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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Bibliographic Details
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=
Description
Summary: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.
ISSN:0974-2069

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