Diffuse Retinal Pigment Epitheliopathy

Twenty-seven patients with characteristic, mostly bilateral, fundus lesions of chronic central serous chorioretinopathy (CSC) and a progressive course, and 80 of their relatives, mainly siblings, were examined. Ophthalmologic examination included assessment of visual acuity, Amsler grid testing, ophthalmoscopy and fluorescein angiography. The fundus findings were classified as normal fundus, multiple areas of retinal pigment epithelium (RPE) atrophy or chronic CSC: RPE atrophy with leakage of fluorescein. In 14 (52%) of the 27 families, 1 or more relatives were affected. Thirty-five (44%) of the 80 investigated relatives had fundus lesions: 22 had chronic CSC in one eye, 20 of these had chronic CSC or RPE atrophy in the fellow eye. Thirteen relatives had RPE atrophy in one or both eyes. The mode of inheritance could not be established.

Section: Discussionsupporting

confidence: 93%

“…Familial central serous chorioretinopathy (CSC) is considered to be rare and only 8 reports exist that document affected relatives [1, 2, 3, 4, 5, 6, 7, 8]. In a previous study, using data pooled from 8 European eye clinics, 11 families having 2–4 members with chronic CSC were identified [5].…”

Section: Introductionmentioning

confidence: 99%

Familial Chronic Central Serous Chorioretinopathy

Weenink

Borsje

Oosterhuis³

2001

“…CSC seems to be the focal form, and DRPE the diffuse form [28]. Von Winning [16] reports a family in which one brother had DRPE, one sister had a typical CSC, and the other brother had DRPE with recurrent CSC.…”

Section: Discussionmentioning

confidence: 99%

“…A variant of central serous chorioretinopathy is the diffuse retinal pigment epitheliopathy (DRPE) with a chronic course, multiple recurrences, often bilateral appearance, extensive alteration of the retinal pigment epithelium (RPE), and a worse visual prognosis than in CSC [16, 17, 18, 19]. We report 2 patients who developed DRPE and 2 patients who presented with recurrent CSC during treatment with systemic corticosteroids for different reasons.…”

Section: Introductionmentioning

confidence: 99%

Retinal Pigment Epithelial Changes Associated with Systemic Corticosteroid Treatment: Report of Cases and Review of the Literature

Spraul¹,

Lang²,

Lang³

1998

Purpose: The aim of our study was to describe two different manifestations of retinal pigment epithelial changes associated with systemic corticosteroid treatment. Methods: We report 4 patients who developed maculopathy during treatment with systemic corticosteroids. Additionally, the literature was reviewed for similar cases. Results: Two patients showed diffuse retinal pigment epitheliopathy. Two patients developed central serous chorioretinopathy with localized changes in the retinal pigment epithelium. A review of the literature revealed 51 patients with similar changes during treatment with corticosteroids. Conclusion: These 4 patients provide further evidence that systemic treatment with corticosteroids may cause central serous chorioretinopathy or diffuse retinal pigment epitheliopathy. Ophthalmologic examination is recommended on a regular basis for patients treated with systemic corticosteroids.

“…Although CSC is generally considered to be a self-limiting condition, some patients may progress to chronic CSC, characterized by diffuse RPE abnormalities with persistent SRF [16, 17]. Chronic CSC, if left untreated, may lead to irreversible atrophic changes of the RPE and photoreceptors, and consequently worst visual prognosis [18].…”

Section: Discussionmentioning

confidence: 99%

Long-Term Outcomes of Full-Fluence and Half-Fluence Photodynamic Therapy for Chronic Central Serous Chorioretinopathy

Son

Kim

2018

Purpose: To evaluate the efficacy and safety of full-fluence photodynamic therapy (PDT) and half-fluence PDT in chronic central serous chorioretinopathy (CSC). Procedures: A retrospective review of CSC patients treated with full-fluence or half-fluence PDT for 12 months was performed. Best-corrected visual acuity (BCVA), central macular thickness (CMT), neural retinal thickness, subfoveal choroidal thickness (SFCT), resolution of subretinal fluid (SRF), and incidence of retinal pigment epithelium (RPE) atrophy at 12, 24, and 36 months were assessed. Results: Thirty-seven and 30 eyes received full-fluence and half-fluence PDT, respectively. The BCVA and CMT improved significantly in both the full-fluence and half-fluence groups at 36 months, without a significant difference between the groups. Both groups showed significant reductions in SFCT with full-fluence (416.8–316.8 µm) being better overall than half-fluence (409.7–349.1 µm, p = 0.002). All patients achieved complete resolution without recurrence after one PDT treatment per eye. A few cases of RPE atrophy occurred, which could be correlated to PDT in both groups during the follow-up. Conclusion: Both treatments were effective and safe in chronic CSC, with significant improvements in anatomic and visual parameters, without recurrence of SRF.