Surgical management of fibrous dysplasia in McCune-Albright syndrome.
Polyostotic Fibrous Dysplasia associated with McCune-Albright Syndrome presents extreme challenges for the treating physician. The bone deformities, frequent fractures and weight bearing bone pain all combine to adversely affect the child's ability to engage in the normal activities of childhood. All too often the family is told that there is no real treatment for the bone disease. As a result of the rare nature of this syndrome, few orthopedic surgeons have had extensive experience with reconstructions for the skeletal deformities. Early surgery to address the upper femoral deformity and the innovative use of off-label internal fixation devices can have a profound effect on these young patients. The parents and patient must have a complete understanding of the effect of further growth on the reconstructions and be prepared to undergo repeated procedures as the deformities change and recur. Bone grafting and the use of common internal fixation devices (plates and screws) are almost always doomed to early failure. The innovative use of intramedullary devices can lead to prolonged ambulation and the eradication of weight bearing bone pain in selected patients. Frequent fractures and deformity can be effectively managed in most patients with these techniques.
Stanton
Robert P
RP
FACS, Department of Orthopaedics, Nemours Children's Clinic, 5153 North 9th Avenue, Pensacola, Florida 3250, USA. rstanton@nemours.org
Diamond
Leigh
L
D005357:Fibrous Dysplasia of Bone / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery*; D005359:Fibrous Dysplasia, Polyostotic / Q000150:complications*; D006801:Humans; D011859:Radiography