Focal congenital hyperinsulinism: spontaneous glycemic improvement before surgery

CONTEXT: Congenital hyperinsulinism (CHI) is the most common genetic cause of hypoglycaemia in infants. Severe CHI may result from a focal pancreatic lesion (FoCHI), which can be surgically removed, although postoperative complications may occur, particularly after pancreatic head resections. OBJECTIVE: To describe the spontaneous glycaemic evolution of FoCHI before surgery and to assess the potential role of conservative management. DESIGN & SETTING: We retrospectively reviewed medical records of patients with FoCHI managed at a single referral centre. CHI severity was classified according to the need for continuous gastric tube feeding (TF) to maintain glycaemic control prior to surgery, defining a TF group and a no-tube feeding (NTF) group. RESULTS: Thirty-nine patients were included: 12 (30.8%) in the NTF group and 27 (69.2%) in the TF group. Patients in the NTF group were diagnosed later (p = 0.022), had less severe initial hypoglycaemia (p = 0.003), and 4/12 did not undergo surgery and were cured of hypoglycaemia before 6 years of age. We developed an exploratory predictive score for early disease evolution, showing a specificity of 97%. Surgery was curative in most patients (96.3% in the TF group, 100% in the NTF group). Long-term complications were uncommon (8.6% vs. 0%) and consisted exclusively of exocrine pancreatic insufficiency, occurring only after pancreatic head surgery (15.8%). CONCLUSION: Approximately 30% of infants with FoCHI demonstrated rapid early improvement. While surgery remains the standard curative treatment, a conservative approach may be considered in selected infants with pancreatic head lesions who show early favourable glycaemic evolution, potentially after surgical exploration to assess postoperative risk.