GRAND ROUNDS : Anterior ischemic optic neuropathy with empty sella

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