Purpose
to examine clinical and angiographic features of children diagnosed with familial exudative vitreoretinopathy [FEVR] but were born prematurely
Methods
A retrospective review of charts and angiograms of patients seen through the Pediatric Retina Service at Jackson Memorial Hospital and Bascom Palmer Eye Institute. IRB approval was obtained.
Results
Participants: We included 16 eyes of 8 infants that had progressive vascular disease clinically consistent with FEVR but were born prematurely. Then we compared them to a control set of 16 eyes of 8 infants with a diagnosis of FEVR who were born at full term. Baseline data including gestational age, birth weight were analyzed between the groups. Through angiography, we compared zones of disease, disk areas of non-perfusion, distance from the ora-serrata, features of the vascular-avascular junction, presence of arteriovenous shunts, vascular dilatation, degree of leakage, and foveal avascularity. In eyes of children with FEVR, regardless of prematurity of birth, FA clearly shows extreme variability in both retinal and choroidal filling patterns and in the clinical course of the disease despite adequate laser treatment.
Conclusions
The clinical similarities in ROP and FEVR have long been recognized. As fluorescein angiography becomes more widely available for pediatrics through the RETCAM system, it may provide valuable information in understanding the pathophysiologic similarities and differences between the diseases. In the future, genetic studies and improved imaging technologies will further increase our knowledge.
Keywords: 706 retinopathy of prematurity •
552 imaging methods (CT, FA, ICG, MRI, OCT, RTA, SLO, ultrasound)