多发性骨骺发育不良髋关节病变的诊治

Diagnosis and treatment of multiple epiphyseal dysplasia in the hip

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,仅见短精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的均有明显的改善,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。

Objective To evaluate the clinical features and treatment of the hip affected by multiple epiphyseal dysplasia（MED）. 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 problems in the hips required surgical treatment. The orthopedic procedures included intertrochanteric extension 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 be performed on moderate and serious cases with MED. Short term results are optimistic but long-term follow up is necessary.