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.
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