Abstract
BACKGROUND: Congenital hyperinsulinemic hypoglycemia (CHI) is a rare but serious condition resulting from genetic abnormalities that cause excessive insulin secretion and recurrent hypoglycemia. It poses significant neurological risks in infancy. When medical therapy fails, pancreatectomy becomes necessary; however, the procedure presents major anesthetic challenges due to unstable glucose levels during surgery. METHODOLOGY: We performed a retrospective study of electronic records of 34 patients <5 years who underwent laparoscopic pancreatectomy at King Faisal Specialist Hospital and Research Center (KFSHRC Riyadh, Saudi Arabia) between 2015 and 2023. RESULTS: Of the 34 patients, 64.7% (n = 22) were male; most (67.6%, n = 23) were aged 4-8 weeks. Nesidioblastosis was the most common pathology (55.9%, n = 19). The mean surgery duration was 177.2 ± 51.7 minutes. Nearly all patients (94.1%, n = 32) were referred to the intensive care unit (ICU), and 85.3% (n = 29) remained intubated postoperatively. All patients received dextrose-containing fluids (5-50% concentration). Intraoperative blood glucose levels ranged from 2.5-33.4 mmol/L, with significant variation between preoperative, intraoperative, and postoperative values (P = 0.005). Blood transfusion was required in 35.3% (n = 12) of patients, with no critically low glucose levels recorded perioperatively. CONCLUSION: Pediatric pancreatectomy for CHI is associated with significant intraoperative glucose fluctuations, necessitating meticulous glucose and fluid management. Nesidioblastosis remains the leading pathology. Most patients require postoperative ICU care with elective ventilation. Early diagnosis, vigilant anesthetic management, and structured postoperative protocols are essential to minimize morbidity.