Hollow visceral myopathy in black South Africans. A report of 14 cases

Familial (hereditary) hollow visceral myopathy is a rare cause of chronic intestinal pseudo-obstruction. Fourteen black patients with the disease, 6 males and 8 females, are described. The earliest morphological lesion is an isolated smooth-muscle degeneration of the muscularis propria of the bowel, especially the colon. The pathological changes are lysis and disappearance of the muscularis propria of the bowel wall, leading to dilatation and intra-luminal stasis. progressive involvement results in large areas of defective muscularis propria. The occasional occurrence of mesenteric arteries showing medial muscular fibrosis supports the concept of a generalized primary smooth-muscle myopathy.