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The histopathology of gastrointestinal motility disorders in children
Sonja Feichter1, William A Meier-Ruge, Elisabeth Bruder
1Department of Pediatric Surgery, Hospital Lucerne, Lucerne, Switzerland.
Insights
Pediatric chronic constipation and gastrointestinal motility disorders stem from issues in muscles, nerves, or connective tissue. Histopathology and enzyme histochemistry aid in diagnosing these complex conditions for better patient management.
Area of Science:
- Pediatric Gastroenterology
- Gastrointestinal Motility
- Histopathology
Background:
- Gastrointestinal motility disorders and chronic constipation are prevalent pediatric issues causing abdominal discomfort.
- Normal peristalsis relies on the coordinated function of muscles, enteric nerves, and connective tissue.
- Malfunctions in these components lead to various motility disorders.
Purpose of the Study:
- To review the causes of pediatric gastrointestinal motility disorders and chronic constipation.
- To highlight the utility of histopathological diagnosis in severe cases.
- To emphasize the role of enzyme histochemistry in diagnosis.
Main Methods:
- Review of literature on pediatric gastrointestinal motility disorders.
- Discussion of histopathological findings in conditions like Hirschsprung disease, hypoganglionosis, and intestinal neuronal dysplasia.
- Evaluation of enzyme histochemistry as a diagnostic tool.
Main Results:
- Motility disorders can result from aganglionosis (Hirschsprung disease), hypoganglionosis, intestinal neuronal dysplasia type B, enteric nervous system immaturity, ganglioneuromatosis, myopathy, or desmosis.
- Histopathological diagnosis is valuable for identifying the underlying cause of severe chronic constipation.
- Enzyme histochemistry is an effective method to complement other diagnostic technologies.
Conclusions:
- Various structural abnormalities of the enteric nervous system, muscles, and connective tissue cause pediatric gastrointestinal motility disorders.
- Accurate histopathological diagnosis is crucial for managing severe chronic constipation.
- An interdisciplinary approach and advanced diagnostic tools like enzyme histochemistry improve patient outcomes.
Abstract:
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.
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