Acute retinal pigment epitheliitis during treatment of hyperprolactinaemia.

BACKGROUND: Acute retinal pigment epitheliitis (ARPE) is a rare, idiopathic and self-limiting disease. The article aims to present ARPE in a patient using D2 dopamine receptor agonists for the treatment of hyperprolactinemia. CASE PRESENTATION: A 28-year-old female during hyperprolactinaemia treatment suffered from a dyschromatopsia and a central visual field defect in the left eye. She noticed a deterioration of vision and discontinued the cabergoline administration. The woman had not been diagnosed with other chronic conditions and exhibited no symptoms of infection. Upon admission, the patient was subjected to a test for COVID-19, which was negative. The ophthalmological examination revealed a decrease in visual acuity to distance in the left eye, which amounted to 18/20 on the Snellen chart. A central scotoma was noted on the Amsler chart and a loss of pigment epithelium was visible on the fundus of the left eye. Fluorescein angiography showed a discrete window defect in the left one, with no signs of leakage. Optical coherence tomography (OCT) scans of the maculae revealed a characteristic change in the photoreceptor layer and retinal pigment epithelium (RPE) in the fovea in the left eye. The electrophysiological tests revealed decreased function of cells in macular region. A magnetic resonance imaging (MRI) of the head and orbits demonstrated an asymmetric pituitary gland without chiasm compression and discrete signal enhancement from the left optic nerve. The patient underwent observation during hospitalisation. She reported improved colour vision and a decreased scotoma in the centre of her visual field. In regular outpatient follow-ups, successive improvements in visual acuity, as well as a decreased RPE damage and outer photoreceptor layer loss during an OCT test were observed. CONCLUSIONS: A case of ARPE is reported in a patient taking medications for hyperprolactinemia. The role of dopamine receptor antagonists in the photoreceptor function and causation of ARPE needs further evaluation.

Unilateral inferior altitudinal visual field defect related to COVID-19.

Ocular manifestations of COVID-19 are still being studied. Posterior segment involvement in viral entities is either direct viral involvement or a delayed immune response to the antigen. A 22-year-old woman presented with history of perceiving absolute inferior scotoma in the right eye for 4 days and history of fever and sore throat 10 days ago. Fundus examination revealed disc edema and vessel tortuosity. Humphreys Field Analyzer confirmed inferior field defect and Optical Coherence Tomography showed superior, nasal and inferior retinal nerve fiber layer thickening in the right eye. Patient was positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) by reverse transcription polymerase chain reaction (RT-PCR) testing. Patient received three doses of injection methylprednisolone over 3 days. There was subjective resolution of scotoma reported 3 weeks posttreatment. We bring forward the first reported case of parainfectious optic neuritis associated with COVID-19.

Diffuse Phlebitis in Patients with Syphilitic Outer Retinopathy.

Purpose: To report two cases of syphilitic outer retinopathy that showed diffuse phlebitis on fluorescein angiography (FA).Methods: Retrospective chart review.Results: The two patients had presentation similar to acute zonal occult outer retinopathy (AZOOR), including hyperautofluorescence on fundus autofluorescence and ellipsoid zone disruption on optical coherence tomography. The main difference from AZOOR was the finding of diffuse phlebitis on FA. Both patients recovered well after antibiotic and steroid treatment.Conclusion: FA is especially important for differentiating syphilitic outer retinopathy from AZOOR. Timely diagnosis and treatment with penicillin and cautious use of steroid usually lead to favorable prognosis.

Long-term Outcome of Zonal Outer Retinopathy in Punctate Inner Choroidopathy or Multifocal Choroiditis.

Purpose: To report the long-term prognosis of punctate inner choroidopathy (PIC) or multifocal choroiditis (MFC) and associated zonal outer retinopathy (ZOR).Method: Retrospective study in patients with PIC/MFC and ZOR with a clinical follow-up of 4 years or longer.Results: There were 14 patients in this study (M: F = 11:3). All patients received systemic steroid therapy. The initial and final median logarithm of minimal angle of resolution of BCVA were 1.00 and 0.22 (p = .002). Ellipsoid zone recovery was noted in all patients. The median visual field loss improved from -6.38 dB to -3.41 dB (p = .035). The median of total area of PIC/MFC lesions enlarged from 6.82 mm2 to 8.77 mm2 (p = .005). Recurrent disease was noted in 4 eyes and maintenance steroid was needed in 3 eyes.Conclusion: With steroid therapy, most patients with PIC/MFC and ZOR had good visual prognosis.

Sífilis ocular imitando una retinopatía oculta externa zonal aguda (AZOOR) / Ocular Syphilis mimicking Acute Zonal Occult Outer Retinopathy (AZOOR)

La sífilis ocular puede simular diversas patologías oftálmicas con múltiples hallazgos oftálmicos posibles. El tratamiento oportuno de estos pacientes puede minimizar el daño visual pero su diagnóstico es a menudo un desafío para el oftalmólogo. Revisamos un caso clínico de un hombre de 45 años, con una presentación rara y atípica de sífilis ocular, sus características clínicas y de imagen, diagnóstico, tratamiento y evolución. El paciente se presentaba con pérdida de visión bilateral indolora, fotopsia y escotomas periféricos con aproximadamente 1 semana de evolución. No eran visibles signos de inflamación ocular. El estudio oftalmológico reveló una presentación similar a retinopatía oculta externa zonal aguda (AZOOR). La sífilis ocular no siempre se presenta con inflamación ocular. Su presentación "similar a AZOOR" es muy rara. Por eso, siempre debemos mantener un alto índice de sospecha de esta afección, ya que el diagnóstico y tratamiento tempranos son esenciales para un mejor pronóstico

Ocular syphilis can simulate various ophthalmic pathologies with multiple possible ophthalmic findings. Timely treatment of these patients can minimize visual damage, but its diagnosis is often a challenge for the ophthalmologist. We review a case report of a 45-year-old man with a rare and atypical clinical presentation of ocular syphilis, its clinical and imaging characteristics, its diagnosis, treatment and evolution. The patient presented with bilateral painless loss of vision, photopsia and peripheral scotomas with about 1 week evolution. There were no signs of ocular inflammation. The complementary ophthalmic study revealed an acute zonal occult outer retinopathy-like ("AZOOR-like") presentation. Ocular syphilis does not always present with ocular inflammation and its presentation as "AZOOR-like" is very rare. So, we should always maintain a high index of suspicion for this condition, as early diagnosis and treatment are essential for a better prognosis

A case report of acute zonal occult outer retinopathy.

PURPOSE: To describe the diagnostic complexities of a patient with acute zonal occult outer retinopathy. CASE REPORT: A healthy female presented with acute bilateral visual field loss and photopsia. On initial examination, her visual acuity, colour vision, fundus examination and fluorescein angiography were normal. Fundus autofluorescence, however, revealed scattered hyperautofluorescence in both eyes and electrodiagnostic tests were abnormal. A differential diagnosis including acute zonal occult outer retinopathy was postulated and serology requested. The patient's past medical history included both breast and endometrial cancer and raised the possibility of cancer-associated retinopathy. A normal full-body positron emission tomography scan, negative antibodies for cancer-associated retinopathy and abnormal electroretinogram led to a diagnosis of acute zonal occult outer retinopathy. CONCLUSION: It has been more than 20 years since Gass first described the syndrome of acute zonal occult outer retinopathy. Typically affecting young, healthy females, acute zonal occult outer retinopathy is characterised by photopsia, minimal fundoscopic changes and electroretinographic abnormalities. Visual field loss is permanent and often associated with slow-progressing degeneration of the retinal pigment epithelial cells. Retinal photoreceptor dysfunction is an uncommon and unrecognised cause of acute visual loss. Acute zonal occult outer retinopathy should be considered as a differential diagnosis in those patients with deceptively normal fundal examinations, abnormal electroretinograms and visual field loss.

Multimodal Imaging of Post-Infectious Unilateral Outer Retinopathy and Choroiditis.

Purpose: To describe with multimodal imaging a case of post-infectious unilateral outer retinopathy with choroiditis. Methods: Retrospective chart review of a case of a 67-year old male who presented following the onset of viral symptoms with an acute onset outer retinopathy, small vessel leakage on fluorescein angiography, and choroidal involvement evident on indocyanine green angiography and near infrared fundus autofluorescence (NIR-AF). Work up for infectious and autoimmune etiologies was negative. Results: Treatment with IV methylprednisolone followed by high dose oral prednisone resulted in improvement in visual acuity, outer retinal reconstitution and resolution of the choroidal changes. Conclusions: Despite this presentation sharing features with both acute zonal occult outer retinopathy (AZOOR) and multifocal choroiditis (MFC), the case is highly atypical of both entities.

Treatment of Acute Zonal Occult Outer Retinopathy With Intravitreal Steroids.

BACKGROUND AND OBJECTIVE: To report on the use of intravitreal steroids in the management of acute zonal occult outer retinopathy (AZOOR), a rare disorder affecting the outer retina. PATIENTS AND METHODS: 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 1 year following intravitreal steroid treatment (range: 14 months to 63 months). RESULTS: Seven eyes received IVTA, six eyes received the dexamethasone intravitreal implant, and one eye received the fluocinolone acetonide implant. 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. CONCLUSION: Intravitreal steroids effectively achieved disease stability in patients with AZOOR. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:504-509.].

Laser Pointer-Induced Maculopathy: More Than Meets the Eye.

PURPOSE: To describe the clinical findings in patients with laser-induced retinal injury. METHODS: Consecutive patients presenting to a tertiary center between January 2014 and December 2015 following inadvertent ocular exposure to a laser pointer were included. Evaluation included a full ophthalmic examination and spectral-domain optical coherence tomography (SD-OCT). RESULTS: Seven young patients (8 eyes) were identified. Their mean age was 18.7 years (median: 16 years; range: 12 to 36 years). In most cases, patients were exposed for several seconds to a 5-mW green laser. At presentation, all patients complained of a central/paracentral scotoma. Snellen best corrected visual acuity (BCVA) at presentation ranged from counting fingers to 6/6. In 5 eyes, a round, well-defined deep yellowish-orange discoloration at the level of the retinal pigment epithelium in the foveola, ranging from 150 to 350 µm in diameter, was noted on ophthalmoscopic examination. Additional findings were macular subhyaloid hemorrhage in 2 eyes and a full-thickness macular hole with cystoid macular edema in 1 eye. In all cases, baseline SD-OCT revealed disruption involving the photoreceptor inner segment/outer segment junction/ellipsoid zone band, and extended toward the inner aspect of the retinal pigment epithelium band, ranging from focal interruption to extensive full-thickness macular hole. All patients received oral corticosteroid treatment with prednisone (0.5 to 1 mg/kg). Follow-up ranged between 2 and 12 months. Over time, improvement in visual acuity to 6/8 and 6/6 was noted in all eyes but one, which remained poor at counting fingers from 2 meters. The visual improvement was associated with complete or near-complete restoration of the integrity of macular structure noted on SDOCT. CONCLUSIONS: Commercial handheld laser pointers may inflict notable macular injury and damage vision permanently. Although good visual recovery was often noted, access to commercially available laser devices is potentially hazardous, especially to minors, and public awareness should be raised. [J Pediatr Ophthalmol Strabismus. 2018;55(5):312-318.].

Vigabatrin: Lessons Learned From the United States Experience.

Vigabatrin was introduced as an antiseizure medication in the United Kingdom in 1989 and was extensively used until 1997 when concerns arose regarding peripheral visual field loss. When the drug was approved in the United States in 2009, it carried a black box warning for the risk of permanent visual loss, and the pharmaceutical company was mandated to create a drug registry to assess for visual deficits. The vigabatrin drug registry has documented a relatively large percentage (37%) of preexisting, baseline visual deficits and a paucity (2%) of potential new visual findings. The vigabatrin vision study, a prospective, longitudinal, single-arm, open-label study, confirmed that adult patients with refractory complex partial seizures had a large number of visual deficits at baseline. An unexpected finding during the first year of therapy with vigabatrin was an increase in retinal thickness on optical coherence tomography. The experience from vigabatrin in the United States emphasizes the importance of baseline eye findings when considering the potential of drug toxicity involving the visual pathways.

Acute Zonal Occult Outer Retinopathy (AZOOR): a case report of vision improvement after intravitreal injection of Ozurdex.

BACKGROUND: AZOOR was first described by Gass in 1993 as a syndrome with rapid loss of one or more extensive zones of the outer retinal segments. It is characterized by photopsia, minimal funduscopic changes, and electroretinographic abnormalities. The efficacy of systemic steroids in treating AZOOR has been previously described and advocated by the concept of autoimmune retinopathy. However, the use of intravitreal of sustained-released steroid had not been mentioned to date. CASE PRESENTATION: A 34-year-old man had sudden onset of central scotoma and photopsia in the left eye. His visual acuity continued deteriorating. The visual field defect demonstrated bilateral enlarged blind spots and altitudinal defects. Fluorescein angiography (FA) showed nonspecific retinal inflammation, and an electroretinogram (ERG) illustrated decreased amplitude of the b wave in both eyes. Optical coherence tomography (OCT) examinations revealed parafoveal loss of the photoreceptor inner/outer segment (IS/OS) junction. Therefore, acute zonal occult outer retinopathy (AZOOR) was diagnosed. 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, consistent with the recovered IS/OS junction disruption. CONCLUSIONS: We herein report a typical case of AZOOR, suggesting that the intravitreal injection of steroid may benefit in certain patients.

[Punctate Inner Choroidopathy]. / Vnitrní teckovitá choroidopatie.

UNLABELLED: Punctate Inner Choroidopathy (PIC) is a rare idiopathic ocular inflammatory disease. It occurs mostly in young, myopic, otherwise healthy women. Symptoms of the disease include acute vision decrease associated with central and paracentral scotoma. In 80% of cases PIC occur bilaterally. We report the case of a woman at the age of twenty-five, who was treated for PIC. Due to the finding of active inflammatory deposit in the retina of the left eye and low visual acuity was recommended corticosteroid therapy with a good therapeutic effect. KEY WORDS: Punctate Inner Choroidopathy (PIC), white dot syndromes.

OCT Angiography Identification of Choroidal Neovascularization Secondary to Acute Zonal Occult Outer Retinopathy.

A 74-year-old female with acute zonal occult outer retinopathy presented with a new lesion suspicious for choroidal neovascularization (CNV) in her right eye. Optical coherence tomography angiography (OCTA) confirmed the presence of CNV. OCTA is a new imaging technique that may help guide diagnosis and management of choroidal neovascular membranes in uveitic diseases.

[Progressive scotoma with perception of photopsia]. / Progredientes Skotom mit Photopsiewahrnehmung.

Acute annular outer retinopathy (AAOR) is a very rare variant of acute zonal occult outer retinopathy (AZOOR). Both variants are characterized by the perception of a progressive visual field defect and can be accompanied by photopsia. In contrast to AZOOR, a progressive whitish and annular demarcation of the involved retina is observed in AAOR using ophthalmoscopy. Optical coherence tomography (OCT) reveals morphological changes predominantly in the outer retinal layers. Although there is no proof of an effective therapy, in the case presented here further progression could be stopped by a combination of antiviral and immunosuppressive therapy.

Improved Photoreceptor Function in Male Acute Zonal Occult Outer Retinopathy.

PURPOSE: To describe male acute zonal occult outer retinopathy (AZOOR) patients with improvement of photoreceptor structure and visual function. CASE SERIES: Medical records for eight eyes in seven patients (mean age, 36.9 years; range, 22 to 57 years) with AZOOR were reviewed retrospectively. Of the seven patients, four were treated with high-dose methylprednisolone therapy and three were not treated. All patients presented with photopsias and severe vision loss in the affected eyes. Visual acuity ranged from 0.2 to 1.5 on a Snellen decimal scale and Humphrey visual field testing showed blind spot enlargement or ring scotomas. Fundus and angiographic examinations found no specific abnormalities, leading to a diagnosis of AZOOR. Spectral domain optical coherence tomography showed attenuation of the photoreceptor inner segment ellipsoid zone. Multifocal electroretinography demonstrated that there were decreased responses at the site of the spectral domain optical coherence tomography abnormalities and corresponding visual field loss. Three patients had a spontaneous resolution with restoration of photoreceptor structure and visual function, and four patients had a visual improvement with restoration of photoreceptor structure and visual function after steroid pulse therapy. CONCLUSIONS: These results suggest that male AZOOR patients may have a tendency of visual improvement both with and without treatment.