Diagnostic and Surgical Management of Nesidioblastosis in a 42-Year-Old Man with Refractory Hypoglycemia.

Abstract

BACKGROUND Nesidioblastosis is an uncommon cause of hypoglycemia and is part of a group of diseases known as non-insulinoma pancreatogenic hypoglycemia syndrome (NIHPS). The objective of this report is to provide a comprehensive discussion on the diagnostic and therapeutic management of a case of nesidioblastosis, a rare and potentially fatal condition. CASE REPORT A 42-year-old non-diabetic man with a history of coronary artery disease and systemic arterial hypertension underwent extensive diagnostic investigation because he had been hospitalized several times over a period of approximately 2 years with a clinical history compatible with persistent refractory hypoglycemia. Despite laboratory and imaging tests, the underlying cause of the hypoglycemia remained unclear. Comprehensive investigation included computed tomography (CT), magnetic resonance imaging (MRI), upper endoscopy, PET/CT, and selective pancreatic arteriography. Persistent hypoglycemia associated with high insulin levels led to the suspicion of nesidioblastosis. After exhausting all clinical therapeutic options and after multidisciplinary discussion considering risks and benefits, we decided to perform total pancreatectomy and splenectomy. Anatomopathological and immunohistochemical examination confirmed the diagnosis of nesidioblastosis. CONCLUSIONS The diagnosis of nesidioblastosis is complex and requires a multidisciplinary approach. The decision to perform a total pancreatectomy was essential to control severe hypoglycemia and improve the patient's quality of life. This case report describes the diagnostic and therapeutic management of persistent endogenous hyperinsulinemic hypoglycemia and highlights the importance of diagnostic accuracy and early therapeutic intervention.