What is the role of surgery in the treatment of choledochal cysts?

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 of the cyst with construction of a biliary-enteric anastomosis to restore continuity with the gastrointestinal tract. [5, 6, 28, 33] According to Jordan and associates, both partial resection of the cyst and internal drainage procedures expose patients to increased risks of cholangitis, pancreatitis, and cholangiocarcinoma. [34, 35]

The positive results of proper surgical treatment were reinforced by Visser and colleagues. [36] These investigators reported a series of 39 adult patients with choledochal cysts. Cholangiocarcinomas or gallbladder cancers were noted in 8 patients (21%) at the initial operation performed by the authors. Seven of these patients had previously undergone a partial cyst excision, drainage procedure, or expectant management. No cancer was noted during the follow-up care of the patients who underwent complete cyst excision.

Shimotakahara and coworkers compared Roux-en-Y hepaticojejunostomy to hepaticoduodenostomy for biliary reconstruction following choledochal cyst excision and concluded that hepaticojejunostomy was a better choice because of an unacceptably high rate of duodenogastric bile reflux (33.3%) in the hepaticoduodenostomy group. [37] See a review of 79 cases by Mukhopadhyay et al. [38]

In a report of three cases of laparoscopic choledochal cyst excision and Roux-en-Y reconstruction in children, one was converted to open operation owing to the involvement of the confluence of the lobar hepatic ducts. [23] All three children did well postoperatively. In a more recent study of 110 patients (55 children, 55 adults) who underwent laparoscopic cyst excision and biliary-enteric reconstruction for type I choledochal cysts and IVA cysts (intrahepatic and extrahepatic fusiform cysts), investigators found an overall complication rate of 10% (including 3 of 6 patients who developed cholangitis that required intervention for anastomotic stricture), with a 2% reexploration rate; there was 1 postoperative death. [28] Children had a significantly lower rate of blood transfusions and shorter operative time; 3 adults required conversion to open laparotomy.

Laparoscopic surgical management of choledochal cysts in a series of 12 adult patients (mean age, 37.3 y) was successful in all patients via complete cyst excision and reconstruction via Roux-en-Y hepaticojejunostomy. [39] No mortalities and no anastomotic complications occurred. The mean operative time was 228 minutes. Patients were discharged from the hospital after an average stay of 5.8 days.

Robotically-assisted laparoscopic resection of choledochal cysts is a relatively recent technique. This technique was used successfully in the management of a 5-year-old child with a type I choledochal cyst. [40] The total robotic operative time was 390 minutes, and the time for the entire procedure was 440 minutes. No complications occurred. The patient was reported well after 6 months of follow-up.

Choledochal cyst excision in 198 children early in the neonatal period has been reported to have a lower complication rate and less hepatic fibrosis, particularly in neonates who underwent excision of a choledochal cyst within the first 30 days of life. [41]

No mortalities occurred in a study of 32 patients, 84% of whom underwent an initial operation with complete cyst excision and Roux-en-Y hepaticojejunostomy and the remaining 16% had revisional surgery for incompletely resected cysts with hepaticojejunostomy. [42] The overall morbidity rate was 44%, with wound infection being the most common (19%). Malignancy was found in only one specimen (3%). The authors emphasized the importance of treating cyst-associated complications, such as pancreatitis and sepsis, before attempting to define cyst anatomy with ERCP or MRCP. This aids in delineating the extent of involvement of the biliary tree and the exact type of choledochal cyst. Furthermore, they reiterated the importance of complete cyst excision and reconstruction with Roux-en-Y hepaticojejunostomy. [42]

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