A retrospective analysis of 6 eyes of 6 patients diagnosed with subfoveal choroidal neovascularization associated with angioid streaks and treated by the authors from October 2003 through december 2006 was conducted. The diagnosis of angioid streaks was based on the typical clinical and angiographic appearance of bilateral broad, irregular, reddish brown or gray lines that radiate from the area around the optic nerve head. Patients with fundus abnormalities associated with other ocular diseases that can cause CNV, such as age-related drusen, were not included in the study. All patients underwent color photography, fluorescein and indocyanine green angiography, and complete ocular examinations with determination of best-corrected visual acuity both before treatment and at all subsequent follow-up visits. Main outcome measures were visual acuity and CNV size. Visual acuity was measured using an Early Treatment of Diabetic Retinopathy Study chart and was approximated to the nearest Snellen equivalent. The greatest linear dimension (GLD) was measured including all lesion components. Photodynamic therapy was administered using the same procedure as in the Treatment of Age-related Macular Degeneration with Photodynamic Therapy (TAP) and Verteporfin in Photodynamic Therapy (VIP) studies.7,8 Verteporfin (Visudyne; Novartis Ophthalmic AG, Hettlinger Switzerland) is reconstituted with sterile water and diluted with 5% dextrose to achieve a drug dose of 6 mg/m² body surface area (BSA) and a total infusion volume of 30 ml. The solution is infused intravenously at a rate of 3 ml/min over 10 minutes. Laser (Coherent, Palo Alto, California, USA) application is carried out at 15 minutes after the start of the infusion. The spot size is determined by measuring the greatest linear dimension of the entire lesion and an additional 1000 µm is added to this value. The time to deliver 50 J/cm² laser dose is 83 seconds. The patients were instructed to avoid direct sunlight or bright indoor light for 48 h after treatment. Retreatments were considered when active leakage was observed at 3 month intervals.

Three patients were men and three patients were women. Their mean age was 61.3±5.50 years (range, 53-68 years.) In four cases the right eye was affected and in two cases, the left eye. Follow-up time ranged from 12 to 38 months with mean of 20.5±10.91 months. Four patients were treated twice and two patients were treated once (Table 1). The mean visual acuity at baseline was 20/100 (range 20/25-20/500), and the mean visual acuity at the last examination was 20/320 (range 20/125-counting finger). Visual acuity of counting fingers was assigned a value of 20/2000. The mean GLD at baseline was 2400±766.81 µm, and the mean GLD at the last examination was 3483±444.59 µm. The mean line change was -4.0 lines (ETDRS charts). One patient improved visual acuity. No unexpected ocular or systemic adverse events were noted in either patient group.

The natural prognosis of CNV in angioid streaks has been reported to be unfavorable insofar as most eyes become legally blind by 1 years.1-3 Different therapeutic approaches have been used in the attempt to treat this vision-threatening disease. Laser photocoagulation has not yielded good results.4-6 This treatment was indicated in non-subfoveal lesions, but frequently the centre of the fovea was finally affected due to relapse of the initial lesion or enlargement of the laser scar.

PDT with verteporfin can reduce vision loss in the more common type of CNV, such as age-related macular degeneration and pathologic myopia.7,8 Thus, PDT with verteporfin has been tested in the treatment of CNV secondary to angioid streaks. The first published case reports and series of PDT in patients with angioid streaks seemed to be particularly promising. Sickenberg et al.9 documented no visual loss in a 52-year-old male patient with 3-month follow-up. Karacorlu et al.10 described eight cases with subfoveal CNV and a mean follow-up of 8 months. The results were quite good, with stabilization or improvement of visual acuity in all cases. In contrast to these reports, recently published series report somewhat less encouraging results of PDT for CNV associated with angioid streaks.12-16 Shaikh et al.12 described an increased in lesion size in seven out of nine CNV treated with multiple sessions. In the largest series published to date, Menchini et al.11 combined the results of 48 eyes out of six centers in Italy. With a minimum follow-up of 12 months, they found a decrease in visual acuity in 32% of patients with subfoveal lesions and in 50% in patients with extra- or juxtafoveal CNV. Overall, 18% had a visual acuity <20/200 at the final examination, and a 62% the lesion size determined by GLD measurements increased comparing baseline to final examination. Browning et al.13 described that PDT might limit the visual loss in the majority of patients through the first 12 months of follow-up.

In our study, treated CNV initially displayed a stabilization of visual acuity (defined as visual loss<3 lines) following PDT. Stabilization of visual acuity was achieved in four patients through the 12 months of follow-up. Visual improvement was obtained in one patient. The mean line change was -4.1 lines (ETDRS charts). Changes in GLD had a tendency to enlarge despite receiving PDT (Figure 1). The mean GLD at baseline was 2400±766.81 µm, and the mean GLD at the last examination was 3483±444.59 µm. In our experience PDT with verteporfin does not seem to be a good treatment to stabilize visual acuity and lesion size in CNV secondary to angioid streaks. However, compared to the aggressive natural course and to the limited treatment options, PDT may at least in part help to stabilize macular function over a limited period of time. We think that PDT should be considered in selected cases until better therapeutic modalities become available. In the future, modifications of the PDT treatment may have the potential to further improve the visual outcome in this population. As the combination of PDT with intravitreal triamcinolone acetonide showed promising results in a group of patients that did initially not show a good response to PDT treatment,17 and combinations of PDT with anti-angiogenic substances are currently investigated in clinical studies.18 We think that these combinations merit further investigation and might possibly enhance the results of the PDT treatment of patients with CNV and angioid streaks.