Early Diagnosis and Management of Aggressive Posterior Neonatal Vitreoretinopathy Presenting in Premature Neonates

Abstract

Purpose : We report a case series of advanced ischemic vitreoretinopathies in premature infants that were more consistent with familial exudative vitreoretinopathy (FEVR) than with retinopathy of prematurity (ROP).

Methods : The charts of three consecutive patients who met ROP screening criteria and were diagnosed with FEVR-like, non-ROP aggressive posterior neonatal vitreoretinopathy were retrospectively reviewed.

Results : Table 1 shows clinical characteristics of each case. Severe, rapidly progressive retinal vascular abnormalities were noted in all cases (Figure 1). Average time to intervention (laser or surgery) was 39 weeks gestational age (range 35-46), and prompt treatment resulted in anatomic stabilization or improvement in the retinal detachment and maintenance of at least light perception vision in all cases.

Conclusions : This series supports the existence of a clinical entity more consistent with a FEVR-like ischemic vitreoretinopathy than ROP in premature infants and suggests that early diagnosis and intervention may mitigate the typical aggressive course and poor prognosis of this condition.

This is an abstract that was submitted for the 2018 ARVO Annual Meeting, held in Honolulu, Hawaii, April 29 - May 3, 2018.

 

Clinical characteristics of three cases of premature infants diagnosed with a non-ROP aggressive posterior neonatal vitreoretinopathy

 

Case 1- Membrane over the disc with macular detachment, retinal dysplasia, and avascularity OU (A-OD, C-OS). FA with marked avascularity, irregular sprouts of neovascularization along the vascular-avascular border, and pinpoint hyperfluorescence beyond the transition zone OU (B: OD, 12sec; D: OS, 82sec). Case 2- Retinal dysplasia with anomalous retinal vasculature and membrane over the disc and arcades with nasal retinal detachment OU (E: OD, G: OS). FA with severe retinal avascularity with irregular sprouts of vascularization beyond the vascular-avascular transition zone and neovascularization over the disc and arcades OU (F: OD, 9sec; H: OS, 42sec). Case 3- Macular tractional retinal detachment OD (I) and unremarkable OS (K). FA revealed severe retinal avascularity OD (J: 1min, 26sec) with bulbous vascular terminals, pinpoint areas of hyperfluorescence beyond the transition zone, and neovascularization from the disc to the temporal avascular-vascular junction; and peripheral avascularity OS (L: 5min, 56sec)