0 Frequency is 1/8000-25000 live births. It is more common in girls.
0 Extrahepatic bile ducts are partially or completely atresia and therefore bile cannot pass into the intestine.
0 The structure of the extrahepatic bile ducts in biliary atresia is anatomically 3 types:
Type 1: Atresia at the level of the common bile duct
Type 2: Atresia at the level of the hepatic duct
Type 3: Atresia at the level of the porta hepatis {most common type}
It occurs as a result of dynamic, progressive sclerosis and fibrosis that starts in the IU period and continues in the postpartum period.
Clinic
• It constitutes 25-33% of newborn's cholestatic jaundice.
• The most prominent signs and symptoms are jaundice, acholic stool and hepatomegaly.
• Dark urine, sclera and conjunctiva have a yellow appearance.
• Jaundice appears in the first week of life. Initially, nutrition and weight gain are normal.
• After a while, anemia, malnutrition and growth retardation occur due to the inability to absorb fat-soluble vitamins and the progression of cirrhotic changes in the liver.
• Intracranial hemorrhages may occur due to vitamin K deficiency.
• the baby's skin and sclera are icteric. The liver surface is hard and the edge is blunt.
• Partal hypertension findings appear after 2 months (splenomegaly, ascites, anemia, prominent anterior abdominal wall veins).
Diagnosis
• If there is conjugated hyperbilirubinemia exceeding 2 weeks in term babies and 3 weeks in premature babies, as well as acholic stool, dark urine, large and hard liver, obstructive jaundice that requires surgery should be considered.
• Duodenal fluid aspiration is a fast, inexpensive and non-invasive test. Babies with atresia do not have bile in the duodenal fluid.
• In ultrasonography; A hyperechoic liver, the presence of a small fibrotic gallbladder, and the inability to visualize the external bile ducts make the diagnosis.
• ERCP, MRCP and hepatobiliary scintigraphy are used in diagnosis.
• The most effective methods in diagnosis; biopsy and hepatobiliary scintigraphy.
• The most prominent finding in biopsy is cholestasis. The most typical histological changes are ductal proliferation and portal fibrosis.
• Differential diagnosis with neonatal hepatitis is important and difficult.
Treatment
• Biliary cirrhosis and death will occur if treatment is not done within the first two months.
• The younger the hepatic portoenterostomy is performed, the better the prognosis.
• The most important factor determining morbidity after portoenterostomy is the degree of destruction of the intrahepatic bile ducts.
• Its treatment is Kasai hepatic portoenterostomy.
• Complications: The most serious is cholangitis; severe hypertension, hypersplenism and cirrhosis.