CASE REPORT

Rev Bras Oftalmol. 2021;80(5):e0045.

CASE REPORT

Keywords:

Retinal detachment; Nephrotic

syndrome; Renal insufciency,

chronic

Descritores:

Descolamento da retina;

Síndrome nefrótica; Insuciência

renal crônica

How to cite:

Henriques S, Lima A, Almeida J, Basto R, Roque J, Coutinho I, Prieto I. Bilateral retinal detachment – when the kidney meets the eye. Rev Bras Oftalmol. 2021:80(5):e0045.

doi:

https://doi.org/10.37039/1982.8551.20210045

Bilateral retinal detachment – when the kidney meets the eye

Descolamento de retina bilateral – quando o rim afeta o olho

Susana Henriques

, Anna Lima

, Júlio Almeida

, Rita Basto

, Joana Roque

, Inês Coutinho

, Isabel Prieto

Ophthalmology Department, Hospital Professor Doutor Fernando Fonseca, EPE, Portugal.

Nephrology Department, Hospital Professor Doutor Fernando Fonseca, EPE, Portugal.

Received on:

Apr 06, 2021

Accepted on:

Jul 31, 2021

Corresponding author:

Susana Henriques

Department of Ophthalmology, Hospital

Professor Doutor Fernando Fonseca

EPE, Portugal

E-mail: [email protected]

Institution:

Hospital Professor Doutor Fernando

Fonseca, EPE, Portugal.

Conict of interest:

the authors declare no conict of interest.

Financial support:

the authors received no nancial support

for this work.

ABSTRACT

Exudative retinal detachment occurs when uid accumulates between the neurosensory retina and the

retinal pigment epithelium. Ocular diseases or multisystem conditions such as nephrotic syndrome may

lead to exudative retinal detachment. This report describes a case of nephrotic syndrome secondary

to minimal change disease, anasarca and bilateral serous macular detachment in an adult patient. A

75-year-old male patient presented to the emergency department with generalized edema, asthenia,

and visual impairment. Medical history included a recent diagnosis of nephrotic syndrome secondary

to minimal change disease, which had been controlled with corticosteroid therapy. At presentation,

best corrected visual acuity was 20/100. Slit-lamp examination revealed xanthelasmas and mild bilateral

eyelid edema and chemosis. Dilated fundus examination conrmed bilateral macular detachment.

The patient did not respond to diuretic therapy. Ttherefore, hemodialysis was started. Two months

later, visual acuity improved to 20/25 and near normal restoration of retinal anatomy was achieved, with

concurrent remission of proteinuria. Exudative retinal detachment is a multifactorial condition. However,

in diseases associated with severe hypoalbuminemia, such as nephrotic syndrome, low oncotic pressure

in choroidal vessels and high interstitial pressure in the choroid may explain retinal detachment. Patients

with chronic kidney disease carry a high risk of ophthalmic disease development. Several mechanisms

that affect ocular vessels, the retina and the choroid are thought to be involved. A multidisciplinary

approach is crucial to resolve the ophthalmic condition and improve overall health.

RESUMO

O descolamento de retina exsudativo ocorre quando o uido se acumula entre a retina neurossensorial

e o epitélio pigmentado da retina. Patologias oculares isoladas ou doenças multissistêmicas, como a

síndrome nefrótica, podem levar ao descolamento de retina exsudativo. Apresenta-se aqui o caso de

um adulto com síndrome nefrótica por doença de lesões mínimas, anasarca e descolamento de retina

exsudativo macular bilateral. Trata-se de um homem de 75 anos de idade, que recorreu ao serviço de

urgência com edema generalizado, astenia e diminuição da acuidade visual. Os antecedentes pessoais

incluíam diagnóstico recente de síndrome nefrótica secundária à doença de lesões mínimas, em uso

de corticoterapia. Na apresentação, a melhor acuidade visual corrigida era 20/100. A biomicroscopia

revelou xantelasmas, edema palpebral leve e quemose nos dois olhos. Fundoscopia mostrou

descolamento macular bilateral. O doente iniciou diuréticos com pouca resposta clínica, tendo sido

adicionada hemodiálise. Vericou-se melhora da acuidade visual para 20/25 e restauração quase

total da anatomia da retina 2 meses após o início do tratamento, coincidindo com a remissão da

proteinúria. A siopatologia dos descolamentos de retina exsudativos é multifatorial, mas, em doenças

com hipoalbuminemia grave, como a síndrome nefrótica, a baixa pressão oncótica e a alta pressão

intersticial na coroide podem explicar o descolamento macular exsudativo. Doentes com doença renal

crônica constituem um grupo de risco para o desenvolvimento de doença ocular, envolvendo vários

mecanismos que afetam vasos, retina e coroide. Uma abordagem multidisciplinar é crucial para a

melhoria da doença oftalmológica e do estado geral do doente.

Henriques S, Lima A, Almeida J, Basto R, Roque J, Coutinho I, Prieto I

Rev Bras Oftalmol. 2021;80(5):e0045.

INTRODUCTION

Exudative retinal detachment (ERD) occurs when uid

accumulates between the neurosensory retina (NSR) and

the retinal pigment epithelium (RPE) in the absence of

retinal tears and/or vitreoretinal traction. Subretinal ex-

udation is thought result from changes in choroidal vas-

cular perfusion and permeability or from impaired pump

activity in the RPE, which lead to uid accumulation in

the subretinal space (SRS).

(1)

Other ocular or multisystem

conditions such as vascular, inammatory, or neoplastic

diseases may also play a role in ERD.

(1)

The prevalence of eye diseases and visual impairment

in patients with chronic kidney disease (CKD) is high, es-

pecially in the presence of concurrent comorbidities,

such as diabetic retinopathy, hypertensive retinopathy or

age-related macular disease (AMD).

(2,3)

Associations be-

tween ERD and CKD have also been reported in patients

undergoing hemodialysis

(4,5)

and in some patients with

nephrotic syndrome (NS), particularly in cases secondary

to diabetes and hypertension.

(6,7)

Nephrotic syndrome is

characterized by severe proteinuria (>3.5 g/dL/24 hour),

hypercholesterolemia and hypoalbuminemia, peripheral

edema and uid overload.

(8)

Diabetic nephropathy, focal

segmental glomerulosclerosis, membranous nephrop-

athy and minimal change disease (MCD) are common

causes of NS in adult patients.

(8)

This report describes a case of MCD and bilateral se-

rous macular detachment in an adult patient.

CASE REPORT

A 75-year-old male patient presented to the emergency

department with generalized edema (limbs, neck, perior-

bital and conjunctiva), asthenia and visual impairment.

Medical history included hypertension and a recent diag-

nosis of NS secondary to biopsy-conrmed MCD, which

had been controlled with corticosteroids the month be-

fore. Laboratory workup revealed acute kidney injury (se-

rum creatinine 4 mg/dL), severe proteinuria (6.5 g/dL) and

hypoalbuminemia (1.44 g/dL), conrming the presence of

active disease. Pleural eusion and ascites were detected

in chest radiographs and abdominal ultrasound, respec-

tively. Serologic screening, autoimmune disease tests and

kidney ultrasound ndings were unremarkable. Initial

routine ophtalmic exam revealed best corrected visual

acuity (BCVA) of 20/100 in both eyes (OU), xanthelasmas

and bilateral mild eyelid edema and chemosis (slit-lamp

examination; Figures 1A to 1D). Dilated fundus examina-

tion revealed bilateral macular detachment (Figures 2A

and 2C), with no signs of hypertensive retinopathy, vitre-

oretinal traction and/or peripheral retinal tears. Spectral-

domain optical coherence tomography (SD-OCT) revealed

dome-shaped macula with serous macular detachment

and cystoid edema in OU, but no other intraretinal ab-

normalities (Figures 2B to 2D). Peripapillary nerve ber

layer (pNFL) and choroidal thickness were normal in OU.

Diuretic therapy was implemented. However, the patient

did not respond, and hemodialysis was initiated to im-

prove edema control. Progressive volume control and

kidney function recovery were achieved. Therefore, the

patient was discharged, and steroid therapy prescribed.

Follow-up assessment one month later revealed BCVA

improvement (20/25 in OU) and restoration of near nor-

mal retinal anatomy on dilated fundus examination

(Figures 2E and 2G) and SD-OCT (Figures 2F to 2H). These

ndings were concurrent with remission of proteinuria

and kidney disease control. Previously undetected sub-

retinal drusenoid deposits were seen in both maculas.

DISCUSSION

Minimal change disease accounts for 70% to 90% of cas-

es of idiopathic NS in children. However, adult patients

are less commonly aected (10% to 15% of cases).

(9)

This

glomerular disorder often manifests as a primary renal

disease. However, associations with other conditions,

such as viral infections, allergies and non-steroidal an-

ti-inammatory therapy have been reported in a minori-

ty of cases. The pathophysiology of the condition has

not been fully determined. Examination of renal biopsy

specimen slides using light microscopy often fails to re-

veal obvious glomerular lesions. However, podocyte foot

Figure 1. Slit-lamp examination showing chemosis in the right (A and B) and the left (C and D) eye.

Bilateral retinal detachment

Rev Bras Oftalmol. 2021;80(5):e0045.

process eacement is typically seen on electron micros-

copy.

(9)

Minimal change disease tends to coincide with

the sudden onset of NS, indicated by severe proteinuria,

minimal hematuria, hypoalbuminemia, hypercholester-

olemia, edema, and hypertension. Prednisone is the rst-

line therapy, but other immunosuppressive drugs can be

used in frequent relapsers, steroid-resistant or steroid-de-

pendent patients. The prognosis in less favorable in adult

patients with acute renal failure.

Exudative retinal detachment is often associated with

other ocular retinal and/or choroidal diseases,

(1)

such as

diabetic retinopathy (DR), central serous chorioretinop-

athy (CSC), posterior scleritis and intraocular tumors.

Systemic causes

(1)

include the Vogt-Koyanagi-Harada

(VKH) syndrome, toxemia of pregnancy, malignant hy-

pertension, and CKD, which are often associated with he-

modialysis and/or corticosteroid therapy.

(5,7)

The RPE and the tight junctions of retinal capillary en-

dothelial cells form the outer and inner blood-retinal barrier

(BRB) respectively. The integrity of the BRB barrier is crucial

to maintain the retina in normal, dry conditions.

(10,11)

In the-

ory, diseases that lead to outer or inner BRB breakdown and

increase the permeability of choroidal capillaries may cause

subretinal uid accumulation.

(10,11)

However, several mecha-

nisms of adhesion of the NSR to the RPE ensure directional

uid transport from the RPE to the choroid, including active

and passive transport across the RPE and pressure gradients

between the vitreous, the retina and the choroid (intraocular

pressure, plasma oncotic pressure and hydrostatic pressure,

respectively). The higher oncotic pressure in the choroid rel-

ative to the vitreous maintains uid dynamics and promotes

the outward ow of water.

(11,12)

Serum albumin is the most important plasma pro-

tein and a major determinant of oncotic pressure in the

circulatory system. Low oncotic pressure in choroidal

vessels in response to hypoalbuminemia facilitates the

outow of water from the intravascular to the interstitial

space, increasing the hydrostatic pressure in the choroi-

dal interstitium.

(13)

Hence, in diseases associated with se-

vere hypoalbuminemia such as NS, this mechanism may

explain retinal detachment in some patients. Few isolated

cases of ERD in hypoalbuminemic patients have been re-

ported to date. These include one case of protein-losing

enteropathy in a 47-year-old female patient with serum al-

bumin levels of 1.4 g/dL and bilateral ERD (Venkatramani

et al.),

(14)

one case of NS secondary to MCD in a 24-year-old

female patient who developed bilateral ERD responsive

to diuretic therapy (De Benedetto et al.)

(15)

and

one case of

ERD in a 52-year-old patient with NS secondary to plasma-

cytoma (Hager et al.).

(16)

The patient described in this case

report had severe hypoalbuminemia and other causes of

ERD could be excluded, given serologic screening, autoim-

mune disease test and ophthalmic imaging ndings were

not consistent with infectious diseases, inammatory con-

ditions or diabetic or hypertensive retinopathy. Also, at the

time of presentation, the patient had not yet started hemo-

dialysis, which is often associated with ERD. Corticosteroid

therapy and central serous chorioretinopathy (CSC) are

common causes of serous macular detachment, the latter

aecting predominantly middle-aged males.

(1)

Exudative retinal detachment is a multifactorial con-

dition. In the case described, two major factors may have

accounted for subretinal uid accumulation: reversal of

pressure gradients from the choroid and the retina in re-

sponse to severe hypoalbuminemia and drop in oncotic

pressure induced by RPE ion pump dysfunction, possibly

induced by the multiple subretinal drusenoid deposits

seen at the level of the RPE on OCT images.

Figure 2. Fundus photographs and spectral-domain optical coherence tomography images acquired prior to (top) and following

one month (bottom) of treatment. Macular detachment can be seen in fundus photographs of the right (A) and the left (C) eye.

Dome-shaped macula with serous macular detachment and cystoid edema can be seen in optical coherence tomography images

of the right (B) and the left eye (D). Cystoid edema and subretinal uid resolution can be seen in post-treatment fundus photo-

graphs and optical coherence tomography images (E to H).

Henriques S, Lima A, Almeida J, Basto R, Roque J, Coutinho I, Prieto I

Rev Bras Oftalmol. 2021;80(5):e0045.

Exudative retinal detachment therapy should be fo-

cused primarily on resolution of the underlying systemic

disease. Corticosteroids are the rst-line therapy for pa-

tients with NS, particularly in cases associated with MCD.

Second-line treatment alternatives include cyclophos-

phamide and/or cyclosporine.

(17)

Indeed, two months

after initiation of treatment with corticosteroids and di-

uretics, functional and structural changes had been re-

verted to almost normality and concurrent remission of

proteinuria was achieved.

In conclusion, patients with CKD carry a high risk of

ophthalmic disease development. Multidisciplinary as-

sessment by ophthalmologists, nephrologists and inter-

nists is vital for prompt diagnosis and therapeutic deci-

sion making in order to prevent severe visual dysfunction.

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