Surgery for Congenital Hyperinsulinism

  • N. Scott AdzickEmail author
Part of the Contemporary Endocrinology book series (COE)


Our multidisciplinary approach to patients with congenital hyperinsulinism can distinguish focal from diffuse disease, localize focal lesions, and permit partial pancreatectomy with cure in almost all patients. Surgery does not cure the diffuse form of congenital hyperinsulinism but can help prevent hypoglycemia and brain damage.


Congenital hyperinsulinism Pancreatectomy 


  1. 1.
    Palladino AA, Stanley CA. A specialized team approach to diagnosis and medical versus surgical treatment of infants with congenital hyperinsulinism. Semin Pediatr Surg. 2011;20:32–7.CrossRefGoogle Scholar
  2. 2.
    Lovvorn HN 3rd, Nance ML, Ferry RJ Jr, et al. Congenital hyperinsulinism and the surgeon: lessons learned over 35 years. J Pediatr Surg. 1999;34:786–92.CrossRefGoogle Scholar
  3. 3.
    Brunelle F, Negre V, Barth MO, et al. Pancreatic venous samplings in infants and children with primary hyperinsulinism. Pediatr Radiol. 1989;19:100–3.CrossRefGoogle Scholar
  4. 4.
    Doppman JL, Miller DL, Chang R, et al. Insulinomas: localization with selective intraarterial injection of calcium. Radiology. 1991;178:237–41.CrossRefGoogle Scholar
  5. 5.
    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;39:270–5.CrossRefGoogle Scholar
  6. 6.
    Hoegerle S, Schneider B, Kraft A, et al. Imaging of a metastatic gastrointestinal carcinoid by F-18-DOPA positron emission tomography. Nuklearmedizin. 1999;38:127–30.CrossRefGoogle Scholar
  7. 7.
    Ribeiro MJ, De Lonlay P, Delzescaux T, et al. Characterization of hyperinsulinism in infancy assessed with PET and 18F-fluoro-L-DOPA. J Nucl Med. 2005;46:560–6.PubMedGoogle Scholar
  8. 8.
    Otonkoski T, Näntö-Salonen K, Seppänen M, et al. Noninvasive diagnosis of focal hyperinsulinism of infancy with [18F]-DOPA positron emission tomography. Diabetes. 2006;55:13–8.CrossRefGoogle Scholar
  9. 9.
    Laje P, States LJ, Zhuang H, et al. Accuracy of PET/CT scan in the diagnosis of the focal form of congenital hyperinsulinism. J Pediatr Surg. 2013a;48:388–93.CrossRefGoogle Scholar
  10. 10.
    Peranteau WH, Bathaii SM, Pawel B, et al. Multiple ectopic lesions of focal islet adenomatosis identified by positron emission tomography scan in an infant with congenital hyperinsulinism. J Pediatr Surg. 2007;42:188–92.CrossRefGoogle Scholar
  11. 11.
    Hussain K, Seppänen M, Näntö-Salonen K, et al. The diagnosis of ectopic focal hyperinsulinism of infancy with [18F]-dopa positron emission tomography. J Clin Endocrinol Metab. 2006;91:2839–42.CrossRefGoogle Scholar
  12. 12.
    von Rohden L, Mohnike K, Mau H, et al. Intraoperative sonography: a technique for localizing focal forms of congenital hyperinsulinism in the pancreas. Ultraschall Med. 2011;32:74–80.CrossRefGoogle Scholar
  13. 13.
    Rahier J, Guiot Y, Sempoux C. Morphologic analysis of focal and diffuse forms of congenital hyperinsulinism. Semin Pediatr Surg. 2011;20:3–12.CrossRefGoogle Scholar
  14. 14.
    Suchi M, MacMullen C, Thornton PS, et al. Histopathology of congenital hyperinsulinism: retrospective study with genotype correlations. Pediatr Dev Pathol. 2003;6(4):322–33.CrossRefGoogle Scholar
  15. 15.
    Suchi M, MacMullen CM, Thornton PS, et al. Molecular and immunohistochemical analyses of the focal form of congenital hyperinsulinism. Mod Pathol. 2006;19:122–9.CrossRefGoogle Scholar
  16. 16.
    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;47:130–5.CrossRefGoogle Scholar
  17. 17.
    Al-Shanafey S. Laparoscopic vs open pancreatectomy for persistent hyperinsulinemic hypoglycemia of infancy. J Pediatr Surg. 2009;44:957–61.CrossRefGoogle Scholar
  18. 18.
    Pierro A, Nah SA. Surgical management of congenital hyperinsulinism of infancy. Semin Pediatr Surg. 2011;20:50–3.CrossRefGoogle Scholar
  19. 19.
    Laje P, Palladino AA, Bhatti TR, et al. Pancreatic surgery in infants with Beckwith-Wiedemann syndrome and hyperinsulinism. J Pediatr Surg. 2013b;48:2511–6.CrossRefGoogle Scholar
  20. 20.
    Laje P, Stanley CA, Adzick NS. Intussusception after pancreatic surgery in children: a case series. J Pediatr Surg. 2010;45:1496–9.CrossRefGoogle Scholar
  21. 21.
    McAndrew HF, Smith V, Spitz L. Surgical complications of pancreatectomy for persistent hyperinsulinaemic hypoglycaemia of infancy. J Pediatr Surg. 2003;38:13–6.CrossRefGoogle Scholar
  22. 22.
    Adzick NS, DeLeon DD, States LJ, Lord K, Bhatti TA, Becker SA, Stanley CA. Surgical treatment of Congenital Hyperinsulinism: Results from 500 pancreatectomies in neonates and children. J Pediatr Surg. 2018:pii: S0022-3468(18)30648-1. [Epub ahead of print].

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of Surgery and the Congenital Hyperinsulinism CenterThe Children’s Hospital of Philadelphia and Perelman School of Medicine at the University of PennsylvaniaPhiladelphiaUSA

Personalised recommendations