Ilizarov Distraction for Congenital Pseudoarthrosis of the Tibia in Adults

Abstract

Objective: The clinical evidence on the management for congenital pseudoarthrosis of the tibia (CPT) in adults is limited. The aim of this study is to assess the functional and radiological outcomes of Ilizarov distraction for treating CPT in adults.

Methods: A retrospective analysis was conducted. Between 2013 and 2022, an Ilizarov distraction technique was performed on 14 adults (14 limbs) with CPT in our limb deformity center. There were seven females and seven males with a mean age of 33.7 (range, 18 ∼ 53) years. The diagnosis of NF-1 was confirmed in seven (50.0%) patients. Eight patients had a history of previous surgical failure. The pseudoarthrosis occurred in the middle and lower tibia in all limbs (six left and eight right). The CPT was classified by Crawford classification and Paley classification. The surgical procedures, external fixation time (EFT), and all outcomes and complications were recorded. The Kolmogorov–Smirnov test was performed to test the normality of the data. The American Orthopedic Foot and Ankle Society (AOFAS) ankle-hindfoot score at the preoperative and final follow-up was compared by using the Wilcoxon’s signed-rank test. The limb-length discrepancy (LLD) and a self-made exercise capacity score at the preoperative and final follow-up were compared by using the student’s t-test. The clinical and radiological outcomes were assessed by the Inan scale.

Results: The mean EFT of Ilizarov fixator was 19.5 months (range, 7.3 ∼ 39.1). At a median follow-up of 26.8 months (IQR, 20.2 ∼ 34.3), bone union of the pseudarthrosis and consolidation of the distraction zone were achieved in all patients. The mean LLD was decreased from 11.3 cm (range, 3.4 ∼ 17.3) preoperatively to 1.1 cm (range, 0.3 ∼ 3.7) (p < 0.05). The mean or median AOFAS ankle-hindfoot score was improved from 53.5 (IQR, 26.5 ∼ 60.5) preoperatively to 63.9 (range, 53 to 73) at final follow-up (p < 0.05). The mean score for exercise capacity were improved from 4.9 (range, 1 to 8) preoperatively to 9.6 (range, 7 ∼ 12) at final follow-up (p < 0.05). According to the criteria described by Inan et al., the clinical results were classified as good in 10 and fair in 4, while the radiological results were classified as excellent in three, good in 8, and fair in 2. The success rate was 92.9%, as refracture was defined as treatment failure and occurred in one patient.

Conclusion: Ilizarov distraction provided a suitable treatment option for the CPT in adults, as it could achieve a high rate of bone union, a good correction of secondary deformity, a low risk of refracture, and consequently restore a relatively functional limb.

Keywords

Bone union / Congenital pseudoarthrosis of the tibia / Deformity correction / Ilizarov distraction

References

[1] Shah H, Rousset M, Canavese F. Congenital pseudarthrosis of the tibia: management and complications. Indian J Orthop. 2012; 46: 616–626.

[2] Ari B, Kuyubasi SN. Bilateral congenital pseudarthrosis of the tibia with neurofibromatosis type 1. J Pak Med Assoc. 2021; 71: 1499–1502.

[3] Hefti F, Bollini G, Dungl P, Fixsen J, Grill F, Ippolito E, et al. Congenital pseudarthrosis of the tibia: history, etiology, classification, and epidemiologic data. J Pediatr Orthop B. 2000; 9: 11–15.

[4] Choi IH, Cho TJ, Moon HJ. Ilizarov treatment of congenital pseudarthrosis of the tibia: a multi-targeted approach using the Ilizarov technique. Clin Orthop Surg. 2011; 3: 1–8.

[5] Cho TJ, Seo JB, Lee HR, Yoo WJ, Chung CY, Choi IH. Biologic characteristics of fibrous hamartoma from congenital pseudarthrosis of the tibia associated with neurofibromatosis type 1. J Bone Joint Surg Am. 2008; 90: 2735–2744.

[6] Madhuri V, Mathew SE, Rajagopal K, Ramesh S, Antonisamy B. Does pamidronate enhance the osteogenesis in mesenchymal stem cells derived from fibrous hamartoma in congenital pseudarthrosis of the tibia? Bone Rep. 2016; 5: 292–298.

[7] Li Z, Liu Y, Huang Y, Tan Q, Mei H, Zhu G, et al. Circ_0000888 regulates osteogenic differentiation of periosteal mesenchymal stem cells in congenital pseudarthrosis of the tibia. iScience. 2023; 26: 107923.

[8] Granchi D, Devescovi V, Baglio SR, Magnani M, Donzelli O, Baldini N. A regenerative approach for bone repair in congenital pseudarthrosis of the tibia associated or not associated with type 1 neurofibromatosis: correlation between laboratory findings and clinical outcome. Cytotherapy. 2012; 14: 306–314.

[9] Shannon CE, Huser AJ, Paley D. Cross-union surgery for congenital pseudarthrosis of the tibia. Children (Basel). 2021; 8: 547.

[10] Liu YX, Yang G, Zhu GH, Tan Q, Wu JY, Liu K, et al. Application of the “telescopic rod” in a combined surgical technique for the treatment of congenital pseudarthrosis of the tibia in children. J Orthop Surg Res. 2021; 16: 532.

[11] Zhong H, Ma S, Cen Y, Ma L, Li D, Liang B, et al. A case report of early unilateral external fixation by 3D printing and computer-assisted and secondary bone graft internal fixation in pseudarthrosis of the tibia surgery. J Int Med Res. 2020; 48: 300060520945518.

[12] McClure PK, Franzone JM, Herzenberg JE. Challenges with Fassier-Duval rod exchanges in congenital pseudarthrosis of the tibia: explant roadblock and solution. J Pediatr Orthop B. 2022; 31: e95–e100.

[13] Paley D. Congenital pseudarthrosis of the tibia: biological and biomechanical considerations to achieve union and prevent refracture. J Child Orthop. 2019; 13: 120–133.

[14] Vander Have KL, Hensinger RN, Caird M, Johnston C, Farley FA. Congenital pseudarthrosis of the tibia. J Am Acad Orthop Surg. 2008; 16: 228–236.

[15] Kumta SM, Spinner R, Hung LK, Leung PC. Congenital pseudarthrosis of the tibia in adults treated by a free vascularized iliac crest graft. Microsurgery. 1994; 15: 598–603.

[16] Miraj F, Aprilya D. Diagnostic and treatment challenge in adult presentation of congenital pseudoarthrosis of the tibia: a case report. Ann Med Surg (Lond). 2020; 58: 112–116.

[17] Bhowmick K, Varghese VD. Retrograde intramedullary nailing for recurrent fracture in congenital pseudarthrosis of the tibia. J Foot Ankle Surg. 2016; 55: 1287–1291.

[18] El-Gammal TA, El-Sayed A, Kotb MM, Saleh WR, Ragheb YF, Refai OA, et al. Crawford type IV congenital pseudarthrosis of the tibia: treatment with vascularized fibular grafting and outcome at skeletal maturity. J Pediatr Orthop. 2021; 41: 164–170.

[19] Westberry DE, Carpenter AM, Tisch J, Wack LI. Amputation outcomes in congenital pseudarthrosis of the tibia. J Pediatr Orthop. 2018; 38: e475–e481.

[20] O’Donnell C, Foster J, Mooney R, Beebe C, Donaldson N, Heare T. Congenital pseudarthrosis of the tibia. JBJS Rev. 2017; 5: e3.

[21] McCarthy RE. Amputation for congenital pseudarthrosis of the tibia. Indications and techniques. Clin Orthop Relat Res. 1982; 166: 58–61.

[22] Khan T, Joseph B. Controversies in the management of congenital pseudarthrosis of the tibia and fibula. Bone Joint J. 2013; 95-B: 1027–1034.

[23] Zayda AI, Mesregah MK, Zalalo SH, Sakr SA. 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: 48.

[24] Li Y, Chen Y, Gan T, Qin B, Liu X, Zhang H. An alternative therapeutic strategy for infected large bone defect and massive soft-tissue loss of leg-is free flap reconstruction inevitable? Int Orthop. 2021; 45: 3033–3043.

[25] Ilizarov GA, Gracheva VI. Bloodless treatment of congenital pseudarthrosis of the crus with simultaneous elimination of shortening using dosed distraction. Ortop Travmatol Protez. 1971; 32: 42–46.

[26] Ilizarov GA. Clinical application of the tension-stress effect for limb lengthening. Clin Orthop Relat Res. 1990; 250: 8–26.

[27] Paley D, Catagni M, Argnani F, Prevot J, Bell D, Armstrong P. Treatment of congenital pseudoarthrosis of the tibia using the Ilizarov technique. Clin Orthop Relat Res. 1992; 280: 81–93.

[28] Crawford AH Jr, Bagamery N. Osseous manifestations of neurofibromatosis in childhood. J Pediatr Orthop. 1986; 6: 72–88.

[29] Kitaoka HB, Alexander IJ, Adelaar RS, Nunley JA, Myerson MS, Sanders M. Clinical rating systems for the ankle-hindfoot, midfoot, hallux, and lesser toes. Foot Ankle Int. 1994; 15: 349–353.

[30] Inan M, El Rassi G, Riddle EC, Kumar SJ. Residual deformities following successful initial bone union in congenital pseudoarthrosis of the tibia. J Pediatr Orthop. 2006; 26: 393–399.

[31] El-Gammal TA, Ali AE, Kotb MM, Saleh WR, Ragheb YF, Refai OA, et al. Congenital pseudarthrosis of the tibia: long-term outcome of treatment with intramedullary vascularized fibular graft combined with Ilizarov distraction. J Pediatr Orthop. 2023; 43: e487–e492.

[32] Ilizarov GA. The tension-stress effect on the genesis and growth of tissues. Part I. The influence of stability of fixation and soft-tissue preservation. Clin Orthop Relat Res. 1989; 238: 249–281.

[33] Ilizarov GA. The tension-stress effect on the genesis and growth of tissues: part II. The influence of the rate and frequency of distraction. Clin Orthop Relat Res. 1989; 239: 263–285.

[34] Seo SG, Lee DY, Kim YS, Yoo WJ, Cho TJ, Choi IH. Foot and ankle function at maturity after ilizarov treatment for atrophic-type congenital pseudarthrosis of the tibia: a comprehensive outcome comparison with normal controls. J Bone Joint Surg Am. 2016; 98: 490–498.

[35] Shabtai L, Ezra E, Wientroub S, Segev E. Congenital tibial pseudarthrosis, changes in treatment protocol. J Pediatr Orthop B. 2015; 24: 444–449.

[36] Ghanem I, Damsin JP, Carlioz H. Ilizarov technique in the treatment of congenital pseudarthrosis of the tibia. J Pediatr Orthop. 1997; 17: 685–690.

[37] El-Rosasy M. Ilizarov techniques for the management of congenital pseudarthrosis of the tibia. Clin Orthop Surg. 2001; 3: 1–8.

[38] Boero S, Catagni M, Donzelli O, Facchini R, Frediani PV. Congenital pseudarthrosis of the tibia associated with neurofibromatosis-1: treatment with Ilizarov’s device. J Pediatr Orthop. 1997; 17: 675–684.

[39] 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: 331–339.

[40] Hissnauer TN, Stiel N, Babin K, Rupprecht M, Hoffmann M, Rueger JM, et al. Bone morphogenetic protein-2 for the treatment of congenital pseudarthrosis of the tibia or persistent tibial nonunion in children and adolescents: a retrospective study with a minimum 2-year follow-up. J Mater Sci Mater Med. 2017; 28: 60.

[41] Ohnishi I, Sato W, Matsuyama J, Yajima H, Haga N, Kamegaya M, et al. Treatment of congenital pseudarthrosis of the tibia: a multicenter study in Japan. J Pediatr Orthop. 2005; 25: 219–224.

[42] Thabet AM, Paley D, Kocaoglu M, Eralp L, Herzenberg JE, Ergin ON. Periosteal grafting for congenital pseudarthrosis of the tibia: a preliminary report. Clin Orthop Relat Res. 2008; 466: 2981–2994.

[43] Agashe MV, Song SH, Refai MA, Park KW, Song HR. Congenital pseudarthrosis of the tibia treated with a combination of Ilizarov’s technique and intramedullary rodding. Acta Orthop. 2012; 83: 515–522.

[44] Yan A, Mei HB, Liu K, Wu JY, Tang J, Zhu GH, et al. Wrapping grafting for congenital pseudarthrosis of the tibia: a preliminary report. Medicine (Baltimore). 2017; 96: e8835.

[45] Zhu GH, Mei HB, He RG, Liu YX, Liu K, Tang J, et al. Combination of intramedullary rod, wrapping bone grafting and Ilizarov’s fixator for the treatment of Crawford type IV congenital pseudarthrosis of the tibia: mid-term follow up of 56 cases. BMC Musculoskelet Disord. 2016; 17: 443.

[46] Lan KC, Wei KT, Lin PW, Lin CC, Won PL, Liu YF, et al. Targeted activation of androgen receptor signaling in the periosteum improves bone fracture repair. Cell Death Dis. 2022; 13: 123.

[47] Dilogo IH, Mujadid F, Nurhayati RW, Kurniawan A. Evaluation of bone marrow-derived mesenchymal stem cell quality from patients with congenital pseudoarthrosis of the tibia. J Orthop Surg Res. 2018; 13: 266.

[48] Shah H, Joseph B, Nair BVS, Kotian DB, Choi IH, Richards BS, et al. What factors influence union and refracture of congenital pseudarthrosis of the tibia? a multicenter long-term study. J Pediatr Orthop. 2018; 38: e332–e337.

[49] Cho T-J, Choi IH, Lee SM, Chung CY, Yoo WJ, Lee DY, et al. Refracture after Ilizarov osteosynthesis in atrophic-type congenital pseudarthrosis of the tibia. J Bone Joint Surg Br. 2008; 90: 488–493.

[50] Singer D, Johnston CE. Congenital pseudarthrosis of the tibia: results, at skeletal maturity, of the Charnley-Williams procedure. JB JS Open Access. 2019; 4: e0004.

[51] Tudisco C, Bollini G, Dungl P, Fixen J, Grill F, Hefti F, et al. Functional results at the end of skeletal growth in 30 patients affected by congenital pseudoarthrosis of the tibia. J Pediatr Orthop B. 2000; 9: 94–102.

[52] Crossett LS, Beaty JH, Betz RR, Warner W, Clancy M, Steel HH. Congenital pseudarthrosis of the tibia. Long-term follow-up study. Clin Orthop Relat Res. 1989; 245: 16–18.

RIGHTS & PERMISSIONS

2024 The Author(s). Orthopaedic Surgery published by Tianjin Hospital and John Wiley & Sons Australia, Ltd.