Corkscrew aortic arch in PHACES syndrome: Multimodal imaging of an unusual morphology of tortuous aortic arch in a rare but well-defined syndrome

PHACES syndrome, a diffuse aortocraniocerebral vasculopathy, is a neural tube migration disorder, characterized by aortic coarctation and aberrant arch branches. Clinical diagnosis, echocardiography, and surgical management of coarctation in this syndrome are challenging due to peculiar morphologica...

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Autores principales: Sheth, Ronak, Singh, Arvind Sahay, Pavithran, Sreeja, Sivakumar, Kothandam
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer - Medknow 2019
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6716328/
https://www.ncbi.nlm.nih.gov/pubmed/31516297
http://dx.doi.org/10.4103/apc.APC_188_18
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author Sheth, Ronak
Singh, Arvind Sahay
Pavithran, Sreeja
Sivakumar, Kothandam
author_facet Sheth, Ronak
Singh, Arvind Sahay
Pavithran, Sreeja
Sivakumar, Kothandam
author_sort Sheth, Ronak
collection PubMed
description PHACES syndrome, a diffuse aortocraniocerebral vasculopathy, is a neural tube migration disorder, characterized by aortic coarctation and aberrant arch branches. Clinical diagnosis, echocardiography, and surgical management of coarctation in this syndrome are challenging due to peculiar morphological differences. Corkscrew aortic arch, an extreme tortuosity of the aortic arch described in arterial tortuosity syndrome, is not reported in PHACES syndrome so far. Multimodal imaging of this unusual corkscrew aortic arch in two patients with PHACES syndrome is presented.
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spelling pubmed-67163282019-09-12 Corkscrew aortic arch in PHACES syndrome: Multimodal imaging of an unusual morphology of tortuous aortic arch in a rare but well-defined syndrome Sheth, Ronak Singh, Arvind Sahay Pavithran, Sreeja Sivakumar, Kothandam Ann Pediatr Cardiol Case Report PHACES syndrome, a diffuse aortocraniocerebral vasculopathy, is a neural tube migration disorder, characterized by aortic coarctation and aberrant arch branches. Clinical diagnosis, echocardiography, and surgical management of coarctation in this syndrome are challenging due to peculiar morphological differences. Corkscrew aortic arch, an extreme tortuosity of the aortic arch described in arterial tortuosity syndrome, is not reported in PHACES syndrome so far. Multimodal imaging of this unusual corkscrew aortic arch in two patients with PHACES syndrome is presented. Wolters Kluwer - Medknow 2019 /pmc/articles/PMC6716328/ /pubmed/31516297 http://dx.doi.org/10.4103/apc.APC_188_18 Text en Copyright: © 2019 Annals of Pediatric Cardiology http://creativecommons.org/licenses/by-nc-sa/4.0 This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Case Report
Sheth, Ronak
Singh, Arvind Sahay
Pavithran, Sreeja
Sivakumar, Kothandam
Corkscrew aortic arch in PHACES syndrome: Multimodal imaging of an unusual morphology of tortuous aortic arch in a rare but well-defined syndrome
title Corkscrew aortic arch in PHACES syndrome: Multimodal imaging of an unusual morphology of tortuous aortic arch in a rare but well-defined syndrome
title_full Corkscrew aortic arch in PHACES syndrome: Multimodal imaging of an unusual morphology of tortuous aortic arch in a rare but well-defined syndrome
title_fullStr Corkscrew aortic arch in PHACES syndrome: Multimodal imaging of an unusual morphology of tortuous aortic arch in a rare but well-defined syndrome
title_full_unstemmed Corkscrew aortic arch in PHACES syndrome: Multimodal imaging of an unusual morphology of tortuous aortic arch in a rare but well-defined syndrome
title_short Corkscrew aortic arch in PHACES syndrome: Multimodal imaging of an unusual morphology of tortuous aortic arch in a rare but well-defined syndrome
title_sort corkscrew aortic arch in phaces syndrome: multimodal imaging of an unusual morphology of tortuous aortic arch in a rare but well-defined syndrome
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6716328/
https://www.ncbi.nlm.nih.gov/pubmed/31516297
http://dx.doi.org/10.4103/apc.APC_188_18
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