Abstract
A 19-month-old girl who had a history of constipation since birth and suspected cytomegalovirus (CMV) infection was admitted. Barium enema revealed no caliber change in the colon, anorectal manometry failed to show sphincter relaxation on rectal distension, and biopsied rectal specimens contained ganglion cells. She underwent rectal myectomy, after which her symptoms improved significantly. Morphologic studies, including electron micrographs of the muscle, demonstrated nerve plexuses with degenerative Schwann cells containing abundant eosinphilic cytoplasmic granules that resembled those of a granular-cell tumor. Granular-cell tumorlike change of the plexus is an unprecedented finding in any form of Hirschsprung's disease or its allied disorders, but appears to have been responsible for her symptoms. CMV as a causative agent is also discussed with a review of the literature.
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