Submucosal hypoganglionosis causing chronic idiopathic intestinal pseudo-obstruction.
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.
Von Boyen
Georg B T
GB
Department of Medicine II (Gastroenterology), University Hospital of Heidelberg at Mannheim, Germany. georg.boyen@medizin.uni-ulm.de
Von der Ohe
Manfred
M
Krammer
Heinz-Jürgen
HJ
Singer
Manfred V
MV
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*