Restoration of elbow active flexion in children with amyoplasia: What is the better age to do the operation?

Abstract

BACKGROUND: The absence of active elbow flexion is the most common problem in children with amyoplasia, leading to daily living difficulties. Many variants of muscle transfer are used for the restoration of active elbow flexion. The pectoralis major and latissimus dorsi muscles are the most used muscles for this purpose; however, the optimal age for these operations is not reported in the literature.

AIM: This study aimed to determine the optimal age of children with amyoplasia for the restoration of active elbow flexion.

MATERIALS AND METHODS: The retrospective study involved 61 patients (90 upper limbs) with amyoplasia (30 [49%] girls and 31 [51%] boys) who were examined and treated between 2011 and 2020. In 46 (51.1%) cases, we used major muscles, and in 44 (48.9%) cases, the latissimus dorsi muscle was used as a donor muscle. All patients were divided into four groups: group 1 included children aged 1–3 years (n = 17, 27.9%); group 2, 3–7 years (n = 30, 49.2%); group 3, 7–11 years (n = 8, 13.1%), and group 4, 12–18 years (n = 6, 9.8%). The clinical examination of the patients was conducted before and after the operation (≥6 months). Statistical data processing was performed using Statistica 10 and SAS JMP 11. To describe the numerical scales, the average value and standard deviation (M ± SD) were used.

RESULTS: The age of the patients at the time of surgery was 5.16 ± 3.72 years, and the postoperative follow-up period was 41.93 ± 30.13 months. Elbow flexion contractures were observed mainly in groups 1–3 (p < 0.05). The greatest changes in indicators such as the strength of forearm flexor muscles, active elbow flexion, and function of the elbow were noted in group 1 (p < 0.05). The same postoperative indicators were worse in group 4 than in younger patients (p < 0.05). Groups 3 and 4 had less strength of the donor muscles than groups 1 and 2 (p < 0.05).

CONCLUSIONS: The retrospective analysis of the results of the restoration of active elbow flexion in children with amyoplasia allowed us to recommend these operations in children aged 1–3 years. The prevention of elbow flexion contractures and the formation of a new stereotype of movement help improve the self-ability of these patients and the treatment results.

Keywords

arthrogryposis / amyoplasia / elbow / flexion / contractures / movement disorders / muscle transfer / latissimus dorsi muscle / pectoralis major muscle

References

[1] Bevan WP, Hall JG, Bamshad M, et al. Arthrogryposis multiplex congenita (amyoplasia): an orthopaedic perspective. J Pediatr Orthop. 2007;27(5):594–600. DOI: 10.1097/BPO.0b013e318070cc76

[2] Bevan W.P., Hall J.G., Bamshad M., et al. Arthrogryposis multiplex congenita (amyoplasia): an orthopaedic perspective // J. Pediatr. Orthop. 2007. Vol. 27. No. 5. P. 594–600. DOI: 10.1097/BPO.0b013e318070cc76

[3] Bevan WP, Hall JG, Bamshad M, et al. Arthrogryposis multiplex congenita (amyoplasia): an orthopaedic perspective. J Pediatr Orthop. 2007;27(5):594–600. DOI: 10.1097/BPO.0b013e318070cc76

[4] Hall JG. Arthrogryposis multiplex congenita: etiology, genetics, classification, diagnostic approach, and general aspects. J Pediatr Orthop B. 1997;6(3):159–166.

[5] Hall J.G. Arthrogryposis multiplex congenita: etiology, genetics, classification, diagnostic approach, and general aspects // J. Pediatr. Orthop. B. 1997. Vol. 6. No. 3. P. 159–166.

[6] Hall JG. Arthrogryposis multiplex congenita: etiology, genetics, classification, diagnostic approach, and general aspects. J Pediatr Orthop B. 1997;6(3):159–166.

[7] Doyle JR, James PM, Larsen LJ, et al. Restoration of elbow flexion in arthrogryposis multiplex congenita. J Hand Surg Am. 1980;5(2):149–152. DOI: 10.1016/s0363-5023(80)80146-8.

[8] Doyle J.R., James P.M., Larsen L.J., et al. Restoration of elbow flexion in arthrogryposis multiplex congenita // J. Hand Surg Am. 1980. Vol. 5. No. 2. P. 149–152. DOI: 10.1016/s0363-5023(80)80146-8

[9] Doyle JR, James PM, Larsen LJ, et al. Restoration of elbow flexion in arthrogryposis multiplex congenita. J Hand Surg Am. 1980;5(2):149–152. DOI: 10.1016/s0363-5023(80)80146-8.

[10] Van Heest A, Waters PM, Simmons BP. Surgical treatment of arthrogryposis of the elbow. J Hand Surg Am. 1998;23(6):1063–1070.

[11] Van Heest A., Waters P.M., Simmons B.P. Surgical treatment of arthrogryposis of the elbow // J. Hand Surg. Am. 1998. Vol. 23. No. 6. P. 1063–1070.

[12] Van Heest A, Waters PM, Simmons BP. Surgical treatment of arthrogryposis of the elbow. J Hand Surg Am. 1998;23(6):1063–1070.

[13] Ezaki M. Treatment of the upper limb in the child with arthrogryposis. Hand Clin. 2000;16(4):703–711.

[14] Ezaki M. Treatment of the upper limb in the child with arthrogryposis // Hand Clin. 2000. Vol. 16. No. 4. P. 703–711.

[15] Ezaki M. Treatment of the upper limb in the child with arthrogryposis. Hand Clin. 2000;16(4):703–711.

[16] Gagnon E, Fogelson N, Seyfer AE. Use of the latissimus dorsi muscle to restore elbow flexion in arthrogryposis. Plast Reconstr Surg. 2000;106(7):1582–1585. DOI: 10.1097/00006534-200012000-00022

[17] Gagnon E., Fogelson, N., Seyfer, A.E. Use of the latissimus dorsi muscle to restore elbow flexion in arthrogryposis // Plast. Reconstr. Surg. Vol. 106. No. 7. P. 1582–1585. DOI: 10.1097/00006534-200012000-00022

[18] Gagnon E, Fogelson N, Seyfer AE. Use of the latissimus dorsi muscle to restore elbow flexion in arthrogryposis. Plast Reconstr Surg. 2000;106(7):1582–1585. DOI: 10.1097/00006534-200012000-00022

[19] Lahoti O, Bell MJ. Transfer of pectoralis major in arthrogryposis to restore elbow flexion: deteriorating results in the long term. J Bone Joint Surg Br. 2005;87(6):858–860. DOI: 10.1302/0301-620X.87B6.15506

[20] Lahoti O., Bell M.J. Transfer of pectoralis major in arthrogryposis to restore elbow flexion: deteriorating results in the long term // J. Bone Joint. Surg. Br. 2005. Vol. 87. No. 6. P. 858–860. DOI: 10.1302/0301-620X.87B6.15506

[21] Lahoti O, Bell MJ. Transfer of pectoralis major in arthrogryposis to restore elbow flexion: deteriorating results in the long term. J Bone Joint Surg Br. 2005;87(6):858–860. DOI: 10.1302/0301-620X.87B6.15506

[22] Chomiak J, Dungl P. Reconstruction of elbow flexion in arthrogryposis multiplex congenita type I. Part I: surgical anatomy and vascular and nerve supply of the pectoralis major muscle as a basis for muscle transfer. J Child Orthop. 2008;2(5):357–364. DOI: 10.1007/s11832-008-0130-0

[23] Chomiak J., Dungl P. Reconstruction of elbow flexion in arthrogryposis multiplex congenita type I. Part I: surgical anatomy and vascular and nerve supply of the pectoralis major muscle as a basis for muscle transfer // J. Child. Orthop. 2008. Vol. 2. No. 5. P. 357–364. DOI: 10.1007/s11832-008-0130-0

[24] Chomiak J, Dungl P. Reconstruction of elbow flexion in arthrogryposis multiplex congenita type I. Part I: surgical anatomy and vascular and nerve supply of the pectoralis major muscle as a basis for muscle transfer. J Child Orthop. 2008;2(5):357–364. DOI: 10.1007/s11832-008-0130-0

[25] Gogola GR, Ezaki M, Oishi SN, et al. Long head of the triceps muscle transfer for active elbow flexion in arthrogryposis. Tech Hand Up Extrem Surg. 2010;14:121–124. DOI: 10.1097/BTH.0b013e3181da07aa

[26] Gogola G.R., Ezaki M., Oishi S.N., et al. Long head of the triceps muscle transfer for active elbow flexion in arthrogryposis // Tech. Hand. Up. Extrem. Surg. 2010. Vol. 14. No. 2. P. 121–124. DOI: 10.1097/BTH.0b013e3181da07aa

[27] Gogola GR, Ezaki M, Oishi SN, et al. Long head of the triceps muscle transfer for active elbow flexion in arthrogryposis. Tech Hand Up Extrem Surg. 2010;14:121–124. DOI: 10.1097/BTH.0b013e3181da07aa

[28] Chomiak J, Dungl P, Včelák J. reconstruction of elbow flexion in arthrogryposis multiplex congenita type I. J Pediatr Orthop. 2014;34(8):799–807. DOI: 10.1097/bpo.0000000000000204

[29] Chomiak J., Dungl P., Včelák J. Reconstruction of elbow flexion in arthrogryposis multiplex congenita type I // J. Pediatr. Orthop. 2014. Vol. 34. No. 8. P. 799–807. DOI: 10.1097/bpo.0000000000000204

[30] Chomiak J, Dungl P, Včelák J. reconstruction of elbow flexion in arthrogryposis multiplex congenita type I. J Pediatr Orthop. 2014;34(8):799–807. DOI: 10.1097/bpo.0000000000000204

[31] Takagi T, Seki A, Kobayashi Y, et al. Isolated muscle transfer to restore elbow flexion in children with arthrogryposis. J Hand Surg Asian Pac. 2016;21(1):44–48. DOI: 10.1142/S2424835516500053

[32] Takagi T., Seki A., Kobayashi Y., et al. Isolated muscle transfer to restore elbow flexion in children with arthrogryposis // J. Hand. Surg. Asian. Pac. 2016. Vol. 21. No. 1. P. 44–48. DOI: 10.1142/S2424835516500053

[33] Takagi T, Seki A, Kobayashi Y, et al. Isolated muscle transfer to restore elbow flexion in children with arthrogryposis. J Hand Surg Asian Pac. 2016;21(1):44–48. DOI: 10.1142/S2424835516500053

[34] Zargarbashi R, Nabian MH, Werthel JD, et al. Is bipolar latissimus dorsi transfer a reliable option to restore elbow flexion in children with arthrogryposis? A review of 13 tendon transfers. Shoulder Elbow Surg. 2017;26(11):2004–2009. DOI: 10.1016/j.jse.2017.04.002

[35] Zargarbashi R., Nabian M.H., Werthel J.D., et al. Is bipolar latissimus dorsi transfer a reliable option to restore elbow flexion in children with arthrogryposis? A review of 13 tendon transfers // J. Shoulder. Elbow. Surg. 2017. Vol. 26. No. 11. P. 2004–2009. DOI: 10.1016/j.jse.2017.04.002

[36] Zargarbashi R, Nabian MH, Werthel JD, et al. Is bipolar latissimus dorsi transfer a reliable option to restore elbow flexion in children with arthrogryposis? A review of 13 tendon transfers. Shoulder Elbow Surg. 2017;26(11):2004–2009. DOI: 10.1016/j.jse.2017.04.002

[37] Oishi S, Agranovich O, Zlotolow D, et al. Treatment and outcomes of arthrogryposis in the upper extremity. Am J Med Genet C Semin Med Genet. 2019;181(3):363–371. DOI: 10.1002/ajmg.c.31722

[38] Oishi S., Agranovich O., Zlotolow D., et al. Treatment and outcomes of arthrogryposis in the upper extremity // Am. J. Med. Genet. C. Semin. Med. Genet. 2019. Vol. 181. No. 3. P. 363–371. DOI: 10.1002/ajmg.c.31722

[39] Oishi S, Agranovich O, Zlotolow D, et al. Treatment and outcomes of arthrogryposis in the upper extremity. Am J Med Genet C Semin Med Genet. 2019;181(3):363–371. DOI: 10.1002/ajmg.c.31722

[40] Agranovich OE, Kochenova EA, Trofimova SI, et al. Restoration of elbow active flexion via latissimus dorsii transfer in patients with arthrogryposis. Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2018;6(3):5–11. (In Russ.). DOI: 10.17816/PTORS6273-75

[41] Агранович О.Е., Коченова Е.А., Трофимова С.И., и др. Использование широчайшей мышцы спины для восстановления активного сгибания в локтевом суставе у больных с артрогрипозом // Ортопедия, травматология и восстановительная хирургия детского возраста. 2018. Т. 6. № 3. C. 5–11. DOI: 10.17816/PTORS6273-75

[42] Agranovich OE, Kochenova EA, Trofimova SI, et al. Restoration of elbow active flexion via latissimus dorsii transfer in patients with arthrogryposis. Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2018;6(3):5–11. (In Russ.). DOI: 10.17816/PTORS6273-75

[43] Sochol KM, Edwards G 3rd, Stevanovic M. Restoration of elbow flexion with a free functional gracilis muscle transfer in an arthrogrypotic patient using a motor nerve to pectoralis major. Hand (NY). 2020;15(5):739–743. DOI: 10.1177/1558944720923412

[44] Sochol K.M., Edwards G. 3rd, Stevanovic M. Restoration of elbow flexion with a free functional gracilis muscle transfer in an arthrogrypotic patient using a motor nerve to pectoralis major // Hand (NY). 2020. Vol. 15. No. 5. P. 739–743. DOI: 10.1177/1558944720923412

[45] Sochol KM, Edwards G 3rd, Stevanovic M. Restoration of elbow flexion with a free functional gracilis muscle transfer in an arthrogrypotic patient using a motor nerve to pectoralis major. Hand (NY). 2020;15(5):739–743. DOI: 10.1177/1558944720923412

[46] Doyle JR, James PM, Larsen LJ, et al. Restoration of elbow flexion in arthrogryposis multiplex congenita. J Hand Surg Am. 1980;5(2):149–152. DOI: 10.1016/s0363-5023(80)80146-8

[47] Doyle J.R., James P.M., Larsen L.J., et al. Restoration of elbow flexion in arthrogryposis multiplex congenital // J. Hand. Surg. Am. 1980. Vol. 5. No. 2. P. 149–152. DOI: 10.1016/s0363-5023(80)80146-8

[48] Doyle JR, James PM, Larsen LJ, et al. Restoration of elbow flexion in arthrogryposis multiplex congenita. J Hand Surg Am. 1980;5(2):149–152. DOI: 10.1016/s0363-5023(80)80146-8

[49] Takagi T, Seki A, Kobayashi Y, et al. Isolated muscle transfer to restore elbow flexion in children with arthrogryposis. J Hand Surg Asian Pac. 2016;21(01):44–48. DOI: 10.1142/s2424835516500053

[50] Takagi T., Seki A., Kobayashi Y., et al. Isolated muscle transfer to restore elbow flexion in children with arthrogryposis // J. Hand. Surg. Asian. Pac. Vol. 2016. Vol. 21. No. 01. P. 44–48. DOI: 10.1142/s2424835516500053

[51] Takagi T, Seki A, Kobayashi Y, et al. Isolated muscle transfer to restore elbow flexion in children with arthrogryposis. J Hand Surg Asian Pac. 2016;21(01):44–48. DOI: 10.1142/s2424835516500053

[52] Steen U, Wekre LL, Vøllestad NK. Physical functioning and activities of daily living in adults with amyoplasia, the most common form of arthrogryposis. A cross-sectional study. Disabil Rehabil. 2018;40(23):2767–2779. DOI: 10.1080/09638288.2017.1357211

[53] Steen U., Wekre L.L., Vøllestad N.K. Physical functioning and activities of daily living in adults with amyoplasia, the most common form of arthrogryposis. A cross-sectional study // Disabil. Rehabil. 2018. Vol. 40. No. 23. P. 2767–2779. DOI: 10.1080/09638288.2017.1357211

[54] Steen U, Wekre LL, Vøllestad NK. Physical functioning and activities of daily living in adults with amyoplasia, the most common form of arthrogryposis. A cross-sectional study. Disabil Rehabil. 2018;40(23):2767–2779. DOI: 10.1080/09638288.2017.1357211

[55] Agranovich OE, Savina MV, Blagoveshchensky ED. Compensatory and adaptive mechanisms in children with congenital multiple arthrogryposis in the absence of active flexion in the elbow joint. Problems of balneology, physiotherapy, and exercise therapy. 2021;98(1):31–37. (In Russ.). DOI: 10.17116/kurort20219801131

[56] Агранович О.Е., Савина М.В., Благовещенский Е.Д. Компенсаторно-приспособительные механизмы у детей с врожденным множественным артрогрипозом с отсутствием активного сгибания в локтевом суставе // Вопросы курортологии, физиотерапии и лечебной физической культуры. 2021. Т. 98. № 1. С. 31–37. DOI: 10.17116/kurort20219801131

[57] Agranovich OE, Savina MV, Blagoveshchensky ED. Compensatory and adaptive mechanisms in children with congenital multiple arthrogryposis in the absence of active flexion in the elbow joint. Problems of balneology, physiotherapy, and exercise therapy. 2021;98(1):31–37. (In Russ.). DOI: 10.17116/kurort20219801131

Funding

Министерство здравоохранения Российской ФедерацииMinistry of Health of the Russian Federation(121031700125-7)

RIGHTS & PERMISSIONS

Agranovich O., Petrova E.V., Batkin S.F., Kenis V.M., Sapogovskiy A.V., Blagoveschenskiy E.D.