Infantile Choledochal Cysts (IFCC) usually present with jaundice, acholic stool and abdominal lump or abdominal distension. If the surgical intervention is delayed, they rapidly progress to liver fibrosis which is considered to be irreversible if progressed to cirrhosis. We present the data of four cases (aged one month to seven months) of IFCC presented with cholangitis managed in one surgical unit in last two years. In one case, cholangitis was treated with prolonged antibiotic course before definitive repair whereas in rest, external drainage of cyst was done in addition to intravenous antibiotic to treat cholangitis. All the infants had features of cholangitis at time of presentation. Total leucocyte count ranged from 18x1000/UL to 30.6x1000/UL. Total bilirubin level at presentation ranged from 8.2 mg/dl to 18 mg/dl and Prothrombin time (INR) ranged from 1.33 to 1.9. Hepatic fibrosis was observed in all cases but cirrhosis was observed in only one case. There was no mortality but one patient had postoperative complication with prolonged hospital stay. External drainage helps in early recovery from cholangitis and better optimization of liver function. It also delays further progression to liver fibrosis by relieving the biliary outflow obstruction while waiting for definitive repair.

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