Fatal intestinal pseudo-obstruction in brown bowel syndrome.

A 52-year-old man, suffering for years from malabsorption due to endemic sprue, developed progressive bowel dysfunction, ie, recurrent ileus and intestinal pseudo-obstruction. Because of partial volvulus formation, ileocecal resection was performed. Histopathologic examination of the resected specimen revealed signs of advanced brown bowel syndrome, with excessive deposits of ceroid-lipofuscin in, and a considerable loss of, smooth-muscle cells and myofibrils. The patient died after surgery, and at autopsy a systemic ceroid lipofuscinosis of smooth-muscle cells was detected. Neuronal ceroid lipofuscinosis was not observed. Serious and eventually fatal bowel dysfunction is rarely seen in brown bowel syndrome, but may occur in advanced stages. Since treatment with vitamin E appears to exert a favorable effect, early diagnosis of brown bowel syndrome seems to be very important.