[Frontonasal dysplasia or the median cleft face syndrome: a case report].

Abstract

Frontonasal dysplasia (FND) is a condition with a more or less severe ocular hypertelorism and, sometimes, a narrowing of the palpebral fissures. The nose is broad and flattened with clefting; it may be bifid or completely divided in two halves. In extreme cases, the central nervous system is concerned and there is a mental deficiency. There is no coronal craniosynostosis in pure FND. Other malformations can be observed. Among the cases reported in the literature, most of them are sporadic but they may be familial. We report here a sporadic case of F.N.D. in which scan examination of the brain shows a possible cephalocele. The literature is briefly reviewed.

• Authors: H Apere, F Serville, B Collet, C Billeaud, B Sandler
• Journal: Journal de genetique humaine (J Genet Hum) Vol. 37 Issue 4-5 Pg. 431-6 (Dec 1989) ISSN: 0021-7743 [Print] Switzerland
• Vernacular Title: La dysplasie frontonasale ou syndrome de la fente médiane de la face. A propos d'une observation.
• PMID: 2635719 (Publication Type: Case Reports, English Abstract, Journal Article)

Topics

• Female
• Humans
• Nose (abnormalities)
• Syndrome