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.
Figure 1. The malformation of the right pinna included an accessory auricle (arrow) composed of cartilage that appeared to be a remnant tragus. Lateral to the canal is a soft-tissue bulge (asterisk), which, when reflected anteriorly, reveals a fully mucosalized fistulous tract.
Figure 2. Coronal computed tomographic image of the right temporal bone. The right external auditory canal (EAC) and middle ear spaces are opacified. There is an absence of the inferior wall of the EAC and floor of the middle ear, with ossifications in the expected region of the floor containing 2 unerupted teeth.
Figure 3. A transoral view of the right lateral pharynx with an absent posterior pillar and tonsil replaced by aberrant lingual tissue. *Aberrant lingual tissue; †anterior pillar.
Figure 4. Elevation of the fistulous tract, with a free-floating tooth extracted from the tympanic ring remnant. *Tooth; patient orientation: †anterior, ‡posterior, §superior, and ∥inferior.
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