Severe constipation with diffuse intestinal myenteric hyperganglionosis

J Pediatr Surg. 1993 Dec;28(12):1630-2. doi: 10.1016/0022-3468(93)90124-4.


The authors report a case of neuronal intestinal dysplasia in a 6-year-old girl. The disease is characterized by hyperplastic ganglia throughout the large and small intestine, associated with severe constipation. To better understand the pathophysiology of this disease the authors investigated the histopathologic, ultrastructural, and immunohistochemical characteristics of the intestinal tissue in this case. The hyperganglionosis was associated with immunohistochemical findings of intact expression of the neuropeptides controlling the peristaltic reflex, through lower expression of calcitonin-gene related peptide. With the recent progress in our understanding of the neural regulation of gastrointestinal function, it may now be possible to begin to understand the complex pathophysiological mechanisms underlying gastrointestinal motility disorders.

Publication types

  • Case Reports

MeSH terms

  • Calcitonin Gene-Related Peptide / analysis
  • Child
  • Colon / innervation*
  • Constipation / etiology*
  • Female
  • Gastrointestinal Motility / physiology
  • Humans
  • Hyperplasia / pathology
  • Intestinal Diseases / complications*
  • Myenteric Plexus / chemistry
  • Myenteric Plexus / pathology*
  • Submucous Plexus / chemistry
  • Submucous Plexus / pathology*
  • Substance P / analysis
  • Vasoactive Intestinal Peptide / analysis


  • Substance P
  • Vasoactive Intestinal Peptide
  • Calcitonin Gene-Related Peptide