Generalised nevus flammeus, episcleral capillary malformation and glaucoma

Description 

A 35-year-old woman presented with no vision, redness, pain and photophobia in the left eye (LE) since 4 months. This was associated with left-sided headache and vomiting too. She had history of poor vision in LE since 2–3 years, and had been treated with topical antiglaucoma medication. There was no significant family history. On examination, visual acuity was 6/6 in right eye (RE) and no perception of light in LE. Intraocular pressure (IOP) was measured as 14 mm Hg in RE and >60 mm Hg in LE. Slit lamp evaluation revealed conjunctival congestion, corneal oedema, fully dilated pupil and white cataract in LE (figure 1). Gonioscopy was within normal limits. RE ocular examination did not reveal any significant anomaly. RE pupil did not show any consensual response, while LE pupil was fixed. Systemic workup revealed nevus flammeus (NF) over the face involving both periocular regions but more...

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