Biliary atresia: the path to diagnosis

Abstract

Biliary atresia (BA) is a congenital rapidly progressive inflammatory disease characterized by cholestasis, increasing destruction of the bile (intra-, extrahepatic) ducts and fibrosclerosis. If jaundice persists after two weeks of age, differential diagnosis is necessary among various diseases of the liver and biliary tract, but very often it remains late and difficult.

The cardinal solution for primary BA is liver transplantation (LT). Kasai operation (KO) - creation of a portojejunoanastomosis - a palliative solution that allows for a partial solution to the problem by the time of LT. When performing LT, the most progressive method is SPLIT-transplantation. Timely diagnosis and treatment (including LT) improves the quality of life (QOL), prevents irreversible changes in the child's body, the rapid development of multiple organ failure and the threat of early death.

Aim - to study the features of differential diagnosis of BA and the effectiveness of various surgical methods of its correction (before the onet of irreversible cirrhotic changes in the liver and multiorgan pathology).

Materials and methods. In 61 children with cholestasis aged 1.5-4.5 months to verify the diagnosis of BA, clinical, biochemical, instrumental examinations were performed in the dynamics before and after treatment, QOL was assessed, physical development (PD) and neuropsychological development (NPD) were assessed according to current orders of the Ministry of Health of Ukraine. Treatment included KO and LT.

Results. Against the background of treatment, biochemical and clinical indicators improved, the level of anxiety decreased. The dynamics of PD and NPD were positive. In 39 children with severe BA, these indicators did not normalize after 6 months, and LT in 37 of them was effective, as evidenced by the reliable normalization of all studied parameters.

Conclusions. Timely multidisciplinary differential diagnostics of BA with histological examination of liver biopsies contributes to the differentiated choice of surgical intervention before the onset of irreversible multiorgan changes in the organs and the death of the child. KO in most cases is a palliative surgical intervention that improves biochemical parameters, PD, NPD indicators. In some cases, KO operation is a sufficient method of surgical correction of BA and normalization of all studied parameters. LT is a modern and most effective method of correcting BA, which leads to the normalization of all studied indicators.

The research was carried out in accordance with the principles of the Declaration of Helsinki. The research protocol was approved by the local ethics committee of the institutions mentioned in the work. Parents' informed consent was obtained for children's participation in the study.

The author declares no conflict of interest.