Single-center experience description of surgical management of diffuse congenital hyperinsulinism in a pediatric non-current cohort.

INTRODUCTION

In this study, we describe surgical indications, procedure selection, and postoperative outcomes in Chinese children with diffuse congenital hyperinsulinism (DCHI). Pancreatic involvement was assessed using 18F-L-Fluoro-3,4-dihydroxyphenylalanine positron emission tomography/computed tomography (18F-L-DOPA PET/CT) to support clinical decision-making.

METHODS

Clinical and genetic characteristics were summarized for 27 children with DCHI, stratified into a surgical group (n = 12) and a non-surgical group (n = 15). Clinical characteristics were summarized descriptively. An exploratory Firth penalized logistic regression was applied to illustrate descriptive patterns in clinical features considered during surgical intervention selection at this center, without implying causal relationships or predictive effects. Metabolic outcomes after subtotal pancreatectomy (STP) and near-total pancreatectomy (NTP) were compared descriptively. Median postoperative follow-up was 2.9 years (range 2.0-8.8 years). Neurodevelopmental outcomes were assessed using standardized tools at variable ages. Kaplan-Meier analysis was applied descriptively to illustrate long-term neurological trends.

RESULTS

Of the 27 patients with medically refractory diffuse congenital hyperinsulinism, 12 (44.4%) required surgical intervention, including subtotal pancreatectomy (STP, n = 6) and near-total pancreatectomy (NTP, n = 6). The median age at diagnosis was 3 days (IQR 2-7), with a male-to-female ratio of 1.25:1. Among the 12 surgical patients, 8 underwent genetic testing, and all (8/8, 100%) were found to harbor ABCC8/KCNJ11 mutations. Overall hypoglycemia control patterns were comparable between the NTP and STP groups, although distinct long-term metabolic trade-offs were observed. At the final follow-up, postoperative diabetes (PD) was observed in 16.7% of NTP patients, while long-term neurodevelopmental outcomes were comparable between the surgical and non-surgical groups.

CONCLUSIONS

Subtotal and near-total pancreatectomy were not observed to result in major perioperative complications in this cohort of Chinese pediatric patients with medically refractory DCHI. Clinicians may need to carefully balance hypoglycemia control against the frequency of postoperative diabetes when selecting the extent of resection. Early diagnosis, timely hypoglycemia management, and structured long-term follow-up are essential to optimize long-term outcomes.