elastin and Hirschsprung-Disease

The aim of this study was to ascertain the incidence of mucosal prolapse changes in Hirschsprung's disease. Twenty-three random, consecutive resection specimens for this condition were analysed for the histological features of prolapse. Eight cases showed histological evidence suggesting mucosal prolapse at the junction between ganglionic and aganglionic bowel. Thickening and splaying of the muscularis mucosae with extension into the lamina propria, and the presence of metaplastic or hyperplastic tubules with goblet cell and cryptal hypertrophy were the dominant histological features found in the eight cases. In addition, an increase in elastic fibres in the lamina propria and diamond-shaped glands were seen to varying degrees in all eight cases. Mucosal prolapse was more prominent in the older patients and is, therefore, related to duration of symptoms. Differential pressures in ganglionic and aganglionic segments of bowel, together with faecal stream and straining are likely to be of pathogenetic significance.

Topics: Child; Child, Preschool; Elastin; Female; Hirschsprung Disease; Humans; Infant; Infant, Newborn; Intestinal Diseases; Intestine, Large; Male; Mucous Membrane; Prolapse