Complications in Caroli Disease

Abstract

Purpose: Caroli disease is a rare inherited disorder characterized by nonobstructive, multifocal areas of saccular dilatation of intrahepatic biliary ducts. It is thought to be a genetically acquired disease, and is linked often to autosomal recessive polycystic kidney disease. It is diagnosed through imaging, and magnetic resonance cholangiopancreatography (MRCP) is emerging as the diagnostic modality of choice. A 62-year-old female with previously diagnosed Caroli disease and polycystic kidney disease presented as a hospital transfer for further evaluation of a complex liver cyst. Prior to admission, she had a complicated course with intermittent severe abdominal pain that was occurring over the past 3 weeks. Initially, when she sought treatment, she was provided an oral narcotic and sent home. The subsequent week, she developed fevers and returned to the outside hospital, was admitted, treated with unknown antibiotics for presumed colitis, and discharged after improvement. A week thereafter, she developed dyspnea on exertion, ascites, and edema of her lower extremities, causing her return to the hospital. During her second outside hospital admission, she was noted to be jaundiced and spiking intermittent fevers. Magnetic resonance imaging (MRI) was performed, which demonstrated a complex liver cyst abutting the upper pole of the right kidney, which appeared infected. Upon arrival to our facility, blood cultures were obtained, intravenous fluids were initiated, and piperacillin-tazobactam was continued. MRI showed a 6.7-cm hepatic cyst in segment VI of the liver with a fluid level, thickened walls, and edema in surrounding hepatic parenchyma. US-guided drainage of 60 mL of purulent material and fluoroscopic-guided 10 french drain was placed via the transhepatic approach. Cyst fluid revealed aerobic culture, showing no growth and anaerobic culture showing Bacteroides fragilis. The patient's abdominal pain improved, she remained afebrile, and blood cultures were negative. She was subsequently discharged with 6 weeks of levofloxacin and metronidazole, with follow-up appointments for her hepatic drain to be evaluated and with infectious disease. Morbidity and mortality from Caroli disease usually comes from complications including cholangitis, hepatolithiasis, biliary abscesses, sepsis, cirrhosis, portal hypertension, cholangiocarcinoma, and renal failure. Prognosis is determined largely by the frequency and severity of cholangitis, which can cause sepsis or death. This case illustrates the potential for complications in Caroli disease and the importance of definitive identification of any source of abdominal pain in these patients. Delay in diagnosis and treatment can lead to significant morbidity and mortality.