Cervical instability as an unusual manifestation of Hajdu-Cheney syndrome of acroosteolysis.
Acroosteolysis is a disease that primarily affects distal areas of extremities, with osteolysis involving the phalanges of the hand most commonly reported. Some authors have noted acroosteolysis to be acquired secondarily to the initiation of an immune complex disease that results in the vascular occlusion of the small arteries of the hand. Although the pathoetiology is unknown, the Hajdu-Cheney syndrome of idiopathic acroosteolysis is distinct from the other forms because a generalized skeletal dysplasia is the most distinct feature, and it is usually not present in early childhood. The purpose of this study is to present an unusual case of cervical instability in a girl with Hajdu-Cheney syndrome. This case not only demonstrates that cervical osteolysis and acroosteolysis can coexist and require primary surgical intervention but also that patients with these concurrent problems need to be monitored closely to identify any subsequent changes that may require secondary surgical stabilization.
Herscovici
D
D
Department of Medical Education, Alfred I. duPont Institute, Wilmington, DE 19899.
Bowen
J R
JR
Scott
C I
CI
D002574:Cervical Vertebrae / Q000000981:diagnostic imaging* / Q000601:surgery; D002648:Child; D005500:Follow-Up Studies; D006801:Humans; D007593:Joint Instability / Q000209:etiology; D008297:Male; D010014:Osteolysis / Q000000981:diagnostic imaging*; D010015:Osteolysis, Essential / Q000150:complications / Q000000981:diagnostic imaging* / Q000601:surgery; D011859:Radiography; D012008:Recurrence; D013123:Spinal Fusion / Q000379:methods