(2021) 15:175
Hayashi et al. J Med Case Reports
https://doi.org/10.1186/s13256-021-02746-0

Open Access

CASE REPORT

Ophthalmological symptoms
in a patient with reversible cerebral
vasoconstriction syndrome: a case report
Rijo Hayashi1* , Shimmin Hayashi1,2 and Shigeki Machida1

Abstract
Background: Reversible cerebral vasoconstriction syndrome is characterized by severe headache with reversible
segmental constriction of the cerebral arteries. We present details on a patient with reversible cerebral vasoconstriction syndrome who initially visited an ophthalmologist because of visual symptoms.
Case presentation: A 34-year-old Japanese woman complained of sustained headache and insomnia starting 2 days
after her first childbirth. In addition to the severe headache, a visual field defect was also observed 10 days later. Best
corrected visual acuity at the initial visit was 20/20 and 20/25 for the right and left eye, respectively. Exudative retinal
detachment was noted surrounding both optic heads. Visual field testing revealed left homonymous hemianopsia,
while magnetic resonance imaging demonstrated the presence of edema and infarction of the bilateral basal nuclei
and right occipital lobe. The homonymous hemianopsia and exudative retinal detachment recovered immediately
after treatment with a free-radical scavenger and anticoagulant. There has been no recurrence of symptoms during
the 4 years of follow-up.
Conclusions: We report a case of reversible cerebral vasoconstriction syndrome with ophthalmological symptoms
that were reversible, including serous retinal detachment and homonymous hemianopsia.
Keywords: Reversible cerebral vasoconstriction syndrome, Ophthalmological symptoms, Thunderclap headache,
Postpartum, Homonymous hemianopsia, Exudative retinal detachment
Background
The incidence of stroke in the peripartum and postpartum periods has increased in recent years and might correspond to the observed increase in pregnancy-induced
hypertension [1, 2]. There are several conditions associated with hypertension, including posterior reversible
encephalopathy, reversible cerebral vasoconstriction syndrome, and cerebral venous sinus thrombosis, along with
an increased risk of stroke and vascular dementia [1].
*Correspondence: lhayashi@dokkyomed.ac.jp
1
Department of Ophthalmology, Saitama Medical Center, Dokkyo
Medical University, 2‑1‑50 Minamikoshigaya, Koshigaya, Saitama
343‑8555, Japan
Full list of author information is available at the end of the article

Reversible cerebral vasoconstriction syndrome (RCVS)
is characterized by reversible constriction of cerebral
arteries associated with severe headache [3, 4], which is
referred to as thunderclap headache [5]. There are various precipitating factors, with the most common being
postpartum [6] and exposure to vasoactive substances
[4, 7]. Patients with RCVS usually visit emergency
rooms because of the severe headache. However, postpartum patients with RCVS usually visit obstetricians.
Visual deficits have only been reported in 29% of RCVS
patients [8], and ophthalmological findings have yet to be
reported in detail. In this report, we present ophthalmological findings for a patient with RCVS who visited our
clinic because of visual field defects.

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Case presentation
A 34-year-old Japanese woman suffered from headache
and insomnia starting 2 days after her first childbirth.
During her pregnancy, she did not experience any complications, including pregnancy-induced hypertension.
However, postpartum hypertension was noted (160–
170 mmHg/90–100 mmHg). She visited our clinic complaining of a visual field defect that had appeared 8 days
after her headache onset.
Corrected visual acuities for the right and left eye
were 20/20 and 20/25, respectively. Intraocular pressure
in both eyes was 15 mmHg. The anterior segments and
lenses appeared normal. Multiple yellowish dots in the
deep retina were noted around the optic nerve head in
both eyes (indicated by the arrows in Fig. 1), and resembled Elschnig’s spots and ischemic choroidal infarcts [9].
Optical coherence tomography (OCT) demonstrated
exudative retinal detachments around the optic nerve
head in both eyes (Fig. 2). Visual field testing conducted
by standard automated perimetry showed left homonymous hemianopsia (Fig. 3a).
We referred the patient to a neurologist for neurological examinations regarding the headache and left homonymous hemianopsia. Results from serum biochemistry
tests and a cerebrospinal fluid examination were normal.
Magnetic resonance imaging (MRI) revealed edema in
the basal ganglions and right occipital lobe (Fig. 3b).
At day 2 after starting treatments with an infusion of
a free-radical scavenger (edaravone, 60 mg per day) and
anticoagulant (heparin sodium, 10,000 U per day), the

Page 2 of 6

Fig. 2 Optical coherence tomography examination near the optic
head at the first visit. Exudative retinal detachments were associated
with both optic heads and corresponded to the peripapillary
exudative edema. a right eye, b left eye

Fig. 1 Fundus photograph at the first visit. Exudative edema was noticed surrounding both of the optic heads. Elschnig’s spots (arrows) and
ischemic choroidal infarcts were also revealed

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Fig. 3 Visual field and magnetic resonance imaging
before treatment. a Left homonymous hemianopsia was
revealed. b MRI at 3 days after onset of the severe headache.
T1-weighted-fluid-attenuated inversion recovery (T1-FLAIR) MRI
revealed edema and infarction in the basal ganglions and right
occipital lobe

left homonymous hemianopsia disappeared (Fig. 4a) and
MRI showed subsidence of the brain edema (Fig. 4b).
The treatment was continued for 3 days, and the patient’s
symptoms improved. After improvement of the visual symptoms, cerebral angiography appeared normal
(Fig. 5), and the yellowish dots in the deep retina (Fig. 6a)
and exudative detachments around the optic nerve heads
were no longer observed (Fig. 6b). There has been no
recurrence of symptoms during the 4 years of follow-up.
The patient gave consent for her clinical details and
clinical images to be published.

Discussion
RCVS is an acute and self-limiting disease commonly
accompanied by acute, severe headache. The most significant contributor to RCVS is being postpartum [10–12],
with hypertension also reported to be a contributing factor [13]. The present patient suffered from both hypertension and severe headache at 2 days after her first
childbirth. However, there was improvement in the headache and visual symptoms within 2 weeks. The clinical
course of this patient was consistent with that for RCVS.

Page 3 of 6

Fig. 4 Visual field and magnetic resonance imaging after treatment.
a Left homonymous hemianopsia improved at 2 days after infusion
of a free-radical scavenger and anticoagulant. b MRI at 1 month after
treatment. The lesions in the basal ganglions and occipital lobe were
no longer visible in the follow-up MRI

On cerebral angiography, RCVS can present as segmental narrowing and dilation of one or more arteries
[4]. However, vasoconstriction in cerebral angiography
is not essential in the diagnosis of RCVS, as the sensitivity has been reported to be only about 70% [14].
The normal angiography observed in RCVS patients
may be due to the timing, as vasoconstriction may not
be revealed during the 2 weeks after the initial clinical
onset [15]. The normal cerebral angiography observed
in our patient occurred after the improvement of symptoms, so the vasoconstriction could have possibly been
in remission. The presence of hemorrhage or aneurysm
should be ruled out for a diagnosis of RCVS [8, 16].
Neither hemorrhage nor aneurysm was found in our
patient, who was correctly diagnosed with RCVS.
Because of visual deficits, the patient presented to
our clinic instead of visiting either the emergency room
or her obstetrician. Blurred vision has been previously reported to be one of the clinical manifestations
of RCVS that is due to brain lesions [8, 10, 12, 13, 17].
Although homonymous hemianopsia was previously
reported in a case examined using a confrontation
visual field test [17], detailed ocular findings have not
been reported in other prior RCVS cases. However,

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Fig. 5 Angiography performed after improvement of homonymous hemianopsia. Normal angiography was observed in the patient following
improvement of the homonymous hemianopsia. a Magnetic resonance venography. b Magnetic resonance angiography

deterioration of visual acuity and visual field defects
have been reported in patients with postpartum posterior reversible encephalopathy syndrome (PRES)
[18–20], which shares several common clinical and
radiologic features with RCVS [21]. Although PRES
frequently exhibits a symmetric distribution of changes
in the parietooccipital lobes with a moderate headache, these findings were not consistent with the results
found in our patient. Vasoconstrictive involvement of
the parietooccipital lobes might be a mechanism for
severe visual deficits [22], and is one possible explanation for the homonymous hemianopsia in our patient.
Exudative retinal detachments have also been reported
in PRES patients with hypertension [23] and in pregnancy [24]. Previous studies have reported finding constriction of choroidal arterioles in the acute ischemic
phase of hypertension [25], followed by patchy filling
of choroidal capillaries [26] and dysfunction of the retinal pigment epithelium [25], which induced exudative
retinal detachment. The exudative retinal detachment
found in our patient may also have been due to the same

mechanism, with the presence of the Elschnig’s spots
supporting the constriction of choroidal arterioles.
Excellent clinical outcome has been reported in 80%
of patients [6], with fatalities reported in less than 1%
of RCVS cases [8, 13, 16]. However, there has been a
higher nonbenign outcome rate reported in postpartum
RCVS, with death in 20% and residual deficits in 30% of
the cases [27]. Thus, close observation of patients with
postpartum RCVS is necessary. In addition, any abnormal ocular findings on diagnostic examinations should
be considered potential visual defects associated with the
initial symptoms of RCVS.

Conclusions
In this study, we reported on a patient with a visual
defect that was an initial symptom of RCVS. This is the
first report to present ophthalmological findings for
RCVS. Although RCVS is known to have a good prognosis, sometimes stroke or neurological sequelae can
complicate the condition, and thus, close observation
of these patients is necessary.

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Fig. 6 Fundus photograph and OCT at 1 month after treatment. a Exudative retinal detachments and exudative edema surrounding the optic
heads were no longer visible in the images. b Exudative retinal detachment and edema were no longer visible in the images

Abbreviations
RCVS: Reversible cerebral vasoconstriction syndrome; OCT: Optical coherence
tomography; MRI: Magnetic resonance imaging; PRES: Posterior reversible
encephalopathy syndrome.

Consent for publication
Written informed consent was obtained from the patient for publication of
this case report and any accompanying images. A copy of the written consent
is available for review by the Editor-in-Chief of this journal.

Acknowledgements
Not applicable.

Competing interests
The authors declare that they have no competing interests.

Authors’ contributions
RH interpreted the patient data and was a major contributor in writing the
manuscript. SH performed the clinical examination. SM drafted the work and
substantively revised it. All authors read and approved the final manuscript.

Author details
Department of Ophthalmology, Saitama Medical Center, Dokkyo Medical
University, 2‑1‑50 Minamikoshigaya, Koshigaya, Saitama 343‑8555, Japan.
2
Lively Eye Clinic, 3‑1‑4 Asahicho, Soka, Saitama 340‑0053, Japan.

Funding
This research did not receive any specific grant from funding agencies in the
public, commercial, or nonprofit sectors.

Received: 26 September 2020 Accepted: 17 February 2021

Availability of data and materials
The datasets used and/or analyzed during the current study are available from
the corresponding author on reasonable request.
Ethics approval and consent to participate
Consent was obtained from the patient. This study adhered to the tenets of
the Declaration of Helsinki.

1

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