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马萨达Ⅰ型遗传性多发性骨软骨瘤前臂畸形尺骨延长治疗的临床和功能结果。

Clinical and Functional Outcomes of Ulnar Lengthening in the Treatment of Masada Type I Forearm Deformities in Hereditary Multiple Osteochondromas.

机构信息

Joint Reconstruction Research Center, Tehran University of Medical Sciences, Tehran, Iran; Children's Hospital of Philadelphia, Philadelphia, PA.

Joint Reconstruction Research Center, Tehran University of Medical Sciences, Tehran, Iran.

出版信息

J Hand Surg Am. 2020 Sep;45(9):876.e1-876.e7. doi: 10.1016/j.jhsa.2020.02.010. Epub 2020 Apr 3.

Abstract

PURPOSE

Forearm deformity is common in hereditary multiple osteochondromas (HMO). The results of ulnar lengthening and osteochondroma excision are generally favorable, both clinically and radiographically. This study aimed to assess the clinical, radiographic, and functional results of forearm reconstruction in patients with Masada type I deformity (osteochondroma in the distal ulna with radial bowing, without radial head dislocation).

METHODS

A retrospective review was performed on patients with HMO who underwent surgical reconstruction for a Masada type I forearm deformity over a 10-year period. Eleven forearms in 10 patients underwent gradual ulnar lengthening with an external fixator. Age at the time of surgery was 5 to 12 years. Radiographic evaluation included ulnar shortening, radial articular angle, and carpal slip. Clinical evaluation included range of motion of the elbow and wrist. Functional outcomes were evaluated with the Disabilities of the Arm, Shoulder, and Hand questionnaire and the Short Form-36 survey.

RESULTS

At an average follow-up of 37.5 months, significant improvement was observed in ulnar shortening, the radial articular angle, and carpal slip. Preoperative average Disabilities of the Arm, Shoulder, and Hand score of 24.2 improved to 17.3. Significant improvement was observed in 3 measures of the Short Form-36 survey: general health, social functioning, and health change.

CONCLUSIONS

In agreement with the literature, surgical reconstruction yielded favorable clinical and radiographic results in Masada type I forearm deformities. The main functional improvement was observed in scales of mental health. The baseline physical impairment was minimal in the absence of radial head dislocation, but although it improved with treatment, it was not necessarily clinically relevant. This study can assist surgeons and families in setting realistic goals and expectations for surgery. There may also be additional long-term benefits to forearm lengthening in HMO, including prevention of radial head dislocation, improved range of motion, and better cosmesis, which future research may help to clarify.

TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic V.

摘要

目的

遗传性多发性骨软骨瘤(HMO)常导致前臂畸形。尺骨延长和骨软骨瘤切除的结果通常在临床和影像学上都是有利的。本研究旨在评估 Masada Ⅰ型畸形(尺骨远端骨软骨瘤伴桡骨弯曲,无桡骨头脱位)患者前臂重建的临床、影像学和功能结果。

方法

对 10 年间接受手术治疗 Masada Ⅰ型前臂畸形的 HMO 患者进行回顾性研究。10 例患者的 11 个前臂采用外固定器进行逐渐尺骨延长。手术时的年龄为 5 至 12 岁。影像学评估包括尺骨缩短、桡腕关节角和腕骨滑移。临床评估包括肘和腕关节的活动范围。使用上肢残疾问卷(DASH)和简明健康调查问卷(SF-36)评估功能结果。

结果

平均随访 37.5 个月后,尺骨缩短、桡腕关节角和腕骨滑移均显著改善。术前平均 DASH 评分为 24.2 分,改善至 17.3 分。SF-36 调查的 3 项指标均显著改善:一般健康状况、社会功能和健康变化。

结论

与文献一致,手术重建在 Masada Ⅰ型前臂畸形中产生了有利的临床和影像学结果。主要的功能改善体现在心理健康量表上。由于没有桡骨头脱位,基线时的身体损伤较小,但治疗后有所改善,但不一定具有临床意义。本研究可以帮助外科医生和家属为手术设定现实的目标和期望。HMO 患者前臂延长可能还有其他长期益处,包括预防桡骨头脱位、改善活动范围和更好的美容效果,未来的研究可能有助于阐明这些益处。

研究类型/证据水平:治疗性 V 级。

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