Clinical aspects of intestinal neuronal dysplasia

The aim of this study was to determine the presence of specific clinical symptoms in intestinal neuronal dysplasia (IND) and whether it correlates to the severity of histopathologic findings.

A group of 44 severe IND and a group of 16 mild IND patients diagnosed by means of a histochemical rectal biopsy were compared with a group of 37 patients with functional constipation (FC) with normal rectal biopsy results.

Patients with severe IND began their symptoms at an earlier age than those with mild IND and FC (5.2 +/- 112 months v 17.5 +/- 23 months and 22.5 +/- 21.8 months, respectively; P <.001). The presence of intestinal obstruction symptoms was more frequent in severe IND patients than in mild IND and FC patients (45.5% v 18.8% v 2.7%, respectively; P <.001). The presence of a fecaloma and soiling were less frequent in the severe IND group than in mild IND and FC groups (20.5% v 56.3% v 59.5%, respectively; P <.001 and 15.9% v 31.3% v 59.5%, respectively; P <.001). Barium enema results showed a lower incidence of rectosigmoid distension in severe IND if compared with mild IND and FC groups (45.5% v 57.1% v 96.9%; P <.001). Internal sphincter relaxation was absent frequently in the severe IND group compared with the FC group (47% v 26.9%, respectively; P <.05).

Intestinal neuronal dysplasia is a distinct histopathologic and clinical entity. Its clinical, radiologic, and manometric presentation correlates to the severity of histochemical findings.