PHACE syndrome: A remarkable phakomatosis

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Airway hemangiomas in PHACE syndrome.

OBJECTIVES/HYPOTHESIS To describe the clinical presentation and airway characteristics of infants with airway hemangiomas and concomitant PHACE syndrome and to determine the prevalence of airway hemangiomas in PHACE subjects at our institution. STUDY DESIGN Case series. METHODS Retrospective review including clinical presentation, airway findings, treatment measures, and outcomes. RESULTS...

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Intracranial infantile hemangiomas associated with PHACE syndrome.

BACKGROUND AND PURPOSE Head and neck infantile hemangiomas are common lesions that are rarely observed in an intracranial location. We report 4 patients with orbital infantile hemangiomas and ipsilateral enhancing intracranial lesions, presumed to be infantile hemangiomas. METHODS Imaging studies and medical records of 4 infants with orbital hemangiomas and enhancing intracranial lesions were...

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Complex aortic coarctation and PHACE syndrome.

In PHACE syndrome, the acronym PHACE stands for the association of posterior fossa malformations, cervicofacial hemangiomas, arterial anomalies, coarctation and eye anomalies. We report our findings in four patients with this syndrome, in whom it was characterized by complex aortic coarctation that required not only preoperative echocardiographic investigation, but also the use of techniques su...

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Large destructive facial hemangioma in PHACE syndrome

We report an infant who presented with large facial hemangioma associated with Dandy-Walker cyst and atrial septal defect. This case is peculiar in that the large facial hemangioma in posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of aorta and other cardiac defects (PHACE) syndrome resulted in massive tissue destruction.

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Association of hearing loss with PHACE syndrome.

BACKGROUND PHACE syndrome describes a spectrum of anomalies associated with large facial infantile hemangiomas and characterized by posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. With improved recognition and imaging practices of infants with PHACE syndrome, additional associations have been identified. To our...

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ژورنال

عنوان ژورنال: Pediatrics & Neonatology

سال: 2021

ISSN: 1875-9572

DOI: 10.1016/j.pedneo.2021.06.001