A Young Woman With Chronic Intestinal Pseudo-obstruction Since Birth

Question: A 24-year-old Caucasian woman was admitted to our hospital for constipation and urinary catheterization for urinary retention and dilated bladder since birth. Left hemicolectomy with transanal pull-through was performed at 1 year of age for megacolon. Hirschsprung’s disease was excluded at histological examination of rectal suction biopsies performed before surgery, and the diagnosis of intestinal neuronal dysplasia (IND) was made according to the presence of ganglion cells hyperplasia in the submucosal layer and in the myenteric plexus of the resected colon. A detrusor sphincter–dyssynergia was diagnosed with a cystography and urodynamic test at infancy. Family history was negative for gastrointestinal diseases.