Spontaneous carotid-cavernous fistula in osteogenesis imperfecta

Abstract

✓ A case of spontaneous carotid-cavernous fistula is presented in a patient suffering from osteogenesis imperfecta tarda type I. The patient was operated on under local anesthesia by intraluminal occlusion of the cavernous carotid artery with a Fogarty catheter, in accordance with Prolo's technique, with total remission except for homolateral loss of vision due to postglaucoma atrophy. The pathogenetic phenomena described in the literature are reviewed, with the suggestion that osteogenesis imperfecta be included with Ehlers-Danlos disease, Marfan's syndrome, and pseudoxanthoma elasticum, as a connective tissue disease with increased weakness of vessel walls that can produce a spontaneous carotid-cavernous fistula. Reports of cases operated on by intraluminal occlusion with various types of balloon-tipped catheters are also reviewed. The good results obtained place this technique among the elective ones for the treatment of carotid-cavernous fistula.