Intestinal neuronal dysplasia and its morphometric evidences

Abstract

The aim of this study was to morphometrically objectify the characteristics of intestinal neuronal dysplasia (IND) B by optic electronic image analysis. Biopsies of 60 children divided into two age groups (8 ± 4 months and 4 years ± 20 months) were examined. Three groups (n = 20) were studied: (1) isolated IND B; (2) Hirschsprung-associated IND B (NAIND), and (3) normal controls. A histotopochemical lactic dehydrogenase (LDH) reaction was used for the morphometric measurement of ganglion size, nerve cell size, and number of nerve cells per ganglion. The submucous neural density was measured with an acetylcholinesterase reaction. The results showed no significant morphometric differences between isolated IND and HAIND. Aging caused an increase in ganglion and nerve cell size. The density of the submucous plexus decreased with age. All parameters measured were significantly different from normal controls. Giant ganglia with a high number of LDH-positive nerve cells (IND: > 7, controls: 4 ± 1 nerve cells/ganglion) were the most relevant diagnostic parameter of IND. The pathogenesis of a dysganglionosis is dominated by abnormal early, genetically caused laminin expression during embryonic life, blocking neuroblast migration (aganglionosis) and prematurely differentiating neuroblasts into myenteric (hypoganglionosis) and submucous plexus (IND). IND B, hypoganglionosis, and aganglionosis are different manifestations of an identical developmental abnormality in which IND is the weakest form.