Endogenous hyperinsulinemic hypoglycemia syndrome: surgical treatment

The endogenous hyperinsulinemic hypoglicemia syndrome (EHHS) can be caused by an insulinoma, or less frequently, by nesidioblastosis in the pediatric population, also known as non insulinoma pancreatic hypoglycemic syndrome (NIPHS) in adults. The aim of this paper is to show the strategy for the surgical treatment of ehhs.

A total of 19 patients with a final diagnosis of insulinoma or NIPHS who were treated surgically from january 2007 until june 2012 were included. We describe the clinical presentation and preoperative work-up. Emphasis is placed on the surgical technique, complications and long-term follow-up.

All patients had a positive fasting plasma glucose test. Preoperative localization of the lesions was possible in 89.4% of cases. The most frequent surgery was distal pancreatectomy with spleen preservation (9 cases). Three patients with insulinoma presented with synchronous metastases, which were treated with simultaneous surgery. There was no perioperative mortality and morbidity was 52.6%. Histological analysis revealed that 13 patients (68.4%) had benign insulinoma, 3 malignant insulinoma with liver metastases and 3 with a final diagnosis of SHPNI. Median follow-up was 20 months. All patients diagnosed with benign insulinoma or NIPHS had symptom resolution.

The surgical treatment of EHHS achieves excellent long-term results in the control of hypoglucemic symptoms.