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Clinical Note
Feb 2012

Congenital Pharyngo-oto-cutaneous Fistula: Surgical Management of an Unusual Anomaly of the First Branchial Apparatus

Author Affiliations

Author Affiliations: Department of Head and Neck Surgery, Kaiser Permanente Medical Center, Oakland, California.

Arch Otolaryngol Head Neck Surg. 2012;138(2):189-192. doi:10.1001/archoto.2011.1072

First branchial anomalies embody a small subset of congenital malformations of the head and neck. Classically, they are duplication abnormalities of the external auditory canal (EAC) and can involve the middle ear and parotid gland. Herein, we describe a 5-year-old girl with unilateral microtia, autism, and Cayler cardiofacial syndrome (asymmetric crying facies and congenital heart disease). She also had a congenital pharyngo-oto-cutaneous fistula resulting in high-volume otorrhea of saliva and formula. We present our experience with surgical correction of this unique condition. To our knowledge, this is the first reported case of a congenital pharyngo-oto-cutaneous fistula involving a derangement of all 3 germ layers of the first branchial apparatus.