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Objective. To perform validation study of the E. Pola classification (2017) and to assess expert consensus on the diagnosis and treatment of infectious lesions of the spine in the national professional community.Material and Methods. A clinical and radiological database on 15 cases of infectious spondylitis, as well as the information about original article by Pola and a Russian translation of the classification and tactical tables from this article, were distributed to 408 orthopedic traumatologists, neurosurgeons and radiologists who have experience in treating patients with spinal pathology and whose data are available in the registers of the relevant professional associations of the Russian Federation. The coincidence/difference in the responses concerning the definition of lesion types and the choice of treatment tactics, as well as proposals for the use of classification were assessed.Results. Answers were obtained from 37 respondents from 11 regions of the Russian Federation. The general interobserver agreement index (Fleiss kappa) for all types of spondylodiscitis was 0.388 (95 % CI 0.374–0.402), including for lesion types: type A – 0.480 (95 % CI 0.460–0.499, type B – 0.300 (95 % CI 0.281–0.320), and type C – 0.399 (95 % CI 0.380–0.419). Agreement levels were higher among radiologists (type A – 0.486, type B – 0.484, and type C – 0.477), orthopedic traumatologists (type A – 0.474, type B – 0.380, and type C – 0.479), and specialists with clinical experience less than 10 years (type A – 0.550, type B – 0.318, and type C – 0.437). The pooled data for all 12 lesion subtypes showed general poor agreement (k = 0.247, CI 0.240–0.253), satisfactory level was found for B3.2 type (k = 0.561, CI 0.542–0.581), good agreement (k > 0.61) was achieved between orthopedic traumatologists for type B3.2 and between radiologists for B3.1 and B3.2 lesion types. Respondents refused to use basic treatment options for type A in 15.1 %, type B in 7.5 % and type C in 3.2 % of answers, while indicating the need for interventions through anterior approach in 24.7 %, 43.0 % and 46.2 %, respectively. Limitations of the classification use depending on the localization and etiology of spondylitis were noted. Authors recommended taking into account the presence of systemic inflammatory response syndrome, mandatory CT scanning, clarification of spinal instability criteria, and the addition of anterior surgical interventions to the treatment algorithm.Conclusion. The Pola classification of spondylodiscitis is currently considered the most successful for tactical algorithms and implementation in broad clinical practice for spondylodiscitis. However, at the stages of its clinical application, there is an unsatisfactory interobserver expert consensus on the types of lesions, and there are limitations related to the etiology, localization and severity of the disease. A modified classification taking into account the identified limitations and including anterior procedures in the tactical options is advisable.

Objective. To analyze the results of the use of minimally invasive technologies in the treatment of patients with degenerative deformity of the lumbar spine.Material and Methods. Design: Single-center, non-randomized continuous retrospective cohort study. The level of evidence is 3b (UK Oxford, version 2009). A total of 57 patients (10 men and 47 women) were operated for degenerative scoliosis of the lumbar spine using minimally invasive techniques. The quality of life indicators using ODI, SF-36, VAS, as well as linear and angulometric parameters of the spine were studied.Results. The age of patients ranged from 37 to 81 years (62/62 [55; 67], hereinafter the data format is mean/median [1; 3rd quartile]). In the postoperative period, patients operated on with MIS techniques showed a statistically significant decrease in pain by 4.3/4.0 [3; 6] points in the lumbar spine, and by 4.3/4.0 [3; 6] points in the legs. Quality of life indicators according to ODI improved by 24/23 [19; 29], and the level of functional adaptation according to the SF-36 questionnaire – by 18/18 [14; 21] in terms of physical parameters and by 18/20 [16; 23] in terms of mental parameters. The deformity angle in the frontal plane according to Cobb decreased by 12.9°/13.0° [10°; 17°], lumbar lordosis changed by 3.3°/2.0° [-1°; 7°], segmental angle L4–S1 – by 1.0°/0.0° [-5°; 7°], and SVA changed by -7.5/-2.0 [-29; 15] mm. As a result of minimally invasive surgical intervention, a good clinical result was obtained in correcting the scoliotic deformity angle from 17.5°/16.0° [11°; 22°] to 4.6°/4.0° [1°; 7°].Conclusion. The study showed the effectiveness of minimally invasive surgical treatment of degenerative scoliosis of the lumbar spine with short-segment fixation, which allowed obtaining satisfactory clinical results in 93 % of cases with a minimum number of complications (7 %).

The interests of society and the modern rhythm of life have shifted the focus towards early rehabilitation, including surgical rehabilitation, and predetermined requirements of functional activity and quality of life in all age groups.The purpose of study – search for ways to optimize the organization of the traumatological and orthopedic service, taking into account the age continuity of nosologies and the inclusion of subgroups of orthopedic diseases of childhood, degenerative diseases, destructive, incl. tumor and traumatic in the model proposed by the authors.Material and methods. Forms of Federal Statistical Observation. The evaluation criteria were data on the prevalence of musculoskeletal diseases for 2019.Results and discussion. An analysis of the primary diagnosis of the orthopedic pathology by regions of the Russian Federation showed extreme variability, and the availability of care does not depend on the population and billing in the regions. The monetary value of the treatment of both planned and urgent pathology has shortcomings in standardization and harmonization between regions. The authors substantiate the new “3DT” organizational concept as the basis for a stable and understandable model of the country’s traumatology and orthopedic service for administrators and the population. The model identifies four areas of directions for assessing the pathology of the musculoskeletal system: “D1” (“children’s” diseases and their outcomes); “D2” (degenerative and involutive pathology); “D3” (destructive diseases of tumor or infectious pathology); “T” (trauma of and its consequences), which have fundamentally different approaches to the organization and planning of medical care.Conclusion. The directions of the “3DT” model require different approaches to the organization of medical care. Groups “D1” and “D2” must be implemented through national (federal) programs, for groups “D3” and “T” the approach of payment for the completed case is applicable. In each sector, it is necessary to indicate the basic, additional and optional amount of assistance. The target indicator for the stability and effectiveness of the model for organizing specialized assistance should be considered to be the provision of a basic level, and the possibility of financing additional assistance cannot be at the expense of providing basic assistance.

The publication highlights the concept of patient executive control (PEC) in pediatric and adolescent orthopedics, as well as the means of achieving it, which is proposed for use in the domestic health care system. The authors identified the key areas of modernization of primary care in the provision of orthopedic and traumatological care to children and adolescents with idiopathic scoliosis. Among them, a special place is occupied by the development and testing of checklists and scales for assessing the orthopedic status for primary care specialists, planning and implementation of a pilot project on early detection of pathology, prescribing and conducting conservative brace treatment in order to reduce the degree of scoliotic deformity of the spine in growing patients, clinical justification for the inclusion of compensation for the costs of implementing this treatment in the compulsory health insurance system (CHI).

Creating a unified information system in Russia is not a trivial task. The main difficulty is not in geographical or cultural features, but in the process of digitalization of the existing system. An important transformation tool is the involvement of professionals in the process of setting goals for system design, as well as testing the results.The purpose of the work is to describe the concept of vertically integrated medical information system (VIMIS) “traumatology and orthopedics” as a digital tool for effective management of the provision of care by profile.Materials. The existing projects of a VIMIS, the routing schemes for patients with injuries and diseases of the musculoskeletal system, the level of organization and provision of medical care in the regions are analyzed.Results. The concept of management of medical care by traumatology and orthopedic the management scheme of the VIMIS project for the profile of traumatology and orthopedics (TO) were developed, an agreement was signed on the scientific and methodological interaction of participants. The project is aimed at the digital transformation of the called tasks, increasing the “digital qualification” to solve the search for tasks in the field of quality control of specialized medical care, efficient use of resources, substantiation of scientific problems. The difference between the profile of “traumatology and orthopedics” is determined by the fact that with the help of VIMIS TO a problem of different directions is solved, including indicators that can be incoherent.Discussion. The project to create a vertically integrated medical system in the field of “traumatology and orthopedics” as a digital transformation of specialized care was based on an analysis of the most successful technological and organizational solutions: modern evidence-based models of treatment and care; artificial Intelligence; intelligent planning and treatment risk stratification; remote patient monitoring and the implant technology industry; advanced professional education platform.Conclusion. The effectiveness of the model should be evaluated for the prospect of piloting with the integration of various regions medical information systems and its verification in the regions. Moreover, it is necessary to explore the sharing of large data sets and lay out the features of their use, given the differences in the nature of the cooperation of consortiums.

Case series, literature review, and technical note. To compare two different approaches to treat the spinal deformity with split cord malformation type I (SCM I). To present a new method of one-stage surgical treatment of congenital spinal deformity with wide bony septum (SCM I). Analysis of the literature on the different types of combined surgical treatment of spinal deformities with SCM I was performed. We have provided our own data on 27 patients treated for congenital spinal deformity and SCM I, one of which underwent Schwab IV type osteotomy at the apex of the deformity through the bony septum and pedicles. Inclusion criteria were presence of spinal deformity in combination with SCM 1, performed surgery to correct spinal deformity, and follow-up period of at least 2 years. The result of the literature review was controversial and requires additional research. The average age of patients was 8.8 ± 6.6 years old. One-stage treatment of SCM I and spinal deformity was performed in 10 patients (group I) and two-stage in 14 patients (group II). Three patients with severe myelodysplasia, SCM I, and congenital kyphoscoliosis underwent correction of spinal deformity without SCM I removing (group III). The group I had the longest surgery duration (mean 289 ± 75 min) and largest blood loss (mean 560 ± 386 ml), a high percentage of deformity correction (mean 69.6%), and the highest rate of complications (60%). The most optimal was the two-stage treatment with the mean surgery duration 191 ± 137 min, mean blood loss 339 ± 436 ml, mean correction rate 63%, and frequency of complications 21%. The average follow-up time was 6.0 ± 2.6 years. One stage surgery associated with a large surgical invasion and a large number of complications. It can be used in some cases, for example when the wide bony septum (SCM I) is localized at the apex of the congenital scoliosis or kyphosis. In all other cases, it is worth adhering to a two-stage treatment. Many new works demonstrate the relative safety and effectiveness of deformity correction without removing the SCM. In our opinion, indications for treatment of spinal deformity without SCM I removing can be the need to perform a shortening ostetomy outside the SCM zone. The remaining cases require a thorough assessment and a balanced decision.

Introduction Morphological, biochemical and histopathological characteristics of paraspinal muscles have been well described in patients with idiopathic scoliosis. However, there is a paucity of literature on morphological picture of paraspinal muscles in patients with severe idiopathic scoliosis. Objective To explore how the magnitude of thoracic spine deformity affects morphological characteristics of paraspinal muscles in patients with severe idiopathic scoliosis. Material and methods A total 21 patients with idiopathic scoliosis were reviewed. The patients were assigned to two groups depending on magnitude of preoperative thoracic spine deformity. A major group consisted of patients with scoliosis of ≥ 60º (n = 11) and control group included patients with a curve < 60º (n=10). Biopsy samples were taken from muscle fragments (multifidus muscle) at the apex of the curve (major arc) at the Th6-Th10 level on the convex side while approaching to the posterior spinal structures during surgical correction of kyphoscoliosis using light microscope. The specimens were examined histologically with light microscope. Results Patients with severe idiopathic scoliosis (≥ 60º) showed evident muscle fiber atrophy and dystrophy of different extent. There were nucleus free areas and homogenized muscle fragments observed with connective tissue layers being swollen and frayed with multiple fibroblasts seen in nearly all the samples. Arterial walls were normally fibrotized. Structural changes ranged from minimal with a curve of 60º to degrading muscles with the fibers replaced by fat and connective tissues with a curve angle of 145º. Conclusion Morphological characteristics of paraspinal muscles have been shown to be severely affected by the magnitude of thoracic spine deformity in patients with severe idiopathic scoliosis. The findings should be considered in surgical correction of kyphoscoliosis with special focus on postoperative vascular and neurotropic therapy to allow wound healing and lower complication rate.

Introduction The problem of treating chronic unstable pelvic injuries is characterized by the complexity of delayed one-step or staged reduction, difficulties in choosing the technology and treatment options, complexity of selecting criteria for evaluating planning along with the effectiveness of treatment. The use of spinal systems to perform reduction manipulations and fixation of the pelvis is described in few clinical observations. Therefore, the assessment of the effectiveness of the sequential use of various fixation systems in one patient was regarded by the authors as a rare opportunity and determined the purpose of the study. Purpose Demonstration of the possibilities of various osteosynthesis methods and their combination in reconstructive surgery of the pelvis in a case of its severe chronic injury. Materials and methods We present a clinical case and a brief analysis of the literature. A case of a patient with post-traumatic pelvic deformity and imbalance syndrome as a leading component of pelvic ring deformity is presented by us. The effectiveness of reconstruction options, including those with the use of spinal systems, was analyzed. The treatment was assessed with radiological study methods (X-ray and CT) with balance evaluation on a digital platform and functional scores for the quality of life. Results Correction of frontal deformity of the pelvis was achieved with compensation for a relative shortening of the left lower extremity, restoration of the center of rotation of the hip joints, and relief of pain in the lumbar region. The functional state according to the Majeed score system and clear radiological signs of stabilization of the pelvic ring while maintaining the position of the acetabulum in the frontal plane prove the effectiveness of hybrid osteosynthesis with the use of spinal fixation systems. Conclusion The leading syndromic complexes are instability with clinical manifestations of non-union and pain along with an imbalance syndrome, manifested by a gross deformation of the pelvic ring. Syndromic evaluation determines the tactics of surgical treatment while a detailed planning of the sequence of intervention, choice of the level of osteotomy, combination of osteosynthesis options using spinal fixation systems provide the solution of reconstructive pelvic surgery tasks in one session.

Background . The surgical treatment of congenital spinal deformity caused by hemivertebra is associated with high rate of complications. A research of a new surgery technique for operation time and blood loss decrease could potentially improve outcomes. The purpose — to evaluate the efficacy of ultrasonic bone scalpel in surgical treatment of pediatric congenital spinal deformities caused by monosegmental hemivertebra. Patients and Methods . Level of Evidence III. The study based on the data of 55 consecutively operated pediatric patients who underwent 59 posterior hemivertebra resection provided by ultrasonic bone scalpel from January 2015 to December 2019. The average age was 4 years and 4 months. 36 hemivertebra were located in thoracic spine and 23 were located in lumbar spine. Total duration of surgery, estimated blood loss (ml and % of circulated blood volume, CBV), complications rate and deformity correction were noted. The influence of posterior instrumentation length and patients age at time of surgery on evaluation parameters was analyzed. 5-year (2015–2019) systematic literature review was performed for compare with obtain results. Results . Total operation time was 131 min ± 33 min for thoracic spine and 165 min ± 50 min for lumbar spine ( p = 0,005). Estimated blood loss was 105 ml ± 74 ml (Me 80 ml) for thoracic resection and 123 ml ± 59 ml (Me 120 ml) — for lumbar ( p = 0,178). The length of posterior instrumentation were not influence on operation time and total blood loss ( p = 0,957; p = 0,967), patients age at time of surgery were not influence on operation time ( p = 0,458), but correlate with total blood loss (p = 0,023). Intraoperative complications was not observed. Four cases of transpedicular screw malposition without neurological deficit were noted (type C acc. Gertzbein-Robbins). Conclusions . Posterior hemivertebra resection with ultrasonic bone scalpel is safe and effective procedure provides decrease of operation time and estimated blood loss.

Design Presentation of clinical cases and literature analysis. Material and methods Clinical observation of patients with congenital cervical spine and shoulder girdle anomalies operated on with neuromonitoring was performed. Operative treatment of Sprengel’s deformity was produced with the use of neuroimaging. Results Surgical treatment resulted in good clinical outcomes without neurological adverse events and good aesthetic and radiological appearance at the descended scapula. Discussion Consideration for intraoperative neuromonitoring should be given to prevent neurological complications at lowering the elevated scapula. Conclusion Review of outcomes showed efficacious application of the most valid methods used to descend the scapula with neuromonitoring. This was supported by clinical and radiological findings of the extent of cranial transposition after appropriate correction and absence of neurological adverse events early postsurgery and at a long-term follow-up. No recurrence of scapular dislocation was observed at a long-term follow-up.

To clarify etiology, clinical features, and diagnostic and treatment options of atlantoaxial dislocation (AAD) due to os odontoideum (OsO) in patients with Down’s syndrome (DS). We described and analyzed three clinical cases of AAD due to OsO in DS patients and reviewed descriptions of similar cases in the scientific sources. According to literature review, more than 80% of DS patients with odontoid ossicles had atlantoaxial instability (AAI). AAI in DS patients with OsO is more often manifested in childhood and adolescence, rarely in adults when ligament relaxation is reduced. Some patients had acute clinical manifestation after a minor trauma without any precursors; in some of the cases, neurological deterioration increased during several years. We found that the earlier surgical treatment of AAD due to OsO in DS patients carries the higher recovery potential. Most patients with DS and OsO had AAI. The method of appropriate treatment in such cases is a posterior screw fixation. Preoperative halo traction and posterior fusion have proved to be a very useful tool in the treatment of AAD due to OsO in DS patients. Even if irreducibility of the AAD established preoperatively, it should not be an absolute indication for anterior decompression. In such cases, an attempt to reduce the AAD should be made under general anesthesia during posterior fixation.

Purpose — to evaluate outcomes of surgical treatment for high-grade spondylolisthesis using bone-disc-bone osteotomy, reduction and fixation through the dorsal approach. Materials and Methods. The authors retrospectively examined a monocenter five-year cohort (IV level of evidence). The study included 10 patients aging from 7 to 22 years (Me — 12 years, M±m — 13.1±4.1 years) who underwent surgery due to high-grade spondylolysis antelisthesis in the period from 2012 to 2017. Displacement was located in L5-S1 segments and corresponded to types 4-6 by AO Spine SDSG classification in all patients. Catamnesis was followed for the period from 1 to 5 years. Surgical procedures included bone-disc-bone osteotomy, L5 reduction and dorsal instrumental multi-bearing (from 2 to 5 spinal motion segments) using reduction transpedicular screws. The following parameters were evaluated: pain syndrome prior and after surgery, sagittal balance, spondylolisthesis mobility on the functional x-rays or CYs, severity grade of anterior spondylolysis, criteria of spontaneous muscular activity and MEPs as well as structure of postoperative complications. Results. L5 displacement prior to surgery was 92.6±25.2%, after surgery — 25.4±16.6% (Z = -2.805, p = 0.005). Patients with sagittal imbalance demonstrated normalization after the surgery allowing to re-classify pathology as “balanced spondylolisthesis”: PI from 67.9±8.6 to 67.5±8.7 (Z = 0,000, p = 1,000), PT from 26.8±13.3 to 20.1±7.1 (Z = -2,090, p = 0.037), SS from 41.3±8.7 to 47.3±9.7 (Z = -1.886, p = 0.059), SA from 34.9±36.3° to 8.6±7.1° (Z = -2.803, p = 0.005). 3 cases of transient L5 radiculopathy with full regress after conservative 6 months’ treatment were reported in the early follow up period (on day 3 after procedure). Pain syndrome dynamics on VAS scale prior to and after the surgery were as follows: spine 8.1±1.0 and 0.5±0.5 (Z = -2.814, p = 0.005), lower limbs 6.8±1.5 and 0.4±0.7 (Z = -2.812, p = 0.005), respectively. Life quality indices by SRS-24 score prior to and after the surgery were 62.6±7.9 and 90.7±12.4 (Z = -2.803, p = 0.005). Mobility of spondylolisthesis was observed in 9 patients. Spondylolisthesis severity by Bridwell classification in late period scored from 1 to 3 points. Conclusion. Use of AO Spine SDSG classification along with assessment of sagittal balance as well as severity of neurological deficit and pain syndrome allow to define the severity grade of spondylolisthesis, while normalization of parameters after the surgery speaks for positive treatment outcome. Extensive release during bone-disc-bone osteotomy at L5-S1 level along with altering tilt angle of the sacrum is the key factor for mobilization and radical correction of pelvic balance in high-grade spondylolisthesis. Outcomes of surgical treatment in the analyzed cohort demonstrate significant improvement in life quality (by SRS-24 score) and reduced pain syndrome (by VAS) in patients. At the same time precise compliance to the procedure protocol and intraoperative neuro-monitoring of MEPs allow to decrease risk of complications.

Short-limbed dwarfism, knock knees and joint hypermobility are the classical clinical criteria encountered in patients with pseudoachondroplasia. Progressive kyphoscoliosis was the main clinical presentation in our patients with pseudoachondroplasia. Preoperative spine radiographs in an 13-years-old-girl showed severe kyphoscoliotic deformity (kyphosis - 70o and scoliosis of Cobbs angle – 55o ). Keywords: Pseudoachondroplasia; COMP gene mutation.

Background: We aimed at reviewing results of surgical correction of spinal deformity due to hemivertebra with regard to length of instrumentation. Methods: Study comprised 117 patients with congenital spinal deformity treated between 2010 and 2018. Patients’ aged 1 to 18 years. Mean follow-up was 3 years (1 to 8). Posterior approach was used in all cases. Patients were subdivided into 4 groups. Result: Unilateral monosegmental fixation (Group I) for 15 children with mean age of 48.3 months. Cobb angle for scoliosis and kyphosis were 31.1 0 + 6.4 0 and 29.3° + 11.9 0 corrected to 7.8 0 + 6.8 0 and 4.7 0 + 4.0 0 respectively. Blood loss was 213.6 mL. Operating time was 165 minutes. Bilateral monosegmental fixation (Group II) for 24 patients with mean age of 53.1 months. Cobb angle for scoliosis and kyphosis were 32.5° + 8.6 0 and 30.9° + 4.3 0 corrected to 5.3 0 + 2.8 0 and 0.2 0 + 11.6 0 respectively. Blood loss was 215.4 mL. Operating time was 160.5 minutes. Bilateral three-segmental fixation (Group III) for 29 patients with mean age of 78.2 months. Cobb angle for scoliosis and kyphosis were 36.6° + 10.6 0 and 37.6° + 14.6 0 corrected to 6.2 0 + 6.1 0 and 5.0 0 + 5.2 0 respectively. Blood loss was 342.7 mL. Operating time was 197.0 minutes. Bilateral polysegmental fixation (Group IV) for 49 children with mean age of 112.7 months. Cobb angle for scoliosis and kyphosis were 40.2° + 14.8 0 and 58.2° + 35.6 0 corrected to 10.7 0 + 9.6 0 and 10.7 0 + 10.3 0 respectively. Blood loss was 549.3 mL. Operating time was 288.8 minutes. Conclusion: Choice of spinal fixation technique and the length of fixation were determined on the basis of patient’s age, magnitude of the deformity and concomitant vertebral abnormalities.

Background Maroteaux-Lamy disease (MPS Type VI) is an autosomal recessive lysosomal storage disorder. Skeletal abnormalities are vast. Early recognition may facilitate timely diagnosis and intervention, leading to improved patient outcomes. The most challenging is when patients manifest a constellation of craniocervical and articular deformities with variable age of onset. Methods We collected 15 patients with MPS VI (aged from 6 years-58 years). From within our practice in Pediatric Orthopedics, we present patients with MPS type VI who were found to manifest a diverse and confusing clinical presentations of hip deformities and cervical cord compression. Stem cell transplants were proposed as treatment tool and enzyme replacement therapy has been instituted in some patients. Results The spectrum of the clinical involvement in our group of patients was supported firstly via the clinical phenotype followed by assessment of the biochemical defect , which has been detected through the deficiency of N-acetylgalactosamine-4-sulfatase (arylsulphatase B) leading to increased excretion of dermatan sulphate. Secondly, through the molecular genetic results, which showed homozygous or compound heterozygous mutation in the ARSB gene on chromosome 5q14. Hip replacements and decompression operations have been performed to restore function and to alleviate pain in the former and life saving procedure in the latter. Conclusions The efforts in searching for the etiological diagnosis in patients with skeletal dysplasia/MPSs has not been rewarding as many had anticipated. This emerged from several facts such as improper clinical documentation, missing diagnostic pointers in radiographic interpretations, limited knowledge in skeletal dysplasia and its variants, and the reliance on underpowered studies. Physicians and radiologists are required to appreciate and assess the diverse phenotypic and the radiographic variability of MPS VI. The importance of considering MPS in the differential diagnosis of other forms skeletal dysplasia is mandatory. Finally, we stress that the value of early diagnosis is to overcome dreadful complications.

Background Children born with multiple congenital contractures have been almost always given the diagnosis of arthrogryposis multiplex congenita. Arthrogryposis is a descriptive term, not a specific disease entity. A heterogeneous group of conditions associated with multiple congenital joint contractures (mostly syndromic) should be considered. Methods The records of seven children (four boys and three girls with aged 6months- 11 years) of different ethnic origins have been included in this study. The constellation of specific craniofacial dysmorphic features, spine malformation complex, and appendicular skeletal abnormalities in addition to camptodactyly, talipes equinovarus and rocker- bottom feet were a cluster of malformation complex encountered in our patients. Via comprehensive clinical and imaging study (3D reconstruction CT scan), definite diagnosis of Escobar syndrome has been approached. Results The clinical and imaging phenotype was the key factor towards etiological understanding, treatment and genotype confirmation. We identified compound heterozygous mutations (c.459dupA [p.Val154Serfs*24] and c.794T>G [p.Leu265Serfs*24] of the CHRNG gene in four patients. Bilateral flexion contractures of the knees have been treated by using Iliazarov external fixator. Simultaneous corrections of scoliosis have been achieved by applying either dual traditional growing rods or single growing rods. Conclusions The clinical and radiological phenotypic characterizations are the fundamental tool in differentiating Escobar from other forms of multiple contractures. The aim of this study are three folds, firstly to demonstrate the importance of detecting the etiological understanding in children presented with multiple contractures, secondly to refute the general conception among the vast majority of pediatricians and orthopedic surgeons that arthrogryposis multiplex is a diagnostic entity. And thirdly, we were able to detect severe spine deformity via 3D reconstruction CT scan, namely unsegmented posterior spinal bar.

Introduction Incidence of adult degenerative scoliosis (ADS) among individuals over 50 years old reaches 68%. Surgical interventions aimed at correcting the spinal deformity in patients of the older age group are accompanied by a high risk of complications. The use of LLIF is associated with lower complications as compared with open anterior or posterior fusion. Materials and methods Seventy-one patients with ADS (13 men, 58 women) were operated at the Federal Neurosurgical Center. Their average age was 60.4/60 (average/median) [55;64.5] (1: 3 quartile) years. The follow-up was from 12 to 18 months. X-ray study, SCT, MRI of the lumbar spine were used. Questionnaire surveys were conducted using the visual analog pain scale (VAS), Oswestry Disability Index (ODI) and the Short Form-36 (SF-36). Deformity correction was estimated in the frontal plane with Cobb’s method. Scoliosis was classified according to SRS-Schwab classification. Parameters of sagittal balance were estimated: PI (Pelvic incidence), SS (Sacral slope), PT (Pelvic tilt), LL (Lumbar lordosis). SVA, PT and PILL (PI minus LL) were defined adjusted for the age. Results Back pain according to VAS relieved from 6.1/6 [4;8] to 2.2/2 [2;3] points (p less 0.001) and was statistically significant at 12 months after the surgery. Leg pain according to VAS decreased from 5.4/5 [4;8] to 2.1/2 [1;3] points (p less 0.001) and was statistically significant at 12 months after the surgery. Functional adaptation according to ODI improved from 51.2/52.2 [38.6;64.1] to 31.8/33.3 [26.1;35.9] (p less 0.001). According to SF36, PH before the surgery was 25.7/24.3 [21.8;28.9] on average and at 12 months after the surgery - 38.7/38.7 [35.4;41.2] (p less 0.001). SF-36 MH before surgery was 27.1/26.3 [21.8;31.4] on average and 12 months later – 41.3/40.6 [36.5;43.7] (p less 0.001). PT before the surgery was 23.3/22° [17.5;28], 12 months later it was 17.9/17° [15;20] (p less 0.001). PI-LL was 11.5/10 ° [4;17.5], 12 months later – 8.4/8 ° [5.5;11.5] (p = 0.11). Transient paresis of femur flexors on the ipsilateral side was observed in five (7 %) cases; transient hyposthesia on the anterior thigh surface occurred in eight (11.2 %) cases. There were two cases of medial malposition (0.4 %) of pedicle screws (474 screws), pseudoarthrosis at two levels (1.2 %) (Grade 4 Bridwell) out of 166 levels performed, and seven (4.2 %) cases of damage to cortical endplates. Conclusion Restoration of local sagittal balance in ADS patients by short-segment fixation using LLIF technology leads to a statistically significant improvement in the quality of life and increases functional adaptation. Few early and late postoperative complications, less intraoperative blood loss and shorter hospital stay make LLIF in combination with MIS transpedicular fixation a method of choice in determining the surgical tactics for ADS in elderly and old age patients.

Introduction Despite a great number of researches on idiopathic scoliosis reported there is still no instrumentation assessment of sensitivity before and after surgical correction of the curve found in literature. The purpose of the work was to explore dynamics in thermal pain sensitivity following correction of severe and mild spinal curves in patients with idiopathic scoliosis. Material and methods The work included results of examination of 25 patients with idiopathic scoliosis. Sampling population was divided into two groups depending on the extent of preoperative curve in major arch: Group I included patients with the curve of ≤ 60o (15 cases); Group II consisted of patients with the curve of > 60o (10 patients). Preoperative neurological examination showed no motor, reflex and sensory impairments. Acute deformity correction and spine stabilization with transpedicular systems were produced in all the cases. Thermal pain sensitivity was explored preoperatively and postoperatively at Th1-S2 dermatomes using electric esthesiometer. Results Disturbed thermal pain sensitivity of various extent was observed preoperatively in the study dermatomes of all patients with idiopathic scoliosis and was not shown to be dependent on the amount of the curve. Positive dynamics in thermal pain sensitivity revealed itself in reduced pain and heat thresholds and restored heat sensitivity and was observed in 41.5 to 54.1 % of the cases Group I following spine deformity correction. Deterioration was seen in 29.2 to 34.7 % of the cases. Group II showed negative dynamics in thermal pain sensitivity in 35.4 to 50 % of the cases with either increased or decreased pain threshold, enhanced heat threshold and loss of heat sensitivity. Sensitivity improved in 29.4 to 31.8 % of the patients. No changes in neurological status were noted in both groups after surgical correction with subclinical changes in thermal pain sensitivity to be considered. Conclusion Positive dynamics in thermal pain sensitivity prevailed in patients with idiopathic scoliosis following baseline curve correction of ≤ 60o. Negative dynamics in thermal pain sensitivity was seen in patients with baseline curve correction of > 60o with a greater risk of neurological complications.

Object of study The existing variety of cervical spine pathologies resulted in a huge number of nosologic classifications and complicates their interpretation. Purpose Based on the analysis of patients with various surgical pathologies of the cervical spine, we suggest a syndromic approach to their evaluation to simplify the choice of a therapeutic algorithm. Study design Retrospective monocentre multicohort study and literature review Materials and methods A retrospective analysis of 336 patients aged 0.9 to 77 years with cervical spine surgical pathology who underwent interventions at the Ilizarov center between 2010 to 2017 was performed. Results Based on the analysis of the symptoms that led to the surgical interventions, the following clinical syndromes were identified: compression-ischemic syndrome, instability syndrome, imbalance syndrome. Conclusion The proposed concept of assessing syndromes identifies the pathology of the cervical spine regarding its clinical manifestations. The use of the syndromic approach will facilitate intra- and interdisciplinary interaction, as well as allows for tactical decisions.

Objective To analyze clinical and radiologic features of pathologic atlantoaxial displacement (PAAD) in pediatric patients and to compose a treatment algorithm for anomaly-related PAAD. Background Criteria of different types of PAAD and treatment algorithms have been widely reported in the literature but are difficult to apply to patients with odontoid abnormalities, C2-C3 block, spina bifida C1, and children. Methods We evaluated results of treatment of 29 pediatric patients with PAAD caused by congenital anomalies of the craniovertebral junction (CVJ), treated in Ilizarov Center in 2009–2017, including 20 patients with atlantoaxial displacement (AAD) and 9 patients with atlantoaxial rotatory fixation. Results There were 14 males (48.3%) and 15 females (51.7%). We singled out 3 groups of patients: nonsyndromic (6 patients, 20.7%), Klippel-Feil syndrome (13 patients, 44.8%), and syndromic (10 patients, 34.5%). Odontoid abnormalities and C1 dysplasia were widely represented in the syndromic group. Local symptoms predominated in the nonsyndromic and KFS groups. In the syndromic group, all patients had AAD and myelopathy. A pronounced decrease of space available for chord C1 and increase of anterior atlantodental interval were noted compared with other groups. Conclusions We present a unified treatment algorithm of pediatric anomaly-related PAAD. Syndromic AAD are often accompanied by anterior and central dislocation and myelopathy and atlantooccipital dissociation. These patients require early aggressive surgical treatment. Nonsyndromic and Klippel-Feil syndrome AAD, atlantoaxial subluxation, and atlantoaxial fixation often manifest by local symptoms and need to eliminate CVJ instability. Existing classifications of symptomatic atlantoaxial displacement are not always suitable for patients with CVJ abnormalities.

Objective To evaluate the optimal timing and type of surgical treatment of myelomeningocele (MMC)-related spinal deformities and long-term follow-up of surgical treatment. Methods We reviewed and presented clinical pictures, treatment strategies and results of 20 patients with MMC-related spinal deformities treated at our center between 2010 and 2017. Results The average patient age was 6.3 years. The average preoperative neurologic status according to a modified Japan Orthopedic Association (mJOA) scale was 7.3 points (Benzel's modification). Average functional status was 41 points according to a functional independent measure scale (FIM). The average angle of kyphosis was 83.7°, that of scoliosis was 36.7°, and that of lordosis was 67° (Cobb angles). The average duration of surgery was 234 minutes, and the average total blood loss was 175 mL. The average angle of kyphosis correction was 61°, that of scoliosis correction was 25°, and that of lordosis correction was 25° (Cobb angles). The average duration of hospitalization was 16.6 days, and the average follow-up was 34.5 months. The total number of complications was 13. Reoperation was required in 9 cases. Neurologic status according to the mJOA scale improved by 0.6 point on average. Functional status according to the FIM increased by 6.6 points on average. Conclusions Early surgical correction of MMC-related spinal deformities improves body balance and quality of life. The dual growing rod technique is safe and effective in cases of moderate neuromuscular spinal deformities at an early age. Kyphectomy is a challenging procedure with high complication rates, especially skin problems, but there are no alternative procedures for cases of heavy rigid kyphosis.

OBJECTIVE. systematization of the electrophysiological phenomena observed during the intraoperative neuro-monitoring with subsequent development of the grading system for results of neuro-physiological testing of pyramidal tract, convenient for surgeon. MATERIAL AND METHODS. 288 patients, at the age of 1–27, under intraoperative neurophysiological monitoring underwent instrumental correction of deformity of the thoracic/thoracolumbar spine. RESULTS. We evaluated in points the changes of motor evoked potentials, observed during the surgical intervention. It was observed that the frequency of changes of their parameters, indicating the danger of iatrogenic lesions, depended on the age of the patient and did not exceed 10 % of the total amount of observations. CONCLUSION. Intraoperative neurophysiological monitoring allows the surgeon and anesthesiologist to correct their actions in a timely fashion; and it minimizes the danger of iatrogenic neurological disorders.

The aim of the present work was to perform an electroneuromyographic assessment of the reactivity and resistance of pyramidal structures of the spinal cord in response to intraoperative exposure to factors associated with the use of instrumented correction of spinal deformities of different etiologies and severities using submersible transpedicular fixation systems. Before treatment and at 4–31 (mean 9.6 ± 0.3) days after surgery, global and stimulated (M responses) electroneuromyography investigations were performed in 135 patients aged 8–50 (mean 16.4 ± 0.6) years (42 male and 93 female) with spinal deformities of different etiologies and severities. maximum intraoperative correction angles for deformities of the spinal column ranged from 3 to 95° (mean 37.2 ± 1.5°). The reactivity of spinal cord structures located at the apex of the base of main deformation arc was assessed in terms of an integral indicator – the index of sensorimotor deficit – (ISD). The lowest ISD values and the clearest post-operative negative trends in this indicator were seen in people with spinal deformities of neuromuscular etiology.

The results of the examination of 30 adolescents of 13-16 years old with scoliosis before and after surgical correction were compared. Intraoperative neurophysiological monitoring was used by the method of transcranial evoked motor potentials. The patients were divided into two groups depending on the presence or absence of neurophysiological signs of damage to nerve structures during the operation.The amplitude of the M-responses of the muscles of the lower limbs in the postoperative period remains at a level close to the initial one, with a noticeable decrease in the amplitude of voluntary electromyography, which is expressed unevenly and to a greater extent in patients with intraoperative signs of hazard for the motor pathways of the spinal cord.Adverse intraoperative events cause significant changes in the state of the motor system of patients with scoliosis and reduce the effectiveness of rehabilitation treatment in the postoperative period.

Objectives We sought to evaluate long-term results of surgical treatment of thoracolumbar segmental spinal dysgenesis (SSD). Methods We analyzed 8 patients with thoracolumbar SSD treated in our institution. Each case was evaluated for specific clinical and radiologic criteria and types and outcomes of treatment. Result The average age of primary surgery was 3.4 years (median 3.4 years, range 1.7−7 years). The average correction of kyphosis was 49.3° (mean 45°, from 25°−75°) and scoliosis 10.6° (mean 10°, from 0°−25°). Average follow-up time was 3.2 years (mean 2.6 years, from 1.8−5.6 years). Neurologic improvement was also achieved in all patients. The Japanese Orthopaedic Association scale score (Benzel's modification) was increased by 2.5 points on average (mean 2.5 points, from 2−5 points). One patient had complications: pseudarthrosis and rod fracture followed by refusion. Conclusions Our treatment strategy provides favorable deformity correction and neurologic improvement. It is limited by immature vertebral structures in newborns and infants, who should be carefully monitored from birth with braces until they reach the age when a fixing tool can be used.

The method of multislice computed tomography has been used to study qualitative and quantitative changes in the spine of the Carassius auratus goldfish that featured multiplanar spinal deformity in the areas of truncal and tail vertebrae.

Study Design Presentation of a clinical case. Object A clinical case of severe congenital kyphoscoliosis in an11-year-old child treated surgically. Method Vertebrotomy beyond the anomaly zone with the PSO technique and dynamic fixation of the spine. Result Correction of spinal deformity. Discussion Surgical treatment of congenital kyphoscoliosis with the PSO technique out the zone of congenital anomaly and implantation of dynamic systems achieve an orthopedic goal with a single intervention via a single approach.

Literature data were used to present the parameters of integral sagittal balance assessment. It is shown that the assessment of the sagittal balance should be performed taking into account the spatial position of the body and the use of the “cone of economy” concept and gravity line. Cervical spine and the pelvis are the key compensatory mechanisms in correction of the sagittal balance changes. The ratio of PI (pelvic incidence) and LL (lumbar lordosis) is the most significant as far as it correlates with the changes in the quality of life. Clinical interpretation of the changes in the sagittal balance should be conducted on the basis of their integration with a mandatory consideration of clinical manifestations and changes in the quality of life.

Purpose Evaluate the dynamics of the pyramidal pathway functional state in spinal deformity surgical correction in patients with genetically caused systemic skeletal pathology based on the formal criteria of a significance degree of the electrophysiological response to a surgical intervention which were developed at the RISC RTO. Materials and Methods The analyzed sample of patients included two groups for comparison. Group 1 (main group) were 28 patients with spinal deformities due to systemic hereditary diseases. Group 2 (control group) consisted of 126 patients with spinal deformities (51 subjects with idiopathic scoliosis, 71 subjects with deformities of congenital genesis, and 4 subjects of other etiology). All the patients underwent instrumental deformity correction with further fixation of the cervical, thoracic, thoracolumbar spine using different variants of internal transpedicular fixation systems. The changes in motor evoked potentials (MEP) were evaluated as ranks 0 to 7 according to the severity of the response observed. Results All the variants of changes in MEPs were observed both in the main group and in the control one. Five types of steady (reproduced in different patients) rank combinations were identified which conformed to the main types of the patient’s motor system response to spinal deformity surgical correction. Conclusion The use of the technology of MEP recording during surgical correction of spinal deformities in patients with systemic genetically caused skeletal pathology is a highly effective tool of preventing the development of intraoperative neurological complications. The proposed system of formal evaluation of the results of neuromonitoring allowed us to conduct a quantitative comparison of the character of intraoperative changes in the functional state of the pyramidal system in patients of different etiological groups depending on their age and intervention duration.

Study Design A descriptive study based on medical records of patients with congenital cervical spine abnormalities. Objective The hypothesis of the study was that patients with congenital cervical spine abnormalities could be divided according to the main pathological syndrome. Summary of Background Data Abnormalities of the cervical spine belong to embryopathies and are a very heterogeneous group. The variety of this pathology made it hard to create a general classification based on a morphological approach. Materials Medical records of 68 patients with congenital cervical spine abnormalities were analyzed and were a clinical material for working out the algorithm of their management. Computer tomography, magnetic resonance imaging (MRI) and selective angiography were used to specify the abnormality structure and preoperative planning. Use of functional positioning was an important feature in all these investigations. Various techniques of surgical treatment such as halo, anterior and posterior fusion, decompression of the cerebral, spinal cord and cervical vertebral arteries, revision of the spinal canal, neurolysis, and meningolysis were used in 28 patients aged from 2 to 47 years old. Results All patients were divided according to leading pathological syndromes. Those were instability, stenosis and brain ischemia. Each group had its own important subgroup. The main surgical steps in management of complex congenital anomalies of the cervical spine were bone fusion or (and) decompression of the brain and spinal cord. Conclusions Selection of the main pathological syndrome or combination of syndromes is a simple and effective way for making the right decision when treating patients with congenital cervical spine abnormalities. Syndromic approach can be used for prognosis as well.

Purpose The purpose of the work was to study the clinical and neuroradiological features of segmental spinal dysgenesis (SSD), congenital anomaly with local hypogenesis of a spinal motion segment and the spinal cord; as well as the selection of an optimal tactics of treating this anomaly. Methods The authors reviewed the literature and present seven clinical cases of their own practice. Results The severity of clinical deficit correlates with the degree of morphological changes in the spinal cord, the level of dysgenesis, as well as with the presence of combined and concomitant developmental anomalies. Early decompression of neural structures and spine stabilization play an important role in improvement of treatment results but the immature bone structures in infants limit the possibilities of early instrumented fixation. Conclusion SSD is a specific developmental anomaly of the spine and spinal cord that features some clinical and radiological peculiarities. This anomaly fills the gap between the infant thoracolumbar kyphosis with segmental subluxation and the syndrome of caudal regression. Early diagnosis and individual approach to determination of an optimal period of surgical treatment and tactics are required in each case

The paper presents a review of 28 domestic and foreign publications with the level of evidence of not less than 2 reporting the treatment results in children with congenital monosegmental anomalies of the spine over the past 15 years. A total of 657 clinical cases were analyzed, including 593 literature cases and 64 cases from authors’ experience. Results of hemivertebra treatment in children using combined approach, extended posterior approach and local posterior approach with transpedicular excision of hemivertebra were analyzed. The following criteria were used for analysis: patient’s age at the time of treatment, magnitude of the local (segmental) curvature before and after surgery, percentage of correction, blood loss volume, duration of surgery, type and length of fixation, and nature and structure of complications. The advantages of transpedicular excision of abnormal vertebra through posterior approach were noted in terms of surgery duration, intraoperative blood loss, risk of neurological complications after comparable correction of deformity, and time required for patient activation and rehabilitation as compared with combined approach, as well as in terms of blood loss volume and correction of kyphotic component as compared with extended posterior approach.

Development of a quantitative indicator for the risk level of intraoperative iatrogenic motor disorders in the process of surgical correction of spinal deformity based on current neurophysiological monitoring data.288 patients 12.6±0.35 y.o. underwent surgical correction of spinal deformities under the control of intraoperative neuromonitoring. The nature of changes in motor evoked potentials was assessed according to the earlier proposed ranking scale. The incidence of different variants of changes in the rank values of the state of the pyramidal system during the operation and the resulting postoperative motor disturbances was calculated.By comparing probabilities of various changes in the conduction properties of pyramidal tracts during surgery with the incidence of the observed motor deficiencies we quantitatively assessed the possible correlation between these phenomena. We propose a method for calculating the risk index for postoperative motor disorders depending on the maximum rank of the pyramidal system's response to surgical aggression.The developed system of ranking evaluation of changes in motor evoked potentials during surgical correction of spinal deformity makes it possible to quantify the risk of postoperative motor disorders and, accordingly, to monitor the level of anxiety for a neurosurgeon during individual stages of surgical intervention.Цель исследования - разработка количественного показателя уровня риска интраоперационного возникновения ятрогенных моторных расстройств в процессе хирургической коррекции деформаций позвоночника на основе данных текущего нейрофизиологического контроля. Материал и методы. 288 больным (12,6±0,35 года) под контролем интраоперационного нейромониторинга проведена хирургическая коррекция деформаций позвоночника. Характер изменения моторных вызванных потенциалов оценивался по предложенной ранее ранговой шкале. Рассчитывалась частота встречаемости разных вариантов изменений значений рангов состояния пирамидной системы в процессе операции и появления в связи с ними послеоперационных моторных нарушений. Результаты. Путем сопоставления вероятностей разной степени изменений проводниковых свойств пирамидных путей во время операции с частотой наблюдений послеоперационного моторного дефицита количественно оценивалась возможная связь между этими феноменами. Предложен способ расчета показателя риска возникновения послеоперационных моторных расстройств в зависимости от максимального ранга реакции пирамидной системы на хирургическую агрессию. Выводы. Разработанная нами система ранговой оценки изменений моторных вызванных потенциалов в процессе хирургической коррекции деформаций позвоночника позволяет количественно выразить степень риска возникновения послеоперационных моторных расстройств и соответственно отслеживать уровень тревоги для нейрохирурга при проведении отдельных этапов оперативного вмешательства.

Study design Retrospective cohort study. Class IV of evidence. Introduction Spinal deformity in patients with mucopolysaccharidosis a problem requiring early treatment Material and methods We analyzed 7cases of mucopolysaccharidosis (MPS), treated in Ilizarov Center in 2012 – 2018. There were 1 patient with MPS IH-type, 3 patients with IVA-type and 3 patients with MPS type VI. The age of the patients ranged from 6 to 26 years-old (average age - 10.3 years-old). All patients had spinal deformity with primary and/or secondary stenosis of the spinal canal. Various surgical treatments were used: 1) two staged surgery was perfomed in one patient with cervical myelopathy and scoliosis: C0-C7 decompression with occipitospondylodesis (OSD) firstly and dual growing rod construction secondly; 2) final fusion in 2 patients with scoliosis; 3) decompression at the stenosis level (cervical spine) with OSD in 4 patients with cervical myelopathy. Results Scoliosis value was from 20° to 65° Cobb, kyphosis from 15° to 80° Cobb. Four patients had vertebral stenosis and myelopathy (Frankel C). All patients had pulmonary and cardiac dysfunctions: vital capacity of the lungs was from 21% to 50% and abnormal ECG. Imbalance in the frontal and sagittal planes was present in 60% of patients. Through surgical treatment correction of the kyphosis was by 68% and scoliosis by 85%. In 4 (50%) patients with spinal stenosis neurologic status improved to Frankel E. Conclusion Patients with MPS quite often have vertebral stenosis and myelopathy, in such patients with combined stenosis of the spinal canal, it is necessary to combine decompression and fusion.

Objective. Evaluation of skin sensitivity and analysis of morphological changes in paravertebral muscles and back skin in kyphoscoliotic deformity projection in patients with type 1 neurofibromatosis (NF-1). Materials and methods. Ten NF-1 patients who underwent surgery to treat kyphoscoliosis were examined. Using an electrical esthesiometer thermal pain sensitivity before the surgery was studied in dermatomes corresponding to the apex of the deformity. Skin and muscle biopsy samples were collected intraoperatively in the projection of the apex of the deformity curve and were subsequently analyzed by light and scanning electron microscopy. Results. Patients with kyphoscoliosis with underlying NF-1 were characterized by abnormal thermal pain sensitivity, pathological structural changes in skin and muscles accompanied by disrupted innervation and blood supply. Discussion . The observed changes may be responsible for lowered postoperative reparative potential of tissues and they must be considered in prevention and prognosis of treatment and rehabilitation efficacy.

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.

Atlanto-axial rotatory fixation (AORF) develops on the background of acute torticollis. Widely adopted terms such as C1 subluxation or atlantooccipital rotational subluxation do not reflect the core of this pathology and carry negative weight in the diagnostics and treatment of AORF. Retrospective analysis of the diagnostics and treatment outcome of 5 children with confirmed AORF diagnosis and literature review were performed. Clinical method, radiography and functional computer tomography were used to verify the diagnosis. De-rotational halo-traction and open correction with screw fixation were applied for treatment. Head position was managed to be improved in all patients. In one case the reduction was performed using correction in suboccipital segments and in other 4 cases the correction and fixation by Harms and de-rotational halo-traction allowed to correct torticollis. The pain syndrome had been arrested completely. Disease outcome resulted in formation of C1-C2 fibrous or bone fusion regardless the method of treatment. The patients with neglected AORF represent a great challenge for diagnostics and treatment. When conservative treatment fails it is necessary to involve de-rotational halo-traction with possible application of open reduction and posterior fusion. The purpose of treatment is to eliminate torticollis and pain using creation of proper C1-C2 alignment. The motions in atlantooccipital joint do not restore due to formation of the fibrous or bone fusion.

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.