Presentation
30 years old female patient present with right loin pain. No previous medical history. No history of trauma. Normal lab investigation
Patient Data
Non-contrast CT KUB performed to look for a urinary stone.
Incidentally found small pancreatic head with non-visualization 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 etiology 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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