DEGENERATED TODANI IA CHOLECOCHAL CYST

Introduction:

Choledochal cysts are a very rare pathology and malignant transformation is the most serious complication (1).

Case report:

In a 72-year-old patient with acute cholangitis a Cholangio-NMR (Fig. 1) and abdominal-CT revealed a mass at the choledochal-pancreas-duodenum crossroads, dilation of the bile duct up to 38 mm and pancreas divisum.

Gastroscopy showed an infiltrated duodenal bulb (biopsies: adenocarcinoma), and a extrinsic luminal bulging.

EUS described a cystic dilation of the common bile duct with solid echogenic content inside it. The EUS-guided puncture was non-specific.

ERCP confirmed an adenomatous papilla (Fig. 2) and a great saccular cyst dilation of the extrahepatic bile duct (Todani Ia). Deep cannulation of the proximal bile duct was possible after a fistulotomy. Stood out that polypoid formations extruded through fistulotomy (Fig. 3); the histopathlogy was adenoma with dysplasia. A 10 Fr plastic prosthesis was placed to ensure biliary drainage.

Our patient was diagnosticated of advanced adenocarcinoma of choledochal cyst and a chemotherapic treatment was iniciated.

Comments:

We present this case due to the unusual aggressiveness of tumor infiltration and endoscopic expression.

After fistulotomy in bulging papillae output of lithiasis or intraampullary ampuloma are observed in some cases, but the extrusion of neoplastic formations is exceptional.

Choledochal cysts are associated to an anomalous arrangement of the pancreaticobiliary duct. Pancreas divisum results from a fusion failure of the pancreatic buds. The coexistence of pancreas divisum and choledochal cyst in adults has been reported in less than 10 well documented cases (2).

1. Jiménez-Ballester MA, Soria-Aledo V, Martín-Lorenzo JG et al. Type II intrapancreatic choledochal malignant cyst in adults: Duodenopancreatectom. Rev Esp Enfem Dig. 2014;106:223 – 6.

2. Ransom-Rodríguez A, Blachman-Braun R, Sánchez-García Ramos E et al. A rare case of choledochal cyst with pancreas divisum: case presentation and literature review. Ann Hepato-Biliary-Pancreatic Surg 2017;21:52.