Four cases of desmosis coli: severe chronic constipation, massive dilatation of the colon, and hypoperistalsis due to of changes in the colonic connective-tissue net.

We present four patients 5, 10, 12, and 17 years of age, each with a long history of severe constipation and hypoperistalsis of the gut. Three had partial or total resection of the colon, all had enterostomies, and only one maintains normal bowel function after reanastomosis. We report the clinical courses, radiologic findings, and operative procedures for all four cases. Preoperative full-thickness biopsies and the resected colon specimens revealed a complete or incomplete lack of the mesh network of collagen. The connective-tissue layer between the circular and longitudinal muscles was missing. Contrary to expectations, the enteric nervous system (ENS) was normal or near-normal in the affected areas. Hypo- and dysganglionosis was found additionally in some proximal segments of colon and/or small bowel. This combination of clinical symptoms and pathological findings is called desmosis of the colon with reference to a working hypothesis in a preliminary report by Meier-Ruge in 1998. Aplastic or hypoplastic desmosis may be the reason for the disturbed gut motility. Histologic examination should thus not only exclude changes of the ENS.