[size=20px]A 5 week old term infant is jaundiced and has acholic stools. Her total serum bilinubin is 9 mg/dl (conjugated 7 mg/dl). Ultrasonography shows a small gallbladder, with no choledochal cyst or other abnormalities. Hepatobiliary (HIDA) scan after five days of phenobarbital treatment shows radionuclide uptake into the liver, but no excretion into the intestine at twenty-four hours. At operative exploration, a small gallbladder is identified. Aspiration of the gall bladder yields a small amount of green fluid. An operative cholangiogram shows free passage of contrast through the common hepatic duct into the duodenum, small right and left extrahepatic ducts, and absence of intrahepatic filling. The most appropriate management is :

 

A. biopsy of the liver and closure of the abdomen

 

B. resection of the extrahepatic ducts and reconstruction with hepatoportoenterostomy (Kasai procedure)

 

C. cholecystoportoenterostomy (gallbladder Kasai procedure)

 

D. irrigation of the extrahepatic ducts through gallbladder with 5% N-acetyleysteine

 

E. transduodenal sphincteroplasty[/size]

Edited December 1, 20205 yr by Admin

B

Think again my friend

C

Think again my friend

A

The correct answer is A. Alagille's syndrome is a congenital cholestatic disorder characterized by a paucity or absence of intrahepatic bile ducts. This syndrome is caused by a mutation or deletion of the JAGI (JaggedI) gene, which encodes a cell surface protein which functions as a ligand for the Notch transmembrane receptors. In the syndromic form (arteriohepatic dysplasia), extrahepatic features such as peculiar facies, vertebral abnormalities, and ocular abnormalities co-exist with cardiovascular and renal abnormalities. Liver transplantation is necessary in up to one half of patients who have hepatic symptoms in infancy. The hepatic clinical manifestations of Alagille's syndrome are varied and range from mild cholestasis to chronic liver failure. Some literature suggests that preoperative percutaneous liver biopsy may be diagnostic of Alagille's syndrome and thus avoid need for surgical exploration, although this remains controversial. Attempts at biliary drainage by hepatoportoenterostomy or cholecystoportostomy generally fail. As well, these repairs may increase the risks of cholangitis and adhesions, which can complicate eventual transplantation surgery. (I. General pediatric surgery B. Hepatobiliary)

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