This document discusses various causes of persistent bilateral visual loss in children and adults. In children, common non-progressive causes include congenital optic nerve anomalies such as optic nerve hypoplasia, morning glory disc anomaly, or optic nerve coloboma. In middle-aged or older adults, common causes of non-progressive bilateral visual loss include non-arteritic anterior ischemic optic neuropathy and glaucoma. Progressive bilateral visual loss can be caused by conditions affecting the optic nerves, chiasm, or retrochiasmal pathways such as pituitary tumors, aneurysms, or toxic/nutritional optic neuropathies. A thorough history, exam, and testing is needed to determine the underlying etiology in each case.

1. Approach to a patient with bilateral
vision loss
Dr. Nishtha Jain
Senior Resident
Department of Neurology
GMC, Kota.

2. Question 1
Patient with visual
loss
Monocular Binocular

3. Question 2
Patient with Bilateral
visual loss
Transient Persistent

4. Persistent bilateral visual loss
Both anterior visual visual pathways
Both optic Nerves
Chiasmal involvement
Retrochiasmal involvement

6. Question 3 Patient with persistent
bilateral visual loss
Non
progressive
Progressive

7. Age of presentation
Associated neurological symptoms
Risk factors
Family history
Any particular sector of visual field
affected
Associated visual symptoms
Additional History

10. A child coming with persistent
non progressive bilateral
visual loss

11. Ophthalmologic conditions
• Anophthalmos and microphthalmos
• Persistent hyperplastic primary vitreous
• Infantile glaucoma
• Retinal dystrophies
• Congenital Cataract
• Retinopathy of prematurity

12. Congenital Tilted Disc
Syndrome
• 1% to 2% of the population.
• The disc is tilted in an inferonasal direction.
• Bilateral in 80% of patients
• Result in myopia and astigmatism.
• Also cause bitemporal hemianopic field defects that can
mimic those seen in chiasmal lesions but defect crosses
the midline(not observed with chiasmal hemianopias).

14. Optic Nerve Coloboma
• Caused by incomplete closure of the embryonic fissure.
• May occur sporadically.
• In bilateral cases with systemic involvement, inherited in
an autosomal dominant pattern.
• Clinical findings- visual field defects and decreased
visual acuity.

15. • An enlarged, sharply
circumscribed, glistening
disc with a deeply
excavated inferior border.
• With time, serous
macular detachment may
occur.

16. Morning Glory Disc Anomaly
• A variant of optic disc coloboma.
• More common in females
• Result of posterior displacement of the nerve and
peripapillary retina
• Due to incomplete closure of the fetal fissure.
• Retinal detachment (typically involving only the posterior
pole) may result.

18. Optic Nerve Hypoplasia
• Most common congenital optic disc anomaly.
• Bilateral in 56% to 92% of patients.
• Localized (typically arcuate and peripheral) visual field
loss is common.
• Defective chiasm/midline development resulting in
retrograde degeneration or defective differentiation of
ganglion cells.
• Proposed risk factors - young maternal age, first parity,
maternal smoking, preterm birth, and the use of fertility
drugs and antidepressant medications.

19. • Superior segmental optic nerve hypoplasia, is
characterized by selective involvement of the superior
portion of the optic nerve head, resulting in a ‘‘topless
optic nerve.’’
• Almost exclusively seen in children of diabetic mothers.
• The triad of optic nerve hypoplasia, absence of the
septum pellucidum, and hypopituitarism is termed septo-
optic dysplasia (de Morsier syndrome).
• MRI is recommended for all children presenting with
optic nerve hypoplasia.

20. • The optic disc is small in
diameter(one-half to one-
third of normal size).
• The nerve may be pale in
color and surrounded by
a yellowish peripapillary
halo circumscribed by a
darker ring of pigment
(double-ring sign).

21. Middle/old age person with
bilateral
sequential/simultaneous
non progressive visual loss

22. Optic disc is swollen
Visual field loss is typically inferior
altitudinal
Risk factors - systemic hypertension,
diabetes mellitus,
hypercholesterolaemia
Non arteritic anterior ischemic
optic neuropathy

23. • Visual acuity spontaneously improves in over 40% by six
months but without improvement in the visual field loss.
• The optic disc swelling resolves to leave optic disc pallor,
often segmental.
• Does not recur in the same eye but there is about 15%
risk of fellow eye involvement.
• Low dose aspirin reduce this risk and is usually
recommended, together with control of risk factors
amenable to treatment.

24. Associated with Jaw Claudication, polymyalgia
rheumatica and constitutional symptoms
Tenderness or absence of pulsation of the
superficial temporal arteries
Fundus shows optic neuropathy with central
retinal artery occlusion
Arteritic anterior ischemic
optic neuropathy

25. • Erythrocyte sedimentation rate (ESR) and C reactive
protein (CRP) are usually raised.
• Temporal artery biopsy - with a specimen of at least 2 cm
in length.
• Emergency systemic steroid treatment.
• Standard initial treatment is oral prednisolone (1–1.5
mg/kg/day).

26. Sudden onset of headache, decreased
acuity and/or visual field loss, altered
mental status, and hormone dysfunction
Visual field defect -superior bitemporal
quadrantanopia
Pitutary Apoplexy

27. • Age - 37 to 57 years
• Male-to-female predominance of 2:1.
• Lumbar puncture may disclose elevated opening
pressure, pleocytosis, increased red blood cells, and
xanthochromia.
• MRI is the imaging study of choice.

28. Stroke

29. Persistent Progressive
Bilateral Visual Loss

31. Causes of Retinopathy
• Diabetes Mellitus
• Hypertension
• Atherosclerosis
• Systemic vasculitis
• Blood dyscrasias
• Systemic infections
• Radiation

33. Fundus Autofluorescence imaging
• Metabolic mapping of naturally and pathologically
occuring flurophores of ocular fundus.
• Flouroscence is mainly derived from lipofuscin.
• Uses : Cystoid macular oedema, wet AMD, Choroidal
neovascularisation, Diabetic macular oedema

34. Optical Coherence Tomography
• Noninvasive imaging technique
• Provides high-resolution, cross-sectional images of the
retina, retinal nerve fiber layer and the optic nerve head.
• Time-domain detection- 400 A-scans per second with an
axial resolution of 8–10 μm in tissue.
• Spectral domain (Fourier domain) – 20000–52000 A-
scans per second and a resolution of 5–7 μm in tissue.

35. • Ultra high-resolution OCT - achieve 3 μm resolution in
tissue.
• Swept-source OCT - the interference spectrum is
measured by photodetectors instead of a spectrometer.
• Used in - Age-related macular degeneration, central
serous chorioretinopathy, polyploidal choroidal
vasculopathy, Diabetic retinopathy, inherited retinal
dystrophies

36. Optic Neuropathy
Classic features of an optic neuropathy:
• (1) central visual loss,
• (2) clear view through the ocular media to the optic
nerve,
• (3)a swollen or pale optic nerve head.

38. Autosomal Dominant
Optic Atrophy
• Also K/A Kjer optic atrophy.
• Characterized by decreased acuity, dyschromatopsia,
abnormal visual fields (often central or cecocentral
scotomas), and optic nerve pallor in the papillomacular
bundle.
• Approximately the time children reach school age.
• Bilateral visual loss is the rule, but may be asymmetric.

39. • Insidious progression of visual loss, but typically not past
the second decade of life.
• Tritanopia (an inability to distinguish between colors in
the blue green section of the spectrum) is the classic
pattern of color loss in patients with dominant optic
atrophy.

40. Leber Hereditary Optic Neuropathy
• Maternally inherited condition.
• Result in degeneration of the retinal ganglion cells and
their axons.
• Young males are most commonly affected
• Typical clinical course - acute visual loss in one eye,
followed weeks to months later by visual loss in the
fellow eye.
• More than 97%of patients - involvement in the second
eye within 1 year.

41. • Visual acuity is variable - 20/200 or worse.
• Significant loss of color vision.
• Central visual field defects.
• Three mitochondrial DNA point mutations : 11778 (69%
of cases), 3460 (13% of cases), and 14484 (14% of
cases).
• 14484 mutation - better visual acuities and better rates of
spontaneous recovery than patients with other
mutations.

42. Funduscopic examination
• circumpapillary
telangiectatic
microangiopathy,
• swelling of the nerve fiber
layer around the disc
(pseudoedema)and
• absence of leakage from
the disk or peripapillary
region on fluorescein
angiography.

43. Toxic Optic Neuropathy
• Portions of the anterior visual pathway (optic nerve,
retina, and chiasm) are susceptible to direct damage
from toxins or indirect damage from nutritional deficiency
states.
• Affect both eyes due to the systemic pathophysiology.
• A detailed history, including medications and habits,
must be obtained.

44. • In patients with bilateral visual loss, serum B12, folate
levels, vitamin assays, complete blood count, blood
chemistries, urinalysis, and a serum lead level (if
appropriate) may be useful in identifying the underlying
cause.
• Treatment is focused on removal of the offending agent,
repletion of deficient vitamins, and improving nutrition
status.

45. Common Causes

46. Glaucomatous Optic Neuropathy

49. Sellar Mass
• Gradually progressive visual loss, particularly if
associated with endocrine dysfunction.
• The classic visual field defect is the bitemporal
hemianopia.
• Other examination features of chiasmal visual field loss
include postfixation blindness.
• Mass or trauma involving the chiasm can result in
seesaw nystagmus.

50. • In children commonly include chiasmal/ hypothalamic
glioma and craniopharyngioma.
• In adults, pituitary adenoma is the most common.
• Of the secreting adenomas, prolactin secreting tumors
are the most common.
• Corticotropin-secreting pituitary tumors are seen most
commonly in women during childbearing years.

52. • Managed by transsphenoidal neurosurgery.
• Prolactin-secreting tumors can be treated with dopamine
agonists.
• In up to 80% of patients, gradual titration of
bromocriptine to a dose of 2.5 mg to 5 mg 3 times a day
can successfully reduce tumor size.

53. ANEURYSM
• Common aneurysm locations resulting in chiasmal
defects include the supraclinoid internal carotid artery,
the junction between the carotid and ophthalmic artery,
and, less commonly, the cavernous or anterior
communicating arteries.
• Neurosurgical clipping, when possible, is preferable to
endovascular embolization.

54. Transient Binocular Visual
Loss

55. Transient Binocular Visual Loss

56. TIAs
• TIAs cause a sudden onset of homonymous binocular
TVL.
• Can result from primary arterial stenosis or occlusion,
secondary occlusion due to embolism from a distant
source, or, less commonly, from arterial dissection.
• Infrequent causes of posterior circulation TIAs include
subclavian steal syndrome and ‘‘bow-hunter’’ syndrome.

57. • Very rarely, giant cell arteritis (GCA) can cause isolated
vertebral arteritis and without producing any of the other
characteristic symptoms.
• The neurologic and ophthalmic examinations are usually
normal between TIAs.
• Abnormalities on cardiovascular examination.

58. • Imaging of the intracranial and extracranial (head and
neck) vessels using Doppler ultrasound and CT or
MR/catheter angiography should be obtained.
• If dissection is suspected, fat-saturated T1-weighted
images of the head and neck should be obtained.
• Ancillary studies, such as screening tests for GCA.

59. Vasculitis
• GCA can cause brief episodes of transient monocular or
binocular visual loss.
• The episodes are characteristically precipitated by
postural maneuvers due to anterior or posterior
circulation compromise.
• As TVL may be a warning symptom for impending
anterior ischemic optic neuropathy, an erythrocyte
sedimentation rate, C-reactive protein, platelet count,
and fibrinogen level should be obtained urgently in all
older patients with TVL.

60. Systemic Hypoperfusion
• Systemic hypoperfusion due to hypotension or impaired
cardiac output can produce brief episodes of transient
binocular visual loss that are sudden in onset
• Characterized by a concentric loss of vision from the
periphery.
• Other symptoms - lightheadedness, syncope, chest pain,
palpitations, or dyspnea.
• Common causes - vasovagal attacks, cardiac
arrhythmias, valvular heart disease (eg, aortic stenosis),
and orthostatic hypotension.

61. Migraine Aura
• Common cause of episodic transient homonymous
visual loss.
• The classic visual aura is the fortification spectrum, in
which an achromatic or black and white figure with an
angulated scintillating edge appears near the center of
the visual field and gradually expands concentrically
toward the periphery over minutes, leaving a bean-
shaped scotoma.

62. • Lasts for 15 minutes or longer, rarely for more than 60
minutes.
• Followed by a severe unilateral throbbing headache.
• In cases where the visual aura is always lateralized to
the same side, the headache precedes the aura, or there
is a persisting neurologic deficit, neuroimaging should be
performed to exclude a structural lesion such as an
arteriovenous malformation.

63. • In basilar-type migraine, there may be an aura producing
transient cortical blindness or homonymous visual field
loss.
• An evaluation for alternative diagnoses, such as
vertebrobasilar ischemia, is indicated, especially in older
patients or those with vascular risk factors.

64. Occipital Seizures
• A sudden onset of binocular elementary positive visual
phenomena with associated visual loss.
• The positive visual phenomena consist of multiple,
brightly colored, small circular spots, circles, or balls.
• They are usually located in the contralateral hemifield.
• Vision is obscured in the area occupied by the
hallucinations from the time of onset.
• The positive visual phenomena usually last for less than
a minute.

65. • Causes- PRES, metabolic encephalopathies,
malformations of cortical development, neoplasms,
vascular lesions, prior head trauma, metabolic diseases
(eg, mitochondrial disease), localized infections, or
idiopathic.

66. PRES
• Transient cortical blindness and a variety of other
homonymous visual field defects.
• Causes - malignant hypertension, preeclampsia, renal
failure or in those taking immunosuppressive treatments.
• characterized by visual symptoms, headache, altered
mental status, and seizures.
• Imaging typically shows bilateral subcortical and cortical
edema in the occipital and occipito-parietal regions.
• With treatment directed toward the underlying cause,
vision usually recovers over 1 to 2 weeks.

67. Angiography and Contrast Media
Exposure
• Due to a breakdown of the blood-brain barrier by the
contrast media with consequent neurotoxic effects.
• Symptoms - headache, altered mental status, and
memory disturbances, typically develop during or shortly
after contrast exposure.
• Extravasation of contrast in the occipital lobes on brain
CT
• Brain MR imaging - increased signal in the occipital
lobes without evidence of infarction.

68. Head Trauma
• Isolated transient cortical blindness may rarely result
from minor blunt head trauma, usually direct occipital
trauma, in children or adolescents.
• The visual loss commonly develops within minutes after
the event.
• Imaging is typically normal.
• Most patients regain vision within minutes to hours.
• Neuroimaging should be obtained to exclude an
intracranial hemorrhage.

69. Papilledema
• Well known cause of episodic TVL.
• Transient visual obscurations, are characterized by
complete or partial loss of vision that lasts for seconds,
followed by a rapid recovery of vision to baseline.
• Precipitated by postural changes and maneuvers that
increase intracranial pressure.
• Occur many times per day.
• Due to transient ischemia of the swollen optic nerve
head.

70. • Neuroimaging should be obtained urgently to exclude a
structural cause, such as a mass lesion, obstructive
hydrocephalus, or venous sinus thrombosis.
• Patients with normal imaging should undergo lumbar
puncture.
• Treatment of the underlying cause.

72. Optic Disc Drusen
• Brief episodes of TVL can occur in patients with optic
disc drusen.
• Visible on funduscopy.
• However, when the drusen are buried, the disc
appearance can mimic that of papilledema and B-scan
ultrasonography or CT may be required to demonstrate
their presence.

74. To conclude
• History
• Thorough examination including fundus and perimetry
• Newer techniques available
• Urgent diagnosis must in certain conditions to prevent
permanent visual loss

75. Thank You

76. Referrences
• Diagnostic approach to visual loss. Newman et al. Continuum (Minneap
Minn) 2014;20(4):785–815.
• Severe Visual Impairment and Blindness in Infants. Gogate et al. Middle
East African Journal of Ophthalmology, Volume 18, Number 2, April - June
2011.
• Ocular and systemic causes of retinopathy in patients without diabetes
mellitus. Venkatramani et al. BMJ 2004;328:625–9.
• Optical coherence tomography – current and future applications. M Adhi et
al. Curr Opin Ophthalmol. 2013 May ; 24(3): 213–221.
• Bilateral visual loss Approach, localization, And Causes. Christopher C.
Glisson. Continuum Lifelong Learning Neurol 2009;15(4).
• Disorders of the anterior visual pathways. S A Madill et al. J Neurol
Neurosurg Psychiatry 2004;75(Suppl IV):iv12–iv19.
• Approach to acute visual loss. Khadilkar et al. Medicine update. 2012.
• Transient visual loss. Thurtell and Rucker. International ophthalmology
clinics. 2009.