Open AccessCorkscrew aortic arch in PHACES syndrome: Multimodal imaging of an unusual morphology of tortuous aortic arch in a rare but well-defined syndrome
Author(s) -
Ronak Sheth,
Arvind Singh,
Sreeja Pavithran,
Kothandam Sivakumar
Publication year - 2019
Publication title -
annals of pediatric cardiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.292
H-Index - 19
eISSN - 0974-2069
pISSN - 0974-5149
DOI - 10.4103/apc.apc_188_18
Subject(s) - aortic arch , medicine , tortuosity , arch , cardiology , aorta , geology , structural engineering , geotechnical engineering , porosity , engineering
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.