Vitrectomy for Massive Vitreoretinal Proliferation as Associated with Von Hippel-Lindau Disease

A 13-year-old female presented progressive unilateral vision loss for a few months in the right eye. Her best corrected visual acuity (BCVA) was 20/100, and fundoscopy revealed a massive macular neovascular proliferation with a small superior temporal vascular tumor. Spectral-domain optical coherence tomography (SD-OCT) presented macular edema associated with a large and thick fibrovascular proliferation. Progressive hyperfluorescence of the tumor was observed during fluorescein angiography. Systemic evaluation revealed vascular lesion located at cerebellar tonsil, which contributed to diagnose Von Hippel-Lindau disease.

Intravitreal bevacizumabe was performed 5 days prior to pars plana vitrectomy (PPV), leading to partial fibrosis of the vascular tumor. During the fibrovascular proliferation peeling, a clivage plan was obtained. Complete remotion was performed carefully, beginning from the optic nerve toward to the macula. The material was removed throw the trocater, followed by tumor photocoagulation and fluid-air exchange. Histopathological analysis of the material revealed capilar proliferation, with immature capillaries, and areas of fibrosis, as result of anti-vascular endothelial growth factor. Immunohistochemistry analysis identified CD34 marker, which was compatible with a recent neovascular proliferation. Four months later, BCVA improved to 20/30, and a better retinal layers organization was observed on both fundoscopy and SD-OCT imaging.