Choledochal Cyst / Biliary
Also covered as: choledochal cyst · cholangitis · pancreatitis · cystic biliary atresia · cholangiocarcinoma · Caroli's disease · biliary ascites · anastomotic stricture
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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Fundamentals
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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
Evidence & Research
3 items
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
Journal of pediatric surgery Article Review: April 2023, IPEG issue
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We’re featuring Journal of Pediatric surgery articles to bring you some of the latest news!
This week we are discussing three articles from the April 2023 issue, the IPEG issue with editor Dr. Mark Wulkan and authors Drs. Luzia Toselli,
podcast13:22 · Jul 2023
Quick Literature Updates Episode 15
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We’re back with fifteenth episode of "Quick Literature Updates" the podcast series that delivers the latest updates in pediatric surgery literature in a quick and digestible format. In each episode, we review articles covering the most inte
video4:12 · Jan 2024
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Choledochal cyst resection with biliary reconstruction remains the definitive treatment, with ongoing debate regarding optimal anastomotic technique and surgical approach. Meta-analysis demonstrates no significant difference in cholangitis incidence between hepaticoduodenostomy and hepaticojejunostomy [e2936-c12, e6861-c12], though some advocate Roux-en-Y reconstruction for reduced bile reflux [e2936-c11, e6861-c11]. Hepaticoduodenostomy offers shorter operative time and decreased fat malabsorption without increased biliary leak risk [e2936-c14, e6861-c14]. Laparoscopic resection yields shorter hospital stays, fewer complications, and lower costs compared to open surgery [e6640-c5, e6640-c6, e6640-c7], with laparoscopic cases more commonly receiving hepaticoduodenostomy [e6640-c4, e7927-c9]. Technical innovations include double hemicircumferential running suture (mean operative time 2.95 hours, mean blood loss 9.05 mL, zero anastomotic complications in 218 patients) [e4122-c11, e4122-c12, e4122-c14, e4122-c15, e4122-c16] and robotic-assisted anastomosis [e2936-c6, e6861-c6]. Giant cysts can be managed laparoscopically via intraoperative aspiration after initial trocar placement [e8465-c22, e8465-c24]. Malignant transformation occurs in approximately 8% of patients by ages 40–60 , with higher risk in those operated after age 5 [e8465-c33, e8465-c34].
- Hepaticoduodenostomy and hepaticojejunostomy show equivalent cholangitis rates and overall complication profiles, though reconstruction choice often correlates with surgical approach. [e2936-c12, e2936-c13, e6861-c12, e6861-c13]
- Laparoscopic resection demonstrates shorter length of stay, fewer complications, and lower costs versus open surgery in national database analysis of 577 pediatric cases. [e6640-c5, e6640-c6, e6640-c7]
- Double hemicircumferential running suture technique in 218 laparoscopic cases achieved zero anastomotic stenosis, fistula, or cholangitis with mean operative time under 3 hours. [e4122-c11, e4122-c14, e4122-c15, e4122-c16]
- Giant choledochal cysts are amenable to laparoscopic excision via intraoperative aspiration after initial trocar placement, avoiding need for preoperative drainage which risks cholangitis. [e8465-c21, e8465-c22, e8465-c24]
- Malignant transformation cumulative incidence reaches 8% by ages 40–60, predominantly in patients operated after age 5; close surveillance warranted for late-operated cohorts. [e8465-c27, e8465-c33, e8465-c35]
For patients & families
A choledochal cyst is an abnormal ballooning of the bile duct — the tube that carries digestive fluid from the liver [e2823-c1, e2936-c2]. Doctors often find these cysts when a child has belly pain, and imaging tests like ultrasound or MRI show the widened duct [e2936-c1, e2936-c2]. The standard treatment is surgery to remove the cyst and reconnect the healthy bile ducts to the intestine so bile can flow normally [e2936-c3, e2936-c5]. Surgeons can perform this operation through small incisions (laparoscopic) or with robotic assistance, and most children recover well — typically eating regular food within a few days and going home within a week [e2936-c7, e2936-c8, e2936-c9, e6640-c2]. Studies show that laparoscopic surgery often means shorter hospital stays and fewer complications compared to traditional open surgery [e6640-c5, e6640-c6]. There are different ways to reconnect the bile ducts, and research suggests both main techniques are safe and effective [e2936-c12, e2936-c13]. Children who have the surgery usually do well long-term, with no further belly pain or weight problems at follow-up visits .
A choledochal cyst is an abnormal ballooning of the bile duct — the tube that carries digestive fluid from the liver [e2823-c1, e2936-c2]. Doctors often find these cysts when a child has belly pain, and imaging tests like ultrasound or MRI show the widened duct [e2936-c1, e2936-c2]. The standard treatment is surgery to remove the cyst and reconnect the healthy bile ducts to the intestine so bile can flow normally [e2936-c3, e2936-c5]. Surgeons can perform this operation through small incisions (laparoscopic) or with robotic assistance, and most children recover well — typically eating regular food within a few days and going home within a week [e2936-c7, e2936-c8, e2936-c9, e6640-c2]. Studies show that laparoscopic surgery often means shorter hospital stays and fewer complications compared to traditional open surgery [e6640-c5, e6640-c6]. There are different ways to reconnect the bile ducts, and research suggests both main techniques are safe and effective [e2936-c12, e2936-c13]. Children who have the surgery usually do well long-term, with no further belly pain or weight problems at follow-up visits .
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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.
epidemiologicalEllen Encisco0:13 ↗
The majority of patients underwent open resection.
epidemiologicalEllen Encisco0:24 ↗
Patients who underwent open resection were more likely to have a Roux-en-Y hepaticojejunostomy.
clinicalEllen Encisco0:24 ↗
Patients who underwent laparoscopic resection were more likely to have a hepaticoduodenostomy.
clinicalEllen Encisco0:24 ↗
Patients who underwent open resection were more likely to have a longer length of hospital stay.
clinicalEllen Encisco0:39 ↗
Patients who underwent open resection were more likely to have more complications.
clinicalEllen Encisco0:39 ↗
Patients who underwent open resection had higher total costs.
clinicalEllen Encisco0:39 ↗
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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