J.Jpn. Surg. Soc.. 81(9): 1223-1227, 1980
Report on the annual meeting
PATHOPHYSIOLOGY AND TREATMENT OF HIRSCHSPRUNG'S DISEASE
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1st Department of Surgery, Hyogo College of Medicine Eizo Okamoto |
In 1964, the author found histologically that, in normal human fetal subjects, the intramural neuroblasts complete their migration from the upper alimentary tract down to the rectum by 12th week of gestation―so called “craniocaudal migration theory.” In Hirschsprung's disease, this migration is considered to be arrested in various levels of the intestines, leaving aganglionic segment.
Histochemical study revealed that there was an extreme proliferation of both the sympathetic and parasympathetic nerve fibers in this aganglionic segment. However, pathophysiological study using aganglionic mouse failed to show that these extrinsic nerve fibers had significant effects on the aganglionic segment of the bowel.
On the other hand, the concept of existence of nonadrenergic inhibitory neuron in the normal intramural plexus has recently been well-established. The cause of the lack of relaxation of the aganglionic segment is considered to be due to an absence of this non-adrenergic inhibitory neuron.
By manometric and cineradiographic examination, defecatory abnormality in Hirschsprung's disease was found to be due to aperistalsis of the aganglionic segment as well as “sphincter achalasia.”
Nowadays, diagnosis and treatment of Hirschsprung's disease have been well established except for an extensive aganglionosis and other similar disease entities. We identified a group of cases with “immaturity of ganglia”. For clarification of immaturity of ganglia, we have been investigating the size of the ganglion cells and their nuclei by means of image analysing computer technique.
This paper summarized our achievements of studies and the present problems in Hirschsprung's disease and the future approach to this disease was discussed.
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