New pathogenetic aspects of gut dysmotility in aplastic and hypoplastic desmosis of early childhood.
The peristaltic movement of the gut is a function of the alternating contraction and relaxation of circular and longitudinal muscles. This movement is induced by a tendon-like connective-tissue net (TCTN) in the circular and longitudinal muscles, which are both rooted in a connective-tissue plexus layer (CTPL). In children with a therapy-resistant aperistaltic or hypoperistaltic syndrome who had normally-developed enteric innervation, a lack of the TCTN in the muscularis propria was observed. Over the last 2 years, 241 children with chronic constipation were investigated histopathologically; 46 children surgically treated by partial resection of the gut or diagnostically investigated by whole-mount biopsy. Fifteen children had a hypoperistalsis syndrome and 3 had an aperistalsis syndrome. All specimens were native and cut in a cryostat. Enteric innervation was examined by acetylcholinesterase and dehydrogenase reactions. The TCTN in the muscularis propria was stained with picric acid/sirius red. In the children with an aperistalsis syndrome, a complete lack of the TCTN in circular and longitudinal muscles was observed. A CTPL was not developed. The children with a hypoperistalsis syndrome had no CTPL, but had a partly-developed TCTN in the circular and longitudinal muscles, which gradually faded in the direction of the plexus layer. Independent of a well-developed enteric nervous system, a lack of the TCTN in longitudinal and circular muscles and a missing CTPL (aplastic desmosis) abolishes the coordinated peristaltic movement of the gut. An isolated lack of the CTPL in the myenteric plexus (hypoplastic desmosis) results in a hypoperistalsis syndrome. An anomaly of the TCTN in the muscularis propria disturbs gut-muscle mechanics, and is another cause of gut dysmotility.
Meier-Ruge
W A
WA
Institut für Pathologie, Universität Basel, Schönbeinstrasse 40, 4003 Basel, Switzerland.
Holschneider
A M
AM
Schärli
A F
AF
D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D003238:Connective Tissue / Q000473:pathology; D003248:Constipation / Q000473:pathology / Q000601:surgery; D003896:Desmosomes / Q000473:pathology*; D003937:Diagnosis, Differential; D017615:Enteric Nervous System / Q000473:pathology; D005260:Female; D005769:Gastrointestinal Motility / Q000502:physiology*; D006627:Hirschsprung Disease / Q000473:pathology / Q000601:surgery; D006801:Humans; D007410:Intestinal Diseases / Q000473:pathology* / Q000601:surgery; D007418:Intestinal Pseudo-Obstruction / Q000473:pathology / Q000601:surgery; D008297:Male; D009130:Muscle, Smooth / Q000473:pathology; D010528:Peristalsis / Q000502:physiology*