Thoracic and Thoracoabdominal Aneurysm in Sisters with Marfan Syndrome: A Case Report

In patients with a family history of Marfan syndrome, the ineidence of recurrent aneurysm and dissection that involves the previous anastomotic site and /or ınore distal segments are higher than normal population. In our institution, during the last 6 years, 10 of 42 patients who underwent a surgical intervention because of aortic dissection and /or thoracoabdoıninal aneurysm had the diagnosis of Marfan syndrome. Two of them were sisters. The first patient previously operated due to infrarenal aneurysm was referred to our institution with the diagnosis of Crawford type IV thoracoabdominal aneurysm. The operation was performed by s iınpl e clamp and using autotransfusion techniques. The second patient with chronic type III dissecting aneurysın underwent to thoracic aorta replacement. Operation was conducted under partial femorofemoral cardiopulmonary by-pass for distal perfusion. During the early and Iate follow-up, we did not notice any neurological deficit or visceral organ ınalperfus ion. The first patient was presented with a sacculer aortic aneurysın 18 ınonths after first operation. However, we los t the patient perioperatively because of the rupture.We believe that the high risk of rec urrent aneurysm in this population necessitates close follow-up after the operation.