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Title:
Ocular Ischemic Syndrome: A case report and review of ocular and systemic
manifestations
Category:
Ocular Disease
Authors:
Raneat Cohen, O.D.
Rebecca Diller, O.D., F.A.A.O
Abstract:
A 72 year-old male complains of painful vision loss in left eye. Vision is NLP, an APD is
present and optic nerve is edematous. Intraocular pressures are elevated and retina is pale
with sclerotic vessels.
I. Case History
A 72 year-old Caucasion male complains of “sudden” painful loss of vision in the left
eye. Ocular history is remarkable for a probable ischemic left CN VI palsy, elevated
optic nerves previously attributed to optic disc drusen, bilateral Hollenhorst plaques, and
mild hypertensive retinopathy OS. Medical history is remarkable for hypertension,
coronary artery disease, hyperlipidemia, and prior cerebrovascular accident. The patient
reports that he recently started acetaminophen with codeine for left periorbital pain.
Other medications include aspirin, lisinopril, simvistatin, carvedilol, and amlodipine
besylate.
II. Pertinent Findings
Vision in the left eye is reduced to NLP (previous visual acuities are 20/30 OD
and 20/70 OS). The pupil is non-reactive and a reverse afferent pupillary defect is
present. Slit lamp exam reveals moderate peri-limbal injection, extensive rubeosis iridis,
and mild anterior chamber reaction in the left. No angle structures are visible with
gonioscopy. Intraocular pressures are 12mmHg OD and 22mmHg OS. The left retina is
pale with sclerotic vessels and the optic nerve is pale and edematous. No retinal
hemorrhages are present. The right fundus is unremarkable. Carotid doppler shows
complete stenosis of the left internal carotid artery (70% noted one year prior) and 80%
stenosis of the right (unremarkable one year prior). Cardiology and vascular surgery are
consulted.
III. Differential Diagnosis
The primary diagnosis is end-stage ocular ischemic syndrome with secondary
neovascular glaucoma. At various stages of the disease, OIS can resemble other vascular
occlusive conditions such as a central retinal artery occlusion, ophthalmic artery
occlusion, and central retinal vein occlusion (Table 1).
IV. Diagnosis and Discussion
Ocular ischemic syndrome (OIS) is a chronic condition which most often results from
carotid stenosis greater than 90%. This leads to vascular hypoperfusion, hypoxia and
ocular ischemia. Patients typically present with unilateral, painful, gradual loss of vision.
Visual prognosis is generally poor for these patients especially in the presence of iris
neovascularization. The 5-year mortality rate in patients with OIS is 40%, with
cardiovascular disease being the leading cause of death. Therefore , it is important to
identify both systemic and ocular manifestations of carotid disease (Table 2), in order to
allow for timely and appropriate management of the condition.
This case is unique both in its presentation and clinical findings. The patient's complaint
of acute vision loss is unusual. This could possibly be explained by an acute embolic
event or possibly a discovery phenomenon as record review indicates gradual loss of
vision over several years. The patient was only experiencing mild pain at the time of
examination but it must be noted that he had self initiated acetaminophen with codeine
for periorbital pain several weeks prior. Although retinopathy in the form of midperipheral hemorrhages is often associated with OIS, retinopathy was no longer present
in this patient due to the advanced stage and chronic nature of his condition.
V. Treatment, management
Treatment of OIS is often unsuccessful in restoring perfusion or preventing progression
of the condition. Panretinal photocoagulation in the presence of neovascularization is
controversial as to whether or not it poses additional benefit. According to research,
panretinal photocoagulation does little to improve visual acuity, however, can minimize
the progression of neovascularization. Palliative care is often implemented.
Cycloablation and even enucleation may be indicated in severe cases.
Evaluation by a neurovascular surgeon is often urgent and the risks and benefits of
surgical and medical treatment options must be thoroughly explored.
Topical combination dorzolamide/timolol BID, prednisolone acetate QID and 5%
homatropine BID were initiated OS for patient comfort. A cardiac consult was ordered to
assess the benefits of open carotid endarterectomy versus a stent of the right internal
carotid artery. No medical intervention was indicated for the left internal carotid artery.
VI. Conclusion
Ocular ischemic syndrome can present with variable clinical findings and
symptomatology, and can therefore be diagnostically challenging for the clinician. This
case highlights an advanced presentation of this condition. Prognosis for these patients is
poor, even post systemic intervention.
Table 1: Review of Differential Diagnosis
Signs
Ocular ischemic syndrome







Central Retinal Artery Occlusion







Ophthalmic artery occlusion






Central retinal vein occlusion





+/- Afferent pupillary
defect (depends on
asymmetry)
Dilated retinal veins, not
tortuous
+/- Cherry red spot
Arterial attenuation
Retinal ischemia
Assymetric retinopathy
Mid-peripheral
hemorrhages
Symptoms
 Gradual, monocular
loss of vision
 Periorbital pain
 +/- Amaurosis fugax
Afferent pupillary defect
+/- Cherry red spot
Arterial attenuation
Segmentation of blood
column
+/- embolus
Retinal ischemia
Normal choroidal perfusion
on angiography

Afferent pupillary defect
+/- Cherry red spot (less
common than CRAO)
Arterial attenuation
Segmentation of blood
column
No embolus visible
Retinal ischemia

+/- Afferent pupillary
defect
(ishchemic/nonischemic)
Diffuse retinal
hemorrhages
Dilated and tortuous retinal
veins
Disc edema
Neovascularization (disc,
retina, or iris)








Sudden, monocular loss
of vision
Usually upon
awakening
+/- Amaurosis fugax
Vision range between
count fingers and hand
motion (unlikely NLP)
Sudden, monocular loss
of vision
Usually upon
awakening
+/- Amaurosis fugax
Vision generally NLP
(worse than CRAO)
Sudden, monocular loss
of vision
Vision generally
20/400 or worse if
ischemic; 20/400 or
better if nonischemic
Table 2: Carotid Occlusive Disease: Systemic and Ocular Manifestations
Systemic Manifestations
Ocular Manifestations
 Transient ischemic attacks
 Transient visual loss
 Difficulty with speech
 Ocular ischemic syndrome – corneal
edema, anterior uveitis, engorged episcleral
 Limb weakness contrallaterally
vessels, orbital pain, iris
 Sensory disturbances contralaterally
neovascularization
 Difficulty with spatial orientation

Retinal pathology- hollenhorst plaque,
 Headaches, often severe
cotton wool spot, asymmetric retinopathy,
dilated retinal veins, optic nerve pallor,
Patient may be asymptomatic
cherry red spot, irregular vessel caliber,
retinal ischemia, sclerotic blood vessels
References:
Kunimoto D, Kanitkar K, Makar M. The Wills Eye Manual. Fourth edition. 2004,
Philadelphia: Lippincott Williams and Wilkins.
Onf T, Paine M, O’Day J. Retinal manifestations of ophthalmic artery hypoperfusion.
Clinical and Experimental Ophthalmology 2002; 30: 284-291
Sanborn G, Magargal L. Arterial Obstructive Disease of the Eye. [cited 08/07/2008];
Available from: file://x:\pages\v3\v3c014.html.
Marks E, Adamczyk D, Thomann K. Primary Eyecare in Systemic Disease. 1995,
Norwalk: Appleton and Lange.
Conflict of interest/acknowledgement of grant support: None
Contact Information:
Raneat Cohen, O.D.
Dayton VA Medical Center
4100 West Third Street
Dayton, OH 45428
[email protected]