Intestinal ganglioneuromatosis--a rare cause of chronic diarrhea

A case of intestinal ganglioneuromatosis is reported. The symptoms were watery diarrhoea and abdominal pain of several months duration. Endoscopic examination of the oesophagus, ventricle, duodenum, colon and rectum was normal. Mucosal biopsies from colon and rectum revealed ganglia cells and thin nerve fibres in the lamina mucosa, giving the diagnosis ganglioneuromatosis. As a consequence of the diagnosis thyroid scintigraphy, CT-scanning of the thyroid and adrenal glands and measurement of serum calcitonin and gastrin were performed. The tests revealed an intrathoracic nodular struma, and beyond this no abnormalities. The relation of intestinal ganglioneuromatosis to Multiple Endocrine Neoplasia type II b is discussed and the necessity of performing mucosal-biopsy from endoscopically normal colonic mucosa in cases of chronic diarrhoea is emphasised.