Near-Total Pancreatectomy for Congenital Hyperinsulinemic Hypoglycemia: A Single-Center Experience From a...

Background and objective Congenital hyperinsulinemic hypoglycemia (CHH) is the leading cause of persistent hypoglycemia in neonates and infants. In high-resource settings, diagnosis and management depend on rapid genetic testing and advanced imaging to differentiate focal from diffuse disease. However, in low-resource settings, limited diagnostic capacity and lack of access to medications often necessitate early surgical intervention. This study aimed to evaluate the clinical features, surgical intervention, and outcomes in infants undergoing pancreatectomy for congenital hyperinsulinism at Al Ribat University Hospital, Sudan. Methods A retrospective descriptive study was conducted, involving all pediatric patients who underwent pancreatectomy for CHH from January 2019 to December 2024. Data were extracted from operative logs and medical records, including demographics, diagnostic investigations, medical therapy, surgical procedures, postoperative complications, histopathology, and one-year outcomes. Descriptive statistics and appropriate inferential statistical tests were used for the analysis. Results Thirty-two patients were included, of whom 19 (59.4%) were female. The majority were diagnosed during the first week of life (n = 19, 59.4%). Diazoxide was administered to 30 (93.75%) patients, with only eight (26.7%) showing a therapeutic response. Restricted access to genetic testing (n = 8, 25%) and imaging (n = 6, 18.8%) resulted in the predominance of near-total pancreatectomy (n = 31, 96.9%). Intraoperative complications were minimal, with bleeding occurring in two cases (6.25%) and vascular injury in one case (3.1%). Early postoperative hypoglycemia was noted in 13 patients (40.6%), whereas hyperglycemia was noted in one patient (3.1%). Surgical complications included wound infection in seven patients (21.9%) and intestinal obstruction in one patient (3.1%). Histopathology confirmed CHH in all cases, with diffuse disease in 27 patients (84.4%). During a one-year follow-up period, three patients (9.4%) died, including two (6.3%) due to recurrent hypoglycemia. Conclusions Near-total pancreatectomy remains a practical and life-saving option for infants with medically unresponsive CHH in low-resource settings where diagnostic and pharmacologic limitations restrict individualized care. Despite limited access to preoperative localization and the predominance of diffuse disease, surgical outcomes were acceptable, with low intraoperative morbidity and manageable rates of postoperative complications. Persistent postoperative hypoglycemia and late mortality highlight the need for structured endocrine follow-up and improved access to medical therapy. Strengthening diagnostic capacity, ensuring medication availability, and establishing coordinated multidisciplinary care pathways are essential to improving long-term outcomes for children with CHH in resource-limited environments.