Educational content from recorded physician discussions — not medical advice. Always talk to your child's care team about your child's situation.
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Choledochal Cyst Podcast
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In this podcast, Dr. Alex Bondoc and Dr. Rae Hanke discuss the intricacies of choledochal cysts. Contributing editor: Dr. Rod Gerardo.Choledochal Cyst- Definition: Congenital cystic dilation of the biliary tree- Epidemiology: We
podcast22:07 · Dec 2020
Choledochal Cysts: In Brief with Dr. Alexander Bondoc
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Are you having difficulty keeping the 5 types of choledochal cyst straight? Each has unique anatomy and management principles. Join Dr. Alexander Bondoc in an overview of Choledochal Cysts for the surgical trainee.
podcast16:55 · May 2022
Outcomes of laparoscopic versus open resection of pediatric choledochal cyst
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"Outcomes of laparoscopic versus open resection of pediatric choledochal cyst"
Ramsey et al, Journal of Pediatric Surgery
https://www.jpedsurg.org/article/S0022-3468(22)00799-0/fulltext
Video: Ellen Encisco
video0:54 · May 2023
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Choledochal cysts are congenital biliary dilations with incidence of 1:100,000 in the West and 1:13,000 in Eastern Asia, showing 3–4× female predominance. The Todani classification defines five types: type 1 (extrahepatic fusiform/saccular), type 2 (diverticulum), type 3 (choledochocele), type 4a/b (multiple intra- and/or extrahepatic), and type 5 (Caroli's disease, intrahepatic only).[e3310-c10–c15] Types 1 and 4 arise from pancreaticobiliary malunion creating a long common channel; reflux of pancreatic enzymes causes epithelial inflammation and degeneration. Type 5 associates with PKHD1 mutation. Presentation ranges from incidental prenatal/infant findings to cholangitis (jaundice, fever) or perforation with biliary ascites; the classic triad is rare. Ultrasound screens; CT/MRCP define anatomy (15–20% have variants) and hilar extent. Surgery is mandatory: complete cyst excision with Roux-en-Y hepaticojejunostomy for types 1/4, diverticulectomy for type 2, ERCP/transduodenal resection for type 3, and resection or transplant for type 5.[e3310-c42–c48] Malignancy risk is 6–30% lifetime (8% for types 1/4), persisting at ~4% post-resection, necessitating lifelong surveillance.
- Types 1 and 4 choledochal cysts stem from pancreaticobiliary malunion; refluxed pancreatic enzymes drive epithelial inflammation and 6–30% lifetime malignancy risk, including post-resection.
- Complete cyst excision is mandatory; Roux-en-Y hepaticojejunostomy is standard for types 1/4, taking the duct to pancreatic taper to avoid remnant cyst.
- MRCP is essential when hilar or intrahepatic disease is suspected; 15–20% of patients have variant biliary anatomy requiring preoperative mapping.
- Prenatal cysts >4.5 cm at 20 weeks correlate with higher postnatal symptoms; neonates require liver biopsy to exclude cystic biliary atresia.
- Lifelong surveillance is mandatory post-resection due to persistent 4% malignancy risk; postoperative cholangitis prophylaxis (Bactrim) is standard for 3–6 months.
For patients & families
Choledochal cyst is a condition where a child is born with an abnormal widening or ballooning of the tubes that carry bile from the liver . It's rare in Western countries (about 1 in 100,000 children) but more common in Eastern Asia (1 in 13,000), and affects girls three to four times more often than boys [e3310-c2, e3310-c3, e3310-c4]. Doctors classify these cysts into five types based on where and how the bile ducts are affected . Many babies and young children are diagnosed by chance when they have an ultrasound for something else, though some develop symptoms like jaundice (yellowing of the skin), fever, or a lump that can be felt in the belly [e3310-c16, e3310-c17, e3310-c18]. Large cysts can sometimes be seen on prenatal ultrasounds around 20 weeks of pregnancy . Surgery is the only treatment because these cysts carry a risk of serious infection and, importantly, an 8% to 30% lifetime risk of developing cancer in the bile ducts or gallbladder [e3310-c33, e3310-c35, e3310-c36]. Even after the cyst is removed, a small cancer risk remains, so children need lifelong medical follow-up [e3310-c37, e3310-c62].
Choledochal cyst is a condition where a child is born with an abnormal widening or ballooning of the tubes that carry bile from the liver . It's rare in Western countries (about 1 in 100,000 children) but more common in Eastern Asia (1 in 13,000), and affects girls three to four times more often than boys [e3310-c2, e3310-c3, e3310-c4]. Doctors classify these cysts into five types based on where and how the bile ducts are affected . Many babies and young children are diagnosed by chance when they have an ultrasound for something else, though some develop symptoms like jaundice (yellowing of the skin), fever, or a lump that can be felt in the belly [e3310-c16, e3310-c17, e3310-c18]. Large cysts can sometimes be seen on prenatal ultrasounds around 20 weeks of pregnancy . Surgery is the only treatment because these cysts carry a risk of serious infection and, importantly, an 8% to 30% lifetime risk of developing cancer in the bile ducts or gallbladder [e3310-c33, e3310-c35, e3310-c36]. Even after the cyst is removed, a small cancer risk remains, so children need lifelong medical follow-up [e3310-c37, e3310-c62].
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Outcomes of laparoscopic versus open resection of pediatric choledochal cyst
The study used the nationwide readmissions database and identified 577 children who underwent choledochal cyst resection between 2016 and 2018.
Host summaryEllen Encisco summarizing a resource — not the host's own clinical position0:13 ↗
The majority of patients underwent open resection.
Host summaryEllen Encisco summarizing a resource — not the host's own clinical position0:24 ↗
Patients who underwent open resection were more likely to have a Roux-en-Y hepaticojejunostomy.
Host summaryEllen Encisco summarizing a resource — not the host's own clinical position0:24 ↗
Patients who underwent laparoscopic resection were more likely to have a hepaticoduodenostomy.
Host summaryEllen Encisco summarizing a resource — not the host's own clinical position0:24 ↗
Patients who underwent open resection were more likely to have a longer length of hospital stay.
Host summaryEllen Encisco summarizing a resource — not the host's own clinical position0:39 ↗
Patients who underwent open resection were more likely to have more complications.
Host summaryEllen Encisco summarizing a resource — not the host's own clinical position0:39 ↗
Patients who underwent open resection had higher total costs.
Host summaryEllen Encisco summarizing a resource — not the host's own clinical position0:39 ↗
Choledochal Cyst Podcast
Choledochal cyst is a congenital cystic dilation of the biliary tree.
clinicalAlex Bondoc0:29 ↗
Incidence of choledochal cysts in the Western world is about 1 in 100,000.
epidemiologicalAlex Bondoc0:36 ↗
In Eastern Asia, choledochal cyst incidence is 1 in 13,000.
epidemiologicalAlex Bondoc0:36 ↗
Choledochal cysts are three to four times more likely in females than in males.
epidemiologicalAlex Bondoc0:36 ↗
There is no genetic predisposition to choledochal cysts, but some genes including PKD1 have been identified in limited studies for type 5 choledochal cysts.
clinicalAlex Bondoc0:36 ↗
The most common classification system for choledochal cysts was developed by a surgeon named Tadani in Japan and includes five types.
clinicalAlex Bondoc1:10 ↗
For types 1 and 4 choledochal cysts, the etiology is likely embryologic, relating to pancreaticobiliary duct junction (pancreaticobiliary malunion), which creates a long common channel of the pancreaticobiliary duct.
clinicalAlex Bondoc1:10 ↗
Reflux of pancreatic enzymes from the head of the pancreas back into the biliary tree causes inflammation, degeneration, and epithelial changes in types 1 and 4 choledochal cysts.
clinicalAlex Bondoc1:10 ↗
In type 5 choledochal cysts, genetic mutation might predispose to more diffuse dilation of both the intra- and extrahepatic biliary trees.
clinicalAlex Bondoc1:10 ↗
Type 1 choledochal cyst is a dilation of the extrahepatic common bile duct only, which can be fusiform or saccular.
clinicalAlex Bondoc2:05 ↗
Type 2 choledochal cyst is a small diverticulum off of the common bile duct.
clinicalAlex Bondoc2:05 ↗
Type 3 choledochal cyst is a choledochocele affecting the portion of the common bile duct in the wall of the duodenum.
clinicalAlex Bondoc2:05 ↗
Type 4a choledochal cyst consists of multiple cysts in both the intra- and extrahepatic biliary tree.
clinicalAlex Bondoc2:05 ↗
Type 4b choledochal cyst consists of multiple cysts in the extrahepatic biliary tree only.
clinicalAlex Bondoc2:05 ↗
Type 5 choledochal cyst is intrahepatic only and diffusely throughout, called Caroli's disease, which can be diffuse or limited to one lobe or one hemi-liver.
clinicalAlex Bondoc2:05 ↗
In younger children such as infants, choledochal cysts are often diagnosed incidentally on axial imaging or ultrasound for other causes.
clinicalAlex Bondoc3:25 ↗
In children, choledochal cysts often present with symptoms consistent with cholangitis, such as jaundice or fever.
clinicalAlex Bondoc3:25 ↗
Patients with choledochal cysts can have a palpable right upper quadrant mass.
clinicalAlex Bondoc3:25 ↗
The triad of jaundice, fever, and palpable right upper quadrant mass (Charcot's triad) is incredibly uncommon as a presentation of choledochal cysts.
clinicalAlex Bondoc3:25 ↗
Giant choledochal cysts in small children can perforate and present with biliary ascites.
clinicalAlex Bondoc3:25 ↗
Giant choledochal cysts can be diagnosed prenatally on week 20 ultrasounds.
clinicalAlex Bondoc3:25 ↗
Prenatally diagnosed choledochal cysts require fetal consultation at a fetal surgery center for establishment of care and counseling, because cystic biliary atresia must be ruled out postnatally.
guidelineAlex Bondoc3:25 ↗
A recent citation demonstrated 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 symptomatology, suggesting these patients may benefit from earlier intervention.
clinicalAlex Bondoc3:25 ↗
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