Choledochal Cysts: In Brief with Dr. Alexander Bondoc
With Dr. Alexander Bondoc · StayCurrentMD
Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
In the Western world the incidence of choledochal cysts is about one in 100,000.
Choledochal cysts have a female predominance.
In Asia, specifically Eastern Asia, choledochal cyst incidence is one in 13,000.
For Todani types 1 and 4, the etiology is likely embryologic relating to the pancreaticobiliary duct junction (pancreaticobiliary malunion).
Pancreaticobiliary malunion is just one theory for the etiology of choledochal cysts and doesn't explain the higher incidence in the Far East.
In adults, the common channel (confluence of biliary and pancreatic ducts) is supposed to be less than 0.9 centimeters, but in pediatric patients any common channel is abnormal.
Reflux of pancreatic enzymes into the biliary tree causes inflammation, degeneration, and epithelial changes.
Type 5 choledochal cyst (Caroli's disease) has an associated genetic mutation in PKHD1.
Type 1 choledochal cyst is a dilation of the extrahepatic common bile duct only, either fusiform or saccular.
Type 2 choledochal cyst is a small diverticulum off the common bile duct.
Type 3 choledochal cyst (choledochocele) is a dilation of the common bile duct in the wall of the duodenum.
Type 4a choledochal cyst consists of multiple cysts in both the intra- and extrahepatic biliary tree.
Type 4b choledochal cyst consists of multiple cysts in the extrahepatic biliary tree only.
Type 5 choledochal cyst (Caroli's disease) has diffuse cystic dilation that can be throughout the liver or limited to one lobe.
In younger children and infants, choledochal cysts are often diagnosed incidentally on axial imaging or ultrasound.
In children, choledochal cysts often present with symptoms consistent with cholangitis such as jaundice or fever, and patients can have a palpable right upper quadrant mass.
Giant choledochal cysts that children are born with can perforate and present with biliary ascites.
Large choledochal cysts can be identified on 20-week fetal ultrasound and should be referred to a fetal center for workup and close monitoring after birth.
Up to 15 to 20 percent of choledochal cyst patients have some kind of variant biliary tract anatomy.
In neonates or newborns with suspected choledochal cyst, liver biopsy is critical to rule out the cystic biliary atresia variant.
While ultrasound and labs are sufficient to diagnose a choledochal cyst, axial imaging such as CT scan is needed before taking patients to the operating room.
MRCP is useful for operative planning when there are questions about anatomic details, specifically for high disease (into the hilum or intrahepatic) to assess the extent of cystic change.
Dr. Bondoc constructs Roux-en-Y hepaticojejunostomies by going 15 to 20 centimeters distal from the ligament of Treitz, performing a jejunojejunostomy in two layers if child size allows, then doing a hepaticojejunostomy with interrupted 5-0 or 6-0 Maxon suture.
Dr. Bondoc does not usually biopsy the liver unless there is something obvious, but does close the mesenteric defect.
Type 2 choledochal cysts require resection of the diverticulum and primary repair of the common bile duct.
For type 3 choledochal cysts, ERCP and sphincterotomy can be therapeutic in some cases.
If a type 3 choledochal cyst is large and obstructing the common duct, a transduodenal approach is often needed to resect the cyst and reconstruct the outflow, sometimes with preoperative stents placed via ERCP.
Type 5 choledochal cysts may require liver transplant if cystic disease is diffuse throughout the liver.
If type 5 cystic burden is limited to one hemi-liver or allows for resection with adequate liver remnant and preserved biliary outflow, it can be addressed surgically with liver resection.
Postoperatively, choledochal cyst patients have an NG tube for about 24 hours to protect the jejunal anastomosis and have temporary biliary drains until eating a regular diet to catch any leaks from the hepaticojejunostomy.
Postoperative complications for choledochal cyst surgery include recurrent cholangitis due to the connection between the biliary tree and enteric system, anastomotic stricture, small bowel obstruction, and reflux gastritis.
Prophylactically, choledochal cyst patients are put on Bactrim postoperatively; Actigall can also be helpful but may be institution-specific.
Lifelong follow-up is critical for choledochal cyst patients because of the possibility of developing future malignancy even after resection of the cyst.
Choledochal cysts are a congenital dilation of the biliary tree.
A recent study showed that if a choledochal cyst was diagnosed prenatally and was larger than 4.5 centimeters at the 20-week anatomy scan, there was a higher rate of postnatal symptoms, suggesting these patients may benefit from earlier intervention.
Surgery is the only true treatment option for choledochal cysts.
The risk of developing cholangiocarcinoma in choledochal cyst patients can be as high as 26 percent according to some studies.
Even after surgical resection of choledochal cysts, some studies suggest there is still about a 4 percent lifetime risk of malignancy, requiring lifelong surveillance.
Patients presenting acutely with choledochal cysts can have pancreatitis or cholangitis, and infection/inflammation can make for a hostile surgical field.
If a choledochal cyst is found incidentally, resection is not an emergency but it is better to schedule surgery sooner rather than later.
For type 1 and type 4 choledochal cysts, the goal is to remove as much of the duct as possible, taking it behind or into the head of the pancreas down to where it tapers, to avoid leaving remnant cysts.
Preoperative MRCP is usually sufficient to identify where the distal cyst tapers behind the pancreas, but intraoperative cholangiogram through the gallbladder is an option if imaging is unclear.
Dr. Bondoc's preference for biliary reconstruction is a Roux-en-Y hepaticojejunostomy, though hepaticoduodenostomy is also an option.