
- Laboratory findings generally not helpful
- Imaging shows: Ventilation-perfusion ratio matched defects; high-resolution CT, bronchiectasis, bronchogram, pruned tree appearance
- Diagnosis confirmed by biopsy
- Treatment includes steroids: 1 mg/kg q24h for 1 month, followed by weaning over several months
GENERAL PREVENTION
Except for prevention of relapse, none known
EPIDEMIOLOGY
• Incidence/prevalence in United States: Unknown
• Predominant age: Reported cases range age 0-70, mean age: 50s
Incidence
Unknown
Prevalence
Unknown
RISK FACTORS
• AIDS
• Immunocompromised patients, including transplant patients
• More likely in smokers
ETIOLOGY
Idiopathic. A complex response to a variety of injuries, such as toxic inhalation; post mycoplasma, viral and bacterial infection; aspiration; immunologic factors; drugs
ASSOCIATED CONDITIONS
• Drug-induced pneumonitis
- Paraquat poisoning
- Amiodarone toxicity
- Acebutolol toxicity
- Amphotericin B
- Bleomycin
- Caramazepine
- Cephalosporins
- Gold
- Minocycline
- Nitrofurantoin
- Phenyltoin
- Sulphamethoxypyridazine
- Sulphasalazine
- Ticloppidine
- Freebase cocaine pulmonary toxicity
- Overdose of L-tryptophan
• Infections
- Chronic infectious pneumonia
- Malaria
- Chlamydia
- Legionella
- Mycoplasma
- Pneumocystis
- Cryptococcus
• Immunocompromise: Bone marrow, lung, renal, transplantation
• Connective tissue diseases
- Rheumatic lung
- Sjogren syndrome
- Polymyositis
- Scleroderma
- Essential mixed cryoglobulinemia
• Miscellaneous
- Cystic fibrosis
- Bronchopulmonary dysplasia
- Renal failure
- CHF
- Adult respiratory distress syndrome
- Chronic eosinophilic pneumonia
- Hypersensitivity pneumonitis
- Histiocytosis X
- Sarcoidosis
- Pneumoconioses
- Radiation pneumonitis

DIAGNOSIS
Think of the possibility
SIGNS AND SYMPTOMS
• Most patients present with a flu-like illness that lasts 4-10 weeks or longer. Most have been treated with antibiotics without success.
• Fever
• Dry cough
• Weight loss
• Dyspnea may be severe
• Bilateral crackles
• Fatigue
TESTS
• Leukocytosis with a normal differential
• Elevated erythrocyte sedimentation rate:
• Negative cultures
• Negative serology for mycoplasma, Coxiella, Legionella, psittacosis, and fungus
• Negative viral studies
- Pulmonary function shows a restrictive/obstructive pattern.
- Flow-volume loop shows terminal airway obstruction.
- Chest radiograph may show patchy alveolar opacities, often in the middle or upper lung area, a ground glass pattern that may have air bronchograms
- The involved area may seem to migrate.
- Ventilation-perfusion ratio scan: Matched patchy defects
Imaging
• Chest radiograph: Often appears more normal than the physical examination
• CT scans more accurately define the distribution and extent of the patchy alveolar opacities with areas of hyperlucency.
• Diagnosis is difficult by CT only
Diagnostic Procedures/Surgery
• Open lung biopsy
• Transbronchial biopsy
• It may be wise to use a trial of steroids as a diagnostic trial, although not all would agree.
• If a diagnostic trial is successful, be prepared to treat the patient for at least 1 year.
Pathological Findings
• Intraluminal fibrosis of distal airspaces is the major pathologic feature.
• Fibroblasts and plugs of inflammatory cells and loose connective tissue fill these distal airways.
• Inflammatory cells are mainly lymphocytes and plasma cells.
• Interstitial fibrosis is present.
• Plugs of edematous granulation tissue in the terminal and respiratory bronchioles and alveolar ducts do not cause permanent damage.
DIFFERENTIAL DIAGNOSIS
• Usual interstitial pneumonitis
• Noninfectious diseases
• Tuberculosis
• Sarcoidosis
• Histoplasmosis
• Berylliosis
• Goodpasture syndrome
• Neoplasm
• Polyarteritis nodosa
• Systemic lupus erythematosus
• Wegener granulomatosis
• Sjogren syndrome
• Chronic eosinophilic pneumonia
• Cryptogenic bronchiolitis
TREATMENT
Inpatient may be required
GENERAL MEASURES
• Monitor blood gases or pulse oximetry.
• Oxygen as necessary
Diet
No special diet
Activity
As tolerated
MEDICATION (DRUGS)
First Line
Prednisone
• For 1-3 months, 60 mg/d
• Then taper over a few weeks to 20 mg (this dose may later be given as alternate-day therapy). Increase length of taper for patients on long-term therapy to avoid precipitating Addisonian crisis.
• Treatment may be needed for 1 year or more.
• Contraindications: Refer to the manufacturer's literature.
• Precautions: Be aware of the patient's Mantoux status and history of peptic ulcer disease. Long-term steroid treatment is associated with significant adverse effects including Cushing syndrome, fluid retention, osteoporosis, hyperkalemia, and poor wound healing.
• Significant possible interactions: Refer to the manufacturer's literature.
Second Line
• Steroids other than prednisone may be used.
• 1 paper reported the use of erythromycin 600 mg a day for 3-4 months after initial control with prednisone.
• Prescribe antimicrobials, if the original infection is persistent. The proper choice depends on the pathogen.
FOLLOW-UP
PROGNOSIS
Complete recovery, but individual case management is mandatory
COMPLICATIONS
• Bronchiectasis
• Death, but with proper treatment, recovery is usually complete without permanent sequelae
PATIENT MONITORING
• Frequent visits, weekly at 1st
• Prednisone must be continued because of the chance of relapse
• Monitor the lung disease and the side effects of prednisone therapy (Mantoux, monthly CBC, funduscopic exam every 3-6 months, serial dual energy x-ray absorptiometry (DEXA) scans for osteoporosis)
REFERENCES
1. Cordier JF, Loire R, Brune J. Idiopathic bronchiolitis obliterans organizing pneumonia. Chest. 1989;96:999-1004.
2. Epler GR, Colby TV, et al. Bronchiolitis obliterans organizing pneumonia. N Engl J Med. 1985;312:152-158.
3. Hardy KA, Schidlow D, Zaeri N. Obliterative bronchiolitis in children. Chest. 1988;93:460-466.
4. Lynch DA. Imaging of small airways diseases. Clin Chest Med. 1993;14:623-634.
5. Mueller NL, Staples CA, Miller RR. Bronchiolitis obliterans organizing pneumonia: CT features in 14 patients. AJR. 1990;154:983-987.
6. St John RC, Dorinsky PM. Cryptogenic bronchiolitis. Clin Chest Med. 1993;14:667-675.
7. http://www.epler.com/boop1.html
8. http://www.emedicine.com/radio/byname/bronchiolitis-obliterans-organizing-pneumonia.html
MISCELLANEOUS
• Other notes: This disease behaves differently than bronchiolitis obliterans. Bronchiolitis obliterans and organizing pneumonia is a restrictive problem; bronchiolitis obliterans is an obstructive problem. Bronchiolitis obliterans causes permanent lung damage; bronchiolitis obliterans and organizing pneumonia is completely reversible.
• See also: Sjogren Syndrome

BRONCHIECTASIS

2009-01-22T07:06:00.001-08:00

BRONCHIECTASIS - Dylan C. Kwait, MD
BASICS
DESCRIPTION
Irreversible dilatation of 1 or more airways accompanied by recurrent bronchial infection/inflammation and chronic mucopurulent sputum production; generally classified into cystic fibrosis (CF) and non-cystic fibrosis (non-CF) bronchiectasis.
ALERT
Geriatric Considerations
Elderly are more likely to need hospitalization for treatment.
Pediatric Considerations
Associated with childhood respiratory infections, CF, and other congenital conditions.
GENERAL PREVENTION
• Immunize against
- Pertussis, measles, Haemophilus influenza type B (in childhood)
- Viral illnesses (influenza)
- Pneumococcal pneumonia
• Genetic counseling in cases in which a congenital condition may increase the likelihood of developing bronchiectasis
• Adequate treatment of all pneumonias
EPIDEMIOLOGY
• Still a significant cause of respiratory morbidity
• Predominant age: Most commonly presents in 6th decade of life (1)[A]
• Predominant sex: Female > Male (1)[A]
Incidence
Incidence has decreased in the US secondary to widespread childhood vaccination against pertussis (2)[A] and the effective treatment of childhood respiratory infections with antibiotics (1)[A].
Prevalence
• Prevalence in the adult US population estimated to be at least 110,000 (1)[A].
• Prevalence is 5 to 10 times higher in those >55 years of age (3)[B]
RISK FACTORS
• Severe respiratory infection in childhood (measles, adenovirus, influenza, pertussis, or bronchiolitis)
• Predisposing congenital condition
• Systemic diseases (e.g., rheumatoid arthritis and connective tissue disorders)
• Chronic rhinosinusitis
• Recurrent pneumonia
• Aspirated foreign body
• Immunodeficiency
PATHOPHYSIOLOGY
"Vicious cycle hypothesis": Transmural infection, generally by bacterial organisms, causes inflammation and obstruction of airways. Damaged airways and dysfunctional cilia foster bacterial colonization, which leads to further inflammation and obstruction (2)[A].
ETIOLOGY
• CF bronchiectasis
• Non-CF bronchiectasis
- Most cases are idiopathic (4)[B].
- The etiologic agent most commonly associated with non-CF bronchiectasis is childhood respiratory infection (2)[A].
- May be associated with many conditions.
ASSOCIATED CONDITIONS
• Mucociliary clearance defects
- Primary ciliary dyskinesia
- Young syndrome (secondary ciliary dyskinesia)
- Kartagener syndrome
• Other congenital conditions
- 1-Antitrypsin deficiency
- Marfan syndrome
- Cartilage deficiency (Williams-Campbell syndrome)
• Chronic obstructive pulmonary disease
• Postinfectious conditions
- Bacteria (H. influenzae and Pseudomonas aeruginosa)
- Mycobacterial infections (TB and MAC)
- Whooping cough
- Aspergillus species
- Viral (HIV, adenovirus, measles, influenza virus)
• Immunodeficient conditions
- Primary
 Hypogammaglobulinemia
- Secondary
 Allergic bronchopulmonary aspergillosis
 Post-transplantation
• Sequelae of toxic inhalation or aspiration (e.g., chlorine, luminal foreign body)
• Rheumatic/chronic inflammatory conditions:
- Rheumatoid arthritis
- Sjogren syndrome
- Systemic lupus erythematosus
- Inflammatory bowel disease
• Miscellaneous
- Yellow nail syndrome

DIAGNOSIS
SIGNS AND SYMPTOMS
Symptoms are commonly present for many years and include
• Chronic cough (90%) (1)[A]
• Sputum: copious and purulent (90%) (1)[A]
• Rhinosinusitis (60-70%) (1)[A]
• Fatigue, may be a dominant symptom (70%) (1)[A]
• Dyspnea (75%) (2)[A]
• Chest pain, may be pleuritic (20-30%)(1)[A]
• Hemoptysis (20-30%) (1)[A]
• Wheezing (20%)(1)[A]
History
It is vital to elicit
• Time course of illness
• Any predisposing factors (either congenital, infectious, and/or exposure-related)
• Immunization history
Physical Exam
• Bi-basal crackles (60%) (1)[A]
• Wheezing (34%) (2)[A]
• Rhonchi (44%) (2)[A]
• Digital clubbing (3%) (2)[A]
TESTS
• Spirometry
- Limited use in diagnosis
- Characterized by moderate airflow obstruction and hyperresponsive airways (1)[A]
- FEV180% predicted and FEV1/FVC 0.7 (3)[B]
• Special tests
- Ciliary biopsy by electron microscopy
Lab
• Sputum culture
- H. influenzae, nontypeable form (42%) (1)[A]
- P. aeruginosa (18%) (1)[A]
- Cultures may also be positive for Streptococcus pneumoniae, Moraxella catarrhalis, MAC, and Aspergillus (1)[A]
- 30-40% of all isolates will show no growth (1)[A]
• Special tests
- Sweat test for CF
- PPD test for TB
- Skin test for Aspergillus
- HIV test
- Serum immunoglobulins to test for humoral immunodeficiency
Imaging
• CT scan
- Noncontrast high-resolution CT (HRCT) is the most important tool used to diagnose bronchiectasis (2)[A].
- Bronchi are dilated and do not taper; varicose constrictions and ballooned cysts may also be appreciated (2)[A].
• Chest radiograph
- Nonspecific findings; sensitivity and specificity are too low to confirm the diagnosis (3)[B]
- Increased lung markings (1)[A]
- May be normal
Diagnostic Procedures/Surgery
Interventional bronchoscopy
• Used to obtain culture and evacuate sputum
Pathological Findings
• Dilatation of airways (2)[A]
• Thickened bronchial walls with necrosis of bronchial mucosa (2)[A]
• Peribronchial scarring (2)[A]
DIFFERENTIAL DIAGNOSIS
• Chronic obstructive pulmonary disease
• Asthma
• CF
• Chronic bronchitis
• Pulmonary tuberculosis
• Allergic bronchopulmonary aspergillosis
TREATMENT
Treatment of non-CF bronchiectasis involves determining the cause of exacerbations, promoting good bronchopulmonary hygiene through daily airway clearance, and surgical resection of damaged lung when necessary.
STABILIZATION
Hemoptysis, although rare, may occur and can be life threatening. Appropriate measures must be taken to minimize blood loss.
GENERAL MEASURES
• Dry powder mannitol improves tracheobronchial clearance (1)[A]
• Maintain hydration (nebulized saline may be used) (2)[A]
• Noninvasive positive-pressure ventilation (2)[A]
Diet
No dietary restrictions
Activity
Regular exercise is recommended.
Physical Therapy
Sputum clearance techniques
• Physiotherapy (percussion and postural drainage)
• Pulmonary rehabilitation (improves exercise tolerance, but does not benefit from Inspiratory muscle training) (1)[A]
MEDICATION (DRUGS)
First Line
• Antibiotics
- May be used in acute exacerbations
- Chronic therapy decreases sputum volume and purulence, but does not diminish the frequency of exacerbations (5)[A].
- Patients may require twice the usual dose and should receive long courses of treatment (7-14 days) (3)[B].
- Selection is complicated by the wide range of pathogens involved and the existence of resistant organisms (culture should be used to direct therapy).
- Augmentin (3)[B]: 500 mg PO q8-12h for 7-10 days (pediatric: Base dosing protocol on amoxicillin content)
- Trimethoprim-sulfamethoxazole (3)[B]: 160 mg TMP/800 mg SMZ PO q12h for 10-14 days (pediatric: 2 months, 8 mg/kg TMP and 40 mg/kg SMZ PO per 24 hours, administered in 2 divided doses q12h for 10 days)
- Doxycycline and cefaclor given orally are also effective (3)[B].
- Nebulized aminoglycosides (tobramycin): 300 mg by aerosol b.i.d. (6)[B]
- Macrolides appear to have immunomodulatory benefits (1)[A].
- Antibiotics should be administered IV in cases of severe infection.
• Bronchodilators
- Chronic use of -2 agonists (e.g., albuterol) effectively reverses airflow obstruction (1)[A].
• Inhaled corticosteroids
- Decrease sputum and tend to improve lung function (3)[B]
- Fluticasone: 110-220 mcg inhaled b.i.d.
• Contraindications: Documented hypersensitivity
• Precautions: Cross-allergy and organ impairment
• Significant possible interactions: Broad-spectrum antibiotics may reduce efficacy of oral contraceptives
Second Line
Other broad-spectrum antimicrobials including antipseudomonals.
SURGERY
Surgery should be considered in cases in which disease is localized and symptoms remain intolerable despite medical therapy. Surgery effectively improves symptoms in 80% of these cases (1)[A].
FOLLOW-UP
PROGNOSIS
• Mortality rate is 13% (death due directly to bronchiectasis) (1)[A].
• Pseudomonas infection is associated with poorer prognosis (1)[A].
COMPLICATIONS
• Hemoptysis
• Recurrent pulmonary infections
• Pulmonary hypertension
• Cor pulmonale
• Lung abscess
PATIENT MONITORING
• Serial spirometry, performed every 2-5 years, should be used to monitor the course of the disease (1)[A].
• Routine microbiological sputum analysis (1)[A].
• Annual influenza and pneumococcal immunizations (3)[B].
REFERENCES
1. King P, Holdsworth S, Freezer N, Holmes P. Bronchiectasis. Intern Med J. 2006;36(11):729.
2. Barker AF. Bronchiectasis. N Engl J Med. 2002;346:1383-1393.
3. Bradley J, Lavery K, Rendall J, Elborn JS. Managing bronchiectasis. Practitioner. 2006;250(1681):194.
4. Pasteur MC, Helliwell SM, Houghton SJ, et al. An investigation into the causative factors in patients with bronchiectasis. Am J Respir Crit Care Med. 2000;162:1277-1284.
5. Evans DJ, Bara AI, Greenstone M. Prolonged antibiotics for purulent bronchiectasis. Cochrane Database of Sys Rev. 2006;(4):CD00284.
6. Lobue PA. Inhaled tobramycin: Not just for cystic fibrosis anymore? Chest. 2005;127:1098.
MISCELLANEOUS
See also: Cystic fibrosis; Chronic obstructive pulmonary disease; Asthma; Pulmonary tuberculosis; Aspergillosis; Kartagener syndrome

BREECH BIRTH

2009-01-22T07:06:00.000-08:00

BREECH BIRTH - Kimberle Vore, MD
BASICS
DESCRIPTION
• At the time of delivery, the fetal buttocks are the presenting part in the maternal pelvis
- Frank breech presentation: The fetal hips are flexed and the knees are extended with the feet near the shoulders; accounts for 60-65% of breech presentations at term.
- Incomplete breech presentation: 1 or both of the fetal hips are incompletely flexed, resulting in some part of the fetal lower extremity as the presenting part. Thus the terms single footling, double footling, knee presentation. Accounts for 25-35% of breech presentations.
- Complete breech: Similar to frank breech except that knees are flexed rather than extended. Accounts for 5% of breech presentations.
• System(s) Affected: Reproductive
ALERT
Pregnancy Considerations
A problem of pregnancy
GENERAL PREVENTION
• External cephalic version
- Conversion of breech to vertex can be attempted after 36 weeks of gestation and if successful allows for vaginal vertex delivery. Success rates 48-78%, with reversion rates back to breech of 2%.
- External cephalic version associated with risk (1-2%) of umbilical cord entanglement, abruptio placenta, preterm labor, premature rupture of membranes, fetal brachycardia, fetal-maternal hemorrhage, and severe maternal discomfort
- Prior to procedure, tocolytics are usually administered and RhoGAM is given to Rh-negative mothers.
- External cephalic version should only be attempted with continuous fetal heart monitoring in the delivery suite, where immediate cesarean delivery can be done
- External cephalic version requires 2 operators, 1 to monitor fetal cardiac activity via ultrasound and holding fetal position, while the 2nd person lifts the buttocks out of the pelvis by abdominal manipulation and then guides the fetal head into the pelvis.
- Contraindications to external cephalic version include multiple pregnancy, nonreassuring fetal monitoring, placenta previa, premature rupture of membranes, abruption, previous uterine surgery, uterine malformation, oligohydramnios, maternal cardiac disease, or major fetal anomalies.
- Successful external cephalic version factors include multiparity, relaxed abdominal wall, adequate amniotic fluid, nonfrank breech, floating presenting part, posterior placenta, and average maternal body weight.
- Failure of external cephalic version associated with maternal obesity, nulliparity, anteriorly located placenta, large fetus, decreased amniotic fluid, frank breech that is engaged in pelvis
• Prevention of fetal anomalies by tight glucose control in diabetics
• Antenatal folate therapy to decrease risk of neural tube detects
EPIDEMIOLOGY
Predominant sex: Female only (affects only women in terms of pregnancy, but affects both sexes of fetuses)
Prevalence
• 3-4% of singleton-term deliveries and up to 15-30% of low-birth-weight infants (2,500 g)
• Breech presentation is common in early pregnancy. At 25-26 weeks, ~20-30% of singleton fetuses are in breech position, but this decreases near term.
RISK FACTORS
• Fetal anomalies including anencephaly, hydrocephalus, trisomy 21 and 21, fetal alcohol syndrome, Potter syndrome, myotomic dystrophy
• Uterine anomalies including bicornate uterus
• Uterine relaxation associated with great parity
• Uterine overdistension as in polyhydramnios or multiple gestation
• Placenta previa
• Placental implantation in cornual-fundal region
• Low-birth-weight or premature infant
• Macrosomia
• Pelvic contractions or irregularly shaped pelvissuch as android or platypelloid pelvis
• Pelvic tumors
• Nulliparity
• Previous history of breech birth
Genetics
Fetal anomalies including anencephaly, hydrocephalus, and trisomy 21 and 18 have higher incidences of breech birth.
ETIOLOGY
Probably a combination of 1 or more of the risk factors listed above
ASSOCIATED CONDITIONS
• See "Risk Factors"
• Congenital hip dislocation is more common in 1st-born (breech) females.

DIAGNOSIS
SIGNS AND SYMPTOMS
• Anus palpable on digital vaginal exam
• Leopold maneuver reveals ballottable head in fundal region
• Mother reports kicking in lower abdomen
• Presenting part not palpable in pelvis near term
TESTS
Imaging
• Ultrasoundconfirms presenting part
• Radiographflat plate of abdomen and pelvimetry to determine extent of head flexion and pelvic measurements (rarely done)
Diagnostic Procedures/Surgery
• Near-term women should be examined to determine presenting part.
• If breech is suspected, an ultrasound should be done to confirm presenting part.
• When breech presentation is confirmed, the option for external version or elective cesarean section should be discussed with the patient.
Pathological Findings
• Congenital malformation among term breech infants: Overall incidence 6-9%
• There is a higher incidence of congenital hip dislocation in infants with breech presentation at term.
DIFFERENTIAL DIAGNOSIS
• In labor, diagnosis is made by vaginal exam and confirmed by ultrasound. Can be confused with face presentation on digital vaginal exam
• In breech presentation, greater trochanter and anus form a straight line. In face presentation, mouth and malar bones form a triangle.
TREATMENT
Inpatient for labor and delivery
GENERAL MEASURES
• Continuous electronic fetal monitoring during labor
• Breech presentation may be converted by external version (see "General Prevention"), but this is not always successful and has risks.
• Currently, the American College of Obstetricians and Gynecologists (ACOG) recommends external version at term and planned cesarean delivery for persistent breech presentation. This recommendation is based on a large randomized clinical trial showing decreased perinatal and neonatal morbidity and mortality in planned breech cesarean delivery (1) [NNT 30] vs planned breech vaginal delivery. There was no difference in maternal morbidity or mortality. (1)[B]
Diet
NPO until delivery accomplished
Activity
Bed rest during labor
SURGERY
• Breech delivery is accomplished either vaginally or by cesarean section
• Most physicians and patients opt for elective cesarean delivery for breech presentation near term, which is usually planned for the 39th week of pregnancy.
• When a patient presents in labor with the fetus in breech position, a decision about a trial of labor or immediate cesarean section must be made. Preferably this decision is made prior to onset of labor.
• Obtain ultrasound to document fetal presentation, check for fetal abnormalities, and estimate fetal weight in deciding candidacy for vaginal delivery.
• The selection for vaginal breech delivery could include
- Breech presentation in advanced labor
- Delivery of a 2nd twin in nonvertex presentation
- Fetus too immature to survive
- Fetus with congenital defects incompatible with life
• Cesarean section procedure
- Prepare for cesarean section by starting IV fluids and obtaining blood type and screen, in all patients, in case needed for emergency.
- A low transverse cesarean section may need to be extended vertically if there is difficulty with head entrapment (this extension produces a weak scar).
- General anesthesia with isoflurane can rapidly relax the uterus and allow delivery of an entrapped after-coming head.
- Delivery is usually accomplished with spinal anesthesia.
- Cord blood gases should be obtained following delivery.
• Vaginal delivery procedures
- Currently not recommended, but may be an option in limited circumstances (see above)
- The candidate for vaginal delivery needs to be attended by a birth attendant skilled in breech delivery, a scrubbed assistant, an anesthesiologist capable of rapid induction of general anesthesia, and an individual skilled in neonatal resuscitation.
- Epidural is preferred anesthesia
- Leave membranes intact as long as possible, to prevent possible cord prolapse
- The patient should not push until fully dilated, due to risk of partial delivery through a cervix that is not fully dilated, which can lead to head entrapment.
- Consider cutting a large episiotomy to allow sufficient room for delivery.
- Use abdominal guidance of fetal head to keep it flexed as it descends into the pelvis.
- The infant should not be touched before the umbilicus crosses the maternal perineum. Traction prior to this point constitutes a complete breech extraction and is associated with higher risk of perinatal morbidity and mortality.
- With the fetal back anterior, maintain downward traction while grasping the fetal hips until the scapula becomes visible.
- Check for nuchal arm.
- As one axilla becomes visible, rotate the infant until the shoulders are oriented anteriorly and posteriorly, allowing their delivery.
- The fetal head is delivered in a face-down position with either piper forceps or manual flexion of the head.
- Cord blood gases should be obtained following delivery.
FOLLOW-UP
PROGNOSIS
• Perinatal morbidity and mortality are much higher in breech births. A large proportion of the deaths are related to congenital abnormalities.
• Successful external cephalic version at term significantly lowers cesarean rate. (2)[A]
• For infants 750-1500 g or 32 weeks gestational age, there is a much higher rate of cerebral hemorrhage and perinatal death associated with vaginal compared to cesarean delivery.
COMPLICATIONS
• Trauma to the head, soft tissue, brachial plexus, and spinal cordnot always prevented by cesarean
• Asphyxia secondary to cord compression or prolapse
• Congenital hip dislocation
PATIENT MONITORING
• Continuous fetal heart rate monitoring should be done during labor and delivery.
• 6-weeks postpartum care as for other deliveries
REFERENCES
1. Term Breech Trial. Lancet. 2000;356(9239):1375
2. External Cephalic Version. ACOG practice bulletin. Num. 13, February. 2000.
3. Committee on Obstetric Practice. ACOG committee opinion. Mode of term singleton breech delivery. Number 265, December 2001. American College of Obstetricians and Gynecologists. Int J Gynaecol Obstet. 2002;77:65-66.
4. Scorza W. Intrapartum management of breech presentation. Clin Perinatol. 1996;23:31-49.
MISCELLANEOUS
• Other notes: Maneuvers of cesarean breech delivery are similar to vaginal breech extraction and can be associated with severe trauma to the infant.
• See also: Placenta Previa; Premature Labor

BREAST-FEEDING

2009-01-22T07:05:00.000-08:00

BREAST-FEEDING - Kathy Mariani, MD
BASICS
DESCRIPTION
Breast-feeding is the natural process of feeding an infant human milk. Nursing an infant directly at the breast is usually done, but in many circumstances the milk may be expressed by hand or a pump to be fed to the infant at another time.
• Maternal benefits include
- Easier postpartum weight loss
- Decreased postpartum bleeding due to oxytocin release
- Increased bonding
- Convenience of feeding
- Delayed postpartum fertility
- Increased sense of well being (endorphin response)
- More rapid and complete reversion of mother's pelvis and uterus to prepuerperal state
- Decreased risk of breast cancer
- Possible decreased risk of osteoporosis
- Economical
• Infant benefits include
- Maternal antibody protection
- Decreased incidence of otitis media
- Decreased upper respiratory infection and sepsis
- Fewer respiratory and gastrointestinal infections
- Decreased incidence of obesity
 Ideal foodeasily digestible, nutrients well absorbed, less constipation
- Increased attachment between mother and baby
- Decreased incidence of allergies in childhood
• Contraindications
- HIV infection
- Active tuberculosis
- Hepatitis is not a contraindication.
- Substances of abuse will pass into human milk; see reference on drugs in lactation
• Physiology
- Stimulation of areola causes secretion of oxytocin.
- Oxytocin is responsible for let-down reflex when milk is ejected from cells into milk ducts.
- Sucking stimulates secretion of prolactin, which triggers milk production. Thus milk is made in response to nursing and increases supply.
• Technique
- Initiate immediately after birth
- Get in comfortable position, usually sitting or reclining with baby's head in crook of mother's arm (side-lying position often useful following cesarean-section delivery)
- Bring baby to mother to decrease stress on back.
- Baby's belly and mother's belly should face each other or touch (belly to belly).
- Initiate the rooting reflex by tickling baby's lips with nipple or finger. As baby's mouth opens wide, mother guides her nipple to back of her baby's mouth while pulling the baby closer. This will ensure that the baby's gums are sucking on the areola, not the nipple.
- Feed every 2-4 hours, 20 minutes per side
• System(s) Affected: Endocrine/Metabolic; Skin/Exocrine
EPIDEMIOLOGY
• Predominant age: 16-45 years
• Predominant sex: Female only
Incidence
According to Healthy People 2010, in 2002, 70% of new mothers initiated breast-feeding, and 29% were doing at least some breast-feeding at 6 months of age. The national goal is 75-50%, respectively

TREATMENT
STABILIZATION
Outpatient
GENERAL MEASURES
See "Patient Teaching."
Diet
• Adequate calorie and protein intake while nursing.
• Drinking cow's milk is not necessary.
• Drink plenty of fluids (8-12 oz. glasses/day).
• Continue prenatal vitamins.
• Fluoride supplement unnecessary
• New National Academy of Science guidelines recommend that children get at least 200 IU/d of vitamin D beginning in the newborn period to prevent rickets. For exclusively breast-fed babies, this will require taking a vitamin supplement such as PolyViSol or Vi-Daylin vitamin drops, 1/2 cc/d.
Activity
No restrictions
FOLLOW-UP
PROGNOSIS
Healthy baby
COMPLICATIONS
• Plugged ducts (mother is well except for) sore lump in 1 or both breasts without fever
- Use moist hot packs on lump prior to and during nursing; more frequent nursing on affected side; ensure good technique
• Mastitis
- Sore lump in 1 or both breasts plus fever and/or redness on skin overlying lump
- Use moist hot packs on lump prior to and during nursing; more frequent nursing on affected side; antibiotics covering for Staphylococcus aureus (the most common organism) for at least 7 days
- Patients can be quite ill with mastitis.
- Other possible sources of fever should be ruled outendometritis, pyelonephritis in particular. Mother should get increased rest, use acetaminophen (Tylenol) as necessary. Fever should resolve within 48 hours or consider changing antibiotics. Lump should also resolve. If it continues, an abscess may be present, requiring surgical drainage.
• Milk supply inadequate
- Check weight gain
- Review signs of adequate supply; review technique, frequency, and duration of nursing.
- Check to see if mother has been supplementing, thereby decreasing her own milk production.
• Sore nipples
- Check technique
- Baby should be taken off the breast by breaking the suction with a finger in the mouth.
- Air-dry nipples after each nursing; no breast creams and do not wash nipples with soap and water; check for signs of thrush in baby and mother
• Engorgement
- Usually develops after milk 1st comes in (day 3 or 4)
- Signs are warm, hard, sore breasts.
- To resolve, offer baby more frequent nursing; may have to hand express a little milk to soften areola enough to let baby latch on; nurse long enough to empty breasts; generally resolves within a day or 2.
• Flat or inverted nipples
- When stimulated, inverted nipples will retract inward, flat nipples remain flat; should check for this on initial prenatal physical
- Nipple shells, a doughnut-shaped insert, can be worn inside the bra during the last month of pregnancy to gently force the nipple through the center opening of the shell.
- Babies can nurse successfully even if the shell does not correct the problem before birth. A lactation consultant or La Leche League member may be a good resource in this situation. Another source: J Human Lactation. 1993;9:27-29.
PATIENT MONITORING
See mother and baby within a few days of hospital discharge if she is a 1st-time breast-feeder.
REFERENCES
1. Berlin CM, Briggs GG. Drugs and chemicals in human milk. Semin Fetal Neonatal Med. 2005;10(2):149-159.
2. Meek J, ed. New Mother's Guide to Breastfeeding. American Academy of Pediatrics. 2002
3. Moreland J, Coombs J. Promoting and supporting breast-feeding. Amer Fam Physician. 2000;61:2093-2100, 2103-2104.
4. Sinusas K, Gagliardi A. Initial management of breast-feeding. Amer Fam Physician. 2001;15;64:981-988.

BREAST CANCER

2009-01-22T07:04:00.001-08:00

BREAST CANCER - Alexandra Sherman, BA
BASICS
DESCRIPTION
• Malignant neoplasm in the breast
• Classified as carcinoma in situ (CIS) or invasive; 70% of all breast cancers possess a component of invasion.
• Age-specific incidence of breast cancer increases sharply until menopause and continues to increase at a slower rate in the geriatric population.
ALERT
Geriatric Considerations
Higher percentage of estrogen receptor-positive tumors (80%) in the geriatric population; correlates with improved disease free survival.
Pregnancy Considerations
• Breast cancer occurs infrequently during pregnancy (2.8%).
• Delay in diagnosis is common, and most series report poorer survival related to advanced stage at diagnosis.
GENERAL PREVENTION
• Mammography to screen for disease
- The U.S. Preventive Services Task Force recommends a mammography with or without clinical breast examination every 1-2 years for women >40.
- The American Cancer Society recommends mammography and a clinical breast examination every year after age 40 and a clinical breast examination every 3 years for ages 20-39.
- The ACOG and AMA recommend mammogram every 1-2 years and an annual clinical breast examination starting at age 40 and then annual mammograms at age 50.
- Albeit controversial, mammography may reduce mortality by 30% in women 50-69; the reduction in mortality is less impressive for women 50 or >70 years.
• Tamoxifen reduces invasive and noninvasive breast cancers by 50% in high-risk women but has significant risk of thromboembolic events and uterine cancer.
- Recently reported results from the STAR trial showed that raloxifene (Evista) was as effective as tamoxifen in preventing invasive breast cancer in postmenopausal women but was associated with less risk for DVT (NNT = 455), pulmonary emboli, and uterine cancer (NNT=370).
EPIDEMIOLOGY
• Breast cancer is the most common malignancy in women in North America.
• Predominant age: 30-80 with peak age 45-65; 77% of cases occur in women >50
• Predominant sex: Female > Male (1% occurs in males)
Incidence
The American Cancer Society estimates that 212,930 new cases will be diagnosed in 2005, with 40,870 deaths (including 460 men).
Prevalence
One in 7 women will develop breast cancer within her lifetime.
RISK FACTORS
• Increased risk occurs in 1st-degree relatives (relative risk 1.7- 2.5), with bilateral disease in premenopausal relatives (relative risk = 10.5), or bilateral disease in postmenopausal relatives (relative risk = 5.0).
• Hormonal risk factors include early menarche, late menopause, nulliparity, 1st full-term pregnancy after age 30, postmenopausal HRT.
• Women with a history of breast cancer or previous breast biopsies revealing atypical changes are at increased risk (5-10 times) for subsequent cancer.
• Exogenous estrogen use, especially in conjunction with progestins, increases risk.
- Premenopausal oral contraceptives have not been shown to increase risk.
• Radiation exposure has been associated with an increased risk of breast cancer.
Genetics
• 10-20% of the patients have a significant family history of breast cancer.
• Women who inherit a mutated BRCA1, a tumor suppressor gene, have a 60-80% lifetime risk of breast cancer and a 33% risk of ovarian cancer. BRCA2 is associated with increased risk of breast cancer in men and women.
- Family history suggestive of breast cancer susceptibility genes include multiple 1st-degree and 2nd-degree relatives with early breast cancer diagnosis and the presence of ovarian cancer.
- 1 in 400 U.S. women carry a germ-line mutation for BRCA1.
• Her-2,neu is an oncogene whose overexpression plays a role in 25-30% of breast cancers; Her-2,neu confers a poor prognosis and has treatment implications.
ASSOCIATED CONDITIONS
Organ disease at metastatic sites

DIAGNOSIS
SIGNS AND SYMPTOMS
• Palpable mass (55%)
• Abnormal mammogram without a palpable mass (35%)
• Color, size (enlargement or shrinkage), or shape changes
• Lymphedema (peau d'orange)
• Dimpling
• Nipple retraction, tenderness, or pain
• Axillary mass
• Bone pain (rare)
• Discharge (bloody discharge is more ominous)
History
Any personal or family history of breast cancer, previous breast biopsy, or recent changes in breasts
TESTS
Bone scan, CT, or ultrasound of abdomen if widespread or recurrent disease is suspected
Lab
Initial lab tests include CBC, liver function tests, chest radiograph, bilateral mammography  ultrasound, estrogen and progesterone receptor determination, FISH for her-2,neu status and S-phase determination. Consider MRI.
Imaging
• Mammography (sensitivity = 50-80% depending on analog versus digital, age, menstrual status, and breast density; positive predictive value = 5-20%)
- Most common abnormalities: Irregular mass, spiculated density, microcalcifications (35%), or architectural distortion.
• Ultrasound may confirm whether a suspicious lump is solid or cystic and help define its size and extent.
Diagnostic Procedures/Surgery
• Nonpalpable lesions: Core biopsy or open excisional biopsy
• Palpable abnormality: Fine-needle aspiration or core-needle biopsy.
Pathological Findings
• Noninvasive cancers: Intraductal (DCIS) or intralobular (LCIS) (carcinoma in situ)
- Intraductal cancers: Micropapillary, cribriform, solid, or comedo. The comedo growth pattern is considered more aggressive.
• Invasive cancers
- Ductal NOS (70%), lobular (5%), Paget disease (2%), inflammatory and miscellaneous (metaplastic, neuroendocrine, or squamous cell carcinomas [1%])
- Patients with invasive intraductal histologies with medullary (6%), colloid/mucinous (3%), tubular, papillary, and adenoid cystic (2%) subtypes have improved survival.
DIFFERENTIAL DIAGNOSIS
• Benign breast disorders, such as abscesses, hematomas, fibroadenomas, fibrocystic change, ductal or lobular hyperplasia, or sclerosing adenosis
• Malignant breast diseases, including sarcomas, lymphomas, or metastatic disease to breast
TREATMENT
Patients treated by a team consisting of a medical oncologist, a surgeon, and a radiation oncologist
GENERAL MEASURES
• Early breast cancer treatment (stage I/II)
- Lumpectomy (wide excision with breast conservation), sentinel node biopsy, hormonal therapy, and radiotherapy is the treatment of choice.
- Combination chemotherapy is also indicated, because most patients have subclinical metastases.
• Treatment of locally advanced breast cancer (stage III)
- Usually multidisciplinary treatment consisting of mastectomy, axillary dissection, radiation, and chemotherapy  tamoxifen
• Treatment of advanced or recurrent disease (stage IV)
- Surgical resection if possible, plus chemotherapy, radiation, hormonal therapy.
Activity
As tolerated.
SPECIAL THERAPY
Radiotherapy
• Postlumpectomy radiation is the mainstay of adjuvant local therapy and has been shown to decrease local recurrence compared to lumpectomy and hormonal therapy alone (1)[A].
• The indications for postmastectomy radiation therapy to the chest wall include patients with 4 positive lymph nodes, the presence of extracapsular extension, and tumor stage of T3 or more.
MEDICATION (DRUGS)
• Combination chemotherapy (most common regimens: CMF, AC or AC + Taxol) reduces the risk of recurrence and improves overall survival in women with tumors >1cm and positive nodes.
- Chemotherapy, notably AC + Taxol improves overall survival by 16.7% in ER- women compared to 4% in ER+ women (2)[B].
• Adjuvant tamoxifen (Nolvadex) (20 mg/d) reduces the risk of recurrence and death for women of all ages by 5-11%, especially in postmenopausal, ER/PR+ women.
• An aromatase-inhibitor, anastrazole (Arimidex) (1 mg/d) may be more effective (NNT = 40 recurrent disease) and better tolerated (NNT = 166 endometrial cancer, NNT = 125 DVT) in ER/PR+, postmenopausal women with localized disease than tamoxifen, although it does increase risk of musculoskeletal problems (NNH = 30 fracture) compared to tamoxifen (3)[A].
• Trastuzumab (Herceptin) (4 mg/kg loading dose, 2 mg/kg maintenance infusion) improves mortality by 1/3 when combined with chemotherapy for early stage HER-2,neu+ breast cancer (4)[A].
• Contraindications: Strict hematologic, renal, hepatic, and cardiac guidelines must be followed for the administration of cytotoxic chemotherapy. Arimidex should not be given to pregnant women.
• Adverse events: See manufacturer' literature. Herceptin associated with CHF.
• Precautions: Monitor for infections and infusion reactions in chemotherapy patients. See "Complications" section for discussion of tamoxifen.
• Significant possible interactions: Drug interactions are common and depend on combinations used. Refer to manufacturer's literature.
First Line
All drugs mentioned above may be used as first-line treatments.
SURGERY
Breast-conserving surgery is appropriate for most breast cancers, because no difference in long-term survival is noted when comparing mastectomy to breast conservation (5)[A]. Axillary node dissection is indicated with all invasive tumors and large noninvasive ones. Identification and biopsy of sentinel nodes may be preferred over axillary dissection because of its lower morbidity rate, but is only appropriate for patients with early-stage breast cancer with clinically negative axillary nodes.
FOLLOW-UP
PROGNOSIS
• 5-year survival
- Stage 0 (noninvasive): 100%
- Stage I (2 cm, no spread): 98%
- Stage II (>2 cm, or spread to axillary lymph nodes): 76-88%
- Stage III (>5 cm or fixed nodes, metastatic disease to the skin, inflammatory changes, chest wall extension, or supraclavicular lymph nodes): 49-56%
- Stage IV (distant metastatic disease): 16%
• The status of the axillary lymph nodes is the most important indicator for disease relapse.
- If any axillary nodes are positive, 60-70% risk of relapse within 5 years.
- If all axillary nodes are negative, 70-80% chance of a long-term cure
COMPLICATIONS
• Postoperative: Lymphedema (5% in modified radical mastectomy), seroma, wound infection, and limited shoulder motion
• Chemotherapy: Nausea, vomiting, alopecia, leukopenia, bladder irritation, stomatitis, fatigue, and menstrual abnormalities
• Tamoxifen: Hot flushes, menstrual irregularities including menopause, vaginal discharge, hypercalcemia, skin rashes, endometrial carcinoma, DVTs, CVAs and interactions with warfarin, erythromycin, cyclosporin, nifedipine, and diltiazem.
• Irradiation: Skin reaction, fatigue, fibrosis (1%), brachial plexopathy (1%), rib fracture (1%), arm edema, pulmonary fibrosis (1%), and rarely 2nd breast malignancy
PATIENT MONITORING
• Up to 60% of patients with invasive disease will relapse within 5 years despite initial therapy.
- Diagnosis of relapse does not impact survival.
• Surveillance for recurrent disease should include physical examination every 4-6 months for 5 years, then yearly. Mammography and routine chemistries should be done annually. Women on tamoxifen should have annual pelvic exams.
• The workup of a suspected recurrence should include CBC, liver function tests, chest radiograph, bone scan, a CT of the affected area, mammogram, and/or a biopsy.
REFERENCES
1. Fisher B, Anderson S, Bryant J, et al. Twenty-year follow-up of a randomized trial comparing total mastectomy, lumpectomy, and lumpectomy plus irradiation for the treatment of invasive breast cancer. NEJM 2002;347:1233-1241.
2. Berry D, Cirrincione C, Henderson C, et al. Estrogen receptor status and outcomes of modern chemotherapy for patients with node positive breast cancer. JAMA 2006;295:1658-1667.
3. Howell A, Cuzick J, Baum M, et al. Results of the ATAC (Arimidex, Tamoxifen, Alone or in Combination) trial after completion of 5 years' adjuvant treatment for breast cancer. Lancet 2005;365(9453):60-62.
4. Romond E, Perez E, Bryant J, et al. Trastuzumab plus adjuvant chemotherapy for operable her-2 positive breast cancer. NEJM 2005;353:1673-1684.
5. Veronesi U, Cascinelli N, Mariani L, et al. Twenty-year follow-up of a randomized study comparing breast-conserving surgery with radical mastectomy for early breast cancer. NEJM 2002;347:1227-1232.

BREAST ABSCESS

2009-01-22T07:04:00.000-08:00

BREAST ABSCESS - Anya S. Koutras, MD; Kristen Burdick, MD
BASICS
DESCRIPTION
• Collection of pus, usually localized.
• Can be associated with lactation or fistulous tracts secondary to squamous epithelial neoplasm or duct occlusion
• System(s) Affected: Skin/Exocrine
• Synonym(s): Mammary abscess; Peripheral breast abscess; Subareolar abscess; Puerperal abscess
ALERT
Pregnancy Considerations
Most commonly associated with postpartum lactation
GENERAL PREVENTION
• Prevention of mastitis
• Early treatment of mastitis with milk expression and cold compresses
• Early treatment with antibiotics
EPIDEMIOLOGY
• Predominant age
- Puerperal abscess: Premenopausal
- Subareolar abscess: Postmenopausal
• Predominant sex: Female
Incidence
• 0.1-0.5% of breast-feeding women
• Puerperal abscess rare after 1st 6 weeks of lactation
RISK FACTORS
• Puerperal mastitis: 5-11% go on to abscess (most often due to inadequate therapy). Risk factors for mastitis are those that result in milk stasis (infrequent feeds, missing feeds)
• Poor latch, damaged nipple, illness in mother or baby, rapid weaning, breast pressure, blocked nipple pore or duct, maternal stress or fatigue, maternal malnutrition
• General factors: Diabetes, rheumatoid arthritis,
• Steroids, silicone/paraffin implants, lumpectomy with radiation, heavy cigarette smoking,
• Nipple retraction
ETIOLOGY
• Delayed treatment of mastitis
• Puerperal abscesses: Blocked lactiferous duct
• Subareolar abscess: Squamous epithelial neoplasm with keratin plugs or ductal extension with associated inflammation
• Peripheral abscess: Stasis of the duct

DIAGNOSIS
SIGNS AND SYMPTOMS
• Tender breast lump, fluctuant, usually unilateral
• Erythema
• Draining pus
• Local edema
• Systemic malaise (though usually less malaise than with mastitis)
• Fever
• Nipple and skin retraction
• Proximal lymphadenopathy
TESTS
Lab
• Leukocytosis
• Elevated sedimentation rate
• Culture and sensitivity of drainage to identify pathogen, usually Staphylococci or Streptococci. E. coli is 3rd most common. Nonlactational abscess associated with anaerobic bacteria.
Imaging
• Ultrasound
• Mammogram
Diagnostic Procedures/Surgery
• Aspiration for culture
• Fine-needle aspiration not accurate to exclude carcinoma
Pathological Findings
• Squamous metaplasia of the ducts
• Intraductal hyperplasia
• Epithelial overgrowth
• Fat necrosis
• Duct ectasia
DIFFERENTIAL DIAGNOSIS
• Carcinoma (inflammatory or primary squamous cell))
• Tuberculosis (may be associated with HIV infection)
• Actinomycosis
• Typhoid
• Sarcoid
• Granulomatous disease
• Syphilis
• Foreign body reactions (e.g., to silicone and paraffin)
• Mammary duct ectasia
• Hydatid cyst
• Sebaceous cyst
TREATMENT
PRE-HOSPITAL
Outpatient, unless systemically immunocompromised or septic
GENERAL MEASURES
• Cold compresses for pain control
• Important to continue to breast-feed or express milk
Diet
• No restrictions
• Lecithin supplementation
Activity
No restrictions
MEDICATION (DRUGS)
• Must combine antibiotics with drainage for cure
• Culture mid-stream sample of milk for mastitis, abscess fluid for breast abscess.
• NSAIDs
• Start with dicloxacillin 500 mg q.i.d. for 10-14 days
• If no response in 24-48 hours, switch to:
• Cephalexin 500 mg q.i.d. for 10-14 days
- Or amoxicillin-clavulanate (Augmentin) 250 mg t.i.d.
• Clindamycin 300 mg t.i.d. if anaerobes suspected
• Contraindications: Allergy to the antibiotic
• Precautions: Refer to manufacturer's profile for each drug
• Significant possible interactions: Refer to manufacturer's profile for each drug
SURGERY
• Aspiration under ultrasound guidance (1,2)[B], (3)[C]
• If aspiration and antibiotics fail, incision and drainage with removal of loculations
• Biopsy of all nonpuerperal abscesses to rule out carcinoma
• Open all fistulous tracts, especially in nonlactating abscesses
FOLLOW-UP
PROGNOSIS
• Good. Complete healing expected in 8-10 days
• Subareolar abscess frequently reoccur, even after I+D and antibiotics; may require surgical removal of ducts.
COMPLICATIONS
Fistula
PATIENT MONITORING
Ensure resolution to exclude carcinoma.
REFERENCES
1. Dener C, Inan A. Breast abscesses in lactating women. World J Surgery. 2003;27:130.
2. Schwarz, RJ, Shrestha R. Needle aspiration of breast abscesses. Am J Surgery. 2001;182:117.
3. Christensen AF, Al-Suliman N, et al. Ultrasound-guided drainage of breast abscesses: Results in 151 patients. Br J Radiol. 2005 Mar; 78(927):186-188.
ADDITIONAL READING
• Cibele B, Schwartz K, Foxman B. Lactation mastitis. JAMA. 2003;289:1609-1612.
• Ng C, Jahanfar S, Teng CL. Antibiotics for mastitis in breastfeeding women (Protocol). Cochrane Database of Systematic Reviews, 2005;(3): CD005458.

BRANCHIAL CLEFT FISTULA

2009-01-22T07:03:00.001-08:00

BRANCHIAL CLEFT FISTULA - Timothy L. Black, MD
BASICS
DESCRIPTION
• A congenital, abnormal tract connecting the skin of the neck with an internal structure, resulting from failure of closure of a branchial cleft
• May involve branchial clefts I-IV, which develop in the 4th gestational week
• System(s) Affected: Skin/Exocrine
ALERT
Pediatric Considerations
Almost all occur in the pediatric age group.
EPIDEMIOLOGY
• Predominant age: By definition, all are present at birth, although they may remain unnoticed for some time. (Branchial cleft cysts may not present until later childhood.) (1)[C]
• Predominant sex: Unknown
Incidence
Unknown
Prevalence
Unknown
RISK FACTORS
Positive family history
Genetics
10% have family history.
ETIOLOGY
• The 1st branchial cleft contributes to the tympanic cavity and eustachian tube. Related fistulae are very rare and tend to be infra- or retroauricular. (Preauricular cysts and sinuses are not thought to be of branchial cleft origin.)
• The 2nd branchial cleft forms the hyoid bone and tonsillar fossa. Related fistulae (most common variant) course between the internal and external carotid arteries. Internal opening usually at level of tonsillar fossa. External opening along anterior border of sternocleidomastoid muscle. (1)[C]
• 3rd and 4th branchial clefts form parathyroid glands, thymus, and portions of thyroid (parafollicular cells). Fistulae are rare; those from 3rd cleft course posterior to carotid artery; both should have external ostia on lower anterior neck. Sinus tracts (also called pyriform sinuses) originate in the pyriform sinus and course adjacent to the thyroid cartilage. (2)[C]
ASSOCIATED CONDITIONS
Microtia and aural atresia occur with failure of development of 1st branchial cleft. (3)[C]

DIAGNOSIS
SIGNS AND SYMPTOMS
• Presence of tiny external opening usually on lower neck along anterior border of sternocleidomastoid muscle
• Spontaneous mucoid drainage
• External openings may also be marked by a skin tag or cartilage.
• Infection may rarely be the presenting sign, with erythema, swelling, pain, or fever.
• 10% are bilateral. (3)[C]
History
History of drainage from cervical area
Physical Exam
Small orifices located in the mid neck, most commonly along the anterior border of the sterno-cleidomastoid muscle (less commonly in the lower neck or post-auricular)
TESTS
Lab
Culture if signs of infection.
Diagnostic Procedures/Surgery
• Sinogram or fistulogram may be done, but is of little value.
• CT of neck with IV contrast occasionally beneficial in 3rd and 4th branchial cleft fistulas/sinus (2)[C]
• Pharyngoscopy may occasionally be useful
Pathological Findings
• Lined by stratified squamous epithelium, may contain hair follicles, sweat glands, sebaceous glands, or cartilage (3)[C]
• Some are lined by ciliated columnar epithelium
DIFFERENTIAL DIAGNOSIS
• External sinuses
• Cystic hygroma
• Dermoid cysts
• Lymphadenopathy
TREATMENT
• Surgical excision
• Outpatient status usually appropriate
SURGERY
• Small transverse incision at external ostium with careful dissection of fistula (1)[C]
• Stepladder incisions may be needed (3)[C]
• End of fistula ligated flush with pharyngeal mucosa. 1st branchial cleft lesions may require larger incision (1)[C]
• Methylene blue injection into fistula may be useful
• Drains are not used.
• Antibiotics only for infection
FOLLOW-UP
PROGNOSIS
Good
COMPLICATIONS
• Facial nerve injury
• Infection
• Carotid artery injury
• Possible recurrence if any epithelium remains
• Neoplastic degeneration of branchial remnants (~250 reported cases) if not resected
PATIENT MONITORING
• Follow at weekly intervals, if infected, until resolution, then excision
• Postoperative visit at 2 weeks
REFERENCES
1. Roback SA, Telander RL. Thyroglossal duct cysts and Branchial cleft anomalies. Sem Ped Surg. 1994;3:142-146.
2. Liberman M, Kay S, Emil S, et al. Ten years of experience with third and fourth branchial remnants. J Ped Surg. 2002;37:685-690.
3. Ashcraft KW, Murphy JP, Sharp RJ, eds. Pediatric Surgery. 3rd ed. Philadelphia, PA: WB Saunders; 2000.
MISCELLANEOUS
Branchial cleft remnants, sinuses, and cysts are also the result of failure of branchial cleft to complete its normal development.

BRAIN INJURY, TRAUMATIC

2009-01-20T05:29:00.000-08:00

BRAIN INJURY, TRAUMATIC - John Herbert Stevenson, MD
BASICS
DESCRIPTION
• Frequently related to rapid deceleration, as occurs ins motor vehicle accidents or diving accidents; may also be due to blunt trauma.
• Traumatic brain injury (TBI) is a dynamic process with initial bleeding followed by secondary injury due to cerebral edema, continued intracranial bleeding, etc.
• Predicting outcome initially is difficult, and patients may improve for years.
• System(s) Affected: Cardiovascular; Endocrine/Metabolic; Nervous
• Synonym(s): Head injury
ALERT
Geriatric Considerations
• Poorer prognosis with increasing age
• Subdural hematomas are common after fall or blow; symptoms may be subtle.
Pediatric Considerations
Outcome for children is more positive, except in severe TBI.
GENERAL PREVENTION
• Safety education
• Seat belts, bicycle and motorcycle helmets
• Protective headgear for contact sports
EPIDEMIOLOGY
• Predominant age: 15-24 years
• Predominant sex: Male > Female
Incidence
• 200/100,000
• 500,000 hospitalizations and 75,000 deaths per year
RISK FACTORS
Alcohol, prior head injury, contact sports; "heading" soccer balls may cause long-term cognitive loss.
ETIOLOGY
• Motor vehicle accident (50%)
• Falls
• Assault
• Child abuse
- Consider if dropped or fell 4 feet (e.g., off bed, couch) and significant injury present
- Subdural more likely to be abuse
- Any retinal hemorrhage (retinal hemorrhage is not caused by seizures or simple head trauma)
ASSOCIATED CONDITIONS
Alcohol and drug abuse

DIAGNOSIS
SIGNS AND SYMPTOMS
Variable and dependent on degree of injury
History
• Loss of consciousness (LOC)
• External signs of head injury
• Headache
• Vomiting
• Amnesia
• Epidural hemorrhage from blunt trauma is generally acute, 30% with a "lucid interval" (initial loss of consciousness [LOC] followed by recovery of consciousness, then LOC secondary to the intracranial bleed)
• Subdural hemorrhage usually has a slower onset and may present weeks after the initial injury, especially in the elderly.
Physical Exam
• Focal signs and symptoms
• Evidence of increased intracranial pressure (ICP) (elevated BP, decreased pulse rate, or slow or irregular breathing [Cushing triad])only 30% have all 3
• Decorticate or decerebrate positioning (both bad prognostic signs)
• Seizures
• Signs of basilar skull fracture: Raccoon eyes, battle sign, hemotympanum, CSF rhinorrhea or otorrhea (see "Tests")
• Unilateral dilated pupil in an alert patient is not consistent with impending herniation, because such patients are always unconscious.
TESTS
• Neuropsychometric testing when able
• CSF rhinorrhea
- Contains glucose, whereas nasal mucus does not
- Check also for the double-halo sign: Put a drop of nasal discharge on filter paper. If it contains CSF and blood, 2 rings appear, a central ring followed by a paler ring.
Lab
• Evaluate for coagulopathy.
• Drug and alcohol screening
Imaging
• CT, noncontrast, is study of choice to review bone windows, tissue windows, and subdural space
- NEXUS II study has demonstrated 8 clinical criteria that, if all absent, indicate a low likelihood of significant TBI
 Evidence of significant skull fracture (depressed, basilar, or diastatic)
 Altered level of alertness
 Neurologic deficit
 Persistent vomiting
 Presence of scalp hematoma
 Abnormal behavior
 Coagulopathy
 Age >65
• Skull radiographs are not helpful in most cases, but can be done to document child abuse.
Diagnostic Procedures/Surgery
• Placement of ICP monitor when indicated
• Serial neurologic exams
Pathological Findings
• Epidural, subdural, or intraparenchymal hemorrhage
• Coup or contra-coup injury
• Evolving, diffuse axonal injury is a principal cause of neurologic sequelae with mild head trauma.
DIFFERENTIAL DIAGNOSIS
Other causes of coma (e.g., drug overdose, infection, metabolic, vascular causes)
TREATMENT
STABILIZATION
• ABCs take priority over head injury.
• C-spine immobilization should be considered in all head trauma.
GENERAL MEASURES
• Acute management depends on severity of injury. Most patients need no interventions.
• Immediate goal: Determine who needs further therapy, imaging studies (CT), and hospitalization to prevent further injury.
• For the severely injured patient
- Avoid hypotension or hypoxia. Head injury causes increased ICP secondary to edema, and perfusion pressure must be maintained.
- Use normal saline for resuscitation fluid.
- Hyperventilation is controversial, but current literature suggests a short duration of hyperventilation, not below PaCO2 30 mm Hg may be beneficial. Prophylactic hyperventilation for those without signs or symptoms of increased intracranial pressure is contraindicated and may cause additional injury secondary to vasoconstriction.
- Hypothermia: Although no difference is seen in mortality, may have marginal benefit especially in patients with elevated ICP refractory to other methods.
- Seizure prophylaxis does not change outcomes (such as death rates) but may prevent seizures. Consider phenytoin for 1 week postinjury.
- Manage breakthrough seizures with lorazepam.
Diet
As tolerated
Activity
See "Activity" under topic "Postconcussive Syndrome" for sports activity management.
MEDICATION (DRUGS)
First Line
• Pain: Morphine 12 mg IV p.r.n.
• Increased ICP
- 0.252 g/kg (0.251 g/kg in children) given over 30-60 minutes in patients with adequate renal function; should not be used unless there is evidence of increased ICP; prophylactic use is associated with worse outcomes.
- 20-40 mg IV to promote diuresis
- Neither furosemide or mannitol should be given to a hypotensive patient.
- Hypertonic saline 2 mL/kg IV decreases ICP without adverse hemodynamic status and may have beneficial effects on immune system and excitatory neurotransmitters.
• Sedation
- Preferred due to short duration of action allowing serial neurologic exams
• Seizures
- 15 mg/kg IV (1 mg/kg/min IV, not to exceed 50 mg/min). Stop infusion if QT interval increases by >50% () 15 mg/kg IV, not to exceed 150 mg/min, if need rapid infusion due to active seizures
- () 12 mg (0.1 mg/kg in children) IV. Preferred over diazepam.
- 15 mg/kg IV at 25-50 mg/min. May give IM.
• Contraindications: Allergy
Second Line
• Diuretics and IV -blockers (e.g., esmolol or labetalol) can be used to maintain mean arterial pressure between 130-70 mm Hg, which may be helpful. However, nitrates may increase ICP.
• Antibiotics (e.g., cefazolin) should be given if penetrating trauma is present. Prophylactic antibiotics are not useful in basilar skull fractures.
SURGERY
Depends on neurosurgical consult
FOLLOW-UP
DISPOSITION
Discharge Criteria
• Abnormal CT
• Abnormal Glasgow coma scale
• Clinical evidence of basilar skull fracture
• Persistent neurological deficits (e.g., confusion, somnolence)
• Patient with no competent adult at home for observation
• Possibly admit: LOC, amnesia, etc.
• Normal hemocrit with return to normal mental status and responsible adult to observe patient at home (see "Patient Monitoring")
Issues for Referral
Consult neurosurgery for
• All penetrating head trauma
• All abnormal head CTs
PROGNOSIS
• Gradual improvement for many
• 30-50% of severe head injuries may be fatal.
• Prolonged coma may be followed by satisfactory outcome.
• Rehabilitation indicated following a significant acute injury. Set realistic goals.
COMPLICATIONS
• Delayed hematomas
• Chronic subdural hematoma, which may follow even "mild" head injury, especially in the elderly. Often presents with headache and decreased mentation.
• Delayed hydrocephalus
• Emotional disturbances and psychiatric disorders resulting from head injury may be refractory to treatment.
• Seizure disordersin 50% of penetrating head injuries, in 20% of severe closed head injuries, and in 5% of head injuries overall. Hematomas significantly increase risk of epilepsy.
• Second-impact syndrome occurs when the central nervous system loses autoregulation. An individual with a minor head injury is returned to a contact sport and, following even minor trauma (e.g., whiplash), the patient will lose consciousness and herniate within 12 minutes, with a 50% mortality. A similar syndrome of "malignant edema" can occur in children with even a single injury.
PATIENT MONITORING
• Any patient discharged should have "head injury instructions" to watch for symptoms indicating the need for further intervention (e.g., changing mental status, worsening headache, focal findings). Give to a competent surrogate who will observe the patient. A patient who deteriorates is not likely to remember or act on any instructions.
• Schedule regular follow-up.
• The postconcussion syndrome can follow mild head injury without LOC and includes headaches, dizziness, fatigue, and subtle cognitive or affective changes.
• Proper counseling, symptomatic management, and gradual return to normal activities is essential to prevent a posttraumatic neurosis that can become refractory to treatment.
MISCELLANEOUS
• Other notes: The Glasgow coma scale is not a linear scale; a score of 14 (normal being 15) represents a moderately severe injury category.
• See also: Brain injurypost acute care issues; Postconcussive syndrome; Seizure disorders

BRAIN INJURYPOST ACUTE CARE ISSUES

2009-01-20T05:28:00.000-08:00

BRAIN INJURYPOST ACUTE CARE ISSUES - Bart M. Demaerschalk, MD, MSc
BASICS
DESCRIPTION
Traumatic brain injury (TBI) is broadly defined as brain injury due to externally inflicted trauma and may result in significant impairment of an individual's physical, cognitive, and psychosocial functioning.
• System(s) Affected: Nervous; Pulmonary; Skin/Exocrine; Endocrine/Metabolic; Renal/Urologic; Gastrointestinal (GI); Musculoskeletal
GENERAL PREVENTION
Improved safety standards and programs designed to minimize injury from vehicular-related events (motor vehicle, motorcycle, bicycle, pedestrian), falls, violence, sports, and recreation provide the best prevention against TBI. (1,2)[C]
EPIDEMIOLOGY
• Predominant age: Highest incidence persons 15-24 years of age and those >75 years, with additional smaller peak in children 5 years.
• Predominant sex: Male > Female (2:1)
Incidence
An estimated 1.2 million-1.7 million Americans sustain TBI per year. Incidence of TBI is 100/100,000 in the US, 230,000 hospitalizations, 50,000 deaths per year, and an estimated 80-90,000 sustain long-term disabilities.
Prevalence
Prevalence estimates range from 2.5 million-6.5 million individuals living with consequences of TBI.
RISK FACTORS
See "Brain Injury, Traumatic"
ETIOLOGY
Motor vehicle, bicycle, or pedestrian-vehicle incidents (50%), falls, acts of violence and assault, and sports and recreation-related injuries are the leading causes of TBI.
ASSOCIATED CONDITIONS
• Psychosis
• Suicide attempts
• Substance abuse
• ADD

DIAGNOSIS
SIGNS AND SYMPTOMS
• Consequences of TBI often influence human functions along a continuum from altered physiological functions, through neurological, psychological, cognitive, and behavioral impairments, to medical problems and disabilities that affect the individual, family, and community.
• Nonneurological complications include pulmonary, metabolic and endocrinological, nutritional, gastrointestinal, musculoskeletal, genitourinary, dermatologic, and chronic pain.
• Most neurological complications are apparent within the 1st days or months following injury. Long-term sequelae include seizures, headache, visual defects, and movement and sleep disorders.
• Cognitive consequences include memory impairment, difficulties in attention and concentration, language deficits, visual perception problems, and poor executive skills, problem solving, reasoning, insight, judgment, planning, information processing, and organization.
• Behavioral problems include decreased ability to initiate responses, verbal and physical aggression, agitation, learning difficulties, shallow self-awareness, altered sexual functioning, impulsivity, and social disinhibition.
• Psychological consequences include mood disorders, personality changes, altered emotional control, depression, and anxiety.
• Social consequences include risk of suicide, divorce, unemployment, economic strain, and alcohol/substance abuse.
ALERT
Pediatric Considerations
• Interactions of physical, cognitive, and behavioral sequelae interfere with new learning. The effect of early TBI may not become apparent until later in the child's development.
TESTS
• Evoked potentials (auditory, visual, and somatosensory)
• Behavioral assessment, neuropsychological testing, and vocational assessment
• Cognitive test for orientation and arousal; use Western Neuro Sensory Stimulation Profile or Galvest. Orient. Amnesia Test
• EEG
Lab
• CBC, electrolytes, blood urea nitrogen creatinine, calcium, albumin, vitamin B12, folate, thyroid stimulating harmone, alkaline phosphatase, aspartate aminotransferase (AST), alanine amino- transferase (ALT), morning cortisol level, urine culture
• Culture, ova, and parasites for diarrhea
• Skin culture
• Culture tracheal site
• Endocrine workup as indicated
Imaging
• Bone scan: Heterotopic ossification
• CT: Hydrocephalus, atrophy, hematoma
• Video pharyngeal fluoroscopic swallowing study
• MRI to evaluate diffuse axonal injury
Diagnostic Procedures/Surgery
• Altered arousalvisual, auditory, and somatosensory evoked potentials
• Neurogenic bladder-check post-void residuals 3-4 times. If >50 cc or 20% of voided volume, urodynamics
• Ultrasound of bladder and kidney: Urolithiasis and hydronephrosis
• Endoscopy: Cause of dysphagia
• Contractures and spasticity: Examination under anesthesia
• Respiratory and neurologic: Sleep/oxygen saturation study, bronchoscopy for stricture
Pathological Findings
• Hydrocephalus with periventricular edema
• Joint contractures results in collagen cross linking: Decreased range of motion
• Heterotopic ossification: Disorganized osteoid calcification in soft tissue
DIFFERENTIAL DIAGNOSIS
Differential Diagnosis of Pain after TBI: (5)
The diagnosis of pain following TBI can be difficult in light of the limitations imposed by cognitive, language, and behavioral deficits. The differential diagnosis includes
• Dysautonomia: Characterized by episodes of tachypnea, hypertension, painful posturing/contractions, and diaphoresis
• Neuropathic pain: Described as burning, shock-like, or pins and needles; Allodynia/hyperpathia. The 3 most common forms are complex regional pain syndrome, central pain syndrome, and peripheral neuropathy.
• Spasticity or spastic dystonia
• Headache: Posttraumatic headache
• Myofascial pain syndrome
• Neurogenic Heterotopic Ossification: Bone formation in soft tissue
• Deep Venous Thrombosis
• Constipation and urinary retention
• Trauma: Fractures, musculoskeletal injuries
• Shoulder: Subluxation, acromioclavicular separation, rotator cuff tendonitis/tear
Differential Diagnosis of Alteration in Functional Capacity/Level after TBI:
Chronic infection (UTI, aspiration pneumonia, GI), depression, hypothyroidism or hydrocephalus, intracerebral hemorrhage, epilepsy/seizures, fractures, tracheal stricture, pain, alcohol or drugs, polypharmacy and/or central nervous system depressant/psychoactive drugs
TREATMENT
GENERAL MEASURES
• Diminished level of arousal: Identify best modality for communication, assess functional skills (proper seating, hand function), behavioral or neuropsychologist. Social work (to assist with family education and long-term planning) and nursing
• Reduce sedatives
• Neurogenic bladdertreat UTI
- If post-void residual 50 cc, then trial of regular voiding routine q2h
- If still incontinent, add oxybutynin
- If still incontinent, try condom catheter during the day; incontinent pads at night.
- If high post-void residuals or high pressure bladder or dyssynergic bladder on urodynamics: Intermittent catheter q4-6h
• Neurogenic bowel: Regular bowel routine
• Contractures and spasticity: Stretching
- If no progress after 4 weeks, consider serial casting or custom made orthotic
- Contractures >45: Consider tendon release
• Heterotopic ossification: Stretch soft tissue to decrease maturation of osteoid, consider orthotics/splinting, bone scan at baseline
• Skin: q2h turning, avoid sitting such as in bed at 45, observe for erythema around tube sites and rule out latex allergy
• Respiratory: Night humidification if has a tracheotomy, may require suctioning
• Endocrine: Monitor fluid balance
• Dental: Assessment and x-rays
• Rehabilitative practices: Rehabilitative programs should be interdisciplinary, comprehensive, and include cognitive and behavioral assessment and intervention. (1) [C]
• Non-pharmacological therapeutic interventions
- Cognitive exercises (including computer-assisted strategies), compensatory devices (memory books, paging systems), psychotherapy, behavior modification, vocational rehabilitation, school setting rehabilitation, nutritional support, music and art therapy, and therapeutic recreation (4)[C]
Diet
• Consult with dietitian
• Ensure adequate hydration; 2-2.5 L of water/day. More if outside or in hot weather.
• Bolus feeds preferred if fed by gastrostomy
• Upright and quiet for 1/2 hour following feeds, as aspiration can occur even with a g-tube
Activity
As tolerated: Outings in wheelchair can be beneficial; skin very sensitive to sun/wind
MEDICATION (DRUGS)
• Psychostimulants may affect speed of cognitive processing, mood, and behavior, but effects on attention, distractibility, and memory are less clear. Methylphenidate 20-40 mg/d in 2 divided doses; Dextroamphetamine. (9)[B]
• Agitation
- Treat epilepsy or depression
- Minimize the use of antipsychotics and benzodiazepines, as they worsen cognition.
- If necessary, use antipsychotics of the atypical class (Clozapine, Olanzepine, Quetiapine, Risperidone, Ziprasidone). (8)[B]
• Abulia and lack of initiative: Amantadine (Symmetrel), bromocriptine, methylphenidate, levodopa (8)[C]
• Epilepsy: American Academy of Physical Medicine and Rehabilitation does not recommend antiepileptic drugs for preventing late (>7 days post TBI) posttraumatic seizures. [B] If epilepsy occurs, avoid phenytoin and phenobarbitaltoo sedating. (6)
• Spasticity caution: Be aware of potential negative consequences of all agents. Dantrolene sodium 25-200 mg/day divided t.i.d.; Baclofen; intrathecal Baclofen; Diazepam, Clonidine, Tizanidine, and Gabapentin; Botulinum toxin injections for focal spasticity. (7)[B]
• Neurogenic bladder: Oxybutynin 2.5 mg t.i.d.-10 mg q.i.d. if bladder pressures low and/or post-void residuals low [B]
• Bowel routine: Stool softener such as docusate sodium (daily) combined with laxative (night before suppository), high fiber and suppository (every other day) to induce bowel movement [C]
• Heterotopic ossification: Indomethacin 25-50 mg t.i.d. If severe, progressive, or history of GI ulceration, then etidronate (Didronel) 20 mg/kg for 6 months or alendronate 20 mg once a day. [C]
• Neurobehavioral problems: Weak evidence that psychostimulants are effective in the treatment of inattention, apathy, and slowness; high-dose beta-blockers in the treatment of agitation and aggression; and anti-convulsants and anti-depressants in the treatment of agitation and aggression with an affective disorder. (3,4)[B]
• Contraindications: Refer to manufacturers' literature
• Precautions: Medications may have significant adverse effects in persons with TBI and can impede rehabilitation progress.
• Significant possible interactions: Refer to manufacturers' literature.
SURGERY
Tendons releases; fundoplasty or gastrostomy; tracheostomy; ventriculoperitoneal or ventriculoatrial shunt
FOLLOW-UP
PROGNOSIS
• Most rapid return of function is during 1st 2 years, but some improve slowly for 5-10 years
• Highly variable (80% of individuals with severe injuries become independent in dressing and self-care at 1 year)
• Negative prognostic factors
- Age >40
- Abnormal pupillary responses
- Prolonged coma (i.e., GCS 9, seven days after injury)
- Abnormal evoked potentials
- Extraocular eye movement abnormalities
COMPLICATIONS
• Major affective disorder (depression, psychosis) in up to 50% of patients
• Family and caregiver burn out
• Substance abuse
• Social isolation
• May be at higher risk of dementia
• Latex allergy to g-tube, catheters
• Dental caries
• Osteoporosis
• Falls
• Aspiration pneumonia
• Pressure ulcers
• Dysphagia, esophagitis
• Bladder incontinence
• Contractures/spasticity
PATIENT MONITORING
• Patients make slow steady gains. Ongoing outcome assessments determine progress (or not) in abilities and medication efficacy.
• Review medical status monthly
REFERENCES
1. NIH Consensus Development Panel on Rehabilitation of Persons with Traumatic Brain Injury. Rehabilitation of persons with traumatic brain injury. JAMA. 1999;282(10):974-983.
2. Lovasik D, Kerr M, Alexander S. Traumatic brain injury research: A review of clinical studies. Crit Care Nurs Q. 2001;23(4):24-41.
3. Shoumitro D, Crownshaw T. The role of pharmacotherapy in the management of behaviour disorders in traumatic brain injury patients. Brain Injury. 2004;18(1):1-31.
4. Glenn MB. A differential diagnostic approach to the pharmacological treatment of cognitive, behavioral, and affective disorders after traumatic brain injury. J Head Trauma Rehab. 2002;17(4):273-283.
5. Ivanhoe CB, Hartman ET. Clinical caveats on medical assessment and treatment of pain after TBI. J Head Trauma Rehab. 2004;19(1):29-39.
6. Bushnik T, Englander J, Duong T. Medical and social issues related to posttraumatic seizures in persons with traumatic brain injury. J Head Trauma Rehab. 2004;19(4):296-304.
7. Zafonte R, Elovic EP, Lombard L. Acute care management of post-TBI spasticity. J Head Trauma Rehab. 2004;19(2):89-100.
8. Elovic EP, Lansang R, Li Y, Ricker JH. The use of atypical antipsychotics in traumatic brain injury. J Head Trauma Rehab. 2003;18(2):177-195.
9. Whyte J, Vaccaro M, Grieb-Neff P, Hart T. Psychostimulant use in the rehabilitation of individuals with traumatic brain injury. J Head Trauma Rehab. 2002;17(4):284-299.
MISCELLANEOUS
• Rehabilitation program guidelines
- Individualized goals: Behavioral approach emphasizing reinforcement of task behavior
- Flexible: Alter to meet changing needs
- Functional (based on activities of daily living): Self care activities involve range of motion exercises
- Consider patient's attention span and best time of day when planning
- Allow for as patient control and choice when able (e.g., choice of clothes, music, etc.)
- Consistency and familiarity
- Quality of life issues vital (e.g., comfort measures, sensory stimulation, address spiritual and/or cultural needs, and positioning)
- For agitated behavior, consider consult with behavioral psychologist to design program integrating medications and behavior therapy techniques. Minimize use of punishment and reinforce correct behavior.
• See also: Brain Injury, Traumatic; Constipation; Dysphagia; Fecal Impaction; Gastroesophageal Reflux Disease; Hemorrhoids; Osteoporosis; Pressure Ulcer; Seizure Disorders; Sleep Apnea, Obstructive; Stomatitis; Stroke (Brain Attack); Stroke Rehabilitation

BRAIN ABSCESS

2009-01-20T05:27:00.000-08:00

BRAIN ABSCESS - PeterKozisek, MD
BASICS
DESCRIPTION
• Single or multiple abscesses within the brain, usually occurring secondary to a focus of infection outside the central nervous system
• May mimic brain tumor, but evolves more rapidly (days to a few weeks)
• Starts as a cerebritis, becomes necrotic, and subsequently becomes encapsulated
• Synonym(s): Cerebral abscess
ALERT
Geriatric Considerations
Age does not affect outcome as much as the abscess size and state of neurological dysfunction at presentation.
Pediatric Considerations
• About 1/3 of the cases in pediatric age group
• Rarely found in infants 1 year of age
• Cyanotic congenital heart disease frequently associated
GENERAL PREVENTION
• Adequate treatment of otitis media, mastoiditis, dental abscess, other predisposing factors
• Prophylactic antibiotics after compound skull fracture or penetrating head wound
EPIDEMIOLOGY
• Incidence/prevalence in the US: Infrequent
• Predominant age: Median age 30-40 years
• Predominant sex: Male > Female (2:1)
RISK FACTORS
• AIDS
• Immunocompromised
• IV drug abuse
Genetics
No known genetic pattern
ETIOLOGY
• Direct extension from otitis, mastoiditis, sinusitis, or dental infection
• Cranial osteomyelitis
• Penetrating skull trauma
• Prior craniotomy
• Bacteremia from lung abscess, pneumonia
• Bacterial endocarditis
• Fungal infection of the nasopharynx
• Toxoplasma gondii (in AIDS patients)
• Cyanotic congenital heart disease
• IV drug use
• No source found in 20%
• Most common infective organisms: Streptococci, staphylococci, enteric Gram-negative bacilli and anaerobes (usually same as source of infection), Nocardia
ASSOCIATED CONDITIONS
• AIDS
• Congenital heart disease

DIAGNOSIS
SIGNS AND SYMPTOMS
History
• Recent onset of headache becoming severe
• Nausea and vomiting
• Mental changes progressing to stupor and coma
• Afebrile or low-grade fever
• Neck stiffness
• Seizures
• Papilledema
• Focal neurological signs depending on location
Physical Exam
• Lumbar puncture often contraindicated
• Search for primary source of infection (chest radiograph, skull film for fracture, or sinus films)
TESTS
• WBC may be normal or mildly elevated
• Culture of abscess contents, predominant organisms include Toxoplasma (AIDS), Staphylococcus (trauma), aerobic or anaerobic bacteria, fungi (rare)
• Blood studies: Mild polymorphonuclear leukocytosis; elevated sedimentation rate
• Special test: Surgical burr hole with aspiration to make a specific bacteriologic diagnosis
ALERT
Drugs that may alter lab results: Prior administration of antibiotics
Imaging
• CT or MRI are the diagnostic methods of choice. The findings are dependent on stages of the abscess.
• Radionuclide 117In-labeled leukocytes may distinguish abscess from neoplasm
Pathological Findings
• Suppuration, liquefaction, and encapsulation, depending on stage of evolution
• Fibrosis
DIFFERENTIAL DIAGNOSIS
• Brain tumors
• Cysticercosis
• Stroke
• Resolving intracranial hemorrhage
• Subdural empyema
• Extradural abscess
• Encephalitis
TREATMENT
PRE-HOSPITAL
Inpatient for close observation, diagnostic evaluation, and specialty consultation (neurology, neurosurgery, or infectious disease)
GENERAL MEASURES
• Palliative and supportive
• Medical therapy
- For surgically inaccessible, multiple abscesses
- For abscesses in early cerebritis stage
- Small (2.5 cm) abscess
- Therapy directed toward most likely organism
Diet
IV fluids if nausea and vomiting present
Activity
Bed rest until infection controlled and abscess evacuated or resolving, then up as tolerated
MEDICATION (DRUGS)
• Antibiotics according to organism if known
• If organism unknown, begin with penicillin G and metronidazole, or chloramphenicol (Chloromycetin), if metronidazole cannot be used
• Add oxacillin or nafcillin if trauma or IV drug user (use vancomycin in penicillin-sensitive patients)
• If Gram-negative organism suspected (otic, GI, GU organ), add 3rd-generation cephalosporin
• Abscess associated with HIV infection assumed to be due to Toxoplasma gondii
- Daily doses of sulfadiazine and pyrimethamine
- Therapy will be lifelong in AIDS patients.
• Anticonvulsants
- Phenytoin until abscess resolved or perhaps longer
- Obtain anticonvulsant levels
• Following a surgical procedure, use corticosteroids to reduce edema, such as Dexamethasone. Taper rapidly. Use is usually limited to 1 week. Continue antibiotics for 6-8 weeks.
• Contraindications: Sensitivity or allergy to any prescribed medications
• Precautions
- Sulfadiazine poorly water-soluble. Patients must maintain adequate hydration or risk developing crystalluria.
- Decrease dosage of penicillin in patients with renal dysfunction
- Monitor serum levels of anticonvulsants.
- A dose of pyrimethamine is required for the treatment of toxoplasmosis, which may approach toxic levels. The patient should be observed for folic acid deficiency and treated with folinic acid (leucovorin), 5-15 mg (PO, IM, IV) if necessary.
• Significant possible interactions: Refer to the manufacturer's literature.
SURGERY
Surgical therapy
• Mandatory when neurologic deficits are severe or progressive
• Used when the abscess is in the posterior fossa
• Abscess drainage via a needle under stereotactic CT guidance through a burr hole under local anesthesia is the most rapid and effective surgical method of treatment and may be repeated if needed.
• Craniotomy: If abscess is large or multilocular
• Abscess resulting from trauma
FOLLOW-UP
PROGNOSIS
Survival: >80% with early diagnosis and treatment
COMPLICATIONS
• Permanent neurological deficits
• Surgical complications
• Recurrent abscess
• Seizures
PATIENT MONITORING
• Postsurgical monitoring as needed
• Serial CT or MRI: To confirm progressive resolution, early detection, and management of complications
REFERENCES
1. Graham DI, Lantos PL, eds. Greenfield's Neuropathology. 9th ed. London: Arnold; 2002.
2. Osenbach RK, Loftus CM. Diagnosis and management of brain abscess. Neurosurg Clin North Am. 1992;3:403-420.
3. Rakel RE, ed. Conn's Current Therapy. Philadelphia, PA: Elsevier Saunders; 2005.
4. Ropper A, Victor M, eds. Adams and Victor's Principles of Neurology. 8th ed. New York, NY: McGraw-Hill; 2005.
5. Rowland LD, ed. Merritt's Textbook of Neurology. 10th ed. Baltimore, MD: Williams  Wilkins; 2000.

BORDERLINE PERSONALITY DISORDER

2009-01-20T05:26:00.000-08:00

BORDERLINE PERSONALITY DISORDER - Heath A. Grames, PhD
BASICS
DESCRIPTION
Beginning no later than adolescence or early adulthood, a consistent and pervasive pattern of an unstable affect and sense of self, impulsivity, and volatile interpersonal relationships. (1)[C]
• Common behaviors and variations (1)[C]
- Self mutilation (pinching, scratching, cutting)
- Suicide (ideation, history of attempts, plans)
- Splitting (idealizing then devaluing people and relationships)
- Presentation of helplessness or victimization
- Emotional pain (may look for physical diagnoses)
- May be high utilizer of medical services
ALERT
Geriatric Considerations
Illness (acute and chronic) may exacerbate borderline personality disorder behaviors and may lead to intense feelings of fear and helplessness. Manifestations may decrease with age. (1)[C]
Pediatric Considerations
Diagnosis is rarely made for children (1)[C]. Must 1st rule out Axis I disorders and behavior related to a general medical condition or to the developmental cycle of the child. For diagnosis, baseline behaviors must be representative of borderline personality disorder.
Pregnancy Considerations
Physical and social changes may induce stress or increase fears, causing increased borderline behaviors.
GENERAL PREVENTION
Tends to be a multi-generational problem. Children, caregivers, and significant others should have time and activities away from the borderline individual, which may help protect them from the disorder.
EPIDEMIOLOGY
• Predominant age: Onset no later than adolescence or early adulthood (may go undiagnosed for years) (1)[C]
• Predominant sex: Female > Male (1)[C]
Prevalence
• General population = ~2% (1)[C]
• Estimated lifetime prevalence = 10-13% (1)[C]
• Estimates of 20-30% (all personality disorders) prevalence in primary care outpatient settings (2)[C]
RISK FACTORS
Physical illness and external social factors may exacerbate borderline personality behaviors.
Genetics
1st-degree relatives are at greater risk for also having this disorder (3)[C] (undetermined whether risk is due to genetics or psychosocial factors).
ETIOLOGY
Undetermined, but generally accepted, that is due to a combination of the following (2)[C]
• Hereditary tempermental traits
• Environment (i.e., history of neglect and abuse, ongoing conflict in home)
• Developmental traits
ASSOCIATED CONDITIONS
• Mood disorders, common (1)[C]
• Anxiety disorders, common (1)[C]
• Substance-related disorders, common (1)[C]
• Eating disorders, common (1)[C]
• PTSD, common (1)[C]
• Co-occurring personality disorders, frequent (1)[C]

DIAGNOSIS
PRE HOSPITAL
• Assess suicide ideation and self-harm behavior
• Assess for psychosis
SIGNS AND SYMPTOMS
See "Description"
History
• Clinic visits for problems that do not have biological findings
• Problems with medical staff members
• Idealizing or unexplained anger at physician
• History of unrealistic expectations of physician (e.g., "I know you can take care of me." "You're the best, unlike my last provider.")
Physical Exam
Scarring from self-mutilating (look on arms and legs where hidden by clothing, but can occur on other parts of the body)
TESTS
Diagnostic Procedures/Surgery
Patient must meet at least 5 of the following (1)[C]
• Attempt to avoid abandonment
• Volatile interpersonal relationships
• Identity disturbance
• Impulsive behavior
- In at least 2 areas
- Impulsive behavior is self-damaging
• Suicidal or self-mutilating behavior
• Mood instability
• Feeling empty
• Unable or difficult to control anger
• Paranoid or dissociative when under stress
DIFFERENTIAL DIAGNOSIS
• Mood disorders (1)[C]
- Look at baseline behaviors when considering borderline vs. mood disorder
• Psychotic disorder (1)[C]
- With borderline, only occurs under intense stress and is not characteristic of disorder
• Other personality disorder (1)[C]
- Consider patients thoughts, feelings, and behavior to differentiate borderline from other personality disorders
- High co-occurrence of borderline and other personality disorders
TREATMENT
PRE-HOSPITAL
• Appropriate psychiatric care must be available.
• Patient may need to be on suicide watch
• Inpatient hospitalization is ineffective in changing Axis II disorder behaviors.
• Inpatient hospital services for conditions related to Axis II disorder should be limited and of short duration to decrease dependence (increased dependence may decrease likelihood of behavior change)
• Hospitalization should be considered for the following
- Adjust medications
- Implement psychotherapy for crisis intervention
- Stabilize patient (psychosocial stressors)
STABILIZATION
If psychotic, consider antipsychotic medications (2)[C]
GENERAL MEASURES
• Focus on patient management rather than "fixing" behaviors.
• Schedule follow-up to relieve patient stress. (4)[C]
• Meet with and rely on treatment team to avoid splitting of team by patient and to provide opportunity for team to discuss issues with patient
• As necessary, refer patient to mental health therapist
Nursing
Nurses can be helpful in managing patient and calling the patient as needed (contact with the patient helps relieve patient stress)
SPECIAL THERAPY
Consider referring patient for specialty mental health services, such as Dialectic Behavioral Therapy (DBT)
MEDICATION (DRUGS)
• There are no medications that treat borderline personality disorder
• Treat symptoms and Axis I disorders (2)[C]
First Line
• Depression/anxiety (4)[C]
- SSRI, Selective Serotonin-reuptake Inhibitors
• Impulsive, aggressive, or history of bi-polar disorder (2)[C]
- Mood stabilizer
• Psychosis, paranoid or hostile behavior, debilitating anxiety (2)[C]
- Atypical antipsychotic
FOLLOW-UP
• Schedule routine follow-up with patient (relieves patient anxiety about medical care relationship with physician) (4)[C]
• Focus should be on medical conditions and co-morbid Axis I disorders (4)[C]
DISPOSITION
Admission Criteria
Refer to inpatient or outpatient psychiatry services if harm to self or others is expressed
• Call police or admit for inpatient services immediately if patient is psychotic and/or presents risk of harm to self or others
Discharge Criteria
• Patient should not present risk of harm to self or others
• Patient should have safety plan
• Routine follow-up should be scheduled with psychiatrist, mental health therapist, or primary care provider
Issues for Referral
• If hospitalized, probably for suicide risk, mood or anxiety disorders, or substance-related disorders
• Urgency for scheduled follow-up depends on community resources (i.e., do outpatient day programs for suicidal patients exist? What substance abuse programs are available?)
- With increased risk for self harm or self-defeating behaviors and low community resources, the patient can/will use increased need for frequent visits
• Treatment of Axis II disorder should include psychotherapy and/or psychiatry.
PROGNOSIS
• Borderline behaviors may decrease with age (1)[C]
• Treatment is complex and takes time
• Medical focus is on patient management and caring for medical and Axis I disorders (5)[C]
PATIENT MONITORING
Monitor for suicidal or other self-harm behaviors
REFERENCES
1. American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders, 4th ed. Washington, DC: American Psychiatric Association; 1994.
2. Ward RK. Assessment and management of personality disorders. American Family Physician. 2004;70:1505-1512.
3. Koenigsberg HW, Woo-Ming AM, Siever LJ. Pharmacological treatments of personality disorders. In: Nathan PE, Gorman JM, eds. A guide to treatments that work, 2nd ed. New York, NY: Oxford University Press; 2002:625-641.
4. Feder A, Robbins SW, Ostermeyer B. Personality disorders. In: Feldman MD, Christensen JF, eds. Behavioral medicine in primary care: A practical guide, 2nd ed. New York, NY: McGraw-Hill; 2003:231-252.
5. Makela EH, Moeller KE, Fullen JE, Gunel E. Medication utilization patterns and methods of suicidality in borderline personality disorder. The Ann Pharmacother. 2006;40:49-52.

BOTULISM

2009-01-20T05:25:00.001-08:00

BOTULISM - John C. Bradford, DO
BASICS
DESCRIPTION
• An intoxication-producing paralytic disease caused by neurotoxins of Clostridium botulinum, the most toxic substances known to science
• The toxin prevents acetylcholine release at presynaptic membranes, blocking neuromuscular transmission in cholinergic nerve fibers.
• 5 forms exist
- Food-borne botulism
- Infantile botulism
- Wound botulism
- Hidden or intestinal botulism
- Inadvertant botulism
- Inhalation (potential sixth form through deliberate release of toxin)
• System(s) Affected: Endocrine/Metabolic; Gastrointestinal (GI); Nervous
• Synonym(s): Sausage poisoning; Kerner disease
ALERT
Pediatric Considerations
Avoid honey in the 1st year of life.
GENERAL PREVENTION
• Avoid giving honey to infants.
• Do not eat or taste food from bulging cans, or if food smells "off."
EPIDEMIOLOGY
• Predominant age
- Food-borne: All ages
- Infantile: 2-4 months (rare after 6 months)
- Wound: Usually younger adult
- Inadvertent: >1 year
• Predominant sex
- Food-borne and infantile: Male = Female
- Wound: Male > Female
- Inadvertent: Female > Male
Incidence
• Average of 110 cases per year (25% food-borne, 72% infant)
• Wound botulism incidence increasing due to IV heroin use and cocaine abuse
• Hidden or intestinal: More common in disorders of the GI tract, such as prior surgery, Crohn disease, or recent antibiotic use
• Inhalation: Only a single incident involving 3 laboratory workers has been described.
RISK FACTORS
• Food-borne: Ingestion of home-canned or prepared foods
• Infantile: From ingestion of honey; breastfeeding (controversial)
• Wound: IV drug use (e.g., black tar heroin) or "skin popping." Sinusitis secondary to cocaine use also reported.
ETIOLOGY
• Ingestion of C. botulinum neurotoxins (A, B, and E most common)
• Food-borne, usually from home-canned vegetables, prepared foods, or foods incubated in anaerobic conditions
• Infantile from ingestion of spores in environment or occasionally in honey
• Wound due to contamination with toxin-producing C. botulinum
• Hidden or intestinal (source yet identified)
• InadvertentIM injections of botulinum toxin

DIAGNOSIS
SIGNS AND SYMPTOMS
• Food-borne
- Onset 2-36 hours after ingestion, as long as 14 days
- Nonspecific findings early (nausea, vomiting, malaise, dizziness, and abdominal distension)
- Dry mouth
- Constipation, urinary retention
- Symmetric descending weakness or paralysis of motor and autonomic nerves, usually beginning with the cranial nerves
- Cranial nerve paralysis (ptosis; extraocular muscle paresis; fixed, dilated pupils; dysphagia)
- Postural hypotension
- Muscle weakness, respiratory paralysis (no sensory deficits)
- Afebrile
- Progression over several days
• Infantile
- Constipation (early sign)
- Loss of head control
- Loss of suck
- Loss of facial expression and verbalization
- Symmetric descending weakness and cranial nerve paresis similar to food-borne form
- Diminished or absent deep tendon reflexes
- Autonomic dysfunction
- Afebrile
- Usual progression over 2-5 days; can be short as a few hours
• Wound
- Onset 4-14 days postinjury
- Findings similar to food-borne botulism, but GI symptoms less common
- May be febrile
• Hidden
- Possible adult variant of infant botulism
- Findings similar to infant botulism
- Inadvertent
- Moderate to marked clinical weakness following IM therapeutic injections
- Autonomic nervous system deficits also reported
- Inhalation
- Likely as irritant upper airway prodrome followed by variable degrees of paralysis
TESTS
• Stool contains organism and toxin
• Serum toxin present in food-borne form
Lab
• Routine testscheck for hypokalemia
• CSF testingnormal helps differentiate from Guillain-Barre syndrome
• Toxin detected in gastric contents, blood, feces, and suspected food and containers
• Confirmation available at Centers for Disease Control and prevention (CDC) and some state laboratories
• Pulmonary function testing
• Disorders that may alter lab results
- Underlying myoneural disease
Diagnostic Procedures/Surgery
Electrophysiology testing can provide presumptive evidence of botulism in patients with the clinical picture and in whom bioassay studies are negative. The most consistent finding is a smaller amplitude evoked muscle action potential on repetitive nerve stimulation with incremental response.
Pathological Findings
Nonspecific
DIFFERENTIAL DIAGNOSIS
• Guillain-Barre syndrome
• Encephalitis
• Tick paralysis
• Myasthenia gravis
• Eaton Lambert myasthenic syndrome
• Basilar artery stroke
• Congenital neuropathy or myopathy
• Sepsis
• Hypokalemic periodic paralysis
• Polio
• Other poisonings (organophosphate, shellfish, Amanita mushrooms, atropine, and aminoglycosides)
• Miller Fisher variant of Guillian-Barre syndrome
• Diphtheritic neuropathy
TREATMENT
Advanced medical and nursing supportive care with special attention to respiratory status; aggressive airway management for those at risk for respiratory failure
GENERAL MEASURES
• Meticulous airway management
• Monitor pulmonary function
• Physical therapy with range of motion exercise and assisted ambulation as tolerated
• Prevention of decubiti
Diet
Nasogastric feedings, if needed
Activity
Bed rest initially
MEDICATION (DRUGS)
First Line
• Antitoxin therapy with trivalent A-B-E antitoxinCall CDC Assistance, (770) 488-7100 for help in locating and acquiring the antitoxin. A single vial IV provides adequate serum levels.
• Most benefit from antitoxin in cases with rapidly progressive paralysis.
• Early administration important
• Horse serum derived: Up to 20% reaction incidence. Consider skin testing or pretreatment with steroids or antihistamines.
• Infantile
- Treatment with Human Botulism Immune Globulin recommended by some authors
- Available only through the California State Health Department (510) 540-2646
• Wound
- Antitoxin therapy with trivalent A-B-E antitoxin, one vial IV and one vial IM, repeat in 2-4 hours if persistent symptoms
- Antibiotics unproven by clinical trial but widely used and recommended
 Penicillin G (3 million units IV q4h in adults)
 Metronidazole (500 mg IV q8h) for penicillin-allergic patients
- Vaccinepentavalent vaccine available
 Efficiency in terrorist attack is unknown
 Newer vaccines being developed
• Contradictions: Previous reaction to horse serum-containing medications represents a relative contraindication to trivalent antitoxin. Human Botulism Immune Globulin has a greatly decreased risk of hypersensitivity reactions.
SURGERY
Wound excision debridement
FOLLOW-UP
PROGNOSIS
• Delay in administering toxin is the most important factor that affects clinical course and outcome
• Mortality: 10-40%
• Mortality for patients >60 years twice that of younger patients
• Full recovery may take months.
COMPLICATIONS
• Aspiration pneumonia
• Nosocomial infection
• Hypoxic tissue damage
• Death
PATIENT MONITORING
Cardiorespiratory monitoring during illness
REFERENCES
1. Horowitz BZ. Botulinum toxin. Crit Care Clin. 2005;21:825-839.
2. Cherington M. Botulinum: Update and review. Semin Neurol. 2004;24(2):155-163.
3. Arnon SS. Botulinum toxin as a biological weapon. JAMA. 2001;285(8):1059-1070.
4. Coffield JA. Botulinum neurotoxin: The neuromuscular junction revisited. Crit Rev Neurobiol. 2003;15(3-4):175-196.
MISCELLANEOUS
• Other notes
- Organism present in stools of 1-2% of healthy individuals
- Release of toxins in the gut may worsen symptoms of infantile botulism by bacterial lysis.
• See also: Food Poisoning; Bacterial

BONE TUMOR, PRIMARY MALIGNANT

2009-01-20T05:25:00.000-08:00

BONE TUMOR, PRIMARY MALIGNANT - Mark R. Dambro, MD
BASICS
DESCRIPTION
• Primary malignant bone tumors are rare. 4 types make up the majority
- Malignant fibrous histiocytoma (malignant fibrous histiocytoma): A pleomorphic sarcoma of storiform pattern without differentiation.
- Osteosarcoma: Similar to malignant fibrous histiocytoma with differentiation to osteoid production
- Chondrosarcoma: Cellular cartilaginous lesion with abundant binucleate cells, myxoid areas, and pushing borders
- Ewing sarcoma: Small, blue-round cell neoplasm
• System(s) Affected: Musculoskeletal
ALERT
Pregnancy Considerations
• Increased growth of musculoskeletal malignancies during pregnancy
• Soft-tissue desmoid tumors have estrogen and progesterone receptors.
GENERAL PREVENTION
None identified
EPIDEMIOLOGY
• Predominant age
- Malignant fibrous histiocytoma: Teens and elderly
- Osteogenic sarcoma: Teens and early 20s
- Chondrosarcoma: Very young and very old
- Ewing sarcoma: Children, teens, and early 20s
• Predominant sex: Male = Female
Incidence
• Rare
• 5,000 bone and soft-tissue sarcomas diagnosed per year in the US
• A practicing orthopedic surgeon may see 1 primary malignant tumor of bone in every 5 years of practice.
• Ewing sarcoma is less common in African Americans.
RISK FACTORS
• Multiple enchondromatosis (Ollier disease): Chondrosarcoma
• Multiple hereditary exostosis: Chondrosarcoma
• Previous irradiation, risk factor for malignant fibrous histiocytoma
• Previous history of bilateral retinoblastoma: Osteosarcoma
Genetics
• Ewing sarcoma has 11/22 chromosomal translocation and EW5-FLI-1 fusion protein.
• Osteosarcoma shows loss of retinoblastoma and p53 suppressor genes and amplification of the genes C-myc, mdm-2, SAS, and cyclin-dependent kinase.
ETIOLOGY
• Generally unknown
• Malignant fibrous histiocytoma often follows irradiation or arises in old bone infarct.
• Osteosarcoma has association with loss of suppressor retinoblastoma and p53 genes.
• Chondrosarcoma may arise in pre-existing enchondroma or exostosis.
ASSOCIATED CONDITIONS
• A higher incidence of chondrosarcoma is seen in patients with multiple hereditary exostosis, multiple enchondromatosis (Ollier disease), and patients with enchondromatosis and hemangiomatosis (Maffucci syndrome).
• Patients with enchondromatosis more often die of gastrointestinal (GI) malignancies than of metastatic chondrosarcoma.

DIAGNOSIS
SIGNS AND SYMPTOMS
• Pain with weight bearing, at rest, and at night
• Swelling
• Tenderness
• Fracture with minor trauma
• Minor injury may bring attention to lesion
TESTS
• A rectal exam should be done to exclude prostate nodules
• Open biopsy or needle biopsy. Needle biopsies may not provide enough tissue for frozen section, touch prep, permanent section, snap freezing, electron microscopy, cytogenetic and molecular studies, DNA indices, immunoperoxidase staining, and immunophenotyping (lymphoma).
• Biopsy of associated soft-tissue mass may lessen the risk of pathologic fracture.
• Biopsy tract should be excised in continuity with the tumor at the time of resection.
Lab
• 50% of osteosarcomas have an elevated alkaline phosphatase.
• Ewing sarcoma may be associated with an elevated ESR and lactate dehydrogenase.
• Prostatic-specific antigen to exclude prostatic carcinoma
• Calcium, phosphate, alkaline phosphatase
• Thyroid function tests to exclude thyroid carcinoma
• Elevated ESR and WBCs in osteomyelitis
• Serum protein electrophoresis and urine electrophoresis to exclude myeloma
Imaging
• Plain films provide the most important information regarding the nature of the lesion and guide further testing.
• Bone scan is done prior to biopsy, to look for other lesions.
• CT scan for cortical destruction and internal calcification or ossification. Abdominal CT, MRI, or renal ultrasound to exclude hypernephroma
• MRI scan determines the extent of marrow involvement and associated soft-tissue mass.
• Chest radiograph and chest CT for metastatic disease
• Mammogram to exclude breast carcinoma
Diagnostic Procedures/Surgery
Bone biopsy
Pathological Findings
• Histology and special studies in combination with radiographic findings confirm the diagnosis.
• Ewing sarcoma expresses MIC-2 protein (CD99).
• Electron microscopy
- Glycogen granules in Ewing sarcoma
- Neurosecretory granules in neuroectodermal tumors
- Birbeck bodies in histiocytosis-X
• Osteosarcoma may express Her-2/neu, indicating, if present, a more aggressive tumor, but one that may respond more favorably to trastuzumab (Herceptin).
DIFFERENTIAL DIAGNOSIS
• Solitary metastatic lesion or myeloma, especially in patients >40 years
• Lymphoma at any age
• Benign bone tumors and benign bone tumors that look aggressive (aneurysmal bone cyst, giant cell tumor, eosinophilic granuloma)
• Infection (osteomyelitis)
• Metabolic bone disease (osteopenia, Paget, hyperparathyroidism)
• Synovial diseases (pigmented villonodular synovitis, synovial chondromatosis, degenerative or inflammatory synovitis)
• Myositis ossificans and repair reaction to trauma
• Avascular necrosis
TREATMENT
Inpatient surgery
GENERAL MEASURES
Diet
No special diet
Activity
Varies with stage of disease and treatment
MEDICATION (DRUGS)
First Line
• These drugs are administered according to specific protocols. Other protocols may be appropriate.
• Malignant fibrous histiocytoma and osteosarcoma
- Doxorubicin (Adriamycin)
- Intra-arterial and intravenous cisplatin
- High-dose methotrexate with leucovorin rescue
- Ifosfamide (with mesna to protect against hemorrhagic cystitis)
- Cyclophosphamide (Cytoxan)
- Dactinomycin (actinomycin-D)
- Bleomycin
• Liposome-encapsulated muramyl tripeptide phosphatidylethanolamine immune-modulating agent for osteosarcoma (under trial in Cancer Center Support Group and Pediatric Oncology Group studies)
• Ewing sarcoma
- Cyclophosphamide
- Vincristine
- Actinomycin D
- Doxorubicin (Adriamycin)
- Ifosfamide
- Etoposide
• Contraindications: Refer to manufacturer's literature.
• Precautions
- Left ventricular dysfunction with Adriamycin. Cumulative dose >450 mg/m2 increases risk. Follow with serial echocardiograms and/or nuclear multiple-gated acquisition ventriculogram scans when cumulative dose >250 mg/m2.
- With high-dose methotrexate, hydration, alkalinization of the urine, and close monitoring of plasma levels are needed.
• Significant possible interactions
- Myelosuppression
- Renal tubular dysfunction with ifosfamide
- Renal and hepatic dysfunction and GI mucositis with methotrexate
- Nephrotoxicity and ototoxicity with cisplatin
Second Line
Ondansetron (Zofran), dronabinol (Marinol), metoclopramide (Reglan), and others for nausea control.
SURGERY
• Resection with adequate margin is required to minimize risk of local persistence.
• For malignant fibrous histiocytoma and osteosarcoma, preresection neoadjuvant chemotherapy treats micrometastatic disease immediately, allows time for ordering replacement prosthesis and bone graft, allows for an in vivo assessment of the chemotherapy responsiveness of the tumor, and may facilitate limb salvage by allowing a "safer" close margin.
• Chondrosarcoma in the extremities should be treated exclusively by surgery unless it is of the mesenchymal or dedifferentiated high-grade variety.
• Ewing sarcoma was traditionally treated with chemotherapy, and surgery was limited to those lesions that were extremely large, associated with pathologic fracture, or involved an expendable bone. Most Ewing sarcoma lesions were irradiated. However, despite irradiation, local recurrence is common in up to 25% with pelvic lesions. Therefore, surgery with limb salvage is increasingly accepted. A dramatic decrease in size in Ewing sarcoma occurs after initial chemotherapy, and a decision can then be made after restaging as to whether to irradiate or to resect the primary lesion.
• The treatment goal is to minimize local recurrence while preserving function. Limb salvage is employed whenever a safe margin can be obtained.
FOLLOW-UP
PROGNOSIS
• With amputation alone, 80% of patients with osteosarcoma had pulmonary metastatic disease by 2 years. With chemotherapy, the 5-year disease free survival rate is 50-85%.
• Favorable prognostic factors for malignant fibrous histiocytoma and osteosarcoma include responsiveness to chemotherapy, distal portions of the extremities, small size, age >10 years.
• Most chondrosarcomas are of lower grade and have a low risk of metastatic spread and low incidence of local recurrence after adequate surgery.
• Malignant fibrous histiocytoma, osteosarcoma, and Ewing sarcoma have an overall 50% survival with combined treatment modalities.
COMPLICATIONS
• Limb salvage with any primary malignant bone tumor is fraught with potential complications.
• Micrometastatic disease may have occurred at the time of presentation and can appear at any time during the course of treatment or follow-up.
• Local recurrence risk for osteosarcoma with limb salvage is 10%.
• Leg-length discrepancy, infection, wound dehiscence, skin coverage problems, arterial and nerve injury, nonunion of bone grafts, and mechanical loosening of prosthetic implants can occur.
• Thoracotomy and continued chemotherapy are often recommended for metastatic disease to the lung.
• Ewing sarcoma, metastatic to the lung, is quite diffuse and is less amenable to thoracotomy.
PATIENT MONITORING
• Patients who require adjuvant chemotherapy are treated with maintenance chemotherapy after resection of the tumor.
• Blood counts for myelosuppression
• Serial echocardiograms when Adriamycin is being used; G-CSF often used to minimize neutropenia
• Chest radiographs obtained every 2 months for the 1st year, every 3 months for the 2nd year, and every 4 months in the 3rd year
• CT scans of the lungs are initially repeated every 6 months during 1st 2 years.
• Ewing sarcoma may recur >5 years after diagnosis.
REFERENCES
1. Enneking WF. Musculoskeletal Tumor Surgery, Vols. 1 and 2. New York: Churchill Livingstone, 1983.
2. Mendelsohn J. Jeremiah Metzger Lecture. Targeted cancer therapy. Trans Amer Clin Climatol Assoc. 2000;111:95-110.
3. Schajowicz F, McGuire MH. Diagnostic difficulties in skeletal pathology. Clin Orthop Rel Res. 1991;240:281-310.
4. Longhi A, Pasini E, Bertoni F, et al. Twenty-year follow-up of osteosarcoma of the extremity treated with adjuvant chemotherapy. J Chemother. 2004;16(6):582-588.
5. Velez-Yanguas MC, Warrier RP. The evolution of chemotherapeutic agents for the treatment of pediatric musculoskeletal malignancies. Orthop Clin North Amer. 1996;27:545-549.
6. Womer RB. The cellular biology of bone tumors. Clin Orthop Rel Res. 1991;262:1221.
MISCELLANEOUS
• Osteosarcoma variants such as parosteal, periosteal, and intraosseous osteosarcoma are lower-grade lesions with a more favorable prognosis; they often do not require chemotherapy. Other variants, postirradiation, and post-Paget osteosarcoma metastasize early.
• Chordoma: Rare malignant bone tumor that develops from the remnants of the primitive notochord. May be located in the sacrum or near the base of the skull. Usual course: Slowly progressive; recurrent; cure possible.

BODY DYSMORPHIC DISORDER

2009-01-20T05:24:00.000-08:00

BODY DYSMORPHIC DISORDER - Jennifer L.Schott, MD
BASICS
DESCRIPTION
According to the DSM-IV-TR, body dysmorphic disorder is a preoccupation with an imagined defect in appearance that causes clinically significant distress or impairment in social, occupational, or other important areas of function that is not accounted for by another mental disorder. If there is a minor physical anomaly, the concern is excessive. (1)
EPIDEMIOLOGY
• May be equally common in men and women
• Different cultural beliefs may influence or amplify preoccupations.
• Usually begins during adolescence with an average age of onset of 17 years (1)
- Adolescents usually present similar to adults
- Can present in childhood, often with refusing to attend school or planning suicide (2)
• Onset can be gradual or abrupt
• Often a delay in diagnosis until 10-15 years after the onset (1)
Prevalence
• 0.7% in the general community
• 5-40% in individuals with Anxiety or Depressive Disorders (3)
• 6-15% in cosmetic surgery patients and in dermatologic clinics (1)
RISK FACTORS
• Genetic predisposition
• Shyness, perfectionism, or anxious temperament
• Childhood adversity
- Teasing or bullying
- Poor peer relationships
- Social isolation
- Lack of support of family
- Sexual abuse
• History of dermatological or other physical stigmata
• Being more aesthetically sensitive than average
• Low self esteem (3)
PATHOPHYSIOLOGY
• Not known
• A cognitive behavioral model has been described in which an external representation of the person's appearance creates a distorted mental image that through selective attention increases the awareness of the image and its specific features. The preoccupation of the distorted image is maintained by different safety and submissive behaviors to decrease the scrutiny by others but actually tends to increase the individual's doubts and reinforces the behavior.
• Possible lesions in the frontostriatal connections, which cause abnormal verbal and nonverbal encoding strategies leading to executive memory deficits (3)
ETIOLOGY
Not known but likely multifactorial involving genetic, biological, and environmental factors
ASSOCIATED CONDITIONS
• Depression
• Social phobia
• Bipolar disorder
• Eating disorders
• Obsessive-compulsive disorder
• Suicide
• Delusional disorder, somatic type (1)

DIAGNOSIS
SIGNS AND SYMPTOMS
• Preoccupation that 1 or more of their features are unattractive, ugly, or deformed
• Can involve any part of the body but usually involves the skin, hair, or facial features (1)
- Women are more likely to be preoccupied with their weight, hips, legs, and breasts
- Men are more likely to be preoccupied with their height, body hair, body build, and genitals (4,5)
• Nature of the preoccupation can change with time
• Have little insight
• Tend to display delusions of reference
• Large amounts of time are consumed by behaviors to examine the perceived defect repeatedly, disguise it, or improve it
- Mirror gazing
- Excessive grooming
- Camouflaging the "defect"
- Skin picking
- Reassurance seeking
- Dieting
- Pursuing dermatological treatment or cosmetic surgery
• Tend to avoid social interactions
• Trouble staying in school, maintaining a job, or maintaining significant relationships
- Tend to be unhappy with results of dermatologic and cosmetic procedures (1)
History
• Determine and validate the patient's concern
• Determine the severity of the disorder
• Quantify the amount of time spent worrying about the "distorted" appearance
• Determine what is done to hide or eliminate the problem
• Determine the degree to which the defect affects their school, job, or social life
• Rule out other psychiatric disorders (6)
Physical Exam
• Important to do a mental status examination
- Look for
 Depression
 Suicidal ideation
 Anxiety
- Rule out organic factors by reviewing
 Orientation
 Memory
 Ability to concentrate
• Rule out actual physical pathology
DIFFERENTIAL DIAGNOSIS
• Normal concerns about appearance
• Eating disorders
• Gender identity disorder
• Major depressive episode
• Narcissistic personality disorder
• Avoidant personality disorder
• Social phobia
• Schizophrenia
• Obsessive-compulsive disorder
• Trichotillomania
• Hypochondriasis
• Delusional disorder, somatic type
• Koro: A culture-related syndrome seen in Southeast Asia
- Involves a preoccupation that the genitals (penis, labia, nipples, or breast) is shrinking and is disappearing into the abdomen (1)
TREATMENT
SPECIAL THERAPY
• Refer to a psychiatrist for diagnosis and therapy
• Cognitive behavior therapy has been shown to be very effective (7)[B]
- Behavioral experiments
- Graded exposure tasks
- Imagery rescripting
- Cognitive restructuring
- Reverse role playing
- Relaxation
• Support groups (7)[C]
• Psychotherapy may be effective (7)[C]
• Therapy with and for family members, spouses, or significant others
MEDICATION (DRUGS)
First Line
Selective Serotonin-reuptake inhibitors (SSRI) (7)[B]
• Not an approved use by the FDA
• Patients with and without a delusional disorder did equally well with an SSRI
• Maximum tolerated dose should be taken for at least 12-16 weeks
• Dosages may need to be higher than typically recommended for an eating disorder (3,6,7)
Second Line
Adding a low dose antipsychotic drug to an SSRI if there is failure to respond to 2 or more SSRIs (2)[C]
SURGERY
Cosmetic surgery and dermatologic procedures may have potential benefits or no benefit
• Difficult patients for dermatologists and plastic surgeons due to tendency to insist on repeated procedures, are often unhappy with the results, and require repeated reassurance (3)
FOLLOW-UP
PROGNOSIS
Continuous course with periods of waxing and waning in the intensity of symptoms
• The longer the duration and the more severe the symptoms, the less the chance of partial or full remission (8)
COMPLICATIONS
• Repeated surgical or dermatological procedures
• Suicide
• Comorbid conditions
• Poor social relations
• Poor self esteem
• Inability or limited ability to function in society
PATIENT MONITORING
• Close monitoring by psychiatrist
• Regular counseling
REFERENCES
1. American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, Fourth Addition, Text Revision. Washington, DC: American Psychiatric Association, 2000;507-510.
2. Albertini RS, Philips KA. Thirty-Three cases of body dysmorphic disorder in children and adolescents. J Am Acad Child Psy. 1999;38:453-459.
3. Veale D. Body dysmorphic disorder. Postgrad Med J. 2004;80:67-71.
4. Perugi G, Akiskal H, Giannotti D, Frare F, Di Vaio S, Cassano G. Gender-related differences in body dysmorphic disorder. J Ner Ment Dis. 1997;185:578-582.
5. Philips KA, Diaz SF. Gender differences in body dysmorphic disorder. J Ner Ment Dis. 1997;185:570-577.
6. Slaughter JR, Sun SM. In pursuit of perfection: A primary care physician's guide to body dysmorphic disorder. Am Fam Physician. 1999;60:1738-1742.
7. Ipser JC, Stein DJ. Pharmacotherapy and psychotherapy for body dysmorphic disorder. The Cochrane Library. 2005;4.
8. Philips KA, Pagano ME, Menard W, Fay C, Stout RL. Predictors of remission from body dysmorphic disorder: A prospective study. J Ner Ment Dis. 2005;193:564-567.

BLEPHARITIS

2009-01-20T05:23:00.000-08:00

BLEPHARITIS - Joshua J. Spooner, PharmD, MS; A. Raquel Matteo-Bibeau, MD
BASICS
DESCRIPTION
• An inflammatory reaction of the eyelid margin
- Usually occurs as seborrheic or as staphylococcal blepharitis.
- Multiple types may coexist.
• System(s) Affected: Skin/Exocrine
• Synonym(s): Granulated eyelids
EPIDEMIOLOGY
• Predominant age: Adult
• Predominant sex: Male = Female
Incidence
One of the most common ocular disorders
RISK FACTORS
• Seborrheic dermatitis
• Contact dermatitis
• Herpes simplex dermatitis
• Varicella-zoster dermatitis
• Acne rosacea
• Diabetes mellitus
• Immunocompromised state (e.g., AIDS, chemotherapy)
• Isotretinoin use
• Dry eye syndromes
ETIOLOGY
• Seborrheic
- Accelerated shedding of skin cells with associated sebaceous gland dysfunction
- Malassezia furfur (formerly Pityrosporum ovale) yeasts often colonize
• Staphylococcal
- Superinfection of Zeis glands of lid margin and meibomian glands posterior to lashes with Staphylococcus aureus
- Usually part of mixed blepharitis
• Meibomian gland dysfunction
- Obstruction and inflammation of the meibomian glands
 Associated with acne rosacea, acne vulgaris, and oral retinoid therapy
• Other types of blepharitis
- Ulcerative blepharitis
 More severe blepharitis with small marginal ulceration and destruction of the hair follicles
- Contact dermatitis/blepharitis
 Develops from type IV hypersensitivity; common causes include ocular medications, topical anesthetics, antivirals, and cosmetics
 May occur with secondary Staphylococcus infection
- Eczematoid blepharitis
 Caused by hypersensitivity reaction to exotoxins and antigens from local flora
 Strong association with eczema, asthma
 Staphylococcal infection common
- Angular blepharitis
 Often caused by Staphylococcus or Moraxella infection
ASSOCIATED CONDITIONS
See "Risk Factors"

DIAGNOSIS
SIGNS AND SYMPTOMS (1)[c]
• Frequently reported in all types of blepharitis
- Burning
- Itching
- Eyelid erythema
- Conjunctival injection (red eyes)
- Lacrimation, tearing
- Tear deficiency
- Foreign body sensation
- Photophobia (light sensitivity)
- Impaired vision
• Staphylococcal
- Recurrent stye (external or internal hordeolum)
- Missing, broken, or, misdirected eyelashes (trichiasis)
- Eyelid deposits: Matted, hard scales; collarettes (ringlike formation around the lash shaft)
- Ulcerations at base of eyelashes (rare)
- Eyelid scarring may occur
• Seborrheic blepharitis
- Eyelid deposits: Dry flakes, oily or greasy secretions on lid margins and/or lashes
- Associated dandruff of scalp, eyebrows
• Meibomian gland dysfunction
- Eyelash misdirection may occur with longstanding disease
- Eyelid deposits: Fatty deposits; may be foamy
- Eyelid margin thickening
- Plugged meibomian gland orifices
- Chalazion (sometimes multiple)
- Eyelid scarring with long-term disease
• Mixed blepharitis
- Signs and symptoms of more than 1 type of blepharitis may be present
History
• Duration of symptoms (1)[C]
• Unilateral or bilateral presentation (1)[C]
• Note any exacerbating conditions (e.g., smoke, allergens, wind, contact lenses, etc.) (1)[C]
• Symptoms related to systemic diseases (1)[C]
• Current and recent medication use (1)[C]
• Recent exposure to infected individuals (1)[C]
Physical Exam
• Test of visual acuity (1)[C]
• External examination (skin and eyelids) (1)[C]
TESTS
Lab
Special tests
• Cultures in atypical blepharitis
• Biopsy in atypical cases for carcinoma
Imaging
Slit-lamp biomicroscopy (1)[C]
• Examine tear film, eyelid margins, eyelashes, tarsal and bulbar conjunctiva, and cornea:
- Reveals loss of lashes (madarosis), whitening of the lashes (poliosis), trichiasis, crusting, eyelid margin ulcers, and lid irregularities
DIFFERENTIAL DIAGNOSIS
• Masquerade syndrome:
- Persistent inflammation and thickening of eyelid margin may indicate squamous cell, basal cell, or sebaceous cell carcinoma masquerading as blepharitis.
 These carcinomas may also mimic styes or chalazia.
- Sebaceous carcinoma of the eyelid has a 22% fatality rate. Up to 1/2 of these potentially fatal sebaceous cell carcinomas may resemble benign inflammatory diseases, particularly chalazia and chronic blepharoconjunctivitis.
- Consider this in all cases of recurrent, persistent, or atypical chalazion; chronic unilateral unresponsive blepharoconjunctivitis; diffuse or nodular tumors of the eyelid; orbital mass developing after removal of an eyelid or caruncular tumor; and any tumor developing in a person with a history of ocular radiotherapy (2)[C].
TREATMENT
Best evidence treatment
• American Academy of Ophthalmology Cornea/External Disease Panel, Preferred Practice patterns Committee. Preferred Practice Pattern: Blepharitis. San Francisco: AAO. 2003.
GENERAL MEASURES
• Appropriate health care: Outpatient
• Promote proper eyelid hygiene (1)[C]
- Apply warm compresses for several minutes once daily to soften adherent encrustations
- The eyelid margins are then gently scrubbed with eyelid cleanser or diluted baby shampoo twice a day, to remove adherent material and clean the meibomian gland orifices (3)[C]
• Brief, gentle massage of the eyelids can help express meibomian secretions in patients with meibomian gland dysfunction (1)[C]
• Discontinue soft contact lenses use during an acute case of blepharitis.
- Chronic recurrent blepharitis requires referral to ophthalmologist for evaluation as to whether patient should continue in lenses.
MEDICATION (DRUGS)
First Line
• Topical treatment to lid, if Staphylococcus likely: Follow eye hygiene with application of bacitracin 500 u/g or (second choice) erythromycin 0.5% ophthalmic ointment:
- Apply with a cotton-tipped applicator
- The frequency and duration of treatment guided by the severity (1)[C].
• Topical corticosteroids (short-term) may be useful for eyelid or ocular surface inflammation.
- The minimum effective dose should be used; long-term use avoided if possible (1,4)[C]
• For patients with meibomian gland dysfunction inadequately controlled with eyelid hygiene, consider: Doxycycline 100 mg/d or tetracycline 1000 mg/d in divided doses, tapered after clinical improvement (2-4 weeks) to doxycycline 50 mg/d or tetracycline 250-500 mg/d (1)[C].
• As aqueous tear deficiency is common in blepharitis, use twice-daily artificial tears in addition to eyelid hygiene and medications.
• Contraindications: Allergy to medication; tetracyclines are not for use in pregnancy, nursing women, or in children 8 years.
• Precautions: Tetracyclines may cause photosensitivity; sunscreen recommended. Corticosteroids may increase intraocular pressure and risk of cataract.
• Significant possible interactions: Tetracyclines; avoid concurrent administration with antacids, dairy products, or iron. May potentiate the effect of warfarin. Broad-spectrum antibiotics may reduce the effectiveness of oral contraceptives; barrier method recommended.
Second Line
• Topical fluoroquinolones (gatifloxacin 0.3%, levofloxacin 0.5%, or moxifloxacin 0.5%) may be helpful for persistent or recurrent staphylococcal blepharitis or for those patients who prefer a solution.
• Seborrheic blepharitis may respond to antifungal agents, such as a short course of itraconazole (5)[C].
FOLLOW-UP
PROGNOSIS
• Symptoms can frequently be improved but are rarely eliminated.
• Long-term eyelid hygiene required for control.
COMPLICATIONS
• Stye and chalazion
• Scarring of eyelid margin
• Corneal infection
PATIENT MONITORING
• Patients should schedule a return visit if their condition worsens despite treatment.
• Return visit intervals for patients with severe disease vary.
• If corticosteroid prescribed, re-evaluate within a few weeks to measure intraocular pressure and determine response to therapy.
REFERENCES
1. American Academy of Ophthalmology Cornea/External Disease Panel, Preferred Practice patterns Committee. Preferred Practice Pattern: Blepharitis. San Francisco: AAO. 2003.
2. Tsai T, O'Brien JM. Masquerade syndromes: Malignancies mimicking inflammation in the eye. Int Ophthalmol Clin 2002;41:115-131.
3. McCulley JP, Shine WE. Changing concepts in the diagnosis and management of blepharitis. Cornea 2000;19:650-658.
4. Abelson MB, Cohane K, Fink K. Blepharitis: Hiding in plain sight. Rev Ophthalmol May 15. 2004.
5. Ninoyima J, et al. A case of seborrheic blepharitis: Treatment with itraconazole. Nippon Ishinkin Gakkai Zasshi 2002;43:189-191.
ADDITIONAL READING
• Lemp MA. Contact lenses and associated anterior segment disorders: Dry eye, blepharitis, and allergy. Ophthalmol Clin North Am 2003;16:463-469.
• McCulley JP, Shine WE. Eyelid disorders: The meibomian gland, blepharitis, and contact lenses. Eye  Contact Lens 2003;29(1S):S93-95.
• Rao NA, Hidayat AA, McLean IW, Zimmerman IE. Sebaceous carcinomas of the ocular adnexa: A clinicopathologic study of 104 cases, with five year follow-up data. Hum Pathol 1982;13:113-122.
• Frucht-Pery J, Sagi E, Hemo I, Ever-Hadani O. Efficacy of doxycycline and tetracycline in ocular rosacea. Am J Ophthalmol 1993;116:88-92.
MISCELLANEOUS
See also: Conjunctivitis; Dry eye syndrome (keratoconjunctivitis sicca)

BLASTOMYCOSIS

2009-01-20T05:22:00.000-08:00

BLASTOMYCOSIS - William G.Gardner, MD, MACP
BASICS
DESCRIPTION
• An uncommon, systemic infection caused by the dimorphic fungus Blastomyces dermatitidis
• System(s) Affected: Pulmonary; Skin/subcutaneous; Bone/joint; Genitourinary; Central nervous system (CNS)
• Synonym(s): North American blastomycosis
ALERT
Geriatric Considerations
Prognosis is worse in elderly patients with significant underlying pulmonary or renal disease.
Pediatric Considerations
Uncommon in children
Pregnancy Considerations
• Amphotericin B is drug of choice
• Azoles should not be used during pregnancy.
GENERAL PREVENTION
• Unknown
• Condoms for sexual encounters
EPIDEMIOLOGY
• Incompletely understood
• Predominant age: Adults, but 10-20% of cases in children in endemic areas
• Predominant sex: Male > Female
Incidence
Ranges from 0.3-1.5 cases per 100,000 population per year
Prevalence
Higher prevalence in endemic areas
• Midwestern, Southcentral US, Great Lakes region of US and Canada
• Large outbreak occurred in Wisconsin
• Sporadic cases around the world
RISK FACTORS
• Occupational or recreational exposure to soil containing spores of Blastomyces dermatitidis
• Residence in endemic areas
• HIV/AIDS or other immunocompromised states (corticosteroids, blood malignancies)
Genetics
No genetic predisposition known
ETIOLOGY
• Infection acquired by respiratory route
• Inhalation of spores of Blastomyces dermatitidis into lungs with lymphohematogenous dissemination to other organ systems
• Primary inoculation of skin may occur rarely.
• Female infection results from sexual contact.
• Reactivation of latent infection or reinfection may occur in immunocompromised patients
ASSOCIATED CONDITIONS
• Most infected persons have no predisposing conditions
• Occasionally occurs in HIV-infected or immunocompromised persons

DIAGNOSIS
SIGNS AND SYMPTOMS
• Acute pulmonary infection
- Onset may be abrupt or insidious
- Incubation period 30-45 days
- Presents as a non-specific flu-like illness
- Fever, chills, myalgias, arthralgias
- Cough nonproductive
- Often self-limiting
- Severe disease and respiratory failure occurs in 10% of cases
• Chronic pulmonary infection
- Chronic pneumonia, indolent onset
- Weight loss, fever, night sweats
- Productive cough, purulent sputum
- Hemoptysis uncommon
- May mimic tuberculosis, other fungal pneumonias, and cancer
• Cutaneous blastomycosis
- Most common extrapulmonary type: 80%
- May occur with or without pulmonary disease
- 2 types of lesions
 Verrucous lesions begin as small papulopustular lesions, become crusted, and have sharp borders, central clearing, scar formation, depigmentation, and microabscesses at periphery.
 Ulcerative lesions (initially pustules) form shallow ulcers with raised edges and granulating base.
- May be mistaken for pyoderma gangrenosum, squamous cell carcinoma, and other chronic infectious lesions (e.g., Sporotrichosis, atypical mycobacteria)
- SC nodules may suppurate forming chronic ulcers.
- Regional adenopathy (uncommon)
• Skeletal blastomycosis
- 10-50% of extrapulmonary cases
- Long bones, vertebrae, ribs, cranium most commonly involved
- Well-circumscribed osteolytic lesions
- May present with contiguous soft tissue abscesses and/or sinus tracts
- Paraspinous abscess in vertebral disease
- Acute or chronic arthritis may result from joint involvement, usually large joints.
• Genitourinary blastomycosis
- Occurs in 10-30% of cases
- Involves prostate, epididymis, and testes
- Enlarged, tender prostate
- May cause outflow obstruction
- Involvement of female genitalia uncommon but can be acquired through sexual contact
• Other
- CNS involvement with acute or chronic meningitis, epidural or cerebral abscesses: More common in AIDS
- Liver, spleen, pericardium, thyroid, gastrointestinal tract, and adrenal gland involved
TESTS
• Special staining of tissue with Gomori methenamine silver stain
• Periodic acid-Schiff's stain colors cell wall pink or red
• Mucicarmine stain helps differentiate from encapsulated Cryptococcus
Lab
• Culture of Blastomyces dermatitidis from tissue or body secretions on Sabouraud's media
- Slow growing
- Identify by highly specific DNA probes
• Yeast forms (5-15 um in diameter, with refractile cell wall, broad-based budding, and no capsule) in tissue or secretions by wet mount
• In pulmonary disease, potassium hydroxide prep of sputum reveals organism in >50%
• Serologic tests have variable sensitivity and low specificity and are not helpful in the diagnosis.
• Skin testing with Blastomycin is not useful.
• Disorders that alter lab results: Lidocaine inhibits growth in bronchoscopic cultures.
Imaging
• CT scan of head for CNS lesions
• CT scan or MRI of spine for vertebral lesions
• Bone scan for skeletal lesions
• Chest x-ray
- Acute pulmonary disease
 Alveolar or interstitial infiltrates
- Chronic pulmonary disease
 Upper lobe fibronodular infiltrates in 50%
 Mass lesions in 30%
 Pulmonary nodules  without cavitation
 Pleural effusion in 10%
 Mediastinal adenopathy in 20%
Diagnostic Procedures/Surgery
• Aspiration of abscess for wet mount and culture
• Needle or surgical biopsy of involved tissue for histology and culture
Pathological Findings
• Early response with polymorphonuclear leukocytes followed by granuloma formation with lymphocytes and macrophages
• Granulomas do not show caseation necrosis.
• Yeast is often found attached to or inside monocytes, macrophages, and giant cells.
DIFFERENTIAL DIAGNOSIS
• Pulmonary
- Acute bacterial pneumonia
- Tuberculosis
- Other fungal diseases
- Bacterial lung abscess
- Empyema
- Bronchogenic carcinoma
• Cutaneous
- Pyoderma gangrenosum
- Bacterial pyoderma
- Cutaneous mycobacterial infection
- Other cutaneous fungal infections (sporotrichosis, histoplasmosis, or ocryptococcosis)
- Squamous cell carcinoma
• Bone
- Bacterial osteomyelitis
- Tuberculosis
- Neoplastic disease
• Genitourinary
- Bacterial prostatitis
- Prostate cancer
- Other fungal infections
- Tuberculosis
TREATMENT
STABILIZATION
• Acute non-life-threatening pulmonary infection may be treated with itraconazole as an outpatient.
• Severe life-threatening infection, CNS disease, or disease in immunocompromised host should be treated initially with intravenous amphotericin B in the hospital.
GENERAL MEASURES
• Systemic antifungal therapy is indicated for all cases of extrapulmonary blastomycosis.
• Systemic antifungal therapy is indicated for all but very mild or asymptomatic pulmonary cases, in which a trial of observation may be appropriate.
Activity
No restrictions after patient is released from hospital
Nursing
Routine care
MEDICATION (DRUGS)
First Line
• Milder forms
- Itraconazole (Sporanox): 200 mg PO b.i.d. for at least 6 months. Take with food; antacids or hydrogen blockers result in lower serum levels. Little drug is excreted in urine; thus genitourinary disease is more resistant to therapy.
- Pediatrics: Non-life-threatening disease treat with itraconazole 5-7 mg/kg/d. Life-threatening or CNS disease treat with amphotericin B.
• Severe forms
- Amphotericin B (Fungizone): 0.5-0.8 mg/kg IV over 4-6 hours daily for a cumulative dose of 1.5-2.5 g (1st dose of amphotericin B is given as a test dose of 1 mg in 200 mL 5% dextrose in sterile water IV over 2-4 hours). If tolerated, give maintenance dose of 0.5-0.8 mg/kg/d. Rigors can be prevented by preinfusion dose of meperidine 50 mg. To reduce infusion-related fever, preinfusion acetaminophen and diphenhydramine.
• Contraindications
- Life-threatening intolerance to amphotericin such as anaphylaxis
- CNS disease: Amphotericin B (total dose 2 g); alternative fluconazole 800 mg/d because of good CNS penetration
• Precautions
- Monitor for hypotension during the infusion.
- Monitor renal function, serum sodium, potassium, and magnesium, complete blood count twice weekly
- Replace potassium and magnesium prn.
- When serum creatinine rises to 1.6 mg/dL (141 umol/L) or greater, dosage interval should be changed to 48 hours.
- Watch for phlebitis at infusion site.
- Consider peripherally inserted central catheter for infusion.
• Significant possible interactions
- Avoid use of potentially nephrotoxic drugs such as aminoglycosides, which may potentiate nephrotoxicity of amphotericin B.
- Itraconazole: Concurrent use of rifampin, phenytoin, or carbamazepine may increase hepatic metabolism, resulting in lower-serum drug levels and treatment failure.
Second Line
• Efficacy of alternative regimens not well established by controlled studies
- Fluconazole 400 mg daily for 6 months for non-life-threatening blastomycosis
- Ketoconazole (Nizoral): 400-800 mg PO daily for 6 months
• Lipid preparations of amphotericin B have not been adequately evaluated in human blastomycosis; they provide an alternative for patients unable to tolerate amphotericin B.
SURGERY
• Surgical debridement of bone lesions if there are areas of devitalized bone
• Surgical drainage of contiguous abscesses, cutaneous abscesses, or pleural empyema
FOLLOW-UP
DISPOSITION
Specialty referral
• Immunocompromised or HIV patients
• Severe pulmonary disease
• Deep abscesses
• Children
Admission Criteria
• Severe pulmonary disease
• Immunocompromised or AIDS patient with severe disease
Discharge Criteria
Clinically stable and responding to therapy
Issues for Referral
Follow-up with infectious diseases physician and primary care physician.
PROGNOSIS
• Cure in >90% with appropriate therapy
• Relapse in 10% of cases
• Immunocompromised and AIDS patients have a poorer prognosis
COMPLICATIONS
• Adverse reactions with amphotericin B are frequent and significant.
• Treatment-induced nephrotoxicity, electrolyte imbalance, and anemia
PATIENT MONITORING
• Monitor closely during early therapy.
• Monitor serum electrolytes, creatinine, and CBC twice weekly during amphotericin B therapy.
• Post-therapy follow-up every 3 months for 2 years, then twice yearly
REFERENCES
1. Chapman SW, et al. Practice guidelines for the management of patients with blastomycosis. Clin Infect Dis. 2000;30:679-683.
2. Pappas PG. Blastomycosis. Sem Resp Crit Care M. 2004;25:113-120.
3. Patel RG, et al. Clinical presentation, radiographic findings, and diagnostic methods of pulmonary blastomycosis: A review of 100 consecutive cases. South Med J. 1999;92:289-295.
4. Crampton TL, et al. Epidemiology and clinical spectrum of blastomycosis diagnosed at Manitoba hospitals. Clin Infect Dis. 2002;34:1310-1316.

BLADDER INJURY

2009-01-20T05:21:00.000-08:00

BLADDER INJURY - Mitchell Cahan, MD; Michael Ford, MD
BASICS
DESCRIPTION
• Injury to the bladder is the result of trauma, either blunt or penetrating.
• Rupture is associated with a full bladder and blunt injury.
• Very rarely, an operative complication or nontraumatic etiology is the cause.
• Classification
- Intraperitoneal rupture
- Extraperitoneal (retroperitoneal) rupture
• Associated ureter/urethral injury
GENERAL PREVENTION
Seat belts
Incidence
~0.5% of trauma patients (1)
Blunt injuries are associated with pelvic fracture in over 95% of cases.
RISK FACTORS
• High-energy mechanism (fall, MVA)
• Pelvic fracture
• Penetrating wound
• Prior bladder/pelvic surgery
• Pelvic radiotherapy
PATHOPHYSIOLOGY
Rupture can lead to urinoma or peritonitis
ETIOLOGY
• High-energy trauma
• Rupture due to increased pressure in nondistensible (full) bladder
• Laceration due to bone fragment or penetrating object (knife, bullet)
ASSOCIATED CONDITIONS
• Pelvic fracture
• Ureteropelvic disjunction
• Urethral injury; almost exclusively in males
• Peritonitis is unusual in bladder injury.

DIAGNOSIS
PRE HOSPITAL
Isolated bladder injury is rare. Typically patient has other serious injuries.
SIGNS AND SYMPTOMS
• Pelvic/suprapubic pain
• Blood at meatus
• Urinary retention
History
• High-energy deceleration injury (fall, MVA)
• Penetrating trauma
- Recent abdominal/pelvic surgery
Physical Exam
• Suprapubic tenderness to palpation
• Blood at meatus
• Scrotal/urethral hematoma
- Free-floating prostate
- High-riding prostate
TESTS
Lab
Blood on urinalysis
• Serum creatinine and K+ are elevated and Na+ is decreased in intraperitoneal ruptures.
• Serum labs are unchanged in extraperitoneal ruptures (3).
Imaging
• Cystography is the gold standard for diagnosis.
• High-resolution CT scans also are acceptable.
ALERT
Retrograde urethrography must be performed before placing a Foley catheter when urethral injury is suspected.
Diagnostic Procedures/Surgery
• Intraperitoneal ruptures and penetrating injuries require urgent operative management.
• Extraperitoneal ruptures may be treated with Foley catheter drainage alone.
- Consider suprapubic tube if drainage is needed for >10 days.
Pathological Findings
• Perivesicular hematoma
• Perforation at dome of bladder (in trigone, near urachus)
• Jagged tear in bladder
DIFFERENTIAL DIAGNOSIS
• Isolated urethral injury
• Isolated pelvic fracture
• Isolated ureteral injury
• Other visceral rupture
TREATMENT
PRE-HOSPITAL
• Cervical spine precautions
• Stabilize hemodynamics.
• Stabilize pelvis.
STABILIZATION
Stabilize pelvis.
GENERAL MEASURES
• Foley catheter placement
• Pain control
• Antibiotics
• Antispasmodics (Ditropan)
• Imaging diagnosis
Diet
No restrictions
Activity
No restrictions
Nursing
Foley to gravity
Physical Therapy
May be necessary for associated pelvic fractures
IV Fluids
Ringer's lactated solution or normal saline for initial resuscitation
MEDICATION (DRUGS)
• Analgesics
• Antibiotics
• Antispasmodics
First Line
• Narcotic pain control (i.e., morphine, hydromorphone); titrate to effect
• Broad-spectrum antibiotics, such as ciprofloxacin 500 mg b.i.d.
• Ditropan 5-10 mg t.i.d. p.r.n. for spasm
ALERT
There is concern about fluoroquinolones causing damage to cartilage in children.
Second Line
• Broad-spectrum antibiotics
• Antispasmodics (i.e., flavoxate)
SURGERY
• Urgent surgery is indicated for intraperitoneal bladder rupture.
• Extraperitoneal rupture usually is manageable conservatively.
FOLLOW-UP
DISPOSITION
Admission Criteria
Admit all patients with bladder rupture for surgery or observation.
Discharge Criteria
• Stable for transfer to rehab or can perform ADLs
• Extraperitoneal ruptures controlled with indwelling Foley catheter if rupture not healed
• No evidence of infection
• Pain controlled
Issues for Referral
• All bladder ruptures should be evaluated by a urologist or surgeon immediately.
• Patient should be seen in follow-up by a urologist.
PROGNOSIS
Full return to normal function
COMPLICATIONS
• Infection
• Peritonitis
• Stricture is a rare complication
• Death
REFERENCES
1. Inaba K, McKenney M, Munera F, et al. Cystogram follow-up in the management of traumatic bladder disruption. J Trauma. 2006;60(1):23-28.
2. Lunetta P, Penttila A, Sajantila A. Fatal isolated ruptures of bladder following minor blunt trauma. Ugeskr Laeger. 2005;167(49):4654-4659.

BLADDER CANCER

2009-01-20T05:20:00.000-08:00

BLADDER CANCER - Margaret E.Thompson, MD
BASICS
DESCRIPTION
Primary malignant neoplasms arising in the urinary bladder
• Most common type is transitional cell carcinoma (90%)
• Other types include adenocarcinoma, small cell carcinoma, and squamous cell carcinoma.
• Rhabdomyosarcoma of the bladder may occur in children
GENERAL PREVENTION
Smoking cessation
EPIDEMIOLOGY
Incidence increases with age (median age at diagnosis = 73 years)
• More common in men than in women (4:1)
• More common in Whites than Asians or African Americans
Incidence
• 36.0 per 100,000 men per year (1)
• 9.1 per 100,000 women per year
Prevalence
As of January 1, 2002, 367,550 men and 131,649 women in the United States (1)
RISK FACTORS
• Smoking is the single greatest risk factor
• Other risk factors
- Occupational carcinogens in dye, rubber, paint, plastics, metal, and automotive exhaust
- Schistosomiasis in Mediterranean (squamous cell cancer)
- History of pelvic irradiation
- Chronic lower urinary tract infection
- Chronic indwelling urinary catheter
- Cyclophosphamide exposure
- High-fat diet
- Chronic low fluid intake
- Slight increase in risk with prostate cancer
Genetics
Hereditary transmission unlikely, though transitional cell carcinoma pathophysiology is related to oncogenes; in particular, p56
PATHOPHYSIOLOGY
• 70-80% is superficial (in lamina propria or mucosa):
- Usually highly differentiated with long survival
• Initial event seems to be activation of an oncogene on chromosome 9
• 20% of tumors are invasive (deeper than lamina propria) at presentation:
- Tend to be high grade with worse prognosis
ETIOLOGY
• See "Risk Factors"
• Activation of oncogene on chromosome 9
ASSOCIATED CONDITIONS
Cigarette smoking

DIAGNOSIS
PRE HOSPITAL
Diagnosis depends on biopsy results obtained by cystoscopy, which is often performed in ambulatory site or as outpatient surgery
SIGNS AND SYMPTOMS
• Hematuriagross or microscopic, usually painless
• May have urinary frequency, urgency, nocturia
• Abdominal or pelvic pain in advanced disease
History
• Hematuria (gross or microscopic) (85-90%)
• Urinary symptomsfrequency, urgency
• Exposures (see "Risk Factors")
Physical Exam
• Normal in early cases
• Pelvic or abdominal mass in advanced disease
• Wasting in systemic disease
TESTS
Urinalysis is the initial test in patients presenting with gross hematuria or urinary symptoms
Lab
• Macroscopic hematuria (55% sensitivity, PPV 0.22 for urologic cancer) (2)[A]
• Urine cytology 54% sensitivity over all, (lower in less-advanced tumors), 94% specific (3)[A]
• Other urine markers
- NMP22: 67% sensitive, 78% specific (3)[A]
- BTA stat: 70% sensitive, 75% specific (3)[A]
• Bottom line: None of the urine markers is sensitive enough to rule out bladder cancer on its own. Cytology is the most specific. (3)[A]
Imaging
Done for staging and evaluating extent of disease, but not for diagnosis itself
• IV push to look at upper tracts if there is suspicion of disease there
• For invasive disease, metastatic workup should include chest x-ray, liver function tests, alkaline phosphatase
• Bone scan should be performed if the patient has bone pain or if alkaline phosphatase is elevated (4)[B]
• Urologic CT scan (abdomen, pelvis, with and without contrast) or MRI 40-98% accurate, with MRI slightly more accurate (4)[B]: Recommended if metastasis is suspected
Diagnostic Procedures/Surgery
• Cystoscopy is the gold standard for diagnosis, but one study showed that 33% of patients had residual tumor after transurethral resection of superficial tumor (4)[B]
• Transurethral resection of the bladder tumor (TURBT) with bladder washings
- Sensitivity of cytology on bladder washings for carcinoma in situ is nearly 100%
ALERT
Any patient who smokes and presents with microscopic or gross hematuria, or irritative voiding symptoms such as urgency and frequency, should be evaluated by cystoscopy for the presence of a bladder neoplasm.
Pathological Findings
• Characterized as superficial or invasive
• 70-80% present as superficial lesion
• Superficial lesions
- Carcinoma-in-situ (CIS, Tis): Flat lesion, high grade
- TaNon-invasive papillary carcinoma (Ta)
- T1Extends into submucosa, lamina propria
• Invasive cancer
- T2Invasion into muscle
 pT2ainvasion into superficial muscle
 pT2binvasion into deep muscle
- T3Invasion into perivesical fat
 pT3amicroscopic
 pT3bmacroscopic
- T4invasion into adjacent organs
 aT4ainvades prostate, uterus, or vagina
 aT4binvades abdominal or pelvic wall
- N1-N3invades lymph nodes
- Mmetastasis to bone or soft tissue
DIFFERENTIAL DIAGNOSIS
Includes differential diagnosis for hematuria
• Other urinary tract neoplasms
• UTI
• Prostatism
• Bladder instability
• Interstitial cystitis
• Uroltihiasis
• Interstitial nephritis
• Papillary urothelial hyperplasia
TREATMENT
STABILIZATION
Generally, hematuria from bladder cancer is not significant enough to cause hemodynamic compromise.
GENERAL MEASURES
Radiotherapy
• In the United States, used for patients with muscle-invasive cancer who are not surgical candidates
• Treatment of choice for muscle-invasive cancer in some European and Canadian centers:
- 65-70 Gy over 6-7 weeks is standard
• Chemotherapy with cis-platin combined with radiotherapy may preserve bladder function
MEDICATION (DRUGS)
• Intravesical Bacillus Calmette-Guerin (BCG) after TURBT in high grade lesions has been shown to decrease recurrence in Ta or T1 tumors (5)[A]
- Common regimen is weekly for 6 weeks, then monthly for 6-12 months
• Intravesical mitomycin C also used
First Line
Chemotherapy is the first-line treatment for metastatic bladder cancer
• Methotrexate-vinblastine-doxorubicin-cisplatin (MVAC) is preferred regimen
SURGERY
• Diffuse carcinoma in situ is treated with intravesical therapy (see Medication)
• Superficial cancer
- TURBT sometimes followed by intravesical therapy
• Invasive cancer
- Radical cystectomy for invasive disease that is confined to the bladder more effective than radical radiotherapy. (6)[A] Urine is diverted via an ileal loop with ostomy or neobladder constructed with intestine.
- Neoadjuvant chemotherapy with cisplatin-methotrexate-vinblastine prior to surgery used by some centers
FOLLOW-UP
• Superficial cancers
- Urine cytology alone has not been shown to be sufficient for follow up
- Cystoscopy every 3 months for 18-24 months, every 6 months for the next 2 years, then annually (7)[C]
• Follow-up for invasive cancers is dependent on the approach to treatment
• Patients treated with BCG require life-long follow-up
DISPOSITION
Admission Criteria
Need for surgery or intensive therapy
Issues for Referral
Patients with microscopic or gross hematuria should be referred to a urologist for cystoscopy
PROGNOSIS
• Superficial bladder cancer
- BCG treatment prevents recurrence vs TURBT alone, difference 30%, NNT 3.3 (7)[A]
- BCG prevents progression vs TURBT alone, difference 8% (7)[A]
• Invasive cancer
- T2 diseaseradical cystectomy results in 60-75% 5-year survival
- T3 or T4 diseaseradical cystectomy results in 20-40% 5-year survival
- Neoadjuvant chemotherapy with cystectomy has led to varying degrees of increased survival.
- Radiation with chemotherapy has led to varying degrees of increased survival.
• Metastatic cancer
- MVAC resulted in mean survival of 12.5 months (8)[C]
COMPLICATIONS
• Superficial bladder cancer
- Local symptoms
 Dysuria, frequency, nocturia, pain, passing debris in urine
 Bacterial cystitis
 Perforation
- General symptoms
 Flu-like symptoms
 Systemic infection
• Invasive cancer
- Symptoms related to definitive treatment, including incontinence, bleeding
- Patients with neobladder at risk for azotemia and metabolic acidosis
PATIENT MONITORING
See "Follow-Up"
REFERENCES
1. National Cancer Institute. SEER Cancer fact sheet, Ries LAG, Eisner MP, Kosary CL, et al., eds. SEER Cancer Statistics Review, 1975-2002, National Cancer Institute. Bethesda, MD. Available at: http://seer.cancer.gov/csr/1975_2002. Accessed February 10, 2006.
2. Buntinx F, Wauters H. The diagnostic value of macroscopic haematuria in diagnosing urological cancers: A meta-analysis. Fam Pract. 1997;14: 63-68.
3. Glas AS, Roos D, Deutekom M, et al. Tumor markers in the diagnosis of primary bladder cancer. A systematic review. J Urol. 2003;169:1975-1982.
4. Kirkali Z, Chan T, Manoharan, M, et al. Bladder cancer: epidemiology, staging, and grading, and diagnosis. Urology. 2005;66(Suppl 6A):4-34.
5. Shelley MD, Court JB, Kynaston H, et al. Intravesical Bacillus Calmette-Guerin in Ta and T1 bladder cancer (Cochrane Review). In: The Cochrane Library, Issue 4, 2005. Chichester, UK: John Wiley and Sons, Ltd.
6. Shelley MD, Barber J, Wilt T, Mason MD. Surgery versus radiotherapy for muscle invasive bladder cancer (Cochrane Review). In: The Cochrane Library, Issue 4, 2005. Chichester, UK: John Wiley and Sons, Ltd.
7. Smith JA, Labasky RF, Montie JE, Rowland RG, Cockett, ATK, Fracchia JA. Report on the management of non-muscle invasive bladder cancer. American Urologic Association monograph. Baltimore, MD: American Urology Association, Inc. 1999.
8. Loehrer PJ, Einhorn LH, Elson PJ, et al. A randomized comparison of cisplatin alone or in combination with methotrexate, vinblastine, and doxorubicin in patients with metastatic urothelial carcinoma: A cooperative group study. J Clin Oncol 1992;10:1066.
9. U.S. Preventive Services Task Force. Screening for bladder cancer in adults: Recommendation statement. Rockville, MD: Agency for Healthcare Research and Quality; 2004.
MISCELLANEOUS
See also: Hematuria

BIPOLAR DISORDER

2009-01-20T05:19:00.000-08:00

BIPOLAR DISORDER - Susan Louisa Montauk, MD
BASICS
DESCRIPTION
• A psychiatric disorder characterized by at least one episode of mania and often involving dramatic "mood swings"; episodes of mania and/or hypomania, and major depression that cause marked impairment and/or hospitalization.
• The symptoms must not be due to a substance (e.g., drug), treatment (e.g., ECT or light therapy), a general medical condition (e.g., hyperthyroidism), or medication
ALERT
Geriatric Considerations
New onset in seniors demands a workup for organic or chemically induced pathology
Pediatric Considerations
• Signs and symptoms must be placed into a developmental context
• There is a large overlap with symptoms of Attention Deficit Hyperactivity Disorder (ADHD) and Oppositional Defiant Disorder
• Children and adolescents experience more rapid cycling and mixed states than adults
• Depression often presents as irritable mood
Pregnancy Considerations
No medications currently used for bipolar I disorder are category A or B
EPIDEMIOLOGY
Incidence
No overall incidence data have been reported.
Prevalence
1.0-1.6% (1)
RISK FACTORS
Genetics (2,3)
• Monozygotic twin concordance 40-70%
• Heritability estimate 0.93
• Several chromosomes implicated
• likely many gene set variations
PATHOPHYSIOLOGY
• Neurotransmitters known to be involved
• Serotonin
• Norepinephrine
• Dopamine
• Brain structures most involved
• MRI findings suggest that abnormalities in prefrontal cortical areas, striatum, and amygdala predate illness onset
ETIOLOGY
• Genetic predisposition (major)
• Life stressors
ASSOCIATED CONDITIONS
Substance abuse (60%) (4), ADHD, Anxiety disorders (e.g., Anorexia nervosa, Bulimia nervosa, Generalized anxiety disorder, Obsessive compulsive disorder, Panic disorder, Post-traumatic stress disorder, Social phobia)

DIAGNOSIS
DSM-IV-R CRITERIA
• Bipolar I disorder requires at least one manic or mixed episode (simultaneous mania and depression). There may be episodes of hypomania or major depression as well.
• Mania
- Distinct period of abnormally and persistently elevated, expansive, or irritable mood, lasting at least 1 week (or any duration if hospitalization is necessary)
- During the period of mood disturbance, 3 or more of the DIGFAST 1 symptoms must persist (4 if the mood is only irritable) and must be present to a significant degree
• Depression
- 5 or more of the 9 symptoms (see Sns and Sxs) must have been present during the same 2-week period and represent change from previous functioning; at least 1 of the symptoms is either (1) or (2).
- Bipolar II (More common in primary care) requires a major depression and at least one hypomanic episode.
SIGNS AND SYMPTOMS
• Mania (DIGFAST)
- Distractibility (attention too easily drawn to unimportant or irrelevant external stimuli)
- Insomnia, decreased need for sleep (e.g., feels rested after only 3 hours of sleep)
- Grandiosity or inflated self-esteem
- Flight of ideas or subjective experience that thoughts are racing
- Agitation or increase in goal-directed activity (socially, at work or school, or sexually)
- Speech pressured/more talkative than usual
- Taking risks: Excessive involvement in pleasurable activities that have a high potential for painful consequences (e.g., financial or sexual)
• Hypomania
- A distinct period of persistently elevated, expansive, or irritable mood, lasting throughout at least 4 days, that is different from usual non-depressed mood but is not severe enough to cause marked impairment in social or occupational functioning
• Depression (DSM)
• Depressed mood most of the day
- Markedly diminished interest or pleasure activities most of the day
- Significant weight loss when not dieting or weight gain
- Insomnia or hypersomnia
• Psychomotor agitation or retardation
• Feel worthless
• Excessive/inappropriate guilt
• Diminished concentration; indecisiveness
• Recurring thoughts of death; suicidal ideation/plan
• Signs and Symptoms More Likely in Bipolar than in Unipolar Depression (5,6)
• Agitation
• Atypical depression symptoms (subjectively restless, leaden paralysis, hypersomnia)
• Feelings of worthlessness
• Hyperphagia
• Hypersomnia
• Melancholia
• Psychomotor retardation
• Suicidal ideation/planning
• Minimal tearfulness
History
• Collateral information makes diagnostics more complete and is often the best source for a clear history.
• HOPI (major points)
- Mood-Mood Disorder Questionnaire and an interview
- Sleep: Longest awake without stimulants and without sleepiness?
- Coexistent conditions? Psychosis?
- Suicide/violence risk
Physical Exam
Base focused exam on history and review of systems
TESTS
• Mood Disorder Questionnaire
- Sensitivity for mania/hypomania 0.73, specificity 0.90 (7)
• Child Behavior Checklist
- For Juvenile Bipolar Disorder
• "Dementia" workup if new onset in seniors
Lab
• No labs help rule in bipolar disorder
• Consider drug/alcohol screen if may help assist in future psychoeducation
• Many mood stabilizer medications must have regular blood draw monitoring
DIFFERENTIAL DIAGNOSIS
Other disorders with mania
Brain tumors
Drug intoxications
Organic mood disorders
Schizoaffective disorder
TREATMENT
PRE-HOSPITAL
• Medication
• Psychotherapy
• Psychoeducation
• Cognitive Behavioral Therapy
• Social Rhythm Therapy
STABILIZATION
Safe environment plus appropriate medication. Useful comparative studies not done
GENERAL MEASURES
Although experts agree that adopting a "healthy lifestyle" is key to better outcomes, there are few clinical trials to access specific diet or exercise effects
MEDICATION (DRUGS)
First Line
Maintenance therapy for Bipolar Disorder often consists of 3-4 of the following psychoactive medications. (8)
Antiseizure Medications
NOTE: Taper any antiseizure med discontinued for reasons other than major side effects.
• Carbamazepine (Carbatrol, Equetro, Tegretol, generic):
- FDA approval: Equetroonly for acute mania and mixed episodes
- Selected warnings: Do not use with TCA or MAOI/Caution with renal or cardiac disease. (Aplastic anemia/agranulocytosis/Preg Cat D)
- Monitoring: Baseline and q3-6 months
• Divalproex sodium (Depakote, generic)
- FDA approval: None
- Selected warnings: Do not use with hepatic or urea cycle disorders. Pancreatitis, polycystic ovary syndrome Preg Cat D. Dose-related hepatic failure and low platelets
- Monitoring: Baseline and q6 months
• Lamotrigine (Lamictal)
- FDA approval: Only for maintenance therapy
- Selected warnings: Titrate slowly (rash). Caution with renal, hepatic, or cardiac impairment. Blood dyscrasias, acute multiorgan failure, deadly hypersensitivity. Chronic ophthal. Preg Cat C.
- Monitoring: Baseline
• Oxcarbazepine (Trileptal)
- FDA approval: None
- Selected warnings: Caution if hypersensitivity to carbamazepine. Severe rash, hyponatremia/Preg Cat C adjust for CrCl.
- Monitoring: Baseline
• Topiramate (Topamax)
- FDA approval: None
- Adult dose: 25 mg/d; increase by 25-50 mg q3-14 days prn/as tolerated. Adjust for CrCl.
- Selected warnings: Possible acidosis in predisposed states. Renal stones, low serum bicarb, acute myopia, oligohidrosis. Preg Cat C.
- Monitoring: Baseline and as needed (prn)
Atypical Antipsychotics
NOTE: All of these drugs have the following possible major side effects: Orthostatic hypotension, Poor ability to reduce core body temperature, Negatively effect glucose regulation, Negatively affect lipid metabolism, Tardive dyskenesia, Increased mortality in elderly with dementia-related psychosis, Seizures, Neuroleptic malignant syndrome, Weight gain. All except aripiprazole may increase prolactin Preg Cat C.
NOTE: All of these drugs need the following
• Monitoring: Blood work and weight at baseline then 4, 8, and 12 weeks; then q3 months
• Aripiprazole (Abilify):
- FDA approval: Acute mania, mixed episodes, and maintenance
- Selected warnings: CVAs in seniors with dementia
• Olanzapine (Zyprexa)
- FDA approval: Acute mania, mixed episodes, and maintenance therapy (Zydis ODT contains phenylalanine)
- Selected warnings: CVAs in seniors with dementia
• Quetiapine (Seroquel)
- FDA approval: Acute mania
- Selected warnings: Cataracts, hypothyroidism
- Monitoring: Eye exam at baseline, then prn
• Risperidone (Risperdal)
- FDA approval: Acute mania and mixed episodes
- Selected warnings: M tabs contain phenylalanine
• Ziprasidone (Geodon)
- FDA approval: Acute mania and mixed episodes
Lithium
Lithium (Lithobid, Eskalith, generic)
FDA approval: Adult acute mania and maintenance therapy
Selected warnings: Use with caution in patients with significant renal or cardiovascular disease, in severely debilitated or dehydrated patients, and sodium-depleted patients (diuretics, Angiotensin Converting Enzyme Inhibitors (ACEIs). Toxicity can lead to diabetes insipidus, seizures, encephalopathic syndrome, arrhythmias, hypothyroidism. Preg Cat D.
Monitoring: At baseline, with dose change, then in 5 days, then q2-3 months  3, then q6-12 months
Second Line
• Antidepressants (not until mood stabilizers are on board) for some patients
• Benzodiazepines
FOLLOW-UP
DISPOSITION
Admission Criteria
Both primarily determined by safety
Issues for Referral
• Experience and comfort level of physician
• Stability of patient
PROGNOSIS
• Most untreated persons will experience manic and/or depression episodes across their lifespan
• Treatment reduces frequency and severity
COMPLICATIONS
In general, the most extreme "complication" is violence toward self or others
PATIENT MONITORING
• Careful medication monitoring
• Regularly scheduled visits to help support medication adherence and healthy lifestyle
REFERENCES
1. Kessler RC, et al. Lifetime and 12-month prevalence of DSM-III-R psychiatric disorders in the United States: Results from the National Comorbidity Study. Arch Gen Psychiatry. 1994;51:8-19.
2. Kieseppa T, et al. High concordance of bipolar I disorder in a nationwide sample of twins. Am J Psychiatry. 2004;161(10):1814-21.
3. Craddock N, Jones I. Molecular genetics of BD. Br J Psychiatry. 2001;41(suppl):128-133.
4. Rush J. Toward an Understanding of BD and Its Origin. J of Clinical Psychiatry 2003;64(suppl 6): 4-8.
5. Keck PE. Evaluating Treatment decisions in Bipolar Depression. CME. Medsacpe. www.medscape.com
6. Mitchell PB, Wilhelm K, Parker G, et al. The clinical features of bipolar depression: A comparison with matched major depressive disorder patients. J Clin Psychiatry. 2001;62:212-216.
7. Hirshfeld RM. Validation of the Mood Disorder Questionnaire Bipolar Depression Bulletin. July 2004.
8. Post RM. Practical approaches to polypharmacy in the long-term management of bipolar disorder. Drug Benefit Trends. 2004;16:329-342.
9. Ketter TA, Ed. Advances in Treatment of Bipolar Disorder. Review of Psychiatry, Vol 24 Amer Psychiatric Publishing
10. Working Group on BD. Practice guidelines for the treatment of patients with BD. Am J Psychiatry 2002;159(Suppl 4):1-50.
11. Vieta E, Goikolea JM. Atypical antipsyhotics: Newer options for mania and maintenance therapy. BD 2005;7(Suppl 4):21-33.
ADDITIONAL READING
• Hirschfeld RM, Vomik LA. Rscognition and diagnosis of bipolar disorder. J Clin Psychiatry. 2004;65(Suppl 15):5-9.
• Vieta E, Pacchiarotti I, Scott J, et al. Evidence-based research on the efficacy of psychologic interventions in bipolar disorders: a critical review. Curr Psychiatry Rep. 2005;7(6):449-455.

BELL PALSY

2009-01-20T05:18:00.001-08:00

BELL PALSY - Dylan C. Kwait, MD
BASICS
DESCRIPTION
• Peripheral lower motor neuron facial palsy, usually unilateral, which arises secondary to inflammation and subsequent swelling and compression of the 7th (facial) cranial nerve and the associated vasa nervorum.
• Synonym(s): Idiopathic facial paralysis
EPIDEMIOLOGY
• Accounts for 60-75% of all cases of unilateral facial paralysis (1)[A]
• Predominant age
- Median age of onset is 40 years, but affects all ages (2)[A].
• Predominant sex: Male = Female (2)[A]
Incidence
• Incidence in the US is 20-30 cases per 100,000 people per year (2)[A].
• Incidence is lowest in children 10 years of age; highest in people 70 years of age (2)[A].
• Incidence is higher among pregnant women (1)[A].
• Occurs with equal frequency on the left and right sides of the face (2)[A].
Prevalence
Affects 40,000 Americans every year (3)[A].
RISK FACTORS
• Pregnancy
• Diabetes mellitus
• Age >30
• Exposure to cold temperatures
• Upper respiratory infection (e.g., coryza, influenza)
Genetics
A genetic predisposition may be associated with Bell palsy, but it is unclear which factors are inherited.
ETIOLOGY
• Results from damage to the 7th (facial) cranial nerve
• Inflammation of the 7th nerve causes swelling and subsequent compression of both the nerve and the associated vasa nervorum
• May arise secondary to reactivation of latent herpes virus (herpes simplex virus type 1 and herpes zoster virus) in cranial nerve ganglia (1)[A].
• May arise secondary to ischemia from arteriosclerosis associated with diabetes mellitus (2)[A].
ASSOCIATED CONDITIONS
• Lyme disease
• Diabetes mellitus
• Hypertension
• Herpes simplex virus
• Herpes zoster virus
• Ramsay Hunt syndrome
• Sjogren syndrome
• Sarcoidosis
• Eclampsia
• Amyloidosis

DIAGNOSIS
SIGNS AND SYMPTOMS
• Weakness on affected side of face, often sudden in onset
• Pain in or behind the ear in 50% of cases (may precede the palsy in 25% of cases) (2)[A]
• Subjective numbness on the ipsilateral side of the face
• Alteration of taste on the ipsilateral anterior 2/3 of the tongue (chorda tympani branch of the facial nerve)
• Hyperacusis (nerve to the stapedius muscle)
• Decreased tear production
History
It is vital to elicit
• Time course of the illness (e.g., rapid vs. slow onset)
• Any predisposing factors (e.g., recent viral infection, trauma, new medications, hypertension, diabetes mellitus)
• Presence of hyperacusis or history of recurrent Bell palsy (both associated with poor prognosis)
• Any associated rash (suggestive of herpes zoster, Lyme disease, or sarcoid)
Physical Exam
• Neurologic examination to determine if the weakness is due to a problem in either the central or peripheral nervous systems
- Flaccid paralysis of muscles on the affected side, including the forehead
 Impaired ability to raise the ipsilateral eyebrow
 Impaired closure of the ipsilateral eye
 Bell phenomenon: Upward diversion of the eye with attempted closure of the lid
 Impaired ability to smile, grin or purse the lips
- Patients may complain of numbness, but on sensory testing, no deficit is present.
- Examine for involvement of other cranial nerves.
• HEENT
- Carefully examine head, neck, and oropharynx to exclude masses.
- Perform pneumatic otoscopic examination.
• Skin
- Examine for erythema migrans (Lyme disease) and vesicular rash (herpes zoster virus).
TESTS
• Electromyography
- Nerve conduction on affected and nonaffected sides can be compared to determine extent of nerve injury.
• Electroneurography
- Evoked potentials of affected and nonaffected sides can be compared.
• CSF analysis
- Not routinely indicated
- CSF protein is elevated in 1/3 of cases.
- CSF cells show mild elevation in 10% of cases with a mononuclear cell predominance.
Lab
• Lyme titer and IgM, IgG, and IgA for B. burgdorferi
• Salivary PCR for herpes simplex virus type 1 or herpes zoster virus (these tests are largely reserved for research purposes) (1)[A]
• IgM, IgG, and IgA titers for varicella zoster virus, cytomegalovirus, rubella, hepatitis A, hepatitis B, and hepatitis C
• ESR
• Blood glucose level
• CBC
• RPR test
• HIV test
Imaging
• Facial radiographs
- Rule out fractures
• CT
- Rule out fractures
- Rule out stroke
• Brain MRI
- Not routinely indicated
- Rule out central pontine, temporal bone, and parotid neoplasms (2)[A]
Diagnostic Procedures/Surgery
Invasive diagnostic procedures are not indicated, because biopsy could further damage the 7th nerve (4)[A].
Pathological Findings
• Dilatation of the vasa nervorum
• Edema of the facial nerve with infiltration of mononuclear cells and possible atrophy
DIFFERENTIAL DIAGNOSIS
• Infectious
- Lyme disease
- Herpes zoster (Ramsay-Hunt syndrome)
- Acute or chronic otitis media
- Malignant otitis externa
- Osteomyelitis of the skull base
- Infectious mononucleosis
- Leprosy
• Trauma injury
- Temporal bone fracture
- Mandibular bone fracture
• Neoplastic (should be considered in cases where onset of palsy is slow and progressive and accompanied by additional cranial nerve deficits and/or headache) (1)[A]
- Tumors of the parotid gland
- Cholesteatoma
- Skull-base tumor
- Carcinomatous meningitis
- Leukemic meningitis
• Cerebrovascular
- Brainstem stroke involving antero-inferior cerebellar artery
- Aneurysm involving carotid, vertebral or basilar arteries
• Other
- Multiple sclerosis
- Myasthenia gravis (should be considered in cases of recurrent or bilateral facial palsy) (2)[A]
- Guillain-Barre syndrome (may also present with bilateral facial palsy) (2)[A]
- Sjogren syndrome
- Sarcoidosis
- Amyloidosis
- Melkersson-Rosenthal syndrome
- Polyneuritis
TREATMENT
GENERAL MEASURES
• Artificial tears should be used to lubricate the cornea.
• The ipsilateral eye should be patched and taped shut at night to avoid drying and infection.
Diet
No restrictions
Activity
No restrictions
MEDICATION (DRUGS)
No evidence suggests that pharmacologic intervention (with anti-inflammatory and/or antiviral agents) or decompression surgery is any more beneficial than watchful waiting in terms of treatment or prevention of long-term effects. (4)[A]
ALERT
Pregnancy Considerations
Steroids should be used cautiously in pregnancy; consult with an obstetrician.
First Line
• Corticosteroids
- Prednisone (5)[B]: Total from 410 mg over 10 days to 760 mg PO over 16 days, tapering dose (adults only)
- Treatment should begin immediately after onset, and should not be instituted if symptoms have been present for >7 days.
- May reduce edema around the 7th cranial nerve; small benefit in adult patients, but use remains controversial.
• Antivirals
- Acyclovir (5)[B]: 400 mg PO 5 times per day for 10 days (adults only)
- Should be instituted within 72 hours, but may be started up to 7 days after onset of symptoms.
- Combination acyclovir and prednisone therapy may improve recovery rates when compared with prednisone alone. (5)[B]
• Contraindications
- Documented hypersensitivity
- Pre-existing infections including tuberculosis and systemic mycosis
• Precautions: Use with discretion in pregnancy, peptic ulcer disease, and diabetes.
• Significant possible interactions: Measles-mumps-rubella, oral polio virus vaccine, and other live vaccines
SURGERY
• Surgical treatment of Bell palsy remains controversial and is reserved for intractable cases (1)[A].
• The 7th cranial nerve is surgically decompressed at the entrance to the meatal foramen where the labyrinthine segment and geniculate ganglion reside. (2)[A]
• Decompression surgery should not be performed >14 days after the onset of paralysis because severe degeneration of the facial nerve is likely irreversible after 2-3 weeks. (2)[A]
FOLLOW-UP
DISPOSITION
Issues for Referral
Patients may need to be referred to an ear, nose, and throat specialist or a neurologist.
PROGNOSIS
• Most achieve complete spontaneous recovery within 2 weeks (3)[A].
• 85% of untreated patients will experience the 1st signs of recovery within 3 weeks of onset. (5)[C].
• Over 80% recover within 3 months (3)[A].
• 16% are left with a partial palsy, motor synkinesis, and autonomic synkinesis (1)[A].
• 5% experience severe sequelae, and a small number of patients experience permanent facial weakness and dysfunction (1)[A].
• Poor prognostic factors include
- Age >60 years
- Complete facial weakness
- Hypertension
- Ramsay-Hunt syndrome
- Absence of recovery at 3 weeks
COMPLICATIONS
• Corneal abrasion or ulceration
• Steroid-induced psychological disturbances; avascular necrosis of the hips, knees, and/or shoulders
• Steroid use can unmask subclinical infection (e.g., tuberculosis).
PATIENT MONITORING
• Patients should start treatment immediately and be followed for 12 months.
• Patients who do not recover complete facial nerve function should be referred to an ophthalmologist for tarsorrhaphy.
REFERENCES
1. Holland NJ, Weiner GM. Recent developments in Bell's palsy. Br Med J. 2004;329:553-557.
2. Gilden DH. Bell's palsy. N Engl J Med. 2004;351:1323-1331.
3. Holten K. How should we manage Bell's palsy? J Fam Pract. 2004;53(10):797-798.
4. Atkin PA. Diagnosis and the management of Bell's palsy. Practitioner. 2003;247(1642):36.
5. Peitersen E. The natural history of Bell's palsy. Am J Otol. 1982;4(2):107-111.
ADDITIONAL READING
Piercy J. Bell's palsy. Br Med J. 2005;330:1374.
MISCELLANEOUS
See also: Herpes simplex virus; Herpes zoster virus; Ramsay-Hunt syndrome; Sjogren syndrome; Sarcoidosis; Amyloidosis; Lyme disease; Diabetes mellitus; Melkersson-Rosenthal syndrome

BEHCET SYNDROME

2009-01-20T05:18:00.000-08:00

BEHCET SYNDROME - Bruce M. Rothschild, MD
BASICS
DESCRIPTION
• Rare multisystem, chronic disease characterized by oral and genital mucocutaneous ulcerations, skin rashes, arthritis, thrombophlebitis, uveitis, colitis, and neurologic symptoms (1-6)[A]
• Endemic in Japan and Northeastern Mediterranean region
• Synonym(s): Ucocutaneous ocular syndrome; Franceschetti-Valero syndrome
ALERT
Geriatric Considerations
Rare
Pediatric Considerations
Rare
Pregnancy Considerations
• Thalidomide contraindicated in pregnancy
• Possible increase in thrombosis and fetal demise
GENERAL PREVENTION
Avoid English walnuts
EPIDEMIOLOGY
• Predominant age: 3rd to 4th decades
• Predominant sex: Male > Female frequently twice as often, whereas as some studies suggest equal frequency
Prevalence (6,8)[A]
• 1/100,000 prevalence in the United States
• In other countries, per 100,000
- Japan: 10
- Iran: 16-100
- Germany: 2
- Saudi Arabia: 20
RISK FACTORS
See "Etiology"
Genetics
• One report in a mother and newborn (9)
• Very rarely familial
ETIOLOGY (2-6)[A], (9)[A]
Unknown:
• Classified as vasculopathy or autoimmune
• HLA-B5 alloantigen relationship
• Possible environmental toxin: Heavy metals, pesticides
• Possibly English walnuts or Ginkgo nuts
• Fibrinolysis abnormality
• 1 report associated with HIV infection (10)
ASSOCIATED CONDITIONS
• Amyloid
• Sweet syndrome

DIAGNOSIS
SIGNS AND SYMPTOMS (2-6)[A], (8)[A]
• Aphthous stomatitis
• Genital ulcers: Painful in the male, usually painless in the female
• Dermal: Papulovesicular, erythema nodosum, pathergy, erythema multiforme, vasculitis, pyoderma
• Ocular: Liritis, iridocyclitis, chorioretinitis, hypopyon, hemorrhage, papilledema, optic atrophy (8)[A]
• Morning stiffness: In 1/3 of patients
• Polyarthritis: Self-limited and predominantly affecting lower extremities
• Thrombophlebitis: Peripheral, pulmonary, cerebral, Budd Chiari syndrome (11)[A]
• Neurologic: Cranial nerve palsy, hemiplegia, intracranial hypertension, meningomyelitis and recurrent meningitis, confusional state (12)[A], (13)[A]
• GI: Aphthous ulcers, colitis, melena
• Pulmonary infiltratespossibly related to thrombosis
• Myopathy/myositis: Rare
• Peripheral gangrene: Rare
• Epididymitis
• Glomerulonephritis: Rare
TESTS (2-6)[A], (8)[A]
• Erythrocyte sedimentation rate elevation, but can be normal
• Immune complexes detected by Raji cell and C1q solid phase assays, but not clinically useful
• Cryoglobulin
• Hypergammaglobulinemia
• Circulating anticoagulation (rare)
• Depression of plasma antithrombin III levels with active disease
• Increased fibrinolytic activity during attacks
• Antineutrophil cytoplasmic antigen antibodies, perinuclear variety
• Demyelinating antibodies in neuro-Behcet syndrome
• Anticardiolipin antibodies (rare), lupus anticoagulants
• Antiendothelial antibodies
• Pathergy
Diagnostic Procedures/Surgery
• Careful history and physical and frequent reevaluation
• Synovial fluid: Inflammatory effusion
• Arteriography: For aneurysms or thrombosis
Pathological Findings
• May be no recognizable changes
• Mononuclear perivascular infiltration
• Mononuclear infiltrate in synovium
• Endothelial cell swelling
• Partial obliteration of vascular lumen
• Neutrophilic dermatitis (Sweet syndrome) (rarely)
DIFFERENTIAL DIAGNOSIS
• Reiter syndrome and other forms of spondyloarthropathy
• Inflammatory bowel disease (Crohn disease and ulcerative colitis)
• Syphilis
• Erythema nodosum
• Aphthous stomatitis
• Herpes simplex
• Stevens-Johnson syndrome
• Vasculitis
• Multisystem disease
• Thrombophlebitis related to coagulation factor deficiency
• Mollaret meningitis
TREATMENT
STABILIZATION
• Usually outpatient
• Inpatient usually required for neurologic complications
GENERAL MEASURES
According to body system involved
Diet
No special diet
Activity
As tolerated
MEDICATION (DRUGS)
First Line (2-6)[A], (14-15)[A], (16)[B]
• Colchicine: 0.6 mg b.i.d.
• Topical ocular steroids
• Prednisone: 1 mg/kg for severe involvement, especially central nervous system
• Azathioprine: 2-3 mg/kg/d PO
• Methotrexate: Use lowest possible dose; perhaps 7.5 mg per week
• Cyclosporine: 1-4 mg/kg, but monitor liver function, creatinine, magnesium, and lipids every 2 weeks for 3 months, then every month (17)[A]
• Resistant cases may require
- Tacrolimus (FK 506) 0.09-0.15 mg/kg/d
- Thalidomide 300 mg/d
- Interferon alpha (15)[A]
- Anticoagulants for patients with anticardiolipin antibodies: Warfarin (Coumadin) to establish PT international normalized ratio 3.0 to 3.5.
• Contraindications
- Thalidomide contraindicated during pregnancy
- Refer to manufacturer's literature
• Precautions
- Refer to manufacturer's literature
- Absorption of drugs such as amitriptyline, diazepam, carbamazepine, phenytoin, and acetaminophen may be reduced in Behcet syndrome. (14)[A]
• Significant possible interactions: Refer to manufacturer's literature.
Second Line
• Levamisole: 100-150 mg 2 days per week
• Chlorambucil: But concern with respect to toxicity, especially its malignant potential
• Cyclophosphamide: 50-100 mg per day in morning.
• The patient should drink 8-10 glasses of water per day and report any blood in the urine.
• Tumor necrosis factor inhibitors (18)[A]
• Stem cell transplantation (19)[B]
• Anti-CD52 antibody (16)[B]
FOLLOW-UP
PROGNOSIS
• Normal life expectancy, except with neurologic involvement
• Possible vision impairment
COMPLICATIONS (2-6), 8[A]
• Death
• Blindness
• Paralysis
• Embolism/thrombosispulmonary, vena cava, peripheral
• Aneurysms
• Amyloidosis
• Thrombotic events, especially when anticardiolipin antibodies present
PATIENT MONITORING
Dependent on severity of system involvement and medication monitoring
REFERENCES
1. International diagnostic study group for Behcet's disease. Evaluation of ("classification") criteria in Behcet's diseaseTowards internationally agreed criteria. Br J Rheumatol. 1992;31:299-308.
2. Kaklamani VG, et al. Behcet's disease. Semin Arthritis Rheu. 1998;27:197-217.
3. Lockwood CM, Hale G, Waldman H, Jayne DR. Remission induction in Behcet's disease following lymphocyte depletion by the anti-CD52 antibody CAMPATH 1-H. Rheumatology (Oxford). 2003;42:1539-1544.
4. Pacor ML, et al. Cyclosporin in Behcet's disease. J Rheumatol. 1994;13:224-227.
5. Shimizu T, et al. Behcet disease. Semin Arthritis Rheu. 1979;8:223-260.
6. Zouboulis CC, Vaiopoulos G, Marcomichelakis N, et al. Onset signs, clinical course, prognosis, treatment and outcome of adult patients with Adamantiades-Behcet's disease in Greece. Clin Exp Rheumatol. 2003;21(suppl 30):19-26.
7. Sushan JJ, Sela EY, Ozcan C. Behcet's disease and pregnancy. Acta Obstet Gynecol Scand 2005;84:939-944 [B].
8. Yoshida A, Kawashima H, Motoyama Y, et al. Comparison of patients with Behcet's disease in the 1980's and 1990's. Ophthalmology 2004;111:810-815 [A].
9. Fam A. Neonatal Behcet syndrome in an infant of a mother with the disease. Ann Rheumatic Dis. 1981;40:509-512.
10. Stein C. J Rheumatol. 1991;18:1427-1428.
11. Huong DL, et al. Arterial lesions in Behcet's disease. J Rheumatol. 1995;22:2103-2113.
12. Akman-Demir G, et al. Seven-year follow-up of neurologic involvement in Behcet syndrome. Arch Neurol. 1996;53:691-768.
13. Gerber S, et al. Long-term MR follow-up of cerebral lesions in neuro-Behcet's disease. Neuroradiology. 1996;38:761-768.
14. Chaleby K. Decreased drug absorption in a patient with Behcet's syndrome. Clin Chem. 1987;33:1679-1681.
15. Hamuryudan V, et al. Systemic interferon alpha-2b treatment in Behcet syndrome. J Rheumatol. 1994;21:1098-1100.
16. Mauer B, Hensel M, Max R, et al. Autologous haematopoietic stem cell transplantation for Behcet's disease with pulmonary involvement: Analysis after 5 years of followup. Ann Rheumatic Dis. 2006;65:127-129 [B].
17. Sakane T, Takeno M, Suzuki N, Inaba G. Behcet's disease. N Engl J Med. 1999;341:1284-1291.
18. Hassard PV, Binder SW, Nelson V, Vasiliauskas EA. Anti-tumor necrosis factor monoclonal antibody therapy for gastrointestinal Behcet's disease: a case report. Gastroenterology. 2001;120:995-999.
19. Melikoglu M, Fresko I, Mat C, et al. Short-term trial of etanercept in Behcet's disease: A double blind, placebo controlled study. J Rheumatol 2005;32:98-105 [B].
20. O'Duffy JD. Behcet's disease. Curr Opin Rheumatol. 1994;6:39-43.

BASAL CELL CARCINOMA

2009-01-20T05:17:00.000-08:00

BASAL CELL CARCINOMA - Melissa A. Fischer, MD, MEd
BASICS
DESCRIPTION
• Basal Cell Carcinoma (BCC) is a common malignant tumor of the skin originating from the basal cells of the epidermis and its appendages
• Rarely metastasizes, but capable of local tissue destruction
ALERT
Geriatric Considerations
Greater frequency in geriatric patients (age 55-75 have 100 incidence of age 20)
Pediatric Considerations
Rare in children, but childhood sun exposure important in adult disease
GENERAL PREVENTION
• Sunscreens (though likely more effective for squamous cell carcinoma) (1)[B]
• Hats, long-sleeve shirts
• Avoid tanning and sunburn, especially during childhood
EPIDEMIOLOGY
• Incidence/prevalence in United States: ~900,000 cases/year
• Predominant age: Generally >40, but incidence is increasing in younger populations
• Predominant sex: Males > Female (although incidence is increasing in females)
Incidence
Lifetime risk of caucasion North American: 30%
RISK FACTORS
• Chronic sun exposure (UV radiation)
• Light complexion
• Tendency to sunburn
• Male sex, although increasing risk in women due to lifestyle changes such as tanning beds/salons
• Family history of skin cancer, basal cell nevus syndrome (rare autosomal dominant)
• 3-4 decades after chronic arsenic exposure, 2 decades after therapeutic radiation, chronic immunosuppression
PATHOPHYSIOLOGY
• UV-induced inflammation and Cyclooxygenase activation in skin
• Patched, Drosophila, Homolog of tumor suppressor gene mutations (familial and sporadic)
• Cytochrome P-450 CYP2D6 and glutathione S-transferase detoxifying enzyme gene mutations (especially in truncal BCC)
ASSOCIATED CONDITIONS
• Xeroderma pigmentosum
• Nevoid BCC syndrome

DIAGNOSIS
SIGNS AND SYMPTOMS
• 70% facial, 15% truncal
• Nodular: Most common (60%), presents as pinkish, pearly papule often with telangiectatic vessel and ulceration, usually on face
• Superficial: (30%) light red, scaly papule or plaque with atrophic center, ringed by translucent micropapules, usually on trunk; more common in men
• Morpheaform: (5-10%) firm, smooth, flesh-colored papule with ill-defined borders
• As the nodules enlarge, central ulceration and crusting can occur
History
Exposure to risk factors, family history
TESTS
Diagnostic Procedures/Surgery
Biopsy and pathologic examination mandatory to confirm diagnosis
Pathological Findings
• Nidus of basal cells extending into dermis
• Characteristic cells resemble normal basal cells with large basophilic, oval nuclei.
• Rare mitoses
• Tumor cells arranged in palisades at periphery
DIFFERENTIAL DIAGNOSIS
• Sebaceous hyperplasia
• Epidermal inclusion cyst
• Intradermal nevi (pigmented and nonpigmented)
• Molluscum contagiosum
• Squamous cell carcinoma
• Nummular dermatitis
• Psoriasis
• Melanoma (pigmented lesions)
TREATMENT
PRE-HOSPITAL
Outpatient unless extensive lesion
GENERAL MEASURES
Activity
No restrictions except to avoid overexposure to sun
MEDICATION (DRUGS)
Topical antibiotics after excision for 24-48 hours (optional)
SURGERY
• Generally first choice, specific treatment selection varies with extent and location of lesion, tumor border distinctiveness (2)[A]
• High-risk areas
- Inner canthus, Nasolabial sulcus, Philtrum, Preauricular area, Retroauricular sulcus, Lip, Temple
• Curettage and electrodesiccation
- Nodular lesion 1 cm, in low-risk area, if not deeply invasive
- Requires specialized training and experience in surgical technique
• Excision
- Useful for lesions in high-risk areas, not as dependent on lesion size
- Poor choice if multiple lesions
- Requires appropriate training
• Cryosurgery
- Reserved for small lesions in low-risk area
- Requires specialized training and equipment
- May want pre- and post-treatment biopsies
• Mohs surgery
- The preferred microsurgically controlled surgical treatment for lesions in high-risk area, for recurrent lesion, if there is an aggressive growth pattern
- Requires referral to appropriately trained dermatologic surgeon
Radiation
• Useful for large lesions, very elderly (life expectancy 15 years) or patients who could not tolerate minor surgical procedures
• Also may be used when preservation of local tissue important, such as near lips and eyelids
Medical
5-Fluorouracil (3)[C]
• inhibits thymidylate synthetase interrupting DNA synthesis
• for superficial lesions in low-risk areas
• primary treatment only
• 5% applied b.i.d. for 3-10 weeks Other non-surgical treatments under investigation: Imiquimod, photodynamic therapy, interferon
FOLLOW-UP
PROGNOSIS
• Proper treatment yields 90-95% cure
• Most recurrences happen within 5 years
• Development of new BCCs: Patients (36%) will develop a new lesion within 5 years
COMPLICATIONS
• Local recurrence and spread
• Usually recurrences will appear within 5 years.
• Metastasis (rare, 0.1%), but metastatic disease usually fatal within 8 mos
PATIENT MONITORING
• Every month for 3 months, then twice yearly for 5 years; yearly thereafter
• Increased risk of other skin cancers (4)[C]
REFERENCES
1. Green A, Williams G, Neale R, et al. Daily sunscreen application and betacarotene supplementation in prevention of baseal-cell and squamous-cell carcinomas of the skin: A randomized controlled trial. Lancet. 1999;354: 723.
2. Bath FJ, Bong J, Perkins W, Williams HC. Interventions for basal cell carcinoma of the skin. Cochrane Database Syst Rev. 2003;CD003412.
3. Goette DK. Topical chemotherapy with 5-Fluorouracil. A review. J Am Acad Dermatol. 1981;4:633.
4. Friedma GD, Tekawa IS. Association of basal cell skin cancers with other cancers (United States). Cancers Cause Control. 2000;11:891.
MISCELLANEOUS
Related terms: Basal cell epithelioma; Rodent ulcer

BARTONELLA INFECTIONS

2009-01-20T05:16:00.000-08:00

BARTONELLA INFECTIONS - Mary H. Hohenhaus, MD
BASICS
DESCRIPTION
• Fastidious intracellular Gram-negative bacilli
- At least 20 distinct species, 8 known to cause disease in humans
- Bartonella henselae and B. quintana most common in North America
• Infections manifest in 2 broad categories
- Localized skin lesions and prominent regional lymphadenitis (cat scratch disease [CSD])
- Bacteremia with localized vascular lesions in various organs and potential for persistent disseminated infection
• System(s) Affected: Cardiovascular; Gastrointestinal; Hemic/Lymphatic/Immunologic; Musculoskeletal; Nervous; Pulmonary; Skin/exocrine
• Synonym(s): Bartonellosis
GENERAL PREVENTION
Vector avoidance
EPIDEMIOLOGY
• CSD: Predominantly children
• Others: Predominantly adults
Incidence
• Carri'on disease: 12.7/100 person-years in endemic areas
• CSD: Estimated 9.3/100,000 in US (~25,000 cases annually)
• Endocarditis: Estimated 3-4% of cases, up to 1/3 of "culture negative" cases
• Others: Unknown
Prevalence
• Seroprevalence studies of B. henselae suggests many childhood infections are asymptomatic.
• Studies of B. quintana in homeless populations suggest seroprevalence of 10%.
RISK FACTORS
• Vector exposure with cutaneous inoculation
- B. bacilliformis: Lutzomyia sandflies, limited to Andean South America
- B. quintana: Human body louse, typically in alcoholic, homeless men
- B. henselae: Domestic cat (especially scratch/bite from kitten)
- Others: Unknown
• Cell-mediated immune dysfunction (particularly in bacillary angiomatosis/bacillary peliosis)
- HIV infection, especially with CD4+ lymphocyte count 100/mcL
- Chronic steroid, immunosuppressant, or alcohol use
PATHOPHYSIOLOGY
• Erythrocyte and endothelial cell invasion
• Stimulation of angiogenesis
ETIOLOGY
• B. bacilliformis: Carri'on disease
• B. quintana: Trench fever, bacillary angiomatosis (subcutaneous and osseous lesions), bacillary peliosis, endocarditis
• B. henselae: CSD, acute and persistent bacteremia, bacillary angiomatosis (hepatosplenic lesions), bacillary peliosis, endocarditis (preexisting valvular disease), neurologic manifestations
• B. clarridgeiae, B. elizabethae, B. grahamii, B. vinsonii, B. washoensis: Case reports of CSD, bacteremia, endocarditis, myocarditis, others

DIAGNOSIS
• Diagnosis of typical CSD traditionally requires at least 3 of the following
- Animal contact (usually cat) resulting in a scratch, abrasion, or ocular lesion
- Positive serologic test
- Characteristic lymph node pathology
- No evidence of other cause
• Diagnosis of other syndromes requires high clinical suspicion and identification of compatible syndrome; if test results are not helpful, response to appropriate antibiotics may be suggestive.
SIGNS AND SYMPTOMS
• Carri'on disease (aka Bartonellosis; usually has 2 distinctive stages: An acute, life-threatening illness associated with high fever, hemolytic anemia, and a chronic, benign cutaneous eruption consisting of raised, reddish-purple nodules.
- Oroya fever (acute bacteremia): In severe cases, abrupt onset 3 weeks after inoculation. Profound anemia, many complications, may be fatal.
- Asymptomatic persistent bacteremia: 15% of untreated Oroya fever survivors
- Verruga peruana: Crops of nodular angiomatous skin lesions months after Oroya fever; mucosal and internal lesions also; involute in months to years
• Typical CSD (up to 90% of cases)
- Days after inoculation 2-3-mm nontender papules develop at the trauma site; progress to reddened then crusted vesicles
- Tender regional adenopathy 1-8 weeks postinoculation; fever, malaise, headache
- Usually involves nodes of upper extremities, neck, head
- Suppuration of nodes common, but only 10% require drainage
- Resolution in 2-4 months for majority
• Atypical CSD
- Parinaud oculoglandular syndrome: Unilateral granulomatous conjunctivitis and preauricular lymphadenitis
- Neuroretinitis: Abrupt, painless unilateral vision loss; macular star exudate, papilledema; self-limited, with return of visual acuity
- Encephalopathy: Rapid progression from headache to lethargy, coma, and seizure; sequelae rare
- Other manifestations self-limited, sequelae rare: Granulomatous hepatitis/splenitis, osteolysis, atypical pneumonitis, fever of unknown origin, mononucleosis-type syndrome, others
• Bacteremia (short-term mortality uncommon)
- B. quintana (urban trench fever, Wolhynia fever, shin-bone fever, quintan fever): Incubation days-weeks; sudden onset of fever, headache, leg pain; self-limited illness may be brief (4-5 days), prolonged (2-6 weeks), most commonly paroxysmal (3-5 episodes of 5 days' duration). Insidious course in HIV.
- B. henselae: If HIV-infected, insidious onset of fatigue, malaise, aches, weight loss, recurring fevers, headache; localizing findings uncommon. If HIV-uninfected, abrupt onset of fever (may persist or relapse), myalgias, arthralgias, headache; localizing findings unusual; may persist without symptoms.
• Endocarditis: Fever, dyspnea, murmur, embolic phenomena; aortic valve involvement most common
• Bacillary angiomatosis: Mostly immunocompromised hosts (e.g., HIV-infected); involves skin (crops of subcutaneous or dermal nodules and/or skin-colored to purple papules; may ulcerate with serous or bloody drainage and crusting), regional lymph nodes, internal organs
• Bacillary peliosis: Involves liver and spleen in immunosuppressed persons; can involve lymph nodes; nonspecific clinical manifestations
• Neurologic syndromes in HIV: Cognitive dysfunction, behavioral disturbances; may be mistaken for dementia, psychiatric disease
TESTS
Lab
• Skin testing reagents: Not recommended
• Giemsa-stained blood smear may show B. bacilliformis adherent to erythrocytes
• Non-bacilliformis species
- Indirect fluorescent antibody and enzyme immunoassay tests are available
 Interpretation complicated by variable correlation between titers and disease stage, lack of uniformity among serologic tests, and cross-reactivity among Bartonella species and other bacteria.
ALERT
Advise lab if Bartonella infection is suspected so that blood, tissue, and cerebrospinal fluid cultures are prepared with appropriate media under optimal conditions; prolonged incubation required.
• Polymerase chain reaction (PCR) and immunohistochemical labeling primarily research tools, although PCR of valve tissue can aid diagnosis of endocarditis
• Drugs that may alter lab results: Antibiotics (cultures falsely negative)
Imaging
Ultrasonography, CT, or ECG as indicated
Diagnostic Procedures/Surgery
• Biopsies for histology/culture of nodules, lymph nodes, or internal organs
• Lumbar puncture if CNS involvement
Pathological Findings
• Verruga peruana: Neovascular proliferation; bacteria uncommonly are identified.
• CSD: Granulomas, stellate necrosis, mixed inflammatory infiltrates; bacilli in tissue may be demonstrable by silver impregnation stains (e.g., Warthin-Starry).
• Endocarditis: Warthin-Starry-stained bacilli may be seen in vegetations.
• Bacillary angiomatosis
- Lobular proliferations of small blood vessels are seen, containing cuboidal endothelial cells interspersed with inflammatory cells, mostly neutrophils.
- Warthin-Starry stain or electron microscopy may show clusters of bacilli
• Bacillary peliosis: Blood-filled cystic structures. Warthin-Starry stain may show surrounding clumps of bacilli.
DIFFERENTIAL DIAGNOSIS
• Typical CSD: Sporotrichosis, histoplasmosis, plague, tularemia, brucellosis, mycobacteria, staphylococci, streptococci, other agents associated with injection drug use; lymphoma; metastatic malignancy
• Atypical CSD: Other agents causing similar syndromes
• Non-bacilliformis bacteremia syndromes
- Immunocompromised: Cryptococcus neoformans, Histoplasma capsulatum, Coccidioides immitis, Mycobacterium avium-complex
- Arthropod exposure: Rickettsial infections, tularemia, plague, babesiosis, borreliosis
- Cat/dog scratch/bite: Pasteurella
- Influenza, infectious mononucleosis, hepatitis
• Endocarditis: Other slow-growing bacteria (Haemophilus, Actinobacillus, Cardiobacterium, Eikenella, Kingella, Coxiella)
• Bacillary angiomatosis/bacillary peliosis: Kaposi sarcoma; pyogenic granuloma, hemangioma
• Neurologic syndrome in HIV: Tertiary syphilis, cryptococcal meningitis, toxoplasmosis, progressive multifocal leukoencephalopathy, alcohol or drug abuse
TREATMENT
STABILIZATION
• Outpatient for uncomplicated infection
• Initial hospitalization may be necessary for IV antibiotics or complications.
GENERAL MEASURES
• CSD: Supportive therapy (e.g., aspiration suppurative nodes)
• Systemic syndromes (including CSD-associated neuroretinitis and encephalopathy): Antibiotics
Activity
Fully active if uncomplicated
MEDICATION (DRUGS)
First Line
• Oroya fever: Chloramphenicol 500 mg (pediatric dose 50-75 mg/dg/d) PO/IV q.i.d. + -lactam for 14 days (not available in US) (1) [B]; ciprofloxacin 500 mg (250 mg for children 7-12 years) PO b.i.d. for 10 days
• Verruga peruana: Rifampin 10/mg/kg/d (not to exceed 600 mg/d in children) for 10 days (1) [B])
• Typical CSD: No clear benefit, although oral azithromycin may speed resolution of extensive lymphadenopathy: Adults and children >45.5 kg: 500 mg on day 1; 250 mg daily on days 2-5; children 45.5 kg: 10 mg/kg on day 1; 5 mg/kg daily on days 2-5 (1) [A]
• Retinitis: Doxycycline 100 mg PO b.i.d. + rifampin 300 mg PO b.i.d. for 4-6 weeks (1) [B]
• Trench fever or chronic B. quintana bacteremia: Doxycycline 200 mg PO daily for 4 weeks + gentamicin 3 mg/kg IV daily for 2 weeks (1) [A]
• Bacillary angiomatosis: Erythromycin 500 mg (pediatric dose 40 mg/kg/d to maximum daily dose of 2 g/d) PO q.i.d. or doxycycline 100 mg PO b.i.d. for 3 months; consider longer course if immunocompromised (1) [B]
• Bacillary peliosis: Erythromycin 500 mg (pediatric dose 40 mg/kg/d to maximum daily dose of 2 g/d) PO q.i.d. or doxycycline 100 mg PO b.i.d. for 4 months; consider longer course if immunocompromised (1) [B]
• Endocarditis (culture positive): Gentamicin 3 mg/kg IV daily for 2 weeks + doxycycline 100 mg PO b.i.d. for 6 weeks (1) [B]
• Endocarditis (culture negative): Gentamicin 3 mg/kg IV daily for 2 weeks + ceftriaxone 2 g IV/IM daily for 6 weeks +/- doxycycline 100 mg PO/IV b.i.d. for 6 weeks (1) [B]
SURGERY
Valve replacement if indicated in endocarditis
FOLLOW-UP
PROGNOSIS
• CSD: Spontaneous resolution usually in 2-4 months without specific therapy
• Other syndromes: With proper treatment, full resolution; if relapse, consider long-term suppressive antibiotics after retreatment
COMPLICATIONS
Relapse, especially in HIV infection
PATIENT MONITORING
Close follow-up after completion of antibiotics to monitor for relapse
REFERENCES
1. Rolain JM, Brouqui P, Koehler JE, et al. Recommendations for treatment of human infections caused by Bartonella species. Antimicrob Agents Chemother 2004;48(6):1921-1933.
ADDITIONAL READING
• Agan BK, Dolan MJ. Laboratory diagnosis of Bartonella infections. Clin Lab Med. 2002;22:937-962.
• Dehio C. Molecular and cellular basis of Bartonella pathogenesis. Ann Rev Microbiol. 2004;58:365-390.
• Slater LN, Welch DF. Bartonella, including cat-scratch disease. In: Mandell, Bennett, Dolin, eds. Principles and Practice of Infectious Diseases, 6th ed. Philadelphia: Churchill Livingstone, 2005.

BARRETT ESOPHAGUS

2009-01-20T05:15:00.000-08:00

BARRETT ESOPHAGUS - Laura Goldman, MD
BASICS
DESCRIPTION
• Replacement of normal squamous epithelium of the distal esophagus with abnormal columnar epithelium as a consequence of gastric acid reflux
• Precursor of adenocarcinoma of the esophagus
• Divided into long (3 cm) and short segments (3 cm)
GENERAL PREVENTION
• Case-controlled studies have shown that aspirin, and NSAIDs may prevent esophageal cancer, but no randomized trials to date (1)[B]
• No evidence that gastric acid-suppression reduces cancer risk (medical or surgical) (1)[A]
• Epidemiological evidence that weight loss, cessation of smoking, and eating fruits and vegetables can decrease cancer risk (2)[C]
EPIDEMIOLOGY
• Most common in white men >55
• Uncommon in Blacks and Asians, Hispanics similar to Caucasians
• Can affect children, but rarely occurs before the age of 5
Prevalence
• Estimates vary widely in studies
- Most patients with Barrett esophagus are not diagnosed (3)[B]
- In patients without gastroesophageal reflux disease (GERD) symptoms 0-25%
- In patients with chronic GERD symptoms, 10%
RISK FACTORS
• Chronic GERD symptoms
• Male
• White
• Most frequent in 55-65 years of age
PATHOPHYSIOLOGY
• Reflux of gastric contents injures mature cells and triggers metaplastic transformation from squamous cells to more resistant columnar cells called specialized intestinal metaplasia
• Columnar cells have higher malignant potential than squamous cells
• Annual incidence of esophageal cancer in patients with Barrett's is 0.5% per year
• A few studies have demonstrated no difference in overall survival in patients with Barrett esophagus compared to the general population. One observational study showed only 4 of 409 patients affected died of esophageal cancer in 10 years. (3)[B]
• Cancers evolve through a sequence of DNA changes that can be recognized by the pathologist as dysplasia, categorized as low grade or high grade depending on severity of changes.
• Develops to full extent over a relatively short period of time, 1 year
ETIOLOGY
Caused by chronic reflux of gastric contents
ASSOCIATED CONDITIONS
• Esophageal cancer is the most rapidly increasing cancer in the United States, with 0.4% annual increase
• Specialized intestinal metaplasia may also be found in the esophagogastric junction, with lower incidence of cancer, but differentiating between this and Barrett's may be difficult.

DIAGNOSIS
SIGNS AND SYMPTOMS
• Barrett esophagus causes no symptoms
• Most patients seen for GERD symptoms
History
• Heartburn, regurgitation, and dysphagia are the most common symptoms of GERD.
• Less common include chest pain, odynophagia, chronic cough, water brash, globus sensation, laryngitis, and asthma
• Weight loss, anorexia, dysphagia, odynophagia, or bleeding may indicate complications of GERD or cancer
Physical Exam
Normal
TESTS
Upper endoscopy with random multiple biopsies is the only test recommended for diagnosis.
Lab
H. pylori testing not indicated; it does not infect the esophagus, and it does not increase the risk of Barrett's or esophageal cancer
Imaging
None
Diagnostic Procedures/Surgery
• Specialized intestinal metaplasia (reddish, velvety appearance) can be seen at endoscopy
• Multiple biopsies are taken of this area.
• Multiple experimental techniques to identify dysplasia have not been shown in studies to increase accuracy of diagnosis (dysplasia is not visible). (1)[A]
Pathological Findings
• Histologic examination must reveal specialized intestinal metaplasia (also called specialized columnar epithelium) to diagnose Barrett esophagus
• Biopsies may or may not demonstrate low-grade or high-grade dysplasia
DIFFERENTIAL DIAGNOSIS
• Erosive esophagitis may make biopsies inadequate, and repeat study after treatment may be necessary.
• Pathology may show 2 types of columnar epithelium that DO NOT have malignant potential: Cardiac epithelium and gastric-fundic type.
• Intraobserver agreement among experienced pathologists for low-grade dysplasia is 50%, while for high-grade dysplasia it is 85%; second expert pathologist opinion is recommended (4)[A]
TREATMENT
GENERAL MEASURES
• The goal of treatment is to control GERD symptoms and detect and treat dysplasia and cancer.
• The efficacy of treatments in reducing the number of deaths from cancer has not been established. (1)[A]
Diet
• Avoid foods that can cause reflux.
• Avoid other acidic foods if known to trigger symptons: Colas, red wine, orange juice
• Weight loss if obese
Activity
• Avoid supine position after eating; avoid tight fitting clothes.
• Smoking cessation
SPECIAL THERAPY
• Treatment of high-grade dysplasia is controversial.
• Esophagectomy is usually recommended. (4)[B] There are multiple endoscopic procedures now available, but none has been shown to decrease long-term risk of cancer. (5)[A] They may be considered in patients who are poor operative candidates (4,5)
- Photodynamic therapy (PDT) is available in some academic centers; efficacy is not established; cancer has been shown to recur after treatment and strictures occur in 40%. (1,5)
- Other endoscopic ablative procedures (thermal, photochemical, radiofrequency); again efficacy unknown and cancers have been reported post-treatment. (5)
- Endoscopic mucosal resection: Involves excision of mucosa down to submucosa; its efficacy is unknown; can be paired with PDT; remains experimental (1)[A]
- Intensive surveillance with endoscopy every 3-6 months and treatment of cancer if it arises; little data to support safety and efficacy (5)[A]
MEDICATION (DRUGS)
• Goal of therapy is the control of the symptoms of GERD (5)[A] and the maintenance of healed mucosa
• Therapy usually does not result in reversal of Barrett esophagus. (4)[A]
First Line
• Once a day proton-pump inhibitor (PPI) therapy for long-segment disease (5)[B]
• H2-receptor antagonist may be sufficient for short-segment disease (5)[B]
Second Line
• If once a day PPI does not control symptoms, b.i.d. dosing is recommended. (4)[A]
• If H2-receptor antagonist does not control symptoms, step-up to PPI. (4)[A]
SURGERY
• Fundoplication is an option to control GERD symptoms, but it has not been shown to reverse Barrett esophagus or decrease risk of cancer (5)[A]
• Esophagectomy is the only treatment of high-grade dysplasia that guarantees cancer-free survival (5)
• Mortality rate from esophagectomy is 8-23%, and 30-50% develop serious post-op complications
ALERT
Geriatric Considerations
If the patient is a poor operative candidate for PDT or other endoscopic ablative procedure, surveillance or no treatment may be preferable; treatment must be individualized.
FOLLOW-UP
• Surveillance is controversial; aim is to detect high-grade dysplasia or early carcinoma
• Only evidence to support improved survival in patients undergoing surveillance is from non-randomized, retrospective studies (1,3)
• Vast majority (96% in one study) of patients with adenocarcinoma of esophagus are NOT known to have Barrett esophagus before diagnosis of cancer (6)[B]
ALERT
Geriatric Considerations
Surveillance should only be done if there is reasonable life expectancy and ability to tolerate treatment of esophageal cancer (4)
DISPOSITION
• American College of Gastroenterology Guidelines for surveillance in Barrett esophagus (4)[C]
- If no dysplasia on 2 consecutive endoscopies with biopsies, 3-yr interval is appropriate
- If low-grade dysplasia, and repeat endoscopy again shows low-grade, yearly endoscopy until no dysplasia
- If high-grade, repeat Esophagogastroduodeno- scopy (EGD) w/biopsies to rule out cancer, expert pathologist confirmation; intervention or EGD every 3 months
Issues for Referral
• Patients should be treated with a PPI prior to endoscopy
• Diagnosis of high-grade dysplasia should be confirmed by a second expert pathologist. (4)[A]
• Patients considering esophagectomy should be referred to a high-volume institution; mortality and morbidity rates have been shown to be inversely related to volume. (4)[A]
PROGNOSIS
• Annual incidence of esophageal cancer in patients with Barrett esophagus is 0.5% per year.
• Low-grade dysplasia may be transient: 7% progress to cancer in 3-7 yrs
• High-grade progresses to cancer in 22% (3-7 yrs) (4)[B]
COMPLICATIONS
Same as GERD: Stricture, bleeding, ulceration
REFERENCES
1. Sampliner RE. Managing Barrett esophagus esophagus: What is new in 2005? Dis Esophagus. 2005;18:17-20.
2. Wang et al. AGA medical position statement: Role of gastroenterologist in the management of EsoCa Gastroenterology. 2005;128.
3. Sharma P, Sidorenko EI. Are Screening and surveillance for Barrett esophagus really worthwhile? Gut. 2005;54:27-32.
4. Sampliner RE and the Practice Parameters Committee of the American College of Gastroenterology. Updated guidelines for the diagnosis, surveillance, and therapy of Barrett Esophagus. Am J of Gastroenterology. 2002;97:1888-1895.
5. Spechler SJ. Barrett Esophagus. N Eng J Med. 2002;346:836-842.
6. Corley DA, Levin TR, et al. Surveillance and survival in Barrett's adenocarcinomas: A population based study. Gastroenterology. 2002;122:3:633-40.
ADDITIONAL READING
UpToDate Online 13.3 has an excellent evidence-based review.

BAROTITIS MEDIA

2009-01-20T05:14:00.001-08:00

BAROTITIS MEDIA - Tyeese Gaines-Reid, DO, MA
BASICS
DESCRIPTION
• Inflammation of the middle ear space (tympanic cavity, eustachian tube, and mastoid air cells) secondary to changes in negative pressure between the external canal and middle ear
• Caused by the inability of the eustachian tube to adequately equilibrate the middle ear air pressure with the moment-to-moment changes in environmental atmospheric pressures while descending or ascending in air (flight) or in water (diving)
- Causes the retraction or protraction of the tympanic membrane, with subsequent inflammation and/or rupture
- May cause asymmetric pressure stimulation of the inner ear and vestibular end organ
- Negative pressure can also cause serous fluid or blood to pool in the middle ear.
• System(s) Affected: Nervous; ENT
• Synonym(s): Dysbarism; Aerotitis; Otitic barotrauma; Middle ear barotrauma
ALERT
Valsalva maneuver can spread nasopharyngeal infection into the middle ear.
Pediatric Considerations
Children have difficulty dilating the eustachian tube even at small pressure changes and therefore are at higher risk (especially with upper-respiratory infection).
Pregnancy Considerations
The nasal congestion often associated with pregnancy increases risk of barotitis media.
GENERAL PREVENTION
• Avoid altitude changes when any risk factors are present for eustachian tube dysfunction.
• Use methods of autoinflation during pressure changes.
EPIDEMIOLOGY
• Predominant age: All ages
• Predominant sex: Male = Female
Incidence
• The most common medical disorder experienced by SCUBA divers
• Also highly prevalent among aircraft flight personnel (especially high-performance jet aircraft), passengers, and sky divers
RISK FACTORS
• Participating in high-risk activities without adequate eustachian tube autoinflation (Valsalva maneuver, swallowing, yawning)
• Any causes of eustachian tube and external ear canal dysfunction
- SCUBA diving
- Airplane flight (especially high performance)
- Sky diving
- High-altitude mountain traveling
- High-altitude elevator rides
- Hyperbaric oxygen chamber therapy
- High-impact sports
• Infants and young children (especially with upper-respiratory infection)
• Upper respiratory infections
• Nasal congestion or allergic rhinitis
• Pregnancy
• Anatomic obstruction in the nasopharynx
ETIOLOGY
• Rapid descent or ascent with eustachian tube obstruction
- Upper-respiratory infections: Sinusitis, rhinitis, tonsillitis, adenoiditis, otitis media
- Overzealous forceful Valsalva maneuver (in ascent with vestibular stimulation)
- Allergic rhinitis
- Nonallergic rhinitis with eosinophilia
- Obstructing nasal polyps
- Deviated nasal septum
- Congenital abnormalities of inner/middle ear (cleft palate)
- Nasopharyngeal tumors
• Rapid descent or ascent with external ear canal occlusion
- Otitis externa (swimmer's ear)
- Impacted cerumen
- Ear plugs
• Trauma to external and middle ear
- Boxing, soccer, water skiing, accidents, etc.
- Overzealous use of cotton swab in cleaning ear canals
• Otalgia and hearing loss occurs as a result of stretching and malformation of the tympanic membrane.
ASSOCIATED CONDITIONS
• Aerosinusitis
• Aerodontalgia
• Face mask squeeze
• Epistaxis
• Alternobaric vertigo
• Unequal caloric stimulation vertigo
• Anxiety (leading to panic attack)
• Temporomandibular joint syndrome
• Inner ear cochlear damage and/or perilymph fistula

DIAGNOSIS
SIGNS AND SYMPTOMS
• Abrupt onset
• Otalgia (ear pain)
• Feeling of fullness or pressure in ear
• Conductive hearing loss
• Dizziness
• Tinnitus, pulsating or constant
• Vertigo
• Nausea and vomiting
• Transient facial paralysis
• With tympanic membrane rupture, leakage of air or fluid from the ear during sneezing or Valsalva
• Crying in children (which is a means of autoinflation)
TESTS
Tympanometry
Imaging
Only to rule out suspected nasopharyngeal tumor or sinusitis
Diagnostic Procedures/Surgery
• Otoscopic exam
• Audiogram: Conductive (middle ear) vs. mixed (inner ear) hearing loss
• Surgical exploration to rule out inner ear involvement if suspected
Pathological Findings
• Tympanic membrane retraction or protraction with hemotympanum or rupture
• Edema of mucosal lining and capillary engorgement with transudation of middle ear effusion
• Inner ear involvement with rupture of the round or oval windows and leakage of perilymph into the middle ear and perilymphatic fistula development
DIFFERENTIAL DIAGNOSIS
• Inner ear barotrauma
• Serous otitis media
• Acute and chronic otitis media
• External otitis
• Myringitis bullosa
• Temporomandibular joint syndrome
TREATMENT
GENERAL MEASURES
• Prevention/avoidance is best
- Avoid flying or diving while risk factors exist, if possible
• Autoinflate the eustachian tube during pressure changes
- Valsalva method (1)[B]
 Patient occludes nose with thumb and index finger pressure on nasal alae, then carefully exhales with mouth closed until ears "pop." This will equalize pressures, relieve pain, and restore hearing. This usually must be repeated several times during descent or ascent.
- Infants: Breastfeeding, or sucking on pacifier or bottle
- 4 years: Chewing gum
- 8 years: Blowing up a balloon
- Adults: Chewing gum, sucking hard candy, or yawning
• Nasal balloon (1)[B]
• If the suggested maneuvers are unsuccessful, return to baseline altitude if possible; autoinflate then resume ascent/descent
• If associated bacterial upper respiratory infection, treat with appropriate antibiotics
• If inner ear exposed, bed rest with head of bed elevated to help drainage
Diet
Avoid food allergens that cause rhinitis.
Activity
• No flying or diving until complete resolution of all signs and symptoms and Valsalva maneuver can be performed
• In severe cases, bed rest
MEDICATION (DRUGS)
First Line
• Antihistamines
- Pseudoephedrine. Start 30-60 minutes prior to exposure
 Studies showed oral pseudoephedrine decreased otalgia in adults but not in children (2)[C].
- Oxymetazoline nasal spray (Afrin, Afrin 12-Hour)
 Beware of rebound congestion after 3-5 days of use
 No statistical significance that it prevents symptoms (2)[C]
- Phenylephrine nasal spray (Neo-Synephrine)
- Antihistamines for allergic component (no data demonstrate benefit except to relieve allergy symptoms)
 Diphenhydramine (Benadryl)
 Loratadine (Claritin)
 Fexofenadine (Allegra)
• Precautions
- All medications must be used on the ground to rule out idiosyncratic reactions that could incapacitate in an airplane or underwater environment.
- Elderly are more susceptible to drug side effects, especially with diphenhydramine
- Caution with hypertension
• Analgesics for pain control
• Tinnitus can be treated with high-dose steroids if given within 3 weeks of onset (3)[C].
SURGERY
If necessary, myringotomy or tympanoplasty
FOLLOW-UP
DISPOSITION
Admission Criteria
Patients with complicating emergencies (e.g., incapacitating pain requiring myringotomy, large tympanic perforation requiring tympanoplasty)
Issues for Referral
Refer to otolaryngology if
• Inner ear is exposed
• Perilymphatic fistula
• Sensorineural hearing loss
PROGNOSIS
• Untreated, simple barotitis media resolves on its own unless secondary to diving.
• Ear block: Hours-days, with complete resolution and return to flight or diving within days-weeks
• Tympanic rupture: Recovery within weeks-months
COMPLICATIONS
• Permanent hearing loss
• Ruptured tympanic membranes
• Serous otitis media
• Chronic tinnitus, vertigo
• Bruising of or bleeding into tympanic membrane
• Fluid exudate in middle ear
• Perilymphatic fistula
• Sensorineural hearing loss
PATIENT MONITORING
• Otoscopic until symptoms clear
• In severe cases, audiograms
REFERENCES
1. Stangerup SE, Klokker M, Yesterhauge S. Point prevalence of barotitis and its prevention and treatment with nasal balloon inflation: A prospective, controlled study. Otol Neurol. 2004;25(2):89-94.
2. Mirza S, Richardson H. Otic barotrauma from air travel. J Laryngol Otol. 2005;119(5):366-370.
3. Duplessis C, Hoffer M. Tinnitus in an active duty navy diver: A review of inner ear barotrauma, tinnitus, and its treatment. Undersea Hyperbaric Med. 2006;33(4):223-230.
ADDITIONAL READING
Internet references
• Emedicine, www.emedicine.com
• MedlinePlus Medical Encyclopedia www.medlineplus.gov
• Up to Date, www.uptodate.com

BALANITIS

2009-01-20T05:14:00.000-08:00

BALANITIS - James P. Miller, MD; Timothy L. Black, MD
BASICS
DESCRIPTION
• Balanitis: Inflammation of glans penis
• Posthitis: Inflammation of the foreskin
• System(s) Affected: Reproductive; Skin/Exocrine
ALERT
Geriatric Considerations
Condom catheters can predispose to balanitis.
Pediatric Considerations
Oral antibiotics predispose male infants to Candida balanitis.
GENERAL PREVENTION
• Proper hygiene and avoidance of allergens
• Circumcision
EPIDEMIOLOGY
• Predominant age: Adult
• Predominant sex: Male only
RISK FACTORS
• Presence of foreskin
• Morbid obesity
• Poor hygiene
• Diabetes
• Nursing home environment
ETIOLOGY
• Allergic reaction (condom latex, contraceptive jelly)
• Infections (Candida albicans, Borrelia vincentii, streptococci, trichomonas)
• Fixed drug eruption (sulfa, tetracycline, barbital)
• Plasma cell infiltration (Zoon balanitis)
• Autodigestion by activated Pancreatic transplant exocrine enzymes

DIAGNOSIS
SIGNS AND SYMPTOMS
History
• Pain
• Drainage
• Dysuria
Physical Exam
• Erythema
• Edema
• Discharge
• Ulceration
• Plaque
TESTS
Lab
• Microbiology culture
• Wet mount
• Serology for syphilis
• Serum glucose
Diagnostic Procedures/Surgery
Biopsy, if persistent
Pathological Findings
Plasma cells infiltration with Zoon balanitis
DIFFERENTIAL DIAGNOSIS
• Leukoplakia
• Lichen planus
• Psoriasis
• Reiter syndrome
• Lichen sclerosus et atrophicus
• Erythroplasia of Queyrat
• Balanitis xerotica obliterans (BXO)
TREATMENT
GENERAL MEASURES
• Appropriate health care: Outpatient
• Warm compresses or sitz baths
• Local hygiene
MEDICATION (DRUGS)
• Fungal
- Clotrimazole (Lotrimin) 1% b.i.d.
- Nystatin (Mycostatin) b.i.d.-q.i.d.
- Fluconazole 150 mg single dose (1)[B]
• Bacterial
- Bacitracin q.i.d.
- Neomycin-polymyxin B-bacitracin (Neosporin) q.i.d.
- If cellulitis, cephalosporin or sulfa drug PO or parenteral
- Dermatitis
- Topical steroids q.i.d.
- Zoon balanitis
- Topical steroids q.i.d.
• BXO
- 0.05% Betamethasone b.i.d. (2)[B]
- 0.1% Tacrolimus b.i.d. (3)[C]
• Contraindications: Refer to manufacturer's profile for each drug.
• Precautions: Refer to manufacturer's profile for each drug.
• Significant possible interactions: Refer to manufacturer's profile for each drug.
SURGERY
Consider circumcision as preventive measure
FOLLOW-UP
DISPOSITION
Admission Criteria
• Uncontrolled diabetes
• Sepsis
Discharge Criteria
Resolution of problem
Issues for Referral
Recurrent infections or development of meatal stenosis
PROGNOSIS
Should resolve with appropriate treatment
COMPLICATIONS
• Meatal stenosis
• Premalignant changes from chronic irritations
• Urinary tract infections
PATIENT MONITORING
• Every 1-2 weeks until etiology has been established
• Persistent balanitis may require biopsy to rule out malignancy or BXO
REFERENCES
1. Stary A, Soeltz-Szoets J, Kiegler C, et al. Comparison of the efficacy and safety of oral fluconazole and topical clotrimazole in patients with candida balanitis. Genitourin Med. 1996;72:98-102.
2. Kiss A, Csontai A, Pirot L, et al. The response of Balanitis xerotica obliterans to local steroid application compared to placebo in children. J Urol. 2001;165:219-220.
3. Pandher BS, Rustin HMA, Kaisary AV. Treatment of Balanitis Xerotica Obliterans with topical tacrolimus. J Urol. 2003;170:923.
MISCELLANEOUS
See also: Reiter Syndrome

BAKER CYST

2009-01-20T05:13:00.000-08:00

BAKER CYST - Gregory R. Czarnecki, DO; John Herbert Stevenson, MD
BASICS
DESCRIPTION
• Aka popliteal cyst; a fluid-filled synovial-lined sac (cyst) arising in the popliteal fossa
• Can be unilateral or bilateral
• Found in both children and adults
EPIDEMIOLOGY
Bimodal distribution: Children 4-7 and adults increasing with age
Prevalence
• Varies by study
• 2.4% in asymptomatic children in 1 prospective study (1)[B]
• 5% in adults by MRI in 1 prospective study; up to 58% in others, limited by sample population and largely retrospective data (2)[B]
RISK FACTORS
• Knee osteoarthritis
• Rheumatoid arthritis
• Meniscal degeneration/tear
• Advancing age
ETIOLOGY
• Extension or herniation of synovial membrane of the knee joint capsule or connection of normal bursa with the joint capsule. This may be the result of increased intraarticular pressure and is commonly seen in association with knee effusions. Direct trauma to the bursa may be the primary cause in children.
• A valvelike mechanism has been described with this connection of bursa and joint allowing primary one-way passage of fluid from the joint to the bursal connection.
• Associated intraarticular pathological findings are rare in children but common in adultsup to 50%. (3)[B]
• Bursa under the medial head of the gastrocnemius or semimembranosus bursa most commonly involved
ASSOCIATED CONDITIONS
• Osteoarthritis
• Rheumatoid arthritis
• Meniscal tear, notably posterior horn of medial meniscus

DIAGNOSIS
SIGNS AND SYMPTOMS
History
• (Often) painless mass arising in the popliteal fossa
• May complain of restricted range of motion or tightness with knee flexion
• Ruptured cyst typically painful with associated swelling causing pseudothrombophlebitis
• Large cysts may cause entrapment neuropathy of the tibial nerve.
• Vascular compression may produce claudication.
Physical Exam
• Examine in full extension and 90 flexion. Mass increases with extension and may disappear on flexion (Foucher sign).
• Most commonly found in medial popliteal fossa
• Mass may be fluctuant or tender
• Transillumination to distinguish cystic vs. solid
TESTS
Imaging
• Ultrasound readily confirms presence and size.
• MRI allows further characterization of association with joint capsule.
• May also detect on arthrography or CT scan
• Radiographs may show soft tissue density posteriorly.
Diagnostic Procedures/Surgery
Arthrography may demonstrate communication with joint capsule.
DIFFERENTIAL DIAGNOSIS
• Aneurysm
• Deep venous thrombosis
• Infection/abscess
• Lipoma
• Fibroma
• Fibrosarcoma
• Hematoma
• Vascular tumor
• Xanthoma
• Any condition causing synovitis
TREATMENT
GENERAL MEASURES
• No treatment if aysmptomatic
• Compressive wrap or sleeve may be used for comfort
• Treat underlying cause if present, (e.g., intraarticular derangement)
Activity
No restrictions
SPECIAL THERAPY
• Aspiration for symptom relief, recurrance common
• Injection with corticosteroid directly into cyst following aspiration, or intraarticular if communicating cyst. If joint communication is present, intraarticular corticosteroid injection may assist regression of cyst. (3)[B]
Physical Therapy
Physical therapy is helpful in improving knee range of motion and strength. It is also helpful if co-existing knee arthritis or stable meniscal tear.
MEDICATION (DRUGS)
Analgesics, NSAIDS for symptomatic relief.
SURGERY
Excision via arthroscopy or open procedure often requires treatment of intraarticular pathology (when present) to prevent recurrence of cyst. (3)[B]
FOLLOW-UP
Routine monitoring providing diagnosis is clear. Size of cyst may vary depending on degree of knee effusion and joint communication.
DISPOSITION
Issues for Referral
Consider whether definitive treatment desired. Despite surgical removal, some cysts recur; treatment of underlying intraarticular pathology, if present, is imperative.
PROGNOSIS
• Variable
• Many cysts remain asymptomatic, and some will regress or resolve with treatment of associated cause without direct treatment of the cyst; in others, size may remain stable or expand; recurrence is common.
• In children, most resolve without treatment.
COMPLICATIONS
• Compartment syndrome in ruptured cyst
• Pain with cyst expansion/dissection or rupture
• Frequent recurrence
PATIENT MONITORING
See above
REFERENCES
1. Seil R, Rupp S, et al. Prevalence of popliteal cysts in children: A sonographic study and review of the literature. Arch Ortho Traum Su. 1999;119:73-75.
2. Rupp S, Seil R, et al. Popliteal cysts in adults: prevalence, associated intraarticular lesions, and results after arthroscopic treatment. Am Sport Med. 2002;30(1):112-115.
3. Handy JR. Popliteal cysts in adults: A review. Semin Arthritis Rheu. 2001;31(2):108-118.
4. Canale. Campbell's Operative Orthopaedics, 10th ed., Mosby, Inc. 2003;894-903.

BABESIOSIS

2009-01-20T05:12:00.001-08:00

BABESIOSIS - Eleftherios Mylonakis, MD; Vassiliki Syriopoulou, MD
BASICS
DESCRIPTION
• Babesiosis is a tick-borne hemolytic disease that is caused by intra-erythrocytic protozoan parasites of the genus Babesia.
• Rarely reported outside the United States. Sporadic cases have been reported from a number of countries, including France, Italy, the former Yugoslavia, the United Kingdom, Ireland, the former Soviet Union, and Mexico. In the United States, infections have been reported in many states, but the most endemic areas are the islands off the coast of Massachusetts (including Nantucket and Martha's Vineyard), New York (including Long Island, Shelter Island, and Fire Island), and in Connecticut. In these areas, asymptomatic human infection seems to be common. (1-4, 6-9)
• Incubation period of babesiosis varies from 5-33 days. Most patients do not recall recent tick exposure. After an infected blood transfusion, the incubation period can be up to 9 weeks.
• System(s) Affected: Cardiovascular Gastrointestinal; Hemic/Lymphatic/Immunologic; Musculoskeletal; Nervous; Pulmonary; Renal/Urologic
ALERT
Geriatric Considerations
Morbidity and mortality is higher in patients >65.
GENERAL PREVENTION
• Avoid endemic regions during the peak transmission months of May to September (especially relevant for asplenic or immunocompromised persons, in whom babesiosis can be a devastating illness).
• Using insect repellant is advised during outdoor activities, especially in wooded or grassy areas
- Products with 10-35% N-diethyl-meta-toluamide (DEET) will provide adequate protection under most conditions.
• Early removal of ticks is important; the tick must remain attached for at least 24 hours before the transmission of Babesia microti occurs. Daily self-examination is recommended for persons who engage in outdoor activities in endemic areas.
• Pets must be examined for ticks because they may carry ticks into the home.
EPIDEMIOLOGY
Predominant age: All ages; most patients present in their 40s or 50s.
Incidence
• Between 1968 and 1993, >450 Babesia infections were confirmed in the United States by blood smears or serologic testing. Prevalence is difficult to estimate because of lack of surveillance, and because infections are often asymptomatic (1,2).
• In a recent 1-year seroconversion study of patients in New York State who were at high risk for tick-borne diseases, antibodies to Babesia microti were seen in 7 of 671 participants (1%). (3)
Prevalence
High-level parasitemia is more common in asplenic patients. Such patients have been treated successfully with exchange transfusion in addition to drugs.
RISK FACTORS
• Exposure to endemic areas
• Transfusion-associated babesiosis and transplacental/perinatal transmission have been reported.
ETIOLOGY
• Babesia microti (in the United States) and Babesia divergens and Babesia bovis (in Europe) cause most infections in humans. Recently, one case of Babesia divergens was reported in the United States.
• A previously unknown species of Babesia (WA-1) was isolated from an immunocompetent man in Washington State who had clinical babesiosis. Researchers also described another probable new babesial species (MO1) associated with the first reported case of babesiosis acquired in the state of Missouri. MO1 is probably distinct from B. divergens, but the two share morphologic, antigenic, and genetic characteristics.
• Ixodid (or hard-bodied) ticks, in particular, Ixodes dammini (Ixodes scapularis) and Ixodes ricinus, are the vectors of the parasite.
ASSOCIATED CONDITIONS
• Coinfection with Borrelia burgdorferi and Babesia microti is relatively common in endemic areas.
• Coinfection with Ehrlichia species may also be seen. Three species of Ehrlichia have been described that infect humans, Ehrlichia chaffeensis, Ehrlichia phagocytophila, and Ehrlichia ewingii. Typically, patients have a nonspecific febrile illness. Rash is uncommon with human granulocytic ehrlichiosis but common with human monocytic ehrlichiosis. Laboratory findings often include leukopenia, thrombocytopenia, and increases in serum hepatic enzyme activities. (4)

DIAGNOSIS
SIGNS AND SYMPTOMS (3)
• Asymptomatic
• High fever (up to 40C [104F])
• Chills
• Diaphoresis
• Gastrointestinal (anorexia, nausea, abdominal pain, vomiting, diarrhea)
• Generalized weakness
• Fatigue
• Myalgia
• Respiratory (cough, shortness of breath)
• Headache
• Hepatomegaly and splenomegaly or evidence of shock
• Rash (uncommon)
• Central nervous system involvement includes headache, photophobia, neck and back stiffness, altered sensorium, and emotional lability
• Jaundice and dark urine may develop later in course of illness
TESTS
Lab
• Mild to severe hemolytic anemia (common nonspecific finding)
• Normal to slightly depressed leukocyte count (common nonspecific finding)
• Typical morphologic picture on the blood smear
• A Wright- or Giemsa-stained peripheral blood smear is most commonly used to demonstrate the presence of intraerythrocytic parasites.
• Rarely, tetrads of merozoites are visible.
• Serologic evaluation with the indirect immunofluorescent antibody test with use of Babesia microti antigen is available in a few laboratories. The cut-off titer for determination of a positive result varies with the particular laboratory protocol used, but in most laboratories, titers of more than 1:64 are considered consistent with Babesia microti infection. Tenfold to 20-fold higher titers can be observed in the acute setting, with a gradual decline over weeks to months. The correlation between the level of the titer and the severity of symptoms is poor.
• Detection of Babesia microti by polymerase chain reaction (PCR) is more sensitive and equally specific for the diagnosis of acute cases, in comparison with direct smear examination and hamster inoculation. PCR-based methods may also be indicated for monitoring of the infection.
Diagnostic Procedures/Surgery
Based on typical morphologic picture on the blood smear in conjunction with epidemiologic information
DIFFERENTIAL DIAGNOSIS
• Bacterial sepsis
• Hepatitis
• Lyme disease
• Ehrlichiosis
• Leishmaniasis
• Malaria
TREATMENT
GENERAL MEASURES
• Appropriate health care: Outpatient or inpatient, depending on symptoms
• Supportive care
MEDICATION (DRUGS)
First Line
• Atovaquone (Mepron): Suspension 750 mg b.i.d. plus azithromycin (Zithromax) 500-1,000 mg/d (5)[B]
• Combination of quinine (Quinamm): 650 mg of salt orally t.i.d. and clindamycin (Cleocin) 600 mg orally t.i.d., or 1.2 g parenterally b.i.d. for 7-10 days is the most commonly used treatment. (Pediatric: Dosage is 20-40 mg/kg/d for quinine and 25 mg/kg/d for Clindamycin.)
• In areas endemic for Lyme disease and ehrlichiosis, it may be advisable to add doxycycline (Vibramycin) 100 mg b.i.d. PO in the management of patients with babesiosis until serologic testing is completed.
• Exchange transfusion, together with antibabesial chemotherapy, may be necessary in critically ill patients. This treatment is usually reserved for patients who are extremely ill (blood parasitemia >10%, massive hemolysis and asplenia).
• Precautions: Clindamycin can lead to Clostridium difficile associated diarrhea.
Second Line
Several other drugs have been evaluated, including tetracycline, primaquine, sulfadiazine (Microsulfon), and pyrimethamine (Fansidar). Results have varied. Pentamidine (Pentam) has proved to be moderately effective in diminishing symptoms and decreasing parasitemia.
FOLLOW-UP
PROGNOSIS
• When left untreated, silent babesial infection may persist for months or even years.
• 139 hospitalized cases in New York State between 1982 and 1993 (3)
- 9 patients (6.5%) died.
- 1/4 of the patients were admitted to the ICU.
- 1/4 of the patients required hospitalization for more than 14 days.
• Alkaline phosphatase levels greater than 125 U/L, white blood cell counts greater than 5  109/L, history of cardiac abnormality, history of splenectomy, presence of heart murmur, and parasitemia values of 4% or higher were associated with disease severity.
COMPLICATIONS
• Congestive heart failure
• Disseminated intravascular coagulation
• Acute respiratory distress syndrome (can occur even a few days after the onset of effective antimicrobial treatment)
• Renal failure and myocardial infarction also have been associated with severe babesiosis.
PATIENT MONITORING
Monitor for complications (congestive heart failure, etc.) and follow parasitemia as needed.
REFERENCES
1. Quick RE, Herwaldt BL, Thomford JW, et al. Babesiosis in Washington State: A new species of Babesia? Ann Intern Med. 1993;119:284-290.
2. Persing DH, Herwaldt BL, Glaser C, et al. Infection with a Babesia-like organism in northern California. N Engl J Med. 1995;332:298-303.
3. White DJ, Talarico J, Chang HG, et al. Human babesiosis in New York State: A review of 139 hospitalized cases and analysis of prognostic factors. Arch Intern Med. 1998;158:2149-2154.
4. Mylonakis E. When to suspect and how to monitor babesiosis. Amer Fam Physician. 2001;63:1969-1974.
5. Krause PJ, Lepore T, Sikand VK, et al. Atovaquone and azithromycin for the treatment of babesiosis. N Engl J Med. 2000 Nov 16;343(20):1454-8.
6. Beattie JF, Michelson ML, Holman PJ. Acute babesiosis caused by Babesia divergens in a resident of Kentucky. N Engl J Med. 2002;29;347(9):697-698.
7. Gelfand JA. Babesia species. In: Mandell GL, Douglas RG, Bennett JE, Dolin R, eds. Mandell, Douglas, and Bennett's Principles and Practice of Infectious Diseases. 6th ed. New York, NY: Churchill Livingstone, 2005:3209-3215.
8. Gutman JD, Kotton CN, Kratz A. Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 29-2003. A 60-year-old man with fever, rigors, and sweats. N Engl J Med. 2003;349(12):1168-1175.
9. Pruthi RK, Marshall WF, Wiltsie JC, Persing DH. Human babesiosis. Mayo Clin Proc. 1995;70:853-862.
MISCELLANEOUS
See also: Lyme Disease

AVIAN FLU

2009-01-20T05:12:00.000-08:00

AVIAN FLU - Sheila M. Seed, BS, Pharm, MPH, RPH; Walter K. Goljan, MD
BASICS
DESCRIPTION
Avian influenza A subtype H5N1 is highly pathogenic and aggressive form of influenza. Presents with influenza-like symptoms, with lower respiratory tract symptoms (limited upper respiratory tract symptoms). Has a high mortality rate in elderly and very young.
GENERAL PREVENTION
• Consider with any patient with influenza-like symptoms who has had close contact with H5N1 or ill poultry.
• Chemoprophylaxis with antivirals should be considered if H5N1 circulating in community.
EPIDEMIOLOGY
Predominate age: All age groups
Prevalence
Rare
RISK FACTORS
• Direct contact with H5N1 virus.
• Contact with infected poultry
• Close contact with infected person
ETIOLOGY
• Infected poultry (domesticated ducks, turkeys, chickens)
• Low incidence of human-to-human transmission in household clusters and health care workers
ASSOCIATED CONDITIONS
Severe respiratory distress (common in severe cases)

DIAGNOSIS
PRE HOSPITAL
• Respiratory infection (incubation period 7 days).
• Standard precautions during transport.
SIGNS AND SYMPTOMS
• Primary phase (1-5)[A]
- Influenza-like symptoms with lower respiratory tract symptoms.
- Temp >100.4F (38C)
- Cough
- Sore throat
- Shortness of breath
- Diarrhea (watery without blood)
- Pleuritic pain
- Bleeding of nose and gums
- Conjunctivitis (rare)
• Secondary acute phase
- Severe respiratory distress
- Pneumonia not responsive to antibiotics
- Multiorgan dysfunction
History
• Known close contact with suspected or confirmed case.
• Close contact with infected poultry
• Travel within 10 days in high-risk area
Physical Exam
• Lab abnormalities (1,3,5)[A]
- Leukopenia (mainly lymphopenia)
- Thrombocytopenia (mild to moderate)
- Elevated aminotransferases (slight-moderate)
- Disseminated intravascular coagulation
- Decreased leukocyte, platelet, and lymphocyte counts (associated with increase risk of death)
• Chest radiograph (1,3,5)[A]
• Consolidation-bilateral and multifocal
- After 7 days-patchy lobar and interstitial infiltrates.
- Pleural effusions with cavitation (less common)
• Respiratory (1,3,5)[A]
- Respiratory distress
- Tachypnea
- Inspiratory crackles
TESTS
Lab
• CBC with differential
• Liver profile
• Chemical profile
• Blood culture
Imaging
Chest radiograph
Diagnostic Procedures/Surgery
• Lab confirmation of H5N1 virus is done case-by-case and requires one of the following (1,4)[A]
- Positive influenza A/H5 (Asian lineage) virus-real time reverse transcription polymerase chain reaction (LRN labs)
- Positive immunofluorescence test for antigen with use of monoclonal antibody against H5.
- Positive viral culture
- 4-fold rise in H5-specific antibody titer in paired serum samples.
DIFFERENTIAL DIAGNOSIS
• Acute respiratory syndrome
• Influenza
• Pneumonia
• SARS
TREATMENT
PRE-HOSPITAL
Standard and droplet precautions
STABILIZATION
• Ventilatory support within 48 hours (1,3)[B]
• Broad-spectrum antibiotics, antivirals agents, with or without corticosteroids until lab confirmation of H5N1 virus (5)[A]
GENERAL MEASURES
Nursing
• Use standard and droplet precautions.
• N-95 masks
MEDICATION (DRUGS)
All patients should receive neuraminidase inhibitors as soon as possible pending results of diagnostic lab tests.
ALERT
The use of amantadine (Symmetrel) and rimantadine (Flumadine) are not considered beneficial unless access to newer agents are unavailable. No vaccine is commercially available.
First Line
• Treatment of mild-moderate cases (1,5)[C]: Oseltamivir (Tamiflu) 75 mg PO b.i.d. for 5 days
• Treatment of severe cases (1,5)[C]: Oseltamivir(Tamiflu) 150 mg b.i.d. for 7-10 days
• Postexposure prophylaxis (1,5)[C]: Oseltamivir (Tamiflu) 75 mg PO once a day for 7-10 days
• Adverse effects: Generally well tolerated; nausea, vomiting, diarrhea, abdominal pain, headache, insomnia, bronchitis, vertigo.
• Drug interactions: Not metabolized by CYP450; drug interactions with drugs metabolized by this system are unlikely. Does not affect metabolism of acetaminophen.
Pediatric Considerations
• Pediatric treatment is weight-based. Safety and efficacy not established for children 1 year of age (1,5)[C]
- Oseltamivir 30 mg PO b.i.d. for 5 days 15 kg.
- Oseltamivir 45 mg PO b.i.d. for 5 days >15-23 kg
- Oseltamivir 60 mg PO b.i.d. for 5 days >23-40 kg
- Oseltamivir 75 mg PO b.i.d. for 5 days >40 kg
• Postexposure prophylaxis (1,5) [C]
- Dosing is weight-based as above but administered once daily for 7-10 days.
Geriatric Considerations
• Renal impairment (1,5)[C]
- Creatinine clearance 10-30 mL/min
 Treatment: Oseltamivir 75 mg PO daily.
 Postexposure prophylaxis: Oseltamivir 75 mg PO every other day or 30 mg PO daily
• Hepatic impairment (1,5)[C]
- No dosage adjustment needed.
Pregnancy Considerations
• Oseltamivir is Category C
• Use with caution only if potential benefits outweigh possible risk.
• Unknown if distributed in breast-milk
Second Line
• Zanamivir (Relenza) is considered second-line agent. Not recommended for patients with underlying respiratory disease (asthma, COPD) (5)[C]
• Treatment (ages 13-65 years)
- Zanamivir 10 mg (2 inhalations) b.i.d. for 5 days.
• Postexposure prophylaxis (ages 13-65)
- Zanamivir 10 mg (2 inhalations) once daily for 7-10 days.
• Adverse effects
- Hypersensitivity reactions: Bronchospasms and allergic-like reactions have occurred.
- Diarrhea, nausea, vomiting, headache, dizziness, sinusitis, cough, throat infections
- Some adverse effects due to lactose in powder of inhaler
• Drug interactions: Not metabolized by CYP450; drug interactions with drugs metabolized by this system are unlikely.
Pediatric Considerations
• Zanamivir is not licensed for use in children 7 years of age for treatment and 5 years for prophylaxis (5)[C].
• Treatment (7-13 years of age): Zanamivir 10 mg (2 inhalations) b.i.d. for 5 days.
• Prophylaxis (5-13 years of age): Zanamivir 10 mg (2 inhalations) once daily 7-10 days.
Geriatric Considerations
No dosage adjustment for renal or hepatic impairment (5)[C]
Pregnancy Considerations
• Zanamivir is Category C
• Use with caution only if potential benefits outweigh possible risk.
• Unknown if distributed in breast-milk
• Other medications (1,5)[C]
- Broad-spectrum antibiotics: Follow hospital protocols for community-acquired pneumonia.
- Corticosteroids: No clear evidence of benefits
- Interferon-: No basis for use
FOLLOW-UP
DISPOSITION
Admission Criteria
If known H5N1 activity in community, or if patient has traveled to country with H5N1 activity, admission should be considered if patient presents with
• Severe acute respiratory illness
• Serious unexplained illness (encephalopathy or diarrhea)
Discharge Criteria
If discharged early, family requires education of proper personal hygiene and infection-control measures. Postexposure prophylaxis given to family members.
PROGNOSIS
Mortality rate is high (some reports >50%). Median time to death was 9 days (range 6-17 days) with or without treatment.
COMPLICATIONS
• Multiorgan failure, acute (1-4)[C]
• Renal dysfunction
• Cardiac compromise
• Cardiac dilatation, supraventricular tachyarrhythmias
• Ventilator-associated pneumonia
• Pulmonary hemorrhage
• Pneumothorax
• Pancytopenia
• Reye syndrome
• Sepsis syndrome without documented bacteremia.
PATIENT MONITORING
Clinical deterioration is rapid.
REFERENCES
1. Beigel JH, Farrar J, et al. Avian influenza A (H5N1) infections in humans. N Engl J Med. 2005;350:1374-1385.
2. World Health Organization. WHO international guidelines on clinical management of humans infected by influenza A (H5N1). February 20, 2004. Accessed Sep 19, 2006 at http://who.int/csr/disease/avian_influenza/guidelines/Guidelines_Clinical%20Management_H5N1_rev.pdf.
3. Chotpitayasunondh T, Ungchusak K, Hanshaoworakul W, et al. Human disease from influenza A (H5N1), Thailand, 2004. Emerg Infect Dis. 2005;11:201-209.
4. Hien TT, Liem NT, Dung NT, et al. Avian influenza A (H5N1) in 10 patients in Vietnam. N Engl J Med. 2004;350:1179-1188.
5. World Health Organization. WHO Rapid advice guidelines on pharmacological management of humans infected with avian influenza A (H5N1) virus. May 2006. Accessed Sep 19, 2006 at http://who.int/csr/disease/avian_influenza/guidelines/pharmamanagement/en.
ADDITIONAL READING
Centers for Disease Control and Prevention CDC: Question  Answers about Avian Influenza http://www.cdc.gov/flu/avian/gen-info/qa.htm.
MISCELLANEOUS
Report any cases to public health officials surveillance is necessary to monitor for possible pandemic.