Appeal type: Medical necessity
Decision: Overturned

Diagnosis:  Acute zonal occult outer retinopathy
Treatment:  Ozurdex

The proposed treatment is medically necessary per standard of care.

The patient is a female with acute zonal occult outer retinopathy (AZOOR), a rare form of posterior uveitis, for whom coverage of an intravitreal dexamethasone implant (67029/J7312) is requested.  The patient had previously tried and failed CellCept and has been treated with intravitreal injections of triamcinolone.  Ozurdex was previously denied as not medically necessary, as insufficient evidence was identified that Ozurdex was effective for treating AZOOR. 

Originally described by Gass in 1992, acute zonal occult outer retinopathy (AZOOR) is an uncommon retinal disorder associated with focal photoreceptor degeneration.  Common features include visual symptoms (photopsias or scotoma) with associated visual field defects and outer retinal abnormalities on imaging (spectral-domain optical coherence tomography [SD-OCT]), fundus autofluorescence (FAF), fluorescein angiography (FA), and indocyanine green angiography (ICG).  Funduscopic examination may initially appear normal, with retinal pigment epithelial (RPE) changes noted later in the disease course.  The etiology of this disease remains poorly understood, though autoimmune and infectious processes have been proposed.

Treatment of patients with AZOOR remains controversial, as neither systemic immunosuppressive nor antimicrobial therapies have shown to be consistently effective. Kuo et al. were the first report a case of a man diagnosed with acute zonal occult outer retinopathy (AZOOR) where, although his vision did not improve under the initial treatment of systemic corticosteroid and calcium channel blocker, remarkable improvement was noticed after the intravitreal injection(IVI) of Ozurdex.  They suggested that that the intravitreal injection of steroid may benefit in certain patients.

Barnes et al. subsequently reported on the use of intravitreal steroids in a retrospective case series of nine eyes of five patients with AZOOR who received intravitreal triamcinolone acetonide (IVTA), dexamethasone intravitreal implant, and/or fluocinolone acetonide implant.  Treatment response was determined by reported symptoms and multimodal imaging findings. Patients were observed for at least one year following intravitreal steroid treatment (range: 14 months to 63 months).  Seven eyes received IVTA, six eyes received the dexamethasone intravitreal implant, and one eye received the fluocinolone acetonide implant.  Most frequently, intravitreal treatments began with IVTA followed by intravitreal steroid delivery implants (dexamethasone or fluocinolone acetonide implants) once a positive response to intravitreal steroids was established.   All patients experienced disease stability or improvement based on symptomatic response and multimodal imaging findings after intravitreal steroids.  One eye developed central serous retinopathy, and another eye a choroidal neovascular membrane.  Five of nine eyes experienced ocular hypertension.  All phakic eyes developed cataracts.  The authors concluded that intravitreal steroids effectively achieved disease stability in patients with AZOOR.

In this case, a patient with AZOOR has tried and failed immunosuppressive treatment (CellCept) and had shown positive response with triamcinolone injection.  Therefore, as there is support in the peer reviewed medical literature for such treatment, coverage of an intravitreal dexamethasone implant (Ozurdex) is established as medically necessary in this case.  Ozurdex implants require less frequent intervention than triamcinolone injections.