Choledochal cysts are cystic structures found along the biliary system (the organs and ducts that produce and store the fluid (bile) made by the liver that helps digest fat and release it to the intestine) and may be found either outside the liver or within the liver.
Choledochal cysts are rare with a reported incidence of 1 in 100,00-150,000 births in the western population. It is thought that choledochal cysts begin at an abnormal bile duct junction into the pancreas. Pancreas perform the important function of producing enzymes that are vital to digestion of food we eat. This abnormality of the location of the choledochal cysts allows for mixing of the pancreatic enzymes which causes local destruction and dilation of the bile duct along its normal pathway.
Choledochal cysts are most commonly isolated abnormalities. However, they can be associated with other fetal problems and so a detailed ultrasound of your baby, including a fetal echocardiogram, a test similar to ultrasound that uses sound waves that “echo” off the structures of the fetus's heart, should be performed.
Babies with choledochal cysts should have additional ultrasound examinations throughout the pregnancy to assess change in size of the cyst.
After birth babies may develop a yellow colour to the eyes and skin, pain in upper-right belly, pale or clay-colored stools, nausea, vomiting and fever and feeding problems. These symptoms can be a sign that the baby has a problem. If your baby has any of these symptoms, you should promptly call your pediatrician. If left untreated, choledochal cysts may cause infections of the bile duct and gallbladder. Occasionally, they rupture. Surgical removal is generally required. Surgery is typically performed before the baby is 6 months old.
The good news about choledochal cysts is that knowing about the cyst during pregnancy allows for early monitoring and appropriate follow-up after your baby is born. Earlier diagnosis that leads to surgical repair at early stages results in fewer complications and a better prognosis.
Choledochal cysts are rare. Most parents will not have a second child with a choledochal cyst. There are rare reports of certain types of choledochal cysts that may be genetically linked. Consultation with a Maternal fetal medicine specialist and/or a geneticist may be helpful.
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Last updated September 2019