Clinical findings precede objective diagnostic testing in the identification of increased ICP in syndromic craniosynostosis.

Autor: David LR; North Carolina Center for Cleft and Craniofacial Deformities, Wake Forest University School of Medicine, Winston-Salem, North Carolina 27157, USA., Velotta E, Weaver RG Jr, Wilson JA, Argenta LC
Jazyk: angličtina
Zdroj: The Journal of craniofacial surgery [J Craniofac Surg] 2002 Sep; Vol. 13 (5), pp. 676-80.
DOI: 10.1097/00001665-200209000-00015
Abstrakt: Crouzon syndrome is an autosomal dominant disorder resulting in facial dysmorphism and craniosynostosis involving multiple cranial sutures. A common but often unrecognized early complication associated with craniosynostosis is a finding of increased intracranial pressure (ICP). This increase in ICP can lead to optic atrophy, neuronal damage, and mental deficits. The case of a 21-month-old girl with Crouzon syndrome is described. Although the child was clinically asymptomatic, a routine ophthalmic exam revealed papilledema and subsequently increased intracranial pressure and craniosynostosis were found. Cranial expansion and bicanthal advancement were performed to relieve the increased pressure. In cases such as these, long-term follow-up is essential because of the progressive nature of the disorder as well as the possibility of a recurrence of elevated intracranial pressure and a need for secondary decompressive surgery.
Databáze: MEDLINE