The risk to have a child who is a carrier like the parents is 50% with each pregnancy. Parents who are close relatives (consanguineous) have a higher chance than unrelated parents to both carry the same abnormal gene, which increases the risk to have children with a recessive genetic disorder.
The chance for a child to receive normal genes from both parents and be genetically normal for that particular trait is 25%. Dominant genetic disorders occur when only a single copy of an abnormal gene is necessary for the appearance of the disease.
Caroli disease is a birth defect distinguished by abnormal prenatal development of the bile duct in the liver. In most cases, the simple or isolated form of Caroli disease is believed to result from a spontaneous genetic change (mutation) that occurs for unknown reasons (sporadic). Caroli’s disease diagnosed by magnetic resonance cholangiopancreatography.
Both forms of Caroli disease are characterized by abnormal widening of the ducts that carry bile from the liver (intrahepatic bile ducts). The simple form, Caroli disease, is apparently much less common than is the more complex form. Both forms are more common in females than among males. The second form of Caroli disease is associated with abnormal formation bands of fibrous tissue in the portal area of the liver (congenital hepatic fibrosis). The portal area of the liver is where the portal vein and the hepatic artery enter the liver.