Vol 10, No 1 (2022)

BACKGROUND: Pathology of the musculoskeletal system ranks first among health disorders of the younger generation. The frequency of posture disorders, scoliosis, and flat feet increases as the child grows older and requires careful monitoring, preventive measures, and long-term rehabilitation measures.

AIM: This investigation studies the formation frequency of lesions of the musculoskeletal system lesions, considering the somatic pathology in adolescent children brought up under various conditions, and addresses the issue of preventive and corrective measures.

MATERIALS AND METHODS: The main group consisted of schoolchildren aged 11–15 from social institutions (n = 60). The comparison group included children from complete families (n = 60). The health assessment was conducted in accordance with the methodological recommendations developed at the Research Institute of Hygiene of Children and Adolescents. The material was copied from forms 112/y, 003/y, 026/y, and the clinical examination results and the conclusions of other specialists were collected. Statistics were evaluated by the Pearson’s χ²-criterion with the Yates correction, with values of p < 0.05.

RESULTS: The health of children from social institutions was significantly worse than that of complete families (p = 0.04). They were 4.8 times more likely to form chronic diseases (p = 0.04), especially those of the central nervous and musculoskeletal systems, digestive organs, blood circulation, and the ear, nose, and throat (ENT) organs were leading (p = 0.001). Lesions of the musculoskeletal system were more often combined (p = 0.02). In the comparison group, functional disorders occurred more often (p = 0.04), and digestive and circulatory organ diseases prevailed. Pathology of the musculoskeletal system occupied the third position and was significantly less common (p = 0.0001).

CONCLUSIONS: The health of children from social institutions is worse than that of schoolchildren from complete families. In them, lesions of the musculoskeletal system occupy second place, the frequency of combined lesions is higher, the increase in orthopedic pathology occurs mainly because of scoliosis, flat feet, and posture disorders. Neurodysplastic and idiopathic forms prevail in the structure of scoliosis. In children with scoliosis, diseases of the central nervous system, digestive organs, and blood circulation are more often registered. In children with flat feet, lesions of the digestive and circulatory organs are more often diagnosed. In children with impaired posture, diseases of ENT organs, circulatory organs, and vision are more often detected. Therefore, the musculoskeletal system pathology must be considered as an interdisciplinary problem, requiring a comprehensive rehabilitation program that involves other specialists.

BACKGROUND: Multiplanar deformities of the proximal femur in children are often accompanied by a high position of the greater trochanter, causing disturbances in the biomechanics of the hip joint and extra-articular impingement syndrome. Progressive anatomical and biomechanical changes in multiplanar deformities of the proximal femur also lead to changes in the system hip joints, the pelvis, and the lumbosacral spine that mutually burden each other. Currently, the domestic literature has isolated publications on assessing the state of sagittal vertebral-pelvic ratios in children with this pathology.

AIM: This study assesses the radiological parameters of sagittal balance in children with multiplanar deformities of the proximal femur with a high position of the greater trochanter. It reveals the relationship between the deformity severity of the proximal femur and spinal and pelvic parameter changes in children.

MATERIALS AND METHODS: The x-ray data of examination of 25 children (25 affected joints) aged from 9 to 15 years with deformities of the proximal femur with a high position of the greater trochanter, in which its apex is located at or above the upper pole of the femoral head, were analyzed. The parameters characterizing the ratio of the femoral head to the greater trochanter in the frontal plane and indicators of the sagittal balance according to lateral skeleton radiographs were evaluated. The obtained data were subjected to statistical processing.

RESULTS: Children with multiplanar deformities of the proximal femur with a high position of the greater trochanter are characterized by a pronounced increase in global lumbar lordosis, excessive pelvic anteversion, and the pelvic tilt toward the affected limb. A direct relationship was found between the severity of the proximal femur disorders and the degree of change in the sagittal spinal-pelvic ratio indices.

CONCLUSIONS: The combination and progression of anatomical changes in the hip joints in children with a high position of the greater trochanter cause pathological compensatory changes in the lumbosacral spine with degenerative dystrophic processes development.

BACKGROUND: Ciliopathies include the large group of hereditary diseases caused by mutations in the genes encoding primary cilia components. The largest type of skeletal ciliopathies is short-rib thoracic dysplasia.

AIM: This study describes the clinical and genetic characteristics of Russian patients with STRD with or without polydactyly caused by mutations in the genes DYNC2H1, DYNC2I2, IFT80, and IFT140.

MATERIALS AND METHODS: A comprehensive examination of 10 unrelated children aged from 9 days to 9 years, with phenotypic signs of short-rib thoracic dysplasia with or without polydactyly, was conducted. The diagnosis was confirmed using genealogical analysis, clinical examination, neurological examination, radiography, and targeted sequencing of a panel consisting of 166 genes responsible for the development of inherited skeletal pathology.

RESULTS: As a result of the molecular genetic analysis, four short-rib thoracic dysplasia genetic variants were identified. Seven patients were diagnosed with short-rib thoracic dysplasia type 3, and three unique patients were diagnosed with types 11, 2, and 9 due to mutations in the DYNC2H1 and DYNC2I2, IFT80, and IFT140 genes, respectively. From the 14 detected variants, six were identified for the first time. As in the previously described patient samples, in the analyzed sample, more than half of the cases were due to a mutation in the DYNC2H1 gene, which is responsible for the SRTD type 3. The differences in the severity of clinical manifestations and the disease course in patients with mutations in certain regions of the gene, which have a different effect on its protein product function, have been shown.

CONCLUSIONS: The results of this molecular genetic study broaden the spectrum of mutations in the DYNC2H1, DYNC212, and IFT140 genes causing short-rib thoracic dysplasia and confirm the usefulness of the whole-exome sequencing as the most informative method for identifying mutations of the genetically heterogeneous short-rib thoracic dysplasia group.

BACKGROUND: One of the most debatable issues in osteochondritis dissecans (OCD) research is bone and cartilage healing assessment during OCD postoperative management. The x-ray scale developed by Wall and colleagues is a commonly used evaluation tool for OCD lesion assessment. This tool has excellent reliability but is associated with radiologic exposure. Also, it provides complete information about bone structure only, even though the articular cartilage is also involved in the pathological process. Lack of cartilage assessment combined with radiation exposure facilitated the development of the novel MRI-based OCD healing assessment tool. It could draw attention to bone and cartilage during healing assessment to improve decision-making in the postoperative period after OCD treatment.

AIM: This study assesses the reliability of a developed novel MRI-based OCD healing assessment tool.

MATERIALS AND METHODS: Ten patients with OCD of the femoral condyle were involved in the current study. A reliability test for the novel MRI-based assessment tool was performed with the expert group comprising six participants to assess 34 MRI studies of 10 patients. From all studies, one study was obligatory for each patient before the operative treatment, and a postoperative MRI study series was performed during the first postoperative year. Each MRI study was examined by each expert twice with a 4-week time lag. The novel MRI-based assessment tool consists of five criteria, of which the common criterion was “general healing,” incorporating all previously described ones. Each criterion was tested, and a two-way mixed-effects intraclass correlation coefficient (ICC) was used to assess intraobserver and interobserver reliability.

RESULTS: The main parameter “general healing” calculations were made first. Two patients achieved full OCD lesion healing with 100 scale points and two patients with 97.5 and 98.5 points, respectively. Other patients reached the cut-off value of 75 points and were defined as “healed with minimally detectable changes on MRI.” Second, a two-way mixed-effects ICC calculation was performed. The “bone marrow extension” parameter reached the value of 0.972, “the extent of the union” – 0.984, “bone structure” – 0.977, and “articular cartilage intensity and structure” – 0.977. The general healing parameter reached the value of 0.993. These values corresponded to the excellent marks according to the guidelines for ICC assessment. The novel MRI-based assessment tool showed excellent intraobserver and interobserver reliability.

CONCLUSIONS: The novel MRI-based assessment tool permits assessing bony and cartilage structures while making decisions about OCD lesion healing in the postoperative period. The novel OCD healing assessment tool has excellent intraobserver and interobserver reliability. Also, it is recommended for use in clinical and research practice since a study revealed a correlation of the MRI healing score with that of the clinical assessment tool.

BACKGROUND: Victims with severe burn injuries require specialized and high-tech care. The treatment results of such patients, as a rule, depend on the timing of the transfer and the volume of surgical treatment aimed at removing necrotic tissues and restoring the skin in conditions of a shortage of donor resources. In this regard, the development and implementation into clinical practice of innovative techniques for the temporary closure of burn wounds with synthetic coatings and the creation of optimal conditions in the wound for engraftment of skin grafts with a high perforation index.

СLINICAL CASE: This paper demonstrates a clinical case of successfully treating a child with a critical area of deep burn injury by flame (“carriage trauma”) using early surgical treatment, innovative wound dressings, and cellular technologies.

DISCUSSION: We consider the transfer to a specialized department of a multidisciplinary hospital essential in successfully treating a patient with a large burn area. Under burn center conditions, surgical treatment was performed to remove the necrotic tissues in the largest part of the deep lesion before inflammatory reactions developed. Subsequently, active surgical techniques were used to prepare the wounds for delayed plastic closure. The use of wound dressings and the introduction of cell technologies into clinical practice ensured successful patient treatment due to the early adaptation of skin autografts and risk minimization of their regression, and acceleration of epithelialization in the cells.

CONCLUSIONS: The early transfer of a severely burned child to a specialized burn center, active surgical techniques, the use of innovative wound dressings and cellular technologies in the treatment of a patient with a critical area of deep burn lesions made it possible to save a life, successfully restore damaged skin, and create optimal conditions for starting early rehabilitation measures.

BACKGROUND: Erythromelalgia is a severe, chronic, progressive disease with periods of exacerbation and remission. A triad of symptoms characterizes the disease: reddening of the extremities, a local increase in skin temperature, and pronounced neuropathic pain syndrome. There are sporadic works in the Russian literature that present data on erythromelalgia, particularly in children. The publications are descriptions of clinical observations with the assessment of the clinical picture of the patient regarding cutaneous manifestations and surgical care at the time of hospitalization, time spent in the hospital, and during the period of his chronic disease exacerbation.

CLINICAL CASE: A clinical case of hereditary erythromelalgia in a 15-year-old adolescent with a detailed description of the disease course since the initial manifestation is presented.

DISCUSSION: During three and a half years, despite early diagnosis and application of consistent pharmacotherapy including nonsteroidal anti-inflammatory drugs, antidepressants, anticonvulsants, antihistamines, opioids, hormonal therapy, local use of lidocaine, ointment with silver content, the disease was progressive, with the resistance of pain syndrome to the treatment, with periods of exacerbation and partial remission.

CONCLUSIONS: The presented clinical observations show the need to assess the patient as a chronic and intractable patient. Considering the lack of understanding of the apparent cause of this disease and its diverse manifestations in the clinical picture, a multidisciplinary approach with a search for new treatment methods, including neurosurgical techniques of chronic pain treatment, is required for patients with erythromelalgia.

BACKGROUND: Treating patients with open forearm fractures is always a difficult task. The complexity of treating these patients is related to the anatomical and biomechanical specifics of the segment. In many cases, fragmented, unstable fractures have serious soft tissue damage due to high-energy trauma, blood supply failure, the risk of infectious complications, prolonged wound healing, compartment syndrome development, delayed union, and contracture formation.

AIM: This article reviews the modern native and foreign literature regarding the treatment of patients with open diaphyseal forearm fractures and identifies the main problems in these injuries.

MATERIALS AND METHODS: The analysis of modern Russian and foreign literature concerning the treatment of patients with open diaphyseal forearm fractures is presented. The analysis was performed using the medical publication databases of CyberLeninka, eLibrary, and PubMed without limiting the search depth.

RESULTS: The high percentage of unsatisfactory results of open fractures, both anatomical and functional, especially of severe type III fractures, presents a problem. This requires the developing methods to achieve fusion to avoid infectious complications and obtain a good functional result.

CONCLUSIONS: Surgical wound management, stabilization of the fracture in the external fixation apparatus, prevention of infection with antibiotics, and early soft tissue coverage are necessary treatment steps to reduce the infection risk. Conversion of the device to submerged fixation allows early functional treatment, including rotational movement restoration. The choice of fixation method, the timing of the conversion, and the type of internal fixation remain a matter of debate.

On January 20, 2022, Mikhail Pavlovich Konyukhov, a remarkable person, doctor, and scientist, Doctor of Medical Sciences, professor, Honored Doctor of the Russian Federation, orthopedic and trauma surgeon of the highest qualification category of H. Turner National Medical Research Center for Сhildren’s Orthopedics and Trauma Surgery, died at the age of 85.