PERFORATION OF THE COLON IN A 15-YEAR-OLD GIRL WITH EHLERS-DANLOS SYNDROME TYPE-IV

A 15-year-old girl presented with severe fecal peritonitis due to a large spontaneous colonic perforation. The sigmoid colon was the site of a cluster of white serosal lesions with omental adhesions, of an appearance identical to that of the edges of the perforation. Her father had died at 30 years of age of spontaneous rupture of an iliac artery aneurysm, preceded by rupture of a splenic artery aneurysm and a spontaneous carotid-cavernous fistula. The clinical diagnosis of Ehlers-Danlos syndrome type IV was made, and confirmed by demonstrating that the patient's cultured fibroblasts are not producing or secreting type III collagen. Spontaneous perforation of the colon is a well-described complication of this syndrome, with a high incidence of recurrence. We recommend total abdominal colectomy to minimize the latter possibility. © 1990.