A premature male infant (born 22 weeks, 3 days; birth weight 649g) was evaluated at 37 weeks and two days post-menstrual age with pre-plus disease and neovascularization consistent with stage 3 ROP in zone 1 in both eyes ( Figure 1 A, 1 B). In the temporal macula, an island of avascular retina was present in both eyes ( Figure 1 A, 1 B, arrow), with fluorescein angiography confirming avascularity ( Figure 1 C, 1 D; double arrows) with late leakage around the avascular island in the right eye ( Figure 1 C). Both eyes were treated with an intravitreal injection of 0.25mg bevacizumab, followed by ROP regression and normal vascularization of prior islands ( Figure 2 A, 2 B). At 64 weeks, fluorescein angiography revealed persistent avascular retina in zone 2 of both eyes ( Figure 2 C,D), which was treated with laser photocoagulation. Similar avascular islands in zone 2 were described by Narang and colleagues, although follow-up imaging did not disclose whether these avascular islands remained after the patient was treated with primary laser photocoagulation. Our report describes a unique case of avascular islands in zone 1 retinopathy of prematurity, with subsequent regression after anti-VEGF treatment. We propose the term “Avascular Island in ROP” (AIR) to describe this entity. Its features are highlighted in this report with the hope that increased awareness of this largely unreported finding may drive future research into clarifying its implications.
