A 34-year-old male diagnosed with St-Weber syndrome in childhood, who presented with acute glaucoma secondary to anterior chamber crystalline luxation and pupillary block, so he underwent emergency surgery.
The examination showed a left hemifacial oportumvine spot on the territory of the first and second trigeminal branches, severe exophthalmos with marked inferior epigcleral rejection of the ocular globe.
In the exploration with posterior cleft lip and palate, a fully dislocated mature cataract was observed in the anterior chamber in contact with the corneal endothelium with pupillary block and intraocular pressure of 60 mm Hg, the underlying hyperprend
1.
After unsuccessful hypotensive medical treatment, the decision was made to perform an intervention, removing intra crystalline lens.
The eye fundus confirmed the suspicion of a superior peripapillary choroidal lesion with associated exudative retinal detachment.
Magnetic resonance imaging (MRI) showed hyperintense lesions on T1 and T2 in leptomeninges of the left frontoparietal region with calcifications and frontal lobe.
A choroidal lesion and two extraconal orbital lesions in the superior nasal quadrant and superior temporalis, well defined, associated with dilatation of the superior ophthalmic vein.
All lesions were compatible with malignant hemangiomas.
1.
Intraocular pressure after surgery was maintained between 10 and 16 mm Hg without need for antihypertensive treatment and final visual acuity was less than 20/200 (balloon view).
Currently, choroidal and orbital lesions remain stable, so they do not require treatment.
