Reviewed By: Pramod Kerkar, MD, FFARCSI

Caroli Disease: An Overview

Caroli disease is an uncommon acquired disorder described by expansion of the intrahepatic bile channels. Jacques Caroli, a gastroenterologist, initially depicted an uncommon inherent condition in 1958 in Paris, France. He depicted it as "nonobstructive saccular or fusiform multi-focal segmental dilatation of the intrahepatic bile ducts"; essentially, he watched an enormous ectasia in the biliary tree bringing about an incessant, frequently life-debilitating hepatobiliary disease. Caroli disease is also known as Communicating Cavernous Ectasia or Congenital Cystic Dilatation of the intrahepatic biliary tree.

There are two sorts of Caroli diseases, the most well-known being the straightforward or isolated situation where the bile ducts are extended by ectasia. The second more mind boggling reason is generally known as Caroli Syndrome. The syndrome is characterized with portal artery hypertension and presence of fibrous tissues in the liver, also commonly known as congenital hepatic fibrosis. The contrasts between the reasons for the two cases have not yet been found. Caroli illness is additionally connected with failure of the liver and polycystic kidney disease. The disease influences around 1 in 1,000,000 individuals, with more reported instances of Caroli syndrome than of Caroli disease.

Caroli Disease

What Are The Causes Of Caroli’s Disease?

The reason gives off an impression of Caroli disease being hereditary; the simpler type is an autosomal dominant trait while the complex form is an autosomal recessive trait. Females are more vulnerable to Caroli disease than males. Family history may incorporate kidney and liver ailment because of the connection between Caroli Disease and Autosomal Recessive Polycystic Kidney Disease (ARPKD). Mutations have been noted in PKHD1, the gene responsible for ARPKD, in patients with Caroli disease. PKHD1 expresses itself principally in the kidneys with lower levels in the liver, pancreas, and lungs, a steady pattern found in the phenotype of the sickness, which essentially influences the liver and kidneys. The hereditary premise for the contrast between Caroli disease and Caroli syndrome has not been characterized.

During an evaluation of 46 instances of Caroli disease before 1990, it was found that 21.7% of the cases were the after effect of an intraheptic cyst or non-obstructive biliary tree widening, 34.7% were connected with congenital hepatic fibrosis, 13% were secluded choledochal cystic expansion, and the rest of the 24.6% had a blend of all the three reasons.

Family studies are important to figure out whether Caroli ailment is because of inheritable reasons.

What Are The Symptoms of Caroli Disease?

The Principal Indications of Caroli disease Commonly Incorporate:

Incidentally jaundice happens. Caroli disease normally happens in the vicinity of different maladies, for example, autosomal recessive polycystic kidney disease, gallstones, cholangitis, biliary ulcer, liver cirrhosis, septicemia, renal failure, and cholangiocarcinoma (7% are affected). People with Caroli disease are 100 times more at danger for cholangiocarcinoma than the general population. After perceiving side effects of related ailments, Caroli disease can be analyzed.

Epidemiology of Caroli Disease

Caroli ailment is regularly found in Asia and analyzed in persons less than 22 years old. Caroli disease sases have likewise been found in both newborn children and grownups. As restorative imaging innovation enhances, the age of diagnosis of the disease decreases.

How Is Caroli Disease Diagnosed?

Advanced imaging strategies permit the determination to be made all the more effortlessly and without intrusive imaging of the biliary tree. Commonly the sickness is restricted to the left side of the liver. Pictures taken by CT-scan, X-beam, or MRI will show amplified intrahepatic bile channels because of ectasia. Utilizing an ultrasound, tubular enlargement of the bile ducts can be observed. On a CT-Scan, Caroli disease can be seen by taking note of the numerous liquid filled tubular structures stretching out to the liver. A high contrast CT must be utilized to recognize the distinction between stones and augmented channels. Gut gas and digestive propensities make it hard to acquire a reasonable sonogram; hence a CT scan is a decent substitution instead. At the point when the walls of the intrahepatic bile duct have projections, it is unmistakably seen as dots or a simple streak. Caroli disease is normally analyzed after this "central dot sign" is recognized on a CT scan or ultrasound. However, cholangiography is the best and last way to view the expanded bile channels as an aftereffect of Caroli disease.

How is Caroli Disease Treated?

The treatment for Caroli disease relies upon clinical components and the area of the biliary anomaly. At the point when the ailment is limited to one lobe of the liver, hepatectomy calms down the symptoms and seems to evacuate the danger of malignancy. Antibiotics are utilized to treat the swelling and irritation of the bile ducts, and ursodeoxycholic acid is given for hepatolithiasis. Ursodiol is given to treat cholelithiasis. In diffuse instances of Caroli disease, treatment alternatives incorporate conservative or endoscopic treatment, inner biliary bypass techniques and liver transplantation in precisely chosen cases. Surgical resection has been utilized effectively as a part of patients with monolobar disease. An orthotopic liver transplant is another choice, utilized just when antibiotics have no impact, along with repeating cholangitis. With a liver transplant, cholangiocarcinoma can be avoided in the long run.

Prognosis of Caroli Disease

Mortality is indirect and brought about by inconveniences. Morbidity is basic and is brought about by problems of sepsis, cholangitis, choledocholithiasis, and also cholangiocarcinoma. These horrible conditions frequently provoke the analysis. Presence of portal hypertension in patients with Caroli disease might bring about different conditions including hematemesis, splenomegaly and melena. These issues can seriously influence the quality of life of the patient. After cholangitis happens, the Caroli disease patients usually expire within 5 to 10 years.


Although quite rare disease, Caroli disease is a life-threatening one when accompanied by other liver and kidney diseases and disorders. A detailed evaluation and a patient-specific approach towards curing the disease can help the patient fight against Caroli disease.

Pramod Kerkar

Written, Edited or Reviewed By:


Pain Assist Inc.

Last Modified On: December 4, 2018

This article does not provide medical advice. See disclaimer

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