Caroli Disease: Liver Abnormalities

Considered the most common cause of childhood liver transplants, Caroli Disease is an inherited complication of the liver bile ducts failing to develop or developed abnormally most often as a dilation, or widening, of the ducts. Considered a disorder of the digestive tract, Caroli Disease can lead to significant health complications as the bile, created in the liver, is unable to pass, correctly, from the liver to the gall bladder and then into the the intestines. The following is an overview of the symptoms of Caroli Disease, methods for diagnosing and treatment options.

Individuals suffering from Caroli Disease will commonly suffer complications associated with the liver disorders. Such complications might include yellowing of the skin and eyes, nausea and vomiting, nose bleeds, fatigue, lack of energy and even decrease in appetite. For many of these individuals, without proper diagnosis, the condition may be dismissed as jaundice, a temporary abnormality of the liver.

Diagnosing Caroli Disease can be achieved through a non-invasive study called Magnetic Resonance Cholangiopancreatography (MRCP). During the MRCP procedure, an MRI study is completed with focus on the pancreas and biliary tracts and emphasis on the liver ducts leading to and away from the organs. It is during this non-invasive procedure that diagnosing of Caroli Disease can be confirmed. For many patients, Caroli Disease is not diagnosed until later into life, commonly in the early 20's, at which point the condition is progressed and requires surgical intervention. Prior to reaching age 20, many individuals, suffering from Caroli Disease, are unsure as to what the origin of the symptoms may be. Upon completion of an MRCP, Caroli Disease is confirmed with 99 percent certainty in the results.

Treating Caroli Disease most often results in liver transplant. Because the development of cholangitis most often leads to death within five to 10 years, many patients will undergo a liver transplant rather quickly. Cholangitis can be acute or chronic. For individuals suffering from Caroli Disease, cholangitis is generally a chronic bacterial accumulation within the biliary ducts which most often results in immune suppressive function and leads to death in up to 40 percent of all cases.

When suffering with any liver associated disorder, understanding the origin of the disorder and the methods for preventing symptoms from being exacerbated is a primary focus of the sufferer. In cases of Caroli Disease, when confirmed through MRCP, the only assured method for recovering from the condition lies in a liver transplant. For more information regarding Caroli Disease and the associated implications on healthcare, visit www.madisonsfoundation.org.