Original Article

Pediatric Cardiology

, Volume 29, Issue 4, pp 793-799

First online:

PHACES Association: A Vasculocutaneous Syndrome

  • Rohit P. RaoAffiliated withDepartment of Pediatrics, Divisions of Cardiology and Critical Care, Medical College of Wisconsin Email author 
  • , Beth A. DroletAffiliated withDepartment of Dermatology, Medical College of Wisconsin
  • , Kristen E. HollandAffiliated withDepartment of Dermatology, Medical College of Wisconsin
  • , Peter C. FrommeltAffiliated withDepartment of Pediatrics, Division of Cardiology, Medical College of Wisconsin

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PHACES association is a spectrum of anomalies that might occur in infants with large facial hemangiomas. Most infants with PHACES association have segmental hemangiomas of the head or neck. Cardiac and cerebrovascular anomalies might be the most important association, as they carry a significant risk of complications. This article summarizes the dermatologic, cardiac, and cerebral vascular findings in a cohort of infants diagnosed with PHACES association. All had large segmental facial hemangiomas and aortic arch abnormalities. Four of the five were not suspected of having arch obstruction prior to imaging studies because of the aberrant origin of both subclavian arteries, and 4/5 required either interventional or surgical repair for arch obstruction. In contrast to classic aortic coarctation, the aortic anomalies found in the cohort had unusually complex and unpredictable anatomic involvement. Cerebral vascular anomalies were identified in 5/5, and 2/5 had neurologic complications secondary to abnormal cerebral vascular supply. It is important for care providers to recognize this association that presents with a cutaneous stigma, as it is associated with potentially lethal and often unrecognized vascular anomalies. Earlier recognition of the associated vascular pathologies might enable preemptive treatments before potentially devastating and irreversible sequelae.


Coarctation Aorta PHACES