Focal congenital hyperinsulinism: spontaneous glycemic improvement before surgery.

Abstract

Context: Congenital hyperinsulinism (CHI) is the most common genetic cause of hypoglycemia 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 glycemic evolution of FoCHI before surgery and to assess the potential role of conservative management.

Methods: We retrospectively reviewed medical records of patients with FoCHI managed at a single referral center. Congenital hyperinsulinism severity was classified according to the need for continuous gastric tube feeding (TF) to maintain glycemic 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 = .022), had less severe initial hypoglycemia (P = .003), and 4/12 did not undergo surgery and were cured of hypoglycemia 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 favorable glycemic evolution, potentially after surgical exploration to assess postoperative risk.