title: Bilateral pial synangiosis in a child with PHACE syndrome.
abstract: The acronym PHACE has been used to denote a constellation of abnormalities: posterior fossa anomalies, facial hemangiomas, arterial anomalies, cardiac anomalies, and eye abnormalities. Approximately 30% of patients with large facial hemangiomas have PHACE syndrome, with the vast majority having intracranial arteriopathy. Few reports characterize neurological deterioration from this intracranial arteriopathy, and even fewer report successful treatment thereof. The authors report on a case of a child with PHACE syndrome who presented with an ischemic stroke from a progressive intracranial arteriopathy and describe her successful treatment with bilateral pial synangiosis. An 8-month old girl diagnosed with PHACE syndrome was found to have bilateral internal carotid artery stenosis. Although initially asymptomatic, a few months after diagnosis she suffered a right frontal and parietal stroke. MRI and cerebral angiography investigations demonstrated progressive intracranial arterial stenosis and occlusion. The patient then underwent indirect cerebral revascularization surgery. At 2-year follow-up, she exhibited clinical improvement with persistent speech and motor developmental delay. Follow-up MRI and cerebral angiography showed no new ischemic events and robust extensive vascular collateralization from surgery. PHACE syndrome is an uncommon disease, and affected patients often have cerebral arteriopathy. Although the underlying natural history of cerebral arteriopathy in PHACE remains unclear, cerebral revascularization may represent a potential therapy for symptomatic patients.
authors.affiliation: Division of Neurosurgery, Department of Surgery, and 
authors.forename: Andrew S
authors.initials: AS
authors.lastname: Jack
authors.affiliation: Division of Neurosurgery, Department of Surgery, and 
authors.forename: Michael M
authors.initials: MM
authors.lastname: Chow
authors.affiliation: Divisions of 2 Pediatric Dermatology.
authors.forename: Loretta
authors.initials: L
authors.lastname: Fiorillo
authors.affiliation: Pediatrics, and.
authors.forename: Thea
authors.initials: T
authors.lastname: Chibuk
authors.affiliation: Pediatric Neurology, Department of Pediatrics, Stollery Children's Hospital, Edmonton, Alberta, Canada.
authors.forename: Jerome Y
authors.initials: JY
authors.lastname: Yager
authors.affiliation: Division of Neurosurgery, Department of Surgery, and 
authors.forename: Vivek
authors.initials: V
authors.lastname: Mehta
mesh_terms: D001017:Aortic Coarctation / Q000150:complications / Q000601:surgery*; D005124:Eye Abnormalities / Q000150:complications / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D020765:Intracranial Arterial Diseases / Q000209:etiology / Q000601:surgery*; D020752:Neurocutaneous Syndromes / Q000150:complications / Q000601:surgery*; D019635:Neurosurgical Procedures / Q000379:methods*; D010841:Pia Mater / Q000601:surgery; D014656:Vascular Surgical Procedures / Q000379:methods*
keywords: ACoA = anterior communicating artery; AIS = arterial ischemic stroke; AP = anteroposterior; CCA = common carotid artery; ECA = external carotid artery; ICA = internal carotid artery; MCA = middle cerebral artery; MMD = moyamoya disease; PCA = posterior cerebral artery; PCoA = posterior communicating artery; PHACE; PHACES association; STA = superficial temporal artery; VA = vertebral artery; arterial ischemic syndrome; hemangioma; pial synangiosis; vascular anomalies; vascular disorders