Friday Pop Quiz #90

PHACES syndrome

The correct answer is E. Coarctation of the aorta.

This patient has a segmental infantile hemangioma on the face and should be evaluated for PHACES syndrome: posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects and coarctation of the aorta (Choice 5), eye anomalies, sternal defects and supraumbilical raphe. Work-up includes MRI/MRA of the head and neck, referral to a cardiologist, and referral to a pediatric ophthalmologist.

Cleft palate (Choice A), diaphragmatic hernia (Choice B), hypospadias (Choice C), imperforate anus (Choice D) are not associated with PHACES syndrome.

References: Bolognia, Jean., Jorizzo, Joseph L.Schaffer, Julie V. (Eds.) (2012) Dermatology /[Philadelphia] : Elsevier Saunders


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