title: Severe idiopathic gastroparesis due to neuronal and interstitial cells of Cajal degeneration: pathological findings and management.
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.
authors.affiliation: Digestive System Research Unit, University Hospital Vall d'Hebron, Autonomous University of Barcelona, 08035-Barcelona, Spain.
authors.forename: N
authors.initials: N
authors.lastname: Zárate
authors.forename: F
authors.initials: F
authors.lastname: Mearin
authors.forename: X-Y
authors.initials: XY
authors.lastname: Wang
authors.forename: B
authors.initials: B
authors.lastname: Hewlett
authors.forename: J D
authors.initials: JD
authors.lastname: Huizinga
authors.forename: J-R
authors.initials: JR
authors.lastname: Malagelada
mesh_terms: D000328:Adult; D005260:Female; D005724:Ganglia / Q000473:pathology; D005746:Gastric Emptying; D018589:Gastroparesis / Q000209:etiology / Q000473:pathology* / Q000628:therapy; D006801:Humans; D009046:Motor Neurons / Q000502:physiology; D009419:Nerve Tissue Proteins / Q000378:metabolism; D013270:Stomach / Q000473:pathology; D013869:Thiolester Hydrolases / Q000378:metabolism; D043222:Ubiquitin Thiolesterase
chemical_list: D009419:Nerve Tissue Proteins; D013869:Thiolester Hydrolases; D043222:Ubiquitin Thiolesterase