A case of oculo-facio-cardio-dental syndrome with integrated orthodontic-prosthodontic treatment

Cleft Palate Craniofac J. 2004 Jan;41(1):84-94. doi: 10.1597/02-133.


Objective: Oculo-facio-cardio-dental (OFCD) syndrome is a very rare condition that requires comprehensive dental management because of associated multiple dental anomalies such as canine radiculomegaly, delayed dentition, oligodontia, persistent primary teeth, microdontia, and macrodontia. This report presents a case of OFCD in a Japanese girl (13 years 1 month old). We analyzed cephalograms, panoramic roentgenograms, and dental casts and discuss our integrated orthodontic-prosthodontic treatment.

Design: The sizes of the tooth crown and root as well as lateral cephalograms were compared with those from a Japanese control group. The outcome of orthodontic treatment was evaluated by comparing cephalograms taken before and after treatment.

Results: Radiculomegaly was found not only in the upper and lower canines but also the upper central incisors and lower first premolars. Macrodontia was found in the upper central incisors, upper canines, lower canines, and lower first premolars. Microdontia was found in the upper lateral incisor. Lateral cephalometric analysis showed a remarkable hypoplastic midface in both the sagittal and vertical dimensions, coupled with a significantly decreased cranial base length and an increased gonial angle. Serial lateral cephalograms during orthodontic treatment from 13 to 23 years of age demonstrated only slight maxillary growth and significant downward mandibular growth with clockwise rotation in addition to pronounced labial tipping of the upper central incisors.

Conclusions: Characteristic dental anomalies together with a unique craniofacial dysmorphology were clarified. Successful oral rehabilitation was achieved by integrated orthodontic-prosthodontic treatment.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple*
  • Adolescent
  • Cataract / congenital*
  • Cephalometry
  • Denture, Partial, Fixed
  • Eye Abnormalities* / surgery
  • Facial Bones / abnormalities
  • Female
  • Heart Septal Defects, Ventricular*
  • Humans
  • Malocclusion / therapy*
  • Orthodontics, Corrective
  • Radiography, Panoramic
  • Skull Base / abnormalities
  • Strabismus / surgery
  • Syndrome
  • Tooth Abnormalities* / diagnostic imaging
  • Tooth Abnormalities* / rehabilitation
  • Tooth Root / abnormalities