Primary and Chronic Intestinal Pseudo Obstruction in a Patient with Probable Ovarian Agenesis

The patient is a 12 year-old girl who has been suffering from episodes of in testinal obstruction since she was 6 years old, leading to severe malnutrition, malabsorption and growth retardation.  Repeated radiological examinations revealed esophageal achalasia, gastric distention, marked small intestinal and mild colonic dilatation. Transmural biopsies from small and large bowels showed normal and mucosa! and muscular layers containing normal neural plexus and ganglionic cells. In laparotomy the ovaries were absent and the uterus was atrophic. Mechanical obstruction, paralytic ileus and metabolic disorders were ruled out by a complete clinical and laboratory evaluations and the diagnosis of a primary and chronic intestinal pseudo-obstruction was made. Whether there is any relation between the intestinal pseudo-obstruction and ovarian agenesis is to be clarified.