Persistent hyperinsulinemic hypoglycemia of infancy (PHHI): Long-term outcome following 95% pancreatectomy.

OBJECTIVE

To evaluate the outcome of neonates and infants with persistent hyperinsulinemic hypoglycemia of infancy (PHHI) who had undergone 95% pancreatectomy, with special emphasis on development of diabetes mellitus (DM).

METHODS

Ten infants diagnosed according to the established criteria of PHHI, and who had undergone 95% pancreatectomy, were followed for a period ranging from 1-20 years (mean 13(4/12) years). A retrospective analysis of their data was carried out with special emphasis on growth and development, glycemic control, neurological status and school performance. The data on weight, glycemic control and daily insulin requirement were compared with a control group of ten children with type 1 DM (DM1), matched for age and sex.

RESULTS

All ten children ultimately developed DM; three of them immediately following pancreatectomy and the remainder after a variable period ranging from 7(1/4)-11(1/2) years (mean: 8(11/12) years). Data on their weight, HbA1c levels and daily insulin requirements when compared to ten children with DM1 showed no statistically significant difference in these biological variables. All the children, with the exception of one who sustained subarachnoid hemorrhage, cerebral edema and seizures in the neonatal period, were neurologically and developmentally normal. None exhibited clinical manifestations of pancreatic exocrine deficiency.

CONCLUSION

95% pancreatectomy is an effective treatment modality in PHHI not responding to medical treatment. In our experience; recurrence of hypoglycemia following surgery is uncommon and responds well to medical treatment. There is a high risk of secondary DM in these children; however, the serious risk of brain damage and neurological abnormalities could be avoided by early surgical intervention. Children in this study did not show any signs or symptoms of malabsorption.