A young adult with biliary atresia and liver decompensation

Biliary atresia is a progressive fibro-obliterative disease of the bile duct, commonly diagnosed within a few months of life. After hepatoportoenterostomy was successfully performed, about one fourth of biliary atresia patients have grown up to the age of twenty with their native liver. However, progression to liver cirrhosis, development of portal hypertension, or cholangitis are the major problems of adult with biliary atresia. Monitoring of these complications and listing for liver transplantation whenever indicated are the key when caring adult with biliary atresia. Figure 1 แสดง CT abdomen พบว่ามีตับโต และมีลักษณะของตับแข็งและ ภาวะความดันของระบบหลอดเลือด portal สูงขึ้น (collateral vessels, splenomegaly, ascites) พบการขยายตัวของท่อน้ำดีในตับ (multifocal dilated ducts and peribiliary cysts along bilateral intrahepatic ducts) และต่อมน้ำเหลืองโตในช่องท้อง (mesenteric, gastrohepatic, hepatoduodenal, aortocaval, para aortic, right anterior diaphragmatic, lower paraesophageal lymph node ขนาดสูงสุด 1.8 ซม.)