А CLINICAL CASE OF TREATMENT OF BILIARY ATRESIA – SPLIT TRANSPLANTATION

Transplantation is the only treatment for congenital and acquired liver malformations in the pediatric population. The most common are biliary atresia, A1 antitrypsin defi ciency, Wilson’s disease, metabolic diseases, acquired diseases complicated by cirrhosis and liver failure. In most cases, patients with biliary atresia require liver transplantation at an early age. In case of late diagnosis and inability to perform portoenterostomy (Kasai procedure), the only treatment option is transplantation at the age of ±1 year. The aim of study. Single- center case analysis of a child with biliary atresia demonstrates the current state of solving this problem in Ukraine with the absence of the possibility of family transplantation and shortage of donor organs. To study the potential development opportunities of SPLIT transplantation in this stage group. Analyze the possibilities of optimizing the process from diagnosis to transplantation with the aim of maximizing the preservation of the child’s physical and intellectual development and reducing the impact of the disease on other organs and systems. Results. In our work we analyzed the experience of treating a patient with uncorrected biliary atresia with late diagnosis. Patient P, blood group A(I) Rh(+) boy, 1st pregnancy, born with weight 2500g, height 50 cm at 39 weeks. Pregnancy: physiologic delivery, Apgar score 9\9. Liver biopsy confi rmed the diagnosis of biliary atresia with signifi cant cirrhosis. On esophagogastroscopy: signs of incipient portal hypertension. Considering these data and the child’s age (85 days), portoenterostomy as a method of treatment was not appropriate. A course of treatment was prescribed and liver transplantation was determined as the only method of treatment for the patient. Unfortunately, the examination of close relatives to determine the possibility of family donation did not give positive results. The donor was a 14-year-old boy, blood type I(O) Rh(+), with a congenital defect of the central nervous system. After prolonged treatment, the patient had a sudden hemorrhage. After brain death was declared, the parents gave their consent for organ transplantation to recipients on the waiting list. SPLIT transplantation was performed by dividing the donor organ for 2 recipients. The left lobe of the liver was transplanted to a patient with biliary atresia at the age of 1 year and 3 months. Three vascular anastomoses (caval, portal and arterial) were performed together with anastomoses between the bile duct and the intestinal tube of the child. The right hepatic lobe was similarly transplanted in an adult patient.The post-operative period was uneventful. Patients were successfully discharged and continue to receive immunosuppressive therapy on an outpatient basis. SPLIT transplantation is a successful way to solve the problem of donor organ shortage. Conclusions. 1. Liver transplantation is the main method of treatment for children with biliary atresia and other liver diseases due to dangerous complications that occur at an early age. 2. Split transplantation is a signifi cant step in the development of Ukrainian transplantology, especially in the conditions of organ shortage. It allows to save the life of two patients in need of transplantation. 3. If the diagnosis is made that liver transplantation is the only method of treatment and the patient is placed on the waiting list, the patient must be under observation and the multidisciplinary team must prepare the patient for transplantation. 4. Transplantation must be performed before signifi cant physical and intellectual irreversible changes. 5. The correct preparation of the patient, the optimalerm and method of transplantation, before lesions of other systems, directly affect the success and result of transplantation.