Unusual association of diseases/symptoms

Case report

Blind spot: blindness as initial presentation of
subarachnoid haemorrhage
André Leonardo Gordinho, Steeve Rosado, Teresa Mesquita, Nuno Ribeiro Ferreira
Summary
A 47-year-old Caucasian man with arterial hypertension
was admitted after a seizure. At the emergency
department, he presented with high blood pressure,
Correspondence to
bilateral vision loss (evidenced by unresponsiveness
Dr André Leonardo Gordinho,
a​ j.l​ eonardogordinho@​gmail.​com to threatening stimuli), right hemiplegia and severe
agitation. The brain CT angiography showed a diffuse
basal cisterns subarachnoid haemorrhage with a
Accepted 27 March 2018
ruptured basilar aneurysm. He was admitted for
neurovascular procedure and embolisation. The patient’s
neurological examination improved but blindness
persisted. A funduscopic examination revealed a left eye
vitreous haemorrhage and diffuse retinal haemorrhages
in the posterior pole. Assuming the haemorrhages
were the cause of blindness, Terson syndrome was
diagnosed. The patient underwent vitrectomy surgery
being discharged 5 days later maintaining left eye
blindness and able to count fingers from 1 m distance
with the right eye. Two months after discharge, he was
re-evaluated at our clinic with left eye blurred vision and
almost normal right eye visual acuity.
Department of Medicine,
Hospital Beatriz Angelo, Loures,
Portugal

Background

Terson syndrome was first described by Albert
Terson in 1900 as a vitreous haemorrhage occurring secondary to subarachnoid haemorrhage.1
Today, Terson syndrome has a broader use and also
includes other forms of vitreous and retinal haemorrhages associated with subarachnoid haemorrhage.2
The presence of this syndrome is associated with
increased mortality and a reported incidence of
3%–28% of subarachnoid haemorrhages.3 4 There
are many theories regarding the pathophysiology of
this syndrome; a more recent one suggests retinal
vessel rupture secondary to venous hypertension
caused by sudden intracranial hypertension.5
This case illustrates a rare presentation of
subarachnoid haemorrhage. Although Terson
syndrome presence is considered a predisposing
factor of poor prognostic outcome, our patient
survived without neurological motor deficits and
partially recovered his visual acuity.

Case presentation
To cite:
Leonardo Gordinho A,
Rosado S, Mesquita T, et al.
BMJ Case Rep Published
Online First: [please
include Day Month Year].
doi:10.1136/bcr-2017222745

A 47-year-old Caucasian man with medical history
of arterial hypertension was admitted to our emergency department after a seizure. At arrival to
the emergency department, physical examination
showed blood pressure was high (165/110 mm Hg),
with a slow pulse rate (45 bpm) and fluent aphasia,
isocoric and isoreactive pupils, bilateral loss of

vision (evidenced by unresponsiveness to threatening stimuli) and right hemiplegia. He had severe
agitation requiring sedation and orotracheal
intubation.
A brain CT angiography was performed which
showed a diffuse basal cisterns subarachnoid haemorrhage complicated with third and fourth intraventricular haemorrhage (Fisher 4) (figure 1) with
a ruptured saccular aneurysm measuring 5x4 mm at
the top of the basilar artery (figure 2) and signs of
intracranial hypertension. He was classified as Hunt
and Hess grade IV which includes patients with
severe clinical deficits and reduced survival rate
(20%): stupor and moderate to severe hemiparesis.
After the brain CT angiography, he was transferred to the neurosurgery department for neurovascular procedure with stenting and embolisation.
The patient was extubated, and significant improvement was reported: absence of aphasia, a right
hemiparesis grade 3, but the blindness persisted.
Repeat CT scan demonstrated the stent on the top
of the basilar artery, reabsorption of the subarachnoid haemorrhage and a right posterior cerebellar
hypodensity. The patient persisted with complaints
of visual loss. A funduscopic examination revealed
a left eye vitreous preretinal haemorrhage and bilateral diffuse retinal haemorrhages in the posterior
pole (figure 3).
After consulting an ophthalmologist, assuming
the haemorrhages were blocking his vision, he was
submitted to vitrectomy with panretinal photocoagulation. Unfortunately, his left eye vision failed to
improve immediately after the vitrectomy, although
it succeeded with the right eye as he was able to
count fingers from 1 m distance. He was discharged
5 days later.

Differential diagnosis

Other potential causes of visual loss were considered in this case. The first presumption on admission
was that the patient had cortical blindness, but the
repeat CT scan did not reveal any lesion in the cerebral area that could support this hypothesis. This
was a case of subarachnoid haemorrhage. It could
raise the possibility of an anterior communicating
artery aneurysm, but the CT angiography and the
neurovascular procedure only confirmed a basilar
artery aneurysm. At last, there could be retinal ischaemia secondary to vasospasm; however, the acute
presentation with consistency in improvement, and
without any fluctuating or worsening symptoms
within the temporal window of 3–7 days make
it less probable. After performing a funduscopic

Leonardo Gordinho A, et al. BMJ Case Rep 2018. doi:10.1136/bcr-2017-222745

1

Unusual association of diseases/symptoms

Figure 3

Retinography of the right and left eyes, patient’s perspective.

examination, the definite diagnosis of Terson syndrome was
made.

Outcome and follow-up

Two months after discharge, he was re-evaluated showing great
improvement: left eye with slightly blurred vision and an almost
normal right eye visual acuity, being able to distinguish fingers from
2 m distance and read with moderate difficulty. At final follow-up,
his visual acuity was 20/30 (right eye) and 20/50 (left eye).

Discussion

Figure 1 Head CT showing diffuse basal cisterns subarachnoid
haemorrhage with third and fourth ventricular haemorrhage (Fisher
grade 4).

Figure 2 CT angiography showing ruptured saccular aneurysm
measuring 5x4 mm at the top of the basilar artery.
2

Terson’s description of this syndrome was published more than
100 years ago, and its pathophysiology has remained doubtful.
There were many possible explanations, from sudden increase
in intracranial pressure that lead to stasis in the venous drainage
of the eye and orbit resulting in haemorrhage to aneurysmal
blood dispersed within cerebrospinal fluid which migrated into
the optic nerve sheath and deposited.6 7 A more recent description suggests that a sudden rise in intracranial pressure is transmitted along the optic sheath leading to venous hypertension
and rupture of retinal vessels.5
A sudden rise in intracranial pressure due to aneurysmal
rupture is compatible with our case which presented clinically
with seizure and agitation. Most of published cases are due to
anterior circulation aneurysmal rupture.5 Terson syndrome from
posterior circulation aneurysmal rupture, as in our case, is rare.
As in this case, many of the diagnosis may be missed initially at
presentation or misinterpreted as cortical blindness.
Mortality has been reported higher in patients with subarachnoid haemorrhage presenting with Terson’s syndrome.3 Our
patient presented with a grade IV Hunt and Hess classification. In
a meta-analysis published by McCarron et al in 2004, patients with
subarachnoid haemorrhage and Terson syndrome who presented
with higher Hunt and Hess grades were much more likely to die
(43% mortality with an average grade of 3.5 vs 9% of whom scored
2.6).8 Although our patient presented with a high Hunt and Hess
grade, he had a better outcome. The funduscopy (gold standard)
gave us the diagnosis, and the vitrectomy was performed as soon as
possible emphasising the importance of early diagnosis and treatment as it is a reversible cause of blindness. Unfortunately, his left
eye vision failed to improve immediately after the vitrectomy, as it
succeeded with the right eye, and until now we do not have any
explanation for that. The early endovascular procedure contributed to the good outcome presented, also showing the importance
of early intervention.
This case illustrates a rare presentation of subarachnoid haemorrhage, although its presence is considered a predisposing
factor of poor prognostic outcome, our patient survived without
neurological motor deficits and recovered part of his visual
acuity. We highlight the importance of a funduscopic examination as many of the diagnosis may be missed or misinterpreted
as cortical blindness.
Leonardo Gordinho A, et al. BMJ Case Rep 2018. doi:10.1136/bcr-2017-222745

Unusual association of diseases/symptoms
Learning points
►► Terson syndrome is underdiagnosed, it may be present in up

to one-third of subarachnoid haemorrhage, and its presence
is associated with increased mortality.
►► Funduscopic examination is essential in patients with new
onset neurological symptoms, especially in subarachnoid
haemorrhage.
►► Evaluation by an ophthalmologist is fundamental as surgical
intervention may recover part or total visual acuity.
Acknowledgements The authors of the article, Blind spot: blindness as initial
presentation ofsubarachnoid haemorrhage, would like to acknowledge the support
and contribution of Dr. Francisco Araújo and Dr. José Lomelino Araújo.
Contributors ALG was involved in data collection regarding the clinical case,
planning, drafting, editing, design and final approval of submitted version. SR was
involved in data collection regarding the clinical case, editing, revising and final
approval of the submitted version. TM and NRF were involved in editing, revising and
final approval of the submitted version.
Funding This research received no specific grant from any funding agency in the
public, commercial or not-for-profit sectors.
Competing interests None declared.

Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article)
2018. All rights reserved. No commercial use is permitted unless otherwise expressly
granted.

References

1	Terson A. De l’hemorrhagie dans le corps vitre au cours de l’hemorrhagie cerebrale. Clin
Ophtalmol 1900;6:309–12.
2 Frizzell RT, Kuhn F, Morris R, et al. Screening for ocular hemorrhages in patients
with ruptured cerebral aneurysms: a prospective study of 99 patients. Neurosurgery
1997;41:529–33. discussion 533-4.
3	Stienen MN, Lücke S, Gautschi OP, et al. Terson haemorrhage in patients suffering
aneurysmal subarachnoid haemorrhage: a prospective analysis of 60 consecutive
patients. Clin Neurol Neurosurg 2012;114:535–8.
4 Fountas KN, Kapsalaki EZ, Lee GP, et al. Terson hemorrhage in patients suffering
aneurysmal subarachnoid hemorrhage: predisposing factors and prognostic
significance. J Neurosurg 2008;109:439–44.
5 Garfinkle AM, Danys IR, Nicolle DA, et al. Terson’s syndrome: a reversible cause of
blindness following subarachnoid hemorrhage. J Neurosurg 1992;76:766–71.
6 Ballantyne AJ. The ocular manifestations of spontaneous subarachnoid haemorrhage. Br
J Ophthalmol 1943;27:383–414.
7 Muller PJ, Deck JH. Intraocular and optic nerve sheath hemorrhage in cases of sudden
intracranial hypertension. J Neurosurg 1974;41:160–6.
8 McCarron MO, Alberts MJ, McCarron P. A systematic review of Terson’s syndrome:
frequency and prognosis after subarachnoid haemorrhage. J Neurol Neurosurg
Psychiatry 2004;75:491–3.

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Leonardo Gordinho A, et al. BMJ Case Rep 2018. doi:10.1136/bcr-2017-222745

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