Congenital hyperinsulinism: the significance of visual evaluation of positron emission tomography and the role of the surgeon in determining the limits of pancreatic resection
- Authors: Sukhotskaya A.A.1, Bairov V.G.1, Mitrofanova L.B.1, Perminova A.A.1, Rijkova D.V.1, Nikitina I.L.1, Amidhonova S.A.1, Kagantsov I.M.1
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Affiliations:
- Almazov National Medical Research Centre
- Issue: Vol 12, No 1 (2022)
- Pages: 19-30
- Section: Original Study Articles
- URL: https://journals.rcsi.science/2219-4061/article/view/123544
- DOI: https://doi.org/10.17816/psaic1221
- ID: 123544
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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.
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##article.viewOnOriginalSite##About the authors
Anna A. Sukhotskaya
Almazov National Medical Research Centre
Email: sukhotskaya_aa@almazovcentre.ru
ORCID iD: 0000-0002-8734-2227
SPIN-code: 6863-7436
Cand. Sci. (Med), Head of Pediatric Surgery Department
Russian Federation, 2, Akkuratova st., Saint Petersburg, 197341Vladimir G. Bairov
Almazov National Medical Research Centre
Email: v-bairov@mail.ru
ORCID iD: 0000-0002-8446-830X
SPIN-code: 6025-8991
Dr. Sci. (Med.), Professor, Head of the Department of Surgical Diseases
Russian Federation, 2, Akkuratova st., Saint Petersburg, 197341Lubov B. Mitrofanova
Almazov National Medical Research Centre
Email: lubamitr@yandex.ru
ORCID iD: 0000-0003-0735-7822
SPIN-code: 9552-8248
Dr. Sci. (Med.), Professor, Department of Pathology, Institute of Medical Education, Chief Researcher, Research Laboratory of Pathomorphology
Russian Federation, 2, Akkuratova st., Saint Petersburg, 197341Anastasiya A. Perminova
Almazov National Medical Research Centre
Email: kulikova9404@gmail.com
ORCID iD: 0000-0002-1946-0029
SPIN-code: 4076-1426
Pathologist, Postgraduate student
Russian Federation, 2, Akkuratova st., Saint Petersburg, 197341Darya V. Rijkova
Almazov National Medical Research Centre
Email: ryzhkova_dv@almazovcentre.ru
ORCID iD: 0000-0002-7086-9153
SPIN-code: 7567-6920
Professor of the Russian Academy of Sciences, Dr. Sci. (Med.), Chief Researcher of the Research Department of Nuclear Medicine and Theranostics of the Institute of Oncology and Hematology, Head of the Scientific and Clinical Association of Nuclear Medicine, Head of Department of Nuclear Medicine and Radiation Technologies, Institute of Medical Education
Russian Federation, 2, Akkuratova st., Saint Petersburg, 197341Irina L. Nikitina
Almazov National Medical Research Centre
Email: nikitina_il@almazovcenrtre.ru
ORCID iD: 0000-0003-4013-0785
SPIN-code: 7707-4939
Dr. sci. (Med.), Professor, Head of the Department of Children’s Diseases, Head of the Research Laboratory of Pediatric Endocrinology
Russian Federation, 2, Akkuratova st., Saint Petersburg, 197341Surayo A. Amidhonova
Almazov National Medical Research Centre
Email: samidkhonova@gmail.com
ORCID iD: 0000-0001-8505-5083
SPIN-code: 2280-6996
Cand. Sci. (Med.), Researcher, Research Laboratory for Surgery of Congenital and Hereditary Pathologies
Russian Federation, 2, Akkuratova st., Saint Petersburg, 197341Ilya M. Kagantsov
Almazov National Medical Research Centre
Author for correspondence.
Email: ilkagan@rambler.ru
ORCID iD: 0000-0002-3957-1615
SPIN-code: 7936-8722
Dr. Sci. (Med.), Head of the Research Laboratory for Surgery of Congenital and Hereditary Pathologies
Russian Federation, 2, Akkuratova st., Saint Petersburg, 197341References
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