BRANCHIAL CLEFT FISTULA

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.