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Visceral neuropathies responsible for chronic intestinal pseudo-obstruction syndrome in pediatric practice: analysis
J Navarro1, E Sonsino, N Boige
1Service de Gastroentérologie Pédiatrique, Hopital Robert Debre, Paris, France.
Insights
Visceral neuropathies in children often present with abdominal issues and malnutrition. Histopathology reveals two main patterns: myenteric plexus or glial cell hyperplasia, impacting nerve function and requiring extensive support.
Area of Science:
- Pediatric Gastroenterology
- Neuropathology
- Gastrointestinal Motility Disorders
Background:
- Visceral neuropathies are rare but serious conditions affecting children's gastrointestinal function.
- Clinical presentation often includes abdominal distension, pain, obstruction, and malnutrition.
- Intestinal bacterial overgrowth is a common complication.
Purpose of the Study:
- To analyze histopathological patterns of visceral neuropathies in children.
- To correlate clinical symptoms with specific neuropathological findings.
- To identify potential associations with other syndromes.
Main Methods:
- Analysis of 26 children (1 day to 15 years) with visceral neuropathies.
- Histopathological examination of aspiration biopsies, surgical rectal biopsies, and ileal/ileocolic biopsies.
- Conventional light microscopy and silver staining techniques were employed.
Main Results:
- Two main histopathological patterns were identified: myenteric plexus hyperplasia (9 patients) and glial cell hyperplasia (15 patients).
- Myenteric plexus hyperplasia showed enlarged ganglionic nodes and altered neurons; four patients had multiple endocrine neoplasia syndrome.
- Glial cell hyperplasia featured sparse ganglion cells and hypertrophic Schwann fibers; 11 patients had neonatal intestinal obstruction. Silver stains revealed abnormalities in the second group.
Conclusions:
- Visceral neuropathies in children exhibit distinct histopathological features, primarily myenteric or glial cell hyperplasia.
- These patterns are associated with specific clinical manifestations, including neonatal intestinal obstruction and risk of medullary thyroid carcinoma.
- Management often requires surgical intervention (intestinal derivation) and prolonged nutritional support (total parenteral nutrition).
Abstract:
Twenty-six children aged from 1 day to 15 years (18 boys and 8 girls) with visceral neuropathies are analyzed. Clinical symptomatology is dominated by abdominal distension, attacks of occlusion, abdominal pain, and malnutrition. Intestine bacterial overgrowth is frequent. From aspiration biopsies, surgical rectal biopsies, and, in some cases, ileal or ileocolic biopsies, histopathological studies revealed two patterns. One group had abnormalities of the myenteric plexus identified by conventional light microscopic studies, with two patterns: [myenteric plexus hyperplasia (9 patients), characterized by large ganglionic nodes, penetration into the mucosal zone, and altered argyrophilic neurons]. Clinically this pattern was observed in four patients with multiple endocrine neoplasia syndrome with risk of medullary thyroid carcinoma. The second pattern observed was characterized by glial cell hyperplasia (15 patients). Ganglion cells are present but are small and sparse, often infiltrated by collagen tissue; Schwann nerve fibers are hypertrophic. Eleven patients presented with neonatal intestinal obstruction. The second group is characterized by normal conventional light microscopic examination, but silver stains revealed important abnormalities of argyrophobic cells (one case) or argyrophilic cells (one case). In the two groups, most of the patients needed intestinal derivation and prolonged nutritional support with total parenteral nutrition.