Choledochal cysts

Choledochal cysts are rare congenital bile duct abnormalities. .33,34
Infants with choledochal cysts can spontaneously present with the following:33
  • Jaundice and acholic stools in early infancy, which should prompt a workup for biliary atresia
  • A palpable mass in the right upper quadrant of the abdomen, with hepatomegaly
Diagnosis
There are no specific laboratory studies for the diagnosis of choledochal cysts, but some may be useful to narrow the differential diagnosis:33
  • Complete blood count
  • Liver function panel
  • Serum amylase and lipase levels
  • Imaging studies:
  • Abdominal ultrasound35,36
  • Abdominal computed tomography scanning and/or magnetic resonance imaging
  • Magnetic resonance cholangiopancreatography, which may be useful for identifying an abnormal pancreatobiliary junction37 and pancreatobiliary abnormalities38

Percutaneous transhepatic cholangiography and endoscopic retrograde cholangiopancreatography are used in addition to the above non-invasive imaging studies if those tests fail to confirm diagnosis.

Back to diagnostic algorithm

33. Tommolino E, Piper MH, Talavera F, Anand BS, Sawyer MAJ. Choledochal cysts. Medscape; 2019.

34. Choledochal cysts. UCSF Department of Surgery, 2019. (Accessed 01 April, 2020, at https://surgery.ucsf.edu/conditions–procedures/choledochal-cysts.aspx.)

35. Sgro M, Rossetti S, Barozzino T, et al. Caroli’s disease: prenatal diagnosis, postnatal outcome and genetic analysis. Ultrasound Obstet Gynecol 2004;23:73-6.

36. Chen CP, Cheng SJ, Chang TY, Yeh LF, Lin YH, Wang W. Prenatal diagnosis of choledochal cyst using ultrasound and magnetic resonance imaging. Ultrasound Obstet Gynecol 2004;23:93-4.

37. Yu ZL, Zhang LJ, Fu JZ, Li J, Zhang QY, Chen FL. Anomalous pancreaticobiliary junction: image analysis and treatment principles. Hepatobiliary Pancreat Dis Int 2004;3:136-9.

38. Fitoz S, Erden A, Boruban S. Magnetic resonance cholangiopancreatography of biliary system abnormalities in children. Clin Imaging 2007;31:93-101.