Neuronal intestinal dysplasia: an entity of chronic intestinal pseudo-obstruction

Abstract

Abnormalities of bowel innervation and ganglion distribution
are represented by a wide spectrum of conditions, including
an entity known as neuronal intestinal dysplasia
[NID].
NID is characterized by localized or disseminated hypertrophic
and immature ganglion cells, hyperplasia of the
myenteric and submucosal plexi with giant ganglia [NID
type B], hypoplasia or aplasia of sympathetic innervation
of the myenteric plexus [NID type A] and sometimes by the
presence of ectopic neural formations in the submucosa and
muscular stratum. The frequency of NID coexisting with
Hirschsprung's disease has been reported to vary from 20-
66%.
Special diagnostic clinical signs do not exist, while a precise
correlation between histology and clinical manifestations
is lacking.
The precise options for therapy have not been clearly established.
If aganglionosis is excluded, treatment is initially
conservative, since clinical and histological improvement
has been reported in cases with mild dysganglionosis, in
the presence of immature ganglion cells in the myenteric
plexus, in atopies of ganglions of the submucosal plexus,
as well as in cases of limited areas NID type B. When symptoms
can not be controled with medical treatment, a temporary
loop colostomy could be undertaken. In cases with
severe obstructive symptoms subtotal colectomy and ileorectal
anastomosis is suggested.
Key words: Constipation, pseudobstruction, neuronal intestinal
dysplasia