TY - JOUR
T1 - Diagnosis and management of choledochal cysts
AU - Brown, Zachary J.
AU - Baghdadi, Azarakhsh
AU - Kamel, Ihab
AU - Labiner, Hanna E.
AU - Hewitt, D. Brock
AU - Pawlik, Timothy M.
N1 - Publisher Copyright:
© 2022 International Hepato-Pancreato-Biliary Association Inc.
PY - 2023/1
Y1 - 2023/1
N2 - Background: Choledochal cysts (CCs) are rare cystic dilatations of the intrahepatic and/or extrahepatic bile ducts. We review the pathophysiology, diagnosis, and management of CCs. Methods: MEDLINE/PubMed and Web of Science databases were queried for “choledochal cyst”, “bile duct cyst”, “choledochocele”, and “Caroli disease”. Data were synthesized and systematically reviewed. Results: Classified according to the Todani Classification, CCs are generally believed to arise secondary to reflux of pancreatic enzymes into the biliary tree due to anomalous pancreaticobiliary duct union. Complications of CCs include abdominal pain, jaundice, cystolithiasis, cholecystitis, pancreatitis, liver abscess, liver cirrhosis and malignant transformation (3–7.5%). Radiological and endoscopic imaging is the cornerstone of CC diagnosis and full delineation of cyst anatomy is imperative for proper management. Management is generally guided by cyst classification with complete cyst excision necessary for CCs with high potential of malignant transformation such as types I and IV. 5-year overall survival after choledochal cyst excision is 95.5%. Conclusion: Most CCs should undergo surgical intervention to mitigate the risk of cyst related complications such as cholangitis and malignant transformation.
AB - Background: Choledochal cysts (CCs) are rare cystic dilatations of the intrahepatic and/or extrahepatic bile ducts. We review the pathophysiology, diagnosis, and management of CCs. Methods: MEDLINE/PubMed and Web of Science databases were queried for “choledochal cyst”, “bile duct cyst”, “choledochocele”, and “Caroli disease”. Data were synthesized and systematically reviewed. Results: Classified according to the Todani Classification, CCs are generally believed to arise secondary to reflux of pancreatic enzymes into the biliary tree due to anomalous pancreaticobiliary duct union. Complications of CCs include abdominal pain, jaundice, cystolithiasis, cholecystitis, pancreatitis, liver abscess, liver cirrhosis and malignant transformation (3–7.5%). Radiological and endoscopic imaging is the cornerstone of CC diagnosis and full delineation of cyst anatomy is imperative for proper management. Management is generally guided by cyst classification with complete cyst excision necessary for CCs with high potential of malignant transformation such as types I and IV. 5-year overall survival after choledochal cyst excision is 95.5%. Conclusion: Most CCs should undergo surgical intervention to mitigate the risk of cyst related complications such as cholangitis and malignant transformation.
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U2 - 10.1016/j.hpb.2022.09.010
DO - 10.1016/j.hpb.2022.09.010
M3 - Review article
C2 - 36257874
AN - SCOPUS:85146364159
SN - 1365-182X
VL - 25
SP - 14
EP - 25
JO - HPB
JF - HPB
IS - 1
ER -