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Ophthalmological Evaluation in
Sellar Suprasellar Tumors
Dr Fakir Mohan Sahu
MCh Senior Resident
AIIMS Bhubaneswar
18/07/2020
Learning Objectives
• Sella, Suprasellar and Chiasmal anatomy
• Visual Pathway and Organisation of Nerve fibres
• Tumours of sellar suprasellar region
• Basic and Morden Neuro-ophthalmological examination
• Approach to different sellar and suprasellar tumor
• Ophthalmological outcome following surgery
Anatomy of sellar and parasellar Region
SELLA
Relationships of the optic nerves and
optic chiasm to the sellar structures
Third ventricle (III).
C-anterior clinoid
D-dorsum sellae.
Pituitary gland
Pear shaped gland
Attached with infundibulum
Ant and post lobe
The diaphragma is thinnest centrally
around the infundibulum and increases
peripherally
The thickness and the degree of incompetence of the diaphragma sella may
determine the ease and rapidity of suprasellar growth of pituitary tumors
Chiasmal Position
Variation in relative position contributes to
clinical variation in the presentation of neuro-
ophthalmological chiasmal syndromes
Normal Visual Pathway
Organisation of Retinal Nerve Fibre layer(RNFL)
Optic Nerve Optic TractOptic Chiasma
• Optic chiasm decussation
Inferior nasal fibers - anteroinferior
Superior nasal fibers - posterosuperior
Lesions and VF defect
Common Sellar Suprasellar tumour
• Pituitary tumours / Apoplexy
• Craniopharyngioma
• Meningioma (Tuberculum sella, Diaphragm sella, Sphenoid wing, Palanum sphenoidale)
• Chiasmatic Glioma / Optic nerve glioma
• Dermoid/Epidermoid
• Germinoma
• Schwannoma
• Chondromas
• Metastasis
• Rathke’s cleft cyst
• Empty Sella Turcica
• 3rd Ventricular tumour extension
Effect of Tumours on vision
Direct effects:
Direct compression.
• Optic chiasm (Normal variant)
• Optic nerve post-fixed chiasm
• Optic tracts  pre-fixed chiasm
• 3rd , 4th, 6th nerves with CS extension
• Third ventricle leading to hydrocephalus
Indirect effects:
• Papilloedema
• Impact of tumour resection
• Post-radiation neuropathy
• Toxic effects due to chemotherapy
• Metastatic lesions to retina or infiltration of optic nerve
• Haematological effects like ischemia, venous engorgement
INVESTIGATION PROTOCOL
• History and physical examination
• Neuro-ophthalmology:
 Visual acuity
Colour vision
 Ophthalmoscopy
 Visual fields
 Pupils
 Extraocular movements
• Neuro-endocrine: Hormone levels- basal hormone and dynamic testing
• Radiology (a) X-Rays
(b) MRI
(c) NCCT/CECT
(d)PET/DSA
• Routine blood investigation
Neuro-ophthalmics of Sellar Suprasellar tumour
Colour Vision
Loss of colour vision precedes other visual deficits
Red perception is lost first described as red desaturation
Ishihara/hardy ritter rand charts used
Neuro-ophthalmics of Sellar Suprasellar tumour
Pituitary adenoma  Primary optic atrophy
primary secondary
Colour of disc white grey
Border of disc Sharp Blurred
Arteries and veins Normal or reduced Arteries thin, veins dilated
Distribution May affect one sector Entire disc affected
Causes Optic nerve/retinal damage Papillitis/papilledema
Lamina cribrosa visible Not visible
Normal Field of vision
• 3D area when fixating at a central target.
Visual fields
90 -100 deg temporally
60 deg nasally
50-60 deg superiorly
60-75 deg inferiorly
With binocular vision , VF of both eyes overlap (60 degrees )
Goldman’s Perimeter
Visual fields are analyzed by
 Confrontation method
 Goldman's perimeter
 Humphrey’s Automated perimeter
Anterior optic chiasmal syndrome
Automated static perimetry
 From Damage to the Anterior Angle of the Optic
Chiasm
 Post –fixed chiasm
 Tumour compressing where optic nerve and the
chiasm
 “Junctional scotoma” C/L eye and I/L temporal
hemianopia
 Asymptomatic scotoma in the superior temporal field of
the opposite eye (Wilbrand’s knee)
Central chiasm syndrome
 Bitemporal defect (quadrantic or hemianopic)
(Infiltrating tumors, such as gliomas, germ cell tumors, and
lymphomas,
 In most cases, visual acuity is normal.
 Suprasellar, infrachiasmatic lesion
(Pituitaryadenoma)
 Compresses the chiasm from below
 Defects are typically superior
 Bitemporal field defects that are denser below
 Suprasellar, suprachiasmatic lesions
(Meningiomas, Craniopharyngiomas)
Bitemporal hemianopia
frequently beginning in the inferior quadrants
 Papilledema suprasellar, supra-chiasmal lesions more
infra-chiasmal lesions
Optic Chiasmal Syndrome
Disturbance of depth perception
 Completely blind triangular area of field with its apex
at fixation  Convergence of the two blind temporal
hemifields
 The image of an object posterior to fixation falls on
blind nasal retinas and thus disappears.
 Difficulty with near tasks
(threading needles, sewing)
Retro-chiasmal syndrome
Posterior lesion  optic tract
Incongruous Homonymous Hemianopia
Posterior lesionCompressing macular fibers
Bitemporal central scotoma
Visual Field Defects Caused by Lesion extension
• Optic nerve or the optic tract optic chiasm: the blind eye usually is on the side of the lesion.
Right optic nerve lesion: blind RE defect temporal field LE
Right optic tract lesion : Lt. Homonymous hemianopia  blindness or near-blindness RE
• Optic chiasm optic nerve or the optic tract:
Blind (or near-blind) eye is always on the side of the extension of the lesion.
Optic chiasm- Bitemporal hemianopia Rt optic nerve leads to RE blind
Rt optic tract, blindness or near-blindness RE.
Radiology
• X- Rays:Lateral & PA Skull + Lateral Sella turcica
Alignment of posterior clinoid processes
Widening of sella
Destruction of sellar floor
Relation of median sphenoidal septum
Aeration of sphenoid sinus
Imaging
Pituitary Macroadenoma
Modified Hardy Wilson Classification
Type A: Tumor bulges into the
chiasmatic cistern
Type B: Tumor reaches the floor of the
3rd ventricle
Type C: Tumor is more voluminous
with extension into the 3rd ventricle up
to the foramen of Monro
Type D: Tumor extends into temporal
or frontal fossa
TYPE E : Extradural spread (
extension into or out of the cavenous
sinus)
Grade 0 : Intrapituitary microadenoma with normal sellar floor
Grade I : Normal-sized sella with asymmetric floor
Grade II : Enlarged sella with an intact floor
Grade III : Localized erosion of sellar floor
Grade IV : Diffuse destruction of floor
Microadenomas – Grades 0 and I
Macroadenomas – Grades II to IV
Size and invasiveness
Craniopharyngioma
Meningioma
Tuberculum sella / Sphenoid wing
Foster Kennedy syndrome
 Optic Atrophy I/L eye
Papilledema in C/L eye
Central scotoma in I/L eye
Anosmia
Pituitary Apoplexy
Pituitary Microadenoma
Cushing’s disease
• Microadenoma/normal
• No compression effect
• Raised intraocular pressure and
Exophthalmos
(seen in up to 1/3rd patients in Cushing's original series)
• Cataracts complication of long term
corticosteroid therapy.
• Central serous chorioretinopathy
Dynamic scan showing delayed contrast uptake by adenoma
Cavernous sinus compression
• CN III, CN IV and CN VI paresis together with neuralgic pain
disturbances over the region (V1 ophthalmic)
• Mild exophthalmos, conjunctival and lid edema.
(Impaired orbital outflow)
• Lesion of the ICA sympathetic: Horner’s syndrome
(ptosis, miosis, and enophthalmos)
 Three lines are drawn between the supraclinoid ICA and
intracavernous ICA on coronal MRI imaging.
Medial tangent
Intercarotid line
Lateral tangent
 These lines are used to define 4 grades of tumor invasion:
Grade 0 = medial to medial tangent
Grade 1 = space between the medial tangent and the intercarotid line
Grade 2 = space between the intercarotid line and the lateral tangent
Grade 3 = tumor extends lateral to the lateral tangent
3A = superior cavernous sinus compartment
3B = inferior cavernous sinus compartment
Grade 4 = complete encasement of intracavernous ICA
Morden Neuro-ophthalmology
• Optic Coherence Tomography (OCT)
RNFL & GCL
Peripapillary RNFL thinning precedes visual field defects.
Detecting subclinical neuro-ophthalmological signs of
Chiasmal syndrome
• Visual Evoked Potentials
Evoked electro physiological potential extracted using signal from EEG activity recorded at the scalp.
Provides diagnostic information regarding the functional integrity of visual system.
Measures the time taken for visual stimuli to travel from eye to occipital cortex.
Particularly useful in infants
Surgical approach
Transcranial approach
• Intradural
Pre-chiasmal: Bifrontal, Pterional, Subfrontal
Retro-chiasmal: Interhemispheric
• Extradural- Orbito-zygomatic
Extracranial approach
• Endoscopic Trans-sphenoidal
• Endoscopic Trans nasal
Endoscopic Trans-ventricular approach
Lamina terminalis corridor
Conclusion
• Neuro-ophthalmological evaluation:
Aids in diagnosis of underlying tumour
 Allow predicting postoperative visual function
Medical clinical history and Neuro-endocrinological exam.
Early treatment leads to better ophthalmological outcome
Protects the patients
and the surgeon…………..
Thank You

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Ophthalmological evalution in sellar suprasellar tumours

  • 1. Ophthalmological Evaluation in Sellar Suprasellar Tumors Dr Fakir Mohan Sahu MCh Senior Resident AIIMS Bhubaneswar 18/07/2020
  • 2. Learning Objectives • Sella, Suprasellar and Chiasmal anatomy • Visual Pathway and Organisation of Nerve fibres • Tumours of sellar suprasellar region • Basic and Morden Neuro-ophthalmological examination • Approach to different sellar and suprasellar tumor • Ophthalmological outcome following surgery
  • 3. Anatomy of sellar and parasellar Region SELLA
  • 4.
  • 5.
  • 6.
  • 7. Relationships of the optic nerves and optic chiasm to the sellar structures Third ventricle (III). C-anterior clinoid D-dorsum sellae. Pituitary gland Pear shaped gland Attached with infundibulum Ant and post lobe The diaphragma is thinnest centrally around the infundibulum and increases peripherally The thickness and the degree of incompetence of the diaphragma sella may determine the ease and rapidity of suprasellar growth of pituitary tumors
  • 8. Chiasmal Position Variation in relative position contributes to clinical variation in the presentation of neuro- ophthalmological chiasmal syndromes
  • 10. Organisation of Retinal Nerve Fibre layer(RNFL) Optic Nerve Optic TractOptic Chiasma • Optic chiasm decussation Inferior nasal fibers - anteroinferior Superior nasal fibers - posterosuperior
  • 11. Lesions and VF defect
  • 12. Common Sellar Suprasellar tumour • Pituitary tumours / Apoplexy • Craniopharyngioma • Meningioma (Tuberculum sella, Diaphragm sella, Sphenoid wing, Palanum sphenoidale) • Chiasmatic Glioma / Optic nerve glioma • Dermoid/Epidermoid • Germinoma • Schwannoma • Chondromas • Metastasis • Rathke’s cleft cyst • Empty Sella Turcica • 3rd Ventricular tumour extension
  • 13. Effect of Tumours on vision Direct effects: Direct compression. • Optic chiasm (Normal variant) • Optic nerve post-fixed chiasm • Optic tracts  pre-fixed chiasm • 3rd , 4th, 6th nerves with CS extension • Third ventricle leading to hydrocephalus Indirect effects: • Papilloedema • Impact of tumour resection • Post-radiation neuropathy • Toxic effects due to chemotherapy • Metastatic lesions to retina or infiltration of optic nerve • Haematological effects like ischemia, venous engorgement
  • 14. INVESTIGATION PROTOCOL • History and physical examination • Neuro-ophthalmology:  Visual acuity Colour vision  Ophthalmoscopy  Visual fields  Pupils  Extraocular movements • Neuro-endocrine: Hormone levels- basal hormone and dynamic testing • Radiology (a) X-Rays (b) MRI (c) NCCT/CECT (d)PET/DSA • Routine blood investigation
  • 15. Neuro-ophthalmics of Sellar Suprasellar tumour Colour Vision Loss of colour vision precedes other visual deficits Red perception is lost first described as red desaturation Ishihara/hardy ritter rand charts used
  • 16. Neuro-ophthalmics of Sellar Suprasellar tumour Pituitary adenoma  Primary optic atrophy primary secondary Colour of disc white grey Border of disc Sharp Blurred Arteries and veins Normal or reduced Arteries thin, veins dilated Distribution May affect one sector Entire disc affected Causes Optic nerve/retinal damage Papillitis/papilledema Lamina cribrosa visible Not visible
  • 17. Normal Field of vision • 3D area when fixating at a central target. Visual fields 90 -100 deg temporally 60 deg nasally 50-60 deg superiorly 60-75 deg inferiorly With binocular vision , VF of both eyes overlap (60 degrees )
  • 18. Goldman’s Perimeter Visual fields are analyzed by  Confrontation method  Goldman's perimeter  Humphrey’s Automated perimeter
  • 19.
  • 20. Anterior optic chiasmal syndrome Automated static perimetry  From Damage to the Anterior Angle of the Optic Chiasm  Post –fixed chiasm  Tumour compressing where optic nerve and the chiasm  “Junctional scotoma” C/L eye and I/L temporal hemianopia  Asymptomatic scotoma in the superior temporal field of the opposite eye (Wilbrand’s knee)
  • 21. Central chiasm syndrome  Bitemporal defect (quadrantic or hemianopic) (Infiltrating tumors, such as gliomas, germ cell tumors, and lymphomas,  In most cases, visual acuity is normal.  Suprasellar, infrachiasmatic lesion (Pituitaryadenoma)  Compresses the chiasm from below  Defects are typically superior  Bitemporal field defects that are denser below  Suprasellar, suprachiasmatic lesions (Meningiomas, Craniopharyngiomas) Bitemporal hemianopia frequently beginning in the inferior quadrants  Papilledema suprasellar, supra-chiasmal lesions more infra-chiasmal lesions
  • 22. Optic Chiasmal Syndrome Disturbance of depth perception  Completely blind triangular area of field with its apex at fixation  Convergence of the two blind temporal hemifields  The image of an object posterior to fixation falls on blind nasal retinas and thus disappears.  Difficulty with near tasks (threading needles, sewing)
  • 23. Retro-chiasmal syndrome Posterior lesion  optic tract Incongruous Homonymous Hemianopia Posterior lesionCompressing macular fibers Bitemporal central scotoma
  • 24. Visual Field Defects Caused by Lesion extension • Optic nerve or the optic tract optic chiasm: the blind eye usually is on the side of the lesion. Right optic nerve lesion: blind RE defect temporal field LE Right optic tract lesion : Lt. Homonymous hemianopia  blindness or near-blindness RE • Optic chiasm optic nerve or the optic tract: Blind (or near-blind) eye is always on the side of the extension of the lesion. Optic chiasm- Bitemporal hemianopia Rt optic nerve leads to RE blind Rt optic tract, blindness or near-blindness RE.
  • 25. Radiology • X- Rays:Lateral & PA Skull + Lateral Sella turcica Alignment of posterior clinoid processes Widening of sella Destruction of sellar floor Relation of median sphenoidal septum Aeration of sphenoid sinus
  • 27.
  • 29. Modified Hardy Wilson Classification Type A: Tumor bulges into the chiasmatic cistern Type B: Tumor reaches the floor of the 3rd ventricle Type C: Tumor is more voluminous with extension into the 3rd ventricle up to the foramen of Monro Type D: Tumor extends into temporal or frontal fossa TYPE E : Extradural spread ( extension into or out of the cavenous sinus) Grade 0 : Intrapituitary microadenoma with normal sellar floor Grade I : Normal-sized sella with asymmetric floor Grade II : Enlarged sella with an intact floor Grade III : Localized erosion of sellar floor Grade IV : Diffuse destruction of floor Microadenomas – Grades 0 and I Macroadenomas – Grades II to IV Size and invasiveness
  • 31. Meningioma Tuberculum sella / Sphenoid wing Foster Kennedy syndrome  Optic Atrophy I/L eye Papilledema in C/L eye Central scotoma in I/L eye Anosmia
  • 32.
  • 35. Cushing’s disease • Microadenoma/normal • No compression effect • Raised intraocular pressure and Exophthalmos (seen in up to 1/3rd patients in Cushing's original series) • Cataracts complication of long term corticosteroid therapy. • Central serous chorioretinopathy Dynamic scan showing delayed contrast uptake by adenoma
  • 36. Cavernous sinus compression • CN III, CN IV and CN VI paresis together with neuralgic pain disturbances over the region (V1 ophthalmic) • Mild exophthalmos, conjunctival and lid edema. (Impaired orbital outflow) • Lesion of the ICA sympathetic: Horner’s syndrome (ptosis, miosis, and enophthalmos)
  • 37.  Three lines are drawn between the supraclinoid ICA and intracavernous ICA on coronal MRI imaging. Medial tangent Intercarotid line Lateral tangent  These lines are used to define 4 grades of tumor invasion: Grade 0 = medial to medial tangent Grade 1 = space between the medial tangent and the intercarotid line Grade 2 = space between the intercarotid line and the lateral tangent Grade 3 = tumor extends lateral to the lateral tangent 3A = superior cavernous sinus compartment 3B = inferior cavernous sinus compartment Grade 4 = complete encasement of intracavernous ICA
  • 38. Morden Neuro-ophthalmology • Optic Coherence Tomography (OCT) RNFL & GCL Peripapillary RNFL thinning precedes visual field defects. Detecting subclinical neuro-ophthalmological signs of Chiasmal syndrome • Visual Evoked Potentials Evoked electro physiological potential extracted using signal from EEG activity recorded at the scalp. Provides diagnostic information regarding the functional integrity of visual system. Measures the time taken for visual stimuli to travel from eye to occipital cortex. Particularly useful in infants
  • 39. Surgical approach Transcranial approach • Intradural Pre-chiasmal: Bifrontal, Pterional, Subfrontal Retro-chiasmal: Interhemispheric • Extradural- Orbito-zygomatic Extracranial approach • Endoscopic Trans-sphenoidal • Endoscopic Trans nasal Endoscopic Trans-ventricular approach Lamina terminalis corridor
  • 40.
  • 41. Conclusion • Neuro-ophthalmological evaluation: Aids in diagnosis of underlying tumour  Allow predicting postoperative visual function Medical clinical history and Neuro-endocrinological exam. Early treatment leads to better ophthalmological outcome Protects the patients and the surgeon…………..