Vaara P, Peltonen J, Poussa M, Merikanto J, Nurminen M, Kaitila I, Ryöppy S
Department of Paediatric Orthopaedics, Hospital for Children and Adolescents, University of Helsinki, Finland.
J Bone Joint Surg Br. 1998 Mar;80(2):315-20. doi: 10.1302/0301-620x.80b2.8329.
We examined 50 patients with diastrophic dysplasia both clinically and radiologically. Two legally aborted fetuses were dissected. The mean age of the patients was 16.2 years (newborn to 38) and the mean follow-up was 11.4 years (3 months to 34 years). The fetal hips and MRI of newborn infants showed congruity and no significant joint deformity. Flexion contracture of the hip became evident later in 93% and was progressive. The radiological appearance of the proximal femoral ossific nuclei was delayed and in 17% of males and 28% of females the ossific nuclei had not appeared by the age of 12 years. Radiological measurements differed considerably from reference values and were related to the rapid and progressive restriction of rotational movement and the increase in flexion contracture. The typical findings were flattening and inferomedial bulking of the femoral head and a double-hump deformation. The changes in the hip led to secondary osteoarthritis before early middle age. We describe the clinical and radiological measurements which define the early degeneration of the joint.
我们对50例脊柱发育不良患者进行了临床和放射学检查。解剖了2例合法流产的胎儿。患者的平均年龄为16.2岁（从新生儿到38岁），平均随访时间为11.4年（从3个月到34年）。胎儿髋关节及新生儿MRI显示关节匹配，无明显关节畸形。93%的患者后期出现髋关节屈曲挛缩，且呈进行性发展。股骨近端骨化核的放射学表现延迟，17%的男性和28%的女性在12岁时骨化核仍未出现。放射学测量结果与参考值有很大差异，且与旋转运动的快速和进行性受限以及屈曲挛缩增加有关。典型表现为股骨头扁平及向内侧下膨隆，呈双峰变形。髋关节的这些变化导致中年早期之前就出现继发性骨关节炎。我们描述了定义关节早期退变的临床和放射学测量方法。
