Abstract
Anomalies of the pancreas organ, especially congenital anomalies, are very uncommon, and can involve the pancreatic ductal as well as the pancreatic extra-ductal systems. While these anomalies usually present as an incidental radiological finding during adulthood, sometimes, they can present as recurrent upper abdominal pain as a presentation of recurrent episodes of acute idiopathic pancreatitis. Thus, these anomalies should be regarded in the differential diagnosis list, as a cause, for recurrent idiopathic pancreatitis, especially in the adult age group of patients. Multiple different variations, mainly in duct course and configuration of the pancreatic ductal system have been reported. In addition, duplication anomalies and cystic dilatation of the pancreatic duct are well known anomalies as well. A combined anomaly of the aforementioned anomalies is even rarer. Herein, we present the case of a male patient, 47 years old, with recurrent admissions to different hospitals due to upper abdominal pain and episodes of severe idiopathic acute pancreatitis. Imaging tests, mainly abdomino-pelvic computed tomography (CT) scan and magnetic resonance imaging (MRI)/magnetic resonance cholangiopancreatography (MRCP) showed a dominant duct of Santorini without divisum along with cystic dilation of the proximal portion of the Santorini duct. Being a very rare pancreatic duct anomaly, the patient was treated by a multidisciplinary team (MDT) of physicians, including pancreas surgeons, gastroenterologists and radiologists. A surgical resection in the form of total pancreatectomy with Roux-en-Y gastrointestinal reconstruction was contemplated. Perioperative and postoperative periods were uneventful. The previously mentioned anomaly is unknown in the English literature and is introduced as new anomaly known as "Kluri".