Surgical treatment of congenital hyperinsulinism: Results from 500 pancreatectomies in neonates and children
Author / Expert
N. Scott Adzick, Diva D. De Leon, Lisa J. States, Katherine Lord, Tricia R. Bhatti, Susan A. Becker, Charles A. Stanley
Topic overview
Abstract
Background
Congenital Hyperinsulinism (HI) causes severe hypoglycemia in neonates and children. We reviewed our experience with pancreatectomy for the various types of HI.
Methods
From 1998 to 2018, 500 patients with HI underwent pancreatectomy: 246 for focal HI, 202 for diffuse HI, 37 for atypical HI (16 for Localized Islet Nuclear Enlargement [LINE], 21 for Beckwith-Wiedemann Syndrome), and 15 for insulinoma. Focal HI neonates were treated with partial pancreatectomy. Patients with diffuse HI who failed medical management underwent near-total (98%) pancreatectomy. Atypical HI patients had pancreatectomies tailored to the PET scan and biopsy findings.
Results
The vast majority of pancreatectomies for focal HI were
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