Indocyanine green-enhanced transpupillary thermotherapy for juxtapapillary retinal capillary hemangioblastoma

Abstract

Purpose: To study the clinical presentation and treatment outcomes of indocyanine green-enhanced transpupillary thermotherapy (ICG-TTT) for treatment-naïve juxtapapillary retinal capillary hemangioblastoma (JRCH).

Methods: A prospective interventional case series. The technique involved ICG dye infusion 45 seconds prior to application of TTT. The main study outcomes were local tumor control, resolution of subretinal fluid (SRF), and improvement in best-corrected visual acuity (BCVA).

Results: Eight eyes of seven patients (5 males and 2 females) were included. The mean age was 26 years (range: 5-56 years). Systemic evaluation revealed von-Hippel Lindau (VHL) disease in five patients. The most common location was the temporal aspect of the optic disc (5 eyes). The mean basal diameter was 2.9 mm (range: 1-8 mm), and tumor thickness was 1.4 mm (range: 1-4 mm). All eight eyes were treated with multiple sessions of ICG-TTT (mean: 3 sessions). Six eyes received adjuvant intravitreal injection of dexamethasone implant (4 eyes) and/or bevacizumab (4 eyes). Post treatment, six eyes (75%) had tumor regression with reduction of SRF. One eye had a partial response with persisting SRF, and one eye showed poor response to TTT for which external beam radiotherapy was performed. At the last follow-up (median: 11 months; range: 6-29 months), the BCVA remained stable in seven eyes and improved in one eye (hand motion to 20/40).

Conclusion: Multiple ICG-TTT sessions can be considered as an alternative treatment option for JRCH with effective local tumor control and SRF resolution.

Figure 1

(a–l): A 34-year-old male presented with bilateral JRCH with macular edema with BCVA of 20/60 and 20/20 in OD and OS, respectively. Fluorescein angiography (FA) demonstrated early hyperfluorescence of the tumor vessels and progressive leakage in the late phase of the angiogram, as shown in Figs. 1(b), 1(c), 1(h), and 1(i). He underwent ICG-guided TTT with intravitreal bevacizumab in both eyes. At 6 weeks follow-up, SRF was persisting; hence, a repeat sitting of ICG-TTT with intravitreal dexamethasone implant was given. At 12 weeks, the BCVA was stable with complete resolution of SRF and tumor control

Figure 2

(a–d): A 5-year-old girl presented with small focal peripheral RCH in the right eye and a large JRCH involving >270° of parapapillary area with SRF. Her BCVA was 20/30 in the right eye and CF CF in the left eye. There was complete regression of lesion in the right eye following one sitting of TTT, while multiple ICG-TTT sessions (a total of 7) were required for the left eye every 8–10 weeks interval along with intravitreal injection of dexamethasone. At the last follow-up, the BCVA remained stable with partial regression of lesion and persisting SRF. (e–h): An 11-year-old boy presented with a large JRCH in the left eye. At presentation, his BCVA was CF CF in the left eye. He underwent one sitting of ICG-TTT with intravitreal bevacizumab, following which there was increase tumor exudation and massive inferior exudative retinal detachment. Considering the size, location, and inferior exudative RD, he was referred for external beam radiation treatment (EBRT). At 2 months post treatment, BVCA remained stable with resolving exudative RD and partial tumor regression

Figure 3

(a–d): A 28-year-old male presented with a small JRCH in the left eye. His BCVA was No PL in the right eye and 20/20 in the left eye. He underwent three sittings of ICG-TTT with an intravitreal dexamethasone implant in the left eye at an interval of 2 months. At the last follow-up, the tumor showed regression with resolution of SRF and stabilization of vision. (e–h): A 24-year-old female was diagnosed to have left eye JRCH with presence of SRF and intraretinal fluid. Her BVCA was No PL in the right eye and 20/20 in the left eye. She was treated with one sitting of ICG-TTT, following which the tumor remained stable with resolution of SRF and stable vision

Figure 4

(a–d): A 56-year-old male presented with exudation and subretinal hemorrhage involving the macula in the right eye. A detailed evaluation showed the presence of an exophytic variant of JRCH with BCVA of hand motions in the right eye. The patient was treated with two sittings of ICG-TTT. At the last follow-up, BCVA improved to 20/40 with complete resolution of SRF and regression of the tumor. (e–h): A 22-year-old male presented with a small, solitary JRCH in the left eye involving the inferotemporal quadrant of the optic disc. He has lost vision in the right eye follwing a failed vitreoretinal surgery for RCH. His BCVA was No PL in the right eye and 20/20 in the left eye. He underwent two sittings of ICG-TTT with intravitreal bevacizumab in the left eye. At last follow-up, the the tumor regressed with stabilization of vision