先天性臀肌挛缩症

Congenital hip contracture

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2 cm,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2～3 d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 review the management of congenital abduction contraction of the hip in children. Methods Two hundred and sixteen children with abduction contraction of the hip were managed in our hospital between 1982 and 2000. Of these, 15 were congenital contractures. The average age of the 15 children at presentation was {6.1} years (3-12 years). There were 9 boys and 6 girls. There were 11 bilateral and 4 unilateral contractures. The common signs included inability to squat with the knees touching each other, inability to cross the legs sitting and pigeon-toe gait. The surgical incision began from a point 2 cm inferior to the postero-superior iliac spine and curved towards the greater trochanter. The contracture tissues were exposed and released. The patient began active and passive hip flexion 2-3 days post-operatively. Results The contracture affected the gluteus maximus, gluteus medius, and even the piriform muscles. In one case, the ischial nerve was compressed by the fibrosed tissue. The post-op follow-up averaged ~8.1 years (ranging from ~2.5 to ~20.3 years). Twelve patients regained normal range of hip motion with full hip flexion. Three patients have limited flexion with the leg adducted, but they can squat with the knees in contact. No complication was observed in the 15 cases. The parents were satisfied with the result. Conclusions The contracted tissues in congenital abduction contraction of the hip may affect the gluteus maximus, medius, minimus muscles and small abduction rotation muscles. Adequate exposure is required for contracture release and to minimize nerve injury.