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Congenital Abnormalities, Pancreatic

Synonyms

Definitions

The pancreas arises from dorsal and ventral anlages that fuse at 6 weeks of gestation. The ventral anlage develops into a part of the pancreatic head and the uncinate process while the dorsal part gives rise to the pancreatic body and tail as well as a second part of the pancreatic head. Congenital variants of the pancreas are seen in approximately 10% of children and include pancreatic agenesis, hypoplasia, ectopia, pancreas divisum, and annular pancreas.

Complete pancreatic agenesis is extremely rare and is usually associated with other major malformations. In agenesis of the dorsal anlage only the pancreatic head develops, e.g., in combination with polysplenia syndrome.

In pancreatic hypoplasia, the exocrine elements or more rarely also the endocrine elements are underdeveloped.

Pancreatic ectopia is defined as pancreatic