title: PHACE(S) Syndrome With Absent Intracranial Internal Carotid Artery and Anomalous Circle of Willis.
abstract: The authors present a case of PHACE(S) (posterior fossa malformations, hemangioma, arterial anomalies, cardiac defects, eye anomalies, and sternal cleft or supraumbilical raphe) syndrome with a right-sided segmental infantile hemangioma, and describe in detail, the associated absent ipsilateral intracranial internal carotid artery and anomalous Circle of Willis. Propranolol therapy led to accelerated, complete involution. Nadolol may reduce the theoretical risk of treating PHACE(S) patients with β-blockers.
authors.affiliation: *Gillies McIndoe Research Institute †Maxillofacial and Burns Unit, Centre for the Study and Treatment of Vascular Birthmarks, Wellington Regional Plastic ‡Department of Paediatrics, Hutt Hospital, Wellington, New Zealand.
authors.forename: Pieta R
authors.initials: PR
authors.lastname: Winter
authors.forename: Tinte
authors.initials: T
authors.lastname: Itinteang
authors.forename: Philip
authors.initials: P
authors.lastname: Leadbitter
authors.forename: Trevor
authors.initials: T
authors.lastname: FitzJohn
authors.forename: Swee T
authors.initials: ST
authors.lastname: Tan
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis*; D001921:Brain / Q000002:abnormalities*; D002343:Carotid Artery, Internal / Q000002:abnormalities*; D002941:Circle of Willis / Q000002:abnormalities*; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging / Q000379:methods*; D013577:Syndrome; D014057:Tomography, X-Ray Computed