Biliary tract intraductal papillary mucinous neoplasm: single-operator cholangioscopy and clearance of mucin obstruction

Biliary tract intraductal papillary mucinous neoplasm (BT-IPMNs) are the counterparts of pancreatic IPMNs, and are characterized as papillary lesions that produce mucin and spread along the biliary mucosa causing obstructive jaundice [1]. These tumors can develop anywhere along the biliary tree, and are considered to be premalignant lesions [2]. We report a case of a BT-IPMN diagnosed by cholangioscopy-guided biopsy, and a novel technique of clearing the biliary tree with a mucolytic solution.

A 49-year-old man presented with obstructive jaundice (serum bilirubin 15 mg/dL) and abdominal pain. Contrast computed tomography showed focal dilatation of bile ducts in segments IV and VIII and dilatation of the common bile duct, with no stones or adenopathy ([Fig. 1 a], [Fig. 1 b]). Endoscopic retrograde cholangiopancreatography (ERCP) detected amorphous filling defects of the common bile duct with poor opacification of the intrahepatic ducts, especially at the right side ([Fig. 2 a], [Fig. 2 b]). As a mucin clot was obstructing bile flow, a 5-minute wash with mucolytic agent (n-acetyl cysteine) through an inflated extractor balloon ([Video 1]) was done to improve clearance. Single-operator cholangioscopy (SpyGlass; Boston Scientific, Natick, Massachusetts, USA) was performed to evaluate the extent and involvement of the tumor growth within the bile duct as well as to provide direct-view biopsies ([Fig. 3]). A protruded, friable 8-mm lesion, located in the right intrahepatic duct, was biopsied and histopathological examination revealed a mucinous papillary neoplasm without dysplasia ([Fig. 4 a], [Fig. 4 b]). The patient recovered without adverse events, his serum bilirubin levels decreased to 2 mg/dL, and a surgical resection was planned.

Fig. 1 a, b Biliary duct dilatation seen at abdominal computed tomography (CT), in a 49-year-old man presenting with obstructive jaundice and abdominal pain.

Fig. 2 a, b Endoscopic retrograde cholangiopancreatography (ERCP) showed amorphous filling defects of the common bile duct and occlusion of the right intrahepatic duct.

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Biliary tract intraductal papillary mucinous neoplasm: clearance of mucin clot and SpyGlass cholangioscopy-guided biopsies.

Fig. 3 Biliary lesion seen at single-operator cholangioscopy.

Fig. 4 a, b Histopathological appearances of a mucinous papillary neoplasm.

BT-IPMN is a rare variant of bile duct tumor, with malignancy varying on several reports to as high as 64 % – 89 % [1] [3] [4]. Clinical presentations include abdominal pain, jaundice, and acute cholangitis. Peroral cholangioscopy can assess the spread of the tumor and allows histological confirmation, providing better information for surgical planning [5]. We undertook a novel approach of mucin removal by injection of a mucolytic agent, thus improving biliary clearance before surgery.

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Competing interests: None

• References

• 1 Ohtsuka M, Shimizu H, Kato A et al. Intraductal papillary neoplasms of the bile duct. Int J Hepatol 2014; Article ID 459091.
• 2 Barton JG, Barrett DA, Maricevich MA et al. Intraductal papillary mucinous neoplasm of the biliary tract: a real disease?. HPB (Oxford) 2009; 11: 684-691
• 3 Wang X, Cai YQ, Chen YH et al. Biliary tract intraductal papillary mucinous neoplasm: report of 19 cases. World J Gastroenterol 2015; 21: 4261-4267
• 4 Rocha FG, Lee H, Katabi N et al. Intraductal papillary neoplasm of the bile duct: a biliary equivalent to intraductal papillary mucinous neoplasm of the pancreas?. Hepatology 2012; 56: 1352-1360
• 5 Moura EG, Franzini T, Moura RN et al. Cholangioscopy in bile duct disease: a case series. Arq Gastroenterol 2014; 51: 250-254