Total Pancreatectomy in a Case of Nesidioblastosis Due to Persisting Hyperinsulinism Following Subtotal Pancreatectomy

  • P. Dohrmann
  • W. Mengel
  • J. Splieth
Part of the Progress in Pediatric Surgery book series (PEDIATRIC, volume 26)


Hypoglycemia with hyperinsulinism persisted in a newborn weighing 6410 g despite treatment with high doses of diazoxide and glucagon, as well as infusions of glucose and somatostatin. A subtotal pancreatectomy was performed after nesidioblastosis had been diagnosed on the basis of the laboratory findings. Due to the persistence of therapy-resistant hypogycemia, a total pancreatectomy preserving the duodenum and the bile duct was done 6 weeks later. With insulin and pancreatic enzyme substitution the now 6-year, 9-month-old child has shown normal, age, appropriate development.


Brain Damage Total Pancreatectomy Efferent Duct Subtotal Pancreatectomy Fetal Islet 
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Bei einem 6410 g schweren Neugeborenen persistierte eine Hypoglykämie mit Hyperinsulinismus trotz hochdosierter Diazoxid- und Glukagonbehandlung sowie Glukoseinfusionen und Somato-statininfusion. Nachdem laborchemisch die Diagnose einer Nesidioblastose gestellt wurde, erfolgte primär die subtotale Pankreasresektion. Bei anhaltenden therapieresistenten Hypoglykämien erfolgte 6 Wochen später die totale Pankreatektomie unter Erhaltung des Duodenums und des Ductus choledochus. Das nunmehr 63/4 Jahre alte Kind hat sich unter Insulin- und Pankreon-substitution altersentsprechend normal entwickelt.


Chez un nouveau-né pesant 6410 g, on constatait une hypoglycémie persistante et une hyper-insulinie en dépit de l’administration à hautes doses de diazoxide et de glucagon et de perfusions de glucose et de somatostatine. Les résultats de l’examen en laboratoire ayant confirmé le diagnostic de nésidioblastose, l’enfant subit d’abord une pancréatectomie subtotale puis, six semaines plus tard, une pancréatectomie totale, conservant le duodénum et le canal cholédoque car l’hypoglycémie résistait au traitement. Cet enfant, qui a maintenant 6 ans et 9 mois, est traité avec une substitution d’insuline et de pancréatine, et sa croissance et son développement sont normaux pour son âge.


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Copyright information

© Springer-Verlag Berlin Heidelberg 1991

Authors and Affiliations

  • P. Dohrmann
    • 1
  • W. Mengel
    • 1
  • J. Splieth
    • 1
  1. 1.Department of General SurgeryPediatric SurgeryKiel 1 (W)Germany

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