Sturge-Weber syndrome and dermatomal facial port-wine stains: incidence, association with glaucoma, and pulsed tunable dye laser treatment effectiveness.
BACKGROUND
Laser therapy is the optimal approach for treating port-wine stains, but whether it is effective for patients with facial dermatomal port-wine stains and Sturge-Weber syndrome is undetermined. This project aimed to verify the incidence of Sturge-Weber syndrome and glaucoma within the population, compare the response in syndromic and nonsyndromic patients with facial dermatomal port-wine stains to laser, and determine the treatment response in relation to dermatome and color.


METHODS
The authors retrospectively reviewed 874 patients with facial port-wine stains (203 displayed a dermatomal pattern). Pretreatment and posttreatment photographs were assessed clinically and recorded on a statistically reliable percentage gradient.


RESULTS
Sturge-Weber syndrome was diagnosed in 30 patients. Twelve patients had glaucoma, with the port-wine stains involving the ophthalmic (V1) division of the trigeminal nerve. The highest proportion of patients with glaucoma came from those with involvement of the maxillary (V2) and/or mandibular (V3) division of the trigeminal nerve. No patients had Sturge-Weber syndrome with V3 port-wine stains alone. After laser therapy, only 45 percent of syndromal patients and 55 percent of nonsyndromic patients had a satisfactory outcome (>50 percent) in color and size reduction of dermatomal port-wine stains.


CONCLUSIONS
The incidence of Sturge-Weber syndrome was 3 percent in patients with a facial port-wine stain. There was an increased risk of Sturge-Weber syndrome with involvement of V1 port-wine stains and no risk with involvement of V3 port-wine stains alone. Laser treatment produced unsatisfactory outcomes in patients with facial dermatomal port-wine stains. V3 port-wine stains responded best and V2 worst to laser.
Hennedige
Anusha A
AA
Livingston, West Lothian, Scotland From St. John's Hospital.
Quaba
Awf A
AA
Al-Nakib
Khalil
K
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D005260:Female; D005901:Glaucoma / Q000150:complications*; D006801:Humans; D015994:Incidence; D007223:Infant; D054025:Lasers, Dye; D008297:Male; D008875:Middle Aged; D019339:Port-Wine Stain / Q000150:complications* / Q000453:epidemiology / Q000532:radiotherapy* / Q000601:surgery*; D012189:Retrospective Studies; D013341:Sturge-Weber Syndrome / Q000150:complications* / Q000453:epidemiology / Q000532:radiotherapy*