Fulminant Progressive Atypical Acute Zonal Occult Outer Retinitis

Purpose: To report a case of unilateral progressive atypical acute zonal outer retinitis in a healthy young female.Case summary: A 32-year-old healthy woman presented with visual disturbance in the left eye for 1 week. Best corrected visual acuity was 0.4 in the left eye. No abnormal findings were detected on brain magnetic resonance imaging and a laboratory work-up. Zonal retinal opacity around the optic nerve disc was noticed in a fundus photo which was consistent with the outer retinal signal defect on optical coherence tomography and a defect in the central visual field from perimetry. Multiple evanescent white dot syndrome was suspected based on fluorescein angiography and indocyanine green angiography. Prednisolone was prescribed. Visual acuity was reduced to hand-motion after 10 days with extended zonal retinal opacity. No virus was detected by multiplex polymerase chain reaction of the aqueous humor. The lesion did not improve despite high-dose intravenous steroid and antiviral treatment.Conclusions: Visual function was lost with the progression of outer retinitis. This case differed from previous cases as the outer retinitis progressed rapidly from the posterior pole to the peripheral retina. It was named fulminant progressive atypical acute zonal outer retinitis.

Paraneoplastic Focal Outer Retinitis and Optic Neuropathy in a Patient with Small Cell Lung Carcinoma and Anti-CRMP5, Anti-HU and Anti-Amphiphysin Antibodies

Our aim is to describe clinical and optical coherence tomographic features of acute paraneoplastic focal outer retinitis associated with optic neuropathy in a patient diagnosed with small cell lung carcinoma. Bilateral focal outer retinitis, bilateral optic disc oedema and vitritis were identified in a patient with progressive bilateral visual loss and ataxia. Spectral domain optical coherence tomography (SD-OCT) revealed various extents of granular hyperreflectivity and atrophy of the macular outer retinal layers. Serum and cerebrospinal fluid positivity for anti-CRMP5, anti-HU and anti-amphiphysin antibodies intensified the search for an underlying malignancy, and a small cell lung carcinoma (SCLC) was finally diagnosed. Local and oral steroids along with systemic chemotherapy and radiotherapy achieved cancer remission and partial recovery from the ophthalmic manifestations. Focal outer retinitis associated with optic disc oedema and antibody positivity facilitated the diagnosis of SCLC in a localized stage. SD-OCT allowed the characterization of this entity, expanding the spectrum of paraneoplastic ophthalmological syndromes.

Recurrent Photoreceptor Loss with Spontaneous Recovery as a Presenting Sign of Syphilitic Outer Retinopathy

A 64-year-old male presented with sudden onset of decreased vision with a central blind spot and shimmering visual symptoms without significant inflammatory findings, and was noted on optical coherence tomography to have localized loss of photoreceptors, which spontaneously resolved after two weeks. This recurred subsequently three times spaced by months of recovery with normal vision and normal photoreceptors. In the fourth episode, the patient developed an arcuate-shaped area of outer retinitis more typical for acute syphilitic posterior placoid chorioretinitis, and the diagnosis was made serologically. With subsequent treatment for syphilis, there was not any recurrence after the treatment and vision recovered to 20/20 with a normal photoreceptor layer.