Differential diagnosis of retinal vasculitis

Abstract

Retinal vaculitis is a sight-threatening inflammatory eye condition that involves the retinal vessels. Detection of retinal vasculitis is made clinically, and confirmed with the help of fundus fluorescein angiography. Active vascular disease is characterized by exudates around retinal vessels resulting in white sheathing or cuffing of the affected vessels. In this review, a practical approach to the diagnosis of retinal vasculitis is discussed based on ophthalmoscopic and fundus fluorescein angiographic findings.

Keywords: Fluorescein Angiography; Retina; Vasculitis.

Figure 1

Fundus photographs of the left eye of a 27-year-old man with strongly positive tuberculin skin test demonstrating thick perivenous sheathing with intraretinal hemorrhages

Figure 2

(a) Anterior segment of a 24-year-old woman with multiple sclerosis showing granulomatous keratic precipitates (b) Fundus photograph showing perivenous sheathing (c) Fluorescein angiogram showing focal venous leakage (d) Magnetic resonance imaging of the brain showing demyelinating lesions

Figure 3

(a and b) Fundus photographs of a 22-year-old man with documented Behçet's disease showing characteristic multiple retinal infiltrates (c) Fluorescein angiogram showing characteristic extensive leakage from all retinal vessels

Figure 4

(a) Fundus photograph of the right eye of a 45-year-old woman with strongly positive tuberculin skin test demonstrating focal sheathing (b) Fluorescein angiogram showing extensive leakage from the retinal veins and optic nerve head

Figure 5

(a) Fluorescein angiogram of a 27-year-old man with strongly positive tuberculin skin test showing peripheral capillary nonperfusion, telangiectasia, abnormal vascular anastomosis, and neovascularization (b) The neovessels demonstrate extensive leakage

Figure 6

(a) Fundus photograph of the right eye of a 23-year-old man with documented Behçet's disease showing extensive perivenous sheathing involving inferotemporal vein with intraretinal hemorrhages and hemorrhagic infarction temporal to the macula (b) Fluorescein angiogram showing delayed filling of the involved vein (c) and late leakage and staining

Figure 7

(a) Fundus photograph of the right eye of a 23-year-old man with documented Behçet's disease showing retinal infiltrates (b) Fluorescein angiogram showing leakage from retinal vessels and the multilobular pattern of cystoid macular edema

Figure 8

(a) Fundus photograph of the left eye of a 23-year-old man with documented Behçet's disease showing juxtapapillary retinal infiltrate and intraretinal hemorrhages (b) Fluorescein angiogram showing leakage from optic nerve head and retinal vessels

Figure 9

(a) Peripheral retina of a 30-year-old woman with acute retinal necrosis showing periarterial vascular sheathing and necrotizing retinitis (b) Fluorescein angiogram showing peripheral occlusive vasculopathy

Figure 10

Fundus photographs of the right (a) and left; (b) eyes of a patient with idiopathic retinal vasculitis, aneurysms, and neuroretinitis showing extensive peripapillary and macular lipid exudates deposition, aneurysmal dilatations along the retinal arterioles, and perivascular sheathing affecting the retinal arterioles

Figure 10c

Fluorescein angiograms showing the aneurysmal changes along the retinal arterioles and peripheral nonperfusion

Figure 10d

Fundus photographs 3 years after treatment with panretinal photocoagulation and systemic steroids showing regression of aneurismal dilatations and absorption of hard exudates

Figure 11

Fundus photographs of the (a) and (b) eyes of a 21-year-old woman with active systemic lupus erythematosus showing bilateral retinopathy consisting of multiple cotton-wool spots, and intraretinal hemorrhages

Figure 12

(a) Fundus photograph of the right eye of a 24-year-old woman with systemic lupus erythematosus and strongly positive lupus anticoagulant in whom increase in disease activity was manifested as a cilioretinal artery occlusion with ischemic retinal whitening in the area of the papillomacular bundle, indicating more extensive ischemia than that producing cotton-wool spots, as well as periarterial sheathing (b) Fluorescein angiogram showing occlusion of the cilioretinal artery; the area of retinal ischemia appears hypofluorescent and obscures the background choroidal flush

Figure 13

(a) Fundus photograph of the right eye showing toxoplasmic focal necrotizing retinitis adjacent to an old scar (b) Midperipheral retina showing extensive perivenous sheathing

Figure 14

(a and b) Fundus photographs of a patient with clinical diagnosis of frosted branch angiitis showing prominent sheathing of the retinal veins, scattered intraretinal hemorrhages, cotton-wool spots, and optic disc swelling and hyperemia

Figure 15

Fundus photograph of the right eye of a 19-year-old male with documented acute lymphoblastic leukaemia showing extensive sheathing of all retinal vessels producing frosted branch-like appearance (courtesy of E. Al-Kahtani, MD, Riyadh, Saudi Arabia)

Figure 16

(a) Fundus photograph of the right eye of a 25-year-old man with strongly positive tuberculin skin test demonstrating thick perivenous sheathing with intraretinal hemorrhages and hemorrhagic infarction (b) Fluorescein angiogram showing leakage and staining of the involved veins

Figure 17

(a) Fundus photograph of the right eye of a 24-year-old man with strongly positive tuberculin skin test demonstrating thick perivenous sheathing and intraretinal hemorrhages (b) Fluorescein angiogram showing retinal nonperfusion

Figure 18

(a and b) Fundus photograph of the right eye of a 28-year-old man with strongly positive tuberculin skin test demonstrating thick perivenous sheathing; with intraretinal hemorrhages and preretinal hemorrhage above optic nerve head (c and d) Fluorescein angiogram showing leakage from the retinal veins, and neovessels on optic nerve head and retinal nonperfusion

Figure 19

Fundus photograph of a 27-year-old man with strongly positive tuberculin skin test demonstrating sclerosed white retinal vessels in the periphery and neovessels Fundus fluorescein angiogram showing peripheral capillary nonperfusion, and leakage from neovessels

Figure 20

Anterior segment of a 30-year-old man with strongly positive tuberculin skin test showing extensive rubeosis iridis. This patient had, in addition, vitreous hemorrhage, retinal ischemia and retinal neovessels

Figure 21

(a) Fundus photographs of the right eye of a 25-year-old man with strongly positive tuberculin skin test demonstrating perivenous sheathing with intraretinal hemorrhages (upper left) and neovessels nasal to optic nerve head (upper right) Fluorescein angiography showing retinal nonperfusion (bottom left) Optical coherence tomography showing macular edema (bottom right) (b) Fundus photographs after treatment with systemic steroids, appropriate antituberculous therapy, and scatter laser photocoagulation (upper left and right) showing resolution of perivenous sheathing and intraretinal hemorrhages and involution of neovessels Optical coherence tomography displays normal anatomy of the macula (bottom)

Figure 22

Fundus photograph of the left eye of a 32-year-old man with strongly positive tuberculin skin test showing vitreous hemorrhage and active fibrovascular tissue on the optic nerve head and along the vascular arcades (left) Fundus photograph after pars plana vitrectomy and endolaser photocoagulation (right) showing clear vitreous cavity and involution of neovessels

Figure 23

(a) Fundus photographs of the right eye of a 27-year-old man with documented Behçet’s disease showing perivenous sheathing, intraretinal hemorrhages, and retinal infiltrates (b) Fluorescein angiogram showing delayed filling of the inferotemporal vein (upper left), late leakage and staining of the involved veins, and retinal nonperfusion (bottom left)

Figure 24

Fundus photograph of a patient with documented sarcoidosis demonstrating segmental perivenous sheathing

Figure 25

Fundus photograph of a patient with rift valley fever virus infection showing retinitis in the macular area (left); Fluorescein angiogram showing occlusive retinal vasculitis (right) (Courtesy of E. Abboud, MD, Riyadh, Saudi Arabia)