Marfan Sendrom'lu Kız Kardeşlerde Torasik ve Torakoabdominal Anevrizma
Yusuf KALKO1, Vural ÖZCAN1, Ufuk ALPAGUT1, Faruk HÖKENEK1, Murat BAŞARAN1, Emin TİRELİ1, Enver DAYIOĞLU1, Aydın KARGI1İstanbul Üniversitesi İstanbul Tıp Fakültesi Kalp damar Cerrahisi Anabilim Dalı, İstanbulFamilya/ aort anevrizma ve disseksiyon/armda ilk operasyondan sonra anasromo: bölgesinde ve/veya daha distalde yeni anevriznıa ve eliseksiyon oluşumu normal popu/asyona oranla daha st kur. Anabilim Dalwuzda son 6 ytlda akw aorr disseksiyonu nedeni ile cerrahi müdahale yapilan 25 olgudan 6'st, roraka/ ve rorakoabdominal anevrizma sebebi ile opere olan 17 vakadan ise 4'ü MatJan sendromlu idi. Bu hasralardan ikisi kt:kardeşri. İlk olgumu: infrarenal abdomina/ aort anevrizmast sebebi ile başka merkezde opere oldukran 2 ytl sonra kliniğimi:de Crawford Tip IV rorakoabdomina/ anevrizma ramst ile yeniden opere edildi. Operasyon basit klemp tekniği ve ototransfüzyon kullamlarak yaptld1. İkinci olgumuz kronik tip lll dissekan aorr anevrizmast nedeni ile opere edildi. Hastaya parsiyel femoro-femoral km·diyopulmoner "by-pass" distal pet/fizyon yöntemi kul/antlarak torasik aort replasmam yaptld1. Operasyon sonrast erken ve geç dönemde nörolojik defisit ve visseral organ malpetfüzyonu gözlenmedi. İlk olgumuz 2. operasyondan yaklaştk 18 ay so1tra torasik sakkiiler anevrizma riiptiirii ile perioperatif dönemde kaybedildi. Rekürren aorr anevrizmalanmn bu hasta grubunda daha stk oluşmast klinik takibin önemini göstermektedir.
Anahtar Kelimeler: Marfan sendromu, familyal torasik anevrizmaThoracic and Thoracoabdominal Aneurysm in Sisters with Marfan Syndrome: A Case Report
Yusuf KALKO1, Vural ÖZCAN1, Ufuk ALPAGUT1, Faruk HÖKENEK1, Murat BAŞARAN1, Emin TİRELİ1, Enver DAYIOĞLU1, Aydın KARGI1In 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.
Keywords: Marfan syndrome, familial thoracic aneurysm| ARAÇLAR |
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