Long-Term Outcomes in Children with Choledochal Malformations

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: