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Neuronal intestinal dysplasia.

R Rintala1, J Rapola, I Louhimo

  • 1Children's Hospital, University of Helsinki, Finland.

Progress in Pediatric Surgery
|January 1, 1989
PubMed
Summary
This summary is machine-generated.

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Neuroenteric disease (NID) presents heterogeneously in patients, often associated with gastrointestinal issues like obstruction or perforation. Its varied presentation suggests NID may be a reactive process rather than a distinct entity.

Area of Science:

  • Gastroenterology
  • Pediatric Surgery
  • Pathology

Background:

  • Neuroenteric disease (NID) is a condition affecting the bowel's neural network.
  • Its clinical presentation and histological findings can be diverse.
  • NID has been observed in various pediatric and adult gastrointestinal conditions.

Purpose of the Study:

  • To present a series of patients with NID.
  • To analyze the histologic and histochemical characteristics of NID.
  • To explore the potential causes and clinical significance of NID.

Main Methods:

  • Histologic and histochemical examination of bowel biopsies from 21 patients with NID.
  • Clinical data review including patient history, associated conditions, and follow-up.

Related Experiment Videos

  • Correlation of clinical presentation with pathological findings.

    • Main Results:

    • NID was observed in a heterogeneous group of patients, including neonates and infants with bowel obstruction/perforation (one fatality).
    • NID was incidentally found in patients with anorectal malformations and Hirschsprung's disease, some with chronic proctitis or obstipation.
    • NID was also identified in children with multiple endocrine neoplasia 2b syndrome and an adult with chronic idiopathic intestinal pseudo-obstruction (CIIP).

    Conclusions:

    • The clinical heterogeneity of NID suggests it may not be a distinct entity.
    • NID could represent a reactive process of the bowel's neuronal network.
    • Both congenital and secondary factors may contribute to the development of NID.