Diagnosis and modern medical-surgical tactics in treatment of biliary atresia in children

Abstract

Background: Biliary atresia (BA) is a serious pediatric condition that tends to progress to cirrhosis, liver failure, and death within a short time. It is the result of a continuous inflammatory, sclerosing, destructive process in the biliary tract and the most common indication for liver transplantation. Material and methods: The study included 46 patients up to 1 year of age hospitalized with cholestasis syndrome in IMSP IM and C, during the years 2014-2019. The basic methods in the diagnosis of BA were the biochemical examination, FGDS, USG doppler dupplex color of the biliary system before and after the meal intake, MRI with cholangiography, dynamic hepatobiliary scintigraphy. Results: Following the analysis of clinical and paraclinical results, surgical pathology was excluded in 25 patients, the diagnosis of BA was established in 11 cases. 6 patients with BA underwent Kasai surgical intervention , a primary liver transplant was performed in 3 cases, and 2 patients died before the surgery. Conclusions: Portoenteroanastamosis ( Kasai operation) performed as early as possible (up to 60 days postnatal) considerably increases life expectancy. The embryonic form of BA is a severe condition that is indicated for the initial liver transplant.The prognosis of untreated biliary atresia is unfavorable, leading to the death of most children in the first 2 years of life due to liver failure. In decopensated late-diagnosed cases, liver transplantation remains the only treatment option.