Vol 15, No 2 (2025)

BACKGROUND: Hirschsprung disease is one of the most complex and severe congenital malformations. The choice of surgical treatment depends on the wide spectrum of disease forms and clinical manifestations. Currently, the transanal endorectal pull-through technique is considered the most promising surgical approach. The greatest challenge is the management of complications such as Hirschsprung-associated enterocolitis and coloanal anastomotic stricture. The implementation of new surgical techniques allows for a reduction in postoperative complications and improvement in treatment outcomes in patients with Hirschsprung disease.

AIM: The study aimed to improve surgical outcomes in patients with Hirschsprung disease based on the analysis of results of transanal endorectal pull-through procedures using the authors’ modification and preventive management of potential complications.

METHODS: An analysis was conducted of surgical treatment of Hirschsprung disease using the transanal endorectal pull-through technique between 2010 and 2024. Since 2015, a modified technique has been used during pull-through surgery: resection of the muscular cuff along the posterior wall of the anal canal following endorectal dissection of the rectum in the submucosal layer. Required morphological and radiological evaluations were carried out. The obtained data were statistically analyzed using the Statistica software package (Version 10-Index, StatSoft Inc.).

RESULTS: Over the past 15 years, 2010–2024, 112 patients under 3 years of age with Hirschsprung disease underwent transanal endorectal pull-through at the Republican Scientific and Practical Center for Pediatric Surgery. A reduction in the incidence of anastomotic strictures was achieved following the introduction of posterior muscular cuff resection during transanal endorectal pull-through (in 2015): from 23.7% (9 out of 38 patients) in 2010–2014 to 8.3% (4 out of 48 patients) in 2015–2019, and further to 3.6% (1 out of 26 patients) in 2020–2024. The incidence of enterocolitis also gradually declined, from 25% (21 of 86 patients) in 2010–2019 to 14.3% (4 of 26 patients) in 2020–2024. It was found that the use of transanal endorectal pull-through with resection of the muscular cuff led to a reduction in postoperative complications.

CONCLUSION: Transanal endorectal pull-through with resection of the posterior muscular cuff in children with Hirschsprung disease, along with preventive management of potential complications, can be considered the optimal technique of surgical treatment for this condition.

BACKGROUND: A review of the scientific data reveals a lack of publications analyzing the opinions of anesthesiologists and intensivists in pediatric departments of anesthesiology, resuscitation, and intensive care on the necessity of using organ dysfunction severity scoring systems in clinical practice.

AIM: The study aimed to analyze the opinions of anesthesiologists and intensivists working in pediatric departments of anesthesiology, resuscitation, and intensive care in the Russian Federation regarding the necessity of applying multiple organ dysfunction scoring systems in clinical practice.

METHODS: The study was observational, uncontrolled, and based on a voluntary anonymous survey conducted through the website of the Association of Pediatric Anesthesiologists and Intensivists of Russia. Descriptive statistics were presented as frequencies and percentages; responses to open-ended questions were categorized based on key qualitative parameters. Pearson’s χ² test was used for comparative analysis of categorical data.

RESULTS: A total of 230 respondents participated in the survey, the majority of them were physicians from regional clinical hospitals (38.7%) with over 10 years of experience (69.6%). The most well-known and frequently used scoring systems were qSOFA, nSOFA, NEOMOD, pSOFA, and the Phoenix Sepsis Score. Regular use of multiple organ dysfunction severity scores was reported by 77 (33.5%) respondents, with the most commonly applied being the qSOFA scale, which has not been validated in the Russian clinical settings.

CONCLUSION: More than 60% of pediatric anesthesiologists and intensivists use multiple organ dysfunction scoring systems irregularly, and their use is more commonly driven by the perceived high risk of mortality than by the need to determine subsequent treatment strategies.

BACKGROUND: Incarcerated inguinoscrotal hernia in children is a serious and relatively common complication (5.2%–24%) that can be life-threatening. Elective surgical repair is considered the most favorable option. Several manual reduction techniques have been proposed as part of conservative management; however, these methods have certain limitations and drawbacks.

AIM: The study aimed to evaluate the advantages of a proposed manual reduction technique for incarcerated inguinoscrotal hernia in boys.

METHODS: Between 2020 and 2023, 67 children with incarcerated inguinal hernias were admitted to the surgical department. Inclusion criteria comprised boys with incarcerated inguinoscrotal hernias of less than 12 hours’ duration, without signs of complications involving the scrotal organs or abdominal cavity. The main group included 17 children who underwent conservative therapy in combination with the new manual reduction technique. The control group consisted of 23 patients who received conservative therapy with the conventional method of gentle compression of the scrotum using all fingers. Following premedication and manual shaping the hernial sac into a funnel—its narrowest point matching the diameter of the external inguinal ring—the funnel was stabilized circumferentially with the fingers of one hand, whereas the other hand gently compressed the remaining part of the sac toward the abdominal cavity until spontaneous reduction of hernia contents was achieved.

RESULTS: Comparison of the parameters of the conventional and the new manual reduction techniques for incarcerated inguinoscrotal hernia revealed significantly higher effectiveness of conservative therapy and a shorter procedure time in the main group using the new method (p < 0.05). The degree of compression applied to the hernial sac in the main group was also lower, which significantly reduced the risk of trauma during the procedure.

CONCLUSION: The proposed manual reduction method for incarcerated hernias in boys demonstrated higher efficacy, faster execution, and reduced trauma risk.

Short bowel syndrome is one of the most challenging conditions in modern pediatric surgery and intensive care, leading to a significant decrease in quality of life, disability, and mortality. This review presents current insights into the pathogenesis, clinical manifestations, diagnosis, and treatment of the condition. Publications focused on the principles of intensive care for pediatric short bowel syndrome were analyzed. Scientific data searches were conducted in the PubMed, Cochrane Central Register of Controlled Trials, eLIBRARY.RU, and CyberLeninka databases for the period from 2001 to December 2024. The search was conducted using the following keywords: синдром короткой кишки / short bowel syndrome, дети/pediatric, интенсивная терапия / intensive care, парентеральное питание / parenteral nutrition, энтеральное питание / enteral nutrition, and осложнения/complication. Inclusion criteria were studies describing the definition, clinical course, and intensive care principles of short bowel syndrome in children. Publications focused solely on adult patients were excluded, except for guidelines illustrating general intensive care principles applicable regardless of age. A total of 62 articles were included. The review discusses the definition, epidemiology, etiology, key risk factors, and pathogenesis of short bowel syndrome, as well as its classification and clinical presentation depending on the extent of intestinal resection. Particular attention is given to intensive care measures and nutritional support, the use of antibiotics and medications for restoring gut microbiota, glucagon-like peptide-2 analog therapy, and chyme reinfusion techniques. The core principles of nutritional support, indications and contraindications for its administration, and complications of long-term parenteral nutrition are examined. Strong evidence is presented supporting early initiation of enteral feeding. Functional gastrointestinal assessment tools and food tolerance diagnostics are described, along with suggested algorithms for initiating enteral support and managing short bowel syndrome–associated diarrhea. The necessity of a multidisciplinary approach for the early rehabilitation of children with short bowel syndrome is emphasized, aimed at restoring physiological function and development.

Hormonal regulation plays a crucial role in human sexual development. Mutations in the androgen receptor gene may cause peripheral androgen resistance, leading to characteristic phenotypic manifestations. In complete androgen resistance, patients develop a female phenotype despite disrupted sexual differentiation, which is mediated by aromatization of testosterone into estrogen. One of the typical complaints reported by patients is the presence of a groin bulge, often bilateral, which is perceived as an inguinal hernia. Surgical treatment in the form of gonadectomy may result in impaired bone mineral metabolism or masculinization; on the other hand, delayed intervention increases the risk of malignancy. This article presents a familial case of Morris syndrome with a previously undescribed AR gene mutation (NM_001011645.3) in two girls aged 14 and 6 years. Both girls were admitted for elective repair of bilateral inguinal hernias observed since birth. During hernioplasty, testes were discovered and biopsied, revealing testicular tissue in the first case and tunica albuginea in the second. Based on intraoperative findings, further evaluation was performed, including ultrasound of the genitourinary system, hormonal testing, medical genetic testing, and consultations with endocrinologist and geneticist. In both cases, androgen insensitivity syndrome was confirmed (a hemizygous AR gene variant was identified in exon 7: HG38, chrX:67721856T> C, c.746T> C, resulting in the amino acid substitution p.Met249Thr). DNA sequencing revealed the AR gene mutation in the mother in a heterozygous state. No pathogenic variants were identified in the father, older sister, or brother. With parental consent, both patients underwent bilateral laparoscopic gonadectomy. The study highlights the importance of age, including in relation to sexual and phenotypic development, when determining the timing of surgical intervention. It also underscores the need for a comprehensive, multidisciplinary approach to address functional, social, and gender-related issues in these patients.

Currently, there are no instrumental or laboratory methods available to assess the extent of ischemic injury to the ovary in cases of torsion. The evaluation of viability of the involved structures is based on visual inspection of the color and appearance of the gonad and fallopian tube. In doubtful cases, a decision is often made in favor of organ preservation in the hope of postoperative functional recovery. This publication presents a clinical case of a 10-year-old girl who had been experiencing intermittent abdominal pain for over three days. Torsion of the right adnexa was corrected surgically in a regional hospital. However, postoperative abdominal pain persisted, and she was hospitalized again 19 days later. Laboratory findings revealed elevated blood glucose levels, signs of hypercoagulation, and active thrombogenesis. Ultrasound imaging demonstrated a fixed, avascular mass measuring 71 × 58 × 89 mm located in the retrovesical space. A repeat laparoscopic surgery was performed. During pelvic revision, an infiltrate consisting of the right ovary, sigmoid colon, and greater omentum was found. The right ovary, which appeared gray, showed no signs of retorsion or perfusion and was excised after aspiration. Histological examination revealed ovarian tissue necrosis. The postoperative course was uneventful. Persistence of pain following adnexal detorsion, absence of blood flow in the affected ovary on transabdominal ultrasound, shortened activated partial thromboplastin time, elevated fibrinogen levels, and increased soluble fibrin–monomer complex may indicate the need for interval laparoscopy.

Inflammatory myofibroblastic tumor is a rare neoplasm of childhood with uncertain biological potential. In more than half of the cases, inflammatory myofibroblastic tumor pathogenesis is associated with ALK gene translocations. The standard treatment is radical surgical treatment. In patients with inoperable, recurrent, or metastatic forms of the disease—which are extremely rare—therapeutic options remain very limited. This article presents a case report of an 8-month-old patient diagnosed with an abdominal inflammatory myofibroblastic tumor. The abdominal mass was initially noticed by the parents. No specific clinical symptoms were observed. Upon examination at the clinic, a multinodular mass was localized to the omentum, ascending colon, and parietal peritoneum. Magnetic resonance imaging and abdominal ultrasound were used as the primary diagnostic modalities during the preoperative stage. Blood tests, including clinical and biochemical parameters, showed no significant abnormalities, and tumor markers were negative. Surgical treatment was indicated due to the presence of a large abdominal mass, with its topography confirmed by contrast-enhanced magnetic resonance imaging angiography. A median laparotomy was performed, followed by tumor excision, subtotal omentectomy, resection of the ascending colon, and colo-colonic anastomosis. The postoperative course was uneventful. The outcome was full recovery. The presented case of an abdominal inflammatory myofibroblastic tumor highlights the need to include this tumor type in the differential diagnosis of abdominal masses in young children. A notable feature of this case is the asymptomatic course and the radical excision of multiple nodules with varying locations, which serves as a predictor of favorable prognosis. No additional therapy was required due to the completeness of surgical treatment.

Purulent kidney lesions occupy a special place in pediatric practice, yet only isolated or small series of cases have been reported to date. This paper presents a clinical case of a 12-day-old patient admitted with lethargy, feeding refusal, fever, and depressed consciousness. Epidemiological history revealed prolonged household contact with varicella. Examination revealed enlargement of the right kidney with multiple diffuse focal changes in the parenchyma, enlargement of the left kidney, multiple microabscesses, signs of ureterohydronephrosis, and bladder wall thickening. Surgical revision of the right kidney was performed via a translumbar approach, with placement of a subcapsular drain and insertion of a cystostomy. The patient received antibacterial, antiviral, and infusion therapy, along with immunological support. Discharge from hospital occurred on day 32 of admission. At follow-up, at the age of 5 months, the Foley cystostomy catheter was removed, and a universal 5Ch ureteral stent was placed in the left ureter, which was removed at 6 months. This clinical case of suppurative pyelonephritis in a newborn with a congenital urinary tract malformation demonstrates the need for staged treatment and formation of a multidisciplinary team of specialists — neonatologists, pediatric surgeons, pediatric urologists, nephrologists, and clinical pharmacologists — for timely diagnosis and successful management.

This article is dedicated to the 70th anniversary of Alexander Yu. Razumovsky, Professor, Corresponding Member of the Russian Academy of Sciences, Head of the Isakov Pediatric Surgery Department of the Pirogov Russian National Research Medical University. It summarizes his ongoing scientific career, professional and management achievements in pediatric surgery. Alexander Razumovsky’s medical education, development as a high-end professional and scientist, and his professional career are connected to the 2nd Moscow “Order of Lenin” State Medical Institute named after N.I. Pirogov (now the Pirogov Russian National Research Medical University). A student of Academician of the Russian Academy of Sciences Yury F. Isakov and the brilliant galaxy of pediatric surgeons of the Filatov Hospital School, he has headed the largest team of researchers and educators in Russia with more than 20 professors since 2012. Professor Alexander Razumovsky’s research and practice include thoracic surgery, abdominal surgery, liver and bile duct surgery, vascular surgery, and endosurgery. Innovative treatments for children with various surgical conditions are presented in more than 20 monographs. Alexander Razumovsky supervised and provided scientific advice on 42 successful theses. Professor Razumovsky is involved in public service as the Chief of Pediatric Surgery of the Department of Healthcare of Moscow and the Central Federal District of the Russian Federation, Chairman of the Presidium of the Russian Association of Pediatric Surgeons, and a member of other surgical associations and journals, including The Russian Journal of Pediatric Surgery, Anesthesia and Intensive Care. Professor Razumovsky’s excellence was recognized by the title of Honored Medical Doctor of the Russian Federation; Science and Technology Prize awarded by the Russian Government; two Ternovsky Prizes, and two Mission (Призвание in Russian) Prizes awarded to the best doctors in Russia. Alexander Razumovsky celebrates his anniversary at the peak of his career with new scientific ideas and promising plans, remaining a wise and talented leader with a strong position on life.