Brb. Schulze et al., Rare dental abnormalities seen in oculo-facio-cardio-dental (OFCD) syndrome: Three new cases and review of nine patients, AM J MED G, 82(5), 1999, pp. 429-435
Oculo-facio-cardio-dental syndrome is a very rare condition. So far, only n
ine cases have been documented. We report on three additional female patien
ts representing the same entity. The clinical findings were: congenital cat
aract, microphthalmia/microcornea, secondary glaucoma, vision impairment, p
tosis, long narrow face, high nasal bridge, broad nasal tip with separated
cartilages, long philtrum, cleft palate, atrial septal defect, ventricular
septal defect, and skeletal anomalies. The following dental abnormalities w
ere found: radiculomegaly, delayed dentition, oligodontia, root dilaceratio
ns (extension), and malocclusion. For the first time, fusion of teeth and h
yperdontia of permanent upper teeth were seen. In addition, structural and
morphological dental changes were noted. These findings expand the clinical
spectrum of the syndrome. (C) 1999 Wiley-Liss, Inc.