Bilateral macrostomia as an isolated deformity and its repair with a modified technique

Abstract

A 3-year-old boy with bilateral congenital macrostomia as an isolated clinical entity is presented. Although this is a rare deformity, it causes functional and cosmetic problems which are difficult to correct. The skin, muscle, and mucosal components of the deformity were separately repaired using a modification of Kaplan's technique.

Bilateral macrostomia as a sole entity

“…In bilateral cases the accurate positioning of the new commissure is more challenging than unilateral cases because of absence of the normal side as a reference [10]. The methods for localizing the position of new commissure include (a) considering the anatomical landmarks like change in vermillion color and in white line, and presence of muscular ridge at the normal commissure site, (b) based on relation to the iris, a vertical plane is dropped from the medial limbus of the iris which corresponds to oral commissure, and (c) referring the standard lip measurement tables for a particular race, age group and gender [14].…”

“…Moreover, bilateral macrostomia is more distressing to the patient because it causes disruption of oral competence on both sides resulting in failure of lower lip to contact the upper lip. An intact oral sphincter is required not only for feeding and speech but also for facial expression, and disruption of orbicularis oris ring causes difficulty in performing these functions [10]. Therefore, an early repair will lead to early restoration of the function of the oral sphincter [6].…”

“…Many techniques of macrostomia repair such as straight-line closure, W-plasty, Z-plasty, double reverse Z-plasty, skin musclevermilion flap and triangular flaps are suggested in the literature [2,[4][5][6]10]. However, the objectives in the repair of macrostomia include symmetry of lips, normal function of the orbicularis oris muscle, accurate positioning and natural look of the oral commissure with prevention of its lateral displacement due to scar contraction, and ultimately, cutaneous closure with minimum scar formation [6].…”

Anesthetic management of a pediatric patient with rare bilateral macrostomia

“…In this case, the anesthesia consisted simply of sevoflurane, nitrous oxide and a muscle relaxant. Although dystonic reactions have been reported in cases using halogenated-volatile anesthetics, drugs potentially provoking dystonic reactions were used in all cases (1). Neither the patient nor his mother took any medicines in this case.…”

“…Although rare, the presence of a perforating foreign body can be associated with life-threatening complications (1). Jacob et al described a case of an embedded tooth brush head in the neck which had been retained for a long time (2).…”