SPONTANEOUS COLON PERFORATION AS THE FIRS T MANIFESTATION OF EHLERS-DANLOS SYNDROME TYPE-IV

Background, - The malignant form of Ehlers-Danlos syndrome type IV owe s its bad reputation to a proneness to spontaneous rupture of bowel or large vessels, which may reveal the disease. Case report, - A girl su ffered acute rupture of the sigmoid at the age of 5 years and rupture of the left colon, twice, at the age of 11 and 13 years, respectively. These ruptures required colostomy and finally colectomy. A proneness to bruisability, history of dislocation of hips, hypermobile joints, o varian cysts and some minor abnormalities of her face resembled that o f the Ehlers-Danlos syndrome which was confirmed by optic and electron ic microscopy of the skin biopsy. Conclusion, - This is the youngest c ase of rupture of bowel reported in Ehlers-Danlos syndrome. Long-term prognosis is influenced by repetition of intestinal ruptures and occur rence of vascular complications.