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Intestinal pseudo-obstruction syndrome in a child with myotonic dystrophy
J F Bruinenberg1, P N Rieu, F M Gabreëls
1Department of Pediatrics, University Hospital Nijmegen, Netherlands.
Insights
Intestinal pseudo-obstruction can be an early sign of myotonic dystrophy in children. Conservative treatment is recommended for these gastrointestinal complications in pediatric myotonic dystrophy patients.
Area of Science:
- Pediatric Gastroenterology
- Neuromuscular Disorders
Background:
- Gastrointestinal symptoms are common in myotonic dystrophy.
- Intestinal pseudo-obstruction is a rare but serious complication.
Abstract:
Gastrointestinal symptoms in myotonic dystrophy are increasingly observed, but major intestinal movement disorders such as intestinal pseudo-obstruction appear to be an infrequent complication. We describe a 13-year-old boy who, after appendectomy, developed intestinal pseudo-obstruction syndrome as the first clinical manifestation of myotonic dystrophy. He developed several similar episodes thereafter, which responded to conservative measures. When a child with myotonic dystrophy presents with an ileus, the diagnosis of intestinal pseudo-obstruction should be considered and therapy should then be conservative.