J.Jpn. Surg. Soc.. 80(11): 1309-1312, 1979

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Report on the annual meeting

HIRSCHSPRUNG'S DISEASE: REVIEW OF THE STUDIES AND FUTURE SCOPE

This paper summarizes an extensive review of the studies in all aspects of Hirschsprung's disease, which have ever been carried out since the first report of this disease entity by Hirschsprung in 1886. Among the highlights of those, recent success in producing aganglionosis in a mouse strain contributed greatly to prove the cranio-caudal migration of the intramural plexus during the fetal life. This success has made a great advance in the studies of hereditary factors in this disease entity. In diagnostic procedures, there has been a progress in radiological technique and histochemical studies which have made it possible for rectal mucosal biopsy to take over the conventional full thickness rectal biopsy. The coordinative mechanism of the anorectal reflexes, in which an absence of dilating reflex of the anorectal canal is known in Hirschsprung's disease, still includes many unknown factors to be studied. Since establishment of the surgical treatment by Swenson based on a concept that the distal aganglionic segment is the cause of disease, Duhamel, Rehbein, Soave and others have reported unique procedures. Although it seems there is not many problems in surgical technique left to be challenged by surgeons, a new technique for the management of extensive aganglionosis including the distal ileum is, at present, in search.
Today, there are disease entities clinically similar to Hirschsprung's disease, of which the cause are not known. Classification based on the clarified pathogenesis of those called as oligoganglionosis, hypoganglionosis, immaturity of ganglia or pseudo-Hirschsprung's disease should be established in the future studies.

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