Clinical findings precede objective diagnostic testing in the identification of increased ICP in syndromic craniosynostosis.
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.
David
Lisa R
LR
North Carolina Center for Cleft and Craniofacial Deformities, Wake Forest University School of Medicine, Winston-Salem, North Carolina 27157, USA.
Velotta
Emily
E
Weaver
R Grey
RG
Wilson
John A
JA
Argenta
Louis C
LC
D003394:Craniofacial Dysostosis / Q000150:complications* / Q000601:surgery; D003398:Craniosynostoses / Q000150:complications* / Q000601:surgery; D019299:Decompression, Surgical; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D005546:Forehead / Q000601:surgery; D005624:Frontal Bone / Q000002:abnormalities; D006801:Humans; D007223:Infant; D019586:Intracranial Hypertension / Q000209:etiology* / Q000517:prevention & control; D009915:Orbit / Q000601:surgery; D010211:Papilledema / Q000209:etiology; D010294:Parietal Bone / Q000002:abnormalities; D012008:Recurrence; D014057:Tomography, X-Ray Computed