title: [Intestinal pseudo-obstruction due to sporadic visceral myopathy].
abstract: We report an unusual case of a patient with sporadic visceral myopathy and involvement of the entire gastrointestinal and urinary tract. Visceral myopathy is a form of chronic idiophatic intestinal pseudo-obstruction characterized by vacuolar degeneration, atrophy and fibrosis of the intestinal propia muscle layer without inflammatory cells. It can be found in childhood and adolescence affecting the gastrointestinal and urinary visceral muscle. The familial occurrence can be found in about 30% of cases and the mode of transmission is autosomal recessive in most families. It is crucial to exclude secondary forms of chronic intestinal pseudo-obstruction and to obtain full thickness intestinal biopsy for the diagnosis. Surgical treatment is only beneficial in cases with isolated segmental involvement of the gastrointestinal tract.
authors.affiliation: Servicio de Clínica Médica, Hospital Zonal General de Agudos San Roque, Gonnet, La Plata, Argentina. pemontalvo@msn.com
authors.forename: Pedro
authors.initials: P
authors.lastname: Montalvo
authors.forename: Liliana
authors.initials: L
authors.lastname: Paz
authors.forename: Elsa
authors.initials: E
authors.lastname: Chiappa
authors.forename: Sandra
authors.initials: S
authors.lastname: Aronne
authors.forename: María
authors.initials: M
authors.lastname: Novelli
authors.forename: María Elena
authors.initials: ME
authors.lastname: Biaiñ
mesh_terms: D000328:Adult; D004108:Dilatation, Pathologic / Q000175:diagnosis / Q000209:etiology; D005260:Female; D006801:Humans; D007418:Intestinal Pseudo-Obstruction / Q000175:diagnosis / Q000209:etiology*; D007421:Intestine, Small / Q000473:pathology; D009135:Muscular Diseases / Q000150:complications* / Q000175:diagnosis; D014515:Ureteral Diseases / Q000473:pathology; D014570:Urologic Diseases / Q000175:diagnosis / Q000209:etiology