Choledochal cysts in infancy and childhood

Abstract

Objective

To study the clinical presentation, management, and results of treatment of 41 consecutive cases of choledochal cyst (CC) managed from 1999 to 2006.

Methods

The age of the patients ranged from 20 days to 11 years. Eleven cases were 1 year or less in age (infantile group), and 30 patients were more than 1 year old (classical pediatric group). Children less than 1 year old presented with jaundice (72%), hepatomegaly (54%) and clay-colored stools (63%); whereas those above one year in age presented with pain (83.3%) and jaundice (47.6%). Three cases had biliary perforation with localized or generalized biliary peritonitis. Ultrasonography (US) diagnosed/suggested CC in all 41 patients, however, contrast-enhanced computed tomographic (CECT) scan (n=12), radionuclide DISIDA scan (n=5), and magnetic resonance cholangiopancreatogram (MRCP) (n=3) also contributed to the diagnosis. 19 patients, including 4 from the ‘infantile’ group, had significant dilatation of intrahepatic biliary ducts.

Results

Nine patients from the ‘infantile’ group had cystic type I CC (type Ic), while 2 patients had fusiform dilatation of the common bile duct (type if disease). In contrast, 21 of the older patients had type If disease while 9 had type Ic disease. Operative management included primary complete excision of the cyst with a Roux-en-Y hepaticodochojejunostomy (HDJ) (n=32), Lilly’s modification of submucosal resection with a HDJ (n=8), and cyst excision with a hepaticodochoduodenostomy (HDD) (n=1). All 3 patients with biliary perforation had primary excision of the CC with a HDJ. Unusual operative findings included accessory hepatic ducts (n=2), anteriorly placed common or right hepatic arteries (n=3), and partially or completely blocked proximal extrahepatic bile ducts (n=3). There were 2 postoperative deaths, one had cirrhotic liver disease with portal hypertension, the other had deep icterus with poor general condition. Both patients succumbed to overwhelming sepsis. One patient required reexploration and refashioning of the HDJ for biliary leak. Short-term follow-up was satisfactory in all 39 patients.

Conclusion

Infants with CC constitute a distinct group with regard to clinical presentation and the pathological anatomy. CC should be kept in mind while evaluating neonates and infants with cholestatic jaundice, and older children with recurrent abdominal pain. Primary excision of the cyst with a HDJ provides satisfactory results in the management of the vast majority of cases of CC including those in infants, massive CC, and in the presence of biliary perforation and peritonitis.