Aganglionosis of the colon. Morphologic investigations in 524 patients

2014 biopsies and surgical specimens of 524 patients with suspected aganglionosis of the colon were analyzed using enzyme histochemical techniques for acetylcholinesterase and lactate dehydrogenase. The diagnosis could be confirmed in 70 patients. Hypoganglionosis was found in 6, neuronal dysplasia (or hyperganglionosis) in 2, and a complete lack of intramural neurons and nerve fibers in 1 patient. The disease was found to occur most often during the first year of life (65.7%) and to be more common in males than females (3.3:1). Enzyme histochemistry is useful for the final diagnosis of aganglionosis provided the biopsy contains mucosa and parts of the submucosa of the colon.