Lenthening and reconstruction progress of achondroplastic short arm deformity_Chinese Journal of Reparative and Reconstructive Surgery

Authors：

GUO Baofeng ¹ ,  QIN Sihe ²

1. The 2nd Department of Orthopedics, Beijing Chuiyangliu Hospital Affiliated to Tsinghua University, Beijing, 100122, P. R. China;
2. Department of Orthopedics, Rehabilitation Hospital of the National Research Center for Rehabilitation Technical Aids, Beijing, 100176, P. R. China;

Corresponding author：

QIN Sihe, Email: qinsihe@163.com

Keywords：

Achondroplasia; short arm deformity; lengthening and reconstruction

DOI：

10.7507/1002-1892.202410047

Video：

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Objective To describe the characteristics of short arm deformity in patients with achondroplasia, and summarize the progress of its lenthening and reconstruction, so as to provide reference for clinical diagnosis and treatment. Methods The literature on the lenthening of upper limb with achondroplastic short arm deformity at home and abroad in recent years was reviewed, and the characteristics, extension methods, postoperative management, effectiveness evaluation, and related complications of short arm deformity were summarized. Results Achondroplastic short arm deformity affect the patient’s daily perineal hygiene activities. Although the upper limb is proportionately shortened, the humerus is mainly short limb deformity. Bilateral humeral lengthening is a common treatment method, and the traditional lengthening tools are mainly external fixation, guided by Ilizarov distraction osteogenesis concept; intramedullary lengthening is the latest treatment method. Lengthening percentage and healing index are commonly used clinical evaluation indexes, and complications such as nerve injury may occur during upper limb lengthening. Conclusion In addition to appearance improvement, achondroplastic short arm lengthening is of great significance in achieving self-management of individual perineal hygiene. Lenthening and reconstruction methods are constantly being innovated and improved.

1.	Horton WA, Hall JG, Hecht JT. Achondroplasia. Lancet, 2007, 370(9582): 162-172.
2.	Shadi M, Musielak B, Koczewski P, et al. Humeral lengthening in patients with achondroplasia and in patients with post-septic shortening: comparison of procedure efficiency and safety. Int Orthop, 2018, 42(2): 419-426.
3.	Kashiwagi N, Suzuki S, Seto Y, et al. Bilateral humeral lengthening in achondroplasia. Clin Orthop Relat Res, 2001(391): 251-257.
4.	Bellus GA, Hefferon TW, Ortiz de Luna RI, et al. Achondroplasia is defined by recurrent G380R mutations of FGFR3. Am J Hum Genet, 1995, 56(2): 368-373.
5.	Kim SJ, Agashe MV, Song SH, et al. Comparison between upper and lower limb lengthening in patients with achondroplasia: a retrospective study. J Bone Joint Surg (Br), 2012, 94(1): 128-133.
6.	Balci HI, Kocaoglu M, Sen C, et al. Bilateral humeral lengthening in achondroplasia with unilateral external fixators: is it safe and does it improve daily life? Bone Joint J, 2015, 97-B(11): 1577-1581.
7.	刘红艳, 李亚军, 邢丽媛, 等. 母亲孕期营养素补充与母婴不良结局的相关研究进展. 临床医学进展, 2023, 13(11): 17349-17355.
8.	Lee FY, Schoeb JS, Yu J, et al. Operative lengthening of the humerus: indications, benefits, and complications. J Pediatr Orthop, 2005, 25(5): 613-616.
9.	McLawhorn AS, Sherman SL, Blyakher A, et al. Humeral lengthening and deformity correction with the multiaxial correction system. J Pediatr Orthop B, 2011, 20(2): 111-116.
10.	Dick HM, Tietjen R. Humeral lengthening for septic neonatal growth arrest. Case report. J Bone Joint Surg (Am), 1978, 60(8): 1138-1139.
11.	Al-Sayyad MJ. Taylor spatial frame in the treatment of upper extremity conditions. J Pediatr Orthop, 2012, 32(2): 169-178.
12.	Liu T, Zhang X, Li Z, et al. Callus distraction for humeral nonunion with bone loss and limb shortening caused by chronic osteomyelitis. J Bone Joint Surg (Br), 2008, 90(6): 795-800.
13.	Yasui N, Kawabata H, Kojimoto H, et al. Lengthening of the lower limbs in patients with achondroplasia and hypochondroplasia. Clin Orthop Relat Res, 1997(344): 298-306.
14.	Pawar AY, McCoy TH, Fragomen AT, et al. Does humeral lengthening with a monolateral frame improve function? Clin Orthop Relat Res, 2013, 471(1): 277-283.
15.	Ruan H, Zhu Y, Liu S, et al. Humeral lengthening and proximal deformity correction with monorail external fixator in young adults. Int Orthop, 2018, 42(5): 1107-1111.
16.	殷海阳, 秦泗河, 石磊, 等. Ilizarov技术治疗软骨发育不全下肢畸形的研究进展. 中华骨与关节外科杂志, 2023, 16(9): 843-848.
17.	秦泗河, 郭保逢, 李旭, 等. 小儿肢体形态与功能重建. 北京: 北京大学医学出版社, 2023: 241-244.
18.	Jager T, Popkov D, Lascombes P, et al. Elastic intramedullary nailing as a complement to Ilizarov’s method for forearm lengthening: a comparative pediatric prospective study. Orthop Traumatol Surg Res, 2012, 98(4): 376-382.
19.	王昌兴, 吴建民, 俞宏亮, 等. 单臂外固定架在肱骨延长中的应用. 中国矫形外科杂志, 2000, 7(5): 505-506.
20.	Tiefenboeck TM, Zak L, Wozasek GE. Intramedullary magnetically actuated limb lengthening in a patient with congenital humeral limb shortening. Injury, 2016, 47(7): 1597-1600.
21.	Kurtz AM, Rozbruch SR. Humerus lengthening with the PRECICE internal lengthening nail. J Pediatr Orthop, 2017, 37(4): e296-e300.
22.	Hammouda AI, Standard SC, Robert Rozbruch S, et al. Humeral lengthening with the PRECICE magnetic lengthening nail. HSS J, 2017, 13(3): 217-223.
23.	Sheridan GA, Falk DP, Fragomen AT, et al. Motorized internal limb-lengthening (MILL) techniques are superior to alternative limb-lengthening techniques: A systematic review and meta-analysis of the literature. JB JS Open Access, 2020, 5(4): e20.00115. doi: 10.2106/JBJS.OA.20.00115.
24.	Zak L, Tiefenboeck TM, Wozasek GE. Innovative technique in extended intramedullary humeral lengthening: A case report. JBJS Case Connect, 2019, 9(3): e0174. doi: 10.2106/JBJS.CC.18.00174.
25.	Morrison SG, Georgiadis AG, Dahl MT. Lengthening of the humerus using a motorized lengthening nail: A retrospective comparative series. J Pediatr Orthop, 2020, 40(6): e479-e486.
26.	Arenas-Miquelez A, Arbeloa-Gutierrez L, Amaya M, et al. Upper limb lengthening in achondroplasia using unilateral external fixation. J Pediatr Orthop, 2021, 41(4): e328-e336.
27.	Ginebreda I, Campillo-Recio D, Cárdenas C, et al. Surgical technique and outcomes for bilateral humeral lengthening for achondroplasia: 26-year experience. Musculoskelet Surg, 2019, 103(3): 257-262.
28.	Cheng SDJ, Wong KPL. Distraction osteogenesis of the humerus: a case of 100% humeral lengthening in a short duration without permanent complications. BMJ Case Rep, 2019, 12(5): e228222. doi: 10.1136/bcr-2018-228222.
29.	Hamanishi C, Yasuwaki Y, Kikuchi H, et al. Classification of the callus in limb lengthening. Radiographic study of 35 limbs. Acta Orthop Scand, 1992, 63(4): 430-433.
30.	Nakano-Matsuoka N, Fukiage K, Harada Y, et al. The prevalence of the complications and their associated factors in humeral lengthening for achondroplasia: retrospective study of 54 cases. J Pediatr Orthop B, 2017, 26(6): 519-525.
31.	Laufer A, Rölfing JD, Gosheger G, et al. What are the risks and functional outcomes associated with bilateral humeral lengthening using a monolateral external fixator in patients with achondroplasia? Clin Orthop Relat Res, 2022, 480(9): 1779-1789.
32.	Paley D. Problems, obstacles, and complications of limb lengthening by the Ilizarov technique. Clin Orthop Relat Res, 1990, (250): 81-104.

1. Horton WA, Hall JG, Hecht JT. Achondroplasia. Lancet, 2007, 370(9582): 162-172.
2. Shadi M, Musielak B, Koczewski P, et al. Humeral lengthening in patients with achondroplasia and in patients with post-septic shortening: comparison of procedure efficiency and safety. Int Orthop, 2018, 42(2): 419-426.
3. Kashiwagi N, Suzuki S, Seto Y, et al. Bilateral humeral lengthening in achondroplasia. Clin Orthop Relat Res, 2001(391): 251-257.
4. Bellus GA, Hefferon TW, Ortiz de Luna RI, et al. Achondroplasia is defined by recurrent G380R mutations of FGFR3. Am J Hum Genet, 1995, 56(2): 368-373.
5. Kim SJ, Agashe MV, Song SH, et al. Comparison between upper and lower limb lengthening in patients with achondroplasia: a retrospective study. J Bone Joint Surg (Br), 2012, 94(1): 128-133.
6. Balci HI, Kocaoglu M, Sen C, et al. Bilateral humeral lengthening in achondroplasia with unilateral external fixators: is it safe and does it improve daily life? Bone Joint J, 2015, 97-B(11): 1577-1581.
7. 刘红艳, 李亚军, 邢丽媛, 等. 母亲孕期营养素补充与母婴不良结局的相关研究进展. 临床医学进展, 2023, 13(11): 17349-17355.
8. Lee FY, Schoeb JS, Yu J, et al. Operative lengthening of the humerus: indications, benefits, and complications. J Pediatr Orthop, 2005, 25(5): 613-616.
9. McLawhorn AS, Sherman SL, Blyakher A, et al. Humeral lengthening and deformity correction with the multiaxial correction system. J Pediatr Orthop B, 2011, 20(2): 111-116.
10. Dick HM, Tietjen R. Humeral lengthening for septic neonatal growth arrest. Case report. J Bone Joint Surg (Am), 1978, 60(8): 1138-1139.
11. Al-Sayyad MJ. Taylor spatial frame in the treatment of upper extremity conditions. J Pediatr Orthop, 2012, 32(2): 169-178.
12. Liu T, Zhang X, Li Z, et al. Callus distraction for humeral nonunion with bone loss and limb shortening caused by chronic osteomyelitis. J Bone Joint Surg (Br), 2008, 90(6): 795-800.
13. Yasui N, Kawabata H, Kojimoto H, et al. Lengthening of the lower limbs in patients with achondroplasia and hypochondroplasia. Clin Orthop Relat Res, 1997(344): 298-306.
14. Pawar AY, McCoy TH, Fragomen AT, et al. Does humeral lengthening with a monolateral frame improve function? Clin Orthop Relat Res, 2013, 471(1): 277-283.
15. Ruan H, Zhu Y, Liu S, et al. Humeral lengthening and proximal deformity correction with monorail external fixator in young adults. Int Orthop, 2018, 42(5): 1107-1111.
16. 殷海阳, 秦泗河, 石磊, 等. Ilizarov技术治疗软骨发育不全下肢畸形的研究进展. 中华骨与关节外科杂志, 2023, 16(9): 843-848.
17. 秦泗河, 郭保逢, 李旭, 等. 小儿肢体形态与功能重建. 北京: 北京大学医学出版社, 2023: 241-244.
18. Jager T, Popkov D, Lascombes P, et al. Elastic intramedullary nailing as a complement to Ilizarov’s method for forearm lengthening: a comparative pediatric prospective study. Orthop Traumatol Surg Res, 2012, 98(4): 376-382.
19. 王昌兴, 吴建民, 俞宏亮, 等. 单臂外固定架在肱骨延长中的应用. 中国矫形外科杂志, 2000, 7(5): 505-506.
20. Tiefenboeck TM, Zak L, Wozasek GE. Intramedullary magnetically actuated limb lengthening in a patient with congenital humeral limb shortening. Injury, 2016, 47(7): 1597-1600.
21. Kurtz AM, Rozbruch SR. Humerus lengthening with the PRECICE internal lengthening nail. J Pediatr Orthop, 2017, 37(4): e296-e300.
22. Hammouda AI, Standard SC, Robert Rozbruch S, et al. Humeral lengthening with the PRECICE magnetic lengthening nail. HSS J, 2017, 13(3): 217-223.
23. Sheridan GA, Falk DP, Fragomen AT, et al. Motorized internal limb-lengthening (MILL) techniques are superior to alternative limb-lengthening techniques: A systematic review and meta-analysis of the literature. JB JS Open Access, 2020, 5(4): e20.00115. doi: 10.2106/JBJS.OA.20.00115.
24. Zak L, Tiefenboeck TM, Wozasek GE. Innovative technique in extended intramedullary humeral lengthening: A case report. JBJS Case Connect, 2019, 9(3): e0174. doi: 10.2106/JBJS.CC.18.00174.
25. Morrison SG, Georgiadis AG, Dahl MT. Lengthening of the humerus using a motorized lengthening nail: A retrospective comparative series. J Pediatr Orthop, 2020, 40(6): e479-e486.
26. Arenas-Miquelez A, Arbeloa-Gutierrez L, Amaya M, et al. Upper limb lengthening in achondroplasia using unilateral external fixation. J Pediatr Orthop, 2021, 41(4): e328-e336.
27. Ginebreda I, Campillo-Recio D, Cárdenas C, et al. Surgical technique and outcomes for bilateral humeral lengthening for achondroplasia: 26-year experience. Musculoskelet Surg, 2019, 103(3): 257-262.
28. Cheng SDJ, Wong KPL. Distraction osteogenesis of the humerus: a case of 100% humeral lengthening in a short duration without permanent complications. BMJ Case Rep, 2019, 12(5): e228222. doi: 10.1136/bcr-2018-228222.
29. Hamanishi C, Yasuwaki Y, Kikuchi H, et al. Classification of the callus in limb lengthening. Radiographic study of 35 limbs. Acta Orthop Scand, 1992, 63(4): 430-433.
30. Nakano-Matsuoka N, Fukiage K, Harada Y, et al. The prevalence of the complications and their associated factors in humeral lengthening for achondroplasia: retrospective study of 54 cases. J Pediatr Orthop B, 2017, 26(6): 519-525.
31. Laufer A, Rölfing JD, Gosheger G, et al. What are the risks and functional outcomes associated with bilateral humeral lengthening using a monolateral external fixator in patients with achondroplasia? Clin Orthop Relat Res, 2022, 480(9): 1779-1789.
32. Paley D. Problems, obstacles, and complications of limb lengthening by the Ilizarov technique. Clin Orthop Relat Res, 1990, (250): 81-104.

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