Desmosis of the colon: a working hypothesis of primary chronic constipation.

Over the last few years, resected specimens of colon from children and young adults have been systematically studied with regard to the vegetative innervation of the colon and its net of stabilizing connective tissue in the muscularis propria. From the basic investigations of Goerttler, it is known that this connective tissue net has important functions in the coordinated movement of longitudinal and circular muscles. The present study reports on cases of chronic constipation, which showed in most cases histopathologically a normal number of nerve cells and ganglia in the myenteric plexus, but abnormalities in the connective tissue net (desmosis). Over 6 years, 236 cases (12 +/- 8 years of age), which were treated surgically for an aganglionosis, hypoganglionosis, hypoperistalsis syndrome and megacolon development, were histopathologically investigated. All surgical specimens were native, caudo-cranially coiled, and were cut 15 microns (equiv. to 4-5 microns paraffin sections) by a cryostat. The vegetative nervous system of the gut was stained with acetylcholinesterase and dehydrogenase reactions. The tissue was also fixed in formalin, embedded in paraffin, and 4 microns sections were stained with hematoxylineosin and picric acid/sirius red. Collagen III; S 100 and PGP 9.5 immunohistochemical reactions were performed. 14 surgical specimens (6%) showed total or focal lack of the connective tissue net in the vicinity of the myenteric plexus. Between the connective tissue net of circular and longitudinal muscles, a continuous connection was missing. These cases were mainly characterized by a hypoperistalsis syndrome or a megacolon without any anomaly of the vegetative innervation. 222 cases (94%) were treated for Hirschsprung's disease or hypoganglionosis without any anomaly of the connective tissue of the muscularis propria. The missing continuity of the connective tissue net in the muscularis propria seems to abolish coordinated alternative movement of circular and longitudinal muscles. Therefore, the lack of coordinated propulsive activity of the colon results in a therapy-resistant chronic constipation, with a hypoperistalsis syndrome.