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Vol 10, No 3 (2022)

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Clinical studies

Assessment of the efficacy of treatment for children with congenital scoliosis with unsegmented bar and rib synostosis

Vissarionov S.V., Asadulaev M.S., Orlova E.A., Ivanova P.A., Shabunin A.S., Murashko T.V., Khardikov M.A., Toriya V.G., Rybinskikh T.S., Rodionova K.N., Kokushin D.N.

Abstract

BACKGROUND: Treatment of children with congenital deformity with unsegmented rod and rib synostosis is an important and topical problem to date. Topical publications present the results of surgical correction efficacy and analysis of treatment complications. The extremely important aspect of treatment efficacy assessment regarding changes in the function of external respiration is still topical.

AIM: This study aimed to analyze the treatment results of children with congenital scoliosis and unilateral segmentation disorder of the lateral surfaces of the vertebral bodies and rib synostosis.

MATERIALS AND METHODS: This is a retrospective monocenter cohort study of the treatment outcomes of 30 patients aged 1–14 years. In the preoperative period, external respiration was evaluated by pulse oscillometry, multi-slice computed tomography, digital X-ray imaging of the craniopelvis in two projections. All patients underwent expanding thoracoplasty with osteotomy of the rib synostosis and fixation with a rib-costal or rib-vertebral distractor. Control studies were performed every 6 months after the surgical intervention. The average follow-up period was 2 years. Nonparametric analysis was applied to estimate the obtained data.

RESULTS: The median (Me) age at the start of treatment was 6 years (interquartile range, 4.25; IQR hereafter). The Me scoliosis before treatment was 74° (IQR, 22.75). The Me scoliosis correction after the first stage of treatment was 16° (IQR, 11) and the second correction achieved 6° (IQR, 13). The Me kyphosis was 15° (IQR, 32), the first intervention improved kyphosis by 4° (IQR, 16), and the second by 6° (IQR, 11).

Complications were represented by the destabilization of the metal construct in six cases, and trophic disorders of soft tissues were noted in four. The assessment of external respiratory function using IOM demonstrates reliable improvement of resistive component, reactive component, and frequency dependence of the resistive component (p < 0,01).

CONCLUSIONS: The assessment of the external respiratory function in young children and analysis of reference values may allow their use as absolute indications for surgical treatment in children with congenital scoliosis with unsegmented bar and rib synostosis.

Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2022;10(3):211-221
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Features of the reparative osteogenesis of the distraction of the tibial regenerate and osteotropic growth factors in patients with achondroplasia at the age of 9–12 years

Lyneva S.N., Menschchikova T.I., Aranovich A.M.

Abstract

BACKGROUND: Despite studies on various issues of distraction osteosynthesis, many morphological aspects of this problem are still insufficiently studied and remained debatable.

AIM: To determine the features of the reparative activity of the regenerate and analyze the content of some osteotropic growth factors in children with achondroplasia.

MATERIALS AND METHODS: Growth factors were determined in serum and blood plasma using equipment from Thermo Fisher (USA). Factor concentrations were determined using ELISA kits: PDGF-AA (R&D Systems, USA), PDGF-BB (R&D Systems, USA), IGF-1 (Immunodiagnostic systems, USA), IGF-2 (Mediagnost, Germany), TGF-β1 (eBioscience, USA), and TGF-β2 (eBioscience, USA). The structural state of the tibial regenerate was determined using by ultrasonography (HITACHI, Japan). Patients with achondroplasia aged 9–12 years (n = 32) were examined at the beginning of distraction (10–20 days), in the middle of distraction (21–40 days), and at the end of distraction (41–63 days).

RESULTS: The ultrasound method showed the dynamic formation of the structural state of the distraction regenerate at the studied stages of distraction. At the same stages of distraction, the concentration of osteotropic growth factors was assessed.

CONCLUSIONS: The serum content of osteotropic growth factors in the blood of children with achondroplasia differs from age-normative values. Growth factors that play a key role in osteogenesis, IGF-1, BMP-4, TGF-β1, and TGF-β2 were reduced, whereas the expressions of IGF-2 and BMP-6 were compensatory increased. At the end of the distraction period, the values of all studied growth factors exceeded the initial values, regardless of their preoperative values and their dynamics at the stages of distraction. The assessment of the dynamics of the concentration of osteotropic growth factors in the blood of patients with achondroplasia during the distraction period and natural growth period indicate the presence of a commonality of processes during the distraction period and prenatal growth of the tibia. Our comprehensive ultrasound study of the structural state of the distraction regenerate of the tibia and biochemical studies of growth factors in the blood of patients with achondroplasia at the age of 9–12 years made it possible to identify the features of reparative osteogenesis of the distraction regenerate of the tibia and the physiological effect of osteotropic growth factors from the viewpoint of the process of reparative regeneration.

Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2022;10(3):223-234
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Reliability of the computed tomography criteria after closed reduction of developmental dislocation of the hip

Khaled L.E., Hesham T.K., Amin A.R., Mohamed A.

Abstract

BACKGROUND: Developmental dislocation of the hip includes femoral head subluxation or dislocation and/or acetabular dysplasia. Closed reduction of the hip should be performed under general anesthesia. Appropriate performance and interpretation of closed reduction are difficult and require experience. The role of computed tomography (CT) in different aspects of treatment of developmental hip dysplasia is well established. It was an accurate way to assess the adequacy of reduction of dislocated hips for patients in spica casts.

AIM: This study aimed to assess the role of CT in the evaluation of closed reduction of developmental hip dislocation in infants and children immobilized in spica casts.

MATERIALS AND METHODS: This study included 16 patients with 20 involved hips who presented with developmental hip dysplasia. The youngest patient was 12 months old, and the oldest was 24 months old, with a mean age of 19.62 ± 4.27 months. There were 15 girls (93.75%) and one boy (6.25%). There were four patients with bilateral hip involvement (25%), and the right side was involved in five hips (31.25%), whereas the left side was affected in 7 (43.75%) hips.

RESULTS: Closed reduction was performed in 20 hips, and according to the post-reduction CT evaluation, the final results were satisfactory in 16 (80%) hips and unsatisfactory in 4 (20%) hips. On the coronal CT cuts, the modified Shenton’s line gave a sensitivity of 75%, specificity of 81.25%, and accuracy of 80%. Second, the calculation of femoral head coverage on coronal CT cuts showed the highest sensitivity of 100%, specificity of 50%, and accuracy of 60%. Lastly, the posterior neck line identified on the axial CT cuts gave a sensitivity of 75%, specificity of 87%, and accuracy of 85%. On comparing and evaluating the three methods, the method that gave the best level of reliability for the adequacy of the reduction was the posterior neckline (82.23 %), followed by modified Shenton’s line (78.75%), and finally femoral head coverage (70%).

CONCLUSIONS: The posterior neck line is the preferred method to confirm the adequacy of hip relocation on multi-slice post-reduction axial CT.

Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2022;10(3):235-245
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Treatment of fractures of the main phalanx of the fingers in children

Gordienko I.I., Tsap N.A., Kutepov S.M.

Abstract

BACKGROUND: Fractures of the bones of the hand and wrist account for 25% of all fractures in children, whereas the phalanges are the most common localization of these injuries and account for 15%–30% of all fractures of the upper limb. To fix fractures of the neck of the middle and main phalanx of the fingers, traumatologists resort to retrograde osteosynthesis with a spoke, which in all cases passes through the articular surface of the distal fragment, thereby blocking the joint adjacent to the fracture. This significantly complicated postoperative rehabilitation to restore movements.

AIM: This study aimed to comparatively analyze the results of extra-articular osteosynthesis of fractures of the distal metaphysis of the main phalanx of children’s fingers.

MATERIALS AND METHODS: A prospective cohort study included 52 children with fractures of the main phalanx of the fingers. The study cohort of children was divided into two groups. The main group included 29 children who underwent osteosynthesis of the distal fragment of the phalanx with spokes according to the author’s method without passing them through the distal or proximal interphalangeal joint. The comparison group included 23 children who, during osteosynthesis, had spokes carried out retrogradely, through the articular surface of the distal phalanx fragment. The total volume of the restored active movements in the proximal interphalangeal joint was compared after 3, 6, and 12 weeks from the moment of surgery, including local signs of inflammation in the needle insertion site after 3 and 7 days from the moment of surgery.

RESULTS: In the main group, signs of inflammation were found only in 10% of the cases, whereas in the comparison group, more serious signs were observed, such as the release of exudate along the spokes in two cases on day 3. The average values of the amplitude of movements at week 3 in the main group are more than two times higher than the average values of the comparison group, 12.06° and 5.56°, respectively. The volume of movements in the main group was restored more than two times more efficiently, and in several patients, by week 12, it was restored to 100° of the total volume of active movements in the joint (p < 0.05).

CONCLUSIONS: The author’s method of extra-articular and antegrade osteosynthesis of the fractures of the neck of the main phalanges in children made it possible to achieve better functional results in comparison with the standard method.

Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2022;10(3):247-253
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Impaired supporting function of the feet in adolescents with congenital cleft lip and palate with a mesial ratio of dentition

Nikityuk I.E., Semenov M.G., Botsarova S.A.

Abstract

BACKGROUND: Impaired occlusal relationships of dental rows can cause adaptive changes in the entire musculoskeletal system, including the feet. Thus, studying the biomechanics of the feet with the possibility of changing the medical rehabilitation program of patients with dentomaxillofacial anomalies of various geneses is important.

AIM: To investigate the plantographic characteristics of the feet in adolescents with congenital cleft lip and palate and combined dentomaxillofacial anomaly with a mesial ratio of dental rows and analyze patterns of distribution of plantar pressure before and after reconstructive operations on the jaws and restoration of facial harmony.

MATERIALS AND METHODS: The study included 31 patients of both sexes aged 15–17 years, who were divided into two groups. The first group consisted of 15 patients with congenital cleft lip and palate after the early stages of reconstructive surgery (cheilorhinoplasty and uranoplasty) and developed a combined dentomaxillofacial anomaly. The second group, with milder lesion, included 16 patients with combined dentomaxillofacial anomaly and do not have congenital cleft lip and palate. Patients had skeletal forms of mesial ratios of dental rows. To correct the bite and restore the aesthetics of the face, all patients underwent simultaneous bone reconstructive (“orthognathic”) surgery on the upper and lower jaws, including genioplasty in some of them, to restore the normal relationship of the jaw bones and harmonize the face. The plantographic characteristics of the feet were studied in patients before surgery and 1–6 months after surgery. The results of these two groups were compared with a pantographic examination of 18 healthy children (control group) without these pathologies in the maxillofacial region and without impairment of the supporting function of the foot.

RESULTS: The first and second groups had a significant decrease in the indices of support on both feet before surgery: t, up to 85 (normal, 96); m, up to 16 (normal, 23); and s, up to 20 (normal, 24), which indicate a decrease in the spring function of the transverse and longitudinal arches and impairment of the supporting function of the feet. It was most pronounced in patients with congenital cleft lip and palate. Deviations in the magnitude of the Clark angle α were multidirectional on the left and right feet, which indicated an abnormally high asymmetry of the load distribution between the feet. Functional relationships between the foot arches were pathologically enhanced to values of rs = 0.83 (normal, rs = 0.14), which indicated a formed pathological support strategy of the feet. After reconstructive operations on the jaws, the biomechanics of the feet in patients with combined dentomaxillofacial anomaly (without congenital cleft lip and palate) tended to normalize.

CONCLUSIONS: It is necessary to consider the possible aggravating effect of the feet with a modified support strategy on the condition of the dentofacial area. Moreover, the comprehensive diagnosis plan of adolescents with congenital cleft lip and palate and combined dentomaxillofacial anomaly and combined dentomaxillofacial anomaly (without congenital cleft lip and palate) should include a study of the supporting function of the feet, considering rehabilitation measures to correct the distribution of plantar pressure.

Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2022;10(3):255-270
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Clinical cases

Abdominal pocketing for the child’s fingers for hand replantation: A clinical case

Bogdanov S.B., Aristov A.M., Kurinnyy S.N., Nikishev V.A., Mukhanov M.L., Vedeneeva A.L.

Abstract

BACKGROUND: The traumatic avulsion of fingers is a severe injury that requires emergency replantation. In the case of crush mechanism and degloving, direct vessel repair, and reconstruction may be impossible. It appears fair to use indirect revascularization by enveloping the phalanges after osteosynthesis with blood-supplied tissues. The most accessible anatomically and functionally are the lower abdominal flap and abdominal pocketing of the hand; the flap pedicle was cut and the hand was released. Staged surgeries could be required for functional and aesthetic reconstruction of acceptable soft tissue envelop of the hand.

CLINICAL CASE: This is a clinical case of successful treatment of traumatic avulsion of II, III, IV fingers on the right hand by phalanx osteosynthesis and abdominal pocketing. The restoration of the functionally and aesthetically acceptable finger was achieved.

DISCUSSION: There is a high risk of necrosis and stump formation after the replantation of severely injured fingers when there was any possibility for direct vessel repair. Another method is the indirect revascularization by blood vessel ingrowth from the flap through the fracture line. This saves the injured limb segment.

CONCLUSIONS: The presented technique of abdominal pocketing allows the replantation of the avulsed and degloved fingers without microsurgical vascular operation and restores them. It appears fair to recommend this technique as a salvage surgery in the case of severely injured soft tissues of the avulsed hand fingers.

Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2022;10(3):271-276
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Two clinical cases of the reconstruction of distal phalanges of hand in children by combination of the regional flap and scrap bone and nailbed graft

Alexandrov A.V., Goncharuk P.V., Evdokimov A.N., Idris L.Y., Smirnov A.A., Khagurov R.A., Samorukova N.N., Alexandrova N.Y.

Abstract

BACKGROUND: The hand and its distal phalanges, especially in children, are among areas mostly exposed to trauma while contacting the environment. Injuries causing loss of soft tissue and bone component are both challenging and interesting. This report presents the treatment results of children with soft tissue and bone defects of distal phalanges, in which reconstructions including combination of regional flaps, bone, and nailbed grafts were performed.

CLINICAL CASE: This report presents clinical cases of the surgical treatment of 4- and 6-year-old children with fingertip injuries who underwent reconstructions including the combination of regional flaps, bone, and nailbed grafts. A combination of the scrap graft was performed in the first case with antegrade homodigital island flap and in the second case with thenar flap. The results were assessed 6 and 7 months after surgery.

DISCUSSION: The primary aim of the surgical treatment of children with distal phalangeal defects is coverage of those defects. The restoration of the bone component of the defect is recommended to prevent postoperative deformations of the distal phalanges. Fingertip replantation or microsurgical transplantation of vascularized tissue complexes are technically difficult and require thorough care and monitoring of the child’s condition in the postoperative period as all these measures may fail.

CONCLUSIONS: The possibility to combine the scrap bone and nailbed graft with existing reliable surgical methods open new prospects for the successful treatment of fingertip injuries.

Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2022;10(3):277-284
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Mucopolysaccharidosis IVB and sensorineural deafness associated with the CDH23 gene: A unique clinical case

Kenis V.M., Gorobets L.V., Dimitrieva A.Y., Zhmurova-Kriventsova A.A., Bychkov I.O., Baydakova G.V., Markova T.V., Zakharova E.Y.

Abstract

BACKROUND: Mucopolysaccharidoses are a group of lysosomal storage diseases belonging to orphan diseases. Certain types of mucopolysaccharides have a typical musculoskeletal findings and radiological changes. The mucopolysaccharidosis IVB is a rare type. Thus, >95% of cases of the mucopolysaccharidosis IV are subtype A.

CLINICAL CASE: Сlinical and radiological changes and genetic examination were performed to a 9-year-old patient with sensorineural deafness who applied to a medical institution with complaints of right hip pain and limping.

DISCUSSION: Based on complaints and radiological changes of the hip joints, the patient was initially diagnosed with Legg–Calve–Perthes disease. The presence of a symmetrical bilateral process, pathognomonic changes in the acetabulum and femoral heads, and an atypical clinic of Legg–Calve–Perthes disease made us suspect mucopolysaccharidos. Enzymatic analysis revealed a significant decrease in the beta-D-galactosidase enzyme activity. In addition, two compound heterozygous variants in the GLB1 gene were identified: the pathogenic variant c.808T>G, inherited from the father, and an insertion of a mobile genetic element, inherited from the mother. Only one variant in the GLB1 gene was detected in the brother (born in 2009), and none of the above GLB1 variants was detected in the older brother (born in 2003). Moreover, the proband (with clinical mucopolysaccharidos IVB) and his brother (born in 2009) (without mucopolysaccharidos IVB) inherited pathogenic CDH23 variants (c.6992T>C and c.805C>T) from their mother and father, respectively, which is consistent with their having sensorineural hearing loss.

CONCLUSIONS: The uniqueness of this clinical case is the presence of the rare type of mucopolysaccharidos and the separate genetic cause of sensorineural hearing loss in a single patient. The diagnosis of mucopolysaccharidos IVB in the proband was confirmed by biochemical and molecular genetic tests, and the diagnosis of CDH23-associated sensorineural deafness in the proband and brother (born in 2009) was confirmed by molecular genetic testing.

Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2022;10(3):285-294
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Review

Molecular foundations of the etiology and pathogenesis of Legg-Calve-Perthes disease and prospects for targeted therapy: A literature review

Shabaldin N.A., Shabaldin A.V.

Abstract

BACKGROUND: The etiology and pathogenesis of the development of Legg–Calve–Perthes disease, despite intensive research, remains not fully understood. Most studies have concluded about the multifactorial genesis of the development of hip osteochondropathy. Moreover, a complete understanding of all elements of pathogenesis leading to the manifestation and the progressive development of aseptic necrosis make it possible to develop targeted antiresorptive therapy. At present, several studies have investigated impaired functioning of signaling pathways that influence bone homeostasis during the development of Legg–Calve–Perthes disease. In addition, impaired metabolism in avascular necrosis is characterized by significant complexity and heterogeneity, which is based on aseptic inflammation associated with ischemic stress. Concepts of antiresorptive therapy were developed based on the results of studies on the pathogenesis of Legg–Calve–Perthes disease. Nevertheless, these treatment algorithms have not achieved wide practical application and require further investigation.

AIM: This study aimed to conduct a literary analysis of the molecular basis of the etiology and pathogenesis of Legg–Calve–Perthes disease and assess the prospects of therapy aimed at correcting bone homeostasis disorders.

MATERIALS AND METHODS: Data sources were PubMed, Medline, Scopus, Web of Science, and RSCI databases, without language restrictions.

RESULTS: The relationship between ischemic stress and the induction of a cytokine cascade with a predominance of the biological actions of proinflammatory cytokines, with parallel activation of intracellular regulatory networks that determine osteoresorptive processes, including due to pyroptosis, is shown. Data on the possibility of various variants of targeted antiresorptive therapy with the use of genetically engineered drugs are presented.

CONCLUSIONS: The pathogenesis of Legg–Calve–Perthes disease is characterized by significant genetic heterogeneity with the induction of various mediators of inflammation, angiogenesis, and osteogenesis, depending on the disease stage. Investigating features of impaired bone homeostasis regulation in the case of Legg–Calve–Perthes disease at the molecular and cellular level opens up opportunities for the development and clinical application of personalized therapy.

Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2022;10(3):295-307
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Screening for adolecent idiopathic scoliosis: A literature review

Lein G.A.

Abstract

BACKGROUND: Despite more than 60 years of screening for adolescent idiopathic scoliosis, it is still a controversial issue in the scientific literature. There are both opponents and supporters of the intervention, represented by government agencies, medical organizations, and individual researchers. Several countries have rejected national scoliosis screening, although some medical associations in these countries believe that screening based on the “Medical Home” model is feasible. By contrast, school-based scoliosis screening has been implemented nationally in a few countries. Given the lack of consensus on this issue, it is useful to systematize conflicting views on screening for adolescent idiopathic scoliosis.

AIM: This study aimed to review publications presenting information on the status of screening for juvenile idiopathic scoliosis to identify unresolved organizational issues.

MATERIALS AND METHODS: Data were searched in the open electronic scientific literature databases (eLIBRARY, PubMed, and Cochrane Library) using the following keywords and phrases: scoliosis screening; screening for adolescent idiopathic scoliosis (AIS); school screening for scoliosis; school scoliosis screening program. The depth of the search was 30 years.

RESULTS: Arguments “for” focus on the need for the early detection of AIS through screening in terms of the effectiveness of timely treatment, proven efficacy of conservative treatment of scoliosis, and reduction of surgical interventions among screened adolescents. The arguments “against” are related to the lack of a unified methodology for screening, high rate of false-positive and false-negative results, unproven effectiveness of screening in reducing the frequency of surgical interventions, economic efficiency, and psychological effect on adolescents and violation of their rights during the event.

CONCLUSIONS: Several organizational issues should be addressed with regard to screening. These include the training of staff who conducts the screening and development of a referral and follow-up system. The screening scheme and methods should be unified through the introduction of noninvasive screening methods to standardize the results and their subsequent uniform interpretation. The referral process for further examination should be standardized according to a defined protocol. The development of a special computer program to assist medical decision-making is relevant.

Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2022;10(3):309-320
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Lectures

Orthopedic management of cerebral palsy: past, present, and future

Baindurashvili A.G., Kenis V.M.

Abstract

Cerebral palsy is a neurological disease accompanied by orthopedic complications. To date, orthopedic treatment of cerebral palsy remains one of the basic approaches. Knowledge of history is the key to understanding the present and a bridge to the future. It gives us insight into the evolution of understanding of the problem, views on the possibilities of treatment, and personalized approach to the patient. The article describes the evolution of knowledge and approaches of treatment to children with cerebral palsy from ancient times to the present day. The main milestones of the neuro-orthopedic approach to diagnosis and treatment are reflected. In improving the surgical treatment of children with cerebral palsy, it is necessary to rely on a multidisciplinary approach, early detection of orthopedic pathology, personalization, taking into account the psychological and social effect of surgical treatment, and development and improvement of surgical techniques.

The lecture was presented at a scientific conference with international participation “Correction of motor disorders in the complex of medical habilitation of children with cerebral palsy.” Saint Petersburg, November 20–21, 2019.

Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2022;10(3):321-330
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Historical Article

H. Turner National Medical Research Center for Children’s Orthopedics and Trauma Surgery is 90 years old

Vissarionov S.V., Baindurashvili A.G., Ovechkina A.V., Zaletina A.V.

Abstract

In 2022, H. Turner National Medical Research Center for Children’s Orthopaedics and Trauma Surgery of the Ministry of Health of the Russian Federation celebrates its 90th Anniversary. It is a unique and the only specialized institution in the world that treats children with orthopedic pathologies. The Center is known not only for its medical achievements, innovations and developments, but also for the amazing atmosphere of care and attention to children. The result of the activities of H. Turner National Medical Research Center is thousands of children who regained their ability to move and enjoy their childhood.

Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2022;10(3):331-338
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Anniversray

Alexey Georgievich Baindurashvili

Abstract

Alexey Georgievich Baindurashvili, MD, PhD, Doctor of Medicine, Professor, Full member of the Russian Academy of Sciences, President of H. Turner National Medical Research Center for Children’s Orthopedics and Trauma Surgery, laureate of the Russian Federation Government Prize, Honored Doctor of the Russian Federation, and Chief Pediatric orthopedic and trauma surgeon of St. Petersburg, celebrates his 75th anniversary.

Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2022;10(3):339-341
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Obituaries

Jean-Jacques Cheneau (1927–2022)

Abstract

On July 14, 2022, at the age of 95, the world-famous orthopedic surgeon Jean-Jacques Cheneau, the creator of the famous corset for the conservative treatment of scoliosis, passed away.

Pediatric Traumatology, Orthopaedics and Reconstructive Surgery. 2022;10(3):343-345
pages 343-345 views


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