Acetabular dysplasia associated with hereditary multiple exostoses. A case report.
Hereditary multiple exostoses is an autosomal dominant disorder characterised by multiple osteochondromata, most commonly affecting the forearm, knee and ankle. Osteochondromata of the proximal femur have been reported to occur in 30% to 90% of affected patients with coxa valga in 25%. Acetabular dysplasia is rare but has been described. This is the first report of a patient requiring surgical intervention. A girl was seen at the age of nine with hereditary multiple exostoses and when 12 developed bilateral pain in the groin. Radiographs showed severely dysplastic acetabula with less than 50% coverage of the femoral heads and widening of the medial joint space. Large sessile osteochondromata were present along the medial side of the femoral neck proximal to the lesser trochanter, with associated coxa valga. The case illustrates the importance of obtaining initial skeletal surveys in children with hereditary multiple exostoses to identify potential problems such as acetabular dysplasia and subluxation of the hip.
Felix
N A
NA
Nemours Children's Clinic, Jacksonville, Florida 32207, USA.
Mazur
J M
JM
Loveless
E A
EA
D000077:Acetabulum / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D001859:Bone Neoplasms / Q000151:congenital / Q000000981:diagnostic imaging / Q000601:surgery; D002648:Child; D005097:Exostoses, Multiple Hereditary / Q000151:congenital* / Q000000981:diagnostic imaging / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D005542:Forearm; D006618:Hip Dislocation, Congenital / Q000000981:diagnostic imaging* / Q000601:surgery; D006621:Hip Joint / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000601:surgery; D006801:Humans; D006811:Humerus; D018216:Osteochondromatosis / Q000151:congenital / Q000000981:diagnostic imaging / Q000601:surgery; D010027:Osteotomy; D011859:Radiography