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Acetabular dysplasia associated with hereditary multiple exostoses. A case report
N A Felix1, J M Mazur, E A Loveless
1Nemours Children's Clinic, Jacksonville, Florida 32207, USA.
The Journal of Bone and Joint Surgery. British Volume
|June 16, 2000
Summary
Hereditary multiple exostoses can lead to severe hip problems like acetabular dysplasia. Early skeletal surveys in children are crucial for identifying and managing these potential complications.
Area of Science:
- Orthopedics
- Genetics
- Pediatric Medicine
Background:
- Hereditary multiple exostoses (HME) is an autosomal dominant disorder causing multiple osteochondromata.
- Commonly affects the forearm, knee, and ankle, with proximal femur involvement in 30-90% of patients.
- Associated coxa valga occurs in 25% of HME patients; acetabular dysplasia is rare but documented.
Observation:
- This report details the first HME patient requiring surgical intervention for hip complications.
- A 12-year-old girl with HME developed bilateral groin pain.
- Radiographs revealed severely dysplastic acetabula with <50% femoral head coverage and medial joint space widening.
Findings:
- Large sessile osteochondromata were noted on the medial femoral neck, proximal to the lesser trochanter.
- Associated coxa valga was present.
- The patient exhibited severe acetabular dysplasia and hip subluxation.
Implications:
- Highlights the critical importance of initial skeletal surveys in children diagnosed with HME.
- Emphasizes the need to screen for potential hip complications, including acetabular dysplasia and subluxation.
- Suggests early identification can guide timely management and surgical intervention if required.
