PHACE Syndrome - A Rare Disorder
As the title says, PHACE syndrome is an extremely rare disorder characterised by problems in multiple systems. It is an association between segmental infantile hemangiomas and malformations of the heart, eyes, major arteries and brain. It's cause is unknown and it is thought to arise sporadically. The term PHACE itself is an acronym for the most common clinical presentation with "P" for posterior fossa brain malformations, "H" for hemangiomas, "A" for arterial anomalies, "C" for cardiac anomalies and coarctation of aorta and "E" for eye and endocrine abnormalities. The treatment differs based on which bodily system is affected. Therefore, the patient requires a team of various specialists including dermatologist, cardiologist, ophthalmologist, neurologist etc. For the hemangioma, systemic steroids and even propranolol therapy have proven to be quite beneficial.
