Abdominal pain, distension, vomiting, and constipation are frequent features and have led initially to confusion with distal intestinal obstruction syndrome.

Newborns with intestinal obstruction typically require surgery, whereas adults with distal intestinal obstruction syndrome typically do not.

Distal intestinal obstruction syndrome (DIOS) involves blockage of the intestines by thickened stool and occurs in individuals with cystic fibrosis.