The histopathology of gastrointestinal motility disorders in children

• Published in: Seminars in pediatric surgery 2009-11, Vol.18 (4), p.206-211
• Main Authors: Feichter, Sonja, Meier-Ruge, William A, Bruder, Elisabeth
• Format: Article
• Language: English
• Subjects: Child; Child, Preschool; Connective tissue; Constipation - etiology; Constipation - pathology; Constipation - physiopathology; Desmosis; Enteric Nervous System - pathology; Enteric Nervous System - physiopathology; Ganglioneuromatosis; Gastrointestinal Motility - physiology; Hirschsprung Disease - etiology; Hirschsprung Disease - pathology; Hirschsprung Disease - therapy; Hirschsprung's disease; Humans; Hypoganglionosis; Infant; Infant, Newborn; Intestinal Mucosa - innervation; Intestinal Mucosa - pathology; Intestinal Mucosa - physiopathology; Intestinal wall; Pediatrics; Surgery
• ISSN: 1055-8586; 1532-9453
• DOI: 10.1053/j.sempedsurg.2009.07.002

Gastrointestinal motility disorders and chronic constipation are common pediatric problems. Symptoms of abdominal discomfort are frequently encountered in the daily practice of pediatricians and pediatric surgeons. Normal peristalsis depends on the interaction between muscles, nerve cells, and tendinous connective tissue of muscularis propria. Malfunction of any of these components results in a motility disorder. Aganglionosis, typically of the left distal colon, is the cause of Hirschsprung disease. Hypoganglionosis constitutes another gastrointestinal motility disorder. In hypoplastic hypoganglionosis, the number of nerve cells and the size of ganglia of the enteric nervous system are reduced, resulting in symptoms similar to aganglionosis. In intestinal neuronal dysplasia type B, submucous plexus development is disturbed. Immaturity of the enteric nervous system, but also ganglioneuromatosis, can be the underlying cause of chronic constipation. Chronic constipation may be caused by a myopathy. Aplasia or atrophy of the tendinous connective tissue of muscularis propria may cause desmosis, which may result in an aperistaltic syndrome. In severe chronic constipation, a histopathological diagnosis of the underlying cause is useful. In the diagnostic approach for most of these causes of chronic constipation, enzyme histochemistry is an efficient tool to complement conventional immunohistochemical and selected molecular technologies. An interdisciplinary approach of a gastrointestinal working group is beneficial in the management of these difficult patients.