JOP. J Pancreas (Online) 2007; 8(4):450-452. JOP. Journal of the Pancreas - http://www.joplink.net - Vol. 8, No. 4 - July 2007. [ISSN 1590-8577] 450 CASE REPORT Dorsal Pancreatic Agenesis Oya Uygur-Bayramiçli 1 , Resat Dabak 2 , Gamze Kiliçoglu 3 , Can Dolapçioglu 1 , Derya Öztas 1 Departments of 1 Gastroenterology and 2 Family Medicine, Kartal State Hospital; 3 Department of Radiology, Haydarpasa Numune Hospital. Istanbul, Turkey ABSTRACT Context Agenesis of the dorsal pancreas is a rare entity and might present with various symptoms. We report a case which presented with chronic pancreatitis. Case report The patient presented with epigastric pain and we found dorsal pancreatic agenesis causing chronic pancreatitis. Conclusions Dorsal pancreatic agenesis can be easily diagnosed with new techniques and its association with clinical syndromes can be better understood. INTRODUCTION During the complex embryologic development of the pancreas, several morphologic anomalies can develop. One of these anomalies is complete agenesis of the dorsal pancreas which is a rare entity and has been reported in the literature only as case reports. This congenital anomaly is accepted mostly as an anatomic variation but it may cause some symptoms such as non-specific abdominal pain and diabetes mellitus [1]. We present a case of dorsal pancreatic agenesis presenting as chronic pancreatitis. CASE REPORT A 44-year-old male patient presented with epigastric pain of a two week duration which worsened after meals. In these two weeks, the patient had lost 4 kg as a result of deceased food intake due to a fear of pain; he also had loose stools. The patient said that he had had three similar pain episodes in the last two years. The past medical records of the patient revealed that he had had mild hyperamylasemia and leukocytosis during those episodes, but they resolved spontaneously after one to two weeks and the patient had not received any treatment. Abdominal ultrasound was said “to be normal” but the images were not available. On physical examination, everything was normal except for abdominal distension. There was no family history of pancreatitis or diabetes and he himself did not have a history of diabetes. He did not drink alcohol or smoke and he did not use any drugs on a regular basis. The biochemical evaluation of the patient revealed hyperamylasemia (250 IU/L; reference range: 30-115 IU/L), leukocytosis (12,000 mm -3 ; reference range: 4,000-11,000 mm -3 ) and a mild elevation of fasting plasma glucose (135 mg/dL; reference range: 80-115 mg/dL). A Van de Kamer test, transaminase, alkaline phosphatase, gamma glutamyl- transferase, bilirubin, CRP, lipase and CA 19- 9 levels were normal. An abdominal ultrasound was performed which showed a large dense pancreatic head. An upper abdominal MRI and MRCP were carried out with the suspicion of a pancreatic malignancy. T2-weighted axial MRI images demonstrated that the pancreatic head and neck were normal in size and shape; however, the body and tail were inexistent. At MRCP, the major pancreatic duct was short and the dorsal duct