Intestinal neuronal dysplasia — Intestinal neuronal dysplasia (IND) is a
pattern of histologic abnormalities in rectal suction biopsies among infants
or older children with chronic constipation. The clinical significance of
the findings are unclear and controversial [42]. Features include
hyperganglionosis, in which the ganglia are giant or ectopic, and increased
acetylcholinesterase staining [42,43]. Similar changes may be seen proximal
to the aganglionic segment in Hirschsprung disease. There is some
controversy whether this histological appearance is a developmental
abnormality of the intestinal submucosal and myenteric parasympathetic
nervous system, a consequence of chronic constipation, or a variant of
normal intestinal development [44]. Considerable inter-observer variation in
interpreting the histologic findings further complicates the diagnosis.
One study examined long-term outcomes in 105 patients presenting with
constipation and histologic abnormalities consistent with IND type B, and
managed conservatively with diet, laxatives, and sometimes anal dilatations
[43]. Most of the patients presented during the first year of life. Ninety-
four percent of patients had normal or near-normal defecation at 5 to 10
years of follow-up, suggesting gradual improvement over time.
