Unilobar Caroli's disease and its management in an 8-year-old girl

Abstract

A case of unilobar Caroli's disease in an 8-year-old girl treated with left hepatectomy is reported here.

INTRODUCTION

Caroli's disease[1] is a rare disease of the hepatobiliary system and has two variants: Caroli disease, characterized by ectasias of the intrahepatic bile ducts without other abnormalities, and Caroli syndrome, in which congenital ductal dilatation is associated with features of congenital hepatic fibrosis, portal hypertension, and renal disease.[2] Choledochal cysts have also been associated with Caroli disease.[3–5]

Partial hepatectomy may be curative in cases when the disease is confined to a single lobe. There are about 140 cases reported in literature managed by partial hepatectomy for unilobar Caroli's disease[6,7] but very few cases have been reported in children less than 10 years of age.

CASE REPORT

An 8-year-old girl was referred with a history of multiple episodes of epigastric pain, high-grade fever, and jaundice since 5 years of age. The episodes were associated with clay colored stools and high colored urine. Liver function tests were normal except for mildly raised serum alkaline phosphatase (Total bilirubin – 0.3 mg%, Serum glutamic oxaloacetic transaminase (SGOT)/Serum glutamic pyruvate transaminase (SGPT) – 47/34 IU/L, and Serum alkaline phosphatase – 996 IU/L). Ultrasonography revealed dilated intrahepatic biliary radicles (IHBR), dilated common bile duct (CBD) with multiple calculi in gall bladder. Magnetic resonance cholangiopancreatography showed atrophy of left lobe of liver, cystic dilatation of left lobar duct and IHBR, prominent right hepatic duct, multiple gall bladder calculi, dilatation of CBD (1 cm) with no evidence of calculus, and smooth narrowing of CBD in its distal part [Figure 1]. The pancreatic duct was normal with short common pancreatobiliary channel (0.8 cm).

Figure 1.

Magnetic resonance cholangiopancreatography images (a) axial and (b) coronal sections showing atrophy of left lobe of liver, cystic dilatation of left lobar duct and intrahepatic biliary radicles, prominent right hepatic duct, multiple gall bladder calculi, dilatation of common bile duct (CBD) (1 cm) with no evidence of calculus, and smooth narrowing of CBD in its distal part

On surgical exploration, the left lobe of liver was atrophied, fibrotic and multiple calculi could be palpated in the left lobe of liver. There was a clear demarcation between the healthy right lobe and the diseased left lobe of liver. The gall bladder was filled with multiple calculi. Right hepatic duct and CBD were dilated with no calculus. The left hepatic duct was significantly dilated with presence of multiple calculi. A stone of size 1 cm × 1 cm was present in a large diverticulum arising from left hepatic duct. Left hepatic artery was anomalous and originating from the left gastric artery. An accessory right hepatic artery originated from the superior mesenteric artery. Left hepatectomy was performed with a Roux-en-Y right hepaticojejunostomy. Histopathology of the resected specimen revealed huge, cystic dilation of large intrahepatic bile ducts, ulcerated duct lining, inspissated bile within the lumen of bile ducts, and chronic inflammatory cells.

The post-operative period was uneventful. She was put on ursodeoxycholic acid to prevent future episodes of primary hepatolithiasis and cholangitis[4] and is asymptomatic at 6-month follow-up.

DISCUSSION

Most patients with Caroli's disease have a unilobar fusiform dilation of the intrahepatic ducts, most commonly involving the left ductal system. In patients with Caroli's disease limited to one lobe of the liver without the presence of concurrent cirrhosis, hepatic resection with or without Roux-en-Y cholangiojejunostomy, remains the treatment of choice. Segmental fibrosis and atrophy do not respond to drainage and mandate segmental resection.[8] Alternative approaches to localized intrahepatic Caroli's disease have included external T-tube biliary decompression or internal drainage via choledochoduodenostomy, Roux-en-Y choledochojejunostomy, or Roux-en-Y hepaticojejunostomy.[9,10] These alternatives are often ineffective because ductal drainage is distal to the intrahepatic cysts. If resection is not feasible in localized disease, Roux-en-Y intrahepatic cholangiojejunostomy to the intrahepatic cyst is preferable. Hepatolithiasis is encountered in a third of the patients with Caroli's disease.

The outcome of patients with Caroli's disease involving both lobes of the liver or associated with portal hypertension from congenital hepatic fibrosis or secondary biliary cirrhosis remains poor.[11] Long-term medical therapy, including antibiotics, analgesics, and litholytic agents, may improve but does not eliminate symptoms permanently. In selected patients with diffuse intrahepatic Caroli's disease but with dominant unilobar disease, extended hepatic resection has been advocated,[12] but long-term benefit remains unproven. Orthotopic liver transplantation appears to be an effective curative option for the treatment of these patients who cannot be operated radically.[13]