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

DOI: 10.4103/apc.APC_188_18

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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