Persistent hyperinsulinemic hypoglycemia of infancy: long-term outcome following subtotal pancreatectomy
Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is the most common cause of persistent hypoglycemia in infants. The current standard treatment is subtotal pancreatectomy (Px). However, the long-term outcome following surgery needs further attention. We analyzed 10 children (7 M, 3 F) 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 hypoglycemia: early (< 1 mo) and late (> or = 1 mo). The seven patients in the early-onset group underwent 85-95% Px between ages of 18 d and 3 mo. Three of them initially treated by 85-90% Px had persistent hypoglycemia postoperatively. Two out of three required a 2nd operation with 95% Px for controlling hypoglycemia, though both still had persistent hypoglycemia and required medication to control blood glucose. The remaining four had 95% Px and had maintained euglycemia postoperatively. 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%, respectively) and maintained euglycemia postoperatively. Despite 65% Px, one developed diabetes 3 yr after surgery. These results suggest that children with early-onset hypoglycemia have more severe hyperinsulinism than those with late-onset hypoglycemia. The former require 95% Px for maintaining euglycemia, but long-term complications with diabetes may be common. In contrast, the latter require lower percentage Px which may reduce the incidence of diabetes in the future.