Rare dental abnormalities seen in oculo-facio-cardio-dental (OFCD) syndrome: Three new cases and review of nine patients

Citation
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
Citations number
9
Categorie Soggetti
Molecular Biology & Genetics
Journal title
AMERICAN JOURNAL OF MEDICAL GENETICS
ISSN journal
01487299 → ACNP
Volume
82
Issue
5
Year of publication
1999
Pages
429 - 435
Database
ISI
SICI code
0148-7299(19990219)82:5<429:RDASIO>2.0.ZU;2-L
Abstract
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.