Surgical treatment of congenital hyperinsulinism

N Scott Adzick

doi:10.1016/j.sempedsurg.2020.150924

Surgical treatment of congenital hyperinsulinism

Semin Pediatr Surg. 2020 Jun;29(3):150924. doi: 10.1016/j.sempedsurg.2020.150924. Epub 2020 May 17.

Author

N Scott Adzick¹

Affiliation

¹ Department of Surgery and the Congenital Hyperinsulinism Center, Children's Hospital of Philadelphia, and the Perelman School of Medicine at the University of Pennsylvania, 3401 Civic Center Boulevard, Philadelphia, PA 19104, United States. Electronic address: adzick@email.chop.edu.

PMID: 32571515
DOI: 10.1016/j.sempedsurg.2020.150924

Abstract

A multidisciplinary approach to patients with congenital hyperinsulinism (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.

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

MeSH terms

Aftercare / methods
Congenital Hyperinsulinism / diagnosis
Congenital Hyperinsulinism / pathology
Congenital Hyperinsulinism / surgery*
Female
Humans
Infant
Infant, Newborn
Laparoscopy
Male
Pancreatectomy / methods*
Positron Emission Tomography Computed Tomography
Postoperative Care / methods
Treatment Outcome