Chronic intestinal pseudo-obstruction as the initial feature of pure autonomic failure

Abstract

Chronic intestinal pseudo-obstruction (CIP) is a rare and highly morbid syndrome characterised by impaired gastrointestinal propulsion together with symptoms and signs of bowel obstruction in the absence of any lesions occluding the gut lumen (tumours, adhesive peritonitis, and so on).1 CIP is thought to have two forms: myogenic and neurogenic.1 Comorbid urinary retention may also occur.2 Postural hypotension is not a feature in CIP. However, we recently had such a CIP patient with profound postural hypotension, which was detected only by a head up tilt test, and he was finally diagnosed as pure autonomic failure (PAF). A 59 year old man gradually (over two months) developed intractable nausea and vomiting immediately after taking meals, although he did not have dysphagia. He also had abdominal distension, discomfort, and mild difficulty in defaecation. At that time, he had no features to suggest autonomic failure affecting other systems than the gut, such as postural dizziness or genitourinary dysfunction. Six months later, he was admitted to a gastrointestinal surgery hospital because of these symptoms, at which time he was emaciated. However, gastric and colonic endoscopy showed no organic lesions. As abdominal x ray findings continued to support the suspicion …