How are choledochal cysts treated?

Updated: Jul 01, 2020
  • Author: Emily Tommolino, MD; Chief Editor: BS Anand, MD  more...
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The treatment of choice for choledochal cysts is complete excision. [28] Patients with type I, II, or IV cysts are recommended for surgical excision due to the risk of malignancy, if they are deemed good surgical candidates. Type III cysts may be managed with endoscopic retrograde cholangiopancreatography (ERCP) with sphincterotomy or endoscopic resection. Primary care physicians who encounter a patient with a choledochal cyst should consult a surgeon.

A retrospective (1996-2015) analysis of 36 adult patients with congenital biliary dilatation suggests that laparoscopic surgery may be effective and provide acceptable outcomes. [29] A different retrospective study (2011-2016) of 20 adults with choledochal cysts (type I, n = 19; type IV, n = 1) who underwent laparoscopic choledochal cyst excision reached similar findings but noted there was one fatality involving bilioenteric anastomosis leak with pseudoaneurysm that led to uncontrolled intraabdominal hemorrhage. [30]

It remains unclear what the optimal therapy is for the rare isolated choledochal cyst involving the proximal cystic duct. [15] Treatment options that have been considered include a bilioenteric reconstruction (because of the wide cystic duct-bile duct junction), as well as cholecystectomy, in combination with examination of the specimen and frozen section (to rule out any abnormalities), with close follow-up owing to the risk of malignancy. [15]

Pregnant women with choledochal cysts are managed on the basis of whether or not cholangitis is present. [31] In those with cysts refractory to medical management, biliary tree decompression is indicated, followed by postpregnancy definitive treatment. [31]

Contributing factors for recurrent biliary obstructions after primary laparoscopic hepaticojejunostomy in children with choledochal cyst appear to include the presence of an aberrant hepatic artery, unresolved hepatic duct strictures, and a poor anastomotic technique. [32] Early surgical correction is important for minimizing liver injury.

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