| Abstract|| |
When Caroli disease (CD), defined as a congenital dilation and ectasia of the segmental intrahepatic bile ducts with or without cystic dilatation of the extrahepatic biliary tree, is associated with congenital hepatic fibrosis, it is termed Caroli syndrome (CS). We describe the case of a 38-year-old woman with CS, having diffuse involvement of the liver, who was successfully treated with orthotopic liver transplantation (OLT).
Keywords: Caroli disease (CD), Caroli syndrome (CS), orthotopic liver transplantation (OLT)
|How to cite this article:|
Sutariya VK, Modi PR, Tank AH. Orthotopic liver transplantation in an adult with Caroli syndrome: Case report. Ann Trop Med Public Health 2016;9:58-60
|How to cite this URL:|
Sutariya VK, Modi PR, Tank AH. Orthotopic liver transplantation in an adult with Caroli syndrome: Case report. Ann Trop Med Public Health [serial online] 2016 [cited 2020 Aug 6];9:58-60. Available from: http://www.atmph.org/text.asp?2016/9/1/58/168720
| Introduction|| |
Caroli disease (CD) is a rare autosomal recessive inherited disorder, characterized by nonobstructive saccular or fusiform segmental dilatations and ectasias of the intrahepatic bile ducts.  The following two types have been mentioned: One pure form and the other associated with hepatic fibrosis, which is known as Caroli syndrome (CS). Both these diseases can be life-threatening if complicated by recurrent attacks of cholangitis, portal hypertension, or cholangiocarcinoma. Although hepatic resection may cure the disease localized to one lobe of the liver, orthotopic liver transplantation (OLT) is the only definitive treatment option for patients with diffuse form of CS. Here, we describe a case report of OLT for CS.
| Case Report|| |
A 38-year-old woman presented with history of recurrent episodes of jaundice, abdominal pain, and fever. She had a history of admission and treatment with antibiotics and intravenous fluids for similar complaints on five instances. Her laboratory investigations were the following: Total serum bilirubin 6.9 mg/dL, direct 5.5 mg/dL, and indirect 1.4 mg/dL, alanine amino transferase 93 IU/L, aspartate amino transferase 97 IU/L, alkaline phosphatase 480 IU/L, serum creatinine 0.46 mg/dL, international normalized ratio for prothrombin time 1.17, hemoglobin 8.8 g/dL, and white blood cell count 8,000 per cubic liter. Her autoimmune antibody profile was negative. Doppler study of her portal venous system was suggestive of dilated portal vein, dilated splenic vein, and splenomegaly. Her computed tomography (CT) scan was suggestive of irregularly dilated intrahepatic biliary radicals with periportal fibrosis in both lobes of the liver [Figure 1]. Magnetic resonance cholangiopancreatography was suggestive of dilated intrahepatic biliary radicals. Her upper gastrointestinal endoscopy was suggestive of grade 3 esophageal varices. Her liver biopsy was suggestive of chronic hepatitis with fibrosis and marked cholestasis. Considering all investigations, she was diagnosed as having CS with recurrent episodes of cholangitis.
|Figure 1: CT scan showing segmental dilatation of intrahepatic biliary radicals involving both lobes of the liver|
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In view of her recurrent nature of cholangitis and extensive disease involving both lobes of the liver, she was offered OLT. Liver transplantation was performed by the modified piggyback technique. Roux-en-Y hepaticojejunostomy was performed with excision of the extrahepatic biliary tree. The diagnosis was confirmed by histology of the explanted liver [Figure 2]. On the fifth postoperative day, the patient developed fever with upper abdominal pain. On CT scan evaluation of her abdomen, collection was encountered in her hepatorenal fossa. She was rediagnosed due to suspension of bile leak. However, no leak was found. Otherwise, her postoperative course was uneventful. She was discharged on the 25th postopearative day. She developed two episodes of rejection, which were successfully managed by steroids. After a 12-month follow-up, she was found to be well-maintained on tacrolimus monotherapy.
|Figure 2: Histopathological examination of explanted liver showing dilated biliary radicals with extensive periportal fibrosis|
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| Discussion|| |
Although initially described as an entity of multiple saccular dilatations of only the intrahepatic ducts, the term CD has been broadly applied to describe any patient with intrahepatic bile duct cysts, regardless of the presence of extrahepatic bile duct cysts. Both CD and CS are believed to be caused by an intrauterine event that arrests ductal plate remodeling at different levels: Larger bile ducts for CD and smaller ducts for CS.  It may be localized to only one lobe or may affect both lobes. It affects both genders equally. The patients usually present with a history suggestive of recurrent cholangitis. However, some patients do present with features of portal hypertension. Diagnosis is usually established by CT scan. Other studies like endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiopancreatography are occasionally required. Liver biopsy is the confirmatory investigation.
Medical management with properly directed antibiotic therapy and ursodeoxycholic acid may stabilize the disease. But most of the patients develop recurrent cholangitis due to bile stasis and hepatolithiasis. Patients with attacks of cholangitis can improve temporarily by biliary drainage procedures. However, bilioenteric drainage procedures are associated with high infectious morbidity. Although hepatic resection is the treatment of choice for monolobar disease with very good long-term results, disease progression in the residual liver has been reported. , Review of the previous literature showed that overall graft and patient survival is no different for patients with CD or CS after OLT, from those patients undergoing OLT for other etiologies. , It also showed that higher mortality, because of infectious complications, in the first year of transplantation prevented better long-term survival. The patients should be free of clinically evident cholangitis at the time of transplantation. Since patients with CD are at risk of developing cholangiocarcinoma and as the outcome of OLT in the presence of cholangiocarcinoma is poor, OLT should be considered timely for patients with CS. 
To conclude, liver transplantation is a form of curative and lifesaving therapy for patients with CS, especially those with recurrent attacks of cholangitis not responsive to medical management and those having decompensated liver disease. 
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Pranjal R Modi
Department of Gastrointestinal Surgery and Liver Transplantation, IKDRC-ITS, Civil Hospital Campus, Asarwa, Ahmedabad, Gujarat, 380016
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2]