The basic defect in Hirschsprung´s disease is the abscence of ganglia and the associated loss of propulsive activity in a segment of rectum and colon. These segments almost always begin at the anocutaneous junction and extends proximally for varying distances.
The commonly used operations have been upon the principles of either resection, as in Swenson´s operation, or exclusion as in Duhamel´s operation, of the functionally incompetent segment. Each of these techniques has problems unique to itself, but both have the inherent problems of aganglionic internal sphincter muscle of anus. If too much of this muscle is resected or bypassed, incontinence results, and if not enough is resected, persistence or recru-descence ensues.
Boley is described herein, Soave´s procedure of endorectal pull through without anastomosis and the technique presently utilized internal bypass of the involved segment. Thus, the normal propulsively active colon can be brought directly to the anocutaneous junction, while the internal sphinter muscle of the anus is retained intact for continence.
This operation includes resection of the mucosa of retained portion of the rectum, the rectum, the aganglionic muscular cuff with its sensory reflexs being retained, and endorecta bypass with normal intestine and primary anastomosis at the anocutaneous junction. Since the entire pelvic dissection is performed within the muscular sleeve, possible damage to the pelvic nerves is avoided.
The present authors describe the four cases with typical roentgenographic appearance of congenital megacolon and the pathologic findings that establish aganglionosis as the histo-logic entity and also performed an endorectal pull-through operation with primary anasto-mosis for Hirschsprung´s disease, and presented with review of the literature.
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