Abstract Ref Number = APCP883
Caroli’s Disease in 4 Years Old Girl
Putu Ayunda Trisnia,I Gusti Ngurah Sanjaya Putra,I Putu Gede Karyana,Ni Nyoman Metriani Nesa
Udayana University supervisor of Department of Child Health Medical School, Udayana UniversitySanglah Hospital
Background : Incidence of Caroli’s disease in population is 1 : 1.000.000 live births with males and females are equally affected. The clinical manifestations are recurrent fever, abdominal pain especially in right hypochondrium, and recurent cholangitis. Various techniques have been performed, but liver transplantation still preferred. Inappropriate management can cause complications such as septicaemia due to recurrent cholangitis, malignancy, and death.
Case Presentation Summary : We report a case of 4 years old girl presented with yellowish of the skin and enlargement of the abdomen since one year ago. Patient was diagnosed with suspect autoimmune hepatitis previously, but didn’t show any improvement with adjuvant therapy. Laboratory finding showed elevated bilirubin level, transaminase serum, and ALP level. Doppler abdominal US revealed hepatomegaly with the sign of chronic liver disease, with cystic lession of the hepatical hilar region of the size about 3.3 x 3.1 cm. Abdominal CT scan showed cyst in lobus caudatus with enlargement of intrahepatic bile duct that supports Caroli’s disease. Patient was planned for a surgery (total or partial hepatectomy) and/or liver transplantation, but family refused both surgeries. After diagnosing Caroli’s disease was made, patient was hospitalized for several times due to infection and malnutrition. Ten months after diagnosed, the patient died during sleep at home.
Learning Points/Discussion : Caroli’s disease should be kept in mind as a differential diagnosis especially in children presented with abdominal pain and jaundice.
Keywords: Caroli's disease Jaundice abdominal CT Scan