Spontaneous free perforation of the small intestine in Crohn's disease.

Spontaneous free perforation of the small intestine is a rare but often dramatic event in the clinical course of Crohn's disease. Fifteen new cases of spontaneous free perforation of the small intestine--nine female patients and six male patients--were discovered in a series of 1000 consecutively evaluated patients with Crohn's disease seen during a period spanning 20 years, for an estimated frequency of 1.5%. Spontaneous free perforation was the presenting clinical feature of Crohn's disease in nine (60%) of the newly discovered cases. Most perforations were located in the ileum rather than in the jejunum, and there were no duodenal free perforations. One patient with extensive intestinal disease presented with concomitant free perforations of the jejunum and ileum, while a second patient had two free ileal perforations that developed independently, separated by about six years. No perforations were the result of a superimposed malignant process, ie, adenocarcinoma or lymphoma. There have been no mortalities, and the subsequent clinical course of these patients has been limited to a minority requiring corticosteroid or immunosuppressive medications, or further surgical resections.