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

Abstract

Gut 2003;52:966-970 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. U nderstanding of both normal and abnormal gastrointestinal function has gained momentum in recent decades. However, gastric motility and, in particular, the relative contribution of all of the factors involved in the coordination of food propulsion are still poorly understood. Gastric emptying reflects the integration of tonic contractions of the proximal stomach (fundic tone), phasic contractions of the antrum, and the inhibitory forces of pyloric and duodenal contractions. These complex phenomena require cooperation between smooth muscle, enteric and autonomic nerves, and interstitial cells of Cajal (ICC).

663 Association of Low Numbers of CD206-Positive Cells With Loss of ICC in the Gastric Body of Patients With Diabetic Gastroparesis

“…We found, using a different cohort of patients from the previously reported study from the gastroparesis clinical research consortium, 13 that there was a decrease in ICC numbers in the circular muscle of gastroparetic samples compared to controls. This is in agreement with a growing number of human studies that show similar levels of loss of ICC in both diabetic and idiopathic gastroparesis 14,[33][34][35][36][37][38][39][40] and a correlation between the degree of ICC loss and gastric emptying in diabetic gastroparesis. 15 The main finding of this study is that there was a significant correlation between number of ICC and CD206 numbers in diabetic gastric tissue and diabetic gastroparetic tissue.…”

Are interstitial cells of Cajal plurifunction cells in the gut?

“…A reduction in the volume of the ICC or damage to their process has been reported in almost every clinical motility disorder that has been investigated, including Hirschsprung's disease (89,90,173), chronic constipation (198), slow-transit constipation (7,67,189), megacolon (189), gastroparesis (199), diabetic mellitus (108), inflammatory bowel disease, animal models of inflammation (102,122,127), pyloric stenosis (92,172,195), achalasia (56,61,63), and chronic idiopathic intestinal obstruction (81,156). However, there are little or no data to correlate the abnormalities of specific subtypes of ICC with specific features of motility dysfunction or clinical symptoms.…”

Analysis of multiple factors of postsurgical gastroparesis syndrome after pancreaticoduodenectomy and cryotherapy for pancreatic cancer

“…Ordog et al [14] suggest that damage to interstitial cells of Cajal may play a key role in the pathogenesis of diabetic gastropathy. Meanwhile, Zarate et al [15] reported that histological and immunohistochemical study of the resected specimen showed hypoganglionosis, neuronal dysplasia, and marked reduction in both myenteric and intramuscular interstitial cells of Cajal in patients with idiopathic gastroparesis. Certainly, PGS after gastric surgery also can be due to muscular, neural, or humoral abnormalities.…”