Intravitreal ranibizumab, photodynamic therapy, and vitreous surgery for the treatment of juxtapapillary retinal capillary hemangioma

Abstract

Dear Editor, Retinal capillary hemangiomas (RCH) are capillary angiomatous hamartomas of the retina and optic nerve head. The median age of diagnosing the disease is 31 years old [1]. Although most RCH is isolated, 10–30% in the 31–40 age group are associated with Von Hippel Lindau disease (VHL). Up to 15% of RCHs are juxtapapillary, where most are located temporally [2]. They are often associated with epiretinal membrane (ERM) formation and serous and tractional retinal detachment of the macula, which leads to a poor prognosis [1]. We report a typical case of isolated juxtapapillary RCH that underwent successful combined therapy with intravitreal ranibizumab injection and photodynamic therapy (PDT) 1 week before PPV and ERM peeling surgery. A 32-year-old woman presented with reduced right eye vision and metamorphopsia for 3 months. At presentation, her best-corrected visual acuity was 20/50 and 20/25 for the right and left eyes, respectively. Right eye fundus examination revealed a reddish mass measuring 2.0 × 1.5 mm in the inferotemporal juxtapapillary region associated with macular subretinal fluid and ERM (Fig. 1a). Fig. 1 Imaging results (color fundus photography, FFA and OCT) of the patient at initial presentation. a Fundus photograph of the right eye showing juxtapapillary retinal capillary hemangioma, measured 2.0 × 1.5 mm, located inferotemporal ... Fundus fluorescein angiogram (FFA) showed early filling of the lesion with late leakage into the subretinal space. Anomalous vascular channels were seen sprouting from the lesion (Fig. 1b). Optical coherence tomography (OCT) confirmed serous detachment of the macula with vitreomacular traction (Fig. 1c). Central foveal thickness was increased to 570 µm (normal values = < 315 µm) [3]. A diagnosis of juxtapapillary retinal capillary hemangioma was made. Systemic workup for Von Hippel Lindau (VHL) disease did not reveal any other tumors. Her visual acuity continued to deteriorate to 20/100 in the subsequent 2 months. She was treated once with PDT using standard fluence at that time, with reduced diameter laser spot centering on the lesion avoiding the optic disc. There was no clinical response and her vision continued to deteriorate to 20/200 3 months after the initial PDT. Combined therapy, using standard fluence PDT and intravitreal injection of ranibizumab were performed 2 months later (7 months since presentation). Reduction of lesion size (1.5 × 1.5 mm) and vascularity was noted 1 day after the combined therapy (Fig. 2a). One week later, PPV and ERM peel were carried out and intravitreal triamcinolone injection (2 mg in 0.05 ml) was given at the conclusion of surgery. Fig. 2 Imaging results (color fundus photography and FFA) of the patient after combined treatment with injection of ranibizumab, PDT, and intravitreal surgery. a Day 1 after combined intravitreal ranibizumab injection and PDT, showing regression of the tumor ... The patient's visual acuity improved to 20/25 after successful surgery and the lesion remained stable for 12 months, up to the latest follow-up (Fig. 2b). The central foveal thickness remained reduced within normal limits at 324 μm. FFA showed hyperfluorescence in the late phase (Fig. 2c). A small superotemporal branch retinal vein occlusion developed secondary to an iatrogenic break resolved spontaneously.