The Vascular Birthmarks Foundation

Dr. Linda Rozell-Shannon, PhD President and Founder

PHACE Syndrome

Here is an acronym that will help you to remember the components of PHACE Syndrome:

P = Posterior Fossa (a tumor or vascular growth located in the rear of the brain and can often be misdiagnosed as a cyst).
H = Hemangioma or other vascular birthmark present either on the outside or inside.
A = Arterial defect in the head and or neck area – a missing or problematic artery.
C = Cardiac problems – these vary from minor to severe.
E = Eye problems – these also vary from minor to severe.

The key with PHACE syndrome is to see a physician who knows how to diagnose and how to treat the complete syndrome.

A team must be consulted which must include a neurologist, cardiologist, opthamologist and vascular birthmark specialist and/or surgeon. There are very few cases of this syndrome accurately diagnosed in this country but there is becoming more and more information. If you suspect your child has PHACE syndrome, consult with a vascular birthmark specialist in order to receive an evaluation and confirmation of the diagnosis.

Kasabach-Merrit Syndrome

Kasabach-Merrit Syndrome is a disease of infancy that does not truly include a hemangioma.  Rather, the vascular Anomaly of KMS is a different type than infantile hemangioma and grows rapidly.  However, the presence at birth of a vascular anomaly usually means that a hemangioma is diagnosed, but it is not a “true” hemangioma.