Neuronal intestinal dysplasia (NID) is a clinical condition similar toHirschsprung’s disease. It was first described by Meier-Ruge in 1971 and is a complex disorder of the intestinal nervous system that can involve the rectum, colon or entire intestine. NID is divided into NID-A associated with the sympathetic nervous system and NID-B associated with the parasympathetic.

Typical histological features of NID include submucosal and myenteric nerve plexus hyperplasia, giant ganglia, ectopic ganglion cells, and increased acetylcholinesterase activity. It can be present alone or in combination with Hirschsprung’s disease, which accounts for 10% to 49% of cases. Itsfrequencyrangesfrom 1 / 4,000 to 1 / 60,000 births.

SYMPTOMS

Common symptoms may include flatulence, vomiting, constipation, and intestinal obstruction.

DIAGNOSIS

There are no specific diagnostic clinical signs, and there is no exact correlation between histology and clinical manifestations. The diagnosis is made by biopsy of the intestinal wall.

TREATMENT

The exact treatment options have not been clearly defined.

Treatment is initially conservative, as clinical and histological improvement has been reported in cases of mild dysganglionosis, in the presence of immature ganglion cells in the myenteric plexus, in atypia of submucosal ganglion cells, and in cases of NID localized in different areas. When symptoms cannot be controlled conservatively, a temporary stoma may be required.In cases with severe obstructive symptoms, total colectomy and / or ileoanal anastomosis or permanent stomaare recommended.