Introduction. The earliest mention of total colonic aganglionosis, as the form of Hirschsprung’s disease, is dated 1926. According to various sources, total colonic aganglionosis is met approximately in 1 per 500 000 live births. Currently, the curative strategy in total colonic aganglionosis is strongly personalized. There are various surgical techniques for managing this severe congenital colon anomaly. Recent achievements in pediatric coloproctology have minimized the mortality rate in children with intestinal neuronal dysplasia and total colonic aganglionosis. However, despite of the evident progress in coloproctology, questions about the volume of reconstructive surgery in total colonic aganglionosis, application of “reservoir” surgical techniques so as to promote child’s further development and socialization are still unsolved.Material and methods. The patient was successfully treated with a reservoir technique which included an ileo-transverse anastomosis by Martin-Kimur with the formed conduit pulled to the anterior abdominal wall. Then, a radical intervention was made which consisted of combined abdominoperineal transanal endorectal pull through (TEPT) of the ileo-transverse conduit/reservoir, of the resection of rudimentary colon and a coloanal anastomosis by Boley.Conclusion. The obtained small intestine-colon reservoir in total aganglionosis allows to preserve motor function due to peristaltic movements of the small intestine wall and to prevent intestinal obstruction, thereby facilitating the physiological function of the intestine. In addition, the colon wall in the reservoir saves the area of absorbing surface and minimizes water-electrolyte disorders.

Authors present a rare clinical case of successful treatment of a 5-year-old child with purulent-necrotic paraproctitis, which developed against the background of a primary immune deficiency state. Clinical case. A 5-year-old boy became acutely ill, against the background of diarrhea with hyperthermia. An area of hyperemia, edema, and tissue infiltration appeared in the perianal region. In anamnesis, with frequent respiratory infections and dysfunction of the intestinal tract in the form of diarrhea. He was admitted to the Regional Childrens Hospital on the 10th day of illness from the regional hospital, where antibiotic treatment was started. Upon admission, the condition was serious in the perianal region with an extensive wound necrotic tissues and a plaque of fibrin. To create favorable conditions for wound healing, a preventive sigmostomy operation was performed. Multidrug-resistant Pseudomonas aeruginosa was found in the wound culture. Thus, courses of antibiotics treatment, infusion therapy, and surgical debridement of the perianal wound and its local treatment were performed. Upon immunological status examination, a primary immunodeficiency was revealed, for which drugs with immunoglobulin G were administered. As a result of the treatment, the wound healed by secondary intention, thus, an operation was performed to close the sigmostoma. The child was discharged in satisfactory condition under the supervision of an immunologist and a surgeon. Discussion. In this case, preventive sigmostomy is justified; however, authors limit themselves to surgical intervention on the perineum in other cases. Pseudomonas aeruginosa shows its pathogenicity in patients with impaired immunity. Therefore, courses of antibiotics, infusion therapy, and immunomodulatory therapy along with surgical treatment, favorable result were achieved. Conclusion. Children with purulent-necrotic paraproctitis, which developed against the background of a primary immune deficiency should be hospitalized as early as possible in a specialized hospital.

BACKGROUND: Pilonidal disease (pilonidal cyst, epithelial coccygeal passage) is a chronic inflammatory disease of the sacrococcygeal region, common among young people, including adolescents, to a certain extent worsens the quality of life. Until now, the etiopathogenesis and surgical treatment methods of the disease have been the subject of wide discussion. AIM: The aim of the study was to evaluate the effectiveness of Gips operations in children with pilonidal disease. MATERIALS AND METHODS: A comparative analysis of 41 patients with pilonidal disease was conducted in the surgical department of the City Ivano-Matryoninsk Children's Clinical Hospital in Irkutsk from January 2020 to August 2021. Planned surgical treatment was performed in 22 patients with primary or recurrent pilonidal cysts, including 15 children using the M. Gips procedure. The comparison group consisted of patients who underwent wide excision of a complex of soft tissues bearing a pilonidal cyst, primary and secondary fistulous passages, followed by wound restoration with a storey suture. RESULTS: The patients comprised 72.7% boys and 27.3% girls with an average age of 15.6 1.5 years and average disease duration of 5.1 2.2 months. The average operation time was 12 4.2 minutes. The motor regime was resumed on the first day after the operation. Patient pain was relieved by administering non-steroidal anti-inflammatory drugs for the first two postoperative days. There were no early postoperative complications. The average hospital stay was 3.8 1.9 days. Complete healing by secondary intention was achieved after an average of 3.9 1.8 weeks. The average follow-up was 7 3.8 months, and 6.7% had early relapses. CONCLUSIONS: Minimally invasive sinusectomy according to the Gips procedure for treating pilonidal disease in adolescents is safe and effective, has a low recurrence rate, allows an early return to daily activities, and provides a good cosmetic result. However, the small number of observations requires further research.

Introduction. One of the actual problems of reconstructive colorectal surgery in childhood is a high frequency of complications after the initial reconstructive procedures for Hirschprung disease. Currently, in the clinical practice there is a wide range of surgical techniques, the combination of which allows to avoid serious outcomes leading to child’s disability. The choice of the volume of repeated surgeries is strictly individual, depending on the nature of complications, types of previously performed surgeries as well as on patient’s initial status. The key for developing a curative tactics is findings after instrumental and morphological examination which were obtained for previous interventions and which often require additional further clarification.Material and methods. In 2010–2021, in the Center of Pediatric Colorectal Anomalies (Irkutsk, Russia) redo endorectal pullthrough surgeries were performed in 18 patients with Hirschprung disease after previous correcting interventions. Surgeons made transanal resection in combination with posterior sagittal perineotomy. Indications for repeated surgical procedures, in most cases, were residual aganglionic segments and the constipation relapses – 11 (61,2%) patients; scar deformation and anus channel stenosis – 5 (27,8%) patients; stricture of coloanal anastomosis – 1 (5,5%) patient; scar stenosis of the neorectum with a rectobulbar fistula – 1 (5,5%) patient.Results. 13 (72,2%) patients had good clinical outcomes: no parents’ complaints, stable rhythms of defecation (from 1 to 4 times per day), feces contents (type 3–6 by Bristol scale of feces). In 4 (22,2%) cases, there was chronic constipation where defecation was 2–3 times a week with preserved defecation urgencies and rare episodes of anal overflow incontinence requiring conservative treatment under the bowel emptying program. One patient had a stricture of coloanal anastomosis which was corrected with its dissection and further dilatation.Conclusion. To confirm indications for redo surgeries, a comprehensive assessment of the colon and perineum with clinical, functional and morphological examination is required. Transanal pull-down is a relatively safe procedure which, in most cases, has good and satisfactory clinical postoperative results and which may be performed even after previous endorectal resections. The rehabilitation program after the discussed surgery is strictly personified and developed under close interaction of a surgeon and a gastroenterologist after assessing the state of the formed coloanal anastomosis and the distal part of the pulled-down intestine.

A rectovestibular / rectovaginal fistula with a normally formed anus, so-called H-type (H-type fistula), is rather rare anorectal malformation which causes a serious problem for surgical correction of the defect. A surgical correction in infancy is associated with technical difficulties because of specific anatomical relationships and perineum topography in this age group. Thus, an improved surgical access which allows to get good anatomical and functional results and a worthy quality of life in such patients in future is a relevant issue. The authors describe a clinical case of successful treatment of H-type rectovestibular fistula with improved perineal access in a 1-year old child.