Liver transplantation in a 7-month-old girl with Caroli's disease

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“…It is a rare disorder, with a prevalence of 1 in 1 million people. 4 The pathology starts in the intrauterine period, 5 and it is characterized by multifocal fibrocystic dilatation of the intrahepatic bile ducts. [4][5][6] Caroli disease can be seen at any age, 4 but it is more common in those in their twenties.…”

“…4 The pathology starts in the intrauterine period, 5 and it is characterized by multifocal fibrocystic dilatation of the intrahepatic bile ducts. [4][5][6] Caroli disease can be seen at any age, 4 but it is more common in those in their twenties. 7 Most of the patients present to the hospital with abdominal pain, fever, hyperbilirubinemia, elevated alkaline phosphatase, hepatomegaly, or symptoms of portal hypertension.…”

“…7 Most of the patients present to the hospital with abdominal pain, fever, hyperbilirubinemia, elevated alkaline phosphatase, hepatomegaly, or symptoms of portal hypertension. 4,7 Caroli disease is characterized by recurrent episodes of biliary obstruction, cholangitis, hepatolithiasis, and liver abscesses. There are 2 treatment options for Caroli disease.…”

“…Up to 87% of patients can be treated with liver resection. 4 To decide between liver transplant or resection, liver functions, portal hypertension, hepatic fibrosis, extent of the disease, and suspect for cholangiocarcinoma must be evaluated. If there are signs of liver failure, liver transplant is recommended.…”

“…If there are signs of liver failure, liver transplant is recommended. 4,7 In the literature, most patients who are treated with liver transplant for Caroli disease are adult patients, 8 and graft and patient survival rates are no different from those shown in patients who undergo orthotopic liver transplant for other causes of chronic liver disease. 7,8 Our patient was an 8-year-old female patient.…”