Statistical data on pediatric congenital musculoskeletal anomalies (malformations) in St. Petersburg

Introduction A scientifically-based knowledge on the incidence, dynamics of detection and structure of congenital malformations of the musculoskeletal system (CMMS) has both practical and scientific significance. For many years, the Turner Institute has close scientific and practical ties with the state public health institution "Diagnostic Medical and Genetic Center" of St. Petersburg which carries out a regional monitoring of congenital disorders in St.Petersburg. Our objective was to present regional statistical data on the CMMS detection and structure in newborns and children of the first and second years of life, morbidity and disability in children aged 0-17 years in connection with congenital malformations of the musculoskeletal system, to study the proportion of CMMS patients in the orthopedic clinic and their need for surgical, including high-tech, treatment, and dispensary follow-ups in outpatient orthopaedic settings. Material and methods Regional statistics of St. Petersburg on prevalence, dynamics and structure of congenital malformations of the musculoskeletal system in children born in St. Petersburg in 2001–2015 were analyzed. The statistical data on the prevalence of congenital malformations of the musculoskeletal system in children aged 0–17 years in St. Petersburg were studied. Based on the studies conducted at the Turner Institute in 2014–2016, the proportion of patients treated for congenital malformations of the musculoskeletal system was revealed as well as their need for surgical treatment. Peculiarities of dispensary follow-ups of CMMS patients at orthopedic consultations of children's clinics of St. Petersburg were analyzed. Results and discussion The prevalence and structure of congenital malformations of the musculoskeletal system in 783,000 children born in St. Petersburg in the period of 2001–2015 are presented in accordance with blocks Q65–Q79 of ICD-10. Diagnostic results of congenital malformations of the musculoskeletal system in newborns (2.70 ‰) and in children of the same group until they reached three years of age (4.21 ‰) were studied in dynamics. Infacnts and children up to 17 years old with congenital malformations of the musculoskeletal system are under regular supervision of orthopedic surgeons. Disability due to congenital anomalies and malformations takes the third place among all the diseases detected in patients aged 0-17 years, established by pediatric medical and social expert boards of St. Petersburg. At the clinic of the Turner Institute, the proportion of patients with CMMS among all admitted patients was 38.6 %. Among them, 78 % of patients with CMMS received surgical treatment, of which 80 % of operations were performed using high technologies. Conclusions Statistics on congenital malformations of the musculoskeletal system in newborns and children under 3 years old and a high level of their disability shows the need in a specialized care for these children, including high-tech surgical treatment

Особенности электрофизиологической активности коры больших полушарий мозга у детей с артрогрипозом

Background. Arthrogryposis is one of the most severe congenital abnormalities of the musculoskeletal system characterized by 2 or more contractures of the large joints, muscle and anterior grey column pathology. One of the main problems making selfcare limited or impossible for the patients is an absence of the active movements in the joints of the upper extremities which can be restored through autologous transplantation from the various donor areas. Processes of the rehabilitation after these operations are associated with neuronal remodeling in the central nervous system both in the spinal cord and the brain, in the cortial regions in particular.The objective is to evaluate possible reflection of arthrogryposis in the amplitude and neurodynamical characteristics of the electroencephalogram (EEG) in children.Materials and methods. Electrophysiological characteristics of the cerebral cortex in children with arthrogryposis and healthy children of the same age were examined. Such EEG characteristics as power and long-range temporal correlations (evaluation of the neuronal activity dynamics) in ranges of 4–8, 8–12, and 12–16 Hz were measured. The results were evaluated in accordance with clinical scales.Results. Data analysis has shown that children with arthrogryposis have significantly decreased EEG power in all of the studied ranges compared to healthy children. Additionally, a significant correlation between EEG power and the level of restoration of motor functions in the upper extremities after autologous transplantation of various muscle groups in the position of the biceps was observed. The obtained results reflect correlation between the electrophysiological parameters of the cerebral cortex and processes associated with arthrogryposis pathology. However, neurodynamical parameters in children with arthrogryposis are similar to those in healthy children. The results allow to state that arthrogryposis is reflected through decreased electrical activity of the cerebral cortex in 4–16 Hz range with preservation of neurodynamic characteristics typical for disease-free children.Conclusion. In this study, a significant difference in EEG power in 4–8, 8–12, and 12–16 Hz ranges between children with arthrogryposis and healthy children was demonstrated. However, there was no difference in such an important neurodynamical characteristic as longrange temporal correlations. It is possible that decreased amplitude of EEG rhythms in children with arthrogryposis is caused by their lower motor activity in general.Введение. Артрогрипоз – один из наиболее тяжелых врожденных пороков развития опорно-двигательного аппарата, характеризующийся наличием 2 и более контрактур крупных суставов, поражением мышц, а также передних рогов спинного мозга. Одной из основных проблем, обусловливающих ограничение или невозможность самообслуживания пациентов, является отсутствие активных движений в суставах верхних конечностей, которое восстанавливается путем аутотрансплантации мышц различных донорских областей. Процессы реабилитации после таких операций связаны в том числе и с нейрональными перестройками в центральной нервной системе как в спинном, так и в головном мозге, в частности в корковых его отделах.Цель исследования – оценить возможное отражение заболевания артрогрипозом у детей в амплитудных и нейродинамических показателях электроэнцефалограммы (ЭЭГ).Материалы и методы. Изучали электрофизиологические показатели активности коры головного мозга у детей с диагнозом артрогрипоза и здоровых детей сходного возраста. Оценивали такие показатели ЭЭГ, как мощность и длинновременные корреляции (метод оценки динамики нейрональной активности) в диапазонах 4–8, 8–12 и 12–16 Гц. Поражения оценивали на основе клинических шкал.Результаты. Анализ данных показал, что у детей с артрогрипозом, по сравнению с детьми без патологий, имеется достоверное снижение мощности ЭЭГ по всем исследованным частотным диапазонам. Кроме того, продемонстрирована достоверная корреляция мощности ЭЭГ со степенью восстановления двигательных функций верхних конечностей после операций по аутотрансплантации различных групп мышц в позицию двуглавой мышцы плеча. Полученные результаты отражают корреляцию электрофизиологических параметров коры головного мозга с процессами, связанными с патологией артрогрипоза. При этом нейродинамические параметры у детей с артрогрипозом не отличаются от таковых у здоровых детей. По результатам можно констатировать факт отражения заболевания артрогрипозом в снижении электрической активности коры больших полушарий головного мозга в частотном диапазоне 4–16 Гц при сохранении нейродинамических показателей, сходных с группой детей без заболевания.Заключение. В данной работе показано достоверное отличие мощности ЭЭГ в диапазонах 4–8, 8–12 и 12–16 Гц у детей с артрогрипозом и здоровых детей. Однако разницы в таком важном нейродинамическом показателе, как длинновременные корреляции, не обнаружено. Возможно, факт снижения амплитуды ритмов в ЭЭГ у больных детей объясняется их более низкой общей моторной активностью

ULTRASONOGRAPHY OF HIP JOINTS IN SRTUCTURE OF NEWBORN ORTHOPEDIC SCREENING (REVIEW)

The problems of epidemiology and pathogenesis of hip dysplasia, advantages and disadvantages of ultrasound diagnostics of a developmental hip dysplasia of the newborn period

TREATMENT OF THE LOW LIMB DEFORMITIES IN YOUNG CHILDREN WITH ARTHROGRYPOSIS (REVIEW)

The review of domestic and foreign literature on a problem of treatment of the low limb deformities in young children with arthrogryposis is revealed in the article. Questions of medical tactics and its peculiarities in one and both sided congenital hip dislocation are touched upon. Basic worldwide tends of hip surgery development and effectiveness of different methods in arthrogriposis are revealed. Views of domestic and foreign authors on a problem of knee joint contractures treatment are represented and different conservative and surgical methods according to severity of deformities are described. Results of clubfoot treatment of different authors are shown in the article. Ponseti method and its possibilities for clubfoot correction in arthrogryposis are specified. Questions of treatment of planovalgus deformities are also touched upon as well as problems of orthotic supply in accordance with type of the deformities and its combination

POSTBURN CICATRICAL FOOT DEFORMITIES IN CHILDREN: CLINICAL ASPECTS AND MANAGEMENT

The cicatrical deformities of the foot and secondary changes in tendons, muscles and in osteoarticular system with respect to the age and scar location were discussed. It was pointed to a high risk of early development of foot deformities, especially in young children up to five years of age. This indicates the need for regular medical check-up. Clinical experience shows that the hypertrophic scars that extend from the lateral surfaces of the foot to the ankle region create preconditions for development of multiplanar foot deformities, especially in the period of intensive growth of the skeleton. Possible complications in the treatment of multiplanar foot deformities in the presence of scar lesions of surrounding tissues associated with an increased risk of trophic disorders and the development of the inflammatory process are considered. Recommendations on stages of surgical treatment are given

REHABILITATION OF CHILDREN WITH CICATRICIAL CONSEQUENCES OF BURNS: FEATURES, ERRORS, POSSIBLE SOLUTIONS

Late results of treatment of thermal injury and its sequelae are presented. According the data obtained, the most severe deformities developing after a thermal injury are the flexion contractures of fingers. The results of the study are evidence of a cardinal importance of an appropriate immobilization of the affected segments and of early functional rehabilitation for prevention of contractures development. Without dynamic keeping the children under observation, secondary skeletal deformities develop which are changes in form of bones and their hypoplasia. This enhances the number and volume of reconstructive procedures needed as well as deteriorates the final outcome of the treatment

SYMPTOM COMPLEX OF CHRONICAL ATLANTOAXIAL SUBLUXATION IN PAEDIATRIC PATIENTS

The objective of this study is to determine the complex of symptoms, including both orthopedic and neurological changes that occur against the backdrop of long-existing disturbances of the atlanto-axial joint. The authors conducted in-depth analysis of the orthopedic and neurological examination of 58 children aged between 10 and 17 years with first diagnosed disturbances of relationship between C1 and C2 vertebrae. Complexes of symptoms are identified from the combination of clinical data obtained with in-depth orthopedic and neurological examinations that are pathognomonic for lateral and posterior atlanto-axial chronic subluxation. Rotational subluxation of C1-C2 vertebral-motor segment was associated with concomitant neurological disorders and additionally with the no gross structural deformity of the spine and the disturbed foot support function in all cases. Using of the study findings will allow to make an adequate diagnosis and to determine the correct choice of additional diagnostic and therapeutic methods for this category of patients on the stage of conventional examination of the patient in clinical practice

Surgical treatment of children with scars on the lower leg and in the area of Achilles tendon using expander dermatension

Treatment of scar deformities of shin and ankle is traditionally a challenge due to significant functional load to the segment and the surface location of the Achilles tendon. Published data indicates that the use of expander dermotension of shin is not widespread and poorly covered in the literature [6]. Available data in the literature devoted to the expander dermotension of shin, are characterized by individual observations. There are no clear guidelines for size selection of the expander, for the protocol of its filling and postoperative regime. The purpose was to study the peculiarities of tissue dermotension of shin and development of recommendations for the use of this method to recover the full skin of shin and area of the Achilles tendon. Materials and Methods. Full restoration of the skin on the leg in the area of the Achilles tendon using tissue dermotension was performed in 24 patients in the clinic of Turner Scientific and Research Institute for Children’s Orthopedics from 2009 to 2014. To perform dermotension we used tissue expanders Eurosilicone (France), st. reg. № FSZ 2010/07171 from 09.06.2010; atraumatic suture material. Results. Complications amounted to 12.5% of all observations, and included: migration of port expander, marginal necrosis on line surgical suture and transient swelling of the foot. Efficacy of treatment was evaluated according to the following criteria: 1) the restoration of full cover in the region of the scar deformation; 2) elimination of adhesions, restore slip anatomical structures; 3) the increase of the amplitude of motion of the ankle joint. All patients achieved clinical improvement 2-3 criteria that considered a good result. Complications did not have a significant impact on the final result of the treatment. Conclusions: 1. Expandera dermatensia is an effective way to full recovery of the skin on the calf and the Achilles tendon. 2. Capsule forming around the expander and part of the flap, which helps eliminate adhesions and restoring slip anatomical structures and to increase range of motion in the ankle joint