Branchial cleft fistula

Branchial cleft fistula

OVERVIEW:

A congenital, abnormal tract connecting the skin of neck with an internal structure, resulting from failure of closure of a branchial cleft.
• May involve branchial clefts I-IV

System(s) affected: Skin/Exocrine
Genetics: 10% have family history
Incidence/Prevalence in USA: Unknown
Predominant age: By definition are all present at birth although may remain unnoticed for some time. (Branchial cleft cysts may not present until later childhood.)
Predominant sex: Unknown

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, fever
• 10% are bilateral

CAUSES:

• 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.
• 3rd and 4th branchial clefts form parathyroid glands, thymus and portions of thyroid (parafollicular cells). Fistulae are rare, those from 3rd cleft course lateral to carotid artery, both should have external ostia on lower anterior neck.

RISK FACTORS:

Positive family history

DIAGNOSIS

DIFFERENTIAL DIAGNOSIS:

• External sinuses
• Cystic hygroma
• Dermoid cysts
• Lymphadenopathy

LABORATORY:

Culture only if signs of infection

Drugs that may alter lab results: N/A
Disorders that may alter lab results: N/A

PATHOLOGICAL FINDINGS:

Lined by stratified squamous epithelium, may contain hair follicles, sweat glands, sebaceous glands, cartilage. Some are lined by ciliated columnar epithelium.

SPECIAL TESTS:

N/A

IMAGING:

N/A

DIAGNOSTIC PROCEDURES:

Sinogram or fistulogram may be done but is of little value

TREATMENT

APPROPRIATE HEALTH CARE:

• Surgical excision
• Outpatient status usually appropriate

GENERAL MEASURES:

N/A

SURGICAL MEASURES:

• Small transverse incision at external ostium with careful dissection of fistula
• Stepladder incisions may be needed
• End of fistula ligated flush with pharyngeal mucosa
• Drains are not used
• Antibiotics only for infection

ACTIVITY:

N/A

DIET:

N/A

PATIENT EDUCATION:

MEDICATIONS

DRUG(S) OF CHOICE:

N/A

Contraindications: N/A
Precautions: N/A
Significant possible interactions: N/A

ALTERNATIVE DRUGS:

N/A

FOLLOW UP

PATIENT MONITORING:

• Follow at weekly intervals, if infected, until resolution, than excision
• Postoperative visit at 2 weeks

PREVENTION/AVOIDANCE:

N/A

POSSIBLE COMPLICATIONS:

• Facial nerve injury
• Infection
• Carotid artery injury
• Possible recurrence if any epithelium remains
• Neoplastic degeneration of branchial remnants (about 250 reported cases) if not resected

EXPECTED COURSE AND PROGNOSIS:

Good

MISCELLANEOUS

ASSOCIATED CONDITIONS:

Microtia and aural atresia occur with failure of development of 1st branchial cleft.

AGE-RELATED FACTORS:

Pediatric: Almost all occur in pediatric age group
Geriatric: N/A
Others: N/A

PREGNANCY:

N/A

SYNONYMS:

N/A

ICD-9-CM:

744.41 branchial cleft sinus or fistula

(see images)

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Medical Diagnosis » B » Branchial cleft fistula