Aggressive posterior retinopathy of prematurity: a review on current understanding

A review of literature was performed, focused on the etiopathogenesis of aggressive posterior retinopathy of prematurity (APROP), the characteristic and atypical clinical features, management strategies, anatomical and visual outcomes. Characteristically APROP has zone I/posterior zone II involvement with prominent plus disease, featureless junction, large vascular loops, flat extra-retinal fibrovascular proliferation, and a rapidly progressive course. The risk factors for APROP are extreme prematurity (birth weight ≤1000 gram and/or gestational age ≤28 weeks), dysregulated oxygen supplementation, intrauterine growth retardation, sepsis, and thrombocytopenia. The uncommon presentations include small zone I disease, a hybrid disease with additional ridge tissue, and APROP in bigger babies with birth weight greater than 1500 g. Laser photocoagulation role is limited by the resultant visual field loss and high refractive error. Although anti-vascular endothelial growth factor injection allows peripheral retinal vascularization; reactivation of disease, systemic absorption of the drug and long-term safety are the chief concerns. Early vitrectomy is required when tractional retinal detachment develops. The visual outcome depends upon the morphology and vascular development of the macula. With the limited yet emerging new understanding of the pathophysiology, a multifaceted rational and individualized treatment strategy is suggested for APROP. Best practices in neonatal intensive care may prevent the occurrence of APROP. Further studies need to be performed for the prevention and safe, effective management of APROP.