Oleg G. Topilin

BACKGROUND: Sublobar lung resections in children include wedge (nonanatomic) resection and anatomic segmentectomy. Segmentectomy is used less frequently due to its technical complexity and the lack of objective patient selection criteria. This study aims to address this gap. The authors evaluated whether patient-specific 3D models can standardize preoperative anatomical assessment and thereby support selection of the optimal extent of resection in pediatric patients. AIM: This study aimed to determine the impact of preoperative 3D modeling on surgical strategy and outcomes in minimally invasive lung resections in children and to identify anatomical and clinical predictors of the extent of resection. METHODS: The study included 32 children (0–17 years) who underwent minimally invasive lung resection for congenital malformations or benign neoplasms (2020–2025). Patients were divided into a 3D modeling group (n = 16) and a standard computed tomography–based planning group (n = 16). Outcomes assessed included type of resection, complications, operative time, and concordance between planned and performed resection. Stepwise logistic regression was used to identify predictors of the extent of resection. In an additional analysis, patients were stratified by type of resection (wedge resection, segmentectomy, lobectomy). Quantitative parameters derived from 3D models—such as lesion-to-lobe volume ratio, zoning, and number of involved segments—were used to refine surgical planning algorithms. RESULTS: 3D-based planning significantly increased the likelihood of performing segmentectomy (odds ratio ≈ 25; p = 0.001), while maintaining high concordance between planned and performed procedures without increasing complication rates. The most significant quantitative predictor of resection extent was the lesion-to-lobe volume ratio (optimal threshold 0.205; AUC = 0.922). The type of resection was also associated with age, number of affected segments, and anatomical location. CONCLUSION: 3D modeling facilitated more frequent use of segmentectomy without increasing complications. The lesion-to-lobe volume ratio and anatomical factors may be used to guide selection of resection type. Incorporation of quantitative 3D parameters may improve the quality of preoperative planning in pediatric lung resections.

Background.Acquired diaphragmatic hernias are rare surgical pathologies. The literature contains limited publications addressing this issue. Postoperative diaphragmatic hernias arise as complications of prior surgical interventions. The broad spectrum of clinical manifestations – ranging from asymptomatic cases to intestinal obstruction and respiratory failure – complicates timely diagnosis and surgical management. Surgical correction of postoperative diaphragmatic hernias, including the choice of surgical approach, requires an individualized strategy. Analysis of the causes of postoperative hernias may help minimize risk factors for their development.Aim.To clarify the etiology of postoperative diaphragmatic hernias in children and determine effective methods for their surgical correction.Methods.A retrospective analysis of 14 cases of children with postoperative diaphragmatic hernias was conducted, including evaluation of clinical data, surgical techniques, and postoperative outcomes.Results.Clinical manifestations varied among patients: 35,7% of cases were asymptomatic, 35,7% presented with intestinal obstruction, and 28,6% exhibited respiratory disturbances, including respiratory failure. Diagnosis of diaphragmatic hernias occurred between 2 weeks and 11 years after the initial surgery. The primary causes of postoperative hernias were intraoperative diaphragmatic injury (14,3%) and surgical technical errors during initial procedures (85,7%). Anatomical integrity of the diaphragm was repaired in all patients. Endoscopic approaches were used in 50% of cases, transthoracic diaphragmatic fixation in 50%, and xenopericardial patch repair in 28,6%. The median surgery duration was 80 minutes. Early postoperative complications were observed in 3 patients, late complications, including recurrences, were absent.Conclusion.Postoperative diaphragmatic hernias may remain asymptomatic and be diagnosed at varying intervals after surgery. Advanced surgical techniques, such as xenopericardial patch repair and transthoracic fixation of the diaphragm to the ribs, enable reliable diaphragmatic reconstruction with minimal postoperative complications. This study highlights the need for standardized surgical protocols to prevent postoperative diaphragmatic hernias.

Background. Primary ciliary dyskinesia (PCD) is an orphan disease, and diagnosis is difficult because there is no gold standard for diagnosis. Aim. Clinical, laboratory-instrumental, genetic characteristics of PCD in children. Materials and methods. From 2009 to 2024, 31 patients with a genetically confirmed diagnosis of PCD were observed as part of a multicenter, open-ended, descriptive pilot longitudinal study. Examination methods: clinical and anamnestic method; X-ray examination and computed tomography of the chest organs and paranasal sinuses, tracheobronchoscopy; sputum/aspirate cultures of the tracheobroncheal tree with determination of sensitivity to antibiotics; transmission electron microscopy, high-speed video microscopy of the ciliated epithelium, cytological examination of bronchoalveolar lavage; monitoring computer pulse oximetry, echocardiography, audiometry, spirometry with bronchodilator test. Results. Respiratory symptoms in the neonatal period have 80% of children with PCD, lateralization defects – 35%, congenital heart defects – 13%, bronchiectasis – 68%, purulent endobronchitis – 62%, year-round rhinitis – 84%, hearing loss, otitis – 65%. The average age of onset of symptoms was 1 [1; 1] weeks, and the verification of diagnosis was 6 [2,5; 8] years. The main pathogens of chronic respiratory infection with PCD are Haemophilus influenzae, Pseudomonas aeruginosa, Staphylococcus aureus. The most common cause of PCD was biallelic variants of the DNAH5 gene. Conclusion. The diagnosis of PCD should be based on the application of the maximum number of diagnostic tests.

Background. Primary ciliary dyskinesia (PCD) is an orphan disease, and diagnosis is difficult because there is no gold standard for diagnosis. Aim. Clinical, laboratory-instrumental, genetic characteristics of PCD in children. Materials and methods. From 2009 to 2024, 31 patients with a genetically confirmed diagnosis of PCD were observed as part of a multicenter, open-ended, descriptive pilot longitudinal study. Examination methods: clinical and anamnestic method; X-ray examination and computed tomography of the chest organs and paranasal sinuses, tracheobronchoscopy; sputum/aspirate cultures of the tracheobroncheal tree with determination of sensitivity to antibiotics; transmission electron microscopy, high-speed video microscopy of the ciliated epithelium, cytological examination of bronchoalveolar lavage; monitoring computer pulse oximetry, echocardiography, audiometry, spirometry with bronchodilator test. Results. Respiratory symptoms in the neonatal period have 80% of children with PCD, lateralization defects – 35%, congenital heart defects – 13%, bronchiectasis – 68%, purulent endobronchitis – 62%, year-round rhinitis – 84%, hearing loss, otitis – 65%. The average age of onset of symptoms was 1 [1; 1] weeks, and the verification of diagnosis was 6 [2,5; 8] years. The main pathogens of chronic respiratory infection with PCD are Haemophilus influenzae, Pseudomonas aeruginosa, Staphylococcus aureus. The most common cause of PCD was biallelic variants of the DNAH5 gene. Conclusion. The diagnosis of PCD should be based on the application of the maximum number of diagnostic tests.

Chest wall resection is performed for a variety of diseases, for primary rib and soft tissue tumors, metastatic lesions, or locally invasive growth of lung and mediastinal tumors being the most common indications. Following the resection phase, it is essential to determine the method of chest wall reconstruction that will restore the structural function, preserve pulmonary biomechanics, reduce the likelihood of residual pleural space, pulmonary hernia, and protect intrathoracic organs. The main objective of this study is to investigate the outcomes of chest wall resection with reconstruction using Codubix material. Material and methods. This retrospective multicenter study included 22 patients who underwent chest wall tumor resection with subsequent Codubix rib endoprosthesis reconstruction from 2019 to 2023. Four medical institutions participated in the study: P.A. Herzen Moscow Cancer Research Institute, Sverdlovsk Regional Oncology Hospital, Morozov Children’s City Clinical Hospital and Kaluga Regional Oncology Hospital. Inclusion criteria were the presence of chest wall tumors, both primary and secondary, removal of more than 2 ribs, resection of the rib arch and the sternum. Results. The median age was 60 years (48—66), 11 (50%) patients were females and 11 (50%) males. Operations for chest wall sarcoma, metastatic lesions, and lung cancer were performed in 9 (40.9%), 4 (18.2%), and 3 (13.6%) patients, respectively. The median number of removed ribs was 3 (2—4), with a maximum of 7. Sternotomy was performed in 9 (40.9%) patients, and subtotal resection of the body or handle of the sternum was carried out in 77.7%. Combined resections were performed in 14 (63.6%) patients. Radical tumor removal (R0) was achieved in 21 (95.5%) patients. Complications were observed in 9 (40.9%) patients, with intermuscular seroma being the most common in three (33.3%), followed by hydrothorax in 2 (22.2%), bilateral pneumonia, acute respiratory failure, and postoperative delirium in 1 (11.1%) patient each. One patient had the Codubix plate removed due to postoperative wound infection. The median overall and recurrence-free survival was not reached, and the 1-year recurrence-free survival was 63.9%, with an overall survival of 86.8%. Conclusion. Reconstruction with Codubix material allows for satisfactory functional and cosmetic results, characterized by a low complication rate and good adaptive properties.

3D-technologies are a promising direction in the development of medicine. Numerous publications in recent years demonstrate the potential use of 3D-modeling in disease diagnosis, surgical planning, intraoperative navigation, development of individual implants, and specialist training. In this article, we present the experience of implementing 3D-technologies in the practice of a pediatric surgeon using three clinical cases where the use of 3D models based on computed tomography data allowed for more accurate diagnosis and surgical planning. The first patient was a 3-year-old boy with vascular compression of the left main bronchus and impaired ventilation of the left lung. After 3D-visualization of the area of interest, an arterial ligament causing the compression was visualized as the main cause. The second patient was a 6-month-old boy with confirmed bilateral chondromesenchymal hamartoma of the chest wall. The volume and staging of the surgical treatment, as well as the most suitable option for defect replacement, were developed after creating a polymer 3D-model of the child’s chest. The third patient was a 17-year-old girl with persistent hypercalcemia after removal of a parathyroid adenoma. A repeat computed tomography revealed an ectopic parathyroid tissue. The use of 3D-models and AR-technology for intraoperative navigation allowed for precise determination of the location of the pathological tissue. These clinical cases demonstrate the potential and effectiveness of using 3D-modeling in pediatric surgery and confirm that this is a promising direction in the development of medicine, opening up new possibilities for clinicians.

Primary ciliary dyskinesia (PCD) is an orphan disease associated with mutations in several genes. It is a ciliopathy, an abnormality of the cilia and flagella. Ciliopathies include the extremely rare Simpson - Golabi - Bemel syndrome (SSGB) type II.The aim of this article is to familiarize the reader with the possibility of simultaneous presence of type II SSGB and PCD in a patient with bronchiectasis (BE).Results. The first clinical observation in the Russian literature is presented withhistory, physical examination, including clinical and morphologic examination, results of additional investigations and initiation of therapy. The case describes a 15-year-old patient with BE and other lesions typical of PCD confirmed on the basis of structural changes in the cilia of the respiratory epithelium of the trachea detected by transmission electron microscopy. The patient had a pathogenic mutation of the OFD1 gene responsible for the development of both type II SSGB and PCD.Conclusion. Several variants of ciliopathies may occur in one patient, and PCD may present as a syndrome.

The purpose of the study was to determine the predictors of severe bronchiectasis (BE) not associated with cystic fibrosis (CF) in children and to evaluate the effectiveness of a stepwise complex conservative therapy. Materials and methods of the study: study design - multicenter cohort prospective pilot study. 67 hospitalized pediatric patients aged from 11 months up to 17 years old (52% boys and 48% girls) with BE caused by previous pneumonia (22%), primary ciliary dyskinesia (22%), bronchial asthma (13%), Williams-Campbell syndrome (7%), bronchial foreign bodies (7%), gastroesophageal reflux disease (6%), bronchopulmonary dysplasia (6%), postinfectious bronchiolitis obliterans (5%), allergic bronchopulmonary aspergillosis (3%), chronic granulomatous disease (3%), AIDS (1%), protracted bacterial bronchitis (1%), and brain-lung-thyroid syndrome (1%) were observed. Predictors of severe BE were determined based on a comparison of groups of patients with mild (up to 4 exacerbations of BE per year) and moderate (from 4 to 6 exacerbations) course of BE (n=31) and patients with severe course of BE (>6 exacerbations per year, n=36). At the second stage of the study, in patients with changes in the severity of the course of BE, who were followed in dynamics (n=42), the frequency of exacerbations was assessed during the year after the appointment of a stepwise complex conservative therapy, which included, depending on the severity, daily drainage massage, exercise therapy, auxiliary devices for the respiratory tract clearance, long-term anti-inflammatory azithromycin, inhaled/intravenous antibiotics, and/or respiratory support. Results: the clinical picture of severe BE occurring with >6 exacerbations per year compared with mild/moderate course of the disease (≤6 exacerbations per year) is characterized by a statistically significantly more frequent registration of dyspnea (86% and 45%, p<0.001), exercise intolerance (69% and 39%, p=0.012), wet rales (89% and 61%, p=0.011), finger clubbing (17% and 0%, p=0.027). The prognostic model for determining the likelihood of severe BE not associated with CF in children includes the age of manifestation, a positive result of bacteriological examination of sputum/aspirates from the respiratory tract, localization of BE in the middle lobe of the right lung and/or lingual segments, and dyspnea. The appointment of gradual conservative therapy for BE, depending on the severity/frequency of exacerbations, makes it possible to statistically significantly reduce the severity of BE (p<0.001) and the median of exacerbations during the year from 9.00 [3.25-12.00] before treatment to 2.00 [1.00-3.00] after treatment (p<0.001). Conclusion: BEs are heterogeneous in severity, which determines the choice of therapy. Conservative therapy of BE not associated with CF in children has a stepwise principle depending on the severity of the course. Its appointment could therefore reduce the frequency of exacerbations.

Aim. To establish the etiological structure and to present clinical and laboratory and instrumental characteristics of bronchiectasis (BE) not associated with cystic fibrosis (CF) in children. Materials and methods. Sixty-seven hospitalised patients with BЕ not related to CF were followed up between 2017 and 2022. Examination methods: clinical-anamnestic method, general clinical laboratory investigations, investigation of allergological and immune status, phagocytosis system, determination of concentration of specific IgE and IgG to fungi of genus Aspergillus, sweat test, radiological examination and computed tomography (CT) of chest organs, bronchoscopy, Bacteriological examination of sputum and/or tracheobronchial aspirates, nasal and/or bronchial ciliary motility, esophagogastroduodenoscopy, 24-hour pH-metry, intra-esophageal combined impedance-pH-metry, genetic study, lung biopsy. Results. Etiologic factors of BЕ not associated with CF in children were severe pneumonia (22%), primary ciliary dyskinesia (22%), bronchial asthma (13%), Williams-Campbell syndrome (7%), bronchial foreign bodies (7%), gastroesophageal reflux disease (6%), Bronchopulmonary dysplasia (6%), postinfectious bronchiolitis obliterans (5%), allergic bronchopulmonary aspergillosis (3%), chronic granulomatous disease (3%), AIDS (1%), prolonged bacterial bronchitis (1%), brain-lung-thyroid syndrome (1%). The clinical picture is characterized by cough (91%), shortness of breath (67%), fever during exacerbation (48%), chest pain (24%), exercise intolerance (55%), drumstick symptom (9%), moist (76%) and dry wheezing (37%). CT-semiotics of BЕ not associated with CF is characterized by localization in one (58%) or several (42%) lobes; traction (42%), non-traction (49%) B and their combination (9%); increased broncho-arterial ratio 0.9; thickening of bronchial wall; "mosaic perfusion"/"air-trap" symptom (9%); more frequent involvement of lower lungs (64%). The main infectious agents in BЕ not associated with CF were Haemophilus influenzae, Pseudomonas aeruginosa, Staphylococcus aureus. Conclusion. On the basis of a multicentre study, the etiological structure, clinical and laboratory and CT-characteristics of non-CF ВE in children were established.