Journal article
Congenital Myenteric Hypoganglionosis
The American journal of surgical pathology, Vol.45(8), pp.1047-1060
01/22/2021
DOI: 10.1097/PAS.0000000000001670
PMID: 33492848
Abstract
Congenital myenteric hypoganglionosis is a rare developmental disorder characterized clinically by severe and persistent neonatal intestinal pseudoobstruction. The diagnosis is established by the prevalence of small myenteric ganglia composed of closely spaced ganglion cells with sparse surrounding neuropil. In practice, the diagnosis entails familiarity with the normal appearance of myenteric ganglia in young infants and the ability to confidently recognize significant deviations in ganglion size and morphology. We review clinical, histologic, and immunohistochemical findings from 12 patients with congenital myenteric hypoganglionosis in comparison with similar data from age-matched controls and clearly delineate the diagnostic features of the condition. Practical guidelines are provided to assist surgical pathologists, who are likely to encounter this condition only infrequently. The diagnosis typically requires full-thickness intestinal biopsy as the abnormality is confined to the myenteric plexus in many patients. Immunohistochemistry for Hu C/D may be used to confirm hypoganglionosis. Reduced staining for calretinin and NeuN implicates a selective deficiency of intrinsic primary afferent neurons in this disease.
Details
- Title: Subtitle
- Congenital Myenteric Hypoganglionosis
- Creators
- Raj P Kapur - University of VirginiaAndrew M Bellizzi - University of Iowa Hospitals and ClinicsSteffan Bond - Providence Alaska Medical CenterHaiying Chen - Hospital for Sick ChildrenJeong S Han - Department of Pathology, Kaiser Oakland Medical Center, Oakland.Robin D LeGallo - University of VirginiaCraig Midgen - Dalhousie UniversityAlysa A Poulin - University of SaskatchewanNaseem Uddin - Southwestern Medical CenterMikako Warren - University of Southern CaliforniaJosé E Velázquez VegaCraig W Zuppan - Loma Linda University
- Resource Type
- Journal article
- Publication Details
- The American journal of surgical pathology, Vol.45(8), pp.1047-1060
- DOI
- 10.1097/PAS.0000000000001670
- PMID
- 33492848
- ISSN
- 0147-5185
- eISSN
- 1532-0979
- Language
- English
- Date published
- 01/22/2021
- Academic Unit
- Pathology
- Record Identifier
- 9984185169902771
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