Megacolon pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Pathophysiology
Aganglionic megacolon
Also called Hirschsprung's disease, it is a congenital disorder of the colon in which nerve cells of the myoenteric or Auerbach's plexus in its walls, also known as ganglion cells, are absent. It is a rare disorder (1:5 000), with prevalence among males being four times that of females. Hirschsprung’s disease develops in the fetus during the early stages of pregnancy. The exact genetic cause remains unsolved, although in familial cases (in which families have multiple affected patients), it seems to exhibit autosomal dominant transmission, with a gene called RET, in chromosome 10, being dominant. Other 7 seven genes seem to be implicated, however. If untreated, the patient can develop enterocolitis.
Toxic megacolon
Toxic megacolon is mainly seen in ulcerative colitis and pseudomembranous colitis, two chronic inflammations of the colon. Its mechanism is incompletely understood. It is probably due to an excessive production of nitric oxide, at least in ulcerative colitis. The prevalence is about the same for both sexes.
Megacolon in Chagas disease
In Central and South America, the most common incidence of chronic megacolon is that observed in ca. 20% of patients affected with Chagas disease, caused by Trypanosoma cruzi, a flagellate protozoan transmitted by the feces of an hematophagous insect, the assassin bugs, or by contamination through blood transfusion or pregnancy. There are several theories on how megacolon (and also megaesophagus) develops in Chagas disease. The Austrian-Brazilian physician and pathologist Fritz Köberle was the first to propose a coherent hypothesis based on the documented destruction of the Auerbach's plexus in the walls of the intestinal tracts of Chagas patients, the so-called neurogenic hypothesis. In this, the destruction of the autonomic nervous system innervation of the colon leads to a loss of the normal smooth muscle tone of the wall and subsequent gradual dilation. His research proved that, by extensively quantifying the number of neurons of the autonomic nervous system in the Auerbach's plexus, that: 1) they were strongly reduced all over the digestive tract; 2) that megacolon appeared only when there was a reduction of over 80% of the number of neurons 3) these pathologies appeared as a result of the disruption of the neurally integrated control of peristalsis (muscular annular contraction) in those parts where a strong force is necessary to impel the luminal bolus of feces; and 4) Idiopathic megacolon and Chagas megacolon appear to have the same etiology, namely the degeneration of the Auerbach's myoenteric plexus.
Why T. cruzi causes the destruction, however, remains to be elucidated: there are evidences for the presence of specific neurotoxins as well as a disordely immune system reaction.