目的：探讨半肢骨骺发育异常（Dysplasia epiphysealis hemimelica，DEH）的MRI影像表现。资料与方法：回顾性分析7例病理证实的DEH患者的临床及MRI影像资料，其中男6例，女1例，年龄1~9岁。扫描序列为常规序列及3D-VIBE序列。结果：①发病部位：7例DEH病变发生于膝关节5例，踝关节2例；2例累及单个骨骺，5例发生于多个骨骺；3例只累及肢体内侧，2例只累及肢体外侧，1例肢体内外侧均受累，1例累及肢体中央部。②合并畸形：3例合并骺早闭，其中1例同时合并骺早闭、髌骨脱位及胫骨弯曲变形；1例合并膝外翻，1例合并踝内翻。③影像表现：7例患者均可见关节周围1个或多个骨软骨性肿物，与邻近固有骨骺信号相似，软骨内骨化中心与固有骨骺融合或分离，表面软骨帽呈T1WI中等信号、VIBE序列高信号。7例DEH病变MRI检查均可清晰显示关节周围软组织的异常。结论：MRI可以清晰显示DEH病变的范围及其解剖关系，明确肿块与固有骨骺的关系，对DEH的诊断、评估以及治疗发挥重要作用。
Abstract
Objective: To investigate the MR imaging features of dysplasia epiphysealis hemimelica(DEH)． Materials and Methods: Clinical records and MR images of 7 cases with DEH confirmed by pathology were retrospectively reviewed, including 6 boys and 1 girl, with ages from 1~9 years old. The conventional MR sequences and 3D-VIBE sequence were performed. Results: ①Location: 5 of 7 cases involved the knees, and 2 cases involved the ankles. Two cases had involvement of single epiphysis, while 5 cases involved multiple epiphyses. Three involved epiphyses were in the medial parts of the limbs, and 2 cases in the lateral parts of the limbs, while 1 case involved both sides, and 1 case involved the central part. ②Complications: premature physeal closure were found in 3 cases, including 1 case with epiphyseal plate closure, dislocation of patella and tibia bending deformation simultaneously, 1 case with the genu valgum, and 1 case with the ankle inversion. ③MR imaging features: 1 or more osteochondral masses were seen around the joint in all cases, which had signal intensity similar to that of normal epiphysis. The osseous components of the osteochondral masses were continuous or separated from the epiphysis. The surface cartilage cap was isointensity in T1WI and hyperintensity in VIBE sequence. Conclusion: MRI can precisely reveal the DEH lesions, and clarify the relationship between the mass and the inherent epiphysis. MRI was essential in the diagnosis, evaluation and treatment of DEH.
关键词
肢畸形 /
先天性 /
磁共振成像
Key words
Limb deformities, congenital /
Magnetic resonance imaging
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