Chinese Journal of Dermatology ›› 2022, Vol. 55 ›› Issue (5): 417-421.doi: 10.35541/cjd.20210211

• Research Reports • Previous Articles     Next Articles

Clinical significance of lesion distribution in children with Sturge-Weber syndrome

Su Wei1, Deng Wei1, Sun Xuefeng2, Zhang Sheng1, Liu Xiaoyan1   

  1. 1Department of Dermatology, Children′s Hospital Affiliated to Capital Institute of Pediatrics, Beijing 100020, China; 2Department of Radiology, Children′s Hospital Affiliated to Capital Institute of Pediatrics, Beijing 100020, China
  • Received:2021-03-11 Revised:2021-08-08 Online:2022-05-15 Published:2022-04-29
  • Contact: Zhang Sheng; Liu Xiaoyan;
  • Supported by:
    National Natural Science Foundation of China(82073461)

Abstract: 【Abstract】 Objective To analyze distribution characteristics of facial port-wine stains and brain imaging features of children with Sturge-Weber syndrome (SWS). Methods Clinical and imaging data were collected from 22 children with confirmed SWS at Department of Dermatology, Children′s Hospital Affiliated to Capital Institute of Pediatrics from July 2017 to August 2020, and retrospectively analyzed. The distribution characteristics of port-wine stains along the facial trigeminal nerve and brain imaging features were investigated in these children with SWS. Results Among the 22 children, 10 were males and 12 were females. Their age ranged from 0.08 to 9.92 years, and the median age was 1.67 years. There were 13 cases of SWS type Ⅰ and 9 cases of SWS type Ⅱ. In terms of the port-wine stain type, 4 children were diagnosed with pink port-wine stains, aged from 0.50 to 2.17 years; 14 were diagnosed with purple port-wine stains, aged from 0.08 to 8.83 years; 4 were diagnosed with thickened port-wine stains, aged from 4.92 to 9.92 years. Port-wine stains were distributed in the ophthalmic (V1) division of trigeminal nerve in 22 patients, in the maxillary (V2) division in 20 patients, and in the mandibular (V3) division in 8 patients. There were 17 children with ocular abnormalities, aged from 0.08 to 9.92 years, including 11 with glaucoma, 5 with elevated intraocular pressure and 2 with visual impairment. Among the children with glaucoma, 7 developed glaucoma within 2 years of age, 8 suffered from unilateral glaucoma, and 3 from bilateral glaucoma, and glaucoma occurred on the same side as port-wine stains. Brain imaging abnormalities were observed in 12 children, and mainly included vascular malformations in the cerebral cortex involving the frontal, parietal, temporal and occipital lobes, as well as cerebral atrophy, punctate hemorrhage, calcification, sulcal widening, midline deviation and increased intraventricular choroid plexus vessels. Eleven children had symptoms of epilepsy and other neurological manifestations, including developmental delay, mental retardation and physical impairment. Conclusion Port-wine stains distributed in the V1 and V2 divisions of the facial trigeminal nerve may indicate a higher risk of SWS, and ophthalmic and brain imaging screening as well as long-term follow-up should be performed.

Key words: Sturge-Weber syndrome, Port-Wine stain, Glaucoma, Epilepsy, Child, Imaging manifestations