[Facial angioma and the Sturge-Weber syndrome: a study of 121 cases]

Abstract

Background: We show a study in 121 patients with port-wine stain (PWS) over the areas of trigeminal nerve sensory innervation, and we analyzed the relations between this distribution, sex and the neuro-ocular disease.

Methods: The population included 68 women between 12 months and 73 years (mean, 23 years; standard deviation, 13.7 years) and 53 men between 12 months and 50 years (mean, 18 years; standard deviation, 12 years). Chi-square test and Fisher test were performed in order to assess statistical significance of our results.

Results: Facial port-wine stains (PWS) were predominantly distributed over the area of innervation of the maxillary branch (V2) of the trigeminal nerve (88% of patients), either isolated or simultaneously involving ophthalmic (V1) and/or mandibular branch (V3). Of the unilateral PWS (86%), 10% were also extrafacially distributed. However, bilateral PWS (14%) extended extrafacially in 53% of the patients. Moreover, the number of patients with extrafacial PWS was increased among those with PWS over V3 (50%). Glaucoma and epilepsy were present in 23 (17%) and 17 (14%) patients, respectively. In all of them PWS affected V1. Among the 19 patients with bilateral PWS, nine of them developed glaucoma and five, epilepsy. Epilepsy was more frequent in men (26%) than in women (4%).

Conclusions: Statistical analysis showed: 1) the PWS is preferentially distributed over the maxillary branch of the trigeminal nerve, 2) bilateral PWS significantly increases the frequency of cutaneous extrafacial PWS, 3) the presence of PWS over the mandibular branch of trigeminal nerve carries a higher risk of cutaneous extrafacial presence of the angioma, 4) only those patients with PWS over the ophthalmic branch may develop glaucoma and/or epilepsy, 5) extrafacial PWS is associated with a higher frequency of glaucoma, 6) men have a higher risk of developing epilepsy than women.