Surgical treatment of congenital hyperinsulinism

Objectives: This study aims to analyze the details of surgical techniques performed in various types of congenital hyperinsulinism (CHI) and review our experience. Patients and methods: Six children with CHI (3 males, 3 females) treated between January 2013 and January 2024 were retrospectively reviewed. Results: Four patients had diffuse type CHI, and two had focal type CHI. The median age at diagnosis was 18.5 days (range, 1 to 54 days). The diagnosis involved 18F-DOPA (18-fluoro-dihydroxyphenylalanine) positron emission tomography (PET)/computed tomography (CT) in two patients and 68Ga-NODAGA-exendin-4 PET/CT in one patient. Mutations in ABCC8/KCNJ1 were detected in five patients. Ultrasonography (n=2), frozen biopsy (n=2), and indocyanine green-guided identification (n=1) were used introperatively. Near total pancreatectomy (95 to 98%) and gastrostomy were performed in four patients with diffuse CHI. Among the two patients with focal lesions, distal pancreatectomy was performed in one patient, and enucleation was performed in the other patient. Conclusion: Knowing the technical details of the surgery, preparation for different surgical approaches to various types of CHI, and being aware of the risky major complications achieve successful outcomes in pancreatic surgery.