Combination of flexible intramedullary nailing and Ilizarov frame for salvage of femur and humerus nonunion in a girl with osteogenesis imperfecta

Background Fixation of pathological long bones with telescopic intramedullary rods is well known to be a technically challenging procedure even in specialist centres, with a high complication rate due to rod migration, hardware failure, nonunion or malunion. However there is very little guidance in the literature regarding salvage treatment options when failure occurs.

Aim We demonstrate a surgical technique that can be used for salvage treatment of both femoral and humeral complex nonunions following Fassier-Duval (FD) rodding in a child with osteogenesis imperfecta (OI).

Case description A 13 year-old girl with OI type VIII presented sequentially with nonunion and deformity of the femur then the humerus following previous FD rods in those segments. The femur was also complicated with metallosis between the steel rod and an overlying titanium plate. Both segments were treated with pseudarthrosis debridement, removal of metalwork and stabilisation with hydroxyapatite (HA)-coated flexible intramedullary nails, with temporary Ilizarov frame to provide enough longitudinal and rotational stability to allow immediate weight-bearing. The femur Ilizarov frame was removed after 64 days, and the femur remained straight and fully healed at 2.5 years. The frame time for the humerus was 40 days, complete union was achieved and upper limb function restored and maintained at 9 months.

Discussion The transphyseal telescopic rod is the traditional implant of choice in terms of treating fractures and stabilising osteotomies for deformity in OI. However, it does not provide enough torsional or longitudinal stability by itself to allow early weight-bearing which is detrimental to bone healing in this vulnerable patient group. The incidence of delayed union or nonunion at osteotomy site in telescopic rod application is not negligible: up to 14.5-51.5 %. Although the technique we have shown in this case may not be applied to all complex OI patients, we believe that the combination of flexible intramedullary nails and Ilizarov frame provides a favourable environment for bone healing in complex or revision cases. As a secondary learning point the initial revision surgery to the left femur demonstrated the perils of using a steel rod and a titanium plate in a biologically active environment which in this case lead to metallosis and lysis.

Conclusion We found the technique of HA-coated flexible intramedullary nails combined with the Ilizarov frame effective in the salvage of failed telescopic rods in both femur and humerus and feel this technique can be used as a salvage option in similar cases worldwide. This case also demonstrates the perils of using different metals in combined internal fixation.

1. Sillence DO, Senn A, Danks DM. Genetic heterogeneity in osteogenesis imperfecta. J Med Genet. 1979;16(2):101-116. doi: 10.1136/jmg.16.2.101

2. Cheung MS, Glorieux FH. Osteogenesis Imperfecta: update on presentation and management. Rev Endocr Metab Disord. 2008;9(2):153-160. doi: 10.1007/s11154-008-9074-4

3. Van Dijk FS, Pals G, Van Rijn RR, et al. Classification of Osteogenesis Imperfecta revisited. Eur J Med Genet. 2010;53(1):1-5. doi: 10.1016/j.ejmg.2009.10.007

4. Glorieux FH. Osteogenesis imperfecta. Best Pract Res Clin Rheumatol. 2008;22(1):85-100. doi: 10.1016/j.berh.2007.12.012

5. Rodriguez Celin M, Kruger KM, Caudill A, et al. A Multicenter Study of Intramedullary Rodding in Osteogenesis Imperfecta. JB JS Open Access. 2020;5(3):e20.00031. doi: 10.2106/JBJS.OA.20.00031

6. Birke O, Davies N, Latimer M, et al. Experience with the Fassier-Duval telescopic rod: first 24 consecutive cases with a minimum of 1-year follow up. J Pediatr Orthop. 2011;31(4):458-464. doi: 10.1097/BPO.0b013e31821bfb50

7. Fassier F. Fassier-Duval Telescopic System: How I Do It? J Pediatr Orthop. 2017;37 Suppl 2:S48-S51. doi: 10.1097/BPO.0000000000001024

8. Sułko J, Oberc A. Advantages and Complications Following Fassier-Duval Intramedullary Rodding in Children. Pilot Study. Ortop Traumatol Rehabil. 2015;17(5):523-30. doi: 10.5604/15093492.1186830

9. Fassier FR. Osteogenesis Imperfecta-Who Needs Rodding Surgery? Curr Osteoporos Rep. 2021;19(3):264-270. doi: 10.1007/s11914-021-00665-z

10. Nicolaou N, Bowe JD, Wilkinson JM, et al. Use of the Sheffield telescopic intramedullary rod system for the management of osteogenesis imperfecta: clinical outcomes at an average follow-up of nineteen years. J Bone Joint Surg Am. 2011;93(21):1994-2000. doi: 10.2106/JBJS.J.01893

11. Shin CH, Lee DJ, Yoo WJ, et al. Dual Interlocking Telescopic Rod Provides Effective Tibial Stabilization in Children With Osteogenesis Imperfecta. Clin Orthop Relat Res. 2018;476(11):2238-2246. doi: 10.1097/CORR.0000000000000429

12. Popkov D, Dolganova T, Mingazov E, et al. Combined technique of titanium telescopic rods and external fixation in osteogenesis imperfecta patients: First 12 consecutive cases. J Orthop. 2020;22:316-325. doi: 10.1016/j.jor.2020.05.017

13. Mingazov ER, Gofman FF, Popkov AV, et al. First use experience with titanium telescopic rod in pediatric limb deformity correction in osteogenesis imperfecta. Genij Ortopedii. 2019;25(3):297-303. doi: 10.18019/1028-4427-2019-25-3-297-303

14. Sterian A, Balanescu R, Barbilian A, et al. Early telescopic rod osteosynthesis for Osteogenesis Imperfecta patients. J Med Life. 2015;8(4):544-547.

15. Cho TJ, Lee K, Oh CW, et al. Locking plate placement with unicortical screw fixation adjunctive to intramedullary rodding in long bones of patients with osteogenesis imperfecta. J Bone Joint Surg Am. 2015;97(9):733-737. doi: 10.2106/JBJS.N.01185

16. Azzam KA, Rush ET, Burke BR, et al. Mid-term Results of Femoral and Tibial Osteotomies and Fassier-Duval Nailing in Children With Osteogenesis Imperfecta. J Pediatr Orthop. 2018;38(6):331-336. doi: 10.1097/BPO.0000000000000824

17. Munns CF, Rauch F, Zeitlin L, et al. Delayed osteotomy but not fracture healing in pediatric osteogenesis imperfecta patients receiving pamidronate. J Bone Miner Res. 2004;19(11):1779-1786. doi: 10.1359/JBMR.040814

18. Popkov D. Use of flexible intramedullary nailing in combination with an external fixator for a postoperative defect and pseudarthrosis of femur in a girl with osteogenesis imperfecta type VIII: a case report. Strategies Trauma Limb Reconstr. 2018;13(3):191-197. doi: 10.1007/s11751-018-0320-3

19. Grossman LS, Price AL, Rush ET, et al. Initial Experience With Percutaneous IM Rodding of the Humeri in Children With Osteogenesis Imperfecta. J Pediatr Orthop. 2018;38(9):484-489. doi: 10.1097/BPO.0000000000000856

20. Hsiao MS, Mormino MA, Esposito PW, Burke BA. Distal humerus atrophic nonunion in a child with osteogenesis imperfecta. J Pediatr Orthop. 2013;33(7):725-729. doi: 10.1097/BPO.0b013e3182a32e69

21. Langlais T, Pannier S, De Tienda M, et al. 'In-Out-In' K-wires sliding in severe tibial deformities of osteogenesis imperfecta: a technical note. J Pediatr Orthop B. 2021;30(3):257-263. doi: 10.1097/BPB.0000000000000785

22. Fassier A. Telescopic rodding in children: Technical progression from Dubow-Bailey to Fassier-Duval™. Orthop Traumatol Surg Res. 2021;107(1S):102759. doi: 10.1016/j.otsr.2020.102759

23. Zeitlin L, Fassier F, Glorieux FH. Modern approach to children with osteogenesis imperfecta. J Pediatr Orthop B. 2003;12(2):77-87. doi: 10.1097/01. bpb.0000049567.52224.fa

24. Esposito P, Plotkin H. Surgical treatment of osteogenesis imperfecta: current concepts. Curr Opin Pediatr. 2008;20(1):52-57. doi: 10.1097/ MOP.0b013e3282f35f03

25. Cox I, Al Mouazzen L, Bleibleh S, et al. Combined two-centre experience of single-entry telescopic rods identifies characteristic modes of failure. Bone Joint J. 2020;102-B(8):1048-1055. doi: 10.1302/0301-620X.102B8.BJJ-2020-0131.R1

26. Rosemberg DL, Goiano EO, Akkari M, Santili C. Effects of a telescopic intramedullary rod for treating patients with osteogenesis imperfecta of the femur. J Child Orthop. 2018;12(1):97-103. doi: 10.1302/1863-2548.12.170009

27. Franzone JM, Kruse RW. Intramedullary nailing with supplemental plate and screw fixation of long bones of patients with osteogenesis imperfecta: operative technique and preliminary results. J Pediatr Orthop B. 2018;27(4):344-349. doi: 10.1097/BPB.0000000000000405

28. Boutaud B, Laville JM. Elastic sliding central medullary nailing with osteogenesis imperfecta. Fourteen cases at eight years follow-up. Rev Chir Orthop Reparatrice Appar Mot. 2004;90(4):304-311. (In French) doi: 10.1016/s0035-1040(04)70125-7

29. Mingazov ER, Popkov AV, Kononovich NA, et al. Results of using transphyseal elastic intramedullary nailing in patients with severe types of osteogenesis imperfecta. Genij Ortopedii. 2016;22(4):6-16.

30. Eliaz N. Corrosion of Metallic Biomaterials: A Review. Materials (Basel). 2019;12(3):407. doi: 10.3390/ma12030407

31. Zartman KC, Berlet GC, Hyer CF, Woodard JR. Combining dissimilar metals in orthopaedic implants: revisited. Foot Ankle Spec. 2011;4(5):318- 323. doi: 10.1177/1938640011422597