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Chapter 26 - Congenital Hyperinsulinism and Pancreatectomy

Published online by Cambridge University Press:  30 November 2019

By
Maureen E. Scollon-McCarthy
Edited by
Adam C. Adler ,
Arvind Chandrakantan  and
Ronald S. Litman
Adam C. Adler
Affiliation:
Texas Children's Hospital
Arvind Chandrakantan
Affiliation:
Texas Children's Hospital
Ronald S. Litman
Affiliation:
The Children's Hospital of Philadelphia
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Summary

In this chapter, the rare yet critical pediatric surgical procedure of pancreatectomy for hyperinsulinism is reviewed. The pathophysiology and diagnostic approach for congenital hyperinsulinism is discussed.The author covers the surgical interventions and their related complex anesthetic management in these patients, particularly with regards to maintenance of glucose homeostasis. The preoperative evaluation, anesthetic implications and regional anesthetic considerations are presented for this complex disease process.

Keywords

HyperinsulinismHypoglycemiaCongenital hyperinsulinismpancreatectomyFDGpositron emission tomography
Type
Chapter
Information
Case Studies in Pediatric Anesthesia , pp. 113 - 117
Publisher: Cambridge University Press
Print publication year: 2019

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References

Suggested Reading

Adzick, NS, Thornton, PS, Stanley, CA, et al. A multidisciplinary approach to the focal form of congenital hyperinsulinism leads to successful treatment by partial pancreatectomy. J Pediatr Surg. 2004;39(3):270–5. PMID: 15017536.CrossRefGoogle Scholar
Arnoux, JB, Verkarre, V, Saint-Martin, C, et al. Congenital hyperinsulinism: current trends in diagnosis and therapy. Orphanet J Rare Dis. 2011;6:63. PMID: 21967988.CrossRefGoogle ScholarPubMed
Hardy, O, Hernandez-Pampaloni, M, Saffer, JR, et al. Diagnosis and localization of focal congenital hyperinsulinism by 18F-fluorodopa PET scan. J Pediatr. 2007;150(2):140–5. PMID: 17236890.CrossRefGoogle ScholarPubMed
Hardy, O, Litman, RL. Congenital hyperinsulinism: a review of the disorder and a discussion of the anesthesia management. Pediatr Anesth. 2007;17(7):616–21.CrossRefGoogle Scholar
Laje, P, Stanley, CA, Palladino, AA, et al. Pancreatic head resection and Roux-en-Y pancreaticojejunostomy for the treatment of the focal form of congenital hyperinsulinism. J Pediatr Surg. 2012;47(1):130–5. PMID: 22244405.CrossRefGoogle ScholarPubMed
Nanni, C, Fanti, S, Rubello, D. 18F-DOPA PET and PET/CT. J Nucl Med. 2007;48(10):1577–9. PMID: 17909255.CrossRefGoogle ScholarPubMed
Yorifuji, T. Congenital hyperinsulinism: current status and future perspectives. Ann Pediatr Endocrinol Metab. 2014;19(2):57–68. PMID: 25077087.CrossRefGoogle ScholarPubMed

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