Introduction:
Choledochal cysts are rare congenital malformations characterized by single or multiple cystic dilatations of the intra- and/or extrahepatic biliary tree. The most widely accepted classification is that of Todani (types I–V).
They are usually diagnosed in childhood (80%) and occur predominantly in women. Their main complications include an increased risk of biliary neoplasms (up to 30% in adults, especially in types I and IV) and recurrent cholangitis, among others.
Treatment generally consists of cyst excision and restoration of biliary-enteric flow, with Roux-en-Y hepaticojejunostomy being the technique of choice. Type III cysts, depending on the subtype, may be treated by sphincterotomy, endoscopic resection (preferred), or surgery.
Sump syndrome, on the other hand, is associated with dysfunction of a surgically created choledochoduodenostomy due to accumulation of debris, biliary sludge, and food remnants in the suprapapillary distal common bile duct, leading to recurrent cholangitis due to bacterial overgrowth. In cases of cholangitis, endoscopic drainage with stent placement is usually required.
Endoscopy:
A 47-year-old woman, who underwent surgery in a private center in 2013 for a choledochal cyst (Todani type IB) treated with choledochoduodenostomy, was admitted for ERCP due to associated sump syndrome.
Cholangiography revealed a large proximal choledochal cyst measuring 30–35 mm and a choledochoduodenal fistula with multiple filling defects. The fistula was cleaned using a Fogarty balloon, yielding abundant biliary sludge and food debris, followed by decompressive sphincterotomy.
Although the guidewire could be advanced through the cyst, the position was extremely unstable, making it impossible to place any type of biliary stent. The procedure was therefore suspended to avoid iatrogenic injury.
The patient was subsequently referred to a tertiary center, where she underwent definitive surgical reintervention.
Conclusions:
The peculiarity of this case lies in the patient’s previous cyst-enterostomy, performed for unknown reasons, which is associated with an increased risk of infection and malignancy. Combined with the technical complexity of ERCP in this setting, surgical management remains the treatment of choice.