Современные подходы к оперативному лечению детей с несовершенным остеогенезом

Солодовникова Е.Н., Жердев К.В., Челпаченко О.Б., Овечкина А.А., Яцык С.П., Тимофеев И.В. Современные подходы к оперативному лечению детей с несовершенным остеогенезом. Педиатрия. Consilium Medicum. 2021; 3: 265–270. DOI: 10.26442/26586630.2021.3.200896

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Solodovnikova EN, Zherdev KV, Сhelpachenko OB, Ovechkina AA, Yatsyk SP, Timofeev IV. Modern approaches to surgical treatment of children with osteogenesis imperfecta. Pediatrics. Consilium Medicum. 2021; 3: 265–270. DOI: 10.26442/26586630.2021.3.200896

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Аннотация

Несовершенный остеогенез (НО) – это гетерогенная группа заболеваний с разным типом наследования, характеризующаяся частыми переломами и деформациями костного скелета. Консервативное и хирургическое лечение детей с данной патологией – неотъемлемая часть мультидисциплинарного подхода. При хирургическом лечении деформаций костей у детей с НО методом выбора являются корригирующие остеотомии, а эффективным способом фиксации – интрамедуллярный остеосинтез. Хирургическое лечение детей с НО направлено на снижение количества переломов, коррекцию деформаций и увеличение двигательной активности. Цель работы: определить показания к проведению интрамедуллярного остеосинтеза длинных трубчатых костей у детей с заболеванием; провести обзор данных международных исследований на тему лечения детей с НО; рассмотреть различные типы металлофиксаторов, их преимущества и недостатки. На сегодняшний день методика интрамедуллярной фиксации является «золотым стандартом» при проведении хирургического лечения детей с НО. Существует явное преимущество телескопических штифтов с точки зрения частоты ревизионных операций, но их применение не исключает наличия послеоперационных осложнений.

Ключевые слова: обзор, несовершенный остеогенез, бисфосфонаты, остеотомия, интрамедуллярный остеосинтез, титановый эластический штифт, телескопическая металлоконструкция

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Osteogenesis imperfecta (OI) is a heterogeneous group of diseases with different types of inheritance, characterized by frequent fractures and deformities of the bone skeleton. Conservative and surgical treatment of children with this pathology is an integral part of a multidisciplinary approach. In the surgical treatment of bone deformities in children with OI, the method of choice is corrective osteotomies, and an effective method of fixation is intramedullary osteosynthesis. Surgical treatment of children with OI is aimed at reducing the number of fractures, correcting deformities and increasing motor activity. Aim of the study to determine the indications for intramedullary osteosynthesis of long tubular bones in children with the disease. To review the studies concerning treatment of children with OI. Consider the different types of metal fixers, their advantages and disadvantages. Today the technique of intramedullary fixation is the "gold" standard in the surgical treatment of children with OI. There is a clear advantage of telescopic rods in terms of the frequency of reoperations, but their use does not exclude the presence of postoperative complications.

Keywords: review, osteogenesis imperfecta, bisphosphonates, osteotomy, intramedullary osteosynthesis, titanium elastic pin, telescopic metal structure

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Список литературы

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23. Fassier FR. Osteogenesis imperfecta. Pediatric lower limb deformities. Springer. Cham, 2016; p. 255-65.
24. Imajima Y, Kitano M, Ueda T. Intramedullary fixation using Kirschner wires in children with osteogenesis imperfecta. J Pediatr Orthop. 2015;35(4):431-4. DOI:10.1097/bpo.0000000000000285
25. Scollan JP, Jauregui JJ, Jacobsen CM, Abzug JM. The Outcomes of Nonelongating Intramedullary Fixation of the Lower Extremity for Pediatric Osteogenesis Imperfecta Patients: A Meta-analysis. J Pediatr Orthop. 2017;37(5):e313-e316. DOI:10.1097/BPO.0000000000000970. PMID: 28277468
26. Persiani P, Ranaldi FM, Martini L, et al. Treatment of tibial deformities with the Fassier–Duval telescopic nail and minimally invasive percutaneous osteotomies in patients with osteogenesis imperfecta type III. J Pediatr Orthop B. 2019;28(2):179-85. DOI:10.1097/BPB.0000000000000536
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30. Bailey RV. Dubow HI. Studies of longitudinal bone growth resulting in an extensible nail. Surg Forum. 1963;14:455-8.
31. Marafioti RL, Westin GW. Elongating intramedullary rods in the treatment of osteogenesis imperfecta. J Bone Joint Surg Am. 1977;59(4):467-72.
32. Fassier A. Telescopic rodding in children: Technical progression from Dubow–Bailey to Fassier–Duval TM. Orthopaedics and Traumatology: Surgery and Research. 2020; p. 102759.
33. 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. JBJS. 2015;97(9):733-7.
34. 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.
35. Fassier F, Duval P. New concept for telescoping rodding in osteogenesis imperfecta: preliminary results. Proceedings of the Annual Meeting of the Pediatric Orthopaedic Society of North America (POSNA). Cancun, Mexico. 2001; p. 101.
36. Makhdom AM, Kishta W, Saran N, et al. Are Fassier–Duval rods at risk of migration in patients undergoing spine magnetic resonance imaging? J Pediatr Orthop. 2015;35(3):323-7.
37. Spahn KM, Mickel T, Carry PM, et al. Fassier–Duval rods are associated with superior probability of survival compared with static implants in a cohort of children with osteogenesis imperfecta deformities. J Pediatr Orthop. 2019;39(5):e392-e6.
38. Ruck J, Dahan-Oliel N, Montpetit K, et al. Fassier–Duval femoral rodding in children with osteogenesis imperfecta receiving bisphosphonates: functional outcomes at one year. J Child Orthop. 2011;5(3):217-24.
39. Ashby E, Montpetit K, Hamdy RC, Fassier F. Functional outcome of forearm rodding in children with osteogenesis imperfecta. J Pediatr Orthop. 2018;38(1):54-9. DOI:10.1097/BPO.0000000000000724
40. Addar A, Hamdy RC, Fassier F, et al. Femoral Fassier–Duval Rodding In Osteogenesis Imperfecta: Long-Term Results In 27 Patients With A Minimum Ten-Year Follow-Up. In Orthopaedic Proceedings. British Editorial Society of Bone and Joint Surgery. 2020;102(Suppl. 8): p. 82.
41. 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. PMID: 25929780
42. Montpetit K, Palomo T, Glorieux FH, et al. Multidisciplinary Treatment of Severe Osteogenesis Imperfecta: Functional Outcomes at Skeletal Maturity. Arch Phys Med Rehabil. 2015; 96(10):1834-9. DOI:10.1016/j.apmr.2015.06.006
43. Мингазов Э.Р., Чибиров Г.М., Попков Д.А. Ортопедические осложнения и ятрогении при коррекции деформаций нижних конечностей у пациентов, страдающих тяжелыми формами несовершенного остеогенеза. Гений ортопедии. 2018:24(2):168-76 [Mingazov ER, Chibirov GM, Popkov DA. Ortopedicheskie oslozhneniia i iatrogenii pri korrektsii deformatsii nizhnikh konechnostei u patsientov, stradaiushchikh tiazhelymi formami nesovershennogo osteogeneza. Genii ortopedii. 2018:24(2):168-76 (in Russian)]. DOI:10.18019/1028-4427-2018-24-2-168-176

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1. Burtsev ME, Frolov AV, Loginov AN, et al. Sovremennyi podkhod k diagnostike i lecheniiu detei s nesovershennym osteogenezom. Ortopediia, travmatologiia i vosstanovitelnaia khirurgiia detskogo vozrasta. 2019;7(Vyp. 2):87-102 (in Russian). DOI:10.17816/PTORS7287-102
2. Ignatovich ON, Namazova-Baranova LS, Margieva TV, et al. Nesovershennyi osteogenez: osobennosti diagnostiki. Pediatricheskaia farmakologiia. 2018;15(3):224-32 (in Russian). DOI:10.15690/pf.v15i3.1902
3. Fratzl-Zelman N, Misof BM, Roschger P, Klaushofer K. Classification of osteogenesis imperfecta. Wien Med Wochenschr. 2015;165(13-14):264-70. DOI:10.1007/s10354-015-0368-3
4. Chu ML, Williams CJ, Pepe G, et al. Internal deletion in a collagen gene in a perinatal lethal form of osteogenesis imperfecta. Nature. 1983;304(5921):78-80. DOI:10.1038/304078a0
5. Marini JC, Forlino A, Bächinger HP, et al. Osteogenesis imperfecta. Nat Rev Dis Prim. 2017;3(1):1-19.
6. Forlino A, Cabral WA, Barnes AM, Marini JC. New perspectives on osteogenesis imperfecta. Nat Rev Endocrinol. 2011;7(9):540-57. DOI:10.1038/nrendo.2011.81
7. 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
8. Palomo T, Vilaça T, Lazaretti-Castro M. Osteogenesis imperfecta: diagnosis and treatment. Curr Opin Endocrinol Diabetes Obes. 2017;24(6):381-8.
9. Tournis S, Dede AD. Osteogenesis imperfecta – a clinical update. Metabolism. 2018;80:27‑37.
10. Amberger J, Bocchini CA, Scott AF, Hamosh A. McKusick's Online Mendelian Inheritance in Man (OMIM). Nucleic Acids Res. 2009;37:D793-6. DOI:10.1093/nar/gkn665
11. Rauch F, Glorieux FH. Osteogenesis imperfecta. The Lancet. 2004;363(9418):1377-85.
12. Bacon S, Crowley R. Developments in rare bone diseases and mineral disorders. Ther Adv Chronic Dis. 2018;9(1):51-60.
13. Thomas IH, DiMeglio LA. Advances in the classification and treatment of osteogenesis imperfecta. Curr Osteoporos Rep. 2016;14(1):1-9.
14. 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
15. Fassier A. Telescopic rodding in children: Technical progression from Dubow–Bailey to Fassier–Duval TM. Orthop Traumatol Surg Res. 2021;107(1S):102759. DOI:10.1016/j.otsr.2020.102759
16. Kusumi K, Ayoob R, Bowden SA, et al. Beneficial effects of intravenous pamidronate treatment in children with osteogenesis imperfecta under 24 months of age. J Bone Miner Metab. 2015;33(5):560-8.
17. Skriabin EG, Komarova IV, Bukseev AN, et al. Vnutriutrobnye perelomy kostei skeleta u plodov s nesovershennym osteogenezom: obzor literatury i sobstvennoe klinicheskoe nabliudenie. Genii ortopedii. 2018;24(4):521-9 (in Russian). DOI:10.18019/1028-4427-2018-24-4-521-529
18. Mingazov ER, Gofman F.F., Popkov AV, et al. Pervyi opyt primeneniia titanovogo teleskopicheskogo sterzhnia pri korrektsii deformatsii konechnostei u detei s nesovershennym osteogenezom. Genii ortopedii. 2019;25(3):297-303 (in Russian). DOI:10.18019/1028-4427-2019-25-3-297-303
19. Bogoraz NA. About surgery for chondrodystrophy. Proceedings of the Rostov State Medical Institute. Rostov, 1940; p. 215-9 (in Russian).
20. Enright WJ, Noonan KJ. Bone plating in patients with type III osteogenesis imperfecta: results and complications. Iowa Orthop J. 2006;26:37-40.
21. Sofield HA, Millar EA. Fragmentation, realignment, and intramedullary rod fixation of deformities of the long bones in children: a ten-year appraisal. J Bone Joint Surg. 1959;41(8):1371-91.
22. Kozhakhmetov OA. Istoriia razvitiia vnutrikostnogo osteosinteza. Nauka i zdravookhranenie. 2012;2:68-70 (in Russian).
23. Fassier FR. Osteogenesis imperfecta. Pediatric lower limb deformities. Springer. Cham, 2016; p. 255-65.
24. Imajima Y, Kitano M, Ueda T. Intramedullary fixation using Kirschner wires in children with osteogenesis imperfecta. J Pediatr Orthop. 2015;35(4):431-4. DOI:10.1097/bpo.0000000000000285
25. Scollan JP, Jauregui JJ, Jacobsen CM, Abzug JM. The Outcomes of Nonelongating Intramedullary Fixation of the Lower Extremity for Pediatric Osteogenesis Imperfecta Patients: A Meta-analysis. J Pediatr Orthop. 2017;37(5):e313-e316. DOI:10.1097/BPO.0000000000000970. PMID: 28277468
26. Persiani P, Ranaldi FM, Martini L, et al. Treatment of tibial deformities with the Fassier–Duval telescopic nail and minimally invasive percutaneous osteotomies in patients with osteogenesis imperfecta type III. J Pediatr Orthop B. 2019;28(2):179-85. DOI:10.1097/BPB.0000000000000536
27. Lascombes P. Flexible intramedullary nailing in children: the Nancy University manual. Springer-Verlag, Berlin, Heidelberg, 2010.
28. Burtsev ME, Frolov AV, Logvinov AN, et al. Khirurgicheskoe lechenie oskolchatogo vnutrisustavnogo pereloma distalnoi treti bedrennoi kosti u patsienta s nesovershennym osteogenezom I tipa. Ortopediia, travmatologiia i vosstanovitel'naia khirurgiia detskogo vozrasta. 2019;7(1):87-96 (in Russian). DOI:10.17816/PTORS7187-96
29. Persiani P, Martini L, Ranaldi FM, et al. Elastic intramedullary nailing of the femur fracture in patients affected by osteogenesis imperfecta type 3: indications, limits and pitfalls. Injury. 2019;50(Suppl. 2):S52-S56. DOI:10.1016/j.injury.2019.01.045
30. Bailey RV. Dubow HI. Studies of longitudinal bone growth resulting in an extensible nail. Surg Forum. 1963;14:455-8.
31. Marafioti RL, Westin GW. Elongating intramedullary rods in the treatment of osteogenesis imperfecta. J Bone Joint Surg Am. 1977;59(4):467-72.
32. Fassier A. Telescopic rodding in children: Technical progression from Dubow–Bailey to Fassier–Duval TM. Orthopaedics and Traumatology: Surgery and Research. 2020; p. 102759.
33. 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. JBJS. 2015;97(9):733-7.
34. 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.
35. Fassier F, Duval P. New concept for telescoping rodding in osteogenesis imperfecta: preliminary results. Proceedings of the Annual Meeting of the Pediatric Orthopaedic Society of North America (POSNA). Cancun, Mexico. 2001; p. 101.
36. Makhdom AM, Kishta W, Saran N, et al. Are Fassier–Duval rods at risk of migration in patients undergoing spine magnetic resonance imaging? J Pediatr Orthop. 2015;35(3):323-7.
37. Spahn KM, Mickel T, Carry PM, et al. Fassier–Duval rods are associated with superior probability of survival compared with static implants in a cohort of children with osteogenesis imperfecta deformities. J Pediatr Orthop. 2019;39(5):e392-e6.
38. Ruck J, Dahan-Oliel N, Montpetit K, et al. Fassier–Duval femoral rodding in children with osteogenesis imperfecta receiving bisphosphonates: functional outcomes at one year. J Child Orthop. 2011;5(3):217-24.
39. Ashby E, Montpetit K, Hamdy RC, Fassier F. Functional outcome of forearm rodding in children with osteogenesis imperfecta. J Pediatr Orthop. 2018;38(1):54-9. DOI:10.1097/BPO.0000000000000724
40. Addar A, Hamdy RC, Fassier F, et al. Femoral Fassier–Duval Rodding In Osteogenesis Imperfecta: Long-Term Results In 27 Patients With A Minimum Ten-Year Follow-Up. In Orthopaedic Proceedings. British Editorial Society of Bone and Joint Surgery. 2020;102(Suppl. 8): p. 82.
41. 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. PMID: 25929780
42. Montpetit K, Palomo T, Glorieux FH, et al. Multidisciplinary Treatment of Severe Osteogenesis Imperfecta: Functional Outcomes at Skeletal Maturity. Arch Phys Med Rehabil. 2015; 96(10):1834-9. DOI:10.1016/j.apmr.2015.06.006
43. Mingazov ER, Chibirov GM, Popkov DA. Ortopedicheskie oslozhneniia i iatrogenii pri korrektsii deformatsii nizhnikh konechnostei u patsientov, stradaiushchikh tiazhelymi formami nesovershennogo osteogeneza. Genii ortopedii. 2018:24(2):168-76 (in Russian). DOI:10.18019/1028-4427-2018-24-2-168-176

Авторы

Е.Н. Солодовникова*, К.В. Жердев, О.Б. Челпаченко, А.А. Овечкина, С.П. Яцык, И.В. Тимофеев

ФГАУ «Национальный медицинский исследовательский центр здоровья детей» Минздрава России, Москва, Россия
*katakrylova0701@gmail

________________________________________________

Ekaterina N. Solodovnikova*, Konstantin V. Zherdev, Oleg B. Сhelpachenko, Anna A. Ovechkina, Sergey P. Yatsyk, Igor V. Timofeev

National Medical Research Center of Children's Health, Moscow, Russia
*katakrylova0701@gmail

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