Acquired intestinal aganglionosis after a lytic infection with varicella-zoster virus

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.