ArticleModern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc2017
A critical appraisal of the morphological criteria for diagnosing intestinal neuronal dysplasia type B.
Article in Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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14 citing papers in PubMed, 22 citations in OpenAlex.
- Clinical characteristics, pathological comorbidities, BMI-for-age Z-score, and machine learning-based exploration of postoperative enterocolitis in iIntestinal neuronal dysplasia: a 20-year retrospective study.Pediatric surgery international · 2026Article
- Intestinal Neuronal Dysplasia Type B as Rare Organic Cause of Refractory Constipation in Adults: A Case Report and Literature Review.Clinics and practice · 2026Article
- Altered enteric neurodevelopment in the Ncx knockout mouse model of intestinal neuronal dysplasia.Pediatric surgery international · 2025Article
- Clinicopathologic features of allied disorders of Hirschsprung disease and status update.World journal of gastrointestinal surgery · 2025Review
- Article
- Article
- Association between Clinical and Histopathological Findings in Intestinal Neuronal Dysplasia Type B: An Advance towards Its Definition as a Disease.Life (Basel, Switzerland) · 2023Article
- Challenges in the diagnosis of intestinal neuronal dysplasia type B: A look beyond the number of ganglion cells.World journal of gastroenterology · 2021Review
- Role of GDNF, GFRα1 and GFAP in aFrontiers in pediatrics · 2021Article
- Effect of Neuroligin1 and Neurexin1 on the Colonic Motility in a Mouse Model of Neuronal Intestinal Dysplasia.Gastroenterology research and practice · 2020Article
- Identification of potential molecular pathogenesis mechanisms modulated by microRNAs in patients with Intestinal Neuronal Dysplasia type B.Scientific reports · 2019Article
- Abnormal serum vitamin A levels and retinoic acid receptor α expression patterns in children with anorectal malformation.Pediatric surgery international · 2019Article
- Observational
- [Chronic Intestinal Pseudo-Obstruction Associated with Type A Intestinal Neuronal Dysplasia].Acta gastroenterologica LatinoamericanaArticle
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5 authors at 1 institution in 1 country.
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Abstract
Intestinal neuronal dysplasia type B is a controversial entity expressed by complex changes in the enteric nervous system. Diagnosis depends on rectal biopsy histopathology and diagnostic criteria, both qualitative and quantitative, have changed over time, hindering the diagnostic practice. We analyzed the morphological criteria for the histological diagnosis of intestinal neuronal dysplasia type B in a series of patients with intestinal neuronal dysplasia type B according to the 1990 Frankfurt Consensus criteria and verified the applicability of the numerical criteria proposed by Meier-Ruge et al in 2004 and 2006. Qualitative criteria adopted for the histological diagnosis of intestinal neuronal dysplasia type B included hyperplasia of the submucous plexus with hyperganglionosis and hypertrophy of the nerve trunks. Quantitative criteria considered more than 20% giant ganglia in the submucosa, with more than eight neurons each on 25 ganglia, and children aged over 1 year. Distal colon surgical specimens from 29 patients, aged 0-16 years, diagnosed with intestinal neuronal dysplasia type B were retrospectively analyzed using sections processed for conventional histology (H&E) and calretinin immunohistochemistry. Hyperplasia of the submucosal nerve plexi with hyperganglionosis and hypertrophy of the nerve trunks was observed in all cases. Ganglia with small, immature neurons were detected in the majority of cases. Quantitative analysis confirmed hyperganglionosis (mean number=10.7 neurons per ganglion) and hypertrophy of the nerve trunks (median=44.6 μm thickness). Neurons showed immunostaining for calretinin, but neuron counts in calretinin-stained sections were lower compared with H&E (P<0.01). No significant differences were verified between children aged under and over 1 year regarding hyperganglionosis (P=0.79), neuron counts (P=0.36), and immature ganglia (P=0.66). Only one patient met the numerical criteria proposed by Meier-Ruge et al in 2004 and 2006. In conclusion, the numerical criteria showed limited applicability when transposed to conventional histopathology. Children aged over 1 year presented very similar histological features of neuronal immaturity to younger children, questioning the need for an age criterion when diagnosing intestinal neuronal dysplasia type B.
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