ABSTRACT Aggressive retinopathy of prematurity (A-ROP) is a rapidly progressive and vision-threatening variant of retinopathy of prematurity (ROP), characterized by posterior location, severe vascular dilation and tortuosity, and the absence of classical staged evolution. With increasing survival of extremely premature infants worldwide, the incidence of A-ROP has risen, particularly in low- and middle-income countries, where neonatal care standards are variable. Understanding the epidemiology and risk factors is essential, as affected infants are often more mature or heavier than those classically at risk, highlighting the multifactorial nature of the disease. Clinical recognition is challenging due to atypical presentations, including “rush disease” and bleb-like posterior detachments, which require a high index of suspicion. Advances in imaging and the updated International Classification of ROP – Third Edition have refined diagnostic criteria and standardized terminology. Pathophysiologically, A-ROP is driven by severe retinal hypoxia and vascular endothelial growth factor (VEGF)-mediated angiogenesis, resulting in aggressive neovascular proliferation and fibrovascular contraction. Management strategies have evolved considerably. Laser photocoagulation, while historically effective, is limited in posterior disease and associated with high myopia and visual field loss. Anti-VEGF therapy has emerged as the preferred first-line treatment, offering rapid regression and preservation of peripheral vascularization, though late recurrence and systemic safety remain concerns. Surgical intervention, particularly early lens-sparing vitrectomy (LSV), is reserved for advanced stages and atypical variants such as bleb-like detachments, but prognosis remains guarded in total retinal detachments. This review synthesizes current evidence on terminology, epidemiology, clinical spectrum, and management of A-ROP, emphasizing early recognition and individualized multimodal therapy to optimize outcomes.
Bhavik Panchal (Thu,) studied this question.