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CAROLI DISEASE AND CAROLI SYNDROME

1999 
Caroli syndrome consists of Caroli disease and congenital hepatic fibrosis [1]. It is an uncommon congenital disorder of the intrahepatic bile ducts. The disease is presumably of autosomal recessive hereditary character [2]. A classical case history of the Caroli syndrome is presented. Non surgical management with endoprosthesis is discussed. Case Report A 10-years-old boy was admitted with complaints of pain in the upper abdomen, fever, vomiting and lump abdomen of 2 1/2 months duration. There was no family history of hepatic disease. He was poorly developed. Weight was 20 kg and height was 128 cm. He was febrile (102 °F). No icterus was present. The abdomen was full in upper part. The liver was firm, nontender and palpable 7 cm below the right costal margin. Features of portal hypertension in the form of ascites, splenomegaly and distended abdominal wall veins were present. Laboratory investigations revealed Hb 6.2 gm%, TLC 12,200/mm3; Liver function tests: S.bilirubin 2.8 mg%, ALT 21 IU/L, AST 56 IU/L, ALP 89 IU/L, Total proteins 8.5 gm/dl, Albumin 3.0 gm/dl, Globulin 5.6 gm/dl and A:G Ratio 0.55:1. HBsAg was negative by RPHA. Serum Amylase was 92 Somogyi Units. Ultrasonography revealed multiple cystic dilatations in both lobes of liver and both kidneys (renal polycystosis). Computed tomography (Fig 1) and endoscopic retrograde cholangiography (Fig 2) confirmed the presence of multiple communicating dilatations of the bile ducts affecting the whole of the liver but mainly of right lobe of liver. Patient was managed with nasogastric aspiration, broad spectrum antibiotics, blood transfusions and other supportive measures. Endoscopic papillotomy and nasobiliary drainage (endoprosthesis) was carried out to decompress the biliary tree. The cholangitis resolved and patient is in the waiting list for liver transplantation. Open in a separate window Fig. 1 Computed tomography scan of liver demonstrating multiple cysts scattered through out the whole liver. A single large cyst is seen in the posterio-inferior aspect of the right lobe of the liver.
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