Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2003 Jul;52(7):966-70.
doi: 10.1136/gut.52.7.966.

Severe idiopathic gastroparesis due to neuronal and interstitial cells of Cajal degeneration: pathological findings and management

N Zárate  1 , F Mearin, X-Y Wang, B Hewlett, J D Huizinga, J-R Malagelada
Affiliations
Case Reports

Severe idiopathic gastroparesis due to neuronal and interstitial cells of Cajal degeneration: pathological findings and management

N Zárate et al. Gut. 2003 Jul.

Abstract

Delayed gastric emptying can be due to muscular, neural, or humoral abnormalities. In the absence of an identified cause, gastroparesis is labelled as idiopathic. We present the case of a patient with severe idiopathic gastroparesis. Pharmacological approaches failed, as well as reduction in gastric emptying resistance with pyloric injection of botulinum toxin and pyloroplasty. Therefore, subtotal gastrectomy was performed. Histological and immunohistochemical study of the resected specimen showed hypoganglionosis, neuronal dysplasia, and a marked reduction in both myenteric and intramuscular interstitial cells of Cajal. To our knowledge, this is the first time these rare histological findings have been described in a patient with idiopathic gastroparesis.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Gastric compliance (volume-pressure relationship) was significantly increased in the patient with idiopathic gastroparesis compared with healthy control values.
Figure 2
Figure 2
Kit immunoreactivity (IR) in the normal corpus (A) and corpus with gastroparesis (B). (A) Kit-IR is distributed throughout the muscle layers. Some kit-IR cells are concentrated at the myenteric plexus level (arrows). A fair amount of intramuscular kit-IR cells are seen in both muscle layers (arrowheads) running parallel to the muscle cells in which they are located. In (B), however, less kit-IR is found in the whole muscle layers except for a few scattered kit-IR cells (arrowheads).
Figure 3
Figure 3
Protein gene product (PGP) 9.5 immunoreactivity (IR) in the corpus of a control (A) and the patient’s (B) stomach. The total amount of nerve structures in (A) is greater than that in (B). Arrows show individual enteric neurones in the myenteric ganglion of control tissue. Arrowheads show PGP 9.5-IR nerve fibres in both circular and longitudinal muscle layers. Compared with control tissue, (B) shows a much weaker reaction in gastroparesis tissue. No nerve cell bodies are found in the myenteric ganglia (arrows). Very little reaction is displayed in muscle layers.
See this image and copyright information in PMC

References

    1. Huizinga JD. Neural injury, repair, and adaptation in the GI tract. IV. Pathophysiology of gastrointestinal motility related to interstitial cells of Cajal. Am J Physiol 1998;275:G381–6. - PubMed
    1. Parkman HP, Harris AD, Krevsky B, et al. Gastroduodenal motility and dysmotility: an update on techniques available for evaluation. Am J Gastroenterol 1995;90:869–92. - PubMed
    1. Vanderwinden JM, Rumessen JJ, Liu H, et al. Intertitial cells of Cajal in human colon and in Hirschsprung’s disease. Gastroenterology 1996;111:901–10. - PubMed
    1. Vanderwinden JM, Liu H, De Laet MH, et al. Study of the interstitial cells of Cajal in infantile hypertrophic pyloric stenosis. Gastroenterology 1996;111:279–88. - PubMed
    1. Isozaki K, Hirota S, Miyagawa J, et al. Deficiency of c-kit cells in patients with a myopathic form of chronic idiopathic intestinal pseudo-obstruction. Am J Gastroenterol 1997;92:332–4. - PubMed

Publication types