Abstract
Transverse facial clefts (macrostomia) are rare disorders that result when the embryonic mandibular and maxillary processes of the first branchial arch fail to fuse due to failure of mesodermal migration and merging to obliterate the embryonic grooves between the maxillary and mandibular processes to form the angle of the mouth at its normal anatomic position. Macrostomia may be seen alone or in association with other anomalies. It may be unilateral, extending along a line from the commissure to the tragus or bilateral. It is usually partial but rarely complete. Transverse facial clefts are more common in males and more common on the left side when unilateral. The goal of macrostomia reconstruction is to achieve functional, symmetrical, and accurate oral commissure with minimal scar. In this paper, we present a six-year-old girl with unilateral macrostomia with preauricular skin tags and malformation of pinna on ipsilateral side treated with vermillion-square flap method. The scar is placed at the upper lip. At two-month followup, the oral commissures are symmetric, the scars are inconspicuous, and the overall balance of facial contour and lip is excellent. We recommend this method for patients with mild to moderate macrostomia.
Highlights
Transverse facial or Tessier No 7 cleft or congenital macrostomia is a rare congenital anomaly [1]. This developmental aberration results from failure of fusion of the maxillary and mandibular processes of the first branchial arch [2, 3]. This explains the frequent association of transverse cleft with developmental anomalies of the first and second branchial arches [2]
Anderson’s (1965) series of 3988 cases of facial clefts treated in Denmark over a period of 30 years gave a figure of 13 patients with macrostomia, an incidence of 0.3% of the total series [5]
We report an excellent result of applying this technique on a 6-year-old girl with unilateral macrostomia
Summary
Transverse facial or Tessier No 7 cleft or congenital macrostomia is a rare congenital anomaly [1] This developmental aberration results from failure of fusion of the maxillary and mandibular processes of the first branchial arch [2, 3]. Anderson’s (1965) series of 3988 cases of facial clefts treated in Denmark over a period of 30 years gave a figure of 13 patients with macrostomia, an incidence of 0.3% of the total series [5] Almost all of these patients had other associated anomalies making isolated cases a rarity. Other etiopathogeneses have been given including those of Mckenzie and Craig [4] who believe the defects of the first branchial arch arise from inadequate arterial blood supply occurring during a period of rapid and critical facial growth and development It could vary from slight widening of the mouth to a cleft. We report an excellent result of applying this technique on a 6-year-old girl with unilateral macrostomia
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