Purpose
To present off-label bevacizumab as a potential therapeutic modality for ocular JXG refractory to management with local corticosteroid therapy.
Methods
Retrospective case series
Results
Case 1: A four-year-old male presented with pain and decreased vision in the right eye. Examination revealed a 1-mm hyphema, which initially cleared with topical atropine 1% drops BID. Upon recurrence 1 week later, gonioscopy revealed a yellow mass on the surface of the iris (Figure 1a), and OCT demonstrated hyperreflective mass on the iris surface, abutting the trabecular meshwork (Figure 1b). Topical atropine 1% drops BID and prednisolone acetate 1% QID were used. Nonetheless, 1 month after discontinuing steroid therapy, the patient returned with recurrent, spontaneous hyphema. Growth of the iris lesion with overlying focal hemorrhage was seen (Figure 1c). Intravitreal bevacizumab (1.25mg/0.05cc) was injected via the pars plana with a 30-gauge, 0.5-inch needle. There was complete involution of the lesion (Figure 1d), with improved visual acuity. Case 2: A 6-month-old female with a history of multifocal cutaneous lesions presented with recurrent subconjunctival hemorrhages OD. There was a 3-mm elevated inferotemporal episcleral lesion (Figure 2a), with gonioscopy demonstrating involvement of the posterior peripheral cornea, angle, and iris (Figure 2b). Following failed therapy with sub-Tenon’s injection of triamcinolone acetonide (40mg/1cc) and topical prednisolone acetate 1% ophthalmic drops, off-label intracameral bevacizumab (1.25mg/0.05cc) was delivered on a 30-gauge needle. 3 months later, gonioscopy showed flattening of the epibulbar component (Figure 2c) and involution of the intraocular lesion (Figure 2d), with minimal residual scarring and no recurrent hemorrhages over the next 36 months. The patient showed reduced astigmatism and no longer required topical glaucoma drops.
Conclusions
There is presently no standard treatment for the ophthalmic manifestations of JXG. The current case series is the first report to demonstrate the efficacy of off-label intraocular bevacizumab for ocular JXG, even in refractory cases. Intraocular bevacizumab offers significant advantages in its lower associated risk of cataract and secondary glaucoma compared to local steroid therapy or radiation. Additionally, it spares the adverse effects of systemic chemotherapeutic agents.