Unilateral macrostomia in the newborn: a rare congenital anomaly of the oral commissure

Abstract

Macrostomia is a rare medical condition, defined as an enlargement of the mouth at the oral commissure. The incidence varies between 1 in 60 000 to 1 in 300 000 live births. Macrostomia is a form of a facial cleft. Macrostomia can present as a unilateral or bilateral anomaly with a partial or complete cleft. Associated anomalies of the surrounding bone, muscle and soft tissue can also be present with or without the presence of a syndrome. Macrostomia results in aesthetic disharmony and also in functional problems. In both cases surgery is the treatment of choice. In cases of macrostomia, additional investigations should be performed to rule out accompanying cardiac and renal anomalies and associated syndromes. A multidisciplinary approach and good collaboration between healthcare providers is essential for optimal care of these patients.

Surgical correction for Tessier number 7 craniofacial cleft using a medially overcorrected design

“…Macrostomia presents both aesthetic and functional problems. Aesthetic problems include an asymmetrical lip contour relative to that on the normal side, while functional problems include feeding difficulties, drooling, and incoherent speech [10]. Therefore, the surgical goals for macrostomia are to create lips that are symmetrical to those on the normal side and to pro- mote normal lip function by properly repairing the orbicularis oris muscle.…”

Depressor Anguli Oris Function in Oral Macrostomia

“…C ongenital macrostomia (transverse facial cleft or lateral cleft lip) is a rare disorder (incidence ranging from 1:60,000 to 1:300,000 live births) resulting from failure of fusion of the maxillary and mandibular portions of the first and second branchial arches as they migrate to the lateral commissures of the mouth. [1][2][3][4][5][6][7] It is approximately 200-times less common than a traditional cleft lip. 8 Congenital macrostomia can present as a unilateral or, less commonly, bilateral anomaly with a partial or complete lateral cleft.…”

“…Extent of clefting ranges from involvement of the orbicularis oris muscle to the tragus, and can be seen in isolation or conjunction with other craniofacial abnormalities. 1,9 Cartilaginous tags, accessory tragi, and preauricular skin are the most frequently observed anomalies in approximately 60% to 70% of unilateral cases; the facial nerve is very rarely involved. 1,2,10,11 Associated morbidities include speech impairment, oral incompetence, and difficulty with facial expression.…”

“…1,9 Cartilaginous tags, accessory tragi, and preauricular skin are the most frequently observed anomalies in approximately 60% to 70% of unilateral cases; the facial nerve is very rarely involved. 1,2,10,11 Associated morbidities include speech impairment, oral incompetence, and difficulty with facial expression. Surgical correction remains the mainstay of treatment to address both aesthetic and functional complications of all variants.…”

“…Congenital macrostomia can present as a unilateral or, less commonly, bilateral anomaly with a partial or complete lateral cleft. Extent of clefting ranges from involvement of the orbicularis oris muscle to the tragus, and can be seen in isolation or conjunction with other craniofacial abnormalities 1,9 . Cartilaginous tags, accessory tragi, and preauricular skin are the most frequently observed anomalies in approximately 60% to 70% of unilateral cases; the facial nerve is very rarely involved 1,2,10,11 .…”