The ectopic accessory parotid system with congenital cheek fistula: An overview and current update

Abstract

Objective: This review attempts to provide a comprehensive, updated overview of the ectopic accessory parotid system (EAPS) from clinical and developmental perspectives and investigates its eligibility to be included in the oculo-auriculo-vertebral spectrum (OAVS).

Review methods: Results of the keyword-based search in the PubMed/MEDLINE, Google Scholar, LILACS, and Cochrane Library were subjected to the given inclusion and exclusion criteria that corroborated with the definition of EAPS. Eleven records were shortlisted, their full texts studied and references cross-checked. Finally, 10 articles collectively describing 16 patients were considered for review.

Results: The average age of presentation was 8.33 years, with 44% of the patients aged below 5 years. Boys were affected 1.67 times more. The cheek fistula was always unilateral, with marginal left predilection. About 94% of the patients were of East Asian descent, chiefly from China and India. Thirteen children had preauricular appendages, mostly ipsilateral, with occasional ipsilateral mandibular hypoplasia (25%). The ectopic parotid was anterolateral to the masseter, lateral to buccinator, and inferior to the Stensen duct. Complete surgical removal of the EAPS (intraoral approach) or transposing the fistula into the oral cavity formed the mainstay of treatment.

Conclusion: The EAPS is a rare craniofacial anomaly presenting with a saliva-draining cheek fistula, associated with ipsilateral preauricular appendages (microtia) and occasional mandibular hypoplasia. Speculative developmental theories suggest dysmorphogenesis of the first two pharyngeal arches, clinically and embryologically relating it with OAVS. This review summarizes the clinical aspects, and establishes isolated EAPS as a mild but discrete phenotype of OAVS irrespective of the presence of other congenital stigmata. Laryngoscope, 127:1351-1360, 2017.

Keywords: Ectopic accessory parotid gland; Goldenhar syndrome; congenital cheek fistula; oculo-auriculo-vertebral spectrum; salivary fistula.