Wada Akifusa, Nakamura Tomoyuki, Yamaguchi Toru, Yanagida Haruhisa, Takamura Kazuyuki, Oketani Yutaka, Kubota Hideaki, Fujii Toshio
Department of Orthopaedic Surgery, Saga Handicapped Children's Hospital, 2215-27 Kinryu, Kinryu-machi, Saga, 849-0906 Japan.
J Child Orthop. 2012 Aug;6(4):261-7. doi: 10.1007/s11832-012-0426-y. Epub 2012 Jul 24.
Kabuki syndrome is characterized by distinctive facial features, skeletal anomalies, persisting fingertip pads with dermatoglyphic abnormalities, postnatal growth deficiency and mental retardation. This report reviews our results in the operative treatment of hip dislocations in patients with Kabuki syndrome.
Between 2001 and 2009, seven dislocated hips (three unilateral and two bilateral hips) in five patients (all girls) were operatively treated at our institution. The operative treatment consists of open reduction, femoral derotation varus osteotomy, pelvic osteotomy (Salter in one and incomplete periacetabular osteotomy in six hips) and capsular plication. The age of the patients at the time of surgery ranged from 2.4 to 5.7 years, with an average of 3.6 years.
The follow-up postoperative period ranged from 3.2 to 6.3 years, with an average of 5.0 years. At the final follow-up, all patients reported no click and no pain, and showed well-contained hips by radiographs. All seven hips were graded as Severin class I-II. One patient presented as having habitual dislocation of the hip 4.4 years after surgery. Computed tomographic (CT) scans revealed posterior acetabular wall deficiency, which was not corrected by the anterolaterally directed Salter osteotomy. The incomplete periacetabular osteotomy provided sufficient posterolateral coverage of the acetabulum.
Operative treatment combining open reduction, femoral derotation varus and incomplete periacetabular osteotomies, and capsular plication provided successful results in patients with Kabuki syndrome who had the characteristics of hip instability such as ligamentous laxity, muscular hypotonia and posterior acetabular wall deficiency.
歌舞伎综合征的特征为独特的面部特征、骨骼异常、伴有皮纹异常的持续指尖垫、出生后生长发育迟缓及智力障碍。本报告回顾了我们对歌舞伎综合征患者髋关节脱位的手术治疗结果。
2001年至2009年期间，我们机构对5例患者（均为女孩）的7个脱位髋关节（3个单侧髋关节和2个双侧髋关节）进行了手术治疗。手术治疗包括切开复位、股骨旋转内翻截骨、骨盆截骨（1例采用Salter截骨，6个髋关节采用不完全髋臼周围截骨）及关节囊折叠术。手术时患者年龄为2.4至5.7岁，平均3.6岁。
术后随访时间为3.2至6.3年，平均5.0年。末次随访时，所有患者均无弹响及疼痛，X线片显示髋关节包容良好。所有7个髋关节均评为Severin I-II级。1例患者术后4.4年出现习惯性髋关节脱位。计算机断层扫描（CT）显示髋臼后壁缺损，前外侧Salter截骨未能纠正。不完全髋臼周围截骨提供了足够的髋臼后外侧覆盖。
对于具有韧带松弛、肌张力低下及髋臼后壁缺损等髋关节不稳定特征的歌舞伎综合征患者，切开复位、股骨旋转内翻及不完全髋臼周围截骨联合关节囊折叠术的手术治疗取得了成功的结果。
