Clinical Manifestations, Diagnosis, and Management of First Branchial Cleft Fistula/Sinus: A Case Series and Literature Review

Abstract

Purpose: First branchial cleft fistula/sinus is a rare congenital developmental deformity that can sometimes be acquired from incision and drainage of a branchial cleft cyst. The aim of the present study was to explore the clinical manifestations, diagnosis, and surgical management of first branchial cleft fistula/sinus in both a large patient series and a review of the pertinent literature.

Materials and methods: The data from 31 cases diagnosed from February 2004 to April 2019 as first branchial cleft fistula/sinus were retrospectively reviewed. The patient demographic data and outcomes were explored. In addition, we performed a literature review of studies reported from 1923 to 2018 for first branchial cleft fistula/sinus and summarized those results.

Results: The present study included 31 patients (15 males, 16 females) with a median age of 4 years. All the patients reviewed had presented with a unilateral first branchial cleft fistula/sinus. The parotid region was the most frequent site of presentation (41.9%) in these cases. The fistula/sinus had occurred on the left side in 13 patients (41.9%) and on the right side in 18 patients (58.1%). Of the 31 patients, 24 (77.4%) had acquired the disease from infection of an existing brachial cleft cyst or incomplete previous excision. Of the 31 cases, 28 (90%) had an intimate relationship between the tract and the facial nerve. Despite this close association, no patient developed postoperative facial nerve palsy. Of the 31 operations, 30 (97%) successfully accomplished complete resection with no recurrence postoperatively. Only 1 patient with a history of multiple recurrences experienced a subsequent recurrence, which was successfully treated with a second surgery.

Conclusions: First branchial cleft fistula/sinus is a frequently misdiagnosed and, therefore, undertreated entity, which leads to recurrence. It is closely associated with the facial nerve and extra auditory canal. The correct diagnosis and meticulous removal can be effectively achieved with minimal risk to the facial nerve.