Congenital hyperinsulinism: the significance of visual evaluation of positron emission tomography and the role of the surgeon in determining the limits of pancreatic resection

Abstract

BACKGROUND: Congenital hyperinsulinism is a complex and multifaceted disease due to genetic disorders, some of which remain unknown. AIM: This investigation shows the value of visual assessment and not just the definition of indices (standardized accumulation index and pancreatic index) when performing PET/CT to determine the surgical correction method of congenital hyperinsulinism. MATERIALS AND METHODS: In 2017, Almazov NMRC implemented modern diagnostic capabilities for diagnosing pancreatic lesions: positron emission tomography with 18F-DOPA and intraoperative express biopsy of the pancreas. RESULTS: From 2017 to October 2021, 48 children were operated on with congenital hyperinsulinism, including 30 with focal forms, in the Department of Pediatric Surgery at Almazov NMRC. This article shows the role of the surgeon, the importance of visual assessment, and not only the determination of indices (standardized accumulation index and pancreatic index) during positron emission tomography to determine the surgical correction method of congenital hyperinsulinism. The presented approach leads to 100% recovery in focal disease forms. CONCLUSION: Partial pancreatectomy was performed in patients with focal forms, subtotal (95%) in atypical, and near total (98%99%) in diffuse forms to cope with hypoglycemia due to congenital hyperinsulinism. The scope of surgical treatment for children with congenital hyperinsulinism is determined by the data of genetic examination, positron emission tomography, and intraoperative express biopsy.