Research Article: Clinical outcomes and predictors of response to photodynamic therapy in symptomatic circumscribed choroidal hemangioma: A retrospective case series

Date Published: May 31, 2018

Publisher: Public Library of Science

Author(s): Yeen-Fey Ho, Anne Chao, Kuan-Jen Chen, An-Ning Chao, Nan-Kai Wang, Laura Liu, Yen-Po Chen, Yih-Shiou Hwang, Wei-Chi Wu, Chi-Chun Lai, Tun-Lu Chen, Conor L. Evans.


To investigate the treatment outcomes and predictors of response to photodynamic therapy (PDT) in patients with symptomatic circumscribed hemangioma (CCH).

This retrospective case series examined 20 patients with symptomatic CCH (10 submacular CCHs and10 juxtapapillary CCHs) who underwent standard PDT (wavelength: 662 nm; light dose: 50J/cm2; exposure time: 83 sec) with verteporfin (6mg/m2), either as monotherapy (n = 9) or in association with other treatments (n = 11), of which 7 received intravitreal injections (IVI) of anti-vascular endothelial growth factor (anti-VEGF). A post-PDT improvement of at least two lines in best-corrected visual acuity (BCVA) was the primary outcome measure. Predictors of response were investigated with binary logistic regression analysis.

Seventeen (85%) patients received one PDT session, and three patients (15%) underwent PDT at least twice. Ten patients (50%) achieved the primary outcome of a post-PDT BCVA improvement of at least two lines. Macular atrophy and recalcitrant cystoid macular edema in 2 patients. Binary logistic regression analysis revealed that younger age (< 50 years) (P = 0.033), pre-PDT BCVA of ≧20/200 (P = 0.013), exudative retinal detachment resolved within one month after PDT (P = 0.007), and a thinner post-PDT tumor thickness (P = 0.015) were associated with the achievement of a post-PDT BCVA improvement. Additional treatments to PDT including IVI anti-VEGF did not appear to improve visual and anatomical outcomes. Symptomatic CCHs respond generally well to PDT. Patients with younger age (< 50 years), pretreatment BCVA≥ 20/200, and thinner foveal edema are most likely to benefit from this approach.

Partial Text

A circumscribed choroidal hemangioma (CCH) is an uncommon, benign choroidal tumor usually located at the posterior pole of the affected eye. Choroidal hemangiomas (CCHs) present as round to oval, reddish-orange choroidal tumors of varying size. They can be asymptomatic, being diagnosed during routine eye examinations or may cause decreased visual acuity because of exudative retinal detachment or cystoid macular edema (CME) [1,2,3]. Different therapeutic approaches including laser photocoagulation, transpupillary thermal therapy (TTT), radiation plaque therapy, external beam radiation, and proton beam radiation have been proposed to treat extramacular CCHs [1,2,4]. However, the use of either laser photocoagulation or TTT is limited by the risk of irreversible foveal damage associated with these methods. Moreover, post-radiotherapy radiation retinopathy is a significant concern [2,4,5]. Owing to a low likelihood of treatment-related visual loss [6,7,8,9,10,11,12], photodynamic therapy (PDT) with verteporfin is currently considered as the first-line treatment modality for patients with CCHs with macular involvement [2].

This study was a single-center retrospective case series. The protocol followed the tenets of the Declaration of Helsinki and was approved by the Institutional Research Ethics Board of the Chang Gung Memorial Hospital (CGMH, 104-A191B, 105-6752C), Linkou, Taiwan. Because of the retrospective nature of the study, the need for informed consent was waived.

Consistent with several previous studies [5,6,7,8,9,10,11,12], the results of our case series confirm that symptomatic CCHs respond generally well to PDT, because 50% of patients exhibited a significant visual improvement. In particular, we identified young age (<50 years) and a pre-PDT BCVA ≥ 20/200 as pretreatment variables significantly associated with an improved post-treatment visual acuity. Lower rates of chorioretinal atrophy or degenerative changes of the posterior pole may contribute to more favorable visual outcomes in younger patients [20,21]. In addition, exudative retinal detachment showing complete resolution within 1 month after PDT and a reduced post-PDT tumor thickness were identified as post-treatment variables associated with an improved BCVA. Although PDT is currently considered as the treatment of choice for symptomatic submacular and peripapillary CCHs [5,6,7,8,9,10,11,12], some patients cannot afford its high cost. In this context, the selection of ideal candidates who are most likely to benefit from this approach is necessary. The present study was primarily designed to address this knowledge gap. A few recent studies have investigated the effectiveness of IVI of anti-VEGF in treating symptomatic CCHs [13,14,15,16]..We also performed an exploratory analyzed whether the combination of PDT with other therapies is significantly associated with more positive outcomes than is PDT alone. Albeit preliminary in nature, our data did not indicate a significant added value of other treatments in combination with PDT when compared with PDT alone. The positive effects of PDT as monotherapy were evident despite the long delay between symptom onset and treatment observed in the current series (median duration of symptoms before presentation: 2 months). The potential correlation between symptom duration before PDT and visual outcomes remains controversial. Some authors have suggested that longer symptom duration may be associated with a poor final visual acuity (≤ 20/200) [1,2], whereas other reports have not [8,9,10,11,12]. In our study, binary logistic regression analysis did not identify symptom duration as a significant predictor of visual outcomes. In contrast, our results indicated that patients aged <50 years with a pre-PDT BCVA score of> 20/200 are ideal candidates for PDT. Notably, most of our patients required only a single PDT session to achieve an improved visual acuity. The factor ‘multiple PDT sessions’ is inversely related to favorable visual outcomes.