Epidemiology of congenital innervation defects of the distal colon.
Congenital colorectal innervation defects were evaluated by studying 3699 colonic mucosal biopsy specimens obtained from 773 patients over a 5-year period (1986-1991). In 358 cases (46.3%) a classifiable defect was present, with aganglionosis in 187 of these patients (52.2%) and hypoganglionosis of the colon in 18 (5.0%). Hypoplastic or aplastic sympathetic innervation (type-A neuronal intestinal dysplasia was found in 2.2% (n = 8) and dysplasia of the parasympathetic submucous plexus (type-B neuronal intestinal dysplasia) in 40.6% (n = 145) of the patients with classifiable defects. Identification of a specific innervation defect was not possible in 229 of the 773 patients (29.6%), 28% of whom exhibited slight dysplasia and 30% immaturity or hypogenesis of the submucous plexus. In 40% of the unclassifiable cases heterotopic nerve cells were found in the muscularis mucosae and/or lamina propria mucosae, while 2% had severe heterotopia with the cells of the myenteric plexus completely displaced into the circular and/or longitudinal muscle layers. These patients generally suffered from severe chronic constipation requiring surgical intervention. Four congenital innervation defects of the colorectum can thus be clearly differentiated at present: aganglionosis (in its various forms), hypoganglionosis, type-A neuronal intestinal dysplasia, and type-B neuronal intestinal dysplasia.
Meier-Ruge
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Institut für Pathologie, Abteilung für Neuropathologie, Basel, Switzerland.
D000110:Acetylcholinesterase / Q000032:analysis; D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003106:Colon / Q000294:innervation*; D003248:Constipation / Q000150:complications; D003937:Diagnosis, Differential; D005260:Female; D006627:Hirschsprung Disease / Q000150:complications / Q000473:pathology*; D006801:Humans; D007223:Infant; D007413:Intestinal Mucosa / Q000294:innervation*; D007770:L-Lactate Dehydrogenase / Q000032:analysis; D008297:Male; D008875:Middle Aged; D013385:Succinate Dehydrogenase / Q000032:analysis
D007770:L-Lactate Dehydrogenase; D013385:Succinate Dehydrogenase; D000110:Acetylcholinesterase