Choledochal malformations (CMs) are uncommon congenital dilatations of the biliary tree—extrahepatic and/or intrahepatic, with no actual anatomical distal ductal obstruction. Named as choledochal cysts, the newer emerging term is choledochal malformations—due to the varied anatomical forms, anomaly not being an actual closed epithelialized sac, and varied theories of pathogenesis. CMs have been either antenatally diagnosed, presented with a spectrum of symptoms at various age groups, or detected incidentally in older age groups. Surgical resection of CMs is warranted once a diagnosis has been made due to the complications involved with untreated CMs—cholestasis, cholangitis, cholelithiasis, pancreatitis, progressive liver injury, perforation, and hepatobiliary malignancies. However, the operative decisions—technique and timing—need to be individualized based on the patient’s comorbidities and CM type. A long-term follow-up of these patients is needed, along with transition of care to our adult surgeon/physician colleagues due to the risks pertaining to postoperative recurrent cholangitis, segmental bile duct stenosis, intrahepatic stone formation, anastomotic stricture, and malignancy.

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Choledochal Malformations

  • Satya Sree Balija,
  • Enono Yhoshu,
  • Subhasis Roy Choudhury

摘要

Choledochal malformations (CMs) are uncommon congenital dilatations of the biliary tree—extrahepatic and/or intrahepatic, with no actual anatomical distal ductal obstruction. Named as choledochal cysts, the newer emerging term is choledochal malformations—due to the varied anatomical forms, anomaly not being an actual closed epithelialized sac, and varied theories of pathogenesis. CMs have been either antenatally diagnosed, presented with a spectrum of symptoms at various age groups, or detected incidentally in older age groups. Surgical resection of CMs is warranted once a diagnosis has been made due to the complications involved with untreated CMs—cholestasis, cholangitis, cholelithiasis, pancreatitis, progressive liver injury, perforation, and hepatobiliary malignancies. However, the operative decisions—technique and timing—need to be individualized based on the patient’s comorbidities and CM type. A long-term follow-up of these patients is needed, along with transition of care to our adult surgeon/physician colleagues due to the risks pertaining to postoperative recurrent cholangitis, segmental bile duct stenosis, intrahepatic stone formation, anastomotic stricture, and malignancy.