A rare syndrome with unusual dental findings: Oculo-facio-cardio-dental syndrome

J Oral Maxillofac Pathol. 2014 May;18(2):331. doi: 10.4103/0973-029X.140928.

Abstract

Radiculomegaly of a tooth is a rare condition. When it is associated with other dental abnormalities, facial dysmorphism and congenital cataracts, the condition is named as oculofaciocardiodental (OFCD) syndrome. This is an X-linked dominant trait, reported only in women, suggesting that it is lethal in males. The most consistent and pathognomic dental finding of OFCD is radiculomegaly (extremely long roots), particularly of the canines and occasionally of other teeth including premolars and incisors, which can easily be diagnosed on dental panoramic radiographs by a dentist or an orthodontist. Till date, 21 cases have been reported worldwide. The aim of this report is to present a new case of a 24 year old female affected by this syndrome and to evaluate it from a dental perspective to call the attention of dentists to this rare anomaly.

Keywords: Congenital cataract; X-linked dominant inheritance; facial dimorphism; radiculomegaly.

Publication types

  • Case Reports