Carotid artery tortuosity is associated with connective tissue diseases

Research output: Contribution to journalArticle

Abstract

BACKGROUND AND PURPOSE: There is a general assumption in the cerebrovascular literature that there is an association between carotid artery tortuosity and connective tissues disease; however, this has not been firmly established. The purpose of this study was to determine the prevalence of carotid artery tortuosity in patients with connective tissue diseases relative to matched controls. MATERIALS AND METHODS: Patients with previous CTA or MRA and a diagnosis of connective tissue diseases were identified and compared with a cohort of age-matched controls. Radiologists blinded to the diagnosis reviewed the images and evaluated the presence of carotid artery tortuosity (including loops, kinks, or coils). Continuous variables were compared using the Student t test, and categoric variables with x 2 tests. RESULTS: One hundred forty-three patients with connective tissue disease and 143 controls were included in this study. Specific diagnoses included Marfan (n = 33), nonvascular Ehlers-Danlos (n = 36), Ehlers-Danlos vascular-type (n = 32), neurofibromatosis type 1 (n = 26), and Loeys-Dietz (n = 16) syndromes. The presence of carotid tortuosity was 44% in connective tissue disease and 16% in controls (P, .001). Of tortuosity manifestations, coils were most prevalent (23% versus 3%; P, .001). Among the various connective tissue diseases, the rates of any carotid tortuosity were 88% for Marfan syndrome, 63% for Loeys-Dietz syndrome, 42% for neurofibromatosis type 1, and 19% for both vascular- and nonvascular-type Ehlers-Danlos syndrome. The positive predictive value of the combination of aortic aneurysm and carotid tortuosity being associated with connective tissue disease was 95.4%. The specificity was 98.6%. CONCLUSIONS: Carotid artery tortuosity is highly associated with connective tissue diseases, particularly Marfan syndrome, Loeys-Dietz syndrome, and neurofibromatosis type 1. Such findings are relevant in risk assessment for vascular complications in connective tissue disease, endovascular treatment planning, and in understanding the pathomechanisms of vascular tortuosity in general.

Original languageEnglish (US)
Pages (from-to)1738-1743
Number of pages6
JournalAmerican Journal of Neuroradiology
Volume40
Issue number10
DOIs
StatePublished - Jan 1 2019

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Connective Tissue Diseases
Carotid Arteries
Neurofibromatosis 1
Loeys-Dietz Syndrome
Blood Vessels
Marfan Syndrome
Ehlers-Danlos Syndrome
Aortic Aneurysm
Students

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Clinical Neurology

Cite this

Carotid artery tortuosity is associated with connective tissue diseases. / Welby, J. P.; Kim, S. T.; Carr, C. M.; Lehman, V. T.; Rydberg, C. H.; Wald, J. T.; Luetmer, P. H.; Nasr, D. M.; Brinjikji, W.

In: American Journal of Neuroradiology, Vol. 40, No. 10, 01.01.2019, p. 1738-1743.

Research output: Contribution to journalArticle

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abstract = "BACKGROUND AND PURPOSE: There is a general assumption in the cerebrovascular literature that there is an association between carotid artery tortuosity and connective tissues disease; however, this has not been firmly established. The purpose of this study was to determine the prevalence of carotid artery tortuosity in patients with connective tissue diseases relative to matched controls. MATERIALS AND METHODS: Patients with previous CTA or MRA and a diagnosis of connective tissue diseases were identified and compared with a cohort of age-matched controls. Radiologists blinded to the diagnosis reviewed the images and evaluated the presence of carotid artery tortuosity (including loops, kinks, or coils). Continuous variables were compared using the Student t test, and categoric variables with x 2 tests. RESULTS: One hundred forty-three patients with connective tissue disease and 143 controls were included in this study. Specific diagnoses included Marfan (n = 33), nonvascular Ehlers-Danlos (n = 36), Ehlers-Danlos vascular-type (n = 32), neurofibromatosis type 1 (n = 26), and Loeys-Dietz (n = 16) syndromes. The presence of carotid tortuosity was 44{\%} in connective tissue disease and 16{\%} in controls (P, .001). Of tortuosity manifestations, coils were most prevalent (23{\%} versus 3{\%}; P, .001). Among the various connective tissue diseases, the rates of any carotid tortuosity were 88{\%} for Marfan syndrome, 63{\%} for Loeys-Dietz syndrome, 42{\%} for neurofibromatosis type 1, and 19{\%} for both vascular- and nonvascular-type Ehlers-Danlos syndrome. The positive predictive value of the combination of aortic aneurysm and carotid tortuosity being associated with connective tissue disease was 95.4{\%}. The specificity was 98.6{\%}. CONCLUSIONS: Carotid artery tortuosity is highly associated with connective tissue diseases, particularly Marfan syndrome, Loeys-Dietz syndrome, and neurofibromatosis type 1. Such findings are relevant in risk assessment for vascular complications in connective tissue disease, endovascular treatment planning, and in understanding the pathomechanisms of vascular tortuosity in general.",
author = "Welby, {J. P.} and Kim, {S. T.} and Carr, {C. M.} and Lehman, {V. T.} and Rydberg, {C. H.} and Wald, {J. T.} and Luetmer, {P. H.} and Nasr, {D. M.} and W. Brinjikji",
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AU - Welby, J. P.

AU - Kim, S. T.

AU - Carr, C. M.

AU - Lehman, V. T.

AU - Rydberg, C. H.

AU - Wald, J. T.

AU - Luetmer, P. H.

AU - Nasr, D. M.

AU - Brinjikji, W.

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