Distraction in a case of otopalatodigital syndrome type II.
BACKGROUND
Otopalatodigital syndrome type II is a rare X-linked recessive disorder with generalized skeletal dysplasia and hearing anomalies. Its features include conductive hearing loss, unusual facies, cleft palate, micrognathia, and overlapping flexed fingers and toes. It is a more lethal variant of otopalatodigital syndrome type I. There are many consistently reported craniofacial and dental findings; however, no case has been published in dental literature.


METHODS
We report a case of otopalatodigital syndrome type II with micrognathia, cleft of the soft palate, and partial anodontia, and discuss the combined orthodontic and surgical management.


CONCLUSIONS
We also discuss the differential diagnosis and consider more recent theories on possible aetiology as well as clinical management strategies for such cases.
Batra
P
P
Maxillofacial and Dental Department, Great Ormond Street Hospital for Children NHS Trust, London WC1N 3JH, UK.
Ryan
F S
FS
Witherow
H
H
Calvert
M L
ML
D000848:Anodontia / Q000235:genetics; D002972:Cleft Palate / Q000235:genetics / Q000601:surgery; D019465:Craniofacial Abnormalities / Q000235:genetics / Q000601:surgery*; D005146:Facial Asymmetry / Q000601:surgery; D019066:Facies; D005385:Fingers / Q000002:abnormalities; D005500:Follow-Up Studies; D040181:Genetic Diseases, X-Linked / Q000235:genetics; D006314:Hearing Loss, Conductive / Q000235:genetics; D006801:Humans; D007223:Infant; D008297:Male; D008312:Malocclusion, Angle Class II / Q000601:surgery; D008336:Mandibular Diseases / Q000601:surgery; D008844:Micrognathism / Q000235:genetics / Q000601:surgery; D010009:Osteochondrodysplasias / Q000235:genetics; D019857:Osteogenesis, Distraction; D010160:Palate, Soft / Q000002:abnormalities; D013577:Syndrome; D014034:Toes / Q000002:abnormalities