Current Status of Pancreatectomy for Persistent Idiopathic Neonatal Hypoglycemia Due to Islet Cell Dysplasia

  • R. M. Filler
  • M. J. Weinberg
  • E. Cutz
  • D. E. Wesson
  • R. M. Ehrlich
Part of the Progress in Pediatric Surgery book series (PEDIATRIC, volume 26)

Summary

A series of 18 children suffering from persistent idiopathic neonatal hypoglycemia (PINH) is reported. Medical and surgical managements are described in detail. All patients subjected to surgery had failed medical treatment. These patients were divided into two groups: 1) 85% pancreatectomy leaving the uncinate process in situ, and 2) 95% pancreatectomy leaving a small rim of pancreatic tissue along the duodenum and the common bile duct. The spleen was preserved in all cases. Two out of 5 children of group 1 required further resection of the pancreas for persistent hypoglycemia and were converted to 95% pancreatectomy. Since 1981 95% pancreatectomy was exclusively employed. Only one patient required insulin for 3 weeks postoperatively. Histo-pathology and immunohistochemistry revealed islet cell dysplasia and islet cell nuclear hypertrophy in the majority of cases. 35% of the patients had focal adenomatosis. Better control of hypoglycemia is achieved by primary 95% pancreatectomy and, thus, 95% pancreatectomy is recommended as the initial procedure in the treatment of PINH.

Keywords

Zinc Sugar Pneumonia Adenoma Radionuclide 

Zusammenfassung

Es wird eine Serie von 18 Kindern mit persistierender, idiopathischer neonataler Hypoglykämie (PINH) vorgestellt. Konservative und chirurgische Behandlungen werden detailliert beschrieben. Bei allen Kindern, die operiert wurden, hatte die konservative Behandlung versagt. Diese Patienten wurden in 2 Gruppen eingeteilt: 1) 85%-Pankreatektomie unter Zurücklassung des Processus uncinatus und 2) 95%-Pankreatektomie unter Zurücklassung eines schmalen Saumes von Pankreasgewebe entlang Duodenum und Ductus choledochus. Die Milz wurde in allen Fällen erhalten; 2 Kinder aus Gruppe 1 mußten sich einer weiteren Pankreasresektion unterziehen und gelangten somit in Gruppe 2. Seit 1981 wird nur noch die 95%-Pankreatektomie durchgeführt. Nur 1 Patient benötigte Insulin für 3 Wochen nach der Operation. Histopathologic und Im-munhistochemie ergaben eine Inselzelldysplasie und Inselzellkernhypertrophie in der Mehrzahl der Fälle. 35% hatten eine fokale Adenomatose. Eine bessere Kontrolle der Hypoglykämien wird durch die primäre 95%-Pankreatektomie erreicht, und deshalb wird die 95 %-Pankreatektomie als primäre Operation zur Behandlung der PINH empfohlen.

Résumé

Les auteurs présentent une série de 18 enfants atteints d’ hypoglycémie idiopathique néonatale persistante. Le traitement, tant médical que chirurgical est décrit en détails. Dans le cas de tous les patients ayant subi une intervention chirurgicale, le traitement médical avait échoué. Ces patients ont été répartis en deux groupes: 1) pancréatectomie à 85%, laissant en place le processus uncinatus pancreatis et 2) pancréatectomie à 95% laissant en place une étroite bordure de tissu pancréatique le long du duodénum et du canal cholédoque. La rate est restée en place dans tous les cas. Deux des enfants du groupe 1 ont dû subir une nouvelle résection du pancréas, l’hypoglycémie persistant, passant ainsi dans le groupe 2 des pancréatectomies à 95%. Depuis 1981, ce type d’intervention fut d’ailleurs le seul à être utilisé. Un seul patient eut besoin d’une administration d’insuline durant les trois semaines suivant l’intervention. Dans la majorité des cas, l’examen histopathologique et immunohistochimique révéla une dysplasie des îlots et une hypertrophie de leurs noyaux. 35% des patients présentaient une adénomatose focale. Le trait-ment de choix de l’hypoglycémie reste donc la pancréatectomie a 95% qui doit être le premier geste thérapeutique pour le traitement de 1’ hypoglycémie idiopathique néonatale persistante.

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Copyright information

© Springer-Verlag Berlin Heidelberg 1991

Authors and Affiliations

  • R. M. Filler
    • 1
  • M. J. Weinberg
    • 4
  • E. Cutz
    • 2
  • D. E. Wesson
    • 1
  • R. M. Ehrlich
    • 3
  1. 1.Department of SurgeryHospital for Sick ChildrenTorontoCanada
  2. 2.Department of PathologyHospital for Sick ChildrenTorontoCanada
  3. 3.Department of EndocrinologyHospital for Sick ChildrenTorontoCanada
  4. 4.University of TorontoTorontoCanada

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