Maurizio Mete*, Grazia Pertile (Negrar, Italy)

Retinal capillary haemangioma (RCH) is one of the most common expressions of Von Hippel-Lindau (VHL) disease. In case of small- or medium-sized RCHs (up to 2 disk diameters), the standard treatment remains laser photocoagulation or cryotherapy. Various treatments have been proposed such as brachytherapy, external beam radiotherapy, transpupillary thermotherapy, photodynamic therapy and anti-VEGF by systemic or intravitreal delivery for large and multiple RCHs, RCH associated with vitreoretinal neovascularization, or combined with exudative and tractional retinal detachment. Surgery can therefore constitute salvage therapy for complicated cases of RCH. We describe 3 cases that underwent vitreoretinal surgery for tractional retinal detachment secondary to VHL. All 3 eyes underwent complete 20-gauge pars plana vitrectomy (PPV), posterior hyaloid detachment, peeling and dissection of preretinal membranes, at both the posterior pole and periphery, as well as those covering the RCHs, coagulation of the afferent arteries of the angiomatous lesions, relaxing retinectomies when needed, and silicon oil exchange. The best-corrected visual acuity (BCVA) improved in all the cases (preoperative and postoperative mean BCVA were namely 0.08 and 0.25), but after 4 to 6 months every eyes developed epiretinal membranes and underwent a second surgery. Regarding the therapeutic option, we think that less invasive therapies like cryotherapy or photocoagulation were not indicated in these cases because of the prominent antero-posterior traction that was responsable for the serous retinal detachment and the ectopia maculae. In particular, the use of anti-VEGF could increases the traction with worsening of the macula displacement.