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Ann Pediatr Cardiol. 2019 Sep-Dec;12(3):333-335. doi: 10.4103/apc.APC_188_18.

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

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


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.


Aortic arch anomaly; PHACES syndrome; corkscrew aorta; hemangioma; neural crest disorder; tortuous aorta

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