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Abstract
This study presents the case of a patient who had jejunal diverticulosis with perforation
and abscess formation as a complication of Fabry's disease. Light microscopy disclosed
glycolipid deposition in the neurons and nerve fibers of the intestinal nerve plexuses
and smooth muscle. Silver stains of the myenteric plexus in the involved segment of
the bowel showed enlarged, granular argyrophobic neurons and a marked decrease in
the number of argyrophilic neurons, with those remaining being enlarged and distorted
by the cytoplasmic glycolipid accumulation. These abnormalities of the myenteric plexus
suggest that jejunal diverticulosis may be the result of a variety of disorders of
the smooth muscle or myenteric plexus, or both. We propose that jejunal diverticulosis
in our patient was a consequence of uncoordinated smooth muscle activity resulting
from Fabry's involvement of myenteric plexus neurons, with mucosal protrusion through
the smooth muscle.