Family Resource Network
Volume 16 Issue 4, Pages 286 - 291
Distraction in a case of otopalatodigital syndrome type II
P. BATRA, F. S. RYAN, H. WITHEROW & M. L. CALVERT
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.