Intestinal Neuronal Dysplasia Type B in Adults: A Controversial Entity

Abstract

Intestinal neuronal dysplasia (IND) is a well-defined entity which raises controversy among authors, described as a congenital malformation of gastrointestinal innervation and caused by dysplastic embryonal development of the enteric nervous system. It is potentially associated with mild and chronic gastrointestinal motility disturbances. IND is rarely reported in adults and especially elderly patients. The present study reports on the case of a 71-year-old man suffering from longstanding idiopathic constipation and who was misdiagnosed for more than 60 years, despite several hospital admissions and a sigmoidectomy in the meantime. On the last admission, the patient presented with megacolon, abdominal pain and X-ray finding of bowel obstruction. Due to massive large bowel dilatation, an exploratory laparotomy failed to reveal any obvious mechanical cause, and a subtotal colectomy and Hartmann's procedure was performed. Bowel continuity was performed 3 months later. Analysis of full-thickness biopsies revealed enlarged myenteric and submucosal neurons as well as an increased number of giant cells and increased acetylcholinesterase activity in the mucosa. The diagnosis of IND was established. The main diagnostic criteria, the underlining pathophysiology and the recommended therapeutic approach of this rare entity are extensively reviewed.

A case of single incision laparoscopic total colectomy for intestinal neuronal dysplasia type B

“…Conservative treatment for IND-B patients has been reported to be effective in 33–64% of the cases [13] , whereas other authors report that IND is a less mild disease than generally thought and a more aggressive management should be concerned. Since colonic peristalsis in IND cases is impaired, surgery is often the appropriate treatment [14] , [15] . An important issue in the surgical treatment of IND-B cases is to specify the colonic segment that has dysganglionosis and a demonstrable innervation defect.…”

Intestinal neuronal dysplasia type B: A still little known diagnosis for organic causes of intestinal chronic constipation

“…In recent years, an increasing number of cases of INDB in adults have been described [8286] . Some of these cases have exhibited symptoms of severe constipation since childhood [82,83] , whereas others experienced the onset of symptoms at adulthood [84] . Some patients develop serious complications, such as chronic intestinal pseudoobstruction, acute bowel obstruction or intestinal infarction [8486] .…”

“…Clinical management must follow the currently used guidelines for the treatment of intestinal chronic con stipation in children, including fecal desimpaction and laxatives [72] . Although there is not a wellestablished role to surgical treatment as in Hirshcprung's disease, there are some reports of this modality of treatment in INDB [83] . Surgical treatment can be performed through different techniques [11,46,63,92] .…”

“…Some case series with a small number of patients showed symptoms improvement after a temporary colostomy [6,46,63] . Several reports described a colonic resection in patients with confirmed diagnosis at 71 years of age [83] .…”

About the Respective Roles of Central and Intraorgan Nervous Systems in Regulation of the Gastrointestinal Tract Physiology and Morphology

“…As to the specific role of intramural nervous system in conditioning intestinal function and morphology, Hirschprung's disease being considered as an intestinal neuropathy with muscular dysfunction [19] constitutes an interesting investigation model. Traumatic interruption of the spine cord, i.e.…”