Idiopathic retinal arterial occlusive vasculitis in the setting of multiple arterial occlusions





Abstract


Purpose


We present a patient with vaso-occlusive retinal vasculitis to summarize this uncommon entity and review the clinical features and management challenges applicable to patients with retinal vasculitis.


Observations


A 76-year-old male presented with sudden-onset severe central vision loss. On examination, vitreous hemorrhage, neovascularization of the optic nerve, peripheral segmental periphlebitis, vessel sclerosis, vascular sheathing, and retinal hemorrhages were observed, and a diagnosis of active vaso-occlusive retinal vasculitis was made. The patient then underwent a complete infectious, inflammatory, and neoplastic workup which returned negative. The patient was treated with locally with a sub-Tenon’s injection of 40 mg triamcinolone on presentation and later with oral prednisone. At three-month follow-up, vision improved to 20/300 with regressing neovascularization and clearing of vitreous hemorrhage in the right eye (OD).


Conclusions


Considering novel associations of occlusive retinal vasculitis, it is important to recognize that idiopathic occlusive retinal vasculitis, although uncommon, can occur and represents a prototypical disease form. It is imperative that these patients have a complete infectious, inflammatory, and neoplastic workup owing to the possible overlap of masquerade clinical signs and symptoms.



Introduction


Retinal vasculitis is a sight-threatening inflammatory condition characterized by perivascular inflammation that may result in vascular occlusion. Causes of retinal vasculitis are classified as either secondary to infection, neoplasia, or other systemic inflammatory diseases. Depending on etiology, retinal vasculitis can be associated with predominant arterial or venous involvement or both. Rarely, retinal vasculitis may occur as an isolated idiopathic condition. Vision loss due to retinal vasculitis can occur secondary to cystoid macular edema and macular ischemia; moreover, retinal neovascularization can produce vitreous hemorrhage, fibrovascular proliferation, or tractional retinal detachment.


Here we present a patient with idiopathic vaso-occlusive retinal vasculitis to summarize this uncommon entity and review the clinical features and management challenges applicable to patients with retinal vasculitis.



Case presentation


A 76-year-old male with a medical history of asthma, arthritis and hypertension presented with sudden-onset severe central vision loss in the right eye (OD). Ophthalmic examination demonstrated a best corrected visual acuity (BCVA) of 2/200 OD and 20/30 in the left eye (OS). Pupillary response, intraocular pressures and anterior segment exam were unremarkable. Dilated fundus examination OD revealed vitreous hemorrhage, neovascularization of the optic disc (NVD), peripheral segmental periphlebitis, vessel sclerosis, vascular sheathing, retinal hemorrhages, and areas of retinal whitening surrounding the macula. No vitreous cells or acute vitritis was noted ( Fig. 1 ). Dilated fundus examination OS revealed mildly sclerotic vessels along with mild vitreous opacities either from vitreous hemorrhage or old inflammation. No signs of acute vitritis were present. ( Fig. 1 ). No hearing loss, tinnitus, or peripheral neuropathy was noted on history and physical examination. Optical coherence tomography (OCT) demonstrated inner retinal thinning in both eyes ( Fig. 2 ). Fluorescein angiography revealed significant late hyperfluorescence secondary to vascular leakage OD, and no abnormal late leakage OS ( Fig. 3 ).




Fig. 1


Fundus photograph of right eye (A) shows vitreous hemorrhage, neovascularization of the optic disc, peripheral segmental periphlebitis, vessel sclerosis, vascular sheathing, and trace retinal hemorrhage. Left eye (B) reveals sclerotic vessels, optic disc pallor, and mild vitreous opacities.



Fig. 2


Optical coherence tomogram illustrates inner retinal thinning secondary to macular ischemia more prevalent in right eye (A) compared to left eye (B).

Jul 10, 2021 | Posted by in OPHTHALMOLOGY | Comments Off on Idiopathic retinal arterial occlusive vasculitis in the setting of multiple arterial occlusions
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