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Esophageal motility in children with Hirschsprung's disease
A Staiano1, E Corazziari, M R Andreotti
1Department of Pediatrics, II School of Medicine, University of Naples, Italy.
Insights
Children with Hirschsprung's disease exhibit abnormal esophageal motility, including increased contraction wave amplitude and simultaneous contractions. These motor dysfunctions extend beyond the colon, persisting even after surgical correction.
Area of Science:
- Gastroenterology
- Pediatric Motility Disorders
- Esophageal Physiology
Background:
- Hirschsprung's disease is a congenital disorder characterized by the absence of ganglion cells in the distal colon, leading to functional obstruction.
- Extracolonic manifestations, particularly gastrointestinal motor dysfunction, are increasingly recognized in Hirschsprung's disease.
Purpose of the Study:
- To investigate esophageal motility in children diagnosed with Hirschsprung's disease.
- To determine if extracolonic motor dysfunction is a feature of Hirschsprung's disease.
- To compare esophageal motility patterns between children with Hirschsprung's disease, idiopathic megacolon, and healthy controls.
Main Methods:
- Esophageal manometry was performed on 12 children with Hirschsprung's disease.
- Manometric data were compared with those from 8 children with idiopathic megacolon and 10 healthy controls.
- Lower esophageal sphincter function was also assessed.
Main Results:
- Children with Hirschsprung's disease demonstrated significantly higher esophageal contraction wave amplitudes compared to controls.
- Simultaneous contractions and double-peaked waves were more prevalent in children with Hirschsprung's disease (57%) versus idiopathic megacolon (10%) and controls (8%).
- These esophageal motility abnormalities persisted post-surgery in Hirschsprung's disease patients, although wave amplitudes normalized.
Conclusions:
- Gastrointestinal motor dysfunction in Hirschsprung's disease is not limited to the colon.
- Observed esophageal motility abnormalities are likely intrinsic to Hirschsprung's disease and not solely a consequence of megacolon or colonic obstruction.
- Esophageal manometry may reveal extracolonic involvement in Hirschsprung's disease.
Abstract:
Esophageal motility was studied in 12 children with Hirschsprung's disease to see if extracolonic motor dysfunction was present in this disorder. Manometric tracings were compared with those from eight children with idiopathic megacolon and from 10 children without esophageal disease or constipation. Amplitude of esophageal contraction waves was significantly higher in children with Hirschsprung's disease than in the other two groups. Swallows that were followed by simultaneous contractions or double-peaked waves were also more common in the group with Hirschsprung's disease (subjects with Hirschsprung's disease, subjects with megacolon, and controls; 57%, 10%, and 8%, respectively). Lower esophageal sphincter characteristics did not differ among the three groups. The increase in simultaneous contractions and double-peaked waves persisted in those children who were reexamined following surgery for Hirschsprung's disease, whereas wave amplitudes fell to a level similar to that observed in the other groups. These data suggest that gastrointestinal motor dysfunction in persons with Hirschsprung's disease is not restricted to the colon, and that some of the observed abnormalities in esophageal motility do not reflect nonspecific responses to megacolon or colonic obstruction.