Aim. To evaluate the organizational structure, logistics, spectrum of surgical interventions, and clinical outcomes of the Department of Minimally Invasive Surgery and Transplantation of the National Children's Medical Center, a specialized pediatric center of the Republic of Uzbekistan. Materials and Methods. The study included 259 pediatric patients consecutively treated in the department between 2020 and 2026. Data from patients with biliary atresia, choledochal cysts, echinococcosis, extrahepatic portal hypertension, congenital portosystemic shunts, malignant liver tumors, as well as patients who underwent liver transplantation and pancreatoduodenal resections were analyzed. Clinical characteristics, intraoperative parameters, complications, and follow-up outcomes were evaluated. Results. The most common conditions were echinococcosis (n = 91), biliary atresia (n = 56), choledochal cysts (n = 54), and extrahepatic portal hypertension (n = 17). Among patients with echinococcosis, liver involvement predominated (n = 67), whereas lung involvement (n = 13), combined lesions (n = 6), and involvement of the spleen, diaphragm, and pelvis were less frequent. Open surgery was performed in 68.1% of patients, laparoscopic surgery in 28.6%, and video-assisted thoracoscopic surgery in 3.3%. Recurrence of echinococcosis was observed in 7.7% of patients and was predominantly associated with poor adherence to postoperative albendazole therapy. Twelve living donor liver transplantations were performed in children aged 5 months to 16 years. The main indications for transplantation were biliary atresia, autoimmune hepatitis, Wilson disease, and Caroli disease. The mean operative time was 523.8 ± 117.0 minutes, and the median blood loss was 200 mL. Vascular complications occurred in 25% of recipients, whereas no biliary complications were observed. Recipient and graft survival reached 100% throughout the follow-up period. All donors were first- or second-degree relatives; Grade II complications according to the Clavien–Dindo classification occurred in 16.7% of donors, with no donor mortality. The cohort also included 20 patients with primary malignant liver tumors (hepatoblastoma and hepatocellular carcinoma) managed through a multidisciplinary approach in collaboration with the pediatric oncology service. The extent of liver resection was determined according to the PRETEXT classification. Experience with extended right hemihepatectomy in infants aged 5–11 months demonstrated R0 resection in all cases, with a mean intraoperative blood loss of 120 mL and no perioperative mortality. Liver transplantation was considered for unresectable tumors according to established oncological selection criteria. Among 17 patients with extrahepatic portal hypertension, all underwent preoperative wedged angiography. A Rex shunt was performed in one patient, a Warren shunt in five patients, and devascularization procedures in two patients. Two patients required reoperation because of shunt thrombosis and stenosis. Between 2023 and 2026, five pancreatoduodenal resections were performed: four for solid pseudopapillary neoplasms of the pancreas and one for insulinoma. One patient developed a gastroenteric anastomotic fistula, while another experienced recurrent insulinoma requiring subtotal pancreatectomy. All patients were discharged and remained under outpatient follow-up. In addition, four patients with congenital portosystemic shunts were treated. Conclusion. The experience of the National Children's Medical Center demonstrates the feasibility of establishing a comprehensive pediatric hepatobiliary surgery and liver transplantation program in a middle-income country. An integrated patient referral system, a multidisciplinary approach, and international collaboration enabled excellent survival outcomes and facilitated the development of a national referral center for pediatric HPB surgery in Central Asia.
| Mualliflar | Semash, Konstantin, Dzhanbekov, Timur, Nasirov, Mansur, Umarov, Qosimjon, Сёмаш, К, Джанбеков, Т, Насиров, М, Умаров, К, Semash, Konstantin, Djanbekov, Timur, Nasirov, Mansur, Umarov, Qosimjon |
|---|---|
| Jurnal | Medical science of Uzbekistan / O'zbekiston tibbiyot ilmi |
| Nashr sanasi | 2025-04-30 |
| Jild | 5 |
| Son | 2 |
| Betlar | 14-32 |
| Til | Rus |
| DOI | 10.56121/msu-2026-2-00008 |
DOI: 10.56121/msu-2026-2-00008 · Maqolaning asl sahifasi
педиатрическая гепатобилиарная хирургия, трансплантация печени, операция Касаи, билиарная атрезия, киста холедоха, эхинококкоз, портальная гипертензия, Рекс-шунт, анастомоз Уоррена, трансплантация печени от живого донора, порто-системные шунты, мини-инвазивная хирургия, Узбекистан, Центральная Азия, pediatric hepatobiliary surgery, liver transplantation, Kasai procedure, biliary atresia, choledochal cyst, echinococcosis, portal hypertension, Rex shunt, Warren shunt, living donor liver transplantation, portosystemic shunts, minimally invasive surgery, Uzbekistan, Central Asia, pediatrik gepatobiliar xirurgiya, jigar transplantatsiyasi, Kasai operatsiyasi, biliar atreziya, xoledox kistasi, exinokokkoz, portal gipertenziya, Reks shunti, Uorren anastomozi, tirik donordan jigar transplantatsiyasi, porto-sistem shuntlar, kam invaziv xirurgiya, Oʻzbekiston, Markaziy Osiyo
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Medical science of Uzbekistan / O'zbekiston tibbiyot ilmi — barcha maqolalar