Bilateral Serous Retinal Detachment as a Presenting Sign of Nephrotic Syndrome

Izzedine, Hassan; Fardeau, Christine; Gauthier, Marion; Fel, Audrey; Attias, Philippe; Benabdellah, N.; Sassi, Mohamed-Ali; Bodaghi, Bahram

doi:10.2169/internalmedicine.53.2720

Cited by 16 publications

(16 citation statements)

References 23 publications

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“…The incidence of this clinical presentation is probably underestimated as it is largely overlooked in these patients. To our knowledge, among the published cases of AL amyloidosis with chorioretinal disease [10][11][12], our case is one of the first to document the rapid progression of fundus alterations and their stabilization after disease remission. Elementary lesions in our case include pachychoroid, large subretinal detachments, which resolved after chemotherapy and subretinal deposits that are hyperautofluorescent leading to masking on angiography.…”

Section: Discussionmentioning

confidence: 64%

“…These alterations would lead to secondary retinal pigment epithelium (RPE) dysfunction accounting for subretinal detachments and impaired photoreceptor outer segment phagocytosis leading to hyperautofluorescent subretinal material accumulation. The nephrotic syndrome could also have a role in the choroidal changes and subretinal detachments through a modification of the oncotic pressure [12,[17][18][19]. By restoring the plasmatic protein balance and decreasing amyloid deposits, the initiation of the first chemotherapy cycles led to the healing of the nephrotic syndrome and the resorption of the subretinal fluid.…”

Section: Discussionmentioning

confidence: 99%

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Progressive chorioretinal involvement in a patient with light-chain (AL) amyloidosis: a case report

2020

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Background: To report an unusual case of light-chain (AL) amyloidosis with progressive bilateral chorioretinal abnormalities documented with short-wavelength autofluorescence, SD-OCT, fluorescein and indocyanine green angiography. Case presentation: Case report of a forty-three-year-old male patient with kappa AL amyloidosis. The patient presented with rapidly progressing pigmented and hyperautofluorescent drusenoid deposits in both eyes, associated with central serous retinal detachments, a pachychoroid and choriocapillaris enlargement. The general assessment revealed a renal failure symptomatic of a nephrotic syndrome, associated with proteinuria composed mainly of free kappa light chains. A kidney biopsy confirmed the diagnosis of kappa AL amyloidosis. Chemotherapy was quickly started. During remission, the extension of drusenoid deposits on the fundus was stopped and a disappearance of the subretinal fluid on SD-OCT was observed. Conclusions: AL amyloidosis is an insidious and potentially fatal condition. This case is one of the first to document the rapid progression of fundus alterations and their stabilization after disease remission. Identifying these specific fundus abnormalities is essential to avoid diagnosis wandering and therapeutic delay.

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Section: Discussionmentioning

confidence: 64%

Section: Discussionmentioning

confidence: 99%

Progressive chorioretinal involvement in a patient with light-chain (AL) amyloidosis: a case report

2020

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“…Among the reported cases, some patients had SRD in the macular region [3–8, 10], while other patients had concurrent macular oedema [3, 8] or RPE detachment and tears [4], or the condition manifested as macular oedema alone [9]. Some patients showed concurrent increases in CT on OCT [6]. Patients suffering from these diseases also typically had hypoalbuminaemia, with blood ALB concentrations of 10–23 g/L, which usually resolved after several weeks of treatment with glucocorticoids, immunosuppressants and diuretics [3–7, 9, 10].…”

Section: Discussionmentioning

confidence: 99%

“…In recent years, some case reports have shown that patients with NS can have bilateral serous retinal detachment (SRD) [3–8], macular oedema [3, 8, 9] and retinal pigment epithelium (RPE) detachment [4], accompanied by an increased choroidal thickness (CT) [6]. SRD and choroidal thickening may also occur in patients with hypoproteinaemia caused by other diseases, such as protein losing enteropathy [7, 10].…”

Section: Introductionmentioning

confidence: 99%

“…SRD and choroidal thickening may also occur in patients with hypoproteinaemia caused by other diseases, such as protein losing enteropathy [7, 10]. Therefore, it is presumed that the pathogenesis may be related to the retention of liquid in the retina and choroid due to hypoproteinaemia, which is the same mechanism that leads to systemic oedema [5, 6, 8]. However, there is no research about the effect of hypoproteinaemia on the retinal thickness (RT) and the CT.…”

Section: Introductionmentioning

confidence: 99%

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Retinal and choroidal thickness in paediatric patients with hypoalbuminaemia caused by nephrotic syndrome

Zhang

Kang

et al. 2019

BMC Ophthalmol

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BackgroundA study was conducted to evaluate the choroidal thickness (CT) and retinal thickness (RT) in paediatric patients with hypoalbuminaemia caused by nephrotic syndrome (NS). We also studied the correlation between the subfoveal choroidal thickness (SFCT) and serum protein concentration.MethodsThis was a cross-sectional study. Fifty-one paediatric patients with hypoalbuminaemia caused by NS and 41 normal subjects were included in the study. Enhanced depth imaging optical coherence tomography (EDI-OCT) was performed to measure the RT and CT. The RT and CT were measured manually at intervals of 0.5 mm along a horizontal line through the macular fovea between 2.5 mm nasal and 2.5 mm temporal to the fovea. Clinical data including measurements of serum proteins were obtained.ResultsThe mean RTs at the T2.5, T2, N1.5, N2, and N2.5 locations and the average macular horizontal RT were slightly greater in the NS group than those in the control group. The mean CTs at all locations were significantly greater in the NS group than those in the control group; the difference was most significant at the fovea (373.8 ± 74.9 μm vs. 280.2 ± 57.1; p < 0.001). The SFCT in patients with NS was correlated with age (r = − 0.307, p = 0.003), body height (r = − 0.320, p = 0.022), body weight (r = − 0.343, p = 0.014), axial length (AL, r = − 0.237, p = 0.023), total protein (TP, r = − 0.302, p = 0.031), albumin (ALB, r = − 0.285, p = 0.042), prealbumin (PA, r = − 0.303, p = 0.033) and 24-h urine volume (UV, r = − 0.298, p = 0.034). Multiple linear regression analysis showed that the TP concentration and body weight had the highest correlation with the SFCT (R2 = 0.220, p < 0.05).ConclusionsThe macular RT is slightly increased and the macular CT is significantly increased in paediatric patients with hypoalbuminaemia caused by NS, indicating fluid accumulation in the retina and choroid. There is a negative correlation between the SFCT and serum TP concentration. Thus, the serum TP concentration is an important indicator of CT in patients with hypoalbuminaemia.

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Exudative retinal detachment secondary to hypertensive crisis related to membranous nephropathy

Yeung

Patrick

2021

Canadian Journal of Ophthalmology

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The present case can provide guidance for following patients with CS in the eye clinic. Retinitis pigmentosa is detected commonly in these patients. 2 Routine OCT may inform treatment decisions given that topical carbonic anhydrase inhibitors may improve CME in retinal dystrophies. 5 In addition to OCT, electroretinogram findings can help characterize and explain vision loss, which is critical as these patients may not verbalize visual complaints. Finally, refractive error should be frequently assessed given that progressive myopia is virtually always present. 2 To further explore the experience with CS among Canadian ophthalmologists, we undertook a survey study. This was approved by the Queen's University Health Sciences and Affiliated Teaching Hospitals Research Ethics Board. The survey (Appendix) was developed using Qualtrics (Provo, UT) and distributed to members of the Canadian Association of Pediatric Ophthalmology and Strabismus by email listserv (76 members).Responses were received from 19 pediatric ophthalmologists (response rate, 25%), with a median of 12 years of practice (range, 2À40 years). Respondents were asked to rate how knowledgeable they were of CS. Three (15.8%) rated "not at all," 5 (26%) "minimally," 10 (53%) "somewhat," 1 (5%) "very," and 0 "extremely." Only 8 (41%) had seen at least 1 patient with CS over their entire career. The range of number of patients ever seen by each ophthalmologist was 0À4. Only 4 respondents (21%) currently follow a patient with this diagnosis in their practice. Among those, only 1 patient has been assessed by ophthalmology before a diagnosis of CS was known; this is the patient herein reported. While limitations of this study include low response rate, potential recall bias, and patients possibly being double-counted if shared between practices, these do not alter our conclusions.Pediatric ophthalmologists in Canada have limited exposure to CS in clinical practice and almost exclusively encounter these patients after the diagnosis has been made. Almost half of pediatric ophthalmologists surveyed believed that they were either not at all or only minimally knowledgeable of CS. This underscores the value of reports describing clinical findings of this rare disease, especially early in its natural history. In the context of myopia, pigmentary retinal changes, undefined developmental delay, and dysmorphic features, an ophthalmologist may be among the first providers with opportunity to initiate referral for genetic testing. This, in turn, has large implications for management of vision loss and family expectations.

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Bilateral Serous Retinal Detachment as a Presenting Sign of Nephrotic Syndrome

Cited by 16 publications

References 23 publications

Progressive chorioretinal involvement in a patient with light-chain (AL) amyloidosis: a case report

Progressive chorioretinal involvement in a patient with light-chain (AL) amyloidosis: a case report

Retinal and choroidal thickness in paediatric patients with hypoalbuminaemia caused by nephrotic syndrome

Exudative retinal detachment secondary to hypertensive crisis related to membranous nephropathy

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