Various treatment modalities have been described for retinal capillary hemangioma. Our purpose is to present a case of juxtapapillary retinal capillary hemangioma treated with photodynamic therapy. A 69-year-old woman with no previous ocular history presented with blurred vision and photopsias in the right eye three months ago. At presentation, her best corrected visual acuity was 6/9 in the right eye and 6/6 in the left eye. The anterior segment was totally normal and IOP was normal in both eyes as well. Dilated fundoscopy revealed a yellowish, well-circumscribed, elevated area with blood vessels, on the inferior margin of the right optic disc, as optic disc edema. Fluorescein angiography and angiogram with indocyanine green confirmed the diagnosis of juxtapapillary retinal capillary hemangioma. The patient was treated with photodynamic therapy with verteporfin and three months later her visual acuity was 6/7.5 in the right eye, while the lesion was slightly smaller. These findings remained stable at the one-year follow-up. In conclusion, photodynamic therapy offers promising anatomical and functional results for juxtapapillary retinal capillary hemangioma, providing visual acuity improvement or even stabilization and restriction of enlargement of the lesion.
Retinal capillary hemangioma (RCH) or hemangioblastoma is an uncommon benign vascular tumor and may occur sporadically (54%) or as a manifestation of von Hippel-Lindau (VHL) disease (46%) [
Treatment depends on the location and size of the RCH and varies from observation to radiotherapy, cryotherapy, transpupillary thermotherapy, laser photocoagulation, photodynamic treatment (PDT), antivascular endothelial growth factor (anti-VEGF) agents, intravitreal triamcinolone, vitreoretinal surgery, or combination of treatment modalities [
A 69-year-old woman, with no previous ocular history, presented with blurred vision and photopsias in the right eye three months ago. At presentation, her best-corrected visual acuity (BCVA) was 6/9 in the right eye and 6/6 in the left eye. Dilated fundoscopy revealed a yellowish, elevated, well-circumscribed lesion with blood vessels, of about one optic disc diameter on the inferior margin of the right optic disc. There were neither peripheral lesions identified nor other ocular abnormalities. Fluorescein angiography (FA) demonstrated early hyperfluorescence of the tumor vessels and some progressive leakage in the late phase of the angiogram (Figure
(a) Through. (c) Fundus photo, fluorescein angiography late phase, and optical coherence tomography at presentation. (d)-(e) Fluorescein angiography early and late phase at presentation. (f)-(g) Indocyanine green angiography early and late phase at presentation.
After explaining to the patient the various treatment modalities against RCH and potential complications, a decision was made to treat the lesion with PDT using Verteporfin (Visudyne, Novartis Pharmaceuticals, Basel, Switzerland). Written informed consent was obtained from the patient. Standard PDT was performed using Visudyne infusion at a dose of 6 mg/m2 body surface area for 10 min. Five minutes after completion of the infusion, light exposure was performed with a diode laser (689 nm) at an intention of 600 mW/cm2 for 83 seconds. The spot size was adjusted to cover the largest diameter of the hemangioma without extension into surrounding retina. A single exposure was applied in one treatment session.
Follow-up visits, including BCVA measurement, fundoscopy, OCT, and FA, were scheduled one month and three months following PDT session and continued up to 12 months. Three months following treatment, BCVA improved to 6/7.5 with no adverse effects from the treatment. There was a slight regression of the tumor, as it is depicted on FA (Figure
(a) Through. (c) Fluorescein angiography early and late phase three months after photodynamic therapy. (d) Though. (f) Fluorescein angiography early and late phase one year after photodynamic therapy, showing stability of the lesion.
The treatment of juxtapapillary RCH remains challenging due to the vicinity to the optic nerve and is based on size and location of the lesion, as well as the presence of associated complications [
Careful observation is recommended if the RCH is very small (up to 500
Moreover, it has been found that patients with RCH have elevated ocular levels of VEGF, suggesting that RCH may depend on VEGF. Currently, in the era of anti-VEGF agents, there are several reports using anti-VEGF injections for the treatment of RCH, presenting variable results. Ach et al. used intravitreal bevacizumab for midperipheral RCH and reported that repeated intravitreal injections have inhibited the growth of the lesion [
Furthermore, combined therapy with anti-VEGF agents and PDT has shown promising results against the treatment of RCH. Mennel et al. reported a case of RCH in the right eye treated with PDT and five injections of bevacizumab, showing improvement in visual acuity and stabilization of the lesion [
Photodynamic therapy using Visudyne is a promising treatment alternative. PDT is a nonthermal, photobiochemical procedure, offering site-specific vascular occlusion and tumor destruction, causing minimal damage to the adjacent neural structures [
As choroidal and retinal hemangioma share a common histopathology in compromised angiomatous vessels, there are various reports suggesting that PDT may have encouraging results in RCH treatment [
The differential diagnosis of a juxtapapillary RCH can be difficult, as it may be easily misdiagnosed as papilloedema, papillitis, granulomatous diseases, or pepipapillary subretinal neovascularization. Therefore, fluorescein angiography is of great importance for accurate diagnosis. Additionally, in cases of RCH, it is essential to rule out the possibility of VHL disease, examining carefully the patient for presence of additional angiomas in the peripheral retina. Furthermore, systemic evaluation should be performed to exclude the presence of tumors in the brain, spinal cord, kidney, and adrenal glands [
Taking as a whole, herein we report a case of juxtapapillary RCH treated with a single session of PDT with Visudyne, presenting improvement in visual acuity from 6/9 to 6/7.5 and slight shrinkage of the lesion size. The patient’s visual acuity, as well as fluorescein angiography image, remained stable at the one-year follow-up, suggesting that PDT may be a treatment alternative with promising results against juxtapapillary RCH. Further studies with large number of patients are needed to reach a safe conclusion.
The authors declare no conflict of interests.