title: Submucosal hypoganglionosis causing chronic idiopathic intestinal pseudo-obstruction.
abstract: A 39-year-old woman presented with recurrent symptoms suggestive of intestinal obstruction. She was put on total parenteral nutrition (TPN) and consequently developed sepsis and endocarditis. TPN was stopped and a venting enterostomy was performed. Biopsies of mucosa and submucosa were taken at surgery; immunohistochemistry for neuronal proteins, protein gene product 9.5 (PGP 9.5) and the glial S-100-protein was done. Many enlarged nerve fiber strands were found in the submucosa. Few small ganglia containing a small number of nerve cells could be observed, suggesting hypoganglionosis. This patient with chronic idiopathic intestinal pseudoobstruction of neurogenic type had a defect in the submucous plexus, whereas visceral neuropathies are usually characterized by defects of the myenteric plexus with normal submucous plexus.
authors.affiliation: Department of Medicine II (Gastroenterology), University Hospital of Heidelberg at Mannheim, Germany. georg.boyen@medizin.uni-ulm.de
authors.forename: Georg B T
authors.initials: GB
authors.lastname: Von Boyen
authors.forename: Manfred
authors.initials: M
authors.lastname: Von der Ohe
authors.forename: Heinz-Jürgen
authors.initials: HJ
authors.lastname: Krammer
authors.forename: Manfred V
authors.initials: MV
authors.lastname: Singer
mesh_terms: D000328:Adult; D002908:Chronic Disease; D005260:Female; D006627:Hirschsprung Disease / Q000150:complications*; D006801:Humans; D007082:Ileum / Q000473:pathology; D007418:Intestinal Pseudo-Obstruction / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D013368:Submucous Plexus / Q000002:abnormalities*