Deformities of the spine and limbs in patients with Duchenne myodystrophy: Clinical features, diagnosis and treatment. Interstate consensus protocol

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