Port wine stains are birthmarks that are deep red in color, similar to the color of port wine. It's caused by a vascular malformation, which means there was an abnormal cluster of capillaries in the skin. As many as one in 300 babies are born with port wine stains. However, for some people, a port wine stain is more than just a birthmark. Here's what you need to know if your baby was born with a port wine stain:
Port wine stain can be a sign of Sturge-Weber syndrome
A port wine stain on the scalp, forehead, or near the eyes can be a sign of Sturge-Weber syndrome, which occurs in roughly one in 20,000 births. In Sturge-Weber syndrome, the abnormal cluster of capillaries affects the brain and eyes. Of course, any medical condition that affects the brain and eyes is concerning.
Sturge-Weber syndrome is a serious medical condition
The medical terminology for Sturge-Weber syndrome is encephalotrigeminal angiomatosis. Breaking that medical term down into understandable English, it directly means:
The excessive capillary formation in, near, and around the area of the trigeminal nerve can lead to serious eye problems, particularly glaucoma, and neurological symptoms, such as seizures. Due to the brain being affected, someone with this condition can have developmental delays and cognitive impairment. Depending on the severity of the condition, weakness and paralysis can also occur.
How is Sturge-Weber syndrome diagnosed and treated?
Everyone with Sturge-Weber has a port wine stain on their head. However, not everyone with a port wine stain has Sturge-Weber. With these factors in mind, your baby's pediatrician should order imaging tests to determine whether or not there is brain damage and damage to the trigeminal nerve and the eyes.
Imaging tests for this include CT scans and MRIs. Your child and their imaging should be sent to a neurologist for further evaluation. Due to the possibility of the eyes being compromised, your pediatrician should also order a consult with a neuro-ophthalmologist to check the eyes.
If Sturge-Weber is diagnosed, treatment options can include anticonvulsant medication to reduce seizure activity, eye drops to decrease eye pressure that can cause glaucoma, and physical therapy to improve muscle strength if there is any weakness. Educational therapies can be ordered if developmental delays are noted.
Contact a medical center like Shriners Hospitals for Children – Cincinnati for more information and assistance.
Share30 August 2017
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