Pancreatic disorders

Pancreatic disorders can be congenital or acquired and can either affect the exocrine or the endocrine component of the gland. This chapter gives an overview of the main pancreatic conditions affecting children and adolescents that require surgical attention. Structural pancreatic anomalies, such as annular pancreas, pancreas divisum, and pancreaticobiliary malunion, are rare but may cause severe symptoms and may require surgical correction. Congenital hyperinsulinism (CHI) is a complex genetic disorder, characterized by excessive insulin secretion from pancreatic β-cells, which results in severe hypoglycemia. CHI can present as a focal, diffuse or atypical lesion. Near-total pancreatectomy is the procedure of choice for diffuse CHI, whereas a localized resection is curative for focal CHI. Pancreatitis is defined as an inflammatory process secondary to the intraductal activation and secretion of pancreatic enzymes, which results in the digestion of the parenchyma. An increasing incidence of pancreatitis in children has recently been reported. In this chapter, we refer to the recent consensus statement of the INSPPIRE consortium for the diagnosis of childhood-onset acute pancreatitis, acute recurrent pancreatitis, and chronic pancreatitis. Pancreatic tumors are rare in the pediatric population, and their diagnosis is often challenging. Herein, we describe benign and malignant childhood tumors of the exocrine and endocrine pancreas according to the World Health Organization classification.