ESPE Abstracts

Background: Congenital Hyperinsulinism (CHI) is the most common cause of recurrent and severe hypoglycaemia in childhood and can be broadly categorised into two subtypes. Diffuse CHI (CHI-D) involving all pancreatic cells is usually treated with medications and rarely subtotal pancreatectomy. Focal CHI (CHI-F) involves a solitary insulin hypersecreting pancreatic lesion and can be cured following surgical lesionectomy. Many patients with CHI-F and CHI-D undergo prolonged hospital admissions and require feeds delivered via nasogastric (NGT) or gastrostomy tubes or administration of parenteral nutrition (PN) to meet nutritional demands. Combined with potentially emetogenic medications, interruption of normal feeding milestones and the possible adverse effect of hyperinsulinism itself, infants are at high risk for the feeding problems frequently observed in severe CHI. We aimed to describe types and incidence of feeding problems in a longitudinal cohort of children with CHI and investigate factors predictive of resolution.

Methods: All patients with CHI admitted to a specialist centre during 2015-2016 were assessed for feeding problems at hospital admission and for three years following discharge, through a combination of specialist speech and language therapy review, parent-report and research telephone clinics.

Results: Twenty-five patients (18% of all patients admitted) with CHI were prospectively identified to have feeding problems related to sucking (n = 6), swallowing (n = 2), vomiting (n = 20) and feed aversion (n = 17). Sixteen (64%) patients required feeding support by NGT/gastrostomy tubes at diagnosis; tube feeding reduced to four (16%) patients by one year and three (12%) patients by three years. Feed aversion resolved slowly with a mean time to resolution of 240 days after discharge; in 15 patients followed up for three years, 6 (24%) continued to report significant feed aversion. The mean time (days) to resolution of feeding problems was lower in those who underwent lesionectomy (n = 4) than in those who did not (30 vs 590, P = 0.009) and significance persisted after adjustment for associated factors in Cox Regression analysis (P = 0.015). No other clinical factors investigated (gene mutation status, neurodevelopmental problems, glucose infusion rate, use of PN, NGT/gastrostomy, glucagon or octreotide) were predictive of faster resolution of feeding problems.

Conclusion: Feeding problems are frequent in patients with CHI and frequently require support over several years following discharge from hospital. Patients often require feeding via the non-oral route and this requirement may persist for years. Successful lesionectomy in focal CHI is the only factor that predicts a significantly shorter time to resolution of feeding problems.