Cystic form of biliary atresia. Treatment experience

Abstract

Background. The cystic form of biliary atresia is a rare form of atresia of the biliary tract, which is a relatively favorable variant of the defect and can be diagnosed antenatally. In practice, it is important not only to suspect this diagnosis, but also to differentiate this variant of impaired development of the external bile duct from the cyst of the common bile duct. This is due to the difference in approaches and methods of surgical treatment of choledochal cysts and biliary atresia. Obliteration (atresia) of the bile ducts in the absence of timely surgical intervention quickly leads to the progression of cirrhosis and the development of liver failure. The method of choice in the treatment of AD is Kasai surgery, often palliative in nature, but allowing to delay the time until liver transplantation. The cyst of the common bile duct rarely requires early surgical treatment, and the risk of cirrhosis is significantly lower. Surgical intervention is aimed at removing the cyst and restoring the flow of bile by anastomosing the external bile ducts with the intestines, which is a radical method of treatment and leads to the recovery of the child. External similarity in ultrasound examination of the fetus and newborn baby of the cystic form of biliary atresia of the bile ducts with a cyst of the common bile duct does not always allow differentiation of one defect from another, which can lead to untimely correction of the defect and an unfavorable outcome.
 Aim. Demonstrate a rare type of biliary atresia.
 Materials and methods. Between 2001 and 2019, 33 patients with biliary atresia were treated in the Childrens City Multidisciplinary Clinical Specialized Center for High Medical Technologies in St. Petersburg, only two patients had a cystic form. Both children were initially treated as patients with bile duct cyst. Children were operated on at the age of 2 and 3.5 months. The first patient underwent surgery Kasai, the second hepaticoyunoanastomosis.
 Results. During the observation period (9 years and 4 years), the synthetic function of the liver is normal, and there are currently no indications for transplantation.
 Conclusion. If a fetus or a newborn with neonatal jaundice is detected during ultrasound examination of a cystic formation in the gates of the liver, it is very important to correctly and quickly make a differential diagnosis between the cystic form of biliary atresia of the biliary tract and the common bile duct cyst.