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A. M. Hendrick, N. Mandava, H. Quiroz-Mercado, S. Oliver, V. Pelak, J. Olson; Bevicizumab for Parry-Romberg Syndrome (PRS) Associated Optic Nerve Edema: An Optical Coherence Tomography (OCT) Case Report. Invest. Ophthalmol. Vis. Sci. 2009;50(13):3261.
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To evaluate by optical coherence tomography the response to intravitreal bevicizumab optic disc nerve fiber layer edema due to PRS with vision loss.
A 26 year-old female presented with an episode of severe vision loss with incomplete resolution in her right eye. Visual acuity initially was 20/200 and was 20/30 on presentation to us 2 weeks into the episode. There was a right APD and enlargement of the blind spot with an arcuate scotoma on automated perimetry. Exam revealed disc edema hard exudate in the right eye. Remaining neurologic exam was unremarkable. Workup including MRI, lumbar punctures, and extensive serologic testing were also unremarkable. The presence right hemifacial atrophy made diagnosis very likely Parry-Romberg syndrome. 1.25mg/0.05ml of bevicizumab was injected with informed consent under standard intravitreal procedures twice 3 months apart.
Pre-injection OCT of the right eye revealed diffuse nerve fiber layer thickening at 197.21um average thickness. This improved to 146.41um after the first injection and worsened to 222.41um 5 months after the second bevicizumab injection.
The pathophysiology of PRS is poorly understood. Vision loss in PRS with optic nerve edema may be ameliorated with intravitreal bevicizumab. The OCT demonstrates improvement in the average thickness of the nerve fiber layer surrounding the optic disc with worsening edema with termination of therapy. This supports investigation into the role of VEGF induced vascular permeability in conditions of optic nerve edema.
Figure 1: OCT of right eye pre-injection. Average thickness 197.21um
Figure 2: OCT of right eye 1 month post injection. Average thickness 146.41um
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