ESPE Abstracts

Introduction: Open near-total pancreatectomy remains the standard treatment for medically unresponsive diffuse Congenital Hyperinsulinism (CHI). This procedure involves significant postoperative morbidity, prolonged recovery and a high risk of adhesions. Minimally invasive surgery (MIS) near-total pancreatectomy in infants with diffuse CHI may reduce these complications but is challenging due to the risk of common bile duct (CBD) injuries. Intraoperative complications can lead to insufficient removal of pancreatic tissue around the CBD, causing persistent severe hypoglycemia or CBD injuries that require complex reconstructive surgery and result in long-term morbidity. Even with open surgery, accurately visualizing these structures is difficult. Fluorescence-guided surgery (FGS) using Indocyanine Green (ICG), an anionic amphiphilic tricarbocyanine dye used clinically for over 60 years, exploits ICG's confinement within the vasculature and rapid biliary excretion, making ICG ideal for MIS. This case series explores using FGS with ICG to enhance MIS near-total pancreatectomy for infants with diffuse CHI.

Method: This retrospective analysis involved five consecutive infants with diffuse CHI who underwent FGS with ICG and laparoscopic near-total pancreatectomy at a single centre between 2021 and 2024.

Results: Four infants had a homozygous ABCC8 variant, and one had a de novo dominant ABCC8 missense variant. All infants had medically unresponsive diffuse CHI and underwent laparoscopic near-total pancreatectomy, with approximately 95% of pancreatic tissue removed. ICG dye was given at 0.5 mg/kg the night before surgery. Rapid recovery was observed, with morphine discontinued and enteral feeds started at a mean of 5 and 3.8 days, respectively. No incidents of bleeding or CBD injury were reported. One infant had a skin infection at the gastrostomy site, and another had acute hepatitis of unclear cause, which resolved. The mean hospital stay post-operation was 44.4 days. Two infants developed hyperglycemia requiring insulin therapy for six weeks after surgery. The mean fasting tolerance on discharge was six hours. None of the infants required glycaemic medications upon discharge and all maintained euglycemia on a standard milk formula, except for one infant who needed carbohydrate supplementation. Additionally, none of the infants showed evidence of pancreatic exocrine insufficiency at discharge.

Conclusion: The success of MIS near-total pancreatectomy in infants with diffuse CHI relies on precise visualization of critical anatomical structures. FGS with ICG significantly improves visualization, enhancing surgical precision, reducing complications, and allowing rapid recovery. This technique advances the management of diffuse CHI in infants, offering a safer, and potentially equally effective alternative to traditional open surgery.