Distraction in a case of otopalatodigital syndrome type II.

Summary.  Introduction.  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. Case report.  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. Conclusion.  We also discuss the differential diagnosis and consider more recent theories on possible aetiology as well as clinical management strategies for such cases.