Two cases of agenesis of the dorsal pancreas and a review of the literature

BMC Gastroenterol. 2020 Apr 6;20(1):94. doi: 10.1186/s12876-020-01245-8.

Abstract

Background: Agenesis of the dorsal pancreas (ADP) is a very rare disease with no specific symptoms, and the pathogenesis is not clear. Some patients will be accompanied by other diseases, such as pancreatic tumor or pancreatitis. But most cases are very atypical and difficult to distinguish. Some syndromes of pancreatic exocrine insufficiency are common in patients with ADP. Here, we report two cases of ADP and summarize the clinical features, diagnosis, and treatment of ADP.

Case presentation: Case A is a 65-year-old Chinese woman who presented with abdominal pain accompanied by nausea, bloating and acid reflux. The enhanced abdominal CT scan found nothing meaningful except the absence of the body and tail of the pancreas. The diagnosis was considered as gastrointestinal dysfunction cause by exocrine pancreatic insufficiency and recovered after symptomatic treatment. Case B is a 61-year-old Chinese woman who presented with abdominal pain accompanied by fever, vomiting and bloating. The abdominal CT showed multiple stones in the gallbladder, and the body and tail of the patient's pancreas were absent. She was diagnosed with cholelithiasis and recovered after laparoscopic cholecystectomy.

Conclusion: Agenesis of the dorsal pancreas (ADP) is a rare congenital disease with an unclear pathogenesis that presents multiple symptoms. It should be considered when the patients have non-specific, persistent and unexplained symptoms such as bloating or uncontrolled blood sugar. Imaging examination is helpful for diagnosis. And it does not require surgical intervention unless it accompanies other diseases, EPI need to be considered when the non-specific gastrointestinal symptoms appear.

Keywords: Agenesis of the dorsal pancreas (ADP); Case report; Pancreatic disease; Pancreatic dysplasia.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged
  • Cholecystectomy, Laparoscopic
  • Cholecystitis, Acute / complications
  • Cholecystitis, Acute / diagnosis*
  • Cholecystitis, Acute / surgery
  • Cholelithiasis / complications
  • Cholelithiasis / diagnosis*
  • Cholelithiasis / surgery
  • Congenital Abnormalities / diagnostic imaging*
  • Congenital Abnormalities / physiopathology
  • Diabetes Mellitus, Type 2 / complications
  • Diabetes Mellitus, Type 2 / drug therapy
  • Enzyme Replacement Therapy
  • Exocrine Pancreatic Insufficiency / complications
  • Exocrine Pancreatic Insufficiency / diagnosis*
  • Exocrine Pancreatic Insufficiency / drug therapy
  • Exocrine Pancreatic Insufficiency / physiopathology
  • Female
  • Gastrointestinal Agents / therapeutic use
  • Gastrointestinal Diseases / diagnosis*
  • Gastrointestinal Diseases / drug therapy
  • Gastrointestinal Diseases / etiology
  • Humans
  • Hypoglycemic Agents / therapeutic use
  • Middle Aged
  • Pancreas / abnormalities*
  • Pancreas / diagnostic imaging
  • Pancreas / physiopathology
  • Pancreatin / therapeutic use
  • Tomography, X-Ray Computed

Substances

  • Gastrointestinal Agents
  • Hypoglycemic Agents
  • Pancreatin

Supplementary concepts

  • Pancreas agenesis, dorsal