Anomalous pancreaticobiliary junction without congenital choledochal cyst

Anomalous pancreaticobiliary junction (APBJ) without congenital choledochal cyst (CCC) carries a high risk of gallbladder carcinoma development. The aim of this study was to obtain information allowing early diagnosis and appropriate management.

The clinical features, imaging findings and surgical outcome of 18 patients with APBJ without CCC were analysed retrospectively.

Fourteen patients had symptoms, including those of acute pancreatitis (five patients). In 16 patients the gallbladder showed abnormalities, including carcinoma (eight) and mucosal hyperplasia (11). Ultrasonography detected gallbladder carcinoma with 100 per cent sensitivity and mucosal hyperplasia with 91 per cent sensitivity. A long common channel was demonstrated by endoscopic retrograde cholangiopancreatography (ERCP) in all patients, endoscopic ultrasonography in nine of ten, and magnetic resonance cholangiopancreatography (MRCP) in five of five. Five of eight patients with gallbladder carcinoma underwent extended cholecystectomy with bile duct excision. Three patients with cancer and eight with no cancer had cholecystectomy alone. None developed bile duct carcinoma or acute pancreatitis after operation. All patients without malignancy remained asymptomatic for a mean follow-up period of 4.7 years.

Prophylactic cholecystectomy is recommended for patients with APBJ without CCC. For early diagnosis of APBJ, gallbladder abnormalities on ultrasonography or acute pancreatitis of unknown aetiology should prompt further investigation with ERCP or less invasive imaging modalities such as endoscopic ultrasonography and MRCP.