Syphilitic Outer Retinopathy: A Case Report and Review of the Literature

2021 ◽  
pp. 247412642110183
Author(s):  
Claudia Amaral ◽  
Leilani Joy ◽  
Hiram Jimenez ◽  
Yousef J. Cruz-Inñigo ◽  
Jan P. Ulloa-Padilla ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Case Report ◽  
Vision Loss ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Oral Prednisone ◽  
Posterior Uveitis ◽  
Central Scotoma ◽  
Demarcation Line

Purpose: This work presents a case of syphilitic outer retinopathy with findings similar to those of acute zonal occult outer retinopathy (AZOOR). We also discuss the clinical characteristics, treatment, and prognosis of this entity. Methods: A case report and systematic literature review are presented. Results: A 56-year-old woman presented with acute vision loss, localized photopsia, a central scotoma, and retinal findings that were all consistent with AZOOR. A further workup led to a diagnosis of syphilis. Oral prednisone and intravenous penicillin resulted in the resolution of the posterior uveitis and the restoration of visual acuity. However, the central scotoma remained at the 3-year follow-up visit. Conclusions: Syphilitic outer retinopathy is a distinct entity characterized by the disruption of the ellipsoid zone visible on optical coherence tomography and a corresponding increase in fundus hyperautofluorescence in the affected areas. Although some patients may present with a demarcation line, as is seen with AZOOR, the fundus is oftentimes unremarkable or may show only subtle retinal pigment epithelium changes. Uveitis resolution and visual acuity restoration may be expected following treatment; however, visual field disturbances may persist.

scholarly journals Combined Hamartoma of the Retina and Retinal Pigment Epithelium in a Patient with Gorlin Syndrome: Spontaneous Partial Resolution of Traction Caused by Epiretinal Membrane

2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
José L. Sánchez-Vicente ◽  
Miguel Contreras-Díaz ◽  
Trinidad Rueda ◽  
Enrique Rodríguez de la Rúa-Franch ◽  
Fredy E. Molina-Socola ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Retinal Pigment Epithelium ◽  
Epiretinal Membrane ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Spontaneous Resolution ◽  
Fundus Examination ◽  
Gorlin Syndrome ◽  
Clinical Context

Purpose. To describe the case of spontaneous resolution of epiretinal membrane in a patient with Combined Hamartoma of the Retina and Retinal Pigment Epithelium (CHR-RPE), in the clinical context of Gorlin Syndrome (GS).Methods. Observational case report of a 12-year-old female patient is presented. The diagnosis of CHRRPE was made by OCT and fundus examination, which showed a mound of disorganized tissue originating from retina and retinal pigment epithelium. Epiretinal membrane (EM) was also detected. Genetic study was performed to confirm the diagnosis of GS.Results. The patient was observed for 39 months, showing spontaneous resolution of the traction caused by the EM and improvement in visual acuity (VA), which was 20/80 at initial presentation, rising to 20/40 after follow-up period.Conclusions. The presence of EM in CHR-REP is a cause of reduction of visual acuity. Management of this condition is controversial; however, we would like to highlight that spontaneous resolution of the traction caused by EM is possible, resulting in recovery of VA.

Acquired retinoschisis and vitreous hemorrhage as unusual findings in choroideremia: Case report

2020 ◽  
pp. 112067212094657
Author(s):  
Dario Pasquale Mucciolo ◽  
Vittoria Murro ◽  
Dario Giorgio ◽  
Andrea Sodi ◽  
Ilaria Passerini ◽  
...  
Keyword(s):  
Case Report ◽  
Retinal Pigment Epithelium ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Vitreous Hemorrhage ◽  
Advanced Stage ◽  
Vascular Abnormalities ◽  
Diagnostic Difficulties ◽  
The Right

Purpose: To report a case of choroideremia characterized by peripheral retinoschisis with vascular abnormalities and vitreous hemorrhage. Observations: A 58-year-old man affected by advanced-stage choroideremia was diagnosed with peripheral retinoschisis in both eyes. Vitreous hemorrhage was present in the right eye with a peculiar clot-like lesion at the periphery. At the 1-year follow-up, the vitreous hemorrhage had reabsorbed and the vascular clot-like lesion in the periphery had almost completely disappeared. Conclusion and importance: We have reported fundoscopic and OCT features of peripheral-acquired retinoschisis with vascular abnormalities in a patient with choroideremia. OCT examination is extremely useful in clinical evaluation of the peripheral retinal alterations in these cases, where the absence of the retinal pigment epithelium and the choriocapillaris pose many diagnostic difficulties.

Diagnosis and treatment of peripheral exudative haemorrhagic chorioretinopathy

2019 ◽  
Vol 104 (6) ◽  
pp. 874-878 ◽  
Author(s):  
Sarah Vandefonteyne ◽  
Jean-Pierre Caujolle ◽  
Laurence Rosier ◽  
John Conrath ◽  
Gabriel Quentel ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Multimodal Imaging ◽  
Chart Review ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Treatment Options ◽  
Treatment Strategies ◽  
Choroidal Melanoma ◽  
Endothelial Growth Factor

PurposePeripheral exudative haemorrhagic chorioretinopathy (PEHCR) is a rare disorder that is often misdiagnosed. The aim of this study was to better characterise PEHCR and to assess treatment options.Material and methodsRetrospective multicentric chart review.ResultsOf 84 eyes (69 patients) with PEHCR referred between 2005 and 2017, the most common referral diagnosis was choroidal melanoma (41.3%). Bilateral involvement was found in 21.7% of cases. Haemorrhagic retinal pigment epithelium detachment was the most common peripheral lesion (53.6%). Maculopathy was associated with peripheral lesions in 65.8% of cases. PEHCR lesions were mostly heterogeneous (58.8%) on B-scan ultrasonography. Choroidal neovascularisation was found in 10 eyes (26.3%) out of 38 eyes that underwent fluorescein angiography. Polyps were observed in 14 eyes (58.3%) out of 24 eyes that underwent indocyanine green angiography. Fifty-one eyes were treated (62.2%). Intravitreal injections (IVTI) of antivascular endothelial growth factor (VEGF) were the most used treatment (36.6%) before laser photocoagulation, photodynamic therapy, vitrectomy and cryotherapy. Only vitrectomy improved visual acuity. Most lesions (65.6%) regressed at the last follow-up visit.ConclusionIn case of PEHCR, multimodal imaging is useful to avoid misdiagnosis, to characterise PEHCR lesions and to guide treatment strategies. Regression of PEHCR lesions was observed in two-thirds of the patients. Vitrectomy improved visual acuity. More than a third of patients underwent anti-VEGF IVTI. Further studies are needed to assess IVTI’s efficacy.

scholarly journals Rapid progression of chorioretinal atrophy in punctate inner choroiditis: a case report

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Yuka Kasuya ◽  
Yuji Inoue ◽  
Satoru Inoda ◽  
Yusuke Arai ◽  
Hidenori Takahashi ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Case Report ◽  
Epiretinal Membrane ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Japanese Woman ◽  
Rapid Progression ◽  
Chorioretinal Atrophy ◽  
Corrected Visual Acuity ◽  
Best Corrected Visual Acuity

Abstract Background The chorioretinal inflammatory lesions occurring in punctate inner choroiditis evolve into punched-out atrophic scars. Typically, the progression is gradual. We report a case of highly myopic punctate inner choroiditis with rapid progression of chorioretinal atrophy. Case presentation A 48-year-old Japanese woman with high myopia presented with decreased visual acuity. Best-corrected visual acuity was 20/28 in the right eye and 20/16 in the left eye; axial length was 29.0 mm and 28.7 mm, respectively. Fundoscopy revealed an epiretinal membrane in the left eye. Three years later, the best-corrected visual acuity in the left eye had decreased to 20/33; at this time, the patient underwent vitrectomy with epiretinal membrane and internal limiting membrane peeling in this eye. Six months later, the best-corrected visual acuity in the left eye decreased suddenly to 20/100. Optical coherence tomography showed a nodule-like lesion in the outer retina with disruption of the retinal pigment epithelium and a focally thickened choroid, compatible with PIC. One month later, the choroidal thickness had decreased. The central chorioretinal atrophy expanded rapidly at a rate of 0.45 mm2/year over the next 3 years, and new areas of patchy focal chorioretinal atrophy developed in the perifovea. Conclusions Rapid progression of chorioretinal atrophy was observed in a patient with punctate inner choroiditis. Because punctate inner choroiditis is often associated with degenerative myopia, the retina is fragile and may be susceptible to mechanical damage. This case report alerts clinicians to the need for careful management of patients with punctate inner choroiditis, especially after vitrectomy.

Serpiginous Choroiditis; Clinical Features, Diagnosis, and Treatment

2021 ◽  
pp. 10-17
Keyword(s):  
Vision Loss ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Treponema Pallidum ◽  
Infectious Agent ◽  
Antimicrobial Treatment ◽  
Serpiginous Choroiditis ◽  
Multiple Recurrences ◽  
Peripapillary Area

Serpiginous choroiditis is a progressive disease that causes atrophy in layers of the choriocapillaris, retinal pigment epithelium, and photoreceptor, due to choroidal inflammation. It typically involves both eyes asymmetrically and spreads from the peripapillary area to the periphery. Although the etiology is unclear, an autoimmune and infectious mechanism seems likely to be the underlying process. The clinical condition is called “multifocal serpiginous choroiditis” if an underlying infectious cause such as Mycobacterium tuberculosis, Treponema pallidum, or herpesviruses is detected. The natural course of the disease usually consists of multiple recurrences of choroidal inflammation over a period of months to years. The vision loss occurs if the fovea is involved during these relapses. Multimodal imaging methods are important in the diagnosis, treatment, follow-up of the disease, and the detection of complications such as choroidal neovascularization. In treatment, it is aimed to suppress inflammation and prevent recurrences via systemic/local corticosteroids and immunomodulatory drugs. If an underlying infectious agent is detected, specific antimicrobial treatment should also be added.

CRB1-associated retinal dystrophies in a Belgian cohort: genetic characteristics and long-term clinical follow-up

2021 ◽  
pp. bjophthalmol-2020-316781
Author(s):  
Mays Talib ◽  
Caroline Van Cauwenbergh ◽  
Julie De Zaeytijd ◽  
David Van Wynsberghe ◽  
Elfride De Baere ◽  
...  
Keyword(s):  
Visual Function ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Posterior Uveitis ◽  
Cone Dystrophy ◽  
Macular Dystrophy ◽  
Genetic Characteristics ◽  
Retinal Dystrophies ◽  
Retinal Structure

AimTo investigate the natural history in a Belgian cohort of CRB1-associated retinal dystrophies.MethodsAn in-depth retrospective study focusing on visual function and retinal structure.ResultsForty patients from 35 families were included (ages: 2.5–80.1 years). In patients with a follow-up of >1 year (63%), the mean follow-up time was 12.0 years (range: 2.3–29.2 years). Based on the patient history, symptoms and/or electroretinography, 22 patients (55%) were diagnosed with retinitis pigmentosa (RP), 15 (38%) with Leber congenital amaurosis (LCA) and 3 (8%) with macular dystrophy (MD), the latter being associated with the p.(Ile167_Gly169del) mutation (in compound heterozygosity). MD later developed into a rod-cone dystrophy in one patient. Blindness at initial presentation was seen in the first decade of life in LCA, and in the fifth decade of life in RP. Eventually, 28 patients (70%) reached visual acuity-based blindness (<0.05). Visual field-based blindness (<10°) was documented in 17/25 patients (68%). Five patients (13%) developed Coats-like exudative vasculopathy. Intermediate/posterior uveitis was found in three patients (8%). Cystoid maculopathy was common in RP (9/21; 43%) and MD (3/3; 100%). Macular involvement, varying from retinal pigment epithelium alterations to complete outer retinal atrophy, was observed in all patients.ConclusionBi-allelic CRB1 mutations result in a range of progressive retinal disorders, most of which are generalised, with characteristically early macular involvement. Visual function and retinal structure analysis indicates a window for potential intervention with gene therapy before the fourth decade of life in RP and the first decade in LCA.

scholarly journals Bevacizumab for Macular Serous Neuroretinal Detachment in Tilted Disk Syndrome

2010 ◽  
Vol 2010 ◽  
pp. 1-3 ◽  
Author(s):  
Paolo Milani ◽  
Alfredo Pece ◽  
Luisa Pierro ◽  
Patrizio Seidenari ◽  
Paolo Radice ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Case Report ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Monthly Interval ◽  
Serous Macular Detachment ◽  
Corrected Visual Acuity ◽  
Total Observation ◽  
Tilted Disk ◽  
Best Corrected Visual Acuity

Background. Tilted disc syndrome (TDS) is a congenital anomaly characterized by “tilting” of the optic disc tipycally associated with myopic astigmatism, visual field defect, inferior staphyloma, and retinal pigment epithelium atrophy. Associated complications such as macular serous neuroretinal detachment are well described; however, ideal therapy for such complication is unknown.Methods. One interventional case report is hereby described. A patient affected by macular serous neuroretinal detachment-complicated tilted disk syndrome underwent a complete ophthalmic examination. Optical coherence tomography and fluorescein angiography were taken at baseline and at scheduled visits. Two intravitreal treatments of bevacizumab (avastin, 1.25 mg/0.05 mL) were performed at monthly interval.Results. At scheduled visit, one month after the second injection, OCT depicted persistence of neuroretinal detachment. Best-corrected visual acuity remain stable as well as metamorphopsia and functional discomfort.Conclusion. Clinical evidence of this brief interventional case report indicates that one patient affected by recent serous macular detachment-complicated TDS did not benefit from 2 consecutive monthly intravitreal Avastin treatments. Best-corrected visual acuity remained stable over a total observation period of 6 months.

Multimodal imaging in iodate-induced toxic retinopathy

2020 ◽  
pp. 112067212092686
Author(s):  
Sebile Çomçalı ◽  
Pınar Topcu Yılmaz ◽  
Cemal Çavdarlı ◽  
Mehmet Numan Alp
Keyword(s):  
Visual Acuity ◽  
Retinal Pigment Epithelium ◽  
Iodine Deficiency ◽  
Vision Loss ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Vitamin Supplementation ◽  
Potassium Iodate ◽  
Intentional Ingestion ◽  
The Right

Introduction Iodine deficiency is a leading cause of preventable physical and mental retardation. Potassium iodate is used for iodine supplementation to prevent iodine deficiency. We herein report a case of toxic retinopathy following intentional ingestion of potassium iodine. Case Presentation A 41-year-old male presented with a 5-day history of blurred vision in both eyes. His visual acuity (VA) was hand motion and his pupillary reactions were sluggish bilaterally. The fundus examination revealed bilaterally diffuse retinal pigment epithelium atrophy and secondary pigmentary changes at the posterior pole, but his peripheral fundus was relatively spared. Choroidal thinning, punctate hyperreflective dots along the retinal pigment epithelium layer, and outer retinal atrophy were the optical coherence tomography findings, which were consistent with widespread areas of retinal pigment epithelium window defects observed on fundus fluorescein angiography. The visual evoked potential test showed no response in the right eye and revealed a delay in the latency and a decrease in the amplitude of the P100 wave in the left eye. Wave b responses of the photoreceptors could not be observed in the patient’s electroretinogram. After a vitamin supplementation protocol consistent with the literature, at the 4-month follow-up visit his visual acuity had improved to 0.3 in the right eye and counting fingers in the left eye. Conclusion Potassium iodate toxicity is a cause of serious retinal and choroidal damage and results in severe vision loss. Hydration, hemodialysis, and antioxidants can be helpful to minimize the complications.

Sjögren Reticular Dystrophy of the Retinal Pigment Epithelium: A Case Report

2003 ◽  
Vol 13 (5) ◽  
pp. 491-495
Author(s):  
M. Rinaldi ◽  
A. Villani ◽  
M. Borrelli ◽  
S. Russo ◽  
L. Cotticelli
Keyword(s):  
Case Report ◽  
Retinal Pigment Epithelium ◽  
Late Onset ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Clinical Manifestations ◽  
Computerized Perimetry ◽  
Fundus Photographs ◽  
First Time

Purpose To describe the clinical manifestations in a patient with Sjögren reticular dystrophy of the retinal pigment epithelium, and the evolution of the disease over a 20-year follow-up period. Case Report A 45-year-old woman with Sjögren reticular dystrophy of the retinal pigment epithelium was seen for the first time in 1983; the patient underwent 20 years of annual check-ups. Results Over the follow-up period, fundus photographs, computerized perimetry, electroretinogram, and electro-oculogram findings had either normal or slightly subnormal outcome. Conclusions The results confirm that this disease involves only the retinal pigment epithelium and should not be considered a central tapeto-retinal degeneration or late onset retinopathy.

scholarly journals Long-Term Follow-Up of Congenital Simple Hamartoma of the Retinal Pigment Epithelium: A Case Report

2018 ◽  
Vol 9 (1) ◽  
pp. 215-220 ◽  
Author(s):  
Yuka Ito ◽  
Masahito Ohji
Keyword(s):  
Visual Acuity ◽  
Retinal Pigment Epithelium ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Japanese Woman ◽  
Initial Examination ◽  
Macular Lesion ◽  
Long Term Follow Up

Background: Congenital simple hamartoma of the retinal pigment epithelium (CSHRPE) is a rare benign tumor of the retinal pigment epithelium characterized by a focal, darkly pigmented nodule in the macular lesion in healthy persons. We report a case of CSHRPE with long-term follow-up. Case: A 41-year-old Japanese woman was incidentally discovered to have a dark lesion on the fundus of the left eye. We evaluated the patient by measuring her best-corrected visual acuity (BCVA) and by slit-lamp biomicroscopy, fundus color photography, and optical coherence tomography (OCT) over a 10-year period. The BCVA gradually declined during the early follow-up period, having decreased from 1.2 to 0.8 in the left eye 3 years after the initial examination, and then has been maintained for the following 7 years. The lesion did not show a change in OCT 10 years after the first examination. Conclusion: It is important to follow a CSHRPE carefully over the long term because visual acuity might decrease.

Sign in / Sign up

Export Citation Format

Share Document