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PHACES association: a vasculocutaneous syndrome
Rohit P Rao1, Beth A Drolet, Kristen E Holland
1Department of Pediatrics, Divisions of Cardiology and Critical Care, Medical College of Wisconsin, Milwaukee, WI, 53226, USA. rrao@chw.org
Pediatric Cardiology
|April 23, 2008
Summary
PHACES association, a condition linked to facial hemangiomas, often involves serious cardiac and cerebrovascular issues. Early recognition of these vascular anomalies is crucial for timely intervention and preventing severe complications in infants.
Area of Science:
- Pediatric Cardiology
- Neurology
- Dermatology
Background:
- PHACES association is a complex condition characterized by multiple congenital anomalies.
- It is primarily associated with large segmental hemangiomas of the head and neck in infants.
- Cardiac and cerebrovascular anomalies are significant comorbidities with high complication risks.
Purpose of the Study:
- To summarize dermatologic, cardiac, and cerebrovascular findings in infants with PHACES association.
- To highlight the importance of recognizing the cutaneous manifestations of PHACES.
- To emphasize the need for early detection of associated vascular pathologies.
Main Methods:
- Retrospective review of a cohort of infants diagnosed with PHACES association.
- Detailed analysis of dermatologic, cardiac, and cerebral vascular findings.
- Evaluation of imaging studies, including those for aortic arch abnormalities.
Main Results:
- All infants presented with large segmental facial hemangiomas and aortic arch abnormalities.
- Aortic arch anomalies were complex and often unsuspected prior to imaging.
- Cerebral vascular anomalies were present in all infants, with 2/5 experiencing neurologic complications.
Conclusions:
- PHACES association requires vigilant recognition due to potentially lethal vascular anomalies.
- Early identification of associated vascular pathologies is critical for preemptive treatment.
- Prompt intervention can prevent devastating and irreversible sequelae in affected infants.
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