Diseases of bile duct in children

Figure 13  A term female infant with hepatic cyst detected by prenatal ultrasonography, presented with jaundice since birth. Repeated abdominal ultrasonography showed fusiform dilatation of extrahepatic and intrahepatic bile ducts, 3.3 cm × 4.5 cm, suggestive of choledochal cyst type IVa (Todani’s classification), containing bile sludge, and another large hepatic cyst in segment IV, 4.7 cm × 4.3 cm. After conservative treatment, clinical jaundice progressed. Liver function test showed total bilirubin/direct bilirubin (TB/DB) of 10.9/1.68 mg/dL, aspartate aminotransferase of 45, alanine aminotransferase of 23, alkaline phosphatase of 150, gamma-glutamyltransferase of 172 U/L, and albumin of 3.9 mg/dL. Computed tomography revealed a cystic lesion measuring 3.7 cm × 5 cm × 4 cm, at the common bile duct area, slight bilateral intrahepatic duct dilatation, a smooth thin wall hypodense lesions measuring 5.1 cm × 4.6 cm × 4.3 cm at hepatic segment 4a/8, and a few small hypodensity lesions at segment 7 that were suspected to be an infected hepatic cyst. After percutaneous aspiration of the hepatic cyst and intensive antibiotics, the cyst enlarged to 5.4 cm × 5.8 cm. Finally, choledochal cyst excision with Roux-en-Y hepaticojejunostomy of the subcutaneous limb was performed. One year later, ultrasonography revealed a reduction in the size of the lobulated cystic lesion to size 2.9 cm × 2.8 cm × 2.1 cm and mild dilatation of the intrahepatic bile ducts at the porta hepatis region. A: Fusiform dilatation of the extrahepatic cyst and intrahepatic bile ducts, measuring 3.3 cm × 4.5 cm, containing bile sludge; B: Another large cyst, 4.7 cm × 4.3 cm, in hepatic segment IV; C: Decreased size of the lobulated cystic lesion at the porta hepatis region with a few small hypoechoic lesions surroundings the cystic lesion, probably cysts or focal IHD dilatation; D: Mild dilatation of the intrahepatic bile ducts at the porta hepatis region.