Bilateral macrostomia as an isolated pathology

Abstract

Objective: Congenital macrostomia is a lateral orofacial cleft between the maxillary and mandibular components of the first branchial arch. Bilateral macrostomia is a poorly characterized malformation, with only 14 cases reported in the literature. The purpose of this study was to compare our experience with the world literature.

Method: A retrospective analysis of 20 cases of bilateral congenital macrostomia was conducted; 6 cases were drawn from 2 maxillofacial surgery units and 14 cases from the world literature. Cases of bilateral congenital macrostomia were compared with cases of unilateral forms using a review of the literature post-1954. Among the six cases identified from the two maxillofacial surgery units, three were treated with linear sutures and three with Z-plasty. Subsequent aesthetic and functional results were analyzed.

Results: Compared to unilateral forms, bilateral macrostomia is more often isolated without ear or skin deformities. Moreover, there are a greater proportion of larger defects among cases with bilateral macrostomia when compared to unilateral macrostomia. Alimentation, phonation, and mouth opening were always normal. The two sides were always symmetric. Only one case presented with the complication of skin contractions during lip movement.

Conclusion: The etiopathogenesis of bilateral macrostomia is unclear. Although over 50% of the reported cases of bilateral macrostomia are isolated, this condition presents a therapeutic challenge. In the case of bilateral forms, the surgeon must define the commissure position without a normal side. Repair thus requires extraoral landmarks and normal measurements.