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Acquired intestinal aganglionosis after a lytic infection with varicella-zoster virus
Authors:Holland-Cunz Stefan  Göppl Max  Rauch Ulrich  Bär Claudia  Klotz Markus  Schäfer Karl-Herbert
Institution:a Department of Pediatric Surgery, University of Heidelberg, 69120 Heidelberg, Germany
b Department of Biotechnology, University of Applied Sciences Kaiserslautern, 66482 Zweibrücken, Germany
c Department of Pediatric Oncology, University of Heidelberg, 69120 Heidelberg, Germany
d Department of Pediatric Surgery, University Hospital Mannheim, Clinical Faculty Univ. Heidelberg, 68123 Mannheim, Germany
Abstract:

Background and Purpose

In this report, we present the first case of an immunologically impaired child surviving a lytic varicella-zoster virus infection affecting the enteric nervous system. In histological findings, myenteric and submucous enteric ganglia were nearly completely absent owing to virus infection.

Methods

A 3-year-old girl with acute lymphoblastic leukemia and generalized varicella-zoster infection developed an ileus. She underwent multiple laparotomies in which histological sections of the entire small intestine could be obtained.

Results

The histological evaluation of these samples showed a generalized aganglionosis with inflammatory residuals. A more detailed immunohistochemical analysis using neuronal (PGP, enolase), glial (S100), and lymphocytic (LCA) antibodies demonstrated a nearly complete neuronal loss.

Conclusion

To our knowledge, this is the first case of a secondary intestinal aganglionosis after varicella-zoster virus infection.
Keywords:Intestinal aganglionosis  Varicella-zoster infection  Acute lymphoblastic leukemia
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