Journal of Postgraduate Medicine
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Year : 1989  |  Volume : 35  |  Issue : 3  |  Page : 186-8  

Congenital cystic biliary disease (a case report).

BV Tantry, SK Yadav, VV Kumar, AA Padhee 

Correspondence Address:
B V Tantry

How to cite this article:
Tantry B V, Yadav S K, Kumar V V, Padhee A A. Congenital cystic biliary disease (a case report). J Postgrad Med 1989;35:186-8

How to cite this URL:
Tantry B V, Yadav S K, Kumar V V, Padhee A A. Congenital cystic biliary disease (a case report). J Postgrad Med [serial online] 1989 [cited 2022 May 25 ];35:186-8
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Developmental abnormalities of bile ducts causing aberrant cyst formation have been grouped together as cystic disease complex syndrome.[1] When congenital dilatation involves intrahepatic bile ducts of a sector, lobe or both halves of liver, it is termed as Caroli's disease. In majority of patients, pure form as originally described by Caroli et al[4] is rare and congenital hepatic fibrosis is commonly associated.[6] Here we are reporting a case of Caroli's disease associated with congenital hepatic fibrosis presenting as prolonged pyrexia.


K.P.S., a 24 year male labourer attended the gastro-enterology clinic with chief complaint of intermittent. moderate grade fever for 72 years. Six months prior to the admission, he had jaundice accompanied by fever which lasted for 15 days. There was no history of abdominal pain, gastro-intestinal haemorrhage and symptoms pertaining to respiratory, cardiovascular and nervous systems. Five years bank he had polyuria for which he was prescribed thiazides and the patient responded.

Examination of the patient revealed mild pallor, icterus and fever (100.4░F). On abdominal examination, liver was 3 cm palpable, firm, smooth and nontender. Spleen was also palpable (12 cm in its long axis). There was no free fluid in the peritoneal cavity. Cardiovascular and respiratory systems were normal.

Investigations showed haemoglobin of 8.5 gm/ dl and normal total and differential leucocyte courts. Platelets were 1,40.000/mm3, reticulocyte count 2% and ESR 150 min/1st hour. Liver function tests showed total serum bilirubin 59.8 Ámol/L, direct bilirubin 35.9 Ámol/L, serum asparate aminotransferase 96 IU/L, alanine aminotransferase 16 IU/L and alkaline phosphatase 720 IU/L. Blood sugar, urea, serum electrolytes and serum osmolality were within normal limits ns were urinary sodium and osmolality. Plain X-ray of the abdomen revealed calcification in the hepatic area. Spleno-portovenogram demonstrated extensive retro-peritoneal collaterals communicating with inferior vena cava. Splenic and portal veins were not visualized. Intravenous pyelogram was normal Abdominal ultrasound revealed multiple hypoechoic areas in the liver containing rounded hyperechoic area with posterior acoustic shadowing. Extrahepatic bile duct was normal and splenic and portal veins were patent [Fig.1]. On percutaneous transhepatic cholangiography multiple intrahepatic cysts communicating with biliary system were found. These biliary cysts contained rounded filling defects (calculi). Common bile duct was normal [Fig.2]. Histopathology of liver- biopsy specimen showed extensive periportal fibrosis with bile duct proliferation and evidence of cholangitis [Fig. 3].


Multiple hepatic cysts harbouring calculi and communicating with intrahepatic bile ducts are typically described in Caroli's disease.[6] Caroli's disease commonly involves both lobes of the liver,[6] however, segmental involvement has also been described[7],[9] and in these patients resectional surgery is favoured.[6] When Caroli's disease is associated with portal hypertension, the commonest cause is congenital hepatic fibrosis, as seen in our case. This association has been described in 10%[7] of the cases.[6] Spleno-portovenogram may reveal characteristic picture in congenital hepatic fibrosis comprising of duplication of intrahepatic portal venous channels,[8] however, in our case this was not observed as portal and splenic vein were not visualised. This non-visualization of splenic and portal vein in intrahepatic portal hypertension has been attributed to excessive blood flow through the collaterals bypassing the portal system.[3]

Congenital hepatic fibrosis is commonly associated with medullary sponge kidney[6] but occasionally with medullary cystic disease also. Adult form of medullary cystic disease manifesting as polyuria without renal failure as found in our case has so far been not described in English literature in association with congenital hepatic fibrosis. In these cases, usual investigations e.g. intravenous pyelogram, ultrasonography or even closed renal biopsy may be normal.[5]

In general, management of Caroli's disease involving both lobes has been unsatisfactory.[7] However, in isolated instances drainage by T-tube, choledochoduodenostomy[6] or transhepatic decompression[9] have been found to be successful. Prognosis of generalized Caroli's disease is still poor. Recurrent cholangitis, liver abscess and septicemia are quite often responsible for fatal outcomes


We sincerely acknowledge the Medical Superintendent Surg. Cmde. A. Jagadishchandran for allowing us to publish this case report and Miss Vatsala for typing this manuscript.


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2Boichis, H., Passwell, J., David, R. an Miller, H.: Congenital hepatic fibrosis and nephronophthisis. Quart. J. Med , 42: 221-233, 1973.
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5Harrison, A. R.: Cystic disease of the kidney. In, "Postgraduate Nephrology" Editor: F. Marsh. Williams Hernemann Medical Books, London. 1985, PP. 411-424.
6Mail, J. C., Ghahremani, G. G. and Boyer, J. L.: Caroli's disease associated with congenital hepatic fibrosis and renal tubular ectasia. A case report. Gastroenterology, 66: 1029-1035, 1974.
7Mercadier, M., Chigot, J. P., Ciot, J. P., Langlois, P. and Lansiaux, P.: Caroli's disease. World J. Surg., 8: 22-29, 1984.
8Odievre, M., Chaumont, P. Montague, J. Ph. and Alagille, D.: Anomalies of the intrahepatic portal venous system in congenital hepatic fibrosis. Radiology, 122: 427-430, 1977.
9Witlin. L. T., Gadacz, T. R., Zuidema G. D. and kreilabaugh, W. W.: Transhepatic decompression of the biliary tree in Caroli's disease. Surgery, 91: 205-209 1982.

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