Chronic primary intestinal pseudo-obstruction

Chronic primary intestinal pseudo-obstruction (CPIP) has received attention despite of its unclear etiology and infrequent occurrence. Recently a patient with this disorder had evidence of a primary visceral neuropathy. Reviewing the literature, we found 30 case reports of CPIP and evaluated their clinicopathologic findings. Presenting symptoms and radiologic findings were nonspecific. Esophageal motility was abnormal in 12 of 14 reports. Intestinal histopathology revealed normal muscle wall, mucosa, and ganglion cells in over 50% of reports. Only 48% of cases demonstrated clinical improvement. Thirty percent (8 of 30) ultimately died. We conclude that CPIP is a perplexing, often fatal entity that can mimic mechanical obstruction in the absence of definite etiology. Primary neurologic or muscular disease may be a possible explanation, but, as yet, definite documentation does not exist.