Recurrent Hepatolithiasis and Hepatic Abscess Secondary to Caroli’s Disease – Case Report

Abstract

Background

Hepatolithiasis is the presence of gallstones in the intrahepatic ducts due to primary, idiopathic or infectious causes (lithogenic bile) or secondary causes such as congenital cysts and strictures or past hepatobiliary surgery. Typically, hepatolithiasis patients presents with the Charcot’s triad (abdominal pain, jaundice, fever) suggestive of cholangitis. It is not uncommon for severe cholangitis to be accompanied with a hepatic abscess. The treatment consists in supportive measures for cholangitis, subsequent stone extraction and surgical removal of strictures and bile drainage. Patients should be closely monitored because of the high risk of recurrence, liver cirrhosis or cholangiocarcinoma.

Case presentation

Our patient is a 61 years old male with the following medical history and presentation: Right nephrectomy before approximately 15 years, for polycystic renal disease. Seven years ago, he underwent the surgical procedure of cholecystectomy and choledocho-duodenal anastomosis for gallbladder and CBD stones. A year later, he undergoes an urgent surgical procedure for an intra-abdominal abcess (right subphrenic). For the next five years the patient had no complaints. Three months prior to the surgery he is admitted to the hospital for persistent, recurring episodes of fever (38.5 °C), which did not respond well to antibiotic therapy. Radiology confirms intrahepatic stones, bilateral cystic dilations of bile ducts (Caroli’s Disease) and an abscess of the VII^th liver segment. The patient continues a conservative treatment until optimal parameters are reached for the eventual procedure. The VII^th liver segment is partially resected, the hepatic calculi are extracted and a hepatico-jejunostomy performed, joining a Roux limb with the biliary confluence.

Discussion

Depending on patient history and current presentation in terms of the severity of the disease and the classification of hepatolithiasis, the surgeon has to weigh and decide upon possible treatment options. Among them we mention supportive measures to treat acute cholangitis, pharmacologic therapy (statins), percutaneous transhepatic cholangioscopic lithotomy, peroral cholangioscopic lithotripsy and surgery.

Conclusion

Patients with hepatolithiasis recurrence experience chronic cholangitis and eventually develop cirrhosis over a period of 10 to 20 years. Furthermore, the incidence of intrahepatic cholangiocarcinoma is about 5% to 10%. These patients require a long-term follow-up because of an increased risk of cancer after 10 to 20 years.

Keywords: General Surgery, Hepatobiliary Surgery, Hepatolithiasis, Caroli’s Disease, Congenital Biliary Cysts.

DOI: 10.7176/ALST/97-01

Publication date: January 31^st 2023