Clinical presentation and management of children with diffuse and focal hyperinsulinism: A review of 223 cases

Abstract

Context: Congenital hyperinsulinism (HI) occurs in two distinct histologic forms: diffuse and focal. Distinguishing between them is essential because a pancreatectomy is curative for focal HI and palliative for diffuse HI. Objective: The purpose of this study was to compare the presentations, treatment, and outcomes of diffuse and focal HI. Design: A retrospective chart review of children who underwent pancreatectomy for hyperinsulinism from December 2004 through September 2012 was conducted. Results: Based on pancreatic histology, 223 children were classified into 3 groups: diffuse (n = 97, 44%), focal (n=114, 51%),andother (n=12, 5%). Children with diffuse vs focal HIhadsignificantly different mean gestational ages (38 vs 39 weeks, P < .0005) and birth weights (3963 vs 3717 g P = .012). Children with focal HI presented at an older age (0.3 vs 0 months, P < .0005) and more frequently with seizures (50 vs25%,P