Midline defects of the orofaciodigital syndrome type VI (Varadi syndrome)

P. D. Wey, J. A. Neidich, L. A. Hoffman, G. S. LaTrenta

Research output: Contribution to journalArticle

13 Scopus citations

Abstract

The orofaciodigital syndromes (OFDS) represent a spectrum of anomalies of the palate, cranium, hands, and feet. Varadi syndrome, designated OFDS type VI, is a rare disorder that is additionally characterized by cerebellar anomalies. The following report is of a patient with OFDS VI and characteristic multiple midline defects: median cleft lip and palate, lingual cleft with nodules, and midline brain malformation. In addition, this case is uniquely associated with the presence of midline (metopic and sagittal) craniosynostoses as well. It is unusual that deformities which result from premature fusion of cranial vault sutures would appear synchronously in a syndrome based on the concept of failure of fusion or coalescence of facial growth centers. The midline represents an independent developmental field, whereby CNS defects and midline anomalies can present concurrently.

Original languageEnglish
Pages (from-to)397-400
Number of pages4
JournalCleft Palate-Craniofacial Journal
Volume31
Issue number5
DOIs
StatePublished - Dec 7 1994
Externally publishedYes

Keywords

  • Varadi syndrome
  • craniosynostosis
  • midline defects
  • orofaciodigital syndromes

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