Dorsal pancreatic agenesis

Agenesis of the dorsal pancreas is an extremely rare congenital pancreatic anomaly. 

While complete agenesis of the dorsal pancreas is extremely rare, partial agenesis of the dorsal pancreas is thought to be more common than ventral pancreatic agenesis ⁴.

Clinical presentation

While many patients with this anomaly are asymptomatic, some patients may present with abdominal pain ^1-3.

Pathology

It results from an embryological failure of the dorsal pancreatic bud to form the body and tail of the pancreas.

Partial dorsal pancreatic agenesis may be appreciated as a short, rounded pancreatic head adjacent to the duodenum with absence of the pancreatic neck, body, and tail. With complete agenesis of the dorsal pancreas, the neck, body, and tail of the pancreas, the duct of Santorini, and the minor duodenal papilla are all absent. With partial agenesis of the dorsal pancreas, the size of the body of the pancreas varies, there is a remnant of the duct of Santorini, and the minor duodenal papilla is present.

Associations

• hyperglycemia: may be present in a significant proportion of cases (up to 50% ^1,6)
• solid pseudopapillary tumor of the pancreas: possible association ²
• polysplenia ^1,5,7
• pancreatitis ⁶
• intestinal malrotation

Radiographic features

CT

• the pancreatic body and tail are hypoplastic or absent to varying degrees with preservation of the pancreatic head ^7,8
• some reports suggest an enlarged pancreatic head (but only in a proportion of cases with this anomaly) ¹

See also

• pancreatic agenesis
• pancreatic ductal variations