A case of atypical retinopathy of prematurity with coexisting occlusive vasculitis

Purpose: To report an atypical case of retinopathy of prematurity (ROP) presenting with severe retinal vascular changes in a preterm infant who did not meet conventional criteria for aggressive ROP. Observations: A male infant, born at 32 weeks and 6 days of gestation with a birth weight of 1400 g, developed significant retinal changes by 4 weeks of postnatal age. Fundus examination revealed bilateral severe plus disease, flat neovascularization, multiple retinal hemorrhages, severe vascular sheathing, extensive perivascular deposits, and arteriovenous shunting within zone I. These findings were atypical and resembled occlusive vasculitis, differing from classical aggressive ROP. During hospitalization, he received non-invasive oxygen therapy for 13 days, with oxygen saturation in the target range of 90–94 %. Although his general condition remained stable and there were no signs of infection, serum interleukin-6 levels were elevated at birth, suggesting possible perinatal inflammation. Intravitreal ranibizumab (0.2 mg) was administered on the day of diagnosis. Retinal vascular abnormalities gradually resolved over 4 weeks following treatment. No recurrence or complications were observed up to a postmenstrual age of 70 weeks. Conclusions and importance: This case shows that severe and unusual retinal changes can develop even in premature infants who are not considered high risk for aggressive ROP based on gestational age or birth weight.Fluctuations in oxygen levels and inflammation may play a role in these unusual forms of ROP, and further research is needed to better understand these causes.