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

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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Main Authors: Ronak Sheth, Arvind Sahay Singh, Sreeja Pavithran, Kothandam Sivakumar
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2019-01-01
Series:Annals of Pediatric Cardiology
Subjects:
Aortic arch anomaly
corkscrew aorta
hemangioma
neural crest disorder
PHACES syndrome
tortuous aorta
Online Access:http://www.annalspc.com/article.asp?issn=0974-2069;year=2019;volume=12;issue=3;spage=333;epage=335;aulast=
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spelling doaj-22792bf925764ada9770f2ef4416fac82020-11-25T02:07:48ZengWolters Kluwer Medknow PublicationsAnnals of Pediatric Cardiology0974-20692019-01-0112333333510.4103/apc.APC_188_18Corkscrew aortic arch in PHACES syndrome: Multimodal imaging of an unusual morphology of tortuous aortic arch in a rare but well-defined syndromeRonak ShethArvind Sahay SinghSreeja PavithranKothandam SivakumarPHACES 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.http://www.annalspc.com/article.asp?issn=0974-2069;year=2019;volume=12;issue=3;spage=333;epage=335;aulast=Aortic arch anomalycorkscrew aortahemangiomaneural crest disorderPHACES syndrometortuous aorta
collection DOAJ
language English
format Article
sources DOAJ
author Ronak Sheth
Arvind Sahay Singh
Sreeja Pavithran
Kothandam Sivakumar
spellingShingle Ronak Sheth
Arvind Sahay Singh
Sreeja Pavithran
Kothandam Sivakumar
Corkscrew aortic arch in PHACES syndrome: Multimodal imaging of an unusual morphology of tortuous aortic arch in a rare but well-defined syndrome
Annals of Pediatric Cardiology
Aortic arch anomaly
corkscrew aorta
hemangioma
neural crest disorder
PHACES syndrome
tortuous aorta
author_facet Ronak Sheth
Arvind Sahay Singh
Sreeja Pavithran
Kothandam Sivakumar
author_sort Ronak Sheth
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_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_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_sort corkscrew aortic arch in phaces syndrome: multimodal imaging of an unusual morphology of tortuous aortic arch in a rare but well-defined syndrome
publisher Wolters Kluwer Medknow Publications
series Annals of Pediatric Cardiology
issn 0974-2069
publishDate 2019-01-01
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.
topic Aortic arch anomaly
corkscrew aorta
hemangioma
neural crest disorder
PHACES syndrome
tortuous aorta
url http://www.annalspc.com/article.asp?issn=0974-2069;year=2019;volume=12;issue=3;spage=333;epage=335;aulast=
work_keys_str_mv AT ronaksheth corkscrewaorticarchinphacessyndromemultimodalimagingofanunusualmorphologyoftortuousaorticarchinararebutwelldefinedsyndrome
AT arvindsahaysingh corkscrewaorticarchinphacessyndromemultimodalimagingofanunusualmorphologyoftortuousaorticarchinararebutwelldefinedsyndrome
AT sreejapavithran corkscrewaorticarchinphacessyndromemultimodalimagingofanunusualmorphologyoftortuousaorticarchinararebutwelldefinedsyndrome
AT kothandamsivakumar corkscrewaorticarchinphacessyndromemultimodalimagingofanunusualmorphologyoftortuousaorticarchinararebutwelldefinedsyndrome
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