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Malrotation; the intestines (or viscera) are twisted


        For parents, caregivers, health care providers, and teachers, concerns and questions often arise regarding the care and well-being of individuals with Cornelia de Lange Syndrome. 

A small number of individuals with CdLS have been reported to have a sudden malformation due to rotation of the intestine (intestinal malrotation). 

Malrotation means that the intestines (or viscera) are twisted, which can cause constipation. Intestinal malrotation can occur during infancy, childhood or adolescence. 


Recommendation(s)

Malrotation; the intestines (or viscera) are twisted

R29
Presentation of any abdominal symptoms in an individual with CdLS, irrespective of age, should prompt consideration of intestinal malrotation.
R30
Evaluation for the presence of intestinal malrotation needs to be discussed and decided together with the family, balancing the potential gain in health and burden for the individual with CdLS.

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