A 39-year-old male patient with decreased vision for 3 days was found to have cystoid macular edema (CME) secondary to papillophlebitis (partial central retinal vein occlusion). Systemic investigation revealed an MTHFR gene mutation. Visual acuity at presentation was 20/200. Color fundus photography revealed optic disc edema, widespread retinal hemorrhage, and tortous dilatation of venous vessels.
Color fundus photography, multicolor image and autofluorescence image of the case with CME secondary to papillophlebitis (partial central retinal vein occlusion)
At presentation, there was a CME appearance on OCT and late fluorescein angiography (FA). In addition, FA showed leakage from retinal vessels and blockage of fluorescence due to hemorrhages.
OCT of the case with CME secondary to papillophlebitis (partial central retinal vein occlusion)
Early and late phase FA of the case with CME secondary to papillophlebitis (partial central retinal vein occlusion)
One week after intravitreal anti-VEGF application, the edema was remarkably reduced, as seen on OCT and multicolor images.
Papillophlebitis is an uncommon ophthalmological pathology with an uncertain etiology. Firstly it was described as an entity like the atherosclerotic central vein occlusion and was misdiagnosed as optic neuritis and papilledema. In fact, this disorder is a syndrome with unilateral optic disc edema and retinal hemorrhages in varying degrees with engorgement and tortuosity of the retinal veins in a healthy adult. Basically it is a subtype of central retinal vein occlusion. Pathophysiologically, it is thought that there is an inflammation of the central retinal vein in the optic nerve head or retrolaminar region. Papillophlebitis is characterized by painless unilateral disc edema and hyperemia, retinal venous engorgement, and a variable extent of intraretinal hemorrhage and macular edema in otherwise healthy adults younger than 50 years. Papillophlebitis may lead to macular edema. There remains no evidence based and universally accepted treatment modality for papillophlebitis. In cases of profound vision loss or macular edema, intravitreal anti-VEGF treatment can be considered.
Credit: M. Giray Ersoz, MD, FEBO
Biruni University School of Medicine, Department of Ophthalmology, Istanbul, Turkey
Instagram accounts: @retina.review and @retina.dr.girayersoz