Some infants who have port-wine stains also have a syndrome called Sturge-Weber (SWS). About 8% of infants with facial port-wine stains of the upper face and forehead will have SWS. This risk increases to about 25% if the port wine stain covers both eyes or the entire half of the face. It occurs equally in males and females. Along with the port wine stain, there may be neurological problems such as seizures, headaches, visual changes, and cognitive and developmental delays.
Seizures are the most common problem in infants with SWS. This is due to abnormal blood vessels on the surface of the brain, and other structural changes that can occur in the brain. There may also be eye abnormalities such as glaucoma (increased pressure in the eye), which most often affects the eye where the port wine stain is present. Less commonly there can be endocrine and other organ abnormalities.
There is a wide range in the problems that children with SWS may have. Some children may have very few health problems, and others may have very serious problems. It is important to have a team of specialists available to care for a patient with SWS. Dermatologists, neurologists and ophthalmologists should evaluate your child on a regular basis. There also should be other specialists, such as plastic surgeons, neurosurgeons, interventional radiologists and physical and occupational therapists on staff to provide treatment when necessary.
The Sturge Weber Foundation has created standards of care; physicians and healthcare providers that have the expertise to provide the best care for patients with Sturge-Weber Syndrome and meet these standards are called Sturge-Weber Centers of Excellence. Lurie Children’s is honored to be named a Sturge-Weber Center of Excellence, one of only 18 centers in the United States.
Klippel-Trenaunay Syndrome is an extremity vascular malformation syndrome with the presence of the three findings: a venous malformation or vein varicosities, a capillary malformation (also called a port-wine stain), and an increase in the size of bone and soft tissues in the affected area. This syndrome is often first noted at birth and often worsens with time. Children with this syndrome should be followed closely by a team of physicians who can address issues as they arise. Leg length difference, pain, clotting problems, infection, swelling and functional issues are possible complications that can occur.
Parkes Weber syndrome also presents with a vascular stain and limb overgrowth in length and girth. In this syndrome, there are also areas where blood is flowing between arteries and veins, called arteriovenous shunts. This shunting is considered a “fast-flow.” Patient with Parkes Weber syndrome may have what has been renamed capillary malformation-arteriovenous malformation syndrome (CM-AVM). Rarely, the excess of blood flow to the arteries can cause heart failure. Children with Parkes-Weber syndrome also must be closely followed by a team of physicians.
These syndromes require close monitoring throughout a child’s life and specialists who will be able to correctly diagnose and care for the syndrome. The Vascular Lesion Center at Lurie Children's includes multidisciplinary specialists that are available to meet your child and develop a specialized plan of care that meets their unique needs. Depending on the location and severity of the lesion, your child may need to be evaluated by many specialists including dermatologists, cardiologists, ophthalmologists, neurologists, radiologists, otolaryngologists, and plastic and general surgeons. Our psychologist and social worker will be able to help you and your child adjust to the changes and special needs that this condition requires
If you’d like to request an appointment with one of our specialists, call 312.227.8521.