Fibrous dysplasia of bone and the Weil-Albright syndrome. A study of thirteen cases with special reference to the orthopaedic treatment.
Four patients with monostotic and nine with polyostotic fibrous dysplasia of bone, including one with the classical Weil-Albright syndrome and a male child who may have had this syndrome, are presented. While monostotic forms affect adults and are amenable to curative surgery, polyostotic involvement may become evident in childhood and early infancy, and represent a difficult therapeutic problem. Screening of the skeleton is essential for proper diagnosis and treatment, and should be achieved by total body scan in preference to a complete radiological survey. Biopsy is of particular importance in monostotic cases. The course of polyostotic fibrous dysplasia of bone is not predictable and depends partly upon the unassessable osteogenic potential of the bone, and also on surgery. The Weil-Albright syndrome and its variants are no worse than polyostotic fibrous dysplasia. Orthopaedic treatment deals with pathological fractures and with the prevention and correction of deformities, particularly of coxa vara. Sufficient stability of diaphyseal and metaphyseal lesions of the femur and tibia can usually be provided by intramedullary fixation, which needs to be left, or exchanged, at least until the end of adolescence. Extensive lesions of the proximal femur make aggressive surgery necessary. Pelvic involvement and destruction of the hip joint preclude surgery and require the use of calipers.
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D005260:Female; D005357:Fibrous Dysplasia of Bone / Q000601:surgery*; D005358:Fibrous Dysplasia, Monostotic / Q000473:pathology / Q000601:surgery*; D005359:Fibrous Dysplasia, Polyostotic / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011629:Puberty, Precocious / Q000473:pathology; D011859:Radiography; D013577:Syndrome