Persistent hyperinsulinemic hypoglycemia of infancy: Long-term outcome following subtotal pancreatectomy

Background: Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is t he most common cause of persistent hypoglycemia in infants, The current sta ndard treatment is subtotal pancreatectomy (Px). However, the long-term out come following surgery needs further attention. Methods: We analyzed 10 children (7M, 3F) with PHHI who underwent partial ( 65-80%) and subtotal (81-95%) Px. Follow-up ranged from 2 to 9.4 yr (mean = 4.2 yr), We divided them into 2 groups based upon the age at onset of hypo glycemia: early (<1 mo) and late (greater than or equal to 1 mo), Results: The seven patients in the early-onset group underwent 85-95% Px be tween ages of 18 d and 3 mo. Three of them initially treated by 85-90% Px h ad persistent hypoglycemia postoperatively. Two out of three required a 2(n d) operation with 95% Px for controlling hypoglycemia, though both still ha d persistent hypoglycemia and required medication to control blood glucose, The remaining four had 95% Px and had maintained euglycemia postoperativel y. One patient developed diabetes 6 yr after surgery. Six of seven patients had delayed development and subnormal IQ, Three patients of the late-onset group (3 mo, 6 mo and 4 yr) underwent partial Px (80%, 65% and 65%, respec tively) and maintained euglycemia postoperatively. Despite 65% Px, one deve loped diabetes 3 yr after surgery. Conclusions: These results suggest that children with early-onset hypoglyce mia have more severe hyperinsulinism than those with late-onset hypoglycemi a. The former require 95% Px for maintaining euglycemia, but long-term comp lications with diabetes may be common. In contrast, the latter require lowe r percentage Px which may reduce the incidence of diabetes in the future.