Badelon O, Bensahel H, Csukonyi Z, Chaumien J P
Department of Pediatric Orthopaedic Surgery, Hospital Robert Debré, Bichat University Medical School, Paris, France.
Clin Orthop Relat Res. 1990 Jun(255):138-43.
Nine patients with Ehlers-Danlos syndrome (EDS) were treated for bilateral congenital dislocation of the hip (CDH). CDH was diagnosed at six months of age in two patients and at two to five years of age in the remaining seven patients. The diagnosis of EDS, based on clinical criteria, was established considerably later. One patient with multiple deformities was only observed; the remaining eight patients were initially treated by closed reduction. Due to difficulties encountered in reduction and especially in stabilization, all hips were subsequently treated surgically, with a total of 42 procedures. Avascular necrosis of the femoral head developed in five hips, four of which had been treated with closed reduction with the patients under general anesthesia. At the follow-up evaluation, six patients had reached adulthood. Clinically, satisfactory results were obtained in 12 of 16 hips but roentgenographically in only six hips. Both femoral and innominate osteotomies are necessary to achieve and maintain the reduction in EDS patients with CDH.
9例埃勒斯-当洛综合征（EDS）患者接受了双侧先天性髋关节脱位（CDH）治疗。2例患者在6个月大时被诊断为CDH，其余7例患者在2至5岁时被诊断。基于临床标准的EDS诊断在相当晚的时候才确立。1例有多种畸形的患者仅进行了观察；其余8例患者最初采用闭合复位治疗。由于复位尤其是稳定过程中遇到困难，所有髋关节随后均接受了手术治疗，共进行了42次手术。5个股骨头出现了缺血性坏死，其中4例是在全身麻醉下对患者进行闭合复位治疗后发生的。在随访评估时，6例患者已成年。临床上，16个髋关节中有12个取得了满意结果，但X线片显示仅6个髋关节结果满意。对于患有CDH的EDS患者，股骨截骨术和无名骨截骨术对于实现并维持复位都是必要的。
