Dorsal Pancreatic Agenesis and Associated Clinical Implications

Dorsal pancreatic agenesis is a rare congenital anomaly characterised by the absence of the pancreatic body and tail due to incomplete development of the dorsal pancreatic bud. Clinically, it spans a spectrum from asymptomatic presentation to endocrine and exocrine dysfunction, including diabetes mellitus, malabsorption and recurrent abdominal pain. Patients may also develop pancreatitis or pancreatic neuroendocrine tumours, reflecting the altered anatomy and compensatory changes in the remaining pancreatic tissue. Diagnosis hinges on cross-sectional imaging and endoscopic techniques that delineate ductal anatomy and exclude mimics such as pancreas divisum. Management remains largely supportive, with enzyme replacement, glycaemic control and pain management forming the mainstays; surgical intervention is reserved for complications such as obstructive lesions or neoplasia. Increasing recognition of this entity underscores its importance in the differential diagnosis of unexplained gastrointestinal and metabolic disorders and prompts investigation into genetic and developmental mechanisms.

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Dorsal Pancreatic Agenesis and Associated Clinical Implications publication trend

The graph below shows the total number of articles in dorsal pancreatic agenesis and associated clinical implications across all publications each year (not limited to Nature Index journals).

Technical terms

Dorsal pancreatic agenesis: congenital absence of the pancreatic body and tail resulting from failure of the dorsal pancreatic bud to develop.

Exocrine pancreatic insufficiency: inadequate secretion of digestive enzymes leading to fat malabsorption, steatorrhea and nutritional deficiencies.

Magnetic resonance cholangiopancreatography (MRCP): non-invasive MRI technique for detailed visualisation of the pancreatic and biliary ductal systems.

Endoscopic ultrasound (EUS): imaging modality combining endoscopy with high-frequency ultrasound to provide detailed assessment and facilitate fine-needle aspiration of pancreatic lesions.

Pancreatic neuroendocrine tumour: neoplasm arising from hormone-secreting islet cells, which may develop in the residual pancreatic head in dorsal agenesis.

References

1. Two cases of agenesis of the dorsal pancreas and a review of the literature. BMC Gastroenterology (2020).
2. Agenesis of the dorsal pancreas with chronic suppurative pancreatitis. Medicine (2021).
3. Agenesis of dorsal pancreas associated with pancreatic neuroendocrine tumor: a case report and review of the literature. Journal of Medical Case Reports (2018).
4. Agenesis of the Dorsal Pancreas: A Rare Cause of Diabetes and Recurrent Upper Abdominal Pain. Cureus (2023).