The Acronym PHACE stands for an association of symptoms that include:

                                                                                          P- Posterior Fossa Malformations                 

                                                                                          H – Hemangioma                                      

                                                                                          A - Arterial Anomalies                                       

                                                                                          C - Cardiac abnormalities/aortic coartation 

                                                                                          E – Eye Abnormalities                                         

                                                                                         S- Sternal cleft defects

Hemangioma is the most common benign tumor of infancy and children.  It has been estimated that 4-5% of the population is diagnosed with a hemangioma.  A small group of patients diagnosed with hemagnioma have associated sympotms that include abnormalities of the brain, heart, vasculature, eyes, aorta, and chest wall.  This group of symptoms  make up the neruocutaneous disorder called PHACE Syndrome.  (other common names have included PHACES, PHACES Syndrome) 

Very little is known of the natural history of PHACE or the long term clinical implications of the disorder.  Historically a broad defination of PHACE included a facial hemagnioma and one or more extracutaneous features.  There has been no standardized means by which physicians diagnosed PHACE.  Physicians have relied on personal experience and consultation with vascular anomaly centers when making the diagnosis of PHACE.  On Noveber 7-8, 2008 leading physicians in the field of vascular anomalies met at Texas Children's Hospital to discuss the criteria for PHACE Syndrome.  The goal of the meeting was to move beyond a descriptive understanding of PHACE towards establishing clinical trials for treating and searching for a genetic basis for the syndrome.   

A Consensus statment has been written and submitted for publication to the medical journal Pediatrics.  We should know if the manuscript has been accepted and if it will be published by February 2009.

Announcements from NOVA:

The primary mission of NOVA is to provide information and support for individuals affected by vascular anomalies.  Vascular Anomalies is the umbrella term that includes hemangioma and vascular malformation and related syndromes.  PHACE is one of those related syndromes. 

NOVA held the first PHACE Syndrome Conference for families, partnering with Texas Children's Hospital November 5-7, 2008.  The meeting was a tremendous success, exceeding our expectations.  It was a time for families to meet, share and learn from eachother.  It was a time for physicians and scientists to share the latest information on PHACE Syndrome and to answer questions from parents about PHACE Syndrome.  Special thanks to Beth Drolet, Ilona Frieden, Peter Frommelt, Maria Garzon, Anita Haggstrom, Judith Hall, Christopher Hess, Geoffrey Heyer, Dawn Siegel and Denise Metry for answering all our questions. 

More Conference Information

NOVA is launching a letter campaign to the NIH for the direct purpose of requesting support for funding of vascular anomaly/PHACE research.  Please follow the link below to the information on how you can help with this effort.

Letter Campaign

PHACES has a varied presentation- NOVA is dedicated to assisting all affected by PHACES

NOVA PROVIDES THIS INFORMATION TO FAMILIES AS A RESOURCE. IT IS NOT INTENDED TO ENGAGE IN THE PRACTICE OF MEDICINE OR TO REPLACE THE PHYSICIAN. NOVA DOES NOT CLAIM TO HAVE MEDICAL KNOWLEDGE. NOVA DOES NOT ENDORSE ANY PARTICULAR PHYSICIAN, TREATING FACILITY OR TREATMENT PROTOCAL. IN ALL CASES NOVA AND ITS BOARD OF DIRECTORS RECOMMENDS THAT YOU SEEK THE OPINION OF A PHYSICIAN EXPERIENCED IN THE MANAGEMENT OF HEMANGIOMAS AND VASCULAR MALFORMATIONS. 

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