title: Surgical treatment of megaduodenum in familial visceral myopathy - report of a case and review of the literature.
abstract: BACKGROUND
  Familial visceral myopathy (VM) is a rare genetic disease that affects intestinal motility and results in pseudo-obstruction. Medical treatments can provide supportive measures but no curative treatment.
  
  
  METHODS
  A 20-year-old male with known diagnosis of VM was referred to our Unit in May 2013 with recurrent episodes of vomiting and hospital admissions not responding to medical treatment. Pre-operative imaging showed megaduodenum with marked delayed transit and normal small and large bowel transit. He underwent an elective Roux-en-Y duodeno-jejunostomy. The post-operative course was uneventful with complete resolution of the symptoms with a 2 years follow-up.
  
  
  CONCLUSIONS
  Due to the early age of presentation, VM affects patient both psychologically and physically. Surgical treatment of megaduodenum in visceral myopathy in the absence of motility disorder of the small bowel seems to achieve satisfactory symptomatic relief and could be considered in this rare cohort of patients.
authors.affiliation: a Hepato-Biliary and Pancreatic Unit , University Hospital Coventry and Warwickshire , Coventry , UK.
authors.forename: Davide
authors.initials: D
authors.lastname: Papis
authors.affiliation: a Hepato-Biliary and Pancreatic Unit , University Hospital Coventry and Warwickshire , Coventry , UK.
authors.forename: Gabriele
authors.initials: G
authors.lastname: Marangoni
mesh_terms: D000713:Anastomosis, Roux-en-Y; D004386:Duodenum / Q000002:abnormalities* / Q000601:surgery; D005315:Fetal Diseases / Q000175:diagnosis* / Q000601:surgery*; D005500:Follow-Up Studies; D006801:Humans; D007418:Intestinal Pseudo-Obstruction / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D007582:Jejunostomy / Q000379:methods; D008297:Male; D035583:Rare Diseases; D018570:Risk Assessment; D012720:Severity of Illness Index; D016896:Treatment Outcome; D001743:Urinary Bladder / Q000002:abnormalities* / Q000601:surgery; D055815:Young Adult
keywords: Duodeno-jejunostomy; familial visceral myopathy; megaduodenum