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Abstract #45790 Published in IGR 13-2
Microstrabismus in a patient with Sturge Weber syndrome
Dimitrova G; Oscar A; Cherninkova SNeuro-Ophthalmology 2011; 35: 91
INRODUCTION: Sturge-Weber syndrome is a rare capillary vascular malformation that may affect the eye, skin and CNS. This congenital neuro-oculocutaneous angiomatosis has various ocular signs: glaucoma, conjunctival and episcleral haemangiomas, diffuse choroidal haemangiomas, heterochromia of the iris. Anisometropia, amblyopia and strabismus have also been reported. AIM: To present a rare case with Sturge-Weber syndrome with a discrepancy between the ophthalmoscopic finding and visual acuity of the two eyes. MATERIAL: A 16 year-old female patient with Sturge Weber syndrome and secondary glaucoma of left eye. METHODS: The patient underwent thorough ophthalmic tests: visual acuity, colour vision, contrast sensitivity, depth perception, ocular motor function, slit-lamp examination, tonometry, ophthalmoscopy, visual field testing, retinoscopy, 4 prism base-out test. RESULTS: Left eye with the secondary glaucoma and severe disc cupping had a normal visual acuity and the nullsoundnull right eye had reduced visual acuity with the best correction of refraction error. As there was no anisometropia or manifest strabismus to explain the amblyopia of the right eye, we looked for a monofixation syndrome. The patient had a positive 4(Delta) base-out test and reduced stereoacuity on Lang test. CONCLUSION: To our knowledge no case of Sturge-Weber syndrome has been described with microstrabismus, the preferred foveal fixation being in the glaucomatous eye with severe disc cupping.
G. Dimitrova. Clinic of Ophthalmology, University Hospital Alexandrovska, Sofia, Bulgaria.
Classification:
9.4.15 Glaucoma in relation to systemic disease (Part of: 9 Clinical forms of glaucomas > 9.4 Glaucomas associated with other ocular and systemic disorders)
