Distraction in a case of otopalatodigital syndrome type II

Int J Paediatr Dent. 2006 Jul;16(4):286-91. doi: 10.1111/j.1365-263X.2006.00714.x.


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.

Publication types

  • Case Reports

MeSH terms

  • Anodontia / genetics
  • Cleft Palate / genetics
  • Cleft Palate / surgery
  • Craniofacial Abnormalities / genetics
  • Craniofacial Abnormalities / surgery*
  • Facial Asymmetry / surgery
  • Facies
  • Fingers / abnormalities
  • Follow-Up Studies
  • Genetic Diseases, X-Linked / genetics
  • Hearing Loss, Conductive / genetics
  • Humans
  • Infant
  • Male
  • Malocclusion, Angle Class II / surgery
  • Mandibular Diseases / surgery
  • Micrognathism / genetics
  • Micrognathism / surgery
  • Osteochondrodysplasias / genetics
  • Osteogenesis, Distraction*
  • Palate, Soft / abnormalities
  • Syndrome
  • Toes / abnormalities