• Lei-Zhou Xia, Xue-Feng Bu, Peng-Cheng Jiang, Feng Yu, Yong-Jun Zhang, and Na-Na Meng.
• Department of General Surgery, Affiliated People's Hospital, Jiangsu University, Zhenjiang, Jiangsu Province, China.
• Medicine (Baltimore). 2021 Dec 10; 100 (49): e28137e28137.

RationaleAgenesis of the dorsal pancreas (ADP) is a rare congenital anomaly of the pancreas. ADP is associated with some other medical problems such as diabetes mellitus, abdominal pain/bloating, pancreatitis, pancreatic neuroendocrine tumor and so on. In this study, we present a case of ADP with chronic suppurative pancreatitis, summarize the clinical characteristics of the reported cases in China and review the correlative literature.Patient ConcernsA 51-year-old Chinese man, with a history of impaired fasting glucose, presented with jaundice, pruritus and dark urine. Laboratory analysis showed abnormal liver function and elevated carbohydrate antigen 19-9.DiagnosesContrast-enhanced computed tomography demonstrated a mass located at the head of pancreas and complete absence of the body and tail of pancreas. Endoscopic retrograde cholangiopancreatography demonstrated an eccentric malignant stricture about 1.6cm of distal common bile duct.InterventionsThe patient underwent pancreaticoduodenectomy because of the suspicion of pancreatic tumor. The postoperative pathological result was chronic suppurative pancreatitis, with moderate hyperplasia in focal ductal epithelium.OutcomesA long-term follow-up shows that the patient is asymptomatic with well-controlled diabetes mellitus and pancreatic exocrine insufficiency.LessonsADP is a quite rare congenital malformation of the pancreas with poorly-understood pathogenesis. The diagnosis of ADP depends on the imaging examination. The therapeutic strategy varies from person to person due to the different accompanying conditions.Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.

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