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Eur J Pediatr Surg 2002; 12(6): 429-431
DOI: 10.1055/s-2002-36853
Case Report

Georg Thieme Verlag Stuttart, New York · Masson Editeur Paris

Intestinal Pseudoobstruction as a Paraneoplastic Syndrome in Ganglioneuroblastoma

B. Wildhaber 1 , F. Niggli 2 , T. Stallmach 3 , U. Willi 4 , U. G. Stauffer 1 , P. Sacher 1
  • 1Department of Paediatric Surgery, University Children's Hospital, Zurich, Switzerland
  • 2Department of Paediatrics, University Children's Hospital, Zurich, Switzerland
  • 3Department of Pathology, University of Zurich, Switzerland
  • 4Department of Radiology, University Children's Hospital, Zurich, Switzerland
Further Information

Publication History

Received: 30 October 2001

Accepted after Revision: 5 November 2001

Publication Date:
27 January 2003 (online)

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Abstract

Intestinal pseudoobstruction may be part of a paraneoplastic syndrome. We report a teenage girl with ganglioneuroblastoma who presented with severe constipation. The intestinal pseudoobstruction was presumed to be due to inflammation of the myenteric plexus with destruction of the ganglion cells caused by antineuronal nuclear antibodies (ANNA or Anti-Hu).

Résumé

La pseudo-obstruction intestinale peut représenter un des éléments d'un syndrome paranéoplasique. Nous présentons le cas d'une jeune fille avec un ganglio-neuroblastome qui a développé une constipation sévère. Cette pseudo-obstruction intestinale est considérée comme la conséquence d'un processus inflammatoire du plexus myentérique avec destruction des cellules ganglionnaires par les anticorps anti-nucléaires (anti-ANNA ou anti-Hu).

Resumen

La pseudoobstrucción intestinal puede ser parte de un síndrome paraneoplásico. Presentamos una adolescente con ganglioneuroblastoma que sufría constipación grave. La pseudoobstrucción intestinal era presumiblemente debida a la inflamación del plexo mientérico con destrución de las células ganglionares causada por antígenos antineuronales nucleares (ANNA o antih-hu).

Zusammenfassung

Eine intestinale Pseudoobstruktion kann als Teil eines neoplastischen Syndroms auftreten. Wir berichten über eine Jugendliche mit einem Ganglioneuroblastom, welche unter einer schweren Obstipation litt. Diese wurde als Folge einer Entzündung des Plexus myentericus mit Zerstörung der Ganglienzellen durch Antikörper gegen Nervenzellnuklei (ANNA oder Anti-Hu) gedeutet.

Key words

Neuroblastoma - Intestinal pseudoobstruction - Paraneoplastic syndrome - Antineuronal nuclear antibodies (ANNA)

Mots-clés

Neuroblastome - Pseudo-obstruction intestinale - Syndrome para-néoplasique - Anticorps anti-nucléaires

Palabras clave

Neuroblastoma - Pseudo-obstrucción intestinal - Sindrome paraneoplásico - Anticuerpos nucleares anti-neuronales (ANNA)

Schlüsselwörter

Neuroblastom - Intestinale Pseudoobstruktion - Paraneoplastisches Syndrom - Antikörper gegen Ganglienzellnuklei (ANNA)

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Dr. Barbara Wildhaber

Section of Pediatric Surgery, University of Michigan Hospitals

Mott F3970, Box 0245

Ann Arbor, Michigan 48109

USA

Email: bwildhab@med.umich.edu