The liver in choledochal cyst.

OBJECTIVE

Hepatic changes due to choledochal cyst have not been given due emphasis in the published literature. In this study the gross and microscopic appearance of the liver in patients with choledochal cyst have been correlated with clinical features, liver function tests and postoperative complications.

METHODS

A retrospective evaluation of patients treated for choledochal cysts between January 1989-December 1998 was undertaken. The case records were reviewed and liver biopsies taken at surgery were analysed.

RESULTS

The case records of 22 patients were reviewed (16 girls, 6 boys; mean age 4.6 years, range 1 month-10 years). The presenting features included jaundice (59%; 85.7% in < 1 year), abdominal pain (59%; 86.7% in > 1 years) and fever in 40.9% cases. A palpable abdominal mass and hepatomegaly was present in 32% cases. A type IV cyst was present in 1 case; all others had type I cysts (14 cystic and 7 fusiform). The liver looked grossly 'normal' in 16 and 'cirrhotic' in 6 cases. Liver biopsies were available for review from 5 of the 'cirrhotic' and 7 of the 'normal' looking livers. All the liver biopsies showed varying degrees of bile duct proliferation, cholestasis, parenchymal damage, inflammatory cell infiltration and pericentral fibrosis. Histological features of cirrhosis were evident in 6 cases (4 'cirrhotic' and 2 'normal' looking livers), 4 of these cases were infants. Liver function tests were deranged in 5 cases with histological features of cirrhosis and hepato-biliary scintigraphy showed obstructive features in 3 of these cases. There was an increased risk of postoperative complications in the cases with histological features of cirrhosis, 2 died from hepatic insufficiency and one each had transient ascitic and biliary leak.

CONCLUSIONS

Liver histology showed significant changes in all the cases of choledochal cyst in whom it was studied; even normal looking livers showed evidence of significant changes. Presence of cirrhosis, more common in infants, correlated with jaundice, deranged liver function tests, obstructive features on hepatobiliary scintigraphy and a greater risk of postoperative complications.