Agenesis of dorsal pancreas

Case contributed by Dr Ahmed Samir Mehany

Presentation

30 years old female patient present with right loin pain. No previous medical history. No history of trauma. Normal lab investigation

Patient Data

Age: 30 years
Gender: Female
CT

Non-contrast CT KUB performed to look for a urinary stone.

Incidentally found small pancreatic head with non-visualisation of the pancreatic neck, body and tail.

Tiny calcified focus is seen at the pancreatic head, yet clear peripancreatic fat.

Case Discussion

Complete pancreatic agenesis is extremely rare and incompatible with life. Dorsal or ventral pancreatic agenesis is also rare with the dorsal agenesis being more common than the ventral agenesis. Complete dorsal pancreatic agenesis is a very rare entity. 

It is usually asymptomatic and discovered incidentally. If symptomatic, common presentations includes diabetes and recurrent pancreatitis.

Pathology:

Pancreatic development begins in the 4th gestational week with 2 separate buds (ventral and dorsal) arising from caudal embryonic foregut.

The embryologic dorsal bud forms the pancreatic body, tail, superior head, Santorini duct, and minor papilla. Agenesis of the dorsal pancreas due to a defect of the dorsal bud.

It is associated with polysplenia/heterotaxy syndrome and bowel malrotation. The exact aetiology unknown, but may be due to a fetal ischemic event or gene mutation (HNF1B).

Differential diagnosis:

  • pancreatic lipomatosis
  • chronic pancreatitis
  • post traumatic pancreatic atrophy
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Case information

rID: 53129
Case created: 2nd May 2017
Last edited: 7th May 2017
Inclusion in quiz mode: Excluded

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