Novel Surgical Technique for Correction of Incomplete Median Cleft Lip Deformity in Oral-Facial-Digital Syndrome Type II.

J Craniofac Surg

Department of Plastic and Reconstructive Surgery, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan.

Published: November 2021

AI Article Synopsis

  • Oral-facial-digital syndromes (OFDSs) are a diverse group of developmental disorders that lead to facial, oral, and limb malformations, with type II being an autosomal recessive condition.
  • In this case, a 5-year-old girl with OFDS type II had an incomplete median cleft lip, along with other medical issues, and previous surgeries.
  • The novel surgical method described focused on repairing the median cleft lip to create a better-defined Cupid's bow and adequate lip fullness, resulting in a successful outcome.

Article Abstract

Oral-facial-digital syndromes (OFDSs) represent a heterogenous group of embryonic development disorders characterized by malformations of the face, oral cavity, and extremities. Oral-facial-digital syndrome type II is an autosomal recessive disease characterized by median cleft lip, gingival frenula, cleft lobulated tongue, and polydactyly. There are few reports on surgical techniques for correction of incomplete median cleft lip. Here we describe a novel surgical method that we used to correct an incomplete median cleft lip in a 5-year-old girl with oral-facial-digital syndrome type II. She had previously undergone surgery for congenital heart disease, oral anomalies, and polydactyly. Cheiloplasty was performed at 5 years and 8 months using a surgical approach that focused on repair of the median tubercle using lateral labial elements. A reasonably good Cupid's bow and median tubercle were achieved. Our technique for surgical correction of moderate incomplete median cleft lip provides adequate philtral height, vermillion fullness, and a good-shaped Cupid's bow.

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http://dx.doi.org/10.1097/SCS.0000000000007720DOI Listing

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