From
Dr. Alexander Bondoc
Choledochal Cysts: Classification, Diagnosis, and Management in Pediatric Patients
With Dr. Alex Bondoc · hosted by Dr. Em Gootee
Chapter 1 of 7 · Fundamentals
Todani classification
Introduction and Classification Overview
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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
There is a type 6 choledochal cyst which is cystic dilation of the cystic duct itself
In the Western world, choledochal cyst incidence is about 1 in 100,000
In Eastern Asia, choledochal cyst incidence is 1 in 13,000
For Todani types 1 and 4, the etiology is likely embryologic, relating to pancreatic biliary duct junction (pancreatic biliary malunion)
Reflux of pancreatic enzymes from the head of the pancreas back into the biliary tree causes inflammation, degeneration and epithelial changes
Type 1 choledochal cyst is a dilation of the extrahepatic common bile duct only, which can be fusiform or saccular
Type 2 choledochal cyst is a small diverticulum off of the common bile duct
Type 4A choledochal cyst has multiple cysts in both the intra and extrahepatic biliary tree
Type 4B choledochal cyst has 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 for other causes
In children, choledochal cysts often present with symptoms consistent with cholangitis, such as jaundice or fever
Patients with choledochal cysts can have a palpable right upper quadrant mass
Giant choledochal cysts that children are born with can perforate and present with biliary ascites
If a choledochal cyst diagnosed prenatally is larger than 4.5 centimeters at the 20-week anatomy scan, there is a higher rate of postnatal symptomatology and these patients may benefit from earlier intervention
Workup for choledochal cysts includes liver function tests with total, direct and indirect bilirubin levels, plus or minus CBC to look for evidence of cholangitis or infection
In infants with suspected choledochal cysts, liver biopsy is critical to rule out cystic biliary atresia variant
MRCP is useful for operative planning when there are questions about anatomic details, specifically for high disease into the hilum or intrahepatic involvement
Up to 15 to 20% of patients with choledochal cysts have some kind of variant biliary tract anatomy
Some patients with choledochal cysts will benefit from ERCP for diagnostic and therapeutic purposes, depending on prior scans and the type of cyst
Anatomic anomalies of the biliary tract can lead to cholangitis as a significant complication due to biliary stasis and superinfection
Even after surgical resection of choledochal cysts, there is about a 4% lifetime risk of malignancy, requiring lifelong surveillance
If a patient with choledochal cyst presents actively infected with pancreatitis or cholangitis, the best approach is to cool the patient off and treat the underlying process before surgery
For type 1 and type 4 choledochal cysts, the surgical 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
Intraoperative cholangiogram through the gallbladder can be used to identify where the distal cyst taper occurs when imaging is unclear or anatomy is aberrant
For type 1 and type 4 choledochal cysts, biliary reconstruction often includes a Roux-en-Y hepaticojejunostomy, though hepaticoduodenostomy is also an option
Dr. Bondoc constructs Roux-en-Y hepaticojejunostomies by going 15 to 20 centimeters distal from the ligament of Treitz for the Roux limb, performing a jejunojejunostomy in two layers when size allows, and creating a hepaticojejunostomy with interrupted absorbable suture (5-0 or 6-0 Maxon)
Dr. Bondoc closes the mesenteric defect after Roux-en-Y reconstruction but does not usually biopsy the liver unless there is something obvious
If a type 3 choledochal cyst is large and obstructing the common duct, transduodenal resection may be required, sometimes with preoperative stents placed via ERCP to identify anatomy
For type 5 choledochal cysts, if the cystic burden is limited to one hemiliver allowing adequate liver remnant and preserved biliary outflow, it can be addressed with liver resection
If type 5 choledochal cyst disease is diffuse throughout the liver, the patient may need to be evaluated for liver transplantation
Short-term to long-term surgical complications after choledochal cyst surgery can include anastomotic stricture, small bowel obstruction, reflux gastritis, and recurrent cholangitis
Lifelong follow-up is needed for choledochal cyst patients because there is still a real possibility for future malignancy even after resection of the cyst
Choledochal cysts are a congenital dilation of the biliary tree
Choledochal cysts have a female predominance
Pancreatic biliary malunion is just one theory for choledochal cyst etiology and doesn't explain the higher incidence in the Far East
In adults, the common channel (confluence of biliary pancreatic ducts) should be less than 0.9 centimeters
In pediatric patients, any common channel is abnormal
Type 5 choledochal cyst (Caroli's disease) has an associated genetic mutation in PKHD1
Type 3 choledochal cyst (choledochocele) is a dilation of the common bile duct in the wall of the duodenum
Large choledochal cysts can be identified on 20-week fetal ultrasound
Labs plus abdominal ultrasound can be diagnostic for choledochal cysts
CT scan is needed before taking children with choledochal cysts to the operating room
The risk for developing cholangiocarcinoma with untreated choledochal cysts can be as high as 26% according to some studies
Preoperative MRCP is usually enough to identify where the taper behind the pancreas is located
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
Postoperatively, choledochal cyst patients have an NG tube for about 24 hours to protect the new jejunal anastomosis
Postoperatively, choledochal cyst patients have temporary biliary drains until eating a regular diet to catch any leaks from the hepaticojejunostomy
Choledochal cyst patients have additional postoperative risk for recurrent cholangitis due to the connection between the biliary tree and the enteric system
Prophylactically, choledochal cyst patients are put on Bactrim postoperatively
Actigall can be helpful postoperatively for choledochal cyst patients, though this may be institution-specific