Search

Study Design: Technical note. Objectives: The objective of this study is to check out safety and rationality of standardized and fast tricks to select trajectory of subaxial cervical pedicle screw (SCPS) insertion, based on simple angles to bony landmarks. Materials and Methods: Stage 1 – Computed tomography (CT)-morphometric analysis of C3–C7 vertebrae of ten patients with cervical degenerative diseases. Stage 2 – SCPS insertion in 6 cadavers, according to the developed technique (59 pedicle screws). Stage 3 – SCPS insertion in 6 patients, according to the developed technique (32 pedicle screws). Results: CT-morphometric analysis showed that the average length of C3–C7 pedicle channels was 32 mm, the average angle between a pedicle axis and an axis of contralateral lamina - 180°, the average angle between a pedicle axis and plane of a posterior surface of a lateral mass amounted to 90° and the coordinates of an optimal entry point – 2 mm from a lateral edge and 2 mm from an upper edge of the lateral mass posterior surface. During the cadaveric study, 39 screws had a satisfactory position (66.1%), 7 screws permissible (11.9%), and 13 screws unacceptable (22%). During the clinical study, 26 screws (81.25%) had satisfactory position, 4 (12.5%) had permissible position, and 2 (6.25%) unacceptable position. Conclusion: Developed and clinically approved a method for simplicity SCPS insertion is relatively safe and cheap. No doubt, it requires further investigation, but the results of primary analysis allow us to recommend it to wide practical application.

The aim of the study was the electroneuromyographic evaluation of the spinal cord pyramidal structures reactivity and resistance in response to the impact of intraoperative factors associated with the application of technologies instrumental correction of spinal deformities of various etiologies and degrees of severity, realized with the help of submersible transpedicular fixation systems. Before treatment and 4-31 (9.6 ± 0.3) days after surgery using methods of global and stimulation (M-responses) electroneuromyography were examined 135 patients 8-50 (16.4 ± 0.6) years (42 male, 93 female) with spinal deformities of various etiologies and severity. The intrao perative maximum angle correction of the spinal column ranged from 3° to 95° (37.2 ± 1.5°). To assess the reactivity of the spinal cord structures located at the top of the primary arc deformation used integral index - the index of sensorimotor deficit (ISD). The lowest values of ISD and most distinct postoperative negative trends of this indicator were observed in persons with spinal deformities of neuromuscular etiology.

A quantitative evaluation of the relationship between the level of postoperative motor deficit and the intensity of reaction of pyramidal tract to the correction of spinal deformity.The correction of spinal deformities of different etiology was performed under neurophysiological control for 87 patients (30 men, 57 women), aged 15.6±0.6 years. Reaction intensity of pyramidal tract was evaluated using the scale developed by the authors.The relationship between the intensity of intraoperative reaction of somatic motor system and EMG-signs of postoperative subclinical motor deficit in temperature-and-pain sensitivity disorders was shown.EMG evaluation of the level of intensity of subclinical motor deficit in combination with changes in temperature-and-pain sensitivity after the spinal deformity correction complies the reaction intensity of somatic motor system to surgical aggression. Neurologists should pay more attention to the patients with V type reaction not only during postoperative period, but before the next stage of surgery.Цель исследования. Количественная оценка связи между уровнем послеоперационного моторного дефицита и интенсивностью реакции пирамидного тракта на коррекцию деформаций позвоночника. Материал и методы. У 87 больных в возрасте 15,6±0,6 года (30 мужского, 57 женского пола) с деформациями позвоночника различной этиологии под нейрофизиологическим контролем проведена коррекция деформации позвоночника. Интенсивность реакции пирамидной системы оценивали по разработанной нами шкале. Результаты. Выявлена связь между интенсивностью интраоперационной реакции пирамидной системы и выраженностью электромиографических (ЭМГ) признаков послеоперационного субклинического моторного дефицита на фоне нарушений температурно-болевой чувствительности. Заключение. ЭМГ-оценка выраженности уровня субклинического моторного дефицита в сочетании с изменениями температурно-болевой чувствительности после коррекции деформации позвоночника соответствует интенсивности реакции пирамидной системы на хирургическую агрессию. Наличие V типа реакции предполагает необходимость повышенного внимания к таким пациентам со стороны невролога не только в послеоперационном периоде, но и перед очередным этапом последующего оперативного вмешательства.

Spondyloepiphyseal dysplasia congenita (SEDC) is an autosomal dominant disorder, characterized by disproportionate dwarfism with short spine, short neck associated with variable degrees of coxa vara. Cervical cord compression is the most hazardous skeletal deformity in patients with SEDC which requires special attention and management. Ten patients with the clinical and the radiographic phenotypes of spondyloepiphyseal dysplasia congenita have been recognized and the genotype was compatible with single base substitutions, deletions or duplication of part of the COL2A1 gene (6 patients out of ten have been sequenced). Cervical spine radiographs showed apparent atlantoaxial instability in correlation with odontoid hypoplasia or os-odontoideum. Instability of 8 mm or more and or the presence of symptoms of myelopathy were the main indications for surgery. Posterior cervical fusion from the occiput or C1–3, decompression of C1–2 and application of autorib transfer followed by halo vest immobilization have been applied accordingly. Orthopedic management of children with spondyloepiphyseal dysplasia congenita (SEDC) should begin with the cervical spine to avoid serious neurological deficits and or mortality.

The aim of this study was electroneuromyographic evaluation of the spinal cord pyramidal structures reactivity when performing the onestage (intraoperative) instrumental correction of spinal deformities of various etiologies and severity, achieved through immersion transpedicular fixation systems.

This study was aimed at revealing the features condition of thermal, pain sensitivity and morphological pattern of the skin of the human back at the apex of spinal deformity in the thoracic area in patients with idiopathic scoliosis with stage III-IV. The study included 41 adolescent with idiopathic scoliosis with stage III-IV. Temperature-pain sensitivity was studied in Th6-Th10 dermatomes on the right and the left. Biopsies (skin) for histological examination were taken intraoperatively in projection corresponding to the apex of the arc scoliosis of the spine. Significant disorders of the sensitivity to temperature and pain were found in the dermatomes to the apex of the thoracic spine deformity that were hyperesthesia, hypoesthesia, or absent thermal sensitivity. Histostrukturnye changes of the skin are significantly decreasing the thickness of the epidermis and dermis, reduction of capillaries and changing the structure of the small vessels, destruction of individual nerve fibers and free nerve endings.

Rationale: The term idiopathic osteoporosis itself is quite a non-specific disease label, which fails to address the etiological understanding. Bone mineral density alone is not a reliable parameter to detect patients at high risk of fracture. The diversity of the clinical phenotypes of discolored teeth, blueness of the sclera, back and joint pain, cardiovascular disease, Diabetes type II, hearing problems and a long list of orthopedic problems are have to be considered. Patients concerns: Our study has been designed in accordance with the clinical and radiological phenotype of eleven index cases with the provisional diagnosis of OI, which was followed by genotypic confirmation. This was followed by the invitation of siblings, parents, grandparents and other relatives to participate in the interviews, and to discuss the impact of the diagnosis. Proper collaboration with these families facilitated the process to identify other subjects with a history of fractures and other deformities/disabilities which were seemingly correlated to heritable connective tissue disorder. In total, 63 patients (27 children and 36 parents/grandparents and relatives) were enrolled in the study. Two groups of children were not included in our study. We excluded children with incomplete documentation and children who manifested de novo mutation. The term idiopathic osteoporosis (IOP) has been given to these families in other Institutes and was considered as a definite diagnosis. IOP was solely based on T scores, BMD and certain laboratory tests. Surprisingly, no single adult patient underwent clinical and or radiological phenotypic characterization. Diagnoses: A constellation of significant disease associations with osteoporotic fracture risk have been encountered. The index cases showed mutations in COL1A1 (17q21.31.q22) and COL1A2 (7q22.1), the genes encoding collagen type I. The phenotype/genotype confirmation in 11 children was the key factor to boost our research and to re-consult each family. Comprehensive clinical and radiological phenotypic documentation has been applied to most of other family subjects who principally received the diagnosis of IOP. Interventions: All adult patients had normal serum calcium and only three patients showed an average of low serum phosphate of 0.7–0.61 mmol/l. Serumcrosslaps in six parents was in the average of (2.9–3.8 nM) and PTH levels were normal in all patients (the average showed 8.73 pg/ml). Outcomes: Our efforts to minimize and constrain the usage of the term idiopathic osteoporosis and to understand the sequence of pathological events that occurred in these families were emphasized. These efforts evolved into a remarkable and unique constellation of clinical findings. Strikingly, fracture represented a portion in a series of skeletal and extra-skeletal deformities and abnormalities which are all correlated to connective tissue disorder. This was achieved mainly through comprehensive phenotype/genotype confirmation, followed by scrutinizing the records of each family, clinical examination of the adults and revising the archives of our Hospitals and other Institutes. Lessons: The sequence of diverse pathological events recorded within each family would be almost incomprehensible without a proper etiological understanding of the natural history of each child/family deformity that led to their occurrences. We wish to stress that, our current study is just an attempt to cover only a tiny fraction of the tip of the iceberg and to profoundly explore one of the most under-estimated causes of idiopathic osteoporosis.

Marked ligamentous hyperlaxity and muscle weakness/wasting associated with awkward gait are the main deficits confused with the diagnosis of myopathy. Seven children (6 boys and 1 girl with an average age of 8 years) were referred to our department because of diverse forms of skeletal abnormalities. No definitive diagnosis was made, and all underwent a series of sophisticated investigations in other institutes in favor of myopathy. We applied our methodology through the clinical and radiographic phenotypes followed by targeted genotypic confirmation. Three children (2 boys and 1 girl) were compatible with the diagnosis of progressive pseudorheumatoid chondrodysplasia. The genetic mutation was correlated with the WISP 3 gene actively expressed by articular chondrocytes and located on chromosome 6. Klinefelter syndrome was the diagnosis in 2 boys. Karyotyping confirmed 47,XXY (aneuploidy of Klinefelter syndrome). And 2 boys were finally diagnosed with Morquio syndrome (MPS type IV A) as both showed missense mutations in the N-acetylgalactosamine-sulfate sulfatase gene. Misdiagnosis can lead to the initiation of a long list of sophisticated investigations.

IntroductionThe bayonet congenital abnormality of cervical-thoracic spine was previously described as a rarest clinical case under the title “congenital upper spine spondylolisthesis ” (Tokgozoglu AM et all., 1994) or “congenital dislocation of the cervical spine” (Shintaki M et all., 2013) without detailed description of anatomy and principles of surgical treatment. Our series include analysis of 10 CTSI cases from 4 clinics.Material and MethodsClinical, X-ray, CT and MRI peculiarities of 10 patients aged from 1 year 8 mns till 15 years were analyzed. The repeated cervical-thoracic abnormality included antero-posterior and cranial-caudad shift of the spine into the cervical spinal canal. All patients had arcs dysplasia, but any case has a complete posterior segmental defects, MMC or split spinal cord syndrome.ResultsAccording to Frankel scale the neurological status was estimated as a type E in one patient, type D – in 4, type C - in 4 and type B in one, incl. two paraparesis and 7 tetraparesis cases. Cr...

Spasticity treatment is one of the key aspects of the contemporary cerebral palsy (CP) rehabilitation that influences on the effectiveness of other methods. The paper presents the first Russian document that unites the recommendations for the BTA treatment of CP and could be used as the guideline for the multilevel injections. The Russian consensus on the multilevel botulinum toxin A (BTA) treatment of spastic CP is based on the international data and the results of national studies. The authors describe typical CP spasticity patterns in the upper and lower extremities, give recommended intervals for the BTA (Abobotulinum toxin A) dosages for the whole injection procedure and for the separate muscles. The method of dosage calculation for functional segments is also described. Attention is paid to the frequency, optimal intervals between the repeated injections and the whole duration of BTA treatment. The authors discuss effectiveness and safety of BTA, factors that potentially influence the results of the injections, including ultrasound and electromyography control, and indications for the continuation and termination of treatment.Преодоление спастичности является одним из ключевых факторов проведения реабилитации пациентов с детским церебральным параличом (ДЦП). В статье представлен первый российский документ, объединяющий мнения экспертов по ботулинотерапии ДЦП и служащий ориентиром при планировании ее применения. Он отражает согласованное мнение российских экспертов о методике выполнения многоуровневых инъекций ботулинического токсина типа А (БТА) при спастических формах ДЦП, основанное как на результатах международных публикаций, так и на собственном клиническом опыте. Выделены наиболее характерные для ДЦП паттерны спастичности верхних и нижних конечностей, представлены рекомендованные диапазоны доз препарата abobotulinumtoxin A на всю инъекционную процедуру, отдельные мышцы и функциональные сегменты конечностей. Обсуждены частота и оптимальные интервалы между повторными инъекциями, общая продолжительность применения ботулинотерапии при ДЦП, необходимость применения методов контроля точности инъекций, контроль эффективности ботулинотерапии, безопасность. Отдельно рассмотрены факторы, влияющие на эффективность ботулинотерапии, определены показания к прекращению и продолжению лечения.Преодоление спастичности является одним из ключевых факторов проведения реабилитации пациентов с детским церебральным параличом (ДЦП). В статье представлен первый российский документ, объединяющий мнения экспертов по ботулинотерапии ДЦП и служащий ориентиром при планировании ее применения. Он отражает согласованное мнение российских экспертов о методике выполнения многоуровневых инъекций ботулинического токсина типа А (БТА) при спастических формах ДЦП, основанное как на результатах международных публикаций, так и на собственном клиническом опыте. Выделены наиболее характерные для ДЦП паттерны спастичности верхних и нижних конечностей, представлены рекомендованные диапазоны доз препарата abobotulinumtoxin A на всю инъекционную процедуру, отдельные мышцы и функциональные сегменты конечностей. Обсуждены частота и оптимальные интервалы между повторными инъекциями, общая продолжительность применения ботулинотерапии при ДЦП, необходимость применения методов контроля точности инъекций, контроль эффективности ботулинотерапии, безопасность. Отдельно рассмотрены факторы, влияющие на эффективность ботулинотерапии, определены показания к прекращению и продолжению лечения.

Rationale: Clinical and radiographic phenotypic characterizations were the base line tool of diagnosis in 3 syndromic disorders in which congenital cervico-thoracic kyphosis was the major deformity. Patients concerns: Directing maximal care toward the radiographic analysis is not only the axial malformation but also toward the appendicular abnormalities was our main concern. We fully documented the diversity of the spine phenotypic malformation complex via the clinical and radiographic phenotypes. Diagnoses: We established the diagnosis via phenotypic/genotypic confirmation in 3 diverse syndromic entities namely acampomelic campomelic dysplasia, Larsen syndrome and Morquio syndrome type A (mucopolysaccharidosis type IV A). Interventions: Surgical interventions have been carried out in the Larsen syndrome and Morquio syndrome type A, resepectively. Outcomes: The earliest the diagnosis is, the better the results are. The necessity to diagnose children in their first year of life has many folds, firstly the management would be in favor of the child's growth and development and secondly, the prognosis could be clearer to the family and the medical staff as well. Our current paper is to sensitize paediatricians, physicians and orthopedic surgeons regarding the necessity to detect the aetiological understanding in every child who manifests a constellation of malformation complex. Lesons: Scoliosis and kyphosis/kyphoscoliosis are not a diagnosis in themselves. Such deformities are mostly a symptom complex correlated to dozens of types of syndromic associations. The rate curve progression and the final severity of congenital spine tilting are related to 3 factors: (a) the type of vertebral malformation present, (b) the patient's phenotype, and (c) the diagnosis.