Choledochal cyst

Choledochal cyst (also known as bile duct cyst or choledochal malformation) is a congenital condition characterized by cystic dilatation of the bile ducts, which are the tubes that carry bile from the liver and gallbladder to the small intestine. These cysts can occur at various points along the biliary tree and are classified into five types (Todani classification), with Type I (fusiform dilatation of the common bile duct) being the most common. The condition primarily affects the hepatobiliary system but can have secondary effects on the pancreas and gastrointestinal tract. The classic triad of symptoms includes abdominal pain (typically in the right upper quadrant), jaundice (yellowing of the skin and eyes), and a palpable abdominal mass, though this complete triad is present in only a minority of patients. In infants, the condition may present with obstructive jaundice and acholic (pale) stools, while older children and adults more commonly experience recurrent abdominal pain, cholangitis (infection of the bile ducts), or pancreatitis. If left untreated, choledochal cysts carry a significant risk of complications including biliary cirrhosis, portal hypertension, cholangiocarcinoma (bile duct cancer), and recurrent cholangitis. The standard treatment for choledochal cysts is surgical excision of the cyst with reconstruction of the biliary tract, most commonly through a Roux-en-Y hepaticojejunostomy (connecting the remaining bile duct directly to a loop of small intestine). Complete cyst excision is strongly recommended over simple drainage procedures because of the well-documented risk of malignant transformation in retained cyst tissue, which increases with age. The condition is more prevalent in females and in East Asian populations, particularly in Japan where incidence rates are notably higher. Early diagnosis and surgical intervention generally lead to excellent long-term outcomes.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Choledochal cyst