Clinical features and treatment of the hip in multiple epiphyseal dysplasia in childhood.

Abstract

Multiple epiphyseal dysplasia is a rare congenital osteochondrodysplasia disorder characterized by a delay in the appearance of the epiphyses; irregular, symmetric epiphyseal formation; mild short stature; and early-onset osteoarthritis. Peripheral weight-bearing and nonweight-bearing joints can be affected. Treatment of the hip deformity in multiple epiphyseal dysplasia is a challenge for orthopedic surgeons.We reviewed the clinical features and treatment options of hip joints affected by multiple epiphyseal dysplasia in 6 young patients (4 boys and 2 girls). Average patient age was 8.8 years (range, 5-14 years). The spectrum of hip joint deformity ranged from mild to severe. Surgical procedures included intertrochanteric extension osteotomy of the femur in 2 patients (4 hips), Staheli acetabular augmentation in 1 patient (2 hips), and trochanter arthroplasty associated with Dega osteotomy in 2 patients (4 hips). One patient did not undergo surgical treatment. All patients were followed up for an average 7.2 years. Joint function improved in the patients treated surgically. The coxa vara deformity was corrected satisfactorily, and the femoral head was covered completely by the acetabulum.Good mid-term outcomes were obtained for the treatment of severe hip deformity by using intertrochanteric extension osteotomy and trochanter arthroplasty. However, because of the inherent nature of this disorder, long-term follow-up of the patients is required.