多发性骨骺发育不良髋关节病变的诊治

目的探讨多发性骨骺发育不良（multiple epiphyseal dysplasia，MED）髋关节病变的临床特点和治疗方法。方法本组6例MED患儿，均有髋关节受累。根据股骨近端的放射学表现，分为三型，Ⅰ型为股骨近端轻度发育不良，髋关节形态基本正常；Ⅱ型为股骨近端明显发育不良，股骨颈短粗，股骨头骨骺小、碎裂呈斑点状，伴不同程度的髋内翻；Ⅲ型为股骨近端严重发育不良，在骨盆前后位片上见不到股骨头影像，仅见短粗三角形的股骨颈残端，大转子高位，有严重的髋内翻畸形。本组Ⅰ、Ⅱ、Ⅲ型患儿各2例。Ⅰ型股骨近端不需手术矫形，Ⅱ型行转子间外展截骨术，Ⅲ型行转子关节成形术。如伴有髋臼发育不良，可同时行髋臼成形术。结果所有病例随访2～5．5年，平均3．5年，髋关节功能、步态及影像学表现较术前均有明显的改善，Trendelenburg征阴性。结论MED髋关节病变，预后不良。畸形轻微可保守治疗，畸形严重可采用外展截骨术、转子关节成形术矫正，短期取得良好的效果，但需长期随访。

Diagnosis and treatment of multiple epiphyseal dysplasia in the hip

Objective To evaluate the clinical features and treatment of the hip affected by mul- tiple epiphyseal dysplasia(MEl)).Methods Six children (4 males and 2 females) with MED were treated in this institute.The average age of these patients was 8.8 years (ranged from 8 - 14 years). Radiographic findings showed epiphyseal abnormality of the hip in all cases.Five of them,the prob- lems in the hips required surgical treatment.The orthopedic procedures included intertrochanteric ex- tension osteotomy for 2 patients,Dega's acetabular osteotomy and trochanter-arthroplasty for 2,and Staheli shelf acetabular augmentation procedure for the other one.Results All the patients had been followed up for 2-5.5 years (mean.3.5 years).The Trendelenburg's sign was reversed.The range of abduction of the hip was obviously improved.Although there was mild pain on the hips in 1 case who underwent Staheli procedure,the femoral head had been contained completely by the acetabulum. Conclusions Conservative treatment may be undertaken for mild deformity,intertrochanteric exten- sion osteotomy and Dega's acetabular osteotomy may he performed on moderate and serious cases with MED.Short-term results are optimistic but long-term follow-up is necessary.