ESPE Abstracts (2022) 95 FC8.6


1Universitätsklinikum Ulm, Ulm, Germany; 2Charité - Universitätsmedizin Berlin, Berlin, Germany; 3Radboud University Medical Centre, Nijmegen, Netherlands

Congenital hyperinsulinism (CHI) is a life-threatening disease and manifests in the majority of cases in the first days after birth. Based on the distribution of affected cells, focal CHI forms are distinguished from diffuse CHI forms. Focal forms occur in most cases due to a paternally inherited heterozygous mutation in a subunit of an ATP sensitive potassium channel (ABCC8, KCNJ11). Within the diagnostic setting, focal forms can be visualized by 18F DOPA PET scan, as a marker for beta-cell secretion activity. However, appropriate intra-operative detection of CHI focus is still pretentious. For this reason, we evaluated whether radio-guided surgery – a procedure which is well established for tumor resection of other entities - may facilitate the surgical approach. For this proof-of-concept study, we used 68Ga-Exendin-4 as a tracer, which binds to GLP1 receptors and is thereby supposed to visualize beta-cell mass. This tracer has already been evaluated for the CHI diagnostic set-up (Boss et al, J Nucl Med. 2022). In 12 CHI patients, 18F-DOPA CT and 68Ga-Exendin-4 PET MRI scan has been performed. Then, during surgery approx. 25 mBq Exendin was injected intravenously. Approximately 30-60 minutes after the injection of the radiotracer, a hand-held cable free positron probe with a spatial resolution of 7 mm for positron radiation was placed over the pancreas and surrounding organs to intraoperatively localize the lesion by radiation signal detection. Radio-guidance led to a convenient and facilitated identification of the affected pancreatic region and removal of the lesion. We analyzed the frequency of complications due to surgery, duration of the operation and clinical outcome and compared the data with 12 operations, performed without radio-guidance. In conclusion, we demonstrate data about specificity and accuracy of 68Ga-Exendin-4 as a diagnostic and therapeutic tool to improve the surgical procedure in patients with focal forms of congenital hyperinsulinism.

Volume 95

60th Annual ESPE (ESPE 2022)

Rome, Italy
15 Sep 2022 - 17 Sep 2022

European Society for Paediatric Endocrinology 

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