Bilateral Extracranial Carotid Artery Aneurysms: A Rare Complication of Marfan Syndrome

Marfan Syndrome (MFS) is an autosomal dominant connective tissue disorder caused by a mutation in the FBN1 gene that encodes for the extracellular matrix protein fibrillin-1. The clinical manifestations of MFS which lead to morbidity and mortality are primarily those involving aortic disease, such a...

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Main Authors: Lee Mendenhall, Karl G. Shaver, Lynne J. Goebel
Format: Article
Language:English
Published: Marshall University 2020-01-01
Series:Marshall Journal of Medicine
Subjects:
Online Access:https://mds.marshall.edu/cgi/viewcontent.cgi?article=1241&context=mjm
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author Lee Mendenhall
Karl G. Shaver
Lynne J. Goebel
author_facet Lee Mendenhall
Karl G. Shaver
Lynne J. Goebel
author_sort Lee Mendenhall
collection DOAJ
description Marfan Syndrome (MFS) is an autosomal dominant connective tissue disorder caused by a mutation in the FBN1 gene that encodes for the extracellular matrix protein fibrillin-1. The clinical manifestations of MFS which lead to morbidity and mortality are primarily those involving aortic disease, such as aneurysmal dilation, aortic regurgitation, and aortic dissection. Despite 60-80% of adults with MFS having some degree of aortic disease, carotid artery aneurysms are rare and usually represent extensions of aortic dissections.1 We report a 70-year-old female with a history of ectopia lentis and family history of MFS who presented to the ED with epistaxis and anemia. An arteriogram revealed an incidental finding of bilateral carotid artery aneurysms a rare phenomenon even in the presence of connective tissue disease. Review of the literature reveals very little data on the clinical presentation, treatment, and outcome of MFS patients with extracranial carotid artery aneurysms (ECAAs). We discuss presentation, etiology, and management options reported for these rare occurrences.
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spelling doaj.art-89de23a2f56c4b8e83780ea36db348092022-12-21T21:17:38ZengMarshall UniversityMarshall Journal of Medicine2379-95362020-01-01615810.33470/2379-9536.1241Bilateral Extracranial Carotid Artery Aneurysms: A Rare Complication of Marfan SyndromeLee Mendenhall0Karl G. Shaver1Lynne J. Goebel 2Marshall UniversityOhio State UniversityMarshall UniversityMarfan Syndrome (MFS) is an autosomal dominant connective tissue disorder caused by a mutation in the FBN1 gene that encodes for the extracellular matrix protein fibrillin-1. The clinical manifestations of MFS which lead to morbidity and mortality are primarily those involving aortic disease, such as aneurysmal dilation, aortic regurgitation, and aortic dissection. Despite 60-80% of adults with MFS having some degree of aortic disease, carotid artery aneurysms are rare and usually represent extensions of aortic dissections.1 We report a 70-year-old female with a history of ectopia lentis and family history of MFS who presented to the ED with epistaxis and anemia. An arteriogram revealed an incidental finding of bilateral carotid artery aneurysms a rare phenomenon even in the presence of connective tissue disease. Review of the literature reveals very little data on the clinical presentation, treatment, and outcome of MFS patients with extracranial carotid artery aneurysms (ECAAs). We discuss presentation, etiology, and management options reported for these rare occurrences.https://mds.marshall.edu/cgi/viewcontent.cgi?article=1241&context=mjmmarfan syndromecarotid aneurysmextracranial carotid artery aneurysm
spellingShingle Lee Mendenhall
Karl G. Shaver
Lynne J. Goebel
Bilateral Extracranial Carotid Artery Aneurysms: A Rare Complication of Marfan Syndrome
Marshall Journal of Medicine
marfan syndrome
carotid aneurysm
extracranial carotid artery aneurysm
title Bilateral Extracranial Carotid Artery Aneurysms: A Rare Complication of Marfan Syndrome
title_full Bilateral Extracranial Carotid Artery Aneurysms: A Rare Complication of Marfan Syndrome
title_fullStr Bilateral Extracranial Carotid Artery Aneurysms: A Rare Complication of Marfan Syndrome
title_full_unstemmed Bilateral Extracranial Carotid Artery Aneurysms: A Rare Complication of Marfan Syndrome
title_short Bilateral Extracranial Carotid Artery Aneurysms: A Rare Complication of Marfan Syndrome
title_sort bilateral extracranial carotid artery aneurysms a rare complication of marfan syndrome
topic marfan syndrome
carotid aneurysm
extracranial carotid artery aneurysm
url https://mds.marshall.edu/cgi/viewcontent.cgi?article=1241&context=mjm
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AT karlgshaver bilateralextracranialcarotidarteryaneurysmsararecomplicationofmarfansyndrome
AT lynnejgoebel bilateralextracranialcarotidarteryaneurysmsararecomplicationofmarfansyndrome