2023IP Indian Journal of NeurosciencesOpen access

A case report on type I sturge weber syndrome with bilateral port-wine stain

Nissi Chrysolite Gongati, Vikas Agrawal, Srinivasulu Poluru

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Abstract

Sturge-Weber syndrome (SWS) is a type of neuro-cutaneous diseases which is seen in 1 in 50,000 populations and affects both the sexes equally. It usually manifests with a facial Port Wine Stain (PWS). Facial port-wine stains are usually isolated findings, however, when associated with cerebral and ocular vascular malformations they form part of the classical triad of Sturge-weber syndrome (SWS). The prevalence of PWS is estimated at three to five children per 1000 live births; there are ~26 million people worldwide with PWS birthmarks. The majority of facial PWS (~90%) are unilateral in a trigeminal dermatomal distribution. Here, we report a case of 39 years old female with SWS who presented with classical triad of SWS i.e., bilateral port-wine stain, epilepsy and severe glaucoma.

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Sturge-Weber syndrome (SWS) is a type of neuro-cutaneous diseases which is seen in 1 in 50,000 populations and affects both the sexes equally. It usually manifests with a facial Port Wine Stain (PWS). Facial port-wine stains are usually isolated findings, however, when associated with cerebral and ocular vascular malformations they form part of the classical triad of Sturge-weber syndrome (SWS). The prevalence of PWS is estimated at three to five children per 1000 live births; there are ~26 million people worldwide with PWS birthmarks. The majority of facial PWS (~90%) are unilateral in a trigeminal dermatomal distribution. Here, we report a case of 39 years old female with SWS who presented with classical triad of SWS i.e., bilateral port-wine stain, epilepsy and severe glaucoma.

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Available abstract

Sturge-Weber syndrome (SWS) is a type of neuro-cutaneous diseases which is seen in 1 in 50,000 populations and affects both the sexes equally. It usually manifests with a facial Port Wine Stain (PWS). Facial port-wine stains are usually isolated findings, however, when associated with cerebral and ocular vascular malformations they form part of the classical triad of Sturge-weber syndrome (SWS). The prevalence of PWS is estimated at three to five children per 1000 live births; there are ~26 million people worldwide with PWS birthmarks. The majority of facial PWS (~90%) are unilateral in a trigeminal dermatomal distribution. Here, we report a case of 39 years old female with SWS who presented with classical triad of SWS i.e., bilateral port-wine stain, epilepsy and severe glaucoma.

Key concepts: Port-wine stain, Sturge–Weber syndrome, Birthmark, Medicine, Dermatology, Stain, Pathology, Laser

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