Tag Archives: choledochal cyst

Choledochal Cyst and Pancreatitis

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SG Hegde et al. JPGN 2024; 78:685–690. An observational study on the prevalence of choledochal cyst with pancreatitis: Geographical implications and management

Choledochal cyst has been associated with acute pancreatitis. Previous studies have found the prevalence of pancreatitis “reported in recent pediatric choledochal cyst (CDC) cohorts is 43.7% and 35%.” South Asia also also has a high prevalence of chronic pancreatitis of 114–200 cases per 100,0000 population; this is mostly idiopathic. This retrospective study over an 11 year timeframe identified 96 children with choledochal cyst. Key findings:

  • 40.2% of children with CDC had pancreatitis based on elevation of enzymes or radiological imaging
  • 47% of those with radiological features of pancreatitis had imaging features of chronic pancreatitis.

The authors note that the incidence of CDC at 1in 1000 live births in South Asia is significantly higher compared to the incidence of 1 in 100,000–150,000 live births in the western population. Thus, their findings may not be generalizable.

My take: Among kids with acute pancreatitis, a history of choledochal cyst is not uncommon. This report indicates that some are likely to develop chronic pancreatitis.

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Long-Term Outcomes in Children with Choledochal Malformations

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I Hyvarinen et al JPGN 2021; 72: 820-825. Long-term Morbidity of Choledochal Malformations in Children

This single-center retrospective study (n=55 median f/u 6 years) provides data on long-term morbidity of choledochal malformations. Key findings:

  • 21% had long-term complications including cholangitis in 9 (>2 episodes in 5) patients, anastomotic stricture in 2, adhesive volvulus in 1 and hepatocellular carcinoma in 1. 
  • Magnetic resonance cholangiography (MRCP) performed 6.4 (3.6–16) years after hepaticojejunostomy, diameters of both main intrahepatic ducts had decreased significantly to 3.0 (2.5–3.5) mm (P = 0.0001) but a distal cyst stump was remaining in 30% with a length of 6.0 (4.0–20) mm

My take: Despite surgical management (hepaticojejunostomy), biliary tract malignancy is still possible in patients with choledochal cysts. Regular CA 19-9 testing is probably worthwhile, especially in teens and older. The authors note that in patients with type 1 choledochal malformations, some have recommended annual liver biochemistries and ultrasonography following successful surgery (J Gastroenterol Hepatol 2019; 34: 966-974).

Figure from JPGNonline twitter feed: