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Updated: Nov 20, 2025
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Immunostaining to Visualize Murine Enteric Nervous System Development
Published on: April 29, 2015
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Congenital Myenteric Hypoganglionosis.
Raj P Kapur1,2, Andrew M Bellizzi3, Steffan Bond4
1Departments of Laboratory Medicine and Pathology.
The American Journal of Surgical Pathology
|January 25, 2021
Summary
Congenital myenteric hypoganglionosis, a rare cause of neonatal intestinal pseudoobstruction, is diagnosed by identifying small, abnormal myenteric ganglia. This study provides diagnostic guidelines and highlights a deficiency in intrinsic primary afferent neurons.
Area of Science:
- Gastroenterology
- Pediatric Pathology
- Developmental Biology
Background:
- Congenital myenteric hypoganglionosis is a rare disorder causing neonatal intestinal pseudoobstruction.
- Diagnosis relies on recognizing deviations in myenteric ganglion size and morphology in infants.
Purpose of the Study:
- To review clinical, histologic, and immunohistochemical findings in congenital myenteric hypoganglionosis.
- To delineate diagnostic features and provide guidelines for surgical pathologists.
- To investigate the underlying neuronal deficiencies in this condition.
Main Methods:
- Analysis of clinical, histologic, and immunohistochemical data from 12 patients.
- Comparison with age-matched control cases.
- Evaluation of full-thickness intestinal biopsies and immunohistochemistry for Hu C/D, calretinin, and NeuN.
Main Results:
- Established diagnostic criteria for congenital myenteric hypoganglionosis based on myenteric plexus abnormalities.
- Confirmed the necessity of full-thickness intestinal biopsies for diagnosis.
- Demonstrated reduced staining for calretinin and NeuN, indicating a deficiency in intrinsic primary afferent neurons.
Conclusions:
- Congenital myenteric hypoganglionosis is characterized by hypoplastic myenteric ganglia and a selective deficiency of intrinsic primary afferent neurons.
- Immunohistochemistry, particularly for Hu C/D, calretinin, and NeuN, aids in confirming the diagnosis.
- Practical guidelines are provided to assist pathologists in diagnosing this infrequent condition.
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