Rubeosis iridis and macular hemorrhages from multiple Corkscrew retinal vessels in a patient with NF1

To report a case of a patient with NF1 presenting with ocular findings of AV malformation, multiple retinal hemorrhages, and neovascular glaucoma in the absence of retinal ischemia.

Review of the medical record was conducted in accordance with the local IRBt.

A 60-year-old female patient with diagnosis of Neurofibromatosis type1 (NF1) and sudden decrease of vision in her left eye was found to have rubeosis iridis and high intraocular pressure (IOP). On fundus exam multiple corkscrew retinal vessels and retinal hemorrhages were present in her left eye. On Optical Coherence Tomography (OCT) the foveal hemorrhages appeared as outer layer hyperreflective retinal infiltrates whereas in the parafoveal area the hyperreflectivity was present between the RPE and neurosensory retina. Fluorescein Angiogram (FA) showed normal perfusion and no areas of leakage or ischemia. Treatment with anti-angiogenics in a timely manner correlated with a good visual outcome.

We present a unique patient with NF1, rubeosis iridis, high IOP, and macular hemorrhages from multiple corkscrew retinal vessels in a well perfused retina, who underwent treatment with a single dose of intravitreal Bevacizumab and had an excellent response.