The aim of the studyis to evaluate the efficiency of triple arthrodesis of foot and its effect on soft tissues blood supply at the surgical site during simultaneous correction of segment deformity in patients with cerebral palsy.Material and Methods. The present study reflects the authors’ experience of triple arthrodesis for correction and stabilization of foot multicomponent deformities of varying severity in 75 patients (136 feet) with cerebral palsy (IIIV level by Gross Motor Function Classification System (GMFCS)) treated in the Ilizarov center in the period from April 2012 to December 2016. The average age of the patients was 16.4±4.3 years (from 11 years 8 months to 43 years 3 months). All patients included into the study had severe arthrosis of hind and midfoot. The main option of foot fixation in this group of patients was internal fixation (elastic threaded wires, compression screws) together with plaster cast immobilization for 6–8 weeks. All patients underwent average of 4.59 surgical elements during a procedure as part of simultaneous multilevel interventions. The blood supply at the surgical site was evaluated by laser and high-frequency Doppler flowmetry before and after all stages of the surgery.Results.Long-term outcomes were evaluated at the average of 19 months after the surgery in 56 (74.7%) patients. 37 patients (66.1%) demonstrated good treatment outcomes and 19 patients (33.9%) — satisfactory outcomes. No unsatisfactory outcomes were observed. The clinical outcome of foot surgery was evaluated using the Angus-Cowell criteria. The obtained significant x-ray enhancement was maintained at the control stages of the follow up. Despite large simultaneous correction of foot deformity, there was no decrease in the parameters of microcirculatory blood supply of the skin, muscles and subcutaneous fat of the foot. The authors observed a stabilized or an increased perfusion of soft tissues.Conclusion.Triple arthrodesis for correction of foot deformities in patients with cerebral palsy and severe arthrosis in hind and midfoot is an efficient method which allows to correct and stabilize gained position of segments. The data of physiological research testify the sparing approach of such procedure and a possibility of an earlier weight-bearing on operated limb.
The constantly changing information about the genetic nature of osteogenesis imperfecta (OI), new approaches to classification and diagnosis, and the growing interest in this pathology require careful analysis and generalization of the available data in the recent literature. Purpose Analysis of recent literature on epidemiology, pathogenesis, etiology, classification of osteogenesis imperfecta. Materials and methods The literature search was carried out in the scientific literature databases of PubMed, Web of Science, Scopus, eLIBRARY, RSCI, and references of the key works. The following inclusion criteria were used: literature reviews, review articles, multicenter studies, cohort studies of patients with osteogenesis imperfecta. Results This systematic review included 18 publications that met the inclusion criteria. All selected publications were published within the period from 2012 to 2020. The study designs were review articles or literature reviews. Discussion Osteogenesis imperfecta is one of the most common types of skeletal dysplasia, a phenotypically and genetically heterogeneous group of hereditary bone diseases. OI is characterized by high variability of manifestations, even within the same type and one family. The results of the analysis of the prevalence of this disease in different population groups remain not fully covered. The degree of genetic heterogeneity of the disease has not yet been determined. Research is ongoing to identify new genes involved in the pathogenesis of OI. The introduction of modern methods for determining mutations in genes contributed to the progress of research into the molecular pathogenesis of osteogenesis imperfecta, and identification of new genes, mutations in which lead to OI. The large number of causative genes complicates the process of creating an optimal classification of OI. The difficulty of creating a comprehensive classification of OI subtypes is the fact that there is no clear phenotype-genotype relationship; based on the mutation, conclusions about its clinical severity cannot be drawn. Conclusion The classification of various types of osteogenesis imperfecta is still the subject of ongoing debate and research.
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