INTESTINAL PSEUDOOBSTRUCTION AS A FEATURE OF MYOTONIC MUSCULAR-DYSTROPHY

We report two cases of intestinal pseudoobstruction caused by visceral smooth muscle involvement due to myotonic muscular dystrophy. Two pat ients with myotonic muscular dystrophy presented with abdominal pain, distention, constipation, and vomiting. The exclusion of mechanical ob struction by plain abdominal radiography, contrast studies, and colono scopy led to the diagnosis of intestinal pseudoobstruction. Diagnosis was confirmed by manometric and cineradiographic findings of abnormal intestinal motility. Conservative management including laxatives and c isapride led to the resolution of the pseudoobstructive syndrome and l ongterm remission without relapses during a two year follow-up. In pat ients with known myotonic dystrophy the occurrence of intestinal pseud oobstruction should be considered in order to avoid unnecessary laparo tomies.