FAMILIAL OCCURRENCE OF AN ARTERIOVENOUS-FISTULA WITH A GIANT PERIMEDULLARY PSEUDOANEURYSM OF THE THORACIC SPINAL-CORD IN 2 YOUNG SIBLINGS

Two siblings, a sister at age 3 years and a brother (l5 years later) a t 4 fears of age, both presented viith similar clinical pictures consi sting of back pain, progressive gait difficulty, lower extremity weakn ess and hyperreflexia, Imaging studies in both cases showed the presen ce of a perimedullary mass with expansion of the bony spinal canal at T-10-T-12 Angiography demonstrated almost identical vascular lesions t hat in each case was being fed by a single left-sided Tg fislulous ves sel that expanded into a giant perimedullary venous pseudoaneurysm. Ea ch malformation was successfully obliterated using endovascular techni ques, balloon occlusion of the feeding vessel in the first case and co il embolization of the pseudoaneurysm in the second. Following treatme nt the neurologic deficits resolved in both patients. These young sibl ings represent the first known case of familial occurrence of very sim ilar giant perimedullary venous pseudoaneurysms of the thoracic spinal cord.