Greig cephalopolysyndactyly syndrome. Report of a sporadic case.

Abstract

A female newborn of healthy parents demonstrates the combination of postaxial hexadactyly type B of the hands (X-ray investigations show in addition duplicated terminal phalanges of the thumbs), preaxial hexadactyly of the toes, partial and total syndactyly of the fingers and toes. These findings are compatible with the diagnosis of Greig cephalopolysyndactyly syndrome. Dyscrania can not be demonstrated. In addition, our patient exhibits genu recurvatum on the left side resulting from intrauterine deformation. Sonography of the head reveals normal formation of the corpus callosum. The neurological status of our baby is inconspicious, and the psychomotor development up to the age of 2 5/12 years normal. The differential diagnosis of this characteristic combination of pre- and postaxial polydactyly has to consider the acrocallosal syndrome. In sporadic cases of Greig cephalopolysyndactyly syndrome it is important to investigate and follow the neurological status of the patients and in particular to use sonography to document the intact corpus callosum. Patients with Greig cephalopolysyndactyly syndrome have a normal development, children with the acrocallosal syndrome are retarded.

• Authors: J Kunze, H J Kaufmann
• Journal: Helvetica paediatrica acta (Helv Paediatr Acta) Vol. 40 Issue 6 Pg. 489-95 ( 1985) ISSN: 0018-022X [Print] Switzerland
• PMID: 3007398 (Publication Type: Case Reports, Journal Article)

Topics

• Child Behavior
• Child Development
• Child, Preschool
• Craniofacial Dysostosis (complications, psychology)
• Female
• Humans
• Motor Skills
• Syndactyly (complications)
• Syndrome