Open AccessAcute Aortic Dissection and Medullary Ischemia in a Patient with Marfan syndrome
Author(s) -
Gustavo Góes,
Eugênio Soares de Albuquerque,
Diana Patricia Lamprea Sepulveda,
José María Castillo,
José Breno de Sousa Filho,
Ricardo de Carvalho Lima,
Dário Celestino Sobral Filho
Publication year - 2019
Publication title -
international journal of innovative research in medical science
Language(s) - English
Resource type - Journals
ISSN - 2455-8737
DOI - 10.23958/ijirms/vol04-i07/663
Subject(s) - medicine , paraplegia , aortic dissection , medullary cavity , marfan syndrome , ischemia , dissection (medical) , connective tissue , connective tissue disease , connective tissue disorder , aortic arch , aortic aneurysm , surgery , spinal cord , cardiology , disease , aorta , pathology , autoimmune disease , psychiatry
Marfan syndrome (MS) is an autosomal dominant inherited connective tissue disease. The systemic complications of this disorder occur due to osteo-articular, cardiovascular, and ophthalmologic alterations. The cardiovascular involvement of MS is characterized mainly by aortic root and arch changes, resulting in increased susceptibility for the development of aneurysms. In the present study, we describe a case of type B abdominal aortic dissection in a patient with MS followed by spinal cord ischemia that rapidly evolved to paraplegia. Although rare, medullary ischemia is associated with a poor prognosis, as noted in the present case, wherein the patient died.