Cheek fistula from the ectopic salivary gland: a variant of the oculo-auriculo-vertebral spectrum.

Objectives/Hypothesis This study aimed to investigate the diagnosis and management of a distinct developmental deformity syndrome characterized by congenital cheek fistula, ectopic accessory parotid gland, and preauricular appendage. Study Design Retrospective study. Methods We analyzed the medical records, radiologic and histopathologic findings, and follow-up data for seven patients (four males) with a congenital cheek salivary fistula. Computed tomography, fistulography, and sialography had been performed for diagnosis. Surgical treatment effect was evaluated. Results The mean age of the patients was 8.1 years (range, 2–16 years). The distinctive clinical feature was a congenital skin orifice lateral to the commissure with saliva discharge during eating. The cheek fistulae were accompanied by ipsilateral preauricular appendages in all seven patients. The skin orifice connected to an ectopic gland anterior to the masseter and inferior to Stensen's duct. Parotid sialography demonstrated an intact Stensen's duct in all cases. Hypoplasia of the ipsilateral mandible could be observed in five cases. Excision of the ectopic gland, skin orifice, and fistula was performed in five cases resulting in optimal treatment outcomes with no recurrent or adverse events. Conclusions A congenital saliva-discharging fistula with an ectopic accessory parotid gland, ipsilateral preauricular appendage, and mandibular hypoplasia constitutes a rare developmental syndrome. Surgical excision can effectively treat congenital cheek salivary fistula. Level of Evidence 4. Laryngoscope, 125:360–364, 2015