Zeitschrift für Kinderchirurgie : organ der Deutschen, der Schweizerischen und der Osterreichischen Gesellschaft für Kinderchirurgie = Surgery in infancy and childhood
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The enzyme histotopochemical characteristics of 18 cases with ultrashort Hirschsprung's disease - investigated during the past 20 years - are analysed. The aganglionic segment, having an extension of 1-3 cm, is characterised by an increase of acetylcholinesterase activity in parasympathetic nerve fibres of the muscularis mucosae. A similar increase of acetylcholinesterase in the nerve fibres of the lamina propria mucosae - as characteristic in Hirschsprung's disease - is usually absent. ⋯ Ultrashort Hirschsprung is a rare disease, although it is observed more often than total aganglionosis of the colon (Zuelzer-Wilson syndrome). The sex ratio, being 5 male male: 1 female, is within the range of Hirschsprung's disease. Hence, enzyme histotopochemical means enable substantiation of ultrashort Hirschsprung's disease as a special form of an aganglionosis of the distal rectum.