Preliminary study of Ilizarov technique in treatment of lower limb deformity caused by achondroplasia_Chinese Journal of Reparative and Reconstructive Surgery

Authors：

ZHENG Xuejian ¹ ,  QIN Sihe ¹ , SHI Lei ¹ , GUO Baofeng ² , ZHAO Jun ¹ , YIN Haiyang ¹

1. Department of Orthopedic Surgery, Rehabilitation Hospital Affiliated to National Research Center for Rehabilitation AIDS, Beijing, 100176, P. R. China;
2. Department of Orthopedics, Chuiyangliu Hospital Affiliated to Tsinghua University, Beijing, 100022, P. R. China;

Corresponding author：

QIN Sihe, Email: qinsihe@163.com

Keywords：

Ilizarov technique; achondroplasia; lower limb deformity; osteotomy correction; limb lengthening

DOI：

10.7507/1002-1892.202210072

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Objective To investigate the surgical method and preliminary effectiveness of Ilizarov technique in the treatment of lower limb deformity caused by achondroplasia. Methods The clinical data of 38 patients with lower limb deformity caused by achondroplasia treated by Ilizarov technique between February 2014 and September 2021 were retrospectively analyzed. There were 18 males and 20 females, the age ranged from 7 to 34 years, with an average of 14.8 years. All patients presented with bilateral knee varus deformity. The preoperative varus angles was (15.2±4.2)°, and knee society score (KSS) was 61.8±7.2. Nine of these patients underwent tibia and fibula osteotomy, 29 cases underwent tibia and fibula osteotomy and bone lengthening at the same time. Full-length bearing position X-ray films of bilateral lower limbs were taken to measure the bilateral varus angles, analyze the healing index, and record the occurrence of complications. KSS score was used to evaluate the improvement of knee joint function before and after operation. Results All 38 cases were followed up 9-65 months, with an average of 26.3 months. Needle tract infection occurred in 4 cases and needle tract loosening occurred in 2 cases after operation, which were improved after symptomatic treatment such as dressing change, Kirschner wire change, and oral antibiotics, and no neurovascular injury occurred in all patients. The external fixator was worn for 3-11 months after operation, with an average of 7.6 months, and the healing index was 43-59 d/cm, with an average of 50.3 d/cm. At last follow-up, the leg was 3-10 cm longer, with an average of 5.5 cm. The varus angles was (1.5±0.2)° and the KSS score was 93.7±2.6, which significantly improved when compared with those before operation (P<0.05). Conclusion Ilizarov technique is a safe and effective method for the treatment of short limb with genu varus deformity caused by achondroplasia, which can improve the quality of life of patients.

Citation： ZHENG Xuejian, QIN Sihe, SHI Lei, GUO Baofeng, ZHAO Jun, YIN Haiyang. Preliminary study of Ilizarov technique in treatment of lower limb deformity caused by achondroplasia. Chinese Journal of Reparative and Reconstructive Surgery, 2023, 37(2): 157-161. doi: 10.7507/1002-1892.202210072 Copy

1.	中国医师协会医学遗传医师分会, 中华医学会儿科学分会内分泌遗传代谢学组, 中华医学会儿科学分会罕见病学组, 等. 软骨发育不全诊断及治疗专家共识. 中华儿科杂志, 2021, 59(7): 545-550.
2.	宋蕊, 张鑫悦, 任远, 等. 胎儿软骨发育不全的临床特点分析. 中华妇产科杂志, 2022, 57(4): 284-288.
3.	Ornitz DM, Legeai-Mallet L. Achondroplasia: Development, pathogenesis, and therapy. Dev Dyn, 2017, 246(4): 291-309.
4.	Horton WA, Hall JG, Hecht JT. Achondroplasia. Lancet, 2007, 370(9582): 162-172.
5.	杜晓杰. 儿童软骨发育不全的诊断与外科治疗. 中国临床医生杂志, 2020, 48(2): 135-137.
6.	Wright MJ, Irving MD. Clinical management of achondroplasia. Arch Dis Child, 2012, 97(2): 129-134.
7.	Paley D. Extensive limb lengthening for achondroplasia and hypochondroplasia. Children (Basel), 2021, 8(7): 540. doi: 10.3390/children8070540.
8.	秦泗河. Ilizarov技术概述. 中华骨科杂志, 2006, 26(9): 642-645.
9.	秦泗河, 夏和桃, 彭爱民, 等. 胫骨与跟腱同步弹性延长器的设计与临床应用. 中华外科杂志, 2004, 42(19): 1157-1160.
10.	Chilbule SK, Dutt V, Madhuri V. Limb lengthening in achondroplasia. Indian J Orthop, 2016, 50(4): 397-405.
11.	Ko KR, Shim JS, Chung CH, et al. Surgical results of limb lengthening at the femur, tibia, and humerus in patients with achondroplasia. Clin Orthop Surg, 2019, 11(2): 226-232.
12.	Elbatrawy YA, Abdelaziz AM, Zidan MAA. Limb lengthening for achondroplasia: Systematic review and meta-analysis. The Egyptian Journal of Hospital Medicine, 2019, 74(7): 1491-1513.
13.	Paley D. Problems, obstacles, and complications of limb lengthening by the Ilizarov technique. Clin Orthop Relat Res, 1990, (250): 81-104.
14.	Donaldson J, Aftab S, Bradish C. Achondroplasia and limb lengthening: Results in a UK cohort and review of the literature. J Orthop, 2015, 12(1): 31-34.
15.	Kim SJ, Balce GC, Agashe MV, et al. Is bilateral lower limb lengthening appropriate for achondroplasia?: midterm analysis of the complications and quality of life Clin Orthop Relat Res, 2012, 470(2): 616-621.
16.	罗楠, 郭锦丽. Ilizarov外固定架针道感染预防的护理研究进展. 实用骨科杂志, 2021, 27(5): 467-470.
17.	臧建成, 秦泗河. 下肢延长术并发症的分类与治疗策略. 中华骨科杂志, 2021, 41(11): 728-736.

1. 中国医师协会医学遗传医师分会, 中华医学会儿科学分会内分泌遗传代谢学组, 中华医学会儿科学分会罕见病学组, 等. 软骨发育不全诊断及治疗专家共识. 中华儿科杂志, 2021, 59(7): 545-550.
2. 宋蕊, 张鑫悦, 任远, 等. 胎儿软骨发育不全的临床特点分析. 中华妇产科杂志, 2022, 57(4): 284-288.
3. Ornitz DM, Legeai-Mallet L. Achondroplasia: Development, pathogenesis, and therapy. Dev Dyn, 2017, 246(4): 291-309.
4. Horton WA, Hall JG, Hecht JT. Achondroplasia. Lancet, 2007, 370(9582): 162-172.
5. 杜晓杰. 儿童软骨发育不全的诊断与外科治疗. 中国临床医生杂志, 2020, 48(2): 135-137.
6. Wright MJ, Irving MD. Clinical management of achondroplasia. Arch Dis Child, 2012, 97(2): 129-134.
7. Paley D. Extensive limb lengthening for achondroplasia and hypochondroplasia. Children (Basel), 2021, 8(7): 540. doi: 10.3390/children8070540.
8. 秦泗河. Ilizarov技术概述. 中华骨科杂志, 2006, 26(9): 642-645.
9. 秦泗河, 夏和桃, 彭爱民, 等. 胫骨与跟腱同步弹性延长器的设计与临床应用. 中华外科杂志, 2004, 42(19): 1157-1160.
10. Chilbule SK, Dutt V, Madhuri V. Limb lengthening in achondroplasia. Indian J Orthop, 2016, 50(4): 397-405.
11. Ko KR, Shim JS, Chung CH, et al. Surgical results of limb lengthening at the femur, tibia, and humerus in patients with achondroplasia. Clin Orthop Surg, 2019, 11(2): 226-232.
12. Elbatrawy YA, Abdelaziz AM, Zidan MAA. Limb lengthening for achondroplasia: Systematic review and meta-analysis. The Egyptian Journal of Hospital Medicine, 2019, 74(7): 1491-1513.
13. Paley D. Problems, obstacles, and complications of limb lengthening by the Ilizarov technique. Clin Orthop Relat Res, 1990, (250): 81-104.
14. Donaldson J, Aftab S, Bradish C. Achondroplasia and limb lengthening: Results in a UK cohort and review of the literature. J Orthop, 2015, 12(1): 31-34.
15. Kim SJ, Balce GC, Agashe MV, et al. Is bilateral lower limb lengthening appropriate for achondroplasia?: midterm analysis of the complications and quality of life Clin Orthop Relat Res, 2012, 470(2): 616-621.
16. 罗楠, 郭锦丽. Ilizarov外固定架针道感染预防的护理研究进展. 实用骨科杂志, 2021, 27(5): 467-470.
17. 臧建成, 秦泗河. 下肢延长术并发症的分类与治疗策略. 中华骨科杂志, 2021, 41(11): 728-736.

Chinese Journal of Reparative and Reconstructive Surgery

Preliminary study of Ilizarov technique in treatment of lower limb deformity caused by achondroplasia

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