Intestinal pseudoobstruction as a feature of myotonic muscular dystrophy

Z Gastroenterol. 1995 Sep;33(9):534-8.


We report two cases of intestinal pseudoobstruction caused by visceral smooth muscle involvement due to myotonic muscular dystrophy. Two patients with myotonic muscular dystrophy presented with abdominal pain, distention, constipation, and vomiting. The exclusion of mechanical obstruction by plain abdominal radiography, contrast studies, and colonoscopy led to the diagnosis of intestinal pseudoobstruction. Diagnosis was confirmed by manometric and cineradiographic findings of abnormal intestinal motility. Conservative management including laxatives and cisapride led to the resolution of the pseudoobstruction syndrome and long-term remission without relapses during a two year follow-up. In patients with known myotonic dystrophy the occurrence of intestinal pseudoobstruction should be considered in order to avoid unnecessary laparotomies.

Publication types

  • Case Reports

MeSH terms

  • Cathartics / administration & dosage
  • Cisapride
  • Female
  • Follow-Up Studies
  • Humans
  • Intestinal Pseudo-Obstruction / diagnostic imaging
  • Intestinal Pseudo-Obstruction / drug therapy
  • Intestinal Pseudo-Obstruction / physiopathology*
  • Male
  • Manometry
  • Middle Aged
  • Muscle, Smooth / diagnostic imaging
  • Muscle, Smooth / drug effects
  • Muscle, Smooth / physiopathology
  • Myotonic Dystrophy / diagnostic imaging
  • Myotonic Dystrophy / drug therapy
  • Myotonic Dystrophy / physiopathology*
  • Piperidines / administration & dosage
  • Radiography
  • Sympathomimetics / administration & dosage


  • Cathartics
  • Piperidines
  • Sympathomimetics
  • Cisapride