First Branchial Cleft Malformation with Duplication of External Auditory Canal

First branchial cleft anomalies are uncommon, accounting for less than 10% of all branchial abnormalities. Their rare occurrence and varied presentation have frequently led to misdiagnosis and inadequate and inappropriate treatment of these conditions leading to repeated recurrences and secondary infection. In this paper, a case of 11-year girl with type 2 first branchial cleft defect is described. She first presented with a nonhealing ulcer of upper neck from childhood. Diagnosis had previously been missed and treated as tubercular ulcer. We confirmed the correct diagnosis by history and computerized tomography fistulogram. The lesion was completely excised with no further recurrence. 1. Introduction First branchial cleft anomalies are a special group of congenital malformations of head and neck. The incidence of these lesions is quite low, accounting for fewer than 10% of all branchial cleft anomalies, and often involves the external auditory canal structures [1, 2]. A wide range of clinical manifestations may be observed and they are usually associated with infection. Symptoms in form of cystic swelling, discharging sinuses, and fistulas occur in the periauricular and cervical region located above a line passing through the hyoid bone. Management is often inadequate with recurrences because of misdiagnosis. Several authors proposed classifications to assist appropriate diagnosis and management of these lesions. First branchial arch anomalies have been classified into two types. Type-1 anomaly is of ectodermal origin, manifests as cyst or sinus in the parotid gland, and appears during early or middle adult life [3, 4]. Type 2 defects are ectodermal and mesodermal origin, containing skin with adnexal structures as well as cartilage, and are associated with a sinus/fistula in the anterior triangle of neck, with a communicating tract to external auditory canal (EAC) manifesting during childhood [3, 4]. This paper reports a case of type 2 first branchial cleft defect (collaural fistula) to highlight specific diagnostic clinical features and discusses the significance of this condition in otolaryngology. The roll of computerized tomography (CT) fistulogram in diagnosis type 2 defect of first branchial cleft is described. 2. Case Report An 11-year girl presented to our department with a nonhealing ulcer in right upper neck and intermittent right ear discharge. The neck ulcer first developed when she was 2 years old and initially started as a swelling which ruptured and formed a fistula with a surrounding ulcer. She had had numerous incision and drainage