Secondary Glaucoma in Patient with Sturge Weber Syndrome at Tertiary Hospital, North East, Nigeria: A Case Report

Background: Sturge-Weber Syndrome (SWS) is a congenital, and sporadic neurocutaneous syndrome occuring as a result of angiomas involving the leptomeninges, and the skin of the face along the ophthalmic and maxillary divisions of the trigeminal nerve. It is characterized by a facial port-wine stain presenting at birth, and glaucoma is a common ocular manifestation.Method: Patient clinical notes from electronic medical records with a diagnosis of SWS was used and the relevant literature reviewed. Informed consent was obtained from the child’s parents, and ethical approval obtained from the Hospital Ethical Committee.Result: An 8 year old female child presented with history of loss of vision, pain and photophobia in the left eye of three (3) years duration. Parents had noticed dark patch on the left side of her face at birth, and later tearing and gradual enlargement of eyes noticed during one year of life. On examination, she had port-wine stain involving the left side of her face. Left eye examination revealed unaided visual acuity (VA) of hand movement (HM), with tortuous conjunctival and episcleral veins, marked corneal epithelial oedema, and a dilated and sluggish pupil. Further view of the lens and posterior segment was not clear due to a hazy media. Intraocular pressure (IOP) was 10 and 35mmHg in the right and left eye respectively.Conclusion: sws is a rare condition and glaucoma is a frequent associated clinical manifestation which if treatment is not commenced on time would lead to blindness. All patients with facial discolouration/port-wine stain in the ophthalmic distribution of the trigeminal nerve that present to the eye clinic should have an evalution to rule out glaucoma during all visits and subsequent regular visits advised.In our clinical setting, treatment may be commenced based on typical clinical symptoms,port-wine stain, and clinically confirmed glaucoma.