Deformities of the spine and limbs in patients with Duchenne myodystrophy: clinical features, diagnosis and treatment. Interstate consensus protocol
S. O. Ryabykh,
P. V. Ochirova,
D. M. Savin,
A. N. Tretjakova,
D. A. Popkov,
T. V. Ryabykh,
Е. N. Shchurova,
M. S. Saifutdinov https://doi.org/10.14531/ss2020.1.61-77
Abstract
Objective. To develop an algorithm for the diagnosis and treatment of orthopedic syndrome in patients with Duchenne muscular dystrophy (DMD) based on an assessment of the evidence level of published data.
Material and Methods. Consensus is a version of the main foreign protocols adapted for use in post-Soviet countries (the basis of consensus is the TREAT NMD protocol: treatnmd.ncl.ac.uk/care/dmd/diagnosis-management-DMD), as well as of works systematized on the basis on evidence level and reflecting modern approaches to the diagnosis and rehabilitation (including surgical) of spinal and limb deformities in patients with Duchenne myodystrophy. The recommendations are based on literature data and the authors’ own experience. Search in electronic databases was performed on the Medline, Embase, Web of Science and Cochrane Library platforms. Preference was given to studies that could be classified as evidence level 2+ and higher according to the ASMOK system. References are given in the order of their mention in the text. The search depth was 5 years. Methods used to assess the quality and strength of the evidence were expert consensus and assessment of significance in accordance with the rating scheme. Methods used to analyze evidence were reviews of published meta-analyzes and systematic reviews with evidence tables.
Results. The consensus reflects aspects of clinical examination, respiratory support and postural control depending on the functional level, conservative and surgical treatment of spinal and limb deformities, anesthesia-related risk assessment, and preoperative, intraoperative and postoperative management of patients with DMD.
Conclusion. Deformities of the spine and lower extremities in DMD are frequent manifestation of the natural history of the underlying disease with the development of secondary orthopedic pathology, causing not only a severe violation of the function of movement and support, but also a violation of the function of internal organs. This requires a detailed assessment of the general somatic and neurological status in general, and the characteristics of the damage to the axial skeleton and extremities in particular. This is achieved by a detailed preoperative multidisciplinary examination to thoroughly assess the risks of complications and to skillfully follow-up a patient depending on functional status and regardless of age. The use of surgical treatment techniques for orthopedic pathology in DMD with proven effectiveness significantly improves self-care, the quality of life of patients and their closest persons, improves the balance of the body, and helps to maintain the function of external respiration and the possibility of verticalization.
Material and Methods. Consensus is a version of the main foreign protocols adapted for use in post-Soviet countries (the basis of consensus is the TREAT NMD protocol: treatnmd.ncl.ac.uk/care/dmd/diagnosis-management-DMD), as well as of works systematized on the basis on evidence level and reflecting modern approaches to the diagnosis and rehabilitation (including surgical) of spinal and limb deformities in patients with Duchenne myodystrophy. The recommendations are based on literature data and the authors’ own experience. Search in electronic databases was performed on the Medline, Embase, Web of Science and Cochrane Library platforms. Preference was given to studies that could be classified as evidence level 2+ and higher according to the ASMOK system. References are given in the order of their mention in the text. The search depth was 5 years. Methods used to assess the quality and strength of the evidence were expert consensus and assessment of significance in accordance with the rating scheme. Methods used to analyze evidence were reviews of published meta-analyzes and systematic reviews with evidence tables.
Results. The consensus reflects aspects of clinical examination, respiratory support and postural control depending on the functional level, conservative and surgical treatment of spinal and limb deformities, anesthesia-related risk assessment, and preoperative, intraoperative and postoperative management of patients with DMD.
Conclusion. Deformities of the spine and lower extremities in DMD are frequent manifestation of the natural history of the underlying disease with the development of secondary orthopedic pathology, causing not only a severe violation of the function of movement and support, but also a violation of the function of internal organs. This requires a detailed assessment of the general somatic and neurological status in general, and the characteristics of the damage to the axial skeleton and extremities in particular. This is achieved by a detailed preoperative multidisciplinary examination to thoroughly assess the risks of complications and to skillfully follow-up a patient depending on functional status and regardless of age. The use of surgical treatment techniques for orthopedic pathology in DMD with proven effectiveness significantly improves self-care, the quality of life of patients and their closest persons, improves the balance of the body, and helps to maintain the function of external respiration and the possibility of verticalization.
About the Authors
S. O. Ryabykh
The Russian Ilizarov Scientific Center for Reconstructive Traumatology and Orthopedics
Russian Federation
Russian Federation
Sergey Olegovich Ryabykh, DMSc, pediatric surgeon, board-certified orthopedic trauma surgeon, vertebrologist, Head of the Clinic of Spine Pathology and Rare Diseases, AOSpine RF Education Officer Ortho, Member of the Presidium of the Russian Association of Spine Surgeons, Member of the Association for the Study and Application of the Method of Ilizarov ASAMI Russia, Member of the All-Russian Society of Orthopedic Trauma Surgeons
6 Marii Ulyanovoy str., Kurgan, 640014
P. V. Ochirova
The Russian Ilizarov Scientific Center for Reconstructive Traumatology and Orthopedics
Russian Federation
Russian Federation
Polina Vyacheslavovna Ochirova, MD, PhD, orthopaedic trauma surgeon, Clinic of Spine Pathology and Rare Diseases
6 Marii Ulyanovoy str., Kurgan, 640014
D. M. Savin
The Russian Ilizarov Scientific Center for Reconstructive Traumatology and Orthopedics
Russian Federation
Russian Federation
Dmitry Mikhailovich Savin, MD, PhD, neurosurgeon, Head of Trauma-Orthopfedic Department No. 9 of the Clinic of Spine Pathology and Rare Diseases
6 Marii Ulyanovoy str., Kurgan, 640014
A. N. Tretjakova
The Russian Ilizarov Scientific Center for Reconstructive Traumatology and Orthopedics
Russian Federation
Russian Federation
Anastasia Nikolaevna Tretjakova, board-certified anesthesiologist-resuscitatoir, Head of Pediatric Anesthesiology and Critical Care Service
6 Marii Ulyanovoy str., Kurgan, 640014
D. A. Popkov
The Russian Ilizarov Scientific Center for Reconstructive Traumatology and Orthopedics
Russian Federation
Russian Federation
Dmitry Arnoldovich Popkov, DMSc, board-certified orthopedic trauma surgeon, Professor of the RAS, Corresponding Member of the French Academy of Sciences, Head of the Clinic for Neuroorthopedics and Systemic Diseases
6 Marii Ulyanovoy str., Kurgan, 640014
T. V. Ryabykh
The Russian Ilizarov Scientific Center for Reconstructive Traumatology and Orthopedics
Russian Federation
Russian Federation
Tatyana Viktorovna Ryabykh, board-certified paediatrist
6 Marii Ulyanovoy str., Kurgan, 640014
Е. N. Shchurova
The Russian Ilizarov Scientific Center for Reconstructive Traumatology and Orthopedics
Russian Federation
Russian Federation
Еlena Nikolayevna Shchurova, DSc in Biology, leading researcher, Clinical Laboratory of the Clinic of Spine Pathology and Rare Diseases
6 Marii Ulyanovoy str., Kurgan, 640014
M. S. Saifutdinov
The Russian Ilizarov Scientific Center for Reconstructive Traumatology and Orthopedics
Russian Federation
Russian Federation
Marat Samatovich Saifutdinov, DSc in Biology, neurophysiologist, leading researcher in the Scientific Laboratory of the Clinic of Spine Pathology and Rare Diseases
6 Marii Ulyanovoy str., Kurgan, 640014
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For citations:
Ryabykh S.O., Ochirova P.V., Savin D.M., Tretjakova A.N., Popkov D.A., Ryabykh T.V., Shchurova Е.N., Saifutdinov M.S. Deformities of the spine and limbs in patients with Duchenne myodystrophy: clinical features, diagnosis and treatment. Interstate consensus protocol. Russian Journal of Spine Surgery (Khirurgiya Pozvonochnika). 2020;17(1):61-77. (In Russ.) https://doi.org/10.14531/ss2020.1.61-77
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