Dr. Annasaheb Lokhande*, Dr. P. V. Kumavat**, Dr. N. S. Bhori***, Dr. Mrs. Savaskar S. V.****, Dr. S. S. Gandhi*****
Dept. of Paediatrics, Dr. V. M. Govt. Medical College, Solapur.*, Dept. of Paediatrics, Dr. V. M. Govt. Medical College, Solapur.**, Dept. of Paediatrics, Dr. V. M. Govt. Medical College, Solapur.***, Dept. of Paediatrics, Dr. V. M. Govt. Medical College, Solapur.****, Dept. of Paediatrics, Dr. V. M. Govt. Medical College, Solapur.*****
Caroli disease is a rare autosomal recessive disorder characterized by cystic dilatation of intrahepatic bile duct. Caroli disease may be a part of spectrum including congenital hepatic fibrosis, choledochal cyst and tubular ectasia of kidney. If dilatation involves one segment or if it is associated with choledochal cyst, it can be surgically treated. In case of bilobar involvement, treatment is mainly palliative and follow-up is needed to detect and treat complications. For those patients who cannot be operated radically, orthotropic liver transplantation appears to be a ray of hope. We report here a case of Caroli disease presented with bile-stained ascitis.

Case Report: Fourteen month old female baby presented with distention of abdomen, yellowish discoloration of urine and eyes and irritability of two months duration. On examination, she was afebrile, tachypneic, pale, icteric and irritable with abdominal distension and free fluid in abdomen, with no organomegaly. Other systems were within normal limits. After hospitalization, distension of abdomen gradually increased. Investigations revealed, Hemoglobin 62 gm% TLC/DLC and Platelet count were in normal range.
  • Sr. Bilirubin total 7.5 mg%
  • Direct 6.0 mg%
  • Indirect 1.5 mg%
  • SGOT 60/U/dl. SGOT - 40 IU/dl
  • Serum Alkaline phosphatase - 460 KA/dl

Ultrasonography of abdomen was suggestive of diffuse liver parenchymal disease, with cystic dilatation of intrahepatic bile duct and Ascitis with internal echoes suggestive of secondary infection. Kidney echotexture and dimensions were normal.

Abdominal paracentesis was suggestive of bile stained ascitic fluid. In view of bile-stained ascitic fluid, some leak was suspected, so percutaneous transhepatic cholangiography was performed. However, no leak was evident on transhepatic cholangiography.

Computed tomography of abdomen showed dilated tortuous right hepatic duct and common hepatic duct (ecstatic dilation of intrahepatic biliary tract). Surgical opinion was taken and repeated therapeutic paracentesis was done. Before surgical intervention was planned, patient went against medical advice and did not appear again.
Caroli's disease was initially reported as communicating cavernous ectasia of biliary tract. Jacques Caroli, a French physician reported this disease as distinct clinical entity, incidence of which is 1 in 150,000 cases. (1)

The dilatation usually involves the entire liver, but cases limited to one segment or one lobe have reported. In the "simple" type of congenital ductular ectasia, the periportal fibrosis is limited.

One postulated theory is that it is combination of disproportionate overgrowth of biliary epithelium and its supporting connective tissue. (1)

A second theory of choledochal cyst: Development concerns abnormal canalization of the bile duct during embryogenesis with distal obstruction. Thus proximal pressure leads to weakening of the common duct wall. (1)

Another hypothesis is that cystic dilatation is a result of ischemic infarctions secondary to peripheral hepatic artery occlusion.
Clinically, females (more common) are sufferers with classic triad of jaundice, right upper quadrant mass (abdominal distension) and abdominal pain. Symptom mimicking biliary colic or acute cholecystitis may be present in a minority of patient.

Associated cystic dilatation of kidneys is seen in 60-80% of the cases, most frequent being medullary sponge kidney. These patients are usuallyasymptomatic from a renal standpoint but may develop renal stone disease and infections. Congenital hepatic fibrosis is also associated with renal cysts but here renal failure dominates the picture. Caroli disease can be associated with choledochal cyst, this type is however inherited in an autosomal dominant pattern. (5)
With rapid advances in non-invasive techniques like Ultrasonography (USG), Computed Tomography (CT) and Magnetic resonance imaging (MRI), Caroli disease, nowadays is more frequently diagnosed. USG is the initial investigation of choice. On ultrasonography, the pure form shows diverticulum-like sacculi of intrahepatic biliary tree, more pronounced towards the center and can be segmental or generalized. The combination of endoscopic retrograde cholangiopancreatography (ERCP) and USG leads to a more reliable diagnosis to the extent of the disease which is essential for appropriate management.(2) CT serves as a useful preoperative roadmap if insufficient data is obtained on USG, however with advent of MRI and MRCP (Magnetic Resonance Cholangiopancreatography), invasive procedures like ERCP and biopsy are rarely indicated. (3)
Plain radiography of the abdomen may very rarely reveal small intrahepatic bile duct calcification. Cholangiography is best suited to depict the multiple ductal dilatation, called the "lollipop tree" aspect.
It is important to demonstrate the communication between cystic ductal dilatation and the bile duct system either by cholangiography or by ultrasound in order to distinguish Caroli disease from polycystic liver disease or multiple simple hepatic cyst. (2)
CT scan is an excellent way to demonstrate the extent of disease. "Central dot sign" corresponding to Intraluminal portal vein sign on USG can be seen on CT. Also shows dilated bile ducts have a random, bizarre pattern and there are focal areas of cystic ectasia. (5)
Radionuclide studies using Tech-99m IDA shows focal defects in the hepatic phase representing dilated bile ducts and gradual increase in activity as the radionuclide concentrates in these ducts.
Cholangiography which was absolutely essential previously to delineate the biliary anatomy and determine the surgical approach, is now rarely indicted with the availability of non-invasive diagnostic methods. Caroli disease can be diagnosed antenatally on USG, by late second or third trimester. The earliest reported cysts are at 25 weeks of gestation. Because these cysts may be initially asymptomatic, prenatal diagnosis facilitates early treatment and prevention of potential complications by regular follow-up.(4) However, some antenatally diagnosed cases show spontaneous regression on subsequent follow-up.
The palliative treatment is mainstay of Caroli disease and needs regular follow-up, as primary bilobar intrahepatic disease cannot be cured surgically. But complications like recurrent cholangitis, hepatic abscesses, biliary calculi and carcinoma needs active surgical management (positioning of an open stent of by percutaneous drainage). However this treatment is not curative and longterm results are not encouraging. If the disease is confined to one lobe, partial lobectomy is the surgical treatment of choice, whereas choledochal cyst is treated by surgical excision and Roux-en-Y anastomosis. Orthotopic liver transplantation appears to be an effective curative option for the treatment of those patients who cannot be operated radically.
References :
  1. Steven Ahrenbt, Herry Apitt, Biliary tract, Sebastian, 17th edition by Elhivir publication : 1998-1597.
  2. Rickes S, Neye H, Wirth J et al. Improved accuracy in diagnosis of intrahepatic bile duct ectasia in Caroli's disease by combination of ultrasound and ECRP. Ultraschall Med. 2000;21(5):223-5.
  3. Husen SZ, Bloom DA, Tolia V. Caroli disease diagnosed in a child by MRCP. Clin imaging 2000;24(5):289-91.
  4. Bratu I, Laberge JM, Kahalife S, et al. Regression of antenatally diagnosed localized caroli disease. J Paediatrics surg. 2000;35(9):1390-3.
  5. Choi, BJ Yeon, CM, Kim SH, HANMC, Caroli disease, central dot sign in CT radiology in 1999, 174:161-63.
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