Choledochal Cyst

Robert Acton, MD, Fritz Karrer, MD, Alan P. Ladd, MD, Loren Berman, MD, Amy J Wagner, MD
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Introduction

Choledochal cyst is a rare congenital cystic dilation of the biliary tract with an incidence that ranges from one in 100,000 to 150,000 in Western countries to 1 in 13,000 in East Asian populations. The disease is mostly that of infancy and childhood with 80% being diagnosed in the first ten years of life. However, the disease may be discovered in adulthood. Theses anomalies of the biliary tree have the potential for malignant degeneration into cholangiocarcinoma and gallbladder carcinoma. Choledochal cysts and related disorders are surgical conditions that should be addressed with the goal of complete surgical extirpation and reconstruction. A variety of presenting signs and symptoms may be demonstrated in this population. The spectrum of disorders includes extra- and/or intrahepatic bile duct morphologic changes that dictate the appropriate management based on the level of ductal abnormality.

see also Choledochal Cyst Excision

Content in this topic is referenced in SCORE Choledochal Cyst overview

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Introduction

Choledochal cyst is a rare congenital cystic dilation of the biliary tract with an incidence that ranges from one in 100,000 to 150,000 in Western countries to 1 in 13,000 in East Asian populations. The disease is mostly that of infancy and childhood with 80% being diagnosed in the first ten years of life. However, the disease may be discovered in adulthood. Theses anomalies of the biliary tree have the potential for malignant degeneration into cholangiocarcinoma and gallbladder carcinoma. Choledochal cysts and related disorders are surgical conditions that should be addressed with the goal of complete surgical extirpation and reconstruction. A variety of presenting signs and symptoms may be demonstrated in this population. The spectrum of disorders includes extra- and/or intrahepatic bile duct morphologic changes that dictate the appropriate management based on the level of ductal abnormality.

see also Choledochal Cyst Excision

Content in this topic is referenced in SCORE Choledochal Cyst overview

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Last updated: November 2, 2020