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. 2019 Jan;54(1):27-32.
doi: 10.1016/j.jpedsurg.2018.10.030. Epub 2018 Oct 5.

Surgical treatment of congenital hyperinsulinism: Results from 500 pancreatectomies in neonates and children

N Scott Adzick  1 , Diva D De Leon  2 , Lisa J States  3 , Katherine Lord  2 , Tricia R Bhatti  4 , Susan A Becker  2 , Charles A Stanley  2
Affiliations

Surgical treatment of congenital hyperinsulinism: Results from 500 pancreatectomies in neonates and children

N Scott Adzick et al. J Pediatr Surg. 2019 Jan.

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 < 50%, and many were 2%-10%. 97% of focal HI patients are cured. For diffuse disease patients, 31% were euglycemic, 20% were hyperglycemic, and 49% required treatment for hypoglycemia; the incidence of diabetes increased with long-term follow-up. All 15 insulinoma patients were cured.

Conclusions: Our approach to patients with focal HI can distinguish focal from diffuse HI, localize focal lesions, and permit partial pancreatectomy with cure in almost all focal patients. Surgery does not cure diffuse disease but can help prevent severe hypoglycemia and brain damage. Surgery can be curative for insulinoma and for some cases of atypical HI.

Level of evidence: Level IV.

Keywords: Beckwith–Wiedemann syndrome; Congenital hyperinsulinism; Insulinoma; Localized islet nuclear enlargement; Multiple endocrine neoplasia-type 1 (MEN1); Pancreatectomy.

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Conflict of interest statement

Conflict of interest: The authors have no conflict of interest to declare.

Figures

Figure 1.
Figure 1.
Types of Hyperinsulinism. The percentages of focal vs diffuse HI cases in this series were 55% and 45%, respectively.“Redo” cases had an initial pancreatectomy performed at another institution.
Figure 2.
Figure 2.
Congenital Hyperinsulinism Paradigm 2018
See this image and copyright information in PMC

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