Functional Assessment of Congenital Radioulnar Synostosis in Children.

Avicenna Journal of Medicine Pub Date : 2025-02-21 eCollection Date: 2025-01-01 DOI:10.1055/s-0045-1802671
Isam Sami Moghamis, Hasan Abuhejleh, Yousef Abuodeh, Hazem Mohamad Nasef, Harris Manova Thanaraj, Ilse Du Preez, Mohamad Alaa Kawas
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Abstract

Background  Congenital radioulnar synostosis (CRUS) is a rare condition caused by segmentation failure in embryonic life. The forearm is usually fixed in pronation, affecting the upper limb and hand functions. Treatment ranges from observation to surgical options to restore limb function and prevent disease recurrence. The study aimed to assess the functional outcome of patients with CRUS. Methods  We conducted a prospective evaluation of eight patients with CRUS between 2010 and 2020. The data involved history, physical examination, and functional assessment using the ABILHAND-Kids functional scale to determine children's adaptation to this deformity and the indication for surgical management. A control sample of four patients was included in the study for comparison. Results  Eight patients with 11 limb deformities were included in the study. The affected limbs' range of motion (ROM) was compared with the ROM of the unaffected limbs and the control patients. There were no statistically significant differences in ROM between the two groups except for fixed pronation deformity of the synostosis ( p  = 0.0133). The average ABILHAND-Kids score for all children was 38.625 ± 3.021, close to the average score of 42. Only one patient with bilateral involvement underwent surgical correction of the deformity 5 years from the initial presentation. Conclusion  CRUS is a rare congenital condition that can affect daily living activities. Surgical correction is rarely indicated and is only preserved for patients with bilateral involvement and significant functional impairment.

Abstract Image

儿童先天性尺桡关节闭锁的功能评估。
背景先天性尺桡关节闭锁(CRUS)是一种罕见的疾病,由胚胎期关节分离失败引起。前臂常内旋固定,影响上肢及手部功能。治疗范围从观察到手术选择,以恢复肢体功能和防止疾病复发。该研究旨在评估CRUS患者的功能结局。方法对2010年至2020年期间8例CRUS患者进行前瞻性评估。数据包括病史、体格检查和使用ABILHAND-Kids功能量表进行功能评估,以确定儿童对这种畸形的适应情况和手术治疗的指征。4例患者作为对照纳入研究进行比较。结果8例肢体畸形11例纳入研究。将患肢活动度(ROM)与未患肢活动度及对照组进行比较。两组间ROM差异无统计学意义(p = 0.0133)。所有儿童的ABILHAND-Kids平均得分为38.625±3.021分,接近平均得分42分。只有1例双侧受累患者在发病5年后接受了畸形手术矫正。结论CRUS是一种罕见的影响日常生活活动的先天性疾病。手术矫正很少指征,仅保留双侧受累和显著功能损害的患者。
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审稿时长
26 weeks
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