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Total Aorta Replacement by Five Times Operations Over 19 Years in Loeys-dietz Syndrome
김응래1, 김경환1, 오홍철1, 한정욱2, 김용한1, 이경학3
서울대학교 의과대학 서울대학교병원 흉부외과학교실1, 순천향대학교 서울병원 흉부외과학교실2, 강원대학교병원 흉부외과학교실3
Background : Loeys-Dietz syndrome is a rare autosomal dominant genetic disorder that affects connective tissue of the body, first described in 2005. It is characterized by e arterial tortuosity, aortic aneurysms and dissections, ocular hypertelorism, bifid uvula, and cleft palate. Patients with Loeys-Dietz syndrome may share some features with Marfan syndrome but tend to have more severely progressive aortic disease with an average age at death of around 26 years. We report a case of Loeys-Dietz syndrome in which the patient underwent whole aorta replacement through multiple operations.
Methods : He had multiple anomalies including cleft palate, bifid uvula, scaphocephaly, and camptodactyly, and was suspected to have a congenital disorder but was diagnosed after 2005. He had patent ductus arteriosus, atrial septal defect, and dilatation of both aortic and pulmonary annulus and underwent surgery for ligation of the patent ductus arteriosus through a left posterolateral thoracotomy at 16 month of age. However, during follow up, the patient had to undergo multiple operations because of the progressive aneurysmal change of the native aorta. 23 months after birth, he underwent aortic root remodeling due to aneurysmal change of the aortic root. At the age of 10, an aortic valve and ascending aorta replacement was performed. 5 years later, the patient developed an acute Stanford type B aortic dissection. Despite strict blood pressure control, a follow up computed tomography performed 2 weeks later showed progressive dilatation of abdominal aorta.
Results : He was consulted to us and we performed thoracoabdominal aorta replacement from proximal descending thoracic aorta to proximal to the renal arteries. During follow up, the remaining native aortic arch and abdominal aorta showed progressive dilatation. We planned staged operation, and he underwent an ascending and total arch replacement operation at the age of 19. However during hospital stay, the patient developed an aortic dissection with impending rupture at the lower abdominal aorta so, emergency operation was performed to replace the remaining native abdominal aorta from superior mesenteric to iliac arteries. Although a reoperation was needed due to bleeding, the patient could be discharged without serious complication.
Conclusion : Because of the aggressive feature of aortic involvement in Loeys-Dietz syndrome, close follow up and adequate surgery is important. Whole aorta follow up rather than specific part of aorta is imperative. We will present video especially about thoracoabdominal aorta and arch replacement including whole history of his aorta operations.
Methods : He had multiple anomalies including cleft palate, bifid uvula, scaphocephaly, and camptodactyly, and was suspected to have a congenital disorder but was diagnosed after 2005. He had patent ductus arteriosus, atrial septal defect, and dilatation of both aortic and pulmonary annulus and underwent surgery for ligation of the patent ductus arteriosus through a left posterolateral thoracotomy at 16 month of age. However, during follow up, the patient had to undergo multiple operations because of the progressive aneurysmal change of the native aorta. 23 months after birth, he underwent aortic root remodeling due to aneurysmal change of the aortic root. At the age of 10, an aortic valve and ascending aorta replacement was performed. 5 years later, the patient developed an acute Stanford type B aortic dissection. Despite strict blood pressure control, a follow up computed tomography performed 2 weeks later showed progressive dilatation of abdominal aorta.
Results : He was consulted to us and we performed thoracoabdominal aorta replacement from proximal descending thoracic aorta to proximal to the renal arteries. During follow up, the remaining native aortic arch and abdominal aorta showed progressive dilatation. We planned staged operation, and he underwent an ascending and total arch replacement operation at the age of 19. However during hospital stay, the patient developed an aortic dissection with impending rupture at the lower abdominal aorta so, emergency operation was performed to replace the remaining native abdominal aorta from superior mesenteric to iliac arteries. Although a reoperation was needed due to bleeding, the patient could be discharged without serious complication.
Conclusion : Because of the aggressive feature of aortic involvement in Loeys-Dietz syndrome, close follow up and adequate surgery is important. Whole aorta follow up rather than specific part of aorta is imperative. We will present video especially about thoracoabdominal aorta and arch replacement including whole history of his aorta operations.
책임저자: 김경환
서울대학교 의과대학 서울대학교병원 흉부외과학교실
연락처 : 김응래, Tel: 02-2072-2348 , E-mail : xion119@naver.com
