Role of PPV in Advanced Retinal Hemangioblastoma-A Systematic Review

In eyes with retinal hemangioblastomas (RH), vision loss may occur despite treatment due to recurrent macular edema, macular pucker, vitreous hemorrhage, and exudative, tractional, or combined retinal detachment. Pars plana vitrectomy (PPV) plays a critical role in managing these complications. This review aims to evaluate the outcomes of PPV in patients with RH.

We searched PubMed/MEDLINE, Embase, Cochrane Library, Scopus, Google Scholar, and Web of Science (2000 to 2025) using the keywords "retinal hemangioblastoma," "capillary hemangioblastoma," "von Hippel-Lindau retina," combined with "vitrectomy," or "pars plana vitrectomy." Given the rarity of the condition, retrospective case series were included if they had ≥4 cases. Single anecdotes and non-English articles were excluded. Two independent reviewers evaluated the studies for qualitative and quantitative synthesis.

Our search yielded 120 results, of which eight studies published between 2011 and 2024 met the inclusion criteria. All were retrospective case series (level IV evidence). A total of 107 eyes from 102 patients were analyzed, with sample sizes ranging from 4 to 23 eyes. Anatomic success, defined as retinal reattachment, ranged from 70% to 100% by final follow-up. However, many eyes remained in the low vision range despite anatomic success.

New tumor development and proliferative vitreoretinopathy (PVR)-related complications remain key challenges in long-term management. While the evidence is limited due to study design and sample size, consistent trends across the literature suggest PPV is anatomically effective, though visual outcomes remain guarded.