Clinical study of Cross-Union surgery for treatment of pseudarthrosis of tibia with neurofibromatosis type 1 in children_Chinese Journal of Reparative and Reconstructive Surgery

Authors：

MO Yueqiang , WU Chunxing , NING Bo ,  WANG Dahui

Department of Pediatric Orthopedics, Children’s Hospital of Fudan University, National Children’s Medical Center, Shanghai, 201102, P. R. China;

Corresponding author：

WANG Dahui, Email: wangdahui@fudan.edu.cn

Keywords：

Neurofibromatosis type 1; pseudarthrosis of the tibia; children; re-fracture; radiolological evaluation

DOI：

10.7507/1002-1892.202406056

Video：

Export PDF Favorites Scan Get Citation

Abstract Full text Figures/Tables Video References Cited by

Objective To evaluate the effectiveness of Cross-Union surgery for the treatment of pseudarthrosis of the tibia (PT) with neurofibromatosis type 1 (NF1). Methods The clinical data of 8 children of PT with NF1 who met the selection criteria between January 2018 and December 2023 was retrospectively analyzed. There were 5 boys and 3 girls, and the operative age ranged from 1.8 to 13.3 years with a median age of 3.5 years. According to Paley classification, there were 2 cases of type 2a, 2 cases of type 3, 2 cases of type 4a, and 2 cases of type 4c. There were 5 cases of first operation and 3 cases of re-fracture after previous operation. Six cases had leg length discrepancy before operation, and 2 of them had shortening over 2.0 cm. Except for 1 case of ankle fusion, the other 7 cases had ankle valgus. Preoperative coronal/sagittal angulation was recorded. Postoperative pseudarthrosis healing and refracture were observed. Leg length discrepancy and tibiotalar angle were measured and recorded before operation and at last follow-up. Inan imaging evaluation criteria was used to evaluate the imaging effect. Results All patients were followed up 12-37 months (mean, 23.5 months). One pseudarthrosis failed to heal at 12 months after operation and healed at 3 months after reoperation, while the other pseudarthrosis healed with a healing rate of 87.5% and a healing time of 4-8 months (mean, 5.3 months). No refracture occurred during the follow-up. At last follow-up, there were 2 new cases with leg length discrepancy, which were 0.7 cm and 1.3 cm, respectively. In 2 cases with the leg length discrepancy more than 2.0 cm before operation, the improvement was from 4.1 cm and 12.6 cm to 2.1 cm and 9.0 cm, respectively. There was no significant difference in leg length discrepancy between pre- and post-operation in 8 cases (P>0.05). At last follow-up, 6 patients still had ankle valgus, and there was no significant difference in the tibiotalar angle between pre- and post-operation (P>0.05); the tibial coronal/sagittal angulation significantly improved when compared with that before operation (P<0.05). According to Inan imaging evaluation criteria, 1 case was good, 6 cases were fair, and 1 case was poor. Conclusion Cross-Union surgery is an effective method for the treatment of PT with NF1 in children, can achieve good bone healing results with a low risk of re-fracture. The surgery may not have significant effects on leg length discrepancy and ankle valgus, and further treatment may be required.

Citation： MO Yueqiang, WU Chunxing, NING Bo, WANG Dahui. Clinical study of Cross-Union surgery for treatment of pseudarthrosis of tibia with neurofibromatosis type 1 in children. Chinese Journal of Reparative and Reconstructive Surgery, 2024, 38(10): 1214-1219. doi: 10.7507/1002-1892.202406056 Copy

1.	Sur ML, Armat I, Sur G, et al. Neurofibromatosis in children: Actually and perspectives. Children (Basel), 2022, 9(1): 40. doi: 10.3390/children9010040.
2.	Wilson BN, John AM, Handler MZ, et al. Neurofibromatosis type 1: New developments in genetics and treatment. J Am Acad Dermatol, 2021, 84(6): 1667-1676.
3.	Tamura R. Current understanding of neurofibromatosis type 1, 2, and schwannomatosis. Int J Mol Sci, 2021, 22(11): 5850. doi: 10.3390/ijms22115850.
4.	王智超, 李青峰. Ⅰ型神经纤维瘤病临床诊疗专家共识 (2021版). 中国修复重建外科杂志, 2021, 35(11): 1384-1395.
5.	Mladenov KV, Spiro AS, Krajewski KL, et al. Management of spinal deformities and tibial pseudarthrosis in children with neurofibromatosis type 1 (NF-1). Childs Nerv Syst, 2020, 36(10): 2409-2425.
6.	简书浪, 毛庆庆, 许思宇, 等. 先天性胫骨前外侧弯曲畸形并发胫骨骨折的危险因素分析. 中华骨科杂志, 2023, 43(17): 1164-1173.
7.	刘喜平, 刘昆. 四合一联合手术治疗CrawfordⅣ型儿童先天性胫骨假关节的疗效分析. 中华创伤骨科杂志, 2023, 25(11): 1003-1007.
8.	Yu H, Li Z, Tan Q, et al. Association between preoperative concurrent fibular pseudarthrosis and risk of postoperative ankle valgus in patients with congenital pseudarthrosis of the tibia. BMJ Paediatr Open, 2023, 7(1): e001763. doi: 10.1136/bmjpo-2022-001763.
9.	Van Royen K, Brems H, Legius E, et al. Prevalence of neurofibromatosis type 1 in congenital pseudarthrosis of the tibia. Eur J Pediatr, 2016, 175(9): 1193-1198.
10.	Banchhor H, Chimurkar V. Congenital pseudoarthrosis of the tibia: A narrative review. Cureus, 2022, 14(12): e32501. doi: 10.7759/cureus.32501.
11.	Shannon CE, Huser AJ, Paley D. Cross-union surgery for congenital pseudarthrosis of the tibia. Children (Basel), 2021, 8(7): 547. doi: 10.3390/children8070547.
12.	Choi IH, Lee SJ, Moon HJ, et al. “4-in-1 osteosynthesis” for atrophic-type congenital pseudarthrosis of the tibia. J Pediatr Orthop, 2011, 31(6): 697-704.
13.	Paley D. Congenital pseudarthrosis of the tibia: Combined pharmacologic and surgical treatment using biphosphonate intravenous infusion and bone morphogenic protein with periosteal and cancellous autogenous bone grafting, tibio-fibular cross union, intramedullary//Zorzi A. Bone grafting. London: InTech, 2012. http://dx.doi.org/10.5772/31149.
14.	Paley D. Congenital pseudarthrosis of the tibia: biological and biomechanical considerations to achieve union and prevent refracture. J Child Orthop, 2019, 13(2): 120-133.
15.	Ibrahim T, Beiri A, Azzabi M, et al. Reliability and validity of the subjective component of the American Orthopaedic Foot and Ankle Society clinical rating scales. J Foot Ankle Surg, 2007, 46(2): 65-74.
16.	Richards BS, Anderson TD. rhBMP-2 and intramedullary fixation in congenital pseudarthrosis of the tibia. J Pediatr Orthop, 2018, 38(4): 230-238.
17.	Vigouroux F, Mezzadri G, Parot R, et al. Vascularised fibula or induced membrane to treat congenital pseudarthrosis of the tibia: A multicentre study of 18 patients with a mean 9.5-year follow-up. Orthop Traumatol Surg Res, 2017, 103(5): 747-753.
18.	Borzunov DY, Chevardin AY, Mitrofanov AI. Management of congenital pseudarthrosis of the tibia with the Ilizarov method in a paediatric population: influence of aetiological factors. Int Orthop, 2016, 40(2): 331-339.
19.	Solla F, Lemoine J, Musoff C, et al. Surgical treatment of congenital pseudarthrosis of the forearm: Review and quantitative analysis of individual patient data. Hand Surg Rehabil, 2019, 38(4): 233-241.
20.	Zayda AI, Mesregah MK, Zalalo SH, et al. Functional and radiological outcomes after treatment of congenital pseudarthrosis of the tibia using the Ilizarov technique: a retrospective single-center study. J Orthop Traumatol, 2022, 23(1): 48. doi: 10.1186/s10195-022-00667-2.
21.	Inan M, El Rassi G, Riddle EC, et al. Residual deformities following successful initial bone union in congenital pseudoarthrosis of the tibia. J Pediatr Orthop, 2006, 26(3): 393-399.

1. Sur ML, Armat I, Sur G, et al. Neurofibromatosis in children: Actually and perspectives. Children (Basel), 2022, 9(1): 40. doi: 10.3390/children9010040.
2. Wilson BN, John AM, Handler MZ, et al. Neurofibromatosis type 1: New developments in genetics and treatment. J Am Acad Dermatol, 2021, 84(6): 1667-1676.
3. Tamura R. Current understanding of neurofibromatosis type 1, 2, and schwannomatosis. Int J Mol Sci, 2021, 22(11): 5850. doi: 10.3390/ijms22115850.
4. 王智超, 李青峰. Ⅰ型神经纤维瘤病临床诊疗专家共识 (2021版). 中国修复重建外科杂志, 2021, 35(11): 1384-1395.
5. Mladenov KV, Spiro AS, Krajewski KL, et al. Management of spinal deformities and tibial pseudarthrosis in children with neurofibromatosis type 1 (NF-1). Childs Nerv Syst, 2020, 36(10): 2409-2425.
6. 简书浪, 毛庆庆, 许思宇, 等. 先天性胫骨前外侧弯曲畸形并发胫骨骨折的危险因素分析. 中华骨科杂志, 2023, 43(17): 1164-1173.
7. 刘喜平, 刘昆. 四合一联合手术治疗CrawfordⅣ型儿童先天性胫骨假关节的疗效分析. 中华创伤骨科杂志, 2023, 25(11): 1003-1007.
8. Yu H, Li Z, Tan Q, et al. Association between preoperative concurrent fibular pseudarthrosis and risk of postoperative ankle valgus in patients with congenital pseudarthrosis of the tibia. BMJ Paediatr Open, 2023, 7(1): e001763. doi: 10.1136/bmjpo-2022-001763.
9. Van Royen K, Brems H, Legius E, et al. Prevalence of neurofibromatosis type 1 in congenital pseudarthrosis of the tibia. Eur J Pediatr, 2016, 175(9): 1193-1198.
10. Banchhor H, Chimurkar V. Congenital pseudoarthrosis of the tibia: A narrative review. Cureus, 2022, 14(12): e32501. doi: 10.7759/cureus.32501.
11. Shannon CE, Huser AJ, Paley D. Cross-union surgery for congenital pseudarthrosis of the tibia. Children (Basel), 2021, 8(7): 547. doi: 10.3390/children8070547.
12. Choi IH, Lee SJ, Moon HJ, et al. “4-in-1 osteosynthesis” for atrophic-type congenital pseudarthrosis of the tibia. J Pediatr Orthop, 2011, 31(6): 697-704.
13. Paley D. Congenital pseudarthrosis of the tibia: Combined pharmacologic and surgical treatment using biphosphonate intravenous infusion and bone morphogenic protein with periosteal and cancellous autogenous bone grafting, tibio-fibular cross union, intramedullary//Zorzi A. Bone grafting. London: InTech, 2012. http://dx.doi.org/10.5772/31149.
14. Paley D. Congenital pseudarthrosis of the tibia: biological and biomechanical considerations to achieve union and prevent refracture. J Child Orthop, 2019, 13(2): 120-133.
15. Ibrahim T, Beiri A, Azzabi M, et al. Reliability and validity of the subjective component of the American Orthopaedic Foot and Ankle Society clinical rating scales. J Foot Ankle Surg, 2007, 46(2): 65-74.
16. Richards BS, Anderson TD. rhBMP-2 and intramedullary fixation in congenital pseudarthrosis of the tibia. J Pediatr Orthop, 2018, 38(4): 230-238.
17. Vigouroux F, Mezzadri G, Parot R, et al. Vascularised fibula or induced membrane to treat congenital pseudarthrosis of the tibia: A multicentre study of 18 patients with a mean 9.5-year follow-up. Orthop Traumatol Surg Res, 2017, 103(5): 747-753.
18. Borzunov DY, Chevardin AY, Mitrofanov AI. Management of congenital pseudarthrosis of the tibia with the Ilizarov method in a paediatric population: influence of aetiological factors. Int Orthop, 2016, 40(2): 331-339.
19. Solla F, Lemoine J, Musoff C, et al. Surgical treatment of congenital pseudarthrosis of the forearm: Review and quantitative analysis of individual patient data. Hand Surg Rehabil, 2019, 38(4): 233-241.
20. Zayda AI, Mesregah MK, Zalalo SH, et al. Functional and radiological outcomes after treatment of congenital pseudarthrosis of the tibia using the Ilizarov technique: a retrospective single-center study. J Orthop Traumatol, 2022, 23(1): 48. doi: 10.1186/s10195-022-00667-2.
21. Inan M, El Rassi G, Riddle EC, et al. Residual deformities following successful initial bone union in congenital pseudoarthrosis of the tibia. J Pediatr Orthop, 2006, 26(3): 393-399.

Chinese Journal of Reparative and Reconstructive Surgery

Clinical study of Cross-Union surgery for treatment of pseudarthrosis of tibia with neurofibromatosis type 1 in children

Abstract Full text Figures/Tables Video References Cited by

Previous Article

Next Article

Format

Content