Four cases with chronic intestinal pseudo-obstruction due to hollow visceral myopathy.

BACKGROUND/AIMS: Chronic intestinal pseudo-obstruction is a rare clinical syndrome characterized by symptoms and signs of intestinal obstruction without any organic lesion obstructing the intestine. Visceral myopathy is one of the etiological causes and full thickness intestinal biopsy is essential for reaching a diagnosis. In this article we describe 4 cases of hollow visceral myopathy; our aim is to stress the importance of full thickness biopsy.

METHODOLOGY: Four cases of hollow visceral myopathy are studied herein. All the patients had recurrent abdominal pain and constipation. The onset of symptoms was early in life or in the second to third decade. A diagnosis was established in all cases by full thickness intestinal biopsy obtained during laparotomy. Associated disorders were noted in 2 cases. One patient had Axenfelt syndrome, non-descended testicles and primary hypogonadism, and another had a diagnosis of Kleinfelter syndrome.

RESULTS: All of the 4 cases were diagnosed to be suffering from hollow visceral myopathy by full thickness intestinal biopsy and 2 had additional disorders as well.

CONCLUSIONS: Patients with chronic intestinal pseudo-obstruction should be carefully evaluated as to whether there is an associated disorder and the diagnosis may be delayed unless full thickness intestinal biopsy is obtained.