Modern approaches to surgical treatment of children with osteogenesis imperfecta

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Abstract

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.

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About the authors

Ekaterina N. Solodovnikova

National Medical Research Center of Children's Health

Email: katakrylova0701@gmail.com
ORCID iD: 0000-0002-8519-1445

Graduate Student, National Medical Research Center of Children's Health

Russian Federation, Moscow

Konstantin V. Zherdev

National Medical Research Center of Children's Health

Email: drzherdev@mail.ru
ORCID iD: 0000-0003-3698-6011

D. Sci. (Med.), National Medical Research Center of Children's Health

Russian Federation, Moscow

Oleg B. Chelpachenko

National Medical Research Center of Children's Health

Author for correspondence.
Email: Chelpachenko81@mail.ru
ORCID iD: 0000-0002-0333-3105

D. Sci. (Med.), National Medical Research Center of Children's Health

Russian Federation, Moscow

Anna A. Ovechkina

National Medical Research Center of Children's Health

Email: asha83@yandex.ru

Graduate Student, National Medical Research Center of Children's Health

Russian Federation, Moscow

Sergey P. Yatsyk

National Medical Research Center of Children's Health

Email: makadamia@yandex.ru
ORCID iD: 0000-0001-6966-1040

D. Sci. (Med.), Prof., Corr. Memb. RAS. National Medical Research Center of Children's Health

Russian Federation, Moscow

Igor V. Timofeev

National Medical Research Center of Children's Health

Email: doktor_timofeev@mail.ru
ORCID iD: 0000-0002-4662-2089

Cand. Sci. (Med.), National Medical Research Center of Children's Health

Russian Federation, Moscow

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Supplementary files

Supplementary Files
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1. Fig. 1. Schemes of osteotomies of the limb long bones proposed by N.A. Bogoraz (1925) [19].

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2. Fig. 2. Technique proposed by H. Sofield and E. Millar [22].

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3. Fig. 3. Surgical treatment using TEN: a – osteosynthesis of the femur TEN; b – TEN slip disorder [29].

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4. Fig. 4. Telescopic FD system [36].

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