Control #: 1137
Title: Impaired vision : how to
optimize imaging and diagnosis
hypotheses
eEdE#: eEdE-107 (Shared Display)
No disclosure
IMPAIRED VISION
HOW TO OPTIMIZE IMAGING AND
DIAGNOSIS HYPOTHESES
F HERAN-DREYFUS1, J SAVATOVSKY1, C VIGNAL-CLERMONT2
With the kind contribution of Drs F Audren2, O Berges1, F Charbonneau1,
M Elmaleh-Berges3, A Leclerc1
1 Imaging department - Fondation Rothschild Paris
fheran@fo-rothschild.fr
2 Neuroophthalmology department - Fondation Rothschild Paris
3 Imaging Department - Pediatric Hospital R Debré Paris
VISUAL LOSS, VISUAL FIELD (VF) ABNORMALITIES
Many causes, disclosed by
Ophthalmological examination: fundus, VF, fluorescein
angiography, Optical Coherence Tomography (OCT)
Complementary imaging (MR, CT Scan, US)
Selection of the appropriate imaging depends on
Ophthalmological data
Age of the patient
Clinical presentation (onset, supposed lesion location)
Based on a daily experience in an ophthalmological
reference center, this practical clinical and imaging
guide aims to improve self-confidence and radiological
know-how of radiologists facing vision impairment.
OPTIMIZATION AND IMPROVEMENT
THEY WILL NEED
Knowledge of the basic vision physiology (anterior and posterior
visual pathways) and of the relationship between clinical data
and location of the lesion.
Use of elementary clinical data: onset of the symptoms, age of
the patient in the choice of the protocol and discussion of the
etiologies.
Understanding and use of MRI protocols with a specific attention
to the image quality.
Analysis of the images.
THESE NOTIONS WILL BE DISPLAYED AND
PROGRESSIVELY ACQUIRED DURING THE READING
Eventually, click on this icon to go to a specific slide, and on the back icon to return to your previous slide
LESION ETIOLOGY
OPHTHALMOLOGICAL ORIENTATION
LOCATION OF THE LESION
Visual pathways organization
On the next slides, we kept the radiological representation of the
brain, with right on the left, knowing that visual field diagrams are
provided opposite to MRI slice orientation.
Notice that the different pathologies will be presented as
unilateral vision loss. Most of these may be bilateral. Slides
summing the bilateral visual loss etiologies will be included at
the end of the presentation.
ANATOMY AND PHYSIOLOGY
NASAL FIBERS
NASAL
RETINA
NASAL
RETINA
NASAL
FIBERS
NASAL
FIBERS
TEMPORAL FIELD
TEMPORAL FIELD
CROSS THE MEDIAN LINE WITHIN THE OPTIC CHIASM
TEMPORAL FIBERS
TEMPORAL
RETINA
TEMPORAL
RETINA
TEMPORAL
FIBERS
TEMPORAL
FIBERS
NASAL FIELD
NASAL FIELD
REMAIN ON THE SIDE OF THE OPTIC NERVE
MACULAR FIBERS
MACULA
MACULAR FIBERS
VISUAL ACUITY
PARTLY CROSS AND PARTLY REMAIN ON THE SAME SIDE
CLINICAL-IMAGING RELATIONSHIP
According to the lesion location
Retina, optic nerve
 VISUAL LOSS
Optic chiasm
 BITEMPORAL HEMIANOPIA
(BTH)
Optic tracts and radiations
 HOMONYMOUS HEMIANOPIA
(HH)
LESION ETIOLOGY: OPHTHALMOLOGICAL ORIENTATION
ONSET OF THE SYMPTOMS
Sudden
Vascular (ischemia, hematoma)
Trauma
Progressive
Rapid (within days): inflammation, toxic
Slow (within weeks): tumor (intrinsic or
extrinsic), degenerative disease
Be careful and open-minded,
as clinical presentation may be misleading…
HOW TO SELECT THE BEST IMAGING METHOD ?
MRI: First choice (unless contra-indicated)
Excellent parenchyma depiction
Analyze of the optic nerve and optic
chiasm
Data about morphology and structure of
the lesion
CT scan: if trauma, extreme emergency
US: neck vessels study
WHICH MRI PROTOCOL ?
OPTIC NERVE LESION SUSPECTED ?
Patient eyes shut, no make up
1 Basic:
Coronal T2 thin slices
from the posterior aspect of ocular bulb to the optic tracts
2 Complementary:
Coronal T1 Fat Sat injected thin slices
Axial T2 and T1 Fat Sat injected thin slices
in the optic nerve plane
If available, dixon* sequences may be used
* Four image sets with different tissue contrast are obtained during one sequence:
in‐phase, out‐of‐phase, water only and fat only.
BRAIN STUDY IS NEEDED ?
1 Basic, depending on the clinical data:
Axial or 3D FLAIR
Diffusion
3D TOF, neck vessels study
Susceptibility sequence (SWI, T2 GE)
Axial or 3D T1 after injection
2 and if necessary:
T2 GE Perfusion sequence
SpectroMR
……………..
Let’s now combine all these essential notions
and meet real life
Back
1 VISUAL DEFECT AFTER TRAUMA
CT scan (bone fragment along the optic pathway)
MRI is useful when no fracture is
depicted and/or to demonstrate optic
nerve section or edema
Optic canal fracture ()
LEFT OPTIC NERVE SECTION
CT Scan
Acute visual loss after head trauma: Optic nerve section ()
Diagnosis keys: linear area of high signal T2, mainly near the orbital apex
T2 DP
T2
T1 Gd Fat Sat
Urolagin SB Kotrashetti SM, Kale TP Balihallimath LJ Traumatic optic neuropathy after maxillofacial trauma: a review
of 8 cases. J Oral Maxillofac Surg.2012 May;70(5):1123-30.
2 SUDDEN UNILATERAL VISUAL LOSS
It mainly means vascular event (same imaging rules as brain
ischemia)
TRANSIENT: embolism ? in emergency, first look for an internal
carotid stenosis
TRANSCIENT VISUAL LOSS
Man 65 Year-old, several recent episodes of sudden
transient right visual loss.
Major stenosis of the right internal carotid ().
EDC
Plaque > 50% diameter reduction
peak systolic velocity > 230 cm/sec (nl <125 cm/s)
CT Scan
MRI
Courtesy of Dr Augustin Lecler , Dr
Frédérique Charbonneau
PERMANENT: optic nerve or retinal ischemia.
Specific visual field (loss of the nasal part of the VF, inferior
altitudinal defect) and fundus appearance (edema).
LEFT OPTIC NERVE ISCHEMIA
Diffusion: positivity is very seldom, revealing an
ischemic area within the optic nerve ()
or papilla ().
B1000
Man 75 Year-old, sudden permanent left visual loss.
Diagnosis keys: Papilledema, VF.
ADC
Papilledema
inferior altitudinal defect
Add a complete brain vascular protocol
Hayreh SS, Zimmerman BVisual field abnormalities in nonarteritic anterior ischemic optic neuropathy: their pattern
and prevalence at initial examination. Arch Ophthalmol.2005 Nov;123(11):1554-62.
3 RAPIDLY PROGRESSIVE UNILATERAL VISUAL LOSS
It generally means optic neuritis (ON) (frequent associated pain
is due to spreading of the posterior inflammation toward the
oculomotor muscles apex insertion). It may be bilateral.
ON have numerous causes, among them
Demyelination: Multiple Sclerosis (MS), Optic
neuromyelitis (ONM), Acute encephalomyelitis (ADEM),
Infection: Lyme disease, viruses, tuberculosis…
Inflammatory diseases: granulomatosis (sarcoidosis),
Behcet disease …
Toxic: radiotherapy...
MULTIPLE SCLEROSIS (MS)
Two cases of acute optic neuritis in patients with MS, known (1 up) and unknown (2 down)
Typical inflammatory lesion: high signal on T2 (), enhanced on T1 after injection ().
Diagnosis keys: small lesion, transient contrast enhancement, white matter lesions.
1
T2
T1 Fat Sat Gd
T2
T1 Fat Sat Gd
2
SARCOÏDOSIS
T2
T2
T1 Gd Fat Sat
Diagnosis keys: involvement of a whole nerve, frequent optic chiasm location (), strong
and long standing contrast enhancement (), associated lesions (e.i. orbital or pituitary
area inflammation, …)
COMPLICATION OF RADIOTHERAPY
T1 Gd Fat Sat
T1 Gd
T2
Diagnosis keys: radiotherapy and long standing enhancement (vasculitis processes) with
high signal lesion on T2 (), enhanced on T1 after injection ().
4 SLOWLY PROGRESSIVE UNILATERAL VISUAL LOSS
1 OPHTHALMOLOGICAL EXAMINATION DISCLOSES A PAPILLEDEMA.
Once drüsen are ruled out, several etiologies must be evocated,
and MRI is performed as soon as possible.
Intracranial hypertension (venous thrombosis, idiopathic,
tumor…)
Optic nerve compression
Optic nerve tumor
Papilledema
OPTIC NERVE GLIOMA
A
Children with optic nerve glioma () isolated (A and B), bilateral
associated to optic chiasm glioma () and NF1 (C ).
Diagnosis keys: thick nerve, discrete visual loss, white matter T2
hypersignal lesions ()
Back to children BTH
C
B
T1
T2
T1 FatSat Gd
A
T2
T2
T1
T2
T1 FatSat Gd
T1 FatSat Gd
OPTIC NERVE MENINGIOMA
Woman 53 year-old. Progressive visual loss. Papilledema. Optic nerve
() surrounded by meningioma ().
Flattening of the posterior eyeball (). Intracrânial extension, major
complication (), depicted with T1 Gd FatSat, mandatory.
Diagnosis keys: Contrast enhanced lesion around an atrophic optic
nerve, calcifications possible.
T1 FatSat Gd
T1 FatSat Gd
T2
T1 FatSat Gd
* Mouton S, Optic nerve sheath meningioma. Experience in Lyon with twenty patients]. Rev Neurol (Paris). 2007
May;163(5):549-59.
IDIOPATHIC INTRACRANIAL HYPERTENSION (ICHT)
Venous MRA
Lumbar punction. CSF
hypertension evaluation.
T2
T2
T1
Overweighed woman 29 year-old, visual loss. Bilateral papilledema.
Typical idiopathic ICHT on MRI: dramatic dilatation of the perioptic spaces (), posterior hypersignal of
both optic nerves on T2 WI (), lateral sinuses stenosis (), arachnoidocele ().
* Woodall MN Bilateral transverse sinus stenosis causing intracranial hypertension. BMJ Case Rep.2013 Aug 20;2013.
* Riggeal BD Clinical course of idiopathic intracranial hypertension with transverse sinus stenosis. Neurology. 2013 Jan
15;80(3):289-95
2 OPHTHALMOLOGICAL EXAMINATION DISCLOSES OPTIC ATROPHY
sometimes during a routine control of the fundus, the patient being
anaware of the visual loss. Even if duration of the atrophy is
impossible to guess, MRI is performed as soon as possible to rule
out optic nerve compression.
The other etiologies are
Sequels of trauma, ON, compression, anything that injured
the nerve may be responsible…
Glaucoma: the papilla is excavated, a specific examination
of the optic chiasm is mandatory. Should it be atrophic, a normal
tension glaucoma is the first pathology to suggest.
Degenerative or toxic affection
NORMAL TENSION (NT) GLAUCOMA*
* low-tension or normal-pressure glaucoma
Glaucoma, 2nd leading cause of visual loss in the world. Slowly
progressive neuronal degenerative process along the visual pathway.
Ophthalmological data associate papilla excavation, visual loss, with
raised or normal intra ocular pressure.
Left l visual loss with papillary excavation . Left optic nerve () and optic chiasm () atrophy.
NT Glaucoma.
Diagnosis keys: optic chiasm atrophy, papillary excavation.
1
3
T2
2
4
T1
Zhang YQ, et al Anterior visual pathway assessment by magnetic resonance imaging in normal-pressure
glaucoma. Acta Ophthalmol. 2012 Jun;90(4):e295-302
Back
5 BITEMPORAL HEMIANOPIA
The optic chiasm is injured, often by a neighbor lesion
Lesion on both nasal fibers pathway:
ONE SITE ONLY, THE OPTIC CHIASMA
Typical visual field
THE PATIENT IS AN ADULT
First cause to suggest: compression
PITUITARY MACROADENOMA
Man 47 year-old. Progressive visual alteration, VF: Bitemporal hemianopia.
Huge lesion (), mass effect on the optic chiasm ().
Diagnosis keys: Tissue lesion growing within and above the sella, no normal pituitary gland disclosed.
T1
T2
T1 Gd
Monteiro ML, Zambon BK Cunha LP Predictive factors for the development of visual loss in patients with pituitary
macroadenomas and for visual recovery after optic pathway decompression. Can J Ophthalmol.2010 Aug;45(4):404-8.
Other: craniopharyngioma, hypophysitis, cavernous sinus or third
ventricle floor lesion
CHORDOID GLIOMA
Man 63 year-old. Progressive visual alteration. VF: Bitemporal hemianopia. Huge suprasellar lesion (),
mass effect on the optic chiasm ().
Diagnosis keys: ovoid mass, well circumscribed, in the region of the hypothalamus/anterior
third ventricle, enhanced uniformly and intensely.
T2
T2
T1
T1 Gd Fat Sat
Pomper MG Chordoid glioma: a neoplasm unique to the hypothalamus and anterior third
ventricle. AJNR Am J Neuroradiol.2001 Mar;22(3):464-9.
More seldom: inflammation (MS, NMO, granulomatosis)
T1 Gd
SARCOIDOSIS
Woman 35 Year-old, bitemporal
hemianopia. Optic chiasm and pituitary
stalk granuloma ().
T1 Gd
Rarely, an abnormal shape of the globes may provoke a pseudo
bitemporal hemianopia.
MYOPIC DEFORMATION
Man, 52 year-old, bitemporal hemianopia noticed during systematic examination. Myopia.
Oblique insertion of optic nerve head
Ectasia of nasal sector of the globes with posterior nasal wall
thinning ()
Flattened temporal aspect of the globes ()
T2
Manfrè L, Vero S, Focarelli-Barone C Lagalla R Bitemporal pseudohemianopia related to the "tilted disk" syndrome: CT,
MR, and fundoscopic findings. AJNR Am J Neuroradiol.1999 Oct;20(9):1750-1.
THE PATIENT IS A CHILD
Two main etiologies: Compression and optic chiasm tumor,
mainly glioma.
CRANIOPHARYNGIOMA
Glioma images
Young 4 year-old boy. Visual loss. Huge suprasellar cystic () and solid () lesion enhanced after
injection (), mass effect on the optic chiasm which is pushed back (). Craniopharyngioma. Complete
resection at control MRI ().
Diagnosis keys: Suprasellar, cystic and solid mass, often calcified (SWI, CT Scan).
T1 Gd
T1 Gd
SWI
T2
Mortini P Neurosurgical treatment of craniopharyngioma in adults and children: early
and long-term results in a large case series. J Neurosurg.2011 May;114(5):1350-9.
T1 Gd post surgery
Back
6 HOMONYMOUS LATERAL HEMIANOPIA
Something is happening beyond the optic chiasm.
Temporal, parietal and occipital lesions on the optic radiation
pathways have many causes. Brain MRI protocol depends mainly on
the onset of the symptomes.
Brutal : Ischemia ? Hematoma? Trauma ?
Progressive : Tumor ? Inflammation ?
You know brain lesions…
Have a special look at the optic tracts !
POST TRAUMA OPTIC
TRACT ATROPHY
Man 42 years-old. Systematic examination discloses a left HH.
MRI: right optic tract atrophy () attributed to a severe
head trauma during childhood.
T2
OPTIC TRACT INFLAMMATION: MS
Woman 36 years-old, complains of the rapid onset of right
homonymous inferior quadranopia, confirmed by the VF ().
Left optic tract lesion, with hypersignal on FLAIR and T2 (),
enhanced after gadolinium injection (). MS.
Right eye
FLAIR
Left eye
T1 Gd
T2
T2
Due to optic radiation vascularization, either posterior cerebral artery
(PCA) or middle cerebral artery (MCA) ischemia may induce HH.
PCA ISCHEMIA
Man 78 year-old complains of sudden visual disturbance. Ophthalmological examination discloses a
discrete right homonymous quadranopia. MRI: acute left CPA ischemia ().
Diagnosis keys: hypersignal diffusion lesion with low ADC, located around the left calcarine sulcus.
FLAIR
FLAIR
B1000
ADC
7 CHILDREN: CONGENITAL DISEASES
The disease may be congenital and revealed by nystagmus,
strabismus.
MRI needs often specific protocol and general anesthesia or sedation.
OPTIC NERVE HYPOPLASIA
Most frequent etiology (12 % of congenital visual disturbances), often
bilateral (85%), isolated or associated to brain malformation such as
schizencephalia, septo optic dysplasia (associated with schizencephalia in
about 50 % of cases), cortical dysplasia, lesion due to perinatal suffering
(periventricular leukomalacia…).
Brodsky MC. Congenital optic disk anomalies. Surv Ophthalmol 1994, 39(2): 89-112.
Brodsky MC, Glasier CM. Optic nerve hypoplasia. Clinical significance of associated central nervous system
abnormalities on magnetic resonance imaging. Arch Ophthalmol 1993, 111(1): 66-74.
SEPTO OPTIC DYSPLASIA
Girl, 8 month-old, bilateral optic atrophy, hypoglycemia.
Absent septum pellucidum()
Optic nerves hypoplasia ()
Corpus callosum hypoplasia ()
Hypoplastic pituitary stalk ()
Small optic chiasm ()
Ectopic neurohypophysis ()
T2
T1
T2
Ferran Kd Septo-optic dysplasia. Arq Neuropsiquiatr.2010 Jun;68(3):400-5.
OPTIC NERVE APLASIA
Rare congenital anomaly consisting of complete absence of the
optic disc and nerve, ganglion cells and nerve fibers, and retinal
blood vessels.
May be associated or not to abnormal eye ball, unilateral or
more rarely bilateral, the latter being sometimes part of major
central nervous system abnormalities.
Chat L et al Value of MRI in the diagnosis of unilateral optic nerve aplasia: a case report] J Radiol.2002 Dec;83 (12
Pt 1):1853-5.
Caputo R, Sodi A, Menchini U Unilateral optic nerve aplasia associated with rudimental retinal vasculature. Int
Ophthalmol 2009 Dec;29(6):517-9.
Margo CE, Hamed LM, Fang E, et al. Optic nerve aplasia. Arch Ophthalmol 1992, 110(11): 1610-3.
OPTIC NERVE APLASIA
Girl, 8 month-old, poor visual attentiveness and nystagmus
MRI: isolated right optic nerve major hypoplasia ().
3D T2
3D T2
3D T2
3D T2
3D T2
3D T1
PAPILLARY MALFORMATIONS
Coloboma : Congenital ocular colobomas result of a failure in closure
of the embryonal fissure. They are uni or bilateral and occur either
as an isolated finding or as part of a complex malformation
syndrome such as gyration abnormalities (60% of cases), CHARGE
syndrome, Joubert syndrome, Aicardi syndrome.
COLOBOMA
Bilateral
coloboma ()
T2
T2
Left coloboma ()
Enlarged and deep papillary excavation
Courtesy of Dr François Audren
CHARGE SYNDROME
Coloboma ()
Heart disease
choanal Atresia ()
Retardal development
Genital hypoplasia
Ear abnormalities (semi circular canal dysplasia )
Courtesy of Dr Monique Elmaleh-Berges
Denis D et al Ocular coloboma and results of brain MRI: preliminary results. J Fr Ophtalmol.2013 Mar;36(3):210-20.
Chang JH, Park DH, Shin JP, Kim IT Two cases of CHARGE syndrome with multiple congenital anomalies. Int
Ophthalmol.2014 Jun;34(3):623-7.
Brodsky MC. Congenital optic disk anomalies. Surv Ophthalmol 1994, 39(2): 89-112
Brown GC, Shields JA, Goldberg RE. Congenital pits of the optic nerve head. II. Clinical studies in humans.
Ophthalmology 1980, 87(1): 51-65.
Cabrera MTn Laterality of brain and ocular lesions in Aicardi syndrome. Pediatr Neurol. 2011 Sep;45(3):149-54.
Morning glory Syndrome: congenital excavated optic disk , early
diagnosed due to poor visual acuity. 28 % of the cases have retinal
detachment.
Coloboma ()
MORNING GLORY
Heart disease
choanal Atresia ()
Retardal development
Genital hypoplasia
Ear abnormalities (semi circular canal
dysplasia )
Ultra sound
T2
T2
Courtesy of Dr Olivier Berges
Koenig SB, Naidich TP, Lissner G. The morning glory syndrome associated with sphenoidal encephalocele. Ophthalmology
1982, 89(12): 1368-73.
Haik BG, Greenstein SH, Smith ME, et al. Retinal detachment in the morning glory anomaly. Ophthalmology 1984, 91(12):
1638-47.
8 BILATERAL VISUAL LOSS
Once retina or eyeball lesions are ruled out, MRI studies anterior
and posterior visual pathways.
LESION OF BOTH OPTIC NERVES AND/OR CHIASM have numerous
causes
Toxic: tobacco, alcohol, methanol, drugs…
Degenerative disease: Leber’s disease and other hereditary
optic neuropathy,
Infection: tuberculosis, virus, Lyme disease…
Compression, infiltration (hemopathy…)
Intracranial hypertension
Glaucoma
inflammation: ADEM, NMO, granulomatosis
Atrophy of any cause
Vassal F Isolated primary central nervous system lymphoma arising from the optic chiasm.
Neurochirurgie.2014 Dec;60(6):312-5.
LEBER DISEASE
Man 32 year-old, complains of subacute severe painless bilateral loss of vision, major on the left side.
Fundoscopy : bilateral optic disc elevation and hyperemia. MRI: hypersignal T2 and FLAIR of the optic
chiasm and optic tracts (), enhancement of the left part of the chiasm ().
Diagnosis keys: young man, bilateral visual loss, atrophy of the optic nerves, more seldom hypertrophy
and inflammatory aspect of the optic nerves and/or chiasm, blood test (mutation).
T2
T2
T2
T1 Gd
FLAIR
Ong E Teaching neuroimages: chiasmal enlargement and enhancement in Leber hereditary optic neuropathy.
Neurology.2013 Oct 22;81(17):e126-7
Lamirel C papilloedema and MRI enhancement of the prechiasmal optic nerve at the acute stage of Leber hereditary optic
neuropathy. J Neurol Neurosurg Psychiatry.2010 May;81(5):578-80..
BILATERAL LESION OF THE OCCIPITAL CORTEX
They provoque a cortical blindness. It is mainly due to
posterior reversible encephalopathy, ischemia (post vertebral
artery catherism), bilateral occipital tumor, subacute sclerosing
panencephalitis, chemotherapy…
It may be transcient (seizure, migraine)
CORTICAL BLINDNESS
Woman 62 year-old, treated for breast cancer, complains of
vision loss. Examination discloses cortical blindness. MRI
displays bi occipital metastases with necrosis () and hyper
perfusion ()
T2
B1000
T1 Gd
rCBV
LOOKING AND SEEING IS PRECIOUS.
Knowledge
of the clinical presentation of visual pathways
disturbances,
of the difference between anterior and posterior
lesion,
of the protocols needed depending on the clinical
data,
of the different aspects of the pathology
is mandatory to help the diagnosis, orientate the
therapeutic approach and preserve at best our patients
vision.