J Korean Ophthalmol Soc.  2016 Dec;57(12):1964-1969. 10.3341/jkos.2016.57.12.1964.

A Case of Repeated Dexamethasone Implantation in a Suspected Patient with IRVAN Syndrome

• Kang MS
• Kwak HW
• Kim ES
• Yu SY

• KMID: 2362717
• DOI: http://doi.org/10.3341/jkos.2016.57.12.1964

Abstract

PURPOSE
In the present study, a case of repeated intravitreal dexamethasone implantation for a suspected idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome associated with recurrent exudative retinal detachment and macular edema is reported.
CASE SUMMARY
A 39-year-old female who underwent steroid pulse therapy due to Vogt-Koyanagi-Harada disease in the left eye was referred for exudative retinal detachment and macular edema. Best corrected visual acuity (BCVA) was 1.0 in the right eye and 0.5 in the left eye. Cystoid macular edema combined with serous retinal detachment was observed on spectral-domain optical coherence tomography. Fluorescein angiography revealed neovascularization and multiple macroaneurysms with fluorescein leakage in the left peripapillary area. Severe peripheral capillary non-perfusion and fluorescein leakage were also observed in both eyes. Intravitreal dexamethasone implantation was performed in the left eye and macular edema showed wax-and-wane pattern. No edema was observed after 4 additional dexamethasone implantations, however, preretinal hemorrhage occurred in the peripapillary area during treatment. Seventeen months after initiation of treatment, BCVA was 0.6 in the left eye and dry macula was maintained.
CONCLUSIONS
Repeated intravitreal dexamethasone implantation was effective for recurrent macular edema in a patient suspected with IRVAN syndrome.

Keyword

Dexamethasone implant; IRVAN syndrome; Macular edema; Ozurdex®

MeSH Terms

Adult
Aneurysm
Capillaries
Dexamethasone*
Edema
Female
Fluorescein
Fluorescein Angiography
Hemorrhage
Humans
Macular Edema
Retinal Detachment
Retinal Vasculitis
Retinitis
Tomography, Optical Coherence
Uveomeningoencephalitic Syndrome
Visual Acuity
Dexamethasone
Fluorescein

Figure

• 

  Figure 1. Serial changes of fundus color photographs and optical coherence tomography images of left eye. At the first visit, fundus color photograph showed normal fundus of right eye (A) and aneurysmal dilatations and neovascularization along the retinal arteriolar tree and optic nerve head with intraretinal exudates (yellow arrowhead) on left eye (B). Optical coherence tomography image showed no macular abnormalities on right eye and macular edema combined with serous detachment of retina on left eye. At 2 month after first implantation, macular edema was reduced (C). At 3 months after first implantation, macular edema recurred and second implantation was performed (D). At 3 months after second implantation, complete resolution of macular edema showed and pan-retinal photocoagulation was performed (E). Macular edema recurred at 4 months after second implantation and third implantation was performed (F). At 3 months after third implantation, pre-retinal hemorrhage and macular edema appeared and fourth implantation was performed (white arrowhead) (G). At 2 months after fifth implantation, macular edema got resolved and a moderate cataract was observed (H).

• 

  Figure 2. At the first visit. (A) Early fluorescein angiography (FA) reveals neovascularization and macroaneurysms clustered at optic nerve (yellow arrowheads). (B) Indocyanine green angiography of early phase reveals multiple aneurysmal dilatations along the upper temporal and lower nasal retinal arterioles (red arrowheads). (C) FA on late phase demonstrates marked increase of leakage. Wide-field fluorescein angiography reveals peripheral retinal capillary nonperfusion and leakage on right eye (D) and left eye (E) (yellow dot line).

Reference

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