Partial agenesis of pancreas

**Definition:** Partial agenesis of the pancreas is characterised by the congenital absence of a critical mass of pancreatic tissue. The severity of the disease depends on the amount of functional pancreatic tissue present. Pancreatic agenesis is commonly associated with other malformations, in particular pancreaticobiliary duct anomalies, leading to acute or chronic pancreatitis, hyperglycaemia (50% of cases), or, more rarely, polysplenia.