Calcifying pancreatitis of a congenital short pancreas: a case report with successful endoscopic papillotomy

We report a case of partial pancreatic agenesis in a 25-yr-old man with insulin-dependent diabetes who exhibited abdominal pains in relation to a calcifying chronic pancreatitis. An endoscopic retrograde pancreatography through the accessory papilla revealed an abnormal duct configuration thought to be consistent with an obstruction of the main duct. Ultrasonography and computerized tomography scanning could not identify any pancreatic tissue in the region of the body or tail. Another endoscopic investigation revealed the main papilla in the third part of the duodenum. The ducts of Santorini and Wirsung were identified. Santorini's duct was dilated and contained calculi. Wirsung's duct was nearly normal. The junction between the two ducts was slightly narrowed. After papillotomy of the accessory papilla, the patient's abdominal pains disappeared. This new case of congenital short pancreas is discussed in relation to four other cases reported in the English and French literature.