QuizWise 39 – Multiple branch retinal artery occlusion and its aftermath

1. Which of the following layers are affected in paracentral acute middle maculopathy?
A. Inner nuclear layer
B. Inner plexiform layer
C. Outer plexiform layer
D.  All of the above

2. Monocular vision loss in CRAO is more commonly due to – 
A. Vitreous hemorrhage
B. Retinal detachment
C. Ischemic optic neuropathy
D. Sub-retinal bleeding

3. Cherry red spot is seen in which of the following conditions? 
A. Central retinal artery occlusion
B. Tay Sachs disease
C. Niemmen Pick disease
D. All of the above

4. Vascular risk factors for CRAO are- 
A. Tobacco intake?
B. Hypertension
C. Hyperlipidemia
D. All of the above

5. The superficial capillary plexus is positioned in which of the retinal layer?
A. Ganglion cell layer
B. Inner plexiform layer
C. Outer plexiform layer
D. Outer nuclear layer

6. Conservative approaches to save vision in CRAO include – 
A. Topical intraocular pressure-lowering agents 
B. Anterior chamber paracentesis
C. Carbogen therapy (inhaling a 95% O2/5% CO2 mixture
D. Ocular massage
E. All of the above

7. Non-embolic causes of BRAO include vasospasm secondary to which of the following conditions?
A. Migraine
B. Cocaine abuse
C. Behcet’s disease
D. All of the above.  

8. Which of the following aspects of vision is affected by CRAO?
A. Central vision
B. Stereopsis
C. Visual field
D. Color vision
E. All of the above

9. Systemic risk factors for the development of BRAO are
A.  Hypertension, 
B. Carotid occlusive disease 
C. Coronary artery disease
D. Hypercholesterolemia
E. All of the above

10. Vascular secondary prevention after CRAO does not include – 
A. Lifestyle modifications
B. Antiplatelet therapy 
C. Regular check-ups with neurologist and ophthalmologist
D. Ocular massage

Answers –

Ans 1 – A. Inner nuclear layer

PAMM is an optical coherence tomography (OCT) finding defined by the presence of a hyperreflective band at the level of the inner nuclear layer (INL) that indicates INL infarction caused by globally impaired perfusion through the retinal capillary system leading to hypoperfusion of the DVC or specifically the DCP. 

Ref – Scharf J, Freund KB, Sadda S, Sarraf D. Paracentral acute middle maculopathy and the organization of the retinal capillary plexuses. Progress in Retinal and Eye Research. 2021 Mar 1;81:100884

Ans 2 – C. Ischemic optic neuropathy

The sudden, painless, monocular visual loss which results from CRAO is due to optic neuropathy which is most often ischemic. 

Ref. Mac Grory B, Schrag M, Biousse V, Furie KL, Gerhard-Herman M, Lavin PJ, Sobrin L, Tjoumakaris SI, Weyand CM, Yaghi S, American Heart Association Stroke Council; Council on Arteriosclerosis, Thrombosis and Vascular Biology; Council on Hypertension; and Council on Peripheral Vascular Disease. Management of central retinal artery occlusion: a scientific statement from the American Heart Association. Stroke. 2021 Jun;52(6):e282-94.

Ans 3 – D. All of the above

A cherry-red spot refers to a red-tinted region at the center of macula surrounded by retinal opacification. Cherry-red spots may be present in a variety of pathologic conditions, including lysosomal storage disorders, retinal ischemia, and retinal infarction. 
It is seen in Central retinal artery occlusion (CRAO), Tay-Sachs disease (GM2 gangliosidosis type 1, infantile amaurotic familial idiocy, B variant GM2 gangliosidosis), GM2 gangliosidosis type 2 or Sandhoff disease, Commotio retinae, Niemann-Pick disease, Sialidosis or mucolipidosis type 1, Toxicity of various drugs like Quinine, Carbon monoxide’

Ref – Tripathy K, Patel BC. Cherry Red Spot. [Updated 2022 May 24]. In: StatPearls Available from: https://www.ncbi.nlm.nih.gov/books/NBK539841/

Ans 4 – D. All of the above

This risk of CRAO increases with age and in the presence of vascular risk factors such as hypertension, hyperlipidemia, diabetes, tobacco exposure, and obesity.

Ref. Mac Grory B, Schrag M, Biousse V, Furie KL, Gerhard-Herman M, Lavin PJ, Sobrin L, Tjoumakaris SI, Weyand CM, Yaghi S, American Heart Association Stroke Council; Council on Arteriosclerosis, Thrombosis and Vascular Biology; Council on Hypertension; and Council on Peripheral Vascular Disease. Management of central retinal artery occlusion: a scientific statement from the American Heart Association. Stroke. 2021 Jun;52(6):e282-94.

Ans 5 – A. Ganglion cell layer 

The NFL capillary plexus and the superficial capillary plexus (SCP), positioned in the ganglion cell layer, collectively constitute the superficial vascular complex or SVC.

Ref – Ophthalmol Vis Sci. 61: 44. Campbell, J. P., M. Zhang, T. S. Hwang, S. T. Bailey, D. J. Wilson, Y. Jia, and D. Huang. 2017. ‘Detailed Vascular Anatomy of the Human Retina by Projection-Resolved Optical Coherence Tomography Angiography’, Scientific Reports

Ans 6 – E. All of the above

The conservative approaches have been used in an effort to restore vision. These include anterior chamber paracentesis, ocular massage, topical intraocular pressure-lowering agents, sublingual isosorbide dinitrate, systemic β-blockade, carbogen therapy (inhaling a 95% O2/5% CO2 mixture), and breathing into a paper bag.Ref. Mac Grory B, Schrag M, Biousse V, Furie KL, Gerhard-Herman M, Lavin PJ, Sobrin L, Tjoumakaris SI, Weyand CM, Yaghi S, American Heart Association Stroke Council; Council on Arteriosclerosis, Thrombosis and Vascular Biology; Council on Hypertension; and Council on Peripheral Vascular Disease. Management of central retinal artery occlusion: a scientific statement from the American Heart Association. Stroke. 2021 Jun;52(6):e282-94.

Ans 7- D. All of the above

Nonembolic causes of BRAO include vasospasm secondary to migraines, cocaine abuse and sildenafil, vasculitidies such as Behcets Disease, coagulopathies, and inflammatory/infectious conditions such as Toxoplasmosis, Herpes Zoster, Lyme disease and Giant Cell Arteritis

Ref – Hayreh SS, Podhajsky PA, Zimmerman MB. Retinal artery occlusion: associated systemic and ophthalmic abnormalities. Ophthalmology. Oct 2009;116(10):1928-1936.

Ans 8 – E. All of the above

CRAO affects central vision (visual acuity), peripheral vision (visual fields), color vision, and stereovision.Ref. Mac Grory B, Schrag M, Biousse V, Furie KL, Gerhard-Herman M, Lavin PJ, Sobrin L, Tjoumakaris SI, Weyand CM, Yaghi S, American Heart Association Stroke Council; Council on Arteriosclerosis, Thrombosis and Vascular Biology; Council on Hypertension; and Council on Peripheral Vascular Disease. Management of central retinal artery occlusion: a scientific statement from the American Heart Association. Stroke. 2021 Jun;52(6):e282-94

Ans 9 – E. All of the above

Risk factors for BRAO include systemic conditions which preclude an individual towards vascular narrowing: hypertension, carotid occlusive disease or atherosclerosis, coronary artery disease, and hypercholesterolemia

Ref – Mason JO, 3rd, Shah AA, Vail RS, Nixon PA, Ready EL, Kimble JA. Branch retinal artery occlusion: visual prognosis. American journal of ophthalmology. Sep 2008;146(3):455-457.

Ans 10 -D. Ocular massage

Secondary prevention (including monitoring for complications) should be a collaborative effort between neurology, ophthalmology, and primary care medicine. Risk factor modification should include pharmacological and lifestyle interventions.Antiplatelet therapy is a reasonable consideration for pharmacological secondary prevention when the cause is cryptogenic or attributed to atherosclerosis.

Ref. Mac Grory B, Schrag M, Biousse V, Furie KL, Gerhard-Herman M, Lavin PJ, Sobrin L, Tjoumakaris SI, Weyand CM, Yaghi S, American Heart Association Stroke Council; Council on Arteriosclerosis, Thrombosis and Vascular Biology; Council on Hypertension; and Council on Peripheral Vascular Disease. Management of central retinal artery occlusion: a scientific statement from the American Heart Association. Stroke. 2021 Jun;52(6):e282-94.


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