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马萨达Ⅰ/Ⅱb 型遗传性多发性骨软骨瘤患者的尺骨逐渐延长:一项平均随访 4.2 年的回顾性研究。

Gradual ulnar lengthening in Masada type I/IIb deformity in patients with hereditary multiple osteochondromas: a retrospective study with a mean follow-up of 4.2 years.

机构信息

Department of Pediatric Orthopedics, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, 1678 Dongfang Road, Shanghai, 200127, People's Republic of China.

出版信息

J Orthop Surg Res. 2020 Dec 9;15(1):594. doi: 10.1186/s13018-020-02137-z.

Abstract

BACKGROUND

Gradual ulnar lengthening is the most commonly used procedure in the treatment of Masada type I/II deformity in patients with hereditary multiple osteochondromas. However, the treatment remains controversial for the recurrence of deformity in growing children. This study aims to evaluate the clinical and radiological outcomes of ulnar gradual lengthening in our clinic.

METHODS

We retrospectively reviewed patients who underwent ulnar lengthening by distraction osteogenesis from June 2008 to October 2017. The carrying angle (CA) and range of motion (ROM) of the forearm and elbow were clinically assessed, and the radial articular angle (RAA) and ulnar shortening (US) were radiologically assessed before lengthening, 2 months after external frame removal, and at the last follow-up.

RESULTS

The current study included 15 patients (17 forearms) with a mean age of 9.4 ± 2.3 years at the index surgery. The mean follow-up period was 4.2 ± 2.4 years. There were 9 patients (10 forearms) with Masada type I deformity and 6 patients (7 forearms) with Masada type IIb deformity. The mean amount of ulnar lengthening was 4.2 ± 1.2 cm. The mean RAA improved from 37 ± 8 to 30 ± 7° initially (p = 0.005) and relapsed to 34 ± 8° at the last follow-up (p = 0.255). There was a minimal deterioration of US yet significant improvement at the last follow-up compared to pre-op (p < 0.001). At the last follow-up, the mean forearm pronation and elbow flexion increased significantly (p < 0.001 and p = 0.013, respectively), and the mean carrying angle also improved significantly (p < 0.001). No patient with type IIb deformity achieved a concentric radial head reduction.

CONCLUSIONS

Gradual ulnar lengthening significantly reduces cosmetic deformity and improves function in patients with Masada type I/IIb deformity. Our results supported early ulnar lengthening for patients with a tendency of dislocation of the radial head.

摘要

背景

在患有遗传性多发性骨软骨瘤的患者中,尺骨逐渐延长是治疗马萨达 I/II 型畸形最常用的方法。然而,对于生长中的儿童畸形的复发,这种治疗方法仍存在争议。本研究旨在评估我们科室尺骨逐渐延长的临床和放射学结果。

方法

我们回顾性分析了 2008 年 6 月至 2017 年 10 月期间接受骨延长术的患者。对前臂和肘部的提携角(CA)和活动范围(ROM)进行临床评估,并在延长前、外固定架去除后 2 个月和最后一次随访时进行桡腕关节角(RAA)和尺骨缩短(US)的放射学评估。

结果

本研究共纳入 15 例患者(17 例前臂),索引手术时的平均年龄为 9.4 ± 2.3 岁。平均随访时间为 4.2 ± 2.4 年。9 例患者(10 例前臂)为马萨达 I 型畸形,6 例患者(7 例前臂)为马萨达 IIb 型畸形。尺骨延长的平均长度为 4.2 ± 1.2cm。RAA 从初始的 37 ± 8°改善到 30 ± 7°(p = 0.005),最后随访时回退到 34 ± 8°(p = 0.255)。与术前相比,US 虽然有轻微恶化,但在最后一次随访时显著改善(p < 0.001)。最后随访时,前臂旋前和肘部屈曲的平均角度显著增加(p < 0.001 和 p = 0.013),平均提携角也显著改善(p < 0.001)。没有 IIb 型畸形患者的桡骨头达到同心复位。

结论

尺骨逐渐延长可显著减轻马萨达 I/IIb 型畸形患者的美容畸形,改善功能。我们的结果支持对有桡骨头脱位倾向的患者进行早期尺骨延长。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fb47/7727121/bc4f86ad60c2/13018_2020_2137_Fig1_HTML.jpg

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