title: Aortic Arch Interruption and Persistent Fifth Aortic Arch in Phace Syndrome: Prenatal Diagnosis and Postnatal Course.
abstract: PHACE is a rare congenital neurocutaneous syndrome where posterior fossa malformations, hemangiomas, cerebrovascular anomalies, aortic arch anomalies, cardiac defects, and eye abnormalities are variably associated. We describe the prenatal detection and the postnatal course of a child with PHACE syndrome with a unique type of aortic arch anomaly consisting of proximal interruption of the aortic arch and persistence of the fifth aortic arch. The fifth aortic arch represented in this case a vital systemic-to-systemic connection between the ascending aorta and the transverse portion of the aortic arch allowing adequate forward flow through the aortic arch without surgical treatment.
authors: Division of Pediatric Cardiology, Azienda Ospedaliero-Universitaria Meyer, Florence, Italy.
authors: Enrico
authors: E
authors: Chiappa
authors: Dermatology Unit, Azienda Ospedaliero-Universitaria Meyer, Florence, Italy.
authors: Antonella
authors: A
authors: Greco
authors: Division of Pediatric Cardiology, Azienda Ospedaliero-Universitaria Meyer, Florence, Italy.|Department of Clinical & Experimental Medicine, Parma University Hospital, Parma, Italy.
authors: Valentina
authors: V
authors: Fainardi
authors: Division of Pediatric Cardiology, Azienda Ospedaliero-Universitaria Meyer, Florence, Italy.
authors: Silvia
authors: S
authors: Passantino
authors: Division of Pediatric Cardiology, Azienda Ospedaliero-Universitaria Meyer, Florence, Italy.
authors: Daniele
authors: D
authors: Serranti
authors: Division of Pediatric Cardiology, Azienda Ospedaliero-Universitaria Meyer, Florence, Italy.
authors: Silvia
authors: S
authors: Favilli
mesh_terms: D000015:Abnormalities, Multiple; D001013:Aorta, Thoracic / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D003937:Diagnosis, Differential; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications* / Q000000981:diagnostic imaging* / Q000601:surgery; D006391:Hemangioma / Q000150:complications* / Q000000981:diagnostic imaging*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011247:Pregnancy; D013577:Syndrome; D014427:Twins; D016216:Ultrasonography, Prenatal
keywords: aorta coarctation; aortic arch; congenital heart defects; echocardiography; hemangioma