Abstract

Circumscribed choroidal haemangioma (CCH) is not usually associated with systemic manifestations (Witschel & Font 1976). It generally presents as an orange-red mass similar to the surrounding choroid and located posterior to the equator (Fig. 1A). When complicated by exudative retinal detachment, subretinal fluid or cystoid macular oedema, CCH may cause visual loss and metamorphopsia. Treatment consists of laser photocoagulation, radiotherapy, photodynamic therapy or transpupillary thermotherapy (TTT) (Jurklies et al. 2003; Gunduz 2004). We retrospectively evaluated the effectiveness and safety of TTT in a large case series (25 eyes of 25 patients with CCH) treated between June 2003 and September 2014 using the TTT technique reported for choroidal melanoma (Forte & Cennamo 2008). Mean treatment age was 55.2 years (range: 34–88). Patients underwent ophthalmological examination, biomicroscopy, tonometry, fundus photography, A-scan and B-scan ultrasonography and optical coherence tomography (OCT) 1 week, and 1 and 3 months post-TTT, then every 3 months in year 1 and every 6 months thereafter (mean follow-up: 49 months; range: 5–135 months). Visual acuity was defined as a variation of at least three Snellen lines, and stable when changes were below ±2 Snellen lines. Treatment end-point was tumour regression and resorption of subretinal fluid with minimal changes in the retina overlying the tumour and in the normal tissue surrounding the tumour. Patients were re-treated if subretinal exudation persisted. Fifteen of 25 CCH were in the posterior pole, four in the macular area, two in the inferior quadrants, three in the superior quadrants and one in the nasal quadrants. Patients complained of visual loss, photopsia or metamorphopsia. Visual symptoms were related to exudative retinal detachment involving the macula in five patients (20%), to macular oedema in two patients (8%) and to fibrovascular retinal fibrosis in one patient (4%). Mean baseline BCVA was 0.36 (range: 0.005–1.0). Fluorescein and indocyanine green angiography performed in five cases revealed minimal hyperfluorescence in the prearterial and arterial phases, moderate hyperfluorescence in the venous phase and extreme hyperfluorescence in the late phase in all five cases (Fig. 1B,C). Mean baseline tumour thickness measured by A-scan ultrasonography was 3.52 mm (range: 2.01–4.65 mm). B-scan showed a high echogenic structure without shadowing and a dome-shaped tumour (Fig. 1D). Ultrasonography showed high internal reflectivity on A-scan (85–100%) with a regular internal structure, no shadowing and vascularity (Fig. 1E). OCT scan through the tumour showed an elevation of the retina–choroid complex and subretinal and intraretinal fluid (Fig. 1F,G). All tumours regressed after TTT (Fig. 1H). Fluorescein angiography and indocyanine green angiography revealed, respectively, extreme hyperfluorescence and hypofluorescence in the scar (Fig. 1I,J). Final visual acuity was improved or stable in 17 of 25 patients (85%); mean tumour thickness decreased from 3.52 to 1.8 mm (p < 0.001) (Fig. 1K,L). Three months post-treatment, mean BCVA was 0.4 (range: 0.008–1). It was improved or stable in almost all patients: in two of four patients with a macular CCH, in 14 of 15 patients with a CCH at the posterior pole and in all patients with a CCH in other sites. Also mean tumour thickness was significantly reduced (1.8 mm; p = 0.0003). A mean of two treatments was administered (85% of patients were treated once, 10% twice and 5% three times). Four patients with macular CCH underwent post-TTT retrobulbar injection of steroids because of persistent subretinal or intraretinal fluid. Post-treatment, preretinal fibrosis occurred in three patients (12%) (Fig. 1M), fibrovascular retinal fibrosis in two (8%) and subfoveal detachment in three (12%). Macular oedema occurred in four patients (16%) who thus underwent retrobulbar steroid injection, and branch retinal vein occlusion occurred in one case (Fig. 1I,J), which was successfully treated by argon laser photocoagulation. In conclusion, TTT is effective and safe for symptomatic CCH not involving the fovea.

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