Case Report

Central Serous Chorioretinopathy in a Case of
Regressed Familial Retinoblastoma

Saeed Karimi1,2, MD; Amir Arabi1,2, MD, MPH; Toktam Shahraki1,2, MD; Sare Safi3, PhD

1Ophthalmic Research Center, Research Institute for Ophthalmology and Vision Science, Shahid Beheshti University of Medical
Sciences, Tehran, Iran

2Department of Ophthalmology, Torfeh Medical Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran
3Ophthalmic Epidemiology Research Center, Research Institute for Ophthalmology and Vision Science, Shahid Beheshti

University of Medical Sciences, Tehran, Iran

ORCID:
Saeed Karimi: http://orcid.org/0000-0002-3231-8414
Amir Arabi: https://orcid.org/0000-0002-6523-7733

Abstract
Purpose: To present a case of central serous chorioretinopathy (CSC) in association with
regressed familial retinoblastoma.
Case Report: A 23-year-old man with regressed unilateral familial retinoblastoma in his left
eye presented with decreased vision of the left eye since two months ago. The patient had
undergone chemotherapy and cryotherapy for the treatment of retinoblastoma 20 years ago.
In the left eye, funduscopy disclosed regressed mass of retinoblastoma, inferonasal to the optic
disc, and focal subfoveal neurosensory detachment. Optical coherence tomography (OCT) and
fluorescein angiography revealed CSC. As there was no sign of recurrence of retinoblastoma
and retinal findings did not show late-onset chemotherapy-related retinopathy, the patient was
diagnosed with CSC and followed up. After two months, visual acuity of the left eye improved,
and repeated macular OCT revealed absorption of the subretinal fluid.
Conclusion: Subretinal fluid accumulation in a patient with regressed retinoblastoma is not
always a sign of tumor recurrence or a treatment-related retinopathy.

Keywords: Central Serous Chorioretinopathy; Recurrence; Retinoblastoma

J Ophthalmic Vis Res 2020; 15 (4): 559–564

INTRODUCTION

Central serous chorioretinopathy (CSC) is a retinal
disease in which there is serous detachment

Correspondence to:

Amir Arabi, MD, MPH. Research Institute for
Ophthalmology and Vision Science, Shahid Beheshti
University of Medical Sciences, No. 23, Paidarfdard St.,
Boostan 9 St., Pasadaran Ave., Tehran 16666, Iran.
E-mail: amir_arab_91@yahoo.com
Received: 16-07-2019 Accepted: 07-01-2020

Access this article online

Website: https://knepublishing.com/index.php/JOVR

DOI: 10.18502/jovr.v15i4.7802

of the neurosensory retina, typically restricted
to the macular area. The disease is believed
to be a disorder with multiple etiologies that
lead to a common pathway of choroidal vascular
abnormality.[1] The pathogenesis is poorly known,
and retrospective studies have recognized a
number of risk factors. These include male sex,[2]
psychological tension,[3] personality type A,[4]

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How to cite this article: Karimi S, Arabi A, Shahraki T, Safi S. Central
Serous Chorioretinopathy in a Case of Regressed Familial Retinoblastoma.
J Ophthalmic Vis Res 2020;15:559–564.

© 2020 JOURNAL OF OPHTHALMIC AND VISION RESEARCH | PUBLISHED BY KNOWLEDGE E 559

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CSCR and RB Association; Karimi et al

Figure 1. (a) Posterior fundus view of the left eye demonstrating the distance between the mass and fovea. (b) Regressed
retinoblastoma with intralesional cavitation, inferonasal to the optic disc.

corticosteroid use,[5, 6] gestation,[7] and,
infrequently, endocrine disorders (such as
Cushing’s syndrome)[8] or tumors with steroid
products.[9]

Retinoblastoma is an inherited pediatric
malignant neoplasia, assumed to be the most
frequent intraocular malignancy in children.[10]
Concerns about patients with a history of
retinoblastoma, especially those treated with
external beam radiotherapy, are related to the
risk of recurrence, secondary malignancies, and
development of radiation retinopathy or retinal
damage secondary to chemotherapy. All these

conditions can develop even after a long period
from the initial treatment.[11–13]

Here, we report a case of regressed
retinoblastoma and coincidental CSC in the
same eye. The association between regressed
retinoblastoma and CSC has not been reported in
the literature.

CASE REPORT

A 23-year-old man presented to the ophthalmology
clinic with a two-month history of blurred vision
in the left eye. The patient had a history of

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CSCR and RB Association; Karimi et al

Figure 2. (A) SD-OCT of the left eye; neurosensory detachment with echo-free subretinal fluid adjacent to a well-defined PED. (B)
EDI-OCT of the same eye; note the increased thickness of the choroid and congested vasculature.

unilateral familial retinoblastoma in the left eye,
which had been treated with cryotherapy and
systemic chemotherapy 20 years before. Medical
and drug histories were otherwise unremarkable.
The patient had a positive family history of
retinoblastoma, as his father and two siblings were
diagnosed with bilateral familial retinoblastoma.

Ophthalmic examination showed a corrected
distance visual acuity of 10/10 in the right eye
and 5/10 in the left eye. Intraocular pressure
was normal, and relative afferent pupillary defect
was negative. Anterior segment examination
showed normal findings. Fundus examination
of the left eye revealed a white elevated
regressed retinoblastoma mass with intralesional
cavitation and focal subfoveal neurosensory
detachment (Figure 1). The right eye was
completely normal. Spectral-domain optical
coherence tomography (OCT) revealed echo-free

subfoveal fluid accumulation and a small pigment
epithelial detachment. Additionally, enhanced
depth imaging OCT of both eyes showed thick
choroid and dilated choroidal vessels (Figure
2). Fluorescein angiography (FAG) of the left
eye revealed an expansile leaking dot near the
fovea in the mid-to-late phase. Indocyanine green
angiography showed hyperpermeability of dilated
choroidal vasculature (Figure 3). Based on clinical
examination and paraclinical findings, a diagnosis
of CSC coincidental with regressed unilateral
familial retinoblastoma was made. As there was no
sign of retinoblastoma recurrence or secondary
malignancy and the clinical findings did not reflect
late-onset retinopathy related to prior treatment, a
close follow-up was planned. During the follow-up
period, oral propranolol was prescribed. At the
two-month re-examination, the best corrected
visual acuity of the affected eye improved to 7/10,

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CSCR and RB Association; Karimi et al

Figure 3. Early and late phases of fluorescein angiography (left) and ICG angiography (right) showing juxtafoveal subretinal leakage
in addition to congestion of choroidal vessels.

Figure 4. Macular SD-OCT of the same eye two months later shows resolved subretinal fluid.

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CSCR and RB Association; Karimi et al

the retinoblastoma mass was unchanged, and
the new macular OCT revealed absorption of the
subretinal fluid (Figure 4).

DISCUSSION

Blurring of vision and new accumulation of
subretinal fluid in a patient with history of
retinoblastoma is usually suggestive of recurrent
retinoblastoma, adverse effect of chemotherapy
on the chorioretinal tissue, or late-onset
radiation retinopathy. As there was no history
of radiotherapy and intra-arterial chemotherapy in
this case, ocular complications of these therapeutic
interventions were not considered for the patient.

Posterior segment side effects of systemic
chemotherapy have been studied for some drugs.
Secondary retinopathy with visual loss develops
with cisplatin use;[14] however, retinal findings of
cisplatin-induced retinopathy are different from
those seen in our case. Moreover, retinal ischemia
and subsequent neovascularization have been
reported in a patient receiving chemotherapy with
bleomycin, etoposide, and cisplatin.[15] However,
FAG of this patient revealed no sign of retinal
ischemia or neovascularization. Subretinal fluid
and choroidal changes have not been reported
in ocular conditions associated with common
agents used for systemic chemotherapy. The
impact of intravenous chemotherapy on choroidal
microvasculature was analyzed in a previous
study, where the authors concluded that systemic
chemotherapy cannot cause a significant change
in choroidal thickness.[16] These findings support
the impression of increased choroidal thickness in
the current case as a primary event.

Recurrence of retinoblastoma may be
manifested by a new retinal tumor, vitreous
seeding, subretinal seeding, or extraocular
findings.[17] Following systemic chemoreduction,
the recurrence rate of retinal tumors ranges from
24 to 44%.[18] The majority of new tumors will be
detected within three years of initial retinoblastoma
diagnosis. In a previous study, most retinal
tumors recurred within five months of initiating
chemoreduction, when the therapeutic period
of chemotherapy had not ended.[19] Although
recurrence of retinoblastoma after 20 years is rare,
complete retinal examination for any active retinal
tumor was performed in our patient, and no sign of
retinal recurrence was detected.

In eyes presenting with exophytic tumors with
subretinal seeding at presentation, recurrence
of subretinal seeding is a particular concern.[17]
Shields et al suggested an average interval of two
months for the recurrence of subretinal seeding
after completion of chemotherapy. However,
calculated recurrence rates of 53 to 62% at one
and three years, respectively, suggested that
subretinal recurrence may happen after several
years.[18] The subretinal seeds may be free running
or fixed to the external retinal surface.[20] Retinal
detachment, in addition to position-dependent
ophthalmoscopic contours, is a characteristic
finding in subretinal seeding of retinoblastoma.[20]
Features of subretinal fluid and its absorption on
follow-up examinations ruled out the probability of
recurrent subretinal seeding as the cause in our
patient.

CSC is an ocular condition characterized
by neurosensory detachment associated with
pathological dilation of choroidal vessels.
A number of systemic disorders, including
autoimmune diseases, hypertension, allergic
respiratory diseases,[2, 21] Helicobacter pylori
infection, obstructive sleep apnea,[22] and
corticosteroid-releasing tumors have been
reported to be related to the pathophysiology
of CSC. Among these, no ocular malignancy
has been shown to be associated with CSC.
Although some conditions with choroidal origin
such as radiation choroidopathy and choroidal
neovascularization have been reported after
treatment of retinoblastoma,[23] in case of
subretinal exudation in an eye with regressed
retinoblastoma, retinoblastoma recurrence or
secondary malignant tumors are considered prior
to choroidal conditions.

This is the first report on the coincidence
of CSC and retinoblastoma in the same eye.
Although blurring of vision and accumulation
of subretinal fluid in a case of retinoblastoma
should be considered as indicators of recurrent
retinoblastoma or treatment adverse effects,
coincidence of CSC and retinoblastoma could be
detected in our patient.

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