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One-bone forearm procedure for Hajdu–Cheney syndrome: a case report

机译:Hajdu–Cheney综合征的单骨前臂手术:一例报告

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摘要

One-bone forearm surgery is generally regarded as one of the last available salvage procedures that could be used to treat patients with longitudinal forearm instability secondary to a congenital, oncologic, or a post-traumatic etiology. We performed this procedure on a 23-year-old patient with longitudinal forearm instability secondary to Hajdu–Cheney syndrome, which is a rare genetic disorder characterized by generalized ligamentous laxity, skeletal dysplasia, acro-osteolysis, and generalized osteoporosis. The patient developed shoulder pain secondary to overuse 28 months following treatment, and was managed conservatively. Eight years after surgery, the patient had not undergone any additional procedures, had no pain, reported a Quick Disabilities of the Arm Shoulder and Hand score of 21, and was completely satisfied with treatment. Although OBF procedure is a radical first-line salvage option, in unique circumstances and appropriate patient selection, it may provide acceptable, durable, and predictable results.
机译:通常,单骨前臂手术被认为是可用于治疗先天性,肿瘤性或创伤后病因继发的纵向前臂不稳的患者的最后一种挽救方法。我们对一名23岁继发于Hajdu–Cheney综合征的前臂纵向不稳定性患者进行了此手术,这是一种罕见的遗传性疾病,其特征是全身性韧带松弛,骨骼发育不良,肢端骨溶解和全身性骨质疏松。患者在治疗后28个月因过度使用而继发肩痛,并进行了保守治疗。手术八年后,患者没有进行任何其他手术,没有疼痛,手臂肩膀和手部快速残疾评分为21,并且对治疗完全满意。尽管OBF手术是一线急救方法,但在特殊情况下和适当的患者选择中,它可能会提供可接受的,持久的和可预测的结果。

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