Almost half of the patients present in infancy, and 40% of
them also have intestinal malrotation.13,15 If
symptoms persist after surgery for malrotation, the child should be
investigated for mechanical obstruction, and if absent, a diagnosis
of chronic intestinal pseudo-obstruction syndrome is considered.
Common symptoms include nausea, vomiting, abdominal distension,
constipation, abdominal pain, and failure to thrive. Diarrhea due
to small bowel bacterial overgrowth and bile acid malabsorption
is also common. Apart from the GI tract, urinary tract and bladder
involvement can result in megacystis and megaureter. In most children
with congenital disease, the clinical course has an illness plateau
with intermittent increases in acuity. Triggers for decompensation
include viral or bacterial infections, central line sepsis, general
anesthesia, psychological stress, and malnutrition.