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.
Proudman
T W
TW
Australian Craniofacial Unit, Women's and Children's Hospital, North Adelaide, South Australia, Australia.
Moore
M H
MH
Abbott
A H
AH
David
D J
DJ
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