Radiographic outcomes of a treatment approach for lower leg deformities in patients with hereditary multiple exostoses.

IF 1 4区 医学 Q4 ORTHOPEDICS
Kenta Sawamura, Masaki Matsushia, Kenichi Mishima, Shiro Imagama
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引用次数: 0

Abstract

Hereditary multiple exostoses (HME) represent a rare skeletal disorder characterized by multiple osteochondromas, often leading to angular deformities in the lower limbs as well as leg length discrepancy (LLD), managed with tension band plates (TBP) for deformity correction. However, the utility of both angular deformity and LLD in HME has not been comprehensively evaluated. In this study, we retrospectively reviewed 25 pediatric patients with HME who visited our institution and reached skeletal maturity between 2012 and 2024, assessing a total of 50 limbs. Surgical indications included patients aged greater than 10 years with an open growth plate, predicted LLD of greater than or equal to 20 mm at the skeletal maturity, and a mechanical axis zone (MAZ) greater than or equal to Zone 2. We categorized the outcomes into four groups: excellent [LLD < 10 mm; mechanical axis percentage (%MA) ≤ ±25%], good (LLD < 15 mm; %MA ≤ ±50%), fair (LLD < 20 mm or at least one limb classified as %MA ≤ ±100%), and poor (worse than the previous categories). We used paired t-tests for statistical analyses. Among the 17 surgically treated patients, TBP was performed on 27 limbs and 60 physes. In most cases, multisite and staged surgeries were required. Angular deformities improved significantly, with the mean hip-knee-ankle angle reduced from 7.8 to 2.7° (lower extremity < 0.01), and 92% of limbs achieved MAZ Zone 1. LLD was corrected from 17.6 to 5.6 mm (P < 0.01) at an average correction rate of 0.47 mm/month. The final outcomes were excellent, good, and fair or poor in 12, 11, and 2 patients, respectively. Major complications were not observed. TBP treatment is effective in correcting both angular deformity and LLD in patients with HME, offering a minimally invasive strategy for comprehensive correction of this complex skeletal dysplasia. Careful surgical planning and timing are essential and staged multisite procedures are often required.

一种治疗遗传性多发性外骨骼增生患者下肢畸形的方法的影像学结果。
遗传性多发性外生骨病(HME)是一种罕见的骨骼疾病,以多发性骨软骨瘤为特征,通常导致下肢角畸形和腿长差异(LLD),采用张力带钢板(TBP)进行畸形矫正。然而,角度畸形和LLD在HME中的应用尚未得到全面评估。在本研究中,我们回顾性分析了2012年至2024年间到我们机构就诊并达到骨骼成熟的25例小儿HME患者,共评估了50条肢体。手术指征包括年龄大于10岁的开放生长板患者,预测骨骼成熟时LLD大于等于20mm,机械轴区(MAZ)大于等于2区。我们将结果分为四组:优秀[LLD]
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来源期刊
CiteScore
2.20
自引率
9.10%
发文量
170
审稿时长
4-8 weeks
期刊介绍: The journal highlights important recent developments from the world''s leading clinical and research institutions. The journal publishes peer-reviewed papers on the diagnosis and treatment of pediatric orthopedic disorders. It is the official journal of IFPOS (International Federation of Paediatric Orthopaedic Societies). Submitted articles undergo a preliminary review by the editor. Some articles may be returned to authors without further consideration. Those being considered for publication will undergo further assessment and peer-review by the editors and those invited to do so from a reviewer pool. ​
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