Pathology of idiopathic megarectum and megacolon

Abstract

Background—The aetiology and pathology of both idiopathic megarectum and idiopathic megacolon are unknown. In particular, it is unknown whether there are abnormalities involving enteric nerves or smooth muscle.

Methods—Resected tissue was examined from 24 patients who underwent surgery for idiopathic megarectum, from six patients who had tissue resected for idiopathic megacolon, and 17 control patients who had surgery for non-obstructing large bowel cancer. Qualitative and quantitative histological examination was performed after staining with haematoxylin and eosin, periodic acid Schiff (PAS), Martius scarlet blue (MSB), and phosphotungstic acid haematoxylin (PTAH). Neural and glial tissue were examined after immunostaining with S100 and PGP9.5.

Results—Compared with controls, patients with idiopathic megarectum had significant thickening of their muscularis mucosae (median 78 v 33 μm, p<0.005), circular muscle (1000 v 633 μm, p<0.005), and longitudinal muscle (1083v 303 μm, p<0.005), despite rectal dilatation. This thickening was relatively greater in the longitudinal than in the circular muscle. Fibrosis of the longitudinal muscle was seen, using MSB staining, in 58%, of circular muscle in 38%, and of muscularis mucosae in 29% of patients. The relation between muscle thickening and fibrosis was variable. The density of neural tissue in the longitudinal muscle seemed to be reduced in patients with idiopathic megarectum. There was no thickening of enteric muscle or alteration in the density of innervation in patients with idiopathic megacolon.

Conclusion—There is notable thickening of the enteric smooth muscle in patients with idiopathic megarectum, but the architecture of the enteric innervation seems to be intact. Functional abnormalities of the latter remain a possible cause of the smooth muscle hypertrophy.