Noncraniofacial manifestations of Crouzon's disease.
Although most interest centers on the craniofacial region in Crouzon's disease, noncraniofacial manifestations are important because they may complicate both diagnosis and management. A population of 59 patients with Crouzon's disease was reviewed to determine the frequency of these deformities. Stylohyoid ligament calcification (50%) and cervical spine (40%) and elbow (18%) abnormalities were the most common. Minor hand deformities (10%), other musculoskeletal deformities (7%), and visceral anomalies (7%) were also seen. Acanthosis nigricans was not present in this population. Recommendations are made for the assessment and management of Crouzon's disease with reference to these areas.
Australian Craniofacial Unit, Women's and Children's Hospital, North Adelaide, South Australia, Australia.
T W
TW
Proudman
M H
MH
Moore
A H
AH
Abbott
D J
DJ
David
D000052:Acanthosis Nigricans / Q000473:pathology; D003394:Craniofacial Dysostosis / Q000473:pathology*; D004550:Elbow / Q000002:abnormalities; D005260:Female; D006228:Hand Deformities, Congenital / Q000473:pathology; D006801:Humans; D006928:Hyoid Bone; D008022:Ligaments / Q000002:abnormalities; D017880:Limb Deformities, Congenital; D008297:Male; D012189:Retrospective Studies; D013131:Spine / Q000002:abnormalities; D013701:Temporal Bone