Ectopic pancreas and the islet cell dysmaturational syndrome

Two cases of ectopic pancreas are described in the setting of the islet cell dysmaturational syndrome. Microscopic and immunostaining studies revealed both lesions to be composed of primarily (80 percent to 90 percent) islet tissue with nuclear hyperchromasia and probable depletion of immunoreactive insulin in one case. Persistent hyperinsulinemic hypoglycemia required a second laparotomy with resection of ectopic pancreas in one case. Awareness of the phenomenon led to successful identification and resection of ectopic islet tissue on first surgery in a subsequent case. Ectopic pancreas is a relatively common developmental pancreatic anomaly, and knowledge of its potential contribution to life-threatening hypoglycemia may obviate the need for multiple surgeries in some cases of islet cell dysmaturational syndrome.