ANOMALOUS PANCREATICOBILIARY JUNCTION WITHOUT CONGENITAL CHOLEDOCHAL CYST

Background Anomalous pancreaticobiliary junction (APBJ) without congen ital choledochal cyst (CCC) carries a high risk of gallbladder carcino ma development. The aim of this study was to obtain information allowi ng early diagnosis and appropriate management. Methods The clinical fe atures, imaging findings and surgical outcome of 18 patients with APBJ without CCC were analysed retrospectively. Results Fourteen patients had symptoms, including those of acute pancreatitis (five patients). I n 16 patients the gallbladder showed abnormalities, including carcinom a (eight) and mucosal hyperplasia (11). Ultrasonography detected gallb ladder 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 patien ts, endoscopic ultrasonography in nine of ten, and magnetic resonance cholangiopancreatography (MRCP) in five of five. Five of eight patient s with gallbladder carcinoma underwent extended cholecystectomy with b ile duct excision. Three patients with cancer and eight with no cancer had cholecystectomy alone. None developed bile duct carcinoma or acut e pancreatitis after operation. All patients without malignancy remain ed asymptomatic for a mean follow-up period of 4.7 years. Conclusion P rophylactic cholecystectomy is recommended for patients with APBJ with out CCC. For early diagnosis of APBJ, gallbladder abnormalities on ult rasonography or acute pancreatitis of unknown aetiology should prompt further investigation with ERCP or less invasive imaging modalities su ch as endoscopic ultrasonography and MRCP.