Early surgical treatment of retinal haemangioblastomas: 10-year follow-up

Abstract

Retinal haemangioblastomas (RHs) are benign vascular tumours that can cause significant visual morbidity. They occur as solitary tumours or as part of von Hippel-Lindau (VHL) disease and may pose considerable challenges in treatment, depending on size, location, multifocality, bilaterality and tumour-related complications (Hajjaj et al., 2022). Small peripheral RHs (<1.5 mm) can be effectively treated with laser photocoagulation, whereas cryotherapy and plaque radiotherapy appear relatively safe and effective for peripheral tumours ranging from 1.5 to 4.5 mm in size. Anti-vascular endothelial growth factor (anti-VEGF) therapy, which may reduce exudation and inhibit RH growth, along with photodynamic therapy (PDT), are considered safer options for juxtapapillary lesions but less effective for complete destruction of peripheral RHs.

While emerging treatments such as systemic sunitinib and belzutifan show promise in managing VHL-related RHs, vitreoretinal surgery still remains inevitable for larger peripheral tumours (>4.5 mm) and tumours complicated by exudation, traction and retinal detachment. Previously, we reported on the early surgical treatment of four patients with a large peripheral RH accompanied by traction and exudation (Van Overdam et al., 2017). Here, we present an extended follow-up of at least 10 years to further discuss their clinical course (Table 1). The insights derived from this follow-up have proven beneficial for subsequent patients, especially those with a more complex presentation.