Published online October 1, 2004
PEDIATRICS Vol. 114 No. 4 October 2004, pp. e520-e522 (doi:10.1542/peds.2003-1180-L)
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ELECTRONIC ARTICLE

Laparoscopic Diagnosis and Cure of Hyperinsulinism in Two Cases of Focal Adenomatous Hyperplasia in Infancy

Monique De Vroede, MD, PhD*, N.M.A. Bax, MD, PhD{ddagger}, Klaus Brusgaard, MSc, PhD§, Mark J. Dunne, PhD|| and Floris Groenendaal, MD, PhD

* Departments of Pediatric Endocrinology
{ddagger} Pediatric Surgery
Perinatology, Wilhelmina Children’s Hospital, University Medical Center Utrecht, Utrecht, Netherlands
§ Department of Clinical Genetics, Odense University Hospital, Odense, Denmark
|| School of Biological Sciences, University of Manchester, Manchester, United Kingdom

Persistent hyperinsulinemic hypoglycemia of infancy or congenital hyperinsulinism of the neonate is a rare condition that may cause severe neurologic damage if the disease is unrecognized or inadequately treated. Current treatment aims to restore normal blood glucose levels by providing a carbohydrate-enriched diet and drugs that inhibit insulin secretion. If medical treatment fails, then surgery is required. Because congenital hyperinsulinism may be caused either by diffuse involvement of pancreatic ß-cells or by a focal cluster of abnormal ß-cells, the extent of pancreatectomy varies. We report on 2 patients with a focal form of the disease for whom diagnosis was made with laparoscopy. Laparoscopic enucleation of the lesion was curative.

Key Words: congenital hyperinsulinism • pancreatectomy • laparoscopy • neurodevelopmental outcome

Abbreviations: PHHI, persistent hyperinsulinemic hypoglycemia of infancy • PVS, pancreatic venous sampling • KATP channel, adenosine triphosphate-sensitive potassium channel • SUR, sulfonylurea receptor • Kir, potassium inward rectifier

Accepted May 18, 2004.