Related Experiment Video
Updated: Apr 9, 2026
Full-Endoscopic Surgery for Hypothalamic Hamartoma Resection
Published on: April 12, 2024
Single-center experience description of surgical management of diffuse congenital hyperinsulinism in a pediatric
Xiaoxiang Li1,2, Congli Chen3,4, Yangmingyue Ji1
1Department of Endocrinology, Genetics and Metabolism, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
Insights
Surgery for diffuse congenital hyperinsulinism (DCHI) in Chinese children, including subtotal and near-total pancreatectomy, showed comparable outcomes. Careful balancing of hypoglycemia control and postoperative diabetes risk is crucial for optimizing long-term results.
Area of Science:
- Pediatric Surgery
- Endocrinology
- Medical Genetics
Background:
- Diffuse congenital hyperinsulinism (DCHI) is a rare condition causing persistent hypoglycemia.
- Medically refractory DCHI often necessitates surgical intervention.
- Accurate assessment of pancreatic involvement is key for surgical planning.
Purpose of the Study:
- To describe surgical indications, procedure selection, and outcomes in Chinese children with DCHI.
- To evaluate the effectiveness of subtotal pancreatectomy (STP) and near-total pancreatectomy (NTP) in managing DCHI.
- To analyze postoperative metabolic and neurodevelopmental outcomes.
Main Methods:
- Retrospective summary of clinical and genetic characteristics for 27 children with DCHI.
- Stratification into surgical (n=12) and non-surgical (n=15) groups.
- 18F-L-DOPA PET/CT used for pancreatic assessment; Firth penalized logistic regression for exploratory analysis; Kaplan-Meier for neurodevelopmental trends.
Main Results:
- 12 out of 27 patients (44.4%) underwent surgery (6 STP, 6 NTP).
- ABCC8/KCNJ11 mutations were found in all tested surgical patients (8/8).
- Hypoglycemia control was similar between STP and NTP; 16.7% of NTP patients developed postoperative diabetes; neurodevelopmental outcomes were comparable between surgical and non-surgical groups.
Conclusions:
- Subtotal and near-total pancreatectomy are safe procedures with no major perioperative complications in this pediatric DCHI cohort.
- Balancing hypoglycemia control with the risk of postoperative diabetes is critical when choosing resection extent.
- Early diagnosis, prompt management, and structured follow-up are vital for optimal long-term outcomes in DCHI.
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.
