What is SWS?
Sturge Weber Syndrome (SWS) is a rare, non-genetic condition resulting from an abnormal development of blood vessels of the skin, eyes and brain. The spectrum of clinical manifestations includes so-called port wine stains usually affecting one side of the face, glaucoma of the eye due to increased pressure within the eye, seizures, stroke-like events, migraine headaches and focal neurologic impairments. The condition poses a number of specific heath risks, due to its progressive, multi-organ involvement.
Most patients develop seizures and progressive neurologic deterioration with hemiparesis, visual impairment and cognitive decline that may start in infancy or childhood. Early diagnosis of brain and eye involvement in infants with a port wine stain is critical to offering effective treatment and potentially modify the severity of the disorder.
Epileptic seizures starting in the first year of life are usually the first and most common symptom of brain involvement in patients with SWS. Early and aggressive treatment of the epilepsy is essential to improving the cognitive outcome, as well as the quality of life, as has been shown in various studies from our and other centers. When medical treatment fails, evaluation for the possibility of epilepsy surgery should be considered early in every patient with SWS.
The challenges of epilepsy surgery in a patient with Sturge Weber Syndrome are complex but surmountable in the hands of experienced epilepsy specialists and surgeons working as a team. At the Cleveland Clinic Epilepsy Center, we have established a multi-disciplinary team of dedicated pediatric and adult epilepsy specialists, neurosurgeons, neuropsychologists, neuroradiologists, functional neuroimaging experts, cognitive and behavior experts, health psychologists and bioethicists to administer epilepsy care to SWS patients. The team meets on a weekly basis to discuss the best individualized plan of care for patients with complex epilepsy.
Specialty care physicians who treat this disorder
Epilepsy, if poorly controlled, can have devastating consequences on the patient's and family's quality of life. In SWS the seizures tend to cluster over a number of days. An individualized treatment plan starting with the first aid approach in the home setting, deciding on the next step of care at the emergency room and transfer to a pediatric facility with expert care for seizure treatment should be put in place in patients with the port wine stain and brain involvement. Ideally the patients should be seen in infancy prior to the onset of seizures to educate and instruct the family and establish a local network of care. A subsequent stepwise approach to epilepsy management should include early surgical evaluation when medication fails to contro seizures and neurologic function and development are at risk.
Skin,This can be treated early with dermatologic laser therapy and surgery.
Eye pressure increase or glaucoma leading to impaired vision is frequent and requires early and serial assessment for medical and surgical management.
Outside of a focused SWS program, fragmented care could come from all directions. Managing a complex disorder affecting different organs, follow up screening, early intervention, treatment, and counseling is challenging for the patient as well as the individual physicians seeing patients in different settings.
Instead, these responsibilities can be delegated to the SWS program, where a dedicated team of specialized physicians in one tertiary care center share the medical information of a patient through electronic medical records, and provide individualized care through a specialized team approach. With this coordination, the program delivers seamless care to patients and their families.
SWS Program Team
Continuing care from experts who understand SWS and other congenital or familial disorders due to vascular malformations is the key to successfully managing the condition, mitigating its complications, and providing the best life possible to each individual and family with SWS.
Patients referred to the Cleveland Clinic SWS Program are in skilled hands with professionals who focus on:
Dermatology, Neuropsychiatry, Counseling, Ophthalmology, Social work, Epilepsy, Genetics, Developmental medicine, Neurosurgery, ENT
Physicians in the SWS program are at the leading edge of SWS treatment and are committed to clinical and translational research and education to improve the lives of patients and families with SWS. Many questions about the disorder are still unanswered. What are the causes and are they preventable? How can we prevent seizures, how can we best treat them? When is a good time to do surgery? What are the best medical or surgical approaches to treating the brain, eye, and skin involvement?
The Cleveland Clinic SWS Program is committed to finding answers to these questions through research initiatives and collaborations.
For More Information or to Schedule an Appointment
216.445.0601
For Referring Physicians
If you have a specific question regarding a patient or our SWS program, contact Ajay Gupta, MD at 216.445.0601 or toll free at 1.800.223.2273, ext. 50601