Surgery in congenital hyperinsulinism-tips and tricks not only for surgeons. A practical guide.

Each form of congenital hyperinsulinism (CHI)-focal, diffuse, atypical-requires its own surgical strategy and technique. Focal CHI is treated by a positron emission tomography/computed tomography-guided, local resection which is confined only to the lesion. As much healthy pancreatic tissue as possible is preserved. On the contrary, the therapeutic mainstay of diffuse CHI must be conservative nowadays. Only in the exceptional cases in which medical treatment fails surgical therapy is warranted to prevent hypoglycemia. However, the extension of resection that is able to cure hyperinsulinism while avoiding diabetes is not known today. The outcome, therefore, is unpredictable. In the rare atypical cases it is important to stop the resection at the right time in order not to finish unnecessarily with a mutilating operation.