Upload
sumeet-agrawal
View
571
Download
0
Embed Size (px)
DESCRIPTION
An interesting case of sudden unilateral visual loss in an elderly woman with empty sella
Citation preview
GRAND ROUNDSANTERIOR ISCHEMIC OPTIC NEUROPATHY
Presented bySumeet Agrawal
PG IIIUCMS and GTB Hospital
• Patient Profile :
– A 60 y woman
– Resident of Delhi
– Home maker
HISTORY
• Presented to Ophthalmology out patient department on 18th with chief complaint of :
– Diminution of vision Left eye
X 4 days
Sudden, Profound
HISTORY• Good vision previously
• Noticed on waking up in the morning
• Associated with
– Headache over left temple (throbbing type)
– Pain on ocular movements (looking up and sideways)
• No complain of redness/photophobia/discharge
• No complain of color desaturation
• No complain of transient visual obscuration
• No complain of phosphenes
• No complain of variation of vision after exercise/ near a heat source
• No complain of scalp tenderness / jaw claudication/ myalgia/ fever/ weight loss
• Complain of central visual field defect
• No complain of proptosis
• No complain of diplopia
• No complain of limb weakness / facial deviation
• No history of antecedent fever / illness
• No history of other neurological symptoms (ataxia, impaired cognition)
• History of spectacle use for about past 5 years (for distance only)
• Not a known case of Diabetes Mellitus/ Hypertension/ Cardiac disease/ Asthma/ thyroid disease
• Decreased hearing (bilateral, progressive) for the past 10 years
• History of head trauma ( frontoparietal region; at around 10 years age; stitches were applied; not associated with loss of consciousness, seizures or vomiting)
• Complain of chronic headache (throbbing, temporo-parietal) relieved with Ibuprofen tablet for past 8-9 years
• Was diagnosed with clinical depression at GTBH 8-9 years back and advised oral medications but patient did not comply with treatment
• Taking tablet alprazolam 0.125 mg 2-3 times every week for sleep deprivation for past 8-9 years associated with malaise and fatigue during daytime
• Complaint of bilateral knee joint pain for past 3-4 years, increased with activity; no complain of small joint pain/ stiffness/ inflammation
• No history of smoking
• No family history of similar complains
GENERAL PHYSICAL EXAMINATION
• Heavy built (weight : 70 kg; height : 156 cm; BMI : 28.8)
• Conscious, alert, cooperative• Oriented to time, place and person• Pulse: 71/min; regular, good volume, symmetrical• BP (Right arm): 127/71 mm of Hg; (Left arm) : 121/67
mm of Hg• Axillary temperature:98.3 degree Fahrenheit• No pallor/icterus/clubbing/cyanosis/lymphadenopathy• No fine tremors in extremities• No evidence of tortuous, cord like vessels on the temple
SYSTEMIC EXAMINATION
• Cardiovascular system– S1 S2 normal
– Peripheral pulses of good volume and symmetrical
– No carotid bruit
– BP in standing position after lying down (postural hypotension): 134/78 mm Hg (pulse 80/min)
• Central nervous system– Cranial nerves intact
– No signs of cerebellar dysfunction
– No gross motor deficit
OCULAR EXAMINATIONRIGHT EYE LEFT EYE
VISUAL ACUITY
Retinoscopy(at 2/3 m under
tropicamide 1.0 %)
DISTANCE : 6/60Near : n18
PR accurateWith spectacles : 6/12p
+6.5 D
+5.50 D
Acc +4.0 Ds/ +1.0 Dcyl X 180 => 6/9p
+2D add -> n6
Finger counting at 1 mPR accurate
+7.0 D
+6.0 D
Acc +4.5 Ds/ +1.0 Dcyl X 180 => Finger counting at
1 m
Color Vision (Ishiharaplates)
Color plates 11/11 All plates show defect
Head posture Erect
Facial symmetry Maintained
RIGHT EYE LEFT EYE
Orbit Nontender, intact margins; normal periorbital sensations
Mild tenderness of superior orbitalmargin; normal periorbital
sensations
Eyeballs Normal position Normal position
Hertels (108 mm) : 16 mm Hertels (108 mm) : 16 mm
Retropulsion test: negative
Hirschberg reflex Central Central
Alternate Cover test Orthophoric
Extraocularmovements
Full and free Full and free (pain during elevation/ adduction/ abduction)
Eyelids No abnormality detected No abnormality detected
Drainage systemPuncta well apposed
Regurgitation test negativePuncta well apposed
Regurgitation test negative
Conjunctiva No congestion/discharge No congestion/discharge
Sclera No nodules/ ectasia No nodules/ ectasia
CorneaCorneal Sensations
ClearIntact
ClearIntact
Anterior Chamber Normal depth; No cells/flare Normal depth; No cells/flare
Iris Brown; normal pattern Brown; normal pattern
Pupil Round, central, 3 mmLight reflex : D + C +;
Accommodation reflex : Normal
Round, central, 4 mmLight reflex : D +; C + RAPD
(grade 1)Accommodation reflex : Normal
Lens Grade 1 nuclear sclerosisPigments on anterior capsule
Grade 1 nuclear sclerosisPigments on anterior capsule
Vitreous Clear Clear
Fundus(78D)
Disc size : Small [1.3 mm(hz), 1.5 mm (vt)]
Shape & margins normalCup:Disc :: 0.2:1 (vt) 0.2:1 (hz)
Neuroretinal rim :Orange-pink, I>S>N>T
A:V :: 2:3Macula : normal
Foveolar reflex : sharpVenous pulsations present
Blurred disc margins (maximum for temporal and inferior margins)
Elevated disc (< 1 mm)Obscuration of Cup
Neuroretinal rim : hyperemic Peripapillary venous dilatation and
tortuosity without sheathingSurface vessel obscuration
Peripapillary hemorrhage superotemporallyA:V :: 2:3
Macula : normalFoveolar reflex : sharp
Venous pulsations present
No abnormalities in the periphery No abnormalities in the periphery
RIGHT EYE LEFT EYE
INTRAOCULAR PRESSURE(Goldman
Applanation)
11:30 AM 16 mm Hg 18 mm Hg
3:00 PM 14 mm Hg 16 mm Hg
GONIOSCOPY
Scleral Spur
Scleral Spur Scleral Spur
Ciliary body band
Scleral Spur
Scleral Spur Ciliary body band
Ciliary body band
AXIAL LENGTH 21.59 mm 21.54 mm
KERATOMETRY Vertical : 46.25 D ; Horizontal : 46.00 D
Vertical : 46.00 D ; vertical : 45.75 D
OCT (RNFL) Picture in the next slide
• AMSLER GRID
– Not able to appreciate a definite scotoma
• VISUAL FIELD
– High number of fixation losses (both eyes)
PROVISIONAL DIAGNOSISNon Arteritic Anterior ischemic optic neuropathy
• POINTS IN FAVOR
• AGE (mean 60 y)
• INCIDENCE
• UNILATERAL
• NO PRECEDING SYMPTOMS
• HYPERMETROPIA
• RAPD
• INCREASED DISC EDEMA FOCALLY
• HYPEREMIC DISC EDEMA
• OTHER EYE SMALL DISC SIZE WITH SMALL CUP (crowded disc)
• POINTS AGAINST
• PAINFUL OCULAR MOVEMENTS
• NO PREDISPOSING SYSTEMIC FACTORS
• PROFOUND LOSS OF VISION
DIFFERENTIALS
ARTERITIC ANTERIOR ISCHEMIC OPTIC NEUROPATHY
• POINTS IN FAVOR
• UNILATERAL SUDDEN ONSET
• PROFOUND VISUAL LOSS
• TEMPORAL HEADACHE
• POINTS AGAINST
• AGE (mean 70 y)
• INCIDENCE
• NO HISTORY OF PREVIOUS TRANSIENT VISUAL LOSS
• NO SCALP TENDERNESS, JAW CLAUDICATION, MYALGIA, FEVER
• HYPEREMIC DISC EDEMA
• NO NODULAR ENLARGEMENT OF TEMPORAL ARTERY
OPTIC NEURITIS
POINTS IN FAVOR
• PAIN ON OCULAR MOVEMENTS
• UNILATERAL
• DISC EDEMA
POINTS AGAINST
• AGE
• INCIDENCE
• NO PHOSPHENES/ UHTHOFF’S SYMPTOMS
• SUDDEN PROFOUND LOSS OF VISION ON DAY 1(non-progressive)
• NO VENOUS SHEATHING
• NORMAL SURROUNDING RETINA
• POINTS IN FAVOR
• AGE (for metastasis, lymphoma)
• OCULAR / ORBITAL PAIN (rapidly progressive tumor)
• POINTS AGAINST
• AGE (for meningioma, bone tumor)
• SUDDEN ONSET
• NO COMPLAIN OF WEIGHT LOSS (metastasis)
• NO PROPTOSIS
• INTACT PERIORBITAL SENSATIONS
• NEGATIVE RETROPULSTION
• ABSENCE OF OPTOCILIARY SHUNT VESSELS
ORBITAL TUMOR (optic nerve sheath meningioma, sphenoid wing meningioma
, lymphoma, bone tumor, metastasis)
PARANASAL SINUSITIS INVOLVING ORBIT and OPTIC NERVE
POINTS IN FAVOR
• Orbital pain
• Pain on elevation
• Tenderness over superior orbital margin (floor of frontal sinus)
POINTS AGAINST
• Site of headache is not specific for sinusitis
• No proptosis
• No fever
MANAGEMENT
• INVESTIGATIONS
– Blood
– Imaging
– Special investigations
• TREATMENT
– Steroids
• COUNSELLING REGARDING PROGNOSIS
– Visual recovery
– Other eye involvement
INVESTIGATIONS
• ESR : 10 mm in first hour
• Hb : 12.5 g%
• TLC : 16500
• Blood Urea : 24
• Serum Creatinine : 1.3 mg%
• Fasting blood sugar : 77 mg%
• 2 hour post prandial blood sugar : 144
• Lipid Profile :
MRI ORBIT: NORMAL STUDY
MRI BRAIN :
NO EVIDENCE OF PERIVENTRICULAR WHITE
MATTER LESIONS
PATIENT HAD AN EMPTY SELLA DETECTED ON MRI BRAIN
NORMAL SELLA ON MRI BRAIN
FFA SHOWED FILLING DEFECT IN THE
INFERIOR HALF OF THE DISC WITH LATE
LEAKAGE
Non arteriticischemic anterior
optic neuropathy
SLEEP APNEA
?
Unknown systemic factors
CROWDED DISC
(hypermetropiceye)
SECTORAL DISC HYPOPERFUSION
EMPTY SELLA
( intermittent prolapse of optic nerve into empty
sella)
REFERRALS
• NEUROLOGY– No abnormalities detected
• ENT evaluation for hearing loss / paranasalsinusitis – Bilateral sensorineural hearing loss
– No evidence of paranasal sinusitis
• MEDICINE opinion for cardiovascular abnormalities – ECG : normal study
– Carotid doppler : normal study
TREATMENT
• Intravenous dexamethasone 8 mg two times a day given for two days
• Oral prednisolone 50 mg once a day after two days
• Tablet ranitidine 150 mg two times a day
• Tablet ciprofloxacin 500 mg two times a day
COURSE
• Decreased orbital pain
• Decreased pain during extraocularmovements
• Mild improvement of visual acuity
– At presentation : finger counting at 1 m
– After 2 days of iv steroid : finger counting at 2 m
– 6 days after presentation :
Plan of management
• Counselling regarding visual prognosis– Affected eye
– Other eye involvement
• Steroids– No definite recommendations
– Slow tapering
• Monitoring – Visual acuity
– Disc appearance
– VEP
Blood supply to the optic nerve
• Superficial nerve fibrelayer– Epipapillary vessels
• From recurrent arterioles of central retinal artery
• Prelaminar region– Short posterior ciliary
arteries• Circle of Haller and Zinn
• Laminar region– Circle of Haller and Zinn– Peripapillary choroidal
vessels
• Retrolaminar region– Pial system (derived from
short posterior ciliarycirculation)
– Central retinal artery
ISCHEMIC OPTIC NEUROPATHY
• Most commonly at the optic nerve head (crowding)
– ANTERIOR ISCHEMIC OPTIC NEUROPATHY
• ARTERITIC
• NON-ARTERITIC
– POSTERIOR ISCHEMIC OPTIC NEUROPATHY
– DIABETIC PAPILLOPATHY
• Insufficiency of ONH circulation
– Structural crowding
– Watershed zone between medial and lateral short posterior ciliary arteries
• Ischemia -> axonal swelling -> microcirculation compromise -> further swelling
AION
• Most common cause of acute optic neuropathy in population > 50 years
• Non-arteritic is the most common form (90-95%)
– 2.5-10 per 1 lac population
• Arteritic form : 0.36 per 1 lac population
Hattenhauer MG, Leavitt JA, Hodge DO, et al. Incidence of nonarteritic anteriorischemic optic neuropathy. Am J Ophthalmol 1997;123:103–107.
RISK FACTORS
Non-Arteritic AION• SYSTEMIC :
– Hypertension – Nocturnal hypotension– Diabetes mellitus– Sleep apnea– Carotid occlusive disease– Smoking– Migraine– Hyperlipidemia – Vasculitis (small vessel)
• OCULAR :– Small discs with small cups
(hypermetropia)– Optic disc drusens
Arteritic AION• SYSTEMIC :
– Giant cell arteritis
– Polyateritis nodosa
– Churg strass syndrome
– Wegener’s granulomatosis
– Rheumatoid arthritis
ARTERITIC AION NON ARTERITIC AION
MEAN AGE 70 years 60 years
GENDER Female > male No relation
ASSOCIATED SYMPTOMS
Headache, scalp tenderness, jaw claudication
Occasional orbital pain
VISUAL ACUITY <6/60 in 76% >6/60 in 61%
OPTIC DISCAPPEARANCE
Pale more than hyperemic edemaNormal to large cup
Hyperemic more than pale edemaSmall cup
ESR >70 (highly raised) 20-40 (mildly raised)
FFA Choroidal (> 30 – 69 s) and disc filling delay
Disc filling delay
NATURAL HISTORY
Poor prognosis for recoveryFellow eye involved in upto 95%
Upto 3 line improvement in about 43% cases
Fellow eye involved in <30% cases
TREATMENT Urgent administration of corticosteroids
Doubtful role of corticosteroids
• ARTERITIC AION
– SUDDEN ONSET VISUAL LOSS
– Preceding transient visual obscuration, headaches, scalp tenderness, jaw claudication (arteritic form)
– ALTITUDINAL FIELD DEFECT
• Segmental ONH involvement
– RAPD
– Disc edema
– Peripapillary disc hemorrhages
– Telangiectasia
DIAGNOSIS
• Arteritic AION– Clinical– Associated cilioretinal artery
occlusion– ESR and CRP– Delayed choroidal with optic disc
filling on FFA
• Non-arteritic AION– Clinical – Normal choroidal filling time on
FFA
TREATMENT
• Arteritic AION (mainly for the other eye)– Ophthalmic emergency
– High dose iv steroids
– Dramatic response
• Non-Arteritic AION– No proven therapy
– Unproven benefits of corticosteroids
– Optic nerve sheath decompression
– Hyperbaric oxygen
– Levodopa
– Aspirin
OUTCOME
• Usually poor visual prognosis
– Worse for the arteritic form
– Visual acuity
– Field defects
– Other eye involvement
POSTERIOR ISCHEMIC OPTIC NEUROPATHY
• Ischemia of the optic nerve not involving the optic nerve head; subsequent pallor
– Vasculitis (Giant cell arteritis)
– Hypotension and anemia
• Blood loss
• Major surgery
– Retrobulbar neuritis
• Younger age group
• Painful ocular movements
DIABETIC PAPILLOPATHY
• Mild form of AION
• Reversible ischemia (prelaminar and superficial part of optic nerve head)
• Type 1 DM (70 %), < 50 years
– Unilateral disc edema
– Mild optic nerve dysfunction
– Absence of intraocular inflammation/ raised ICT
• Vision 6/12 or better
• Macular edema
• Hyperemic disc edema
• Telangiectasia of surface vessels (mistaken for NVD)
• Diabetic retinopathy usually present but not necessarily
• Crowded fellow disc
• Good prognosis
• Full recovery in 2 to 10 months
EMPTY SELLA
• Primary empty sella
– Congenitally incompetent diaphragma sellae
– Allows herniation of the arachnoid membrane and cerebrospinal fluid into the sella turcica
– Flattening of the pituitary gland,
• Secondary empty sella
– Benign intracranial hypertension
– Pituitary disease
– Surgery, radiation or apoplexy
• Usually free of clinical symptoms (incidental finding)
– Headache
– Visual field defects
– Nontraumatic cerebrospinal fluid rhinorrhea
– pituitary hormone abnormalities
• Assess for
– Hormonal abnormalities (GH deficiency , mild hyperprolactinemia )
– Global hypopituitarism is rare
– Diabetes insipidus
– Visual field defects
• Posterior dislocation of optic nerve and chiasm,
• Optic nerve compression
• Partial prolapse of optic tracts into the sella with optic nerve and optic chiasm strain
• Sagging of the optic nerves and chiasm
• Vascular compromise
• Subsequent infarction
THANK YOU