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