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


Department of Pediatric Cardiology, Madras Medical Mission, Chennai, Tamil Nadu, India

Correspondence Address:
Dr. Kothandam Sivakumar
Department of Pediatric Cardiology, Madras Medical Mission, 4A, Dr. J J Nagar, Mogappair, Chennai - 600 089, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/apc.APC_188_18

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Year : 2019  |  Volume : 12  |  Issue : 3  |  Page : 333-335

 

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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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Department of Pediatric Cardiology, Madras Medical Mission, Chennai, Tamil Nadu, India

Correspondence Address:
Dr. Kothandam Sivakumar
Department of Pediatric Cardiology, Madras Medical Mission, 4A, Dr. J J Nagar, Mogappair, Chennai - 600 089, Tamil Nadu
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/apc.APC_188_18

Rights and Permissions

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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