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Clinicopathologic Reports, Case Reports, and Small Case Series
September 2001

Intravitreal Triamcinolone for Refractory Cystoid Macular Edema Secondary to Birdshot Retinochoroidopathy

Arch Ophthalmol. 2001;119(9):1380-1383. doi:

Birdshot retinochoroidopathy is a chronic, bilateral uveitic disorder. Originally described by Ryan and Maumenee,1 it is characterized by posterior segment inflammation in the presence of multiple depigmented choroidal lesions symmetrically scattered throughout the postequatorial retina. The cause is presumed to be autoimmune and more than 90% of patients test positive for the HLA-A29 serotype.2 Approximately half of affected eyes develop cystoid macular edema (CME), and this represents a major cause of considerable visual loss from this condition.3 A rationale for treatment with corticosteroids has been established based on the inflammatory nature of the disease. However, systemic and periocular corticosteroids have failed to produce significant improvement in most treated patients.4 We report 2 cases of refractory CME secondary to birdshot retinochoroidopathy that were successfully treated with intravitreal injections of triamcinolone acetonide.

Report of Cases
Case 1

A 60-year-old woman was diagnosed with birdshot retinochoroidopathy 3 years prior to initial examination. She was positive for HLA-A29 and had a fundus appearance consistent with this condition (Figure 1). This included multiple creamy yellow choroidal lesions posterior to the equator bilaterally. The anterior vitreous showed mild cells with some vitreous debris.

Figure 1.
Fundus appearance of case 1 is
consistent with a diagnosis of birdshot retinochoroidopathy.

Fundus appearance of case 1 is consistent with a diagnosis of birdshot retinochoroidopathy.

At initial examination, she complained of chronic floaters with occasional photopsia. However, she noted acute blurring of vision and distortion in the left eye during the prior 6 weeks. Her best-corrected visual acuity was 20/20 OD and 20/60 OS. Intraocular pressures were 14 mm Hg bilaterally. Anterior segment examination findings were normal with the exception of mild nuclear sclerotic cataracts. Indirect ophthalmoscopy and slitlamp biomicroscopy showed a normal optic disk and retinal vasculature. The right macula was normal, and the left macula showed intraretinal thickening involving the fovea with a cystoid appearance. The retinal periphery showed symmetric birdshot lesions as described.

Fluorescein angiography was obtained and showed leakage in a petalloid pattern involving the left fovea. Optical coherence tomography (OCT) confirmed CME with intraretinal thickening measured at 540 µm.

The patient was given ketorolac topical drops 4 times a day. At 3 months, her visual acuity remained 20/60 OS and OCT showed no improvement in CME (Figure 2). The patient was offered an intravitreal triamcinolone injection to treat residual edema. Informed consent was obtained, and the patient underwent injection of 4 mg of triamcinolone acetonide (Kenalog 40; Apothecon, Princeton, NJ) in 0.1 mL. The injection was performed under topical anesthesia through the pars plana inferiorly using a 27-gauge needle. Immediately after the injection, the patient described a transient visual perturbation owing to the opaque corticosteroid compound suspended in the vitreous cavity. This had resolved over the next 2 days. Within 10 days, OCT showed reduction of macular thickness to 240 µm with improvement of visual acuity to 20/50 OS. At 2 months, CME resolved completely with a return of OCT macular thickness to 190 µm (Figure 3). Her visual acuity improved to 20/25 OS at this interval. After 6 months of follow-up, the patient maintains this level of acuity and has shown no recurrence of CME. Macular thickness remains normal at 190 µm as measured by OCT. The greatest intraocular pressure measured during the follow-up period was 18 mm Hg. The patient showed no progression of cataract during this interval.

Figure 2.
Optical coherence tomograph (OCT)
depicts extensive cystoid macular edema (arrows) that failed conservative
therapy.

Optical coherence tomograph (OCT) depicts extensive cystoid macular edema (arrows) that failed conservative therapy.

Figure 3.
Optical coherence tomograph (OCT)
after intravitreal corticosteroid injection shows near resolution of cystoid
macular edema (arrows).

Optical coherence tomograph (OCT) after intravitreal corticosteroid injection shows near resolution of cystoid macular edema (arrows).

Case 2

A 38-year-old woman was diagnosed with birdshot retinochoroidopathy on initial presentation based on characteristic fundus findings. Findings from HLA-A29 testing were positive. Slitlamp biomicroscopy showed considerable neovascularization of the right optic disc along with venous sheathing. Both maculas showed trace CME. The peripheries were notable for symmetric, creamy yellow birdshot lesions scattered throughout the postequatorial region. The vitreous showed 1+ cells bilaterally. Panretinal photocoagulation was performed on the right eye, and the patient was followed clinically for 3 years with stable visual acuity at the 20/40 level.

On an emergency visit, the patient reported acute visual loss in the right eye accompanied by central distortion during the prior week. Her best-corrected visual acuity measured 20/400 OD and 20/60 OS. Intraocular pressures were 12 mm Hg and 15 mm Hg, respectively. The anterior segments were normal with the exception of rare cells in each anterior chamber. Examination of the anterior vitreous revealed 2+ cells on the right and 1+ cells on the left. Fundus examination of the right eye showed persistent disc neovascularization with a band of new preretinal hemorrhage beneath the inferotemporal arcade. The right macula showed considerable CME. Although the left macula showed mild CME, she had remained essentially stable in this eye and was not symptomatic until visual loss occurred on the right.

Fluorescein angiography confirmed leakage from disc neovascularization as well as CME in a petalloid pattern. The OCT measured the intraretinal thickening at 290 µm. The patient was treated with further panretinal photocoagulation and a sub-Tenon injection of triamcinolone acetonide (40 mg/mL). At 1-month follow-up, her visual acuity remained at 20/200 OD. The CME showed no response to therapy and actually increased to 370 µm on OCT (Figure 4). The patient was followed up for 1 additional month without a clinical response. At this time, she was offered an intravitreal triamcinolone injection to treat residual edema. After obtaining informed consent, she was injected with 0.1 mL of triamcinolone acetonide (40 mg/mL) through the pars plana inferiorly. She experienced a transient visual perturbation from the opaque intravitreal corticosteroid suspension lasting 2 days. At 1 month, her visual acuity improved to 20/100 OD. The macular thickness was reduced to 220 µm on OCT. Her visual acuity gradually recovered to 20/50 OD at 3 months with a corresponding macular thickness of 140 µm on OCT (Figure 5). At 6 months following intravitreal corticosteroid injection, she maintains a stable macular thickness of 140 µm and visual acuity measures 20/30 OD without correction. Of note, neovascularization of the disc has shown regression at 6 months. It is unclear whether this is an effect of prior photocoagulation and/or antiangiogenic effect from the corticosteroid. Intraocular pressure never exceeded 16 mm Hg during the follow-up interval, and there was no evidence of cataract formation during this time.

Figure 4.
Optical coherence tomograph (OCT)
confirms intraretinal thickening consistent with cystoid macular edema that
also failed conservative therapy (arrows).

Optical coherence tomograph (OCT) confirms intraretinal thickening consistent with cystoid macular edema that also failed conservative therapy (arrows).

Figure 5.
Optical coherence tomograph (OCT)
after intravitreal corticosteroid confirms resolution of cystoid macular edema
(arrows).

Optical coherence tomograph (OCT) after intravitreal corticosteroid confirms resolution of cystoid macular edema (arrows).

Comment

Birdshot retinochoroidopathy often is seen with CME, a common cause of visual loss in this uveitic condition. Current treatments target the inflammatory nature of the disorder. Corticosteroids represent the mainstay of therapy, but systemic and periocular routes of administration have produced disappointing results in controlling inflammation and preserving visual acuity. Cyclosporine A has shown promise owing to its potent immunosuppressive effect, but its role has not been fully established in birdshot retinochoroidopathy.4,5 Two patients are described who had CME that responded promptly to intravitreal administration of triamcinolone acetonide, an injectable corticosteroid suspension. Both showed marked improvement in macular thickness with a corresponding dramatic increase in visual acuity maintained for 6 months of follow-up.

The rationale for intravitreal corticosteroids parallels that established for other routes of corticosteroid administration, specifically the anti-inflammatory effect. However, the intravitreal route alleviates the pharmacologic issues of penetration and bioavailability. A potent dose of medication is delivered directly to its site of action with a rapid onset. With this more aggressive approach, concerns arise regarding adverse events associated with the corticosteroid medication and the injection procedure.

Specifically, corticosteroids have been associated with a rise in intraocular pressure as well as the development of cataracts. All routes of corticosteroid administration share these risks, although the risk may be theoretically amplified with injection into the eye. The injection procedure itself introduces unique risks of endophthalmitis, retinal detachment, and hemorrhage. Larger studies of corticosteroid injections for other conditions have not shown significant morbidity associated with the intravitreal injection procedure.6,7 However, this intervention may best be reserved for those truly refractory cases that have failed standard topical, regional, and oral routes of delivery. Of note, neither treated patient experienced any adverse effects related to the drug or the injection procedure. Both experienced transient visual disturbance lasting a few days owing to the opaque nature of triamcinolone suspended in the vitreous cavity. The risks seem justified based on the failure of more conservative approaches in the presence of progressive visual loss.

Intravitreal corticosteroid injection seems to be a viable optionfor the treatment of refractory CME owing to birdshot retinochoroidopathy. Preliminary results show prompt resolution of edema with corresponding improved visual acuity. Improvement in visual acuity lags resolution of macular edema temporally, with recovery of retinal function after restoration of structural integrity. The duration of effect exceeds 6 months in both treated patients. Further study is warranted to evaluate the long-term risks and benefits associated with this promising treatment modality for CME complicating birdshot retinochoroidopathy.

Corresponding author: Jay S. Duker, MD, 750 Washington St, Box 450, Boston, MA 02111.

References
1.
Ryan  SJMaumenee  AE Birdshot retinochoroidopathy. Am J Ophthalmol. 1980;8931- 45
2.
Nussenblatt  RBMittal  KKRyan  S Birdshot retinochoroidopathy associated with HLA-A29 antigen and immune responsiveness to retinal S-antigen. Am J Ophthalmol. 1982;94147- 158
3.
Duker  JS Birdshot retinochoroidopathy. Guyer  DRYannuzzi  LAChang  SShields  JAGreen  WRedsRetina-Vitreous-Macula Philadelphia, Pa WB Saunders Co1999;565- 568
4.
Vitale  ATRodriguez  AFoster  CS Low-dose cyclosporine therapy in the treatment of birdshot retinochoroidopathy. Ophthalmology. 1994;101822- 831Article
5.
LeHoang  PGirard  BDeray  G  et al.  Cyclosporine in the treatment of birdshot retinochoroidopathy. Transplant Proc. 1988;20suppl 4128- 130
6.
Danis  RPCiulla  TAPratt  LMAnliker  W Intravitreal triamcinolone acetonide in exudative age-related macular degeneration. Retina. 2000;20244- 250Article
7.
Challa  JKGillies  MCPenfold  PLGyory  JFHunyor  ABBillson  FA Exudative macular degeneration and intravitreal triamcinolone: 18 month follow-up. Aust N Z J Ophthalmol. 1998;26277- 281Article
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