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Other Names for this Disease
- Encephalofacial angiomatosis
- Encephalotrigeminal angiomatosis
- Fourth phacomatosis
- Leptomeningeal angiomatosis
- Meningeal capillary angiomatosis
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Your QuestionI have a son who is 18 months old. He has Sturge-Weber and we are wondering what the percentage is that his birthmark will protrude from his face. What are the pros and cons of laser surgery? If we were to get the surgery, when would be the best time to get it done?
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Questions on this page
- How might a port-wine stain associated with Sturge-Weber syndrome change over time?
- What are some of the benefits and risks of laser treatment for port-wine stains associated with Sturge-Weber syndrome?
- Is there an ideal time to undergo laser treatment for port-wine stains associated with Sturge-Weber syndrome?
A port-wine stain associated with Sturge-Weber syndrome usually is obvious at birth. However, its appearance changes with age and its size increases as the affected individual grows. In the newborn period, it is usually flat and light pink in color. It typically darkens with age to a deep red, "port-wine" appearance, and vascular ectasias (dilations and/or expansions) develop. The vascular ectasias produce nodules and superficial outgrowths, which lead to overgrowth of the underlying soft tissues and sometimes the bone. Dental abnormalities may occur as a result of gingival thickening if this area is affected. By the fifth decade of life, 65 percent of individuals with a facial port wine stain have hypertrophy and nodularity within the lesion. Left untreated, a port-wine stain can lead to complications including bleeding. In many cases it is not possible to predict exactly when or how much the port-wine stain will grow; this can vary amongst affected individuals.
Last updated: 3/14/2012
Pulsed dye laser (PDL) remains the treatment of choice for the majority of children with a port-wine stain (PWS). Laser treatment of port-wine stains may produce good cosmetic results, with a low incidence of adverse skin changes and other side effects. A major benefit of laser treatment for a PWS is that it can help to minimize psychological problems associated with the social consequences of having a PWS. It has been shown that large facial port-wine stains are associated with an increase in mood and social problems in children older than 10 years of age.Most experts agree that there is little risk associated with the use of PDL in a child with Sturge-Weber syndrome (SWS), provided that anticonvulsant therapy is maintained and that adequate care is taken. The level of pain associated with laser treatment varies. Management of anesthesia should be carefully planned to minimize the potential for secondary effects. Few children with SWS achieve complete clearance of their PWS with laser treatment; PDL does have limitations when large areas or dermatomal patterns are involved.
Last updated: 3/14/2012
While some authors have reported there is no significant evidence that treatment for a port-wine stain at an earlier age is preferable, most experts agree that earlier treatment may minimize the psychological burden an affected individual may develop in relation to the cosmetic defect or from having laser treatment. It has been shown that large facial port-wine stains are associated with an increase in mood and social problems in children older than 10 years of age. Furthermore, multiple treatments are needed, so earlier treatment (when the lesion is initially smaller) may reduce the number of laser flashes per treatment as well as the total number of treatments needed.
Last updated: 3/14/2012
- John B Bodensteiner. Sturge-Weber syndrome. UpToDate. UpToDate; 2012;
- Port Wine Stain. The Sturge-Weber Foundation. http://www.sturge-weber.org/. Accessed 3/13/2012.
- Léauté-Labréze C, Boralevi F, Pedespan JM, Meymat Y, Taïeb A. Pulsed dye laser for Sturge-Weber syndrome. Arch Dis Child. November 2002; 87(5):434-435. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1763097/pdf/v087p00434.pdf. Accessed 3/13/2012.
- Hennedige AA, Quaba AA, Al-Nakib K. Sturge-Weber syndrome and dermatomal facial port-wine stains: incidence, association with glaucoma, and pulsed tunable dye laser treatment effectiveness. Plast Reconstr Surg. April 2008; 121(4):1173-1180.
- Masanori Takeoka. Pediatric Sturge-Weber Syndrome. eMedicine. January 5, 2010; http://emedicine.medscape.com/article/1177523-overview. Accessed 3/13/2012.