Omphalomesenteric duct remnants (ODR) including Meckel diverticula often present with symptoms of bowel obstruction. Their histologic features are varied and include heterotopic gastrointestinal mucosa and/or pancreatic tissue within the wall. Abnormalities of the submucosal plexus of Meissner, however, have not been documented in the literature. Thus, we have examined a number of ODR for evidence of ganglion abnormalities. Fifty-three cases of ODR were retrieved from our archives, along with 25 nonduodenal small bowel control specimens obtained from autopsies of patients without clinical or pathologic evidence of enteropathy. Histologic criteria for the diagnosis of abnormal hypercellular/heterotopic ganglia (HHG) in ODR were defined as a single submucosal ganglion/plexus containing >10 neurons, or >5 submucosal ganglions per high-power (X40) field, or heterotopic ganglion cells within the muscularis mucosa or the lamina propria. HHG, histologically indistinguishable from intestinal neuronal dysplasia type B, were found in more than half of the ODR (ODR: 51.9%, n = 50 vs. control: 4%, n = 25, P = 3.6 X 10−6), particularly those excised for obstructive complications (ODR with acute abdomen: 65.7%, n = 35, vs. ODR without acute abdomen: 33.3%, n = 15, P = 0.035). HHG were present in equal numbers of inflamed and noninflamed ODR (inflamed: 53.6%, n = 28, vs. noninflamed: 59.1%, n = 22, P = 0.70). A similar incidence was found in ODR with heterotopia and without (with heterotopia: 61.1%, n = 18, vs. without: 53.1%, n = 32, P = 0.59). ODR frequently contained HHG histomorphologically similar to those found in intestinal neuronal dysplasia, type B (IND-B). The overrepresentation of HHG in symptomatic ODR patients suggests an association with bowel obstruction. The data did not demonstrate a relationship with either inflammation or heterotopia.
Challenges in the diagnosis of intestinal neuronal dysplasia type B: A look beyond the number of ganglion cells
