遗传性多发性外骨骼增生伴尺骨偏斜

S. Shah
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引用次数: 0

摘要

遗传性多发性外生骨病是一种非常罕见的骨骼发育不良,其特征是在整个骨骼中形成大量带梗或无梗的软骨覆盖的良性肿瘤,称为骨软骨瘤,特别是在肋骨、椎骨、骨盆和长骨的生长板周围。很少会出现前臂问题,如桡骨弯曲畸形、尺骨缩短和桡肱脱位或半脱位。我们报告一例20岁的女性,她表现为左侧尺远端外生,导致尺短和桡骨弯曲,旋前和旋前活动范围受限。其他主诉为上肢和下肢多发非压痛性硬骨性肿块。切除尺远端外植骨后,旋前和旋后活动范围明显改善。遗传性多发性外生骨疣与前臂畸形虽然非常罕见,但现在和保守治疗除非骨肿胀体现任何疼痛或相关畸形等并发症。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Hereditary Multiple Exostoses with Ulnar Hemimelia
Hereditary Multiple Exostoses is a skeletal dysplasia that is very rare and defined by formation of numerous cartilage capped benign tumours either pedunculated or sessile known as osteochondromas throughout skeleton especially around the growth plates of ribs, vertebrae, pelvis and long bones. Rarely it can present forearm problems such bowing deformity of radius, ulnar shortening and radiocapitellar dislocation or subluxation. We are presenting a case of 20 year old female who presented with left distal ulnar exostosis resulting in ulnar shortening and radial bowing with restricted supination and pronation range of movement. Other complaint was of multiple non tender bony hard lumps in both upper and lower limbs. Excision of distal ulnar exostosis was done which resulted in marked improvement in pronation and supination range of movement. Hereditary multiple exostoses with forearm deformities though very rare but can present and the treatment is conservative except if any bony swelling manifests any complications such as pain or associated deformity.
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