Acquired hypoganglionosis results in reduced gut motility and constipation.
The typical onset of symptoms is during adolescence or as an adult.
Conditions associated with acquired hypoganglionosis:
(1) Chaga's disease
(2) chronic intestinal pseudo-obstruction
(3) enteric myopathy secondary to mutations in ACTG2
(4) enteric neuropathy
(5) Waardenburg-Shah syndrome
Histologic features:
(1) degeneration of ganglion cells in the colon wall
(2) reduction in ganglion cells in the myenteric and submucosal plexuses
(3) gliosis
In some cases the reduction in ganglion cells may follow bowel wall inflammation.
The reduction in ganglion cells may be limited to a colon segment. If so, then excision of the involved segment can improve gut motility.
The diagnosis requires exclusion of late-onset Hirschsprung's disease.
