Congenital hyperinsulinism

Abstract

Hyperinsulinism is the most common cause of hypoglycemia in infants. In many cases conservative treatment is not effective and surgical intervention is required. Differentiation between diffuse and focal forms and localization of focal lesions are the most important issues in preoperative management. We present a case of persistent infancy hyperinsulinism. Clinical presentation, conservative treatment modalities, diagnostic possibilities of focal and diffuse forms, and surgical treatment, which led to total recovery, are discussed.

Hiperinsulinismo congénito del recién nacido: A propósito de un caso clínico

“…This could be identified with ultrasonography, endosonography, computed tomography or abdominal MRI 24,25 . However, these procedures fail to define whether the HC lesion is focal or diffuse, which is crucial to decide the treatment, plan the extension of pancreatic resection and evaluate the prognosis [25][26][27][28] . Recent studies emphasize the contribution of Fluorine 18-L-3,4 Dihydroxyphenylalanine Positron Emission Tomography (18F-DOPA-PET/CT scan) which reports a 96% accuracy in differentiating diffuse or focal disease, making precise the localization of a focal lesion in up to 100% of cases 3,25,27,28 .…”

“…However, these procedures fail to define whether the HC lesion is focal or diffuse, which is crucial to decide the treatment, plan the extension of pancreatic resection and evaluate the prognosis [25][26][27][28] . Recent studies emphasize the contribution of Fluorine 18-L-3,4 Dihydroxyphenylalanine Positron Emission Tomography (18F-DOPA-PET/CT scan) which reports a 96% accuracy in differentiating diffuse or focal disease, making precise the localization of a focal lesion in up to 100% of cases 3,25,27,28 . It should be done when it is verified that the HC is persistent (usually after 1 month of life) and could be overlooked if the genetic study demonstrates diffuse disease 28 .…”

“…Recent studies emphasize the contribution of Fluorine 18-L-3,4 Dihydroxyphenylalanine Positron Emission Tomography (18F-DOPA-PET/CT scan) which reports a 96% accuracy in differentiating diffuse or focal disease, making precise the localization of a focal lesion in up to 100% of cases 3,25,27,28 . It should be done when it is verified that the HC is persistent (usually after 1 month of life) and could be overlooked if the genetic study demonstrates diffuse disease 28 . 18F-DOPA is not available in our country.…”

“…The most frequent complications of subtotal surgery are infections in the acute phase, development of Diabetes Mellitus, and deficiency in exocrine pancreatic function in the long term 6,28 . In a cohort of children with subtotal pancreatectomy for HC, it was reported that up to 60% required maintaining medical treatment because hypoglycemia persisted.…”

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“…Although they present with similar clinical manifestations, they differ in the management required. Focal forms of CHI, unlike the diffuse forms are amenable to surgery without any long term sequlae [4]. Owing to the fact that, near-total pancreactectomy often leads to postoperative hypoglycemias in 60% children and insulin-dependent diabetes mellitus by puberty in most, and that many diffuse forms of HI show spontaneous resolution after years of treatment, an aggressive medical treatment approach for diffuse disease is preferred to surgery [5,6].…”