title: [Surgical management of a dissecting aneurysm of the thoracic aorta in a patient with Marfan's syndrome - case report].
abstract: Marfan's Syndrome is a rare connective tissue disease whose surgical treatment presents several challenges. The existing criteria and recommendations on how and when to surgically approach this disease is almost exclusively confined to the root and ascending aorta, in contrast to the variability of indications on the involvement of the remaining segments of the thoracic and abdominal aorta, due to the multiplicity, morphology and topography of these involvements. This case report is related to a 39 year old woman with Marfan's syndrome, with a type B chronic dissection and a segmentar aneurysm of the proximal descending thoracic aorta with 6,1cm of maximum diameter. The clinical and imagiologic evaluation pointed out the absence of any disorder of the heart, root or ascending aorta. The patient underwent surgical management of the dissecting aneurysm through a transthoracic approach to the aneurysm, with fenestration of the distal aorta and exclusion of the aneurism and intercostal arteries. The efficacy and result of the procedure in addition to the rarity of the vascular pathology presented by the patient, even in the context of a Marfan, completely justify the presentation and disclosure of this clinical case.
authors.affiliation: Clínica Universitária de Cirurgia Vascular do Hospital de Santa Maria, Lisboa, Portugal.
authors.forename: A
authors.initials: A
authors.lastname: Dinis Da Gama
authors.forename: João Luís
authors.initials: JL
authors.lastname: Marcelino
authors.forename: Angélica
authors.initials: A
authors.lastname: Damião
authors.forename: Ana
authors.initials: A
authors.lastname: Evangelista
authors.forename: Augusto
authors.initials: A
authors.lastname: Ministro
mesh_terms: D000328:Adult; D000784:Aortic Dissection / Q000209:etiology / Q000235:genetics / Q000601:surgery*; D017545:Aortic Aneurysm, Thoracic / Q000209:etiology / Q000235:genetics / Q000601:surgery*; D005260:Female; D006801:Humans; D008382:Marfan Syndrome / Q000150:complications / Q000235:genetics; D010375:Pedigree