The results of the use of the telescopic intramedullary system in the surgical treatment of patients with osteogenesis imperfecta types I and III

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Background. The main orthopedic manifestation of osteogenesis imperfecta is multiple fractures. In severe disease, frequent fractures lead to malunion and deformity, which increases the risk of refracture. Surgical treatment of children with osteogenesis imperfecta is aimed at reducing the risk of complications and improving the quality of life of the child. The Fassier-Duval telescopic intramedullary system has taken the leading place among the “growing” metal fixators. In this study, we conduct a comparative analysis of the results of a longitudinal study between patients with osteogenesis imperfecta types I and III, as well as an assessment of motor activity.

Aim. To evaluate the efficiency of using the telescopic intramedullary system depending on the type of osteogenesis imperfecta.

Materials and methods. A prospective analysis of surgical treatment of 20 children with types I and III was carried out. Group I included 8 patients with type I NO, group II included 12 with type III. The average age of patients was 8.2 years. Surgical treatment was carried out on the basis of the neuroorthopedic department with orthopedics of the National Medical Research Center for Children's Health. A total of 48 intramedullary telescopic pins were installed. The average period of postoperative dynamic follow-up was 42 months. The analysis of the results of surgical treatment was carried out according to the following criteria: the frequency of migrations of metal structures, the formation of bone deformities, the number of revisions, as well as the number of bone fractures with an installed metal fixator. Evaluation of the results of motor activity was carried out using two scales: Gillette Functional Assesment Questionnaire (Gillette FAQ) and Hoffer–Bullock.

Results. There were no statistical differences in the number of complications in the two groups. Fractures among patients with type I occurred more often than in patients with type III by 19.3%. The frequency of migrations in the I study group was 7.1% (1 segment), in the II group 35.3% (12 segments). The formation of deformation was the main factor in the revision intervention, which in our case amounted to 83.3%. In the group of patients with type I, the number of repeated operations was 7.1% (1 segment in 1 patient), in the group of patients with type III – 17.6% (6 segments in 4 patients). All patients in the postoperative period had II or I level of motor capabilities on the Hoffer–Bullock scale. According to the Gillette FAQ scale in the postoperative period, the subjects showed a result above 4 points.

Conclusion. The Fassier-Duval telescopic rod is a modern and reliable metal fixator. Despite the absence of statistical differences in the two study groups (p-value2<0.005), there was an increase in the tendency to complications in severe course with NO. Correction of varus deformation when restoring normal values of DN>125° and HEA within 25–38° is the prevention of the re-formation of the truth of Coxa Vara. The motor activity of children with severe course did not statistically differ from children with mild course (p-value2>0.005) in the postoperative period with an average follow-up period of 42 months, regardless of the initial low motor capabilities in children with type III.

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About the authors

Katerina N. Solodovnikova

National Medical Research Center for Children's Health

Author for correspondence.
ORCID iD: 0000-0002-8519-1445

Graduate Student

Russian Federation, Moscow

Konstantin V. Zherdev

National Medical Research Center for Children's Health; Sechenov First Moscow State Medical University (Sechenov University)

ORCID iD: 0000-0003-3698-6011

D. Sci. (Med.)

Russian Federation, Moscow; Moscow

Ivan P. Pimburskiy

National Medical Research Center for Children's Health

ORCID iD: 0009-0002-5274-3941


Russian Federation, Moscow

Oleg B. Сhelpachenko

National Medical Research Center for Children's Health

ORCID iD: 0000-0002-0333-3105

D. Sci. (Med.)

Russian Federation, Moscow

Margarita A. Soloshenko

Petrovsky National Research Centre of Surgery

ORCID iD: 0000-0002-6150-0880

Cand. Sci. (Med.)

Russian Federation, Moscow

Sergey P. Yatsyk

National Medical Research Center for Children's Health

ORCID iD: 0000-0001-6966-1040

D. Sci. (Med.), Prof., Corr. Memb. RAS

Russian Federation, Moscow

Andrey S. Butenko

National Medical Research Center for Children's Health


Surgeon, Traumatologist-Orthopedic

Russian Federation, Moscow

Igor V. Timofeev

National Medical Research Center for Children's Health

ORCID iD: 0000-0002-4662-2089

Cand. Sci. (Med.), Assoc. Prof.

Russian Federation, Moscow


  1. Игнатович О.Н., Намазова-Баранова Л.С., Маргиева Т.В., и др. Несовершенный остеогенез: особенности диагностики. Педиатрическая фармакология. 2018;15(3):224-32 [Ignatovich ON, Namazova-Baranova LS, Мargieva ТV, et al. Osteogenesis Imperfecta: Diagnostic Feature. Pediatric pharmacology. 2018;15(3):224-32 (in Russian)]. doi: 10.15690/pf.v15i3.1902
  2. Oduah G, Firth G, Pettifor JM, Thandrayen K. Management of osteogenesis imperfecta at the Chris Hani Baragwanath Hospital. SAOJ. 2017;16(2):19-25.
  3. Van Dijk FS, Sillence DO. Osteogenesis imperfecta: Clinical diagnosis, nomenclature, and severity assessment. Am J Med Genet A. 2014;164A(6):1470-81. doi: 10.1002/ajmg.a.36545. Erratum in: Am J Med Genet A. 2015;167A(5):1178.
  4. Thomas IH, DiMeglio LA. Advances in the classification and treatment of osteogenesis imperfecta. Curr Osteoporos Rep. 2016;14(1):1-9.
  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. Lin HY, Lin SP, Chuang CK, et al. Incidence of the mucopolysaccharidoses in Taiwan, 1984-2004. Am J Med Genet A. 2009;149A(5):960-4. doi: 10.1002/ajmg.a.32781
  7. Бурцев М.Е., Фролов А.В., Логвинов А.Н., и др. Современный подход к диагностике и лечение детей с несовершенным остеогенезом. Ортопедия, травматология и восстановительная хирургия детского возраста. 2019;7(2):87-102 [Burtsev ME, Frolov AV, Logvinov AN, et al. Current approach to diagnosis and treatment of children with osteogenesis imperfecta. Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2019;7(2):87-102 (in Russian)]. doi: 10.17816/PTORS7287-102
  8. Hefny H, Elmoatasem EM, Nassar W. Valgus osteotomy by external fixation for treatment for developmental coxa vara. Strategies Trauma Limb Reconstr. 2013;8(3):161-7. doi: 10.1007/s11751-013-0178-3
  9. Hoffer MM, Bullock M. The functional and social significance of orthopedic rehabilitation of mentally retarded patients with cerebral palsy. Orthop Clin North Am. 1981;12(1):185-91.
  10. Novachek TF, Stout JL, Tervo R. Reliability and validity of the Gillette Functional Assessment Questionnaire as an outcome measure in children with walking disabilities. J Pediatr Orthop. 2000;20(1):75-81.
  11. Joseph B, Rebello G, Kant CB. The choice of intramedullary devices for the femur and the tibia in osteogenesis imperfecta. J Pediatr Orthop B. 2005;14(5):311-9.
  12. Hidalgo Perea S, Green DW. Osteogenesis imperfecta: treatment and surgical management. Curr Opin Pediatr. 2021;33(1):74-8. doi: 10.1097/MOP.0000000000000968
  13. 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):331-6.
  14. Musielak BJ, Woźniak Ł, Sułko J, et al. Problems, Complications, and Factors Predisposing to Failure of Fassier-Duval Rodding in Children With Osteogenesis Imperfecta: A Double-center Study. J Pediatr Orthop. 2021;41(4):e347-52. doi: 10.1097/BPO.0000000000001763
  15. Lee RJ, Paloski MD, Sponseller PD, Leet AI. Bent Telescopic Rods in Patients With Osteogenesis Imperfecta. J Pediatr Orthop. 2016;36(6):656-60. doi: 10.1097/BPO.0000000000000509
  16. Sterian AG, Ulici A. Revision Rates for Osteogenesis Imperfecta Patients Treated with Telescopic Nails. A follow-up Study After a 7-year Experience. J Med Life. 2020;13(4):543-7. doi: 10.25122/jml-2020-0161
  17. Wirth T. The orthopaedic management of long bone deformities in genetically and acquired generalized bone weakening conditions. J Child Orthop. 2019;13(1):12-21. doi: 10.1302/1863-2548.13.180184
  18. Kaur S, Kulkarni KP, Kochar IS, Narasimhan R. Management of lower limb deformities in children with osteogenesis imperfecta. Indian Pediatr. 2011;48(8):637-9. doi: 10.1007/s13312-011-0103-0

Supplementary files

Supplementary Files
1. Fig. 1. Bilateral true Coxa Vara in a patient with type III OI.

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2. Fig. 2. Data on motor activity in the preoperative and postoperative periods assessed using the Hoffer–Bullosk scale (Y axis is the number of patients).

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