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

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. Patient 1 was a 32-year-old woman with VHL disease and a blind fellow eye. Vitrectomy with feeder vessel ligation was performed (without vitreoschisis-induced vitreous cortex remnants (VCR) detection and removal), followed by lesion excision in the superotemporal far-periphery for recurrent exudation and traction at 17 months (Figure 1). Sixty-six months later, additional surgeries were performed to address retinal detachment due to a new RH with proliferative vitreoretinopathy (PVR) formation (following insufficient laser treatment and follow-up). More frequent follow-up visits enabled early and effective treatment of new RHs (Figure 2). Patient 2 was a 28-year-old woman without VHL disease. During the 10-year follow-up period after vitrectomy with VCR removal and lesion excision in the temporal mid-periphery, no complications or new RHs developed (Figure 3). Patient 3 was a 30-year-old woman with VHL. Sixty-nine months after vitrectomy with membranectomy, VCR removal and lesion excision in the temporal mid-periphery (without 360-degree laser), multiple RHs of varying sizes were observed and effectively lasered (12 months after the previous visit; Figure 4). Subsequently, a small inferotemporal far-peripheral retinal break with some subretinal fluid developed, managed with laser and SF6-gas. New RHs could be detected and treated earlier by more frequent surveillance in both eyes (Figure 5). Patient 4 was a 19-year-old woman without VHL. After vitrectomy with VCR removal (up to the mid-periphery) and lesion excision in the inferior far-periphery (with SF6-gas, without sclerotomy suturing), a mild vitreous haemorrhage occurred 1 week post-operatively, which resolved spontaneously. However, 8 months later, additional membranectomy was performed to release progressive traction at the inferior border of the area where VCR were removed, thereby preventing retinal detachment (Figure 6). There were no other complications, and no new RHs developed during the 10-year follow-up. Complete vitreous removal reduces the risk of post-therapeutic PVR formation. In contrast, this risk will be increased by blood-retinal barrier disruption resulting from extensive laser, cryotherapy, plaque radiotherapy or surgery for large or multiple tumours in the presence of attached vitreous. Therefore, early complete vitrectomy with repeated targeted use of triamcinolone acetonide (TA) for vitreous and VCR visualisation is recommended, including posterior vitreous detachment (PVD) induction, shaving with indentation at the vitreous base and removal of VCR from the retinal surface posterior to the vitreous base. Internal limiting membrane (ILM) peeling over the macula and around lesions may be beneficial to ensure complete removal of tumour-related epiretinal glial proliferation, preretinal fibrosis and VCR (Van Overdam et al., 2019, 2022). At the time of Patient 1's initial surgery, the role of VCR in post-operative PVR formation was not fully clarified. The complete removal of peripheral VCR was not yet part of our vitrectomy procedure, potentially contributing to the subsequent complex clinical course. This also applies, in part, to Patient 4. Additional targeted TA-staining could have revealed the presence of VCR over the retinal periphery after extending the PVD up to the vitreous base in this patient (Figure 7). Complete removal of tumours and associated tractional membranes prevents incomplete lesion destruction and subsequent tumour growth, traction and exudation (Patient 1). To reduce the risk of post-operative PVR formation, post-operative haemorrhage must be prevented (Patient 4). For better closure of feeder and draining vessels and to avoid post-operative hypotony, additional methods like feeder vessel ligation, intraocular diathermy forceps, silicon oil tamponade and sclerotomy sutures may be useful (Van Overdam et al., 2017, 2018). In addition to laser around the excision site, 360-degree laser may prevent post-therapeutic retinal detachment (Patient 3). Regular patient surveillance is vital for the early detection and effective treatment of new lesions, especially in high-risk VHL patients. Early lesion detection can be enhanced through fluorescein angiography, particularly with wide-field imaging (Figures 2 and 5). The surveillance frequency for VHL patients should be aligned with their clinical course, yet a 6–12-month interval proved too long (Patient 3).