Rapid and durable recovery of visual function in a patient with von hippel-lindau syndrome after systemic therapy with vascular endothelial growth factor receptor …
Ophthalmology, 2002•Elsevier
PURPOSE: To present a case of rapid and durable recovery of visual function in a patient
with von Hippel-Lindau syndrome and optic nerve head hemangioblastoma after systemic
treatment with the vascular endothelial growth factor (VEGF) receptor inhibitor SU5416.
DESIGN: Interventional case report. METHODS: Visual function parameters, including visual
acuity, automated visual field, and contrast sensitivity, were evaluated using standardized
methods repeatedly over a 15-month period. Fundus photographs and fluorescein …
with von Hippel-Lindau syndrome and optic nerve head hemangioblastoma after systemic
treatment with the vascular endothelial growth factor (VEGF) receptor inhibitor SU5416.
DESIGN: Interventional case report. METHODS: Visual function parameters, including visual
acuity, automated visual field, and contrast sensitivity, were evaluated using standardized
methods repeatedly over a 15-month period. Fundus photographs and fluorescein …
PURPOSE
To present a case of rapid and durable recovery of visual function in a patient with von Hippel-Lindau syndrome and optic nerve head hemangioblastoma after systemic treatment with the vascular endothelial growth factor (VEGF) receptor inhibitor SU5416.
DESIGN
Interventional case report.
METHODS
Visual function parameters, including visual acuity, automated visual field, and contrast sensitivity, were evaluated using standardized methods repeatedly over a 15-month period. Fundus photographs and fluorescein angiograms were also obtained. Central nervous system lesions were monitored by computed tomography (CT) and magnetic resonance imaging (MRI) scans. Treatment involved the systemic administration of the VEGF receptor inhibitor SU5416.
MAIN OUTCOME MEASURES
Clinical presentation, visual acuity using Early Treatment Diabetic Retinopathy Study protocol, Humphrey automated perimetry, (Zeiss Humphrey Systems, Dublin, CA) Vistech contrast sensitivity (Vistech Consultants Inc., Dayton, OH) Farnsworth (Farnsworth-Munsell Color Services, New Windsor, NY) dichotomous panel D-15, retinal photography, fluorescein angiography, and CT and MRI scans.
RESULTS
Within 4 weeks of therapy, visual acuity had improved from 20/32−2 to 20/16−1, the visual field had expanded from being circumferentially constricted to within 8° of fixation to normal, and contrast sensitivity improved in all but the lowest spatial frequency (1.5 cycles/degree). No change was observed in lesion size by fundus photography. Improvement has been maintained over 18 months with intermittent SU5416 therapy.
CONCLUSIONS
We report rapid, extensive, and durable recovery of visual function after systemic administration of the VEGF receptor inhibitor SU5416 to a patient with VHL syndrome and optic nerve head hemangioblastoma. These findings suggest that continued evaluation of VEGF inhibitors for ocular neovascular disorders is warranted.
Elsevier
