半肢骨骺发育异常的影像特征

Imaging feature of dysplasia epiphysealis hemimelica

目的探讨半肢骨骺发育异常（DEH）的影像特征。方法回顾性分析经病理证实的13例DEH患者的临床资料，男10例、女3例。年龄1．5-19．0岁，中位年龄9．5岁。13例均行X线检查，其中1例行CT检查、1例行MR检查、1例行CT和MR检查。结果13例DEH病变均发生于下肢。发病部位共32处；8例为多发性，累及全骨骺者6例；发生于肢体内侧23处、外侧3处。Azouz分型局限型5例、典型型8例。合并踝外翻4例、膝外翻3例、踝内翻2例、足外翻2例。8例干骺端增宽变形，1例先期钙化带部分消失，骨骺早期闭合。结论影像上DEH主要表现为单侧或双侧肢体的一个或多个骨化中心偏心性增大，早期可见散在的斑点状或不规则钙化、骨化，晚期钙化或骨化可融合呈团状。肿块压迫邻近骨组织引起骨骼变形、移位，甚至压迫性骨缺损。

Objective To investigate the imaging features of dysplasia epiphysealis hemimelica （DEH）. Methods Thirteen cases of DEH confirmed by pathology （between 1.5 to 19.0 years old） underwent X-ray scan. Additionally, CT scan was performed in one patient, MR scan was performed in one, and CT and MR scan were both perfomled in one. Results The lesions all occurred in lower limb. There were 32 lesions, and multiple lesions were detected in 8 cases. Lesions involved holo-epiphyses in 6 cases. Twenty-three lesions were in the medial limbs, 3 were in the lateral limb. With Azouz classification, the limited form was identified in 5 cases, while the classic form was identified in 8 cases. Among 13 cases, there were 4 cases with ankle eversion, 3 cases with the knock knee, 2 cases with the ankle inversion, and 2 cases with the strephexopodia. The metaphysis of 8 cases were widened and deformed, and the provisional zone of calcification of 1 case disappeared partly and the epiphysis closed prematurely. Conclusions One or multiple eccentric enlarged ossification center of unilateral or bilateral limb is the imaging feature of DEH. There is sporadic calcification or ossification on the prophase. The calcification or ossification can fuse each other to form agglomerate at the advanced stage. The bone can deform and shift when compressed by the mass, and bone defect can even occur.