Closed duodenal duplication cyst involving the major duodenal papilla versus ampullary choledochocele.

Marius L Calin, MD, Gabriel Arevalo, MD, Katie Harris, MD, Mark Lybik, MD, Maurice E Arregui, MD, FACS. Saint Vincent Hospital

Type 3 choledochal cyst or choledochocele is the rarest form and accounts for 5% of all bile duct cysts. These cysts are located within the duodenal wall and the mucosal histology further differentiates them between choledochocele and duodenal duplication cysts. The challenge in the approach is related to the proximity to the ampulla and the impact on the pancreaticobiliary drainage. To date, there is only one case described of a closed choledococele when the ampulla opens within the cyst cavity and the biliopancreatic drainage in the duodenum is provided via the duct of Santorini. Our patient had a similar anatomy.

This video presents the surgical procedure performed for a 41-year-old women diagnosed with a duodenal cyst that was obstructing the lumen. The cyst was temporarily decompressed by endoscopic ultrasound guided aspiration. The patient later underwent open transduodenal cystectomy with internal marsupialization along with cholecystectomy. Intraoperatively, the major papilla was found to be located within the cavity and appeared to drain into the cyst. No other communication between the cyst and the duodenal lumen was found. It appeared that the drainage was provided via the dorsal accessory duct. The pathology was consistent with duodenal duplication cyst. She had an uncomplicated postoperative course.

The late presentation of the patient in her early 40s and the absence of pancreatitis and biliary obstruction makes this case particularly interesting. This strange anatomical configuration is based upon the major papilla opening into a closed ampullary diverticulum and it would require the presence of a patent Santorini duct in order to drain the biliary and pancreatic secretions into the duodenum. It appears to be the second case of this type presented so far in the literature. The goal of the surgical approach was to resect the cyst and to establish a patent biliopancreatic drainage into the duodenum.

This new interesting anatomical configuration of “ampulary choledococele” should be considered whenever the major papilla opens in a closed cystic cavity.

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