Caroli Disease: Symptoms, Causes, Treatment, Prognosis, Diagnosis, Epidemiology

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 “non-obstructive fusiform or saccular multi-focal dilatation of 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 referred to as Congenital Cystic Dilatation of Intrahepatic Biliary Tree or Communicating Cavernous Ectasia.

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 disorder while the complex form is believed to be autosomal recessive. Females tend to get Caroli disease more when compared to males. There may also be a family history of kidney and liver ailment because of its association with a condition like Autosomal Recessive Polycystic Kidney Disease. 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 kidneys and liver. The hereditary premise for the contrast between Caroli syndrome and Caroli disease 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 presenting features of Caroli disease include:

In some cases there is also jaundice observed. Caroli disease normally develops in association with some other medical conditions like gallstones, autosomal recessive polycystic kidney disease, cholangitis, liver cirrhosis, biliary ulcer, renal failure, septicemia, and cholangiocarcinoma where around 10% of people get 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 CT, Caroli disease can be seen by taking note of the numerous liquid filled structures in the shape of a tube which stretches out to the liver. This requires testing with a high resolution CT 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 these dots in the bile duct are seen on CT or ultrasound. However it is best to have a cholangiography as this is perhaps the best way to diagnose Caroli Disease since this gives the best view of the expanded bile channels.

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 mode of treatment in patients with mono-lobar disease. Liver transplant is also a mode of treatment but it is only done when antibiotics fail to provide any relief in addition to a repeat 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, M.D., FFARCSI, DA
Pramod Kerkar, M.D., FFARCSI, DA
Written, Edited or Reviewed By: Pramod Kerkar, M.D., FFARCSI, DA Pain Assist Inc. This article does not provide medical advice. See disclaimer
Last Modified On:December 26, 2018

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