Bilateral macrostomia as a sole entity

Congenital macrostomia is frequently associated with other anomalies and syndromes, but isolated cases are rare. It occurs due to failure of fusion of maxillary and mandibular processes of the first branchial arch to form the oral commissure. The clinical manifestations are variable, ranging from slight involvement of oral commissure to complete division of the face along a line from oral commissure to tragus. It is more common in males than females and unilateral form is six times more common than the bilateral one. Macrostomia causes esthetic disharmony, functional problems, anesthetic challenges and psychological impact on both the child and the family. Various techniques of macrostomia repair such as straight-line closure, W-plasty, Z-plasty, double reverse Z-plasty, skin muscle-vermilion flap and triangular flaps are described in the literature. However various problems remain like asymmetric closure, deviation/drooping and distortion of the commissure, goldfish mouth deformity, distortion of nasolabial fold and scar formation. The key point in the repair is the reconstruction of a muscular commissure. With Z- or W-plasty the scar is more conspicuous and distortion of nasolabial fold can also occur. We report a rare case of asyndromic bilateral macrostomia in a 7-year-old male repaired by Pfeifer wave line incision technique. This technique is simple, easy to master and yields good functional and cosmetic results. In the case presented herein, after 2 years of follow-up the scar was minimal and no lateral deviation of the commissure or distortion of the nasolabial fold was noted.