Choledochal cysts in infants and children.

ABSTRACT

OBJECTIVE: To study the clinical spectrum and management of choledochal cyst in children below 12 years of age. DESIGN: Descriptive study. SETTING: Tertiary care hospital. METHODS: Twenty three children with choledochal cysts were managed between January 1991 to September 1997 and their clinical details, investigations and management were recorded. Choledochal cyst was diagnosed by ultrasonography and confirmed by ERCP or peroperative cholangioram (POC) Children were treated with antibiotics and/or percutaneous transhepatic biliary drainage if there was cholangitis and subsequently subjected to surgery (excision of the cyst and jejunal loop interposition hepaticoduodenostomy). RESULTS: The median age of these children was 3 years with an almost equal sex ratio. Predominant presentation was jaundice in 18, pain abdomen in 15, fever in 12, and lump abdomen in 9 cases. The classical triad of jaundice, pain and lump was present in only 4 cases. ERCP conducted in 7 and POC in 14 cases yielded positive findings in all. Clinically there were two distinct forms of presentation (i) infantile form (< or = 1 year) comprised 9 infants which presented with jaundice in all, acholic stool in 6, lump abdomen in 4 but only one had classical triad; and (ii) childhood form (> 1 year) presented with pain abdomen in 12 and jaundice and cholangitis in 9 subjects each. Type I cyst was seen in 20 and type IVa in 3. Two children refused surgery, and the rest underwent surgery. Three infants died after surgery, the remaining 18 were alive and well on follow-up (median 25 months). Secondary biliary cirrhosis was seen in 6, extra hepatic biliary artresia in 2 and congenital hepatic fibrosis in 1 on histology. CONCLUSIONS: Choledochal cysts present in two clinically distinct forms. Infantile form is an important cause of cholestasis of infancy. Early diagnosis and referral is essential to prevent complications and death, and prognosis after surgery is good.