目的总结儿童先天性臀肌挛缩症的诊断和治疗心得。方法自1982年~2000年共治疗216例臀肌挛缩症,其中,15例为先天性臀肌挛缩症。平均年龄6.1岁(3~12岁),男9例,女6例,双侧11例,单侧4例。15例患儿共同的体征为双膝并拢后不能下蹲,翘“二郎腿”动作受限,八字脚步态,尖臀征明显。手术采用臀部大切口由髂后上棘下2cm,沿髂骨翼向下弧形至股骨大转子后侧,再向前切口暴露,广泛松解挛缩组织,术后2~3d开始在床上中立位或内收位屈髋活动。结果术中见挛缩范围广泛,臀大、中、小肌及髂胫束均有变性纤维化,甚至在梨状肌等一些小的肌肉也有挛缩。有1例术中发现,坐骨神经被挛缩的变性组织压迫而变扁。术后随访时间最长为20年4个月,最短为2年6个月,平均为8年2个月。15例中,非常满意的12例,髋关节活动基本达到正常或接近正常,可以在内收位做屈髋活动,能翘“二郎腿”。3例只能在中立位屈髋活动,内收位做屈髋活动时轻度受限,但双下肢并拢时下蹲无受限。15例中无一例出现手术并发症,家长均感满意。结论先天性臀肌挛缩症不单纯是臀肌的挛缩,大部分存在臀大、中、小肌以及髋关节外展外旋肌群的挛缩所致。大切口可充分暴露,彻底松解挛缩变性的肌肉组织,使手术获得成功,并避免损伤坐骨神经。 Objective To summarize the diagnosis and treatment of congenital gluteal muscle contracture in children. Methods A total of 216 cases of gluteal muscle contracture were treated from 1982 to 2000, of which 15 cases were congenital gluteal muscle contracture. The mean age was 6.1 years (range, 3 to 12 years). There were 9 males and 6 females, 11 bilateral and 4 unilateral. Common signs of 15 cases of children can not squat together after the knees close together, Alice “erlang leg” limited movement, character foot gait, sharp hip sign obvious. Surgical use of the buttocks large incision from the posterior superior iliac spine 2cm, arc down the iliac wing to the back of the greater trochanter, and then forward incision exposure, extensive release of contracture organizations, 2 ~ 3d after bed neutral Or internal hip flexion activities. Results The intraoperative findings of a wide range of contracture, hip, middle and small muscles and iliotibial tract have denatured fibrosis, and even some small muscles such as piriformis also contracture. In one case, intraoperative findings showed that the sciatic nerve became flattened by the degeneration of contracted tissue. The longest postoperative follow-up was 20 years and 4 months, the shortest was 2 years and 6 months, with an average of 8 years and 2 months. In 15 cases, 12 patients were very satisfied. The activities of hip joint reached the normal or nearly normal level, and the hip flexion activities could be performed in the internal position. Only 3 cases of hip flexion in the neutral position, the adduction of hip flexion activities slightly limited, but when the lower limbs closed squat without restriction. None of 15 patients had surgical complications, and both parents were satisfied. Conclusions Congenital gluteal muscle contracture is not simply the contracture of the gluteal muscle, but it is caused by the contracture of the large, middle and small muscles of the hip as well as the outward swollen hip joint. Large incision can be fully exposed, completely loosen the contracture of degenerative muscle tissue, the operation was successful, and to avoid damage to the sciatic nerve.