Journal
JOURNAL OF CRANIOFACIAL SURGERY
Volume 32, Issue 8, Pages E741-E742Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/SCS.0000000000007720
Keywords
Incomplete median cleft lip correction; Mohr syndrome; oral-facial-digital syndrome
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Oral-facial-digital syndrome type II is a rare autosomal recessive genetic disease characterized by features such as median cleft lip. The article describes a novel surgical method for repairing incomplete median cleft lip, which provided the patient with a good cosmetic outcome.
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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