Sturge-Weber syndrome (SWS) is a rare congenital neurocutaneous syndrome is characterized by unilateral facial cutaneous vascular malformations affecting the eye and skin in association with ipsilateral leptomeningeal angiomatosis. The incidence of SWS is ~ 1:50,000 infants, with no predilection for the sex. Glaucoma in SWS patients shows a bimodal peak of age development: an early-onset (congenital) form affecting ~ 60% of patients and a late onset form during childhood and adolescence (40% of cases). Association of glaucoma with SWS is unilateral, most frequently as open-angle glaucoma leading to progressive visual field loss. Our case illustrated the need of regular follow up and early intervention in the patients with cutaneous port wine stain (PWS) in the distribution of trigeminal nerve to prevent loss of vision due to glaucoma.

Keywords: Struge weber syndrome, Port wine strain, Glaucoma, Leptomeningeal angiomatosis

1. Jing, L. Nan-yan, Z. Xiao-jun, W. Jian-dong, M. A. Henghui, and Z. Ru-song, “Sturge-Weber syndrome:a case report and review of literatures,” Chinese Medical Journal, vol. 123, no. 1, pp.117–121, 2010.
2. Welty LD. Sturge-Weber syndrome: A case study. Neonatal Netw 2006; 25: 89-98.
3. Comi AM, Mehta P, Hatfield LA, Dowling MM. Sturge-Weber syndrome associated with other abnormalities: a medical record and literature review. Arch Neurol. 2005; 62: 1924–1927.
4. Lee CW, Choi DY, Oh YG, Yoon HS, Kim JD. An infantile case of Sturge-Weber syndrome in association with Klippel-Trenaunay-Weber syndrome and phakomatosis pigmentovascularis. J Korean Med Sci. 2005; 20: 1082–1084.
5. Sonmez FM, Aksoy A, Sari A, Erpolat S, Ozkaya AK. Phacomatosis pigmentovascularis type IIB associated with Sturge-Weber syndrome: a case report and review of the literature. Genet Couns. 2013; 24: 247–250.
6. Recupero SM, Abdolrahimzadeh S, De Dominicis M, Mollo R. Sturge-Weber syndrome associated with nevus of Ota. Eye (Lond) 1998; 12: 212–213.
7. Abdolrahimzadeh S, Scavella V, Felli L, et al. Ophthalmic alterations in the Sturge Weber syndrome, Klippel Trenaunay syndrome and the phakomatosis pigmentovascularis: an independent group of conditions? Biomed Res Int. 2015 Article ID 786519:11 pages.
8. Moe P, Seay AR. Neurologic and muscular disorders. In: Hay WW, Hayward AR, Levin MJ, Sondheimer JM, editors. Current Pediatric Diagnosis and Treatment, 16th edn. Singapore: McGraw Hill; 2003. p. 717-92
9. Sinawat S, Auvichayapat N, Auvichayapat P, Yospaiboon Y, Sinawat S. 12-year retrospective study of Sturge-Weber syndrome and literature review. J Med Assoc Thai. 2014; 97(7): 742–750.
10. Sujansky E, Conradi S. Sturge-Weber syndrome: age of onset of seizures and glaucoma and the prognosis for affected children. J Child Neurol. 1995; 10: 49.
11. Abdolrahimzadeh S, Scavella V, Felli L, et al. Ophthalmic alterations in the Sturge Weber syndrome, Klippel Trenaunay syndrome and the phakomatosis pigmentovascularis: an independent group of conditions? Biomed Res Int. 2015 Article ID 786519:11 pages

12. Boniuk M. Hemangioma of the choroids. A clinicopathologic study of 71 cases and a review of the literature. Surv Ophthalmol. 1976; 20: 415–431.

Priyanka Sharma

Department of OBG, Bhandari Hospital Hamirpur (H.P), India