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    Vitrectomy-Assisted Excision: An Alternative Approach of Juxtapapillary Hemangioblastoma in VHL Disease

    AAO2021 Video Program
    Retina/Vitreous

    Purpose To present an alternative approach for the diagnosis and treatment of juxtapapillary hemangioblastoma (JHB). Methods Educative video illustrating the surgical case of a JHB by multimodal imaging. A comparison with other techniques to treat extrapapillary HB, such as transpupillary thermotherapy, photodynamic therapy, and radiotherapy, is done. Results An 18-year-old woman was referred with an epiretinal membrane (ERM) in the right eye. Multiple retinal HBs were observed, and a brain MRI study confirmed Von Hippel Lindau (VHL) disease. Cryotherapy and laser were performed with suitable results; however, 4 years after, the vision decreased to 20/200 and a juxtapapillary mass associated with complex ERM and macular hole (MH) appeared. Swept source optical coherence tomography angiography revealed a high-flow lesion, with late hyperfluorescence, diagnosing a JHB. A three-port pars plana vitrectomy was undertaken, removing the lesion. Two months after, 20/70 vision was achieved, the MH remained closed and foveal contour was recovered. Conclusions The excision of HB in a juxtapapillary location is an alternative and secure choice compared to other therapies. Following the recent AAO guidelines, this pathology requires a multidisciplinary assessment.