DISC EDEMA Prof. Vasudev Anand Rao

CAUSES UNILATERAL BILATERAL Papillitis Papilledema Anterior Ischemic optic neuropathy Neuroretinitis Papillophlebitis Ischemic CRVO Anterior compressive optic neuropathies (orbital tumors) Infiltrative optic neuropathies Ocular hypotony Foster-Kennedy syndrome BILATERAL Papilledema Hypertension Diabetic papillopathy Advanced Graves disease Cavernous sinus thrombosis Carotid cavernous fistula Leber hereditary optic neuropathy

PAPILLEDEMA: “optic disc swelling” Conventionally the term refers to hydrostatic non-inflammatory optic disc swelling that results from raised intracranial tension.

ETIOLOGY 1. Intracranial space occupying lesion neoplasm (location of the tumor is more important than size) abscess/inflammatory mass hemorrhage/infarct A-V malformation 2. Obstruction of ventricular system 3. Cerebral edema 4. Impaired CSF absorption by arachnoid villi: Meningitis Raised venous pressure SAH/trauma Communicating hydrocephalus 5. Severe systemic hypertension 6. Idiopathic (pseudo tumor cerebri): 7. Decreased size of cranial vault: Craniosynostosis Thickening of skull 8. Hypersecretion of choroids plexus tumor

CLINICAL FEATURES SYMPTOMS Ocular: Visual acuity-normal in early ,decreased when established and grossly affected when atrophic Amaurosis fugax(spasm of arteries) Central vision affected late(selective loss of peripheral neurons) Diplopia(assoc. 6th cranial nerve palsy in raised ICT) General: Headache (bifrontal/occipital) more in the morning, aggravated by coughing straining Projectile vomiting Loss of consciousness/ focal neurological deficits

CLINICAL FEATURES SIGNS PUPILLARY REACTION -normal until optic atrophy sets in 2. FUNDOSCOPY EARLY PAPILLEDEMA Hyperemia/elevation of disc Blurred margins Loss of SVP Superficial hemorrhage

CLINICAL FEATURES ESTABLISHED/FULLY DEVELOPED PAPILLEDEMA: Engorged & tortuous veins Numerous flame shaped hemorrhages Cotton wool spots, hard exudates Peripapillary edema (paton’s lines) Retinal folds/macular star

CLINICAL FEATURES Optic disc pale & elevated (champagne cork CHRONIC/VINTAGE PAPILLEDEMA: Optic disc pale & elevated (champagne cork appearance) Disc obliterated Opticociliary shunts

CLINICAL FEATURES ATROPHIC PAPILLEDEMA: Pale grey disc with reactive gliosis Narrow and sheathed vessels Retina shows pigmentary changes and choroidal folds

CLINICAL FEATURES 3. FIELD CHANGES Early-normal Established-enlargement of blind spot Chronic-peripheral constriction End stage-total loss 4. FLUORESCEIN ANGIOGRAPHY To differentiate true and pseudopapilledema Dilatation of surface capillaries and leakage of dye in the late phase 5. NEUROIMAGING Features of raised ICT-silver beaten appearance with erosion of posterior clinoid process and dorsum sellae Cause of raised ICT may be identified.

UNILATERAL PAPILLEDEMA Asymmetric Foster Kennedy syndrome Seen in patients with frontal lobe/olfactory lobe tumors, meningiomas of olfactory groove/sphenoidal wing, characterized by optic atrophy on the side of the tumor caused by direct pressure on the nerve and papilledema on the opposite side because of raised ICT. Prior optic atrophy, congenital abnormality in disc, high myopia

PSEUDOTUMOR CEREBRI Or Benign Intracranial hypertension Defined by 4 criteria Increased intracranial pressure Normal or small ventricles No evidence of intracranial mass lesion Normal CSF composition Usually idiopathic seen in young obese women

ETIOLOGY Endocrine causes Addison’s disease Hypoparathyroidism Hyperthyroidism Hypothyroidism Menopause Menarche Pregnancy Drugs Vitamin A Tetracycline Steroids OCP Phenytoin Indomethacin Growth hormone lithium

TREATMENT Weight loss Acetazolamide Lumbar puncture Surgical decompression (ventriculo-peritomeal shunt)

DIFFERENTIAL DIAGNOSIS - Ocular 1. PAPILLITIS Papillitis Papilledema 1.Presentation U/L B/L 2.Vision Sudden loss Unimpaired initially 3.Pupil RAPD present RAPD absent 4.Media Hazy near posterior vitreous Media clear 5.Pain/tendeness of eyeball Present Absent 6.Hemorrhages/exudates Less More(in established) 7.Disc swelling +2 to +3D >+3D 8.Field defects central/centrocaecal scotoma Enlargement of blind spot, later peripheral constriction. 9.X-ray skull Normal Silver beaten appearance, erosion of dorsum sellae,post clinoid 10.CT/MRI Demyleinating ICSOL etc.

DIFFERENTIAL DIAGNOSIS - Ocular 2. PSUEDOPAPILLEDEMA Hypermetropia: Crowded nerve fibers at disc. More in children, no enlargement of blind spot Astigmatism Optic nerve head drusen: Calcium containing refractile bodies within substance of optic nerve head. Seen in USG. Autofluorescence Hazy media

DIFFERENTIAL DIAGNOSIS - Ocular 3. AION/LHON/TOXIC AMBLYOPIAS 4. OCULAR HYPOTONY Effusion from choroidal vessels 5. RAISED INTRAOCULAR PRESSURE: Obliteration of peripapillary vessels by raised IOP 6. CRVO

OPTIC NEURITIS : “Inflammation of the optic nerve” ETIOPATHOGENESIS IDIOPATHIC DEMYELINATING (Always Retrobulbar) Isolated a/w multiple sclerosis neuromyelitis optica schilder’s disease

ETIOPATHOGENESIS INFECTIOUS AND PARAINFECTIOUS LOCAL: Orbital cellulites Sinusitis Teeth, tonsil Meninges, brain or base of skull. SYSTEMIC: VIRAL-measles, mumps, rubella, chickenpox, herpes, CMV and EBV. BACTERIAL-T.B,syphilis,cat scratch disease,lyme’s FUNGAL-cryptococcosis,histoplasmosis PARASITImalaria,pneumocystis,toxoplasma,toxocara,cysticercosis VACCINES:BCG,DPT,TT,HepB,variola and influenza

ETIOPATHOGENESIS 4.IMMUNE RELATED LOCAL Uveitis, sympathetic ophthalmitis. SYSTEMIC sarcoidosis, Wegener’s polyarteritis nodosa, SLE etc. 5.METABOLIC Anemia Diabetes Starvation 6.DRUGS AND TOXINS INH, ethambutol, etanercept, INFa, tobacco, alcohol, quinine.

CINICAL FEATURES Commonly unilateral, more in females and mean age is 30-35 yrs. SYMPTOMS Triad of Loss of central vision Eye pain Decreased colour vision Other Altered perception of moving objects Worsening of symptoms with elevation of body temperature(uhthoff sign)

CINICAL FEATURES SIGNS Decreased visual acuity Tenderness Marcus gunn pupil (RAPD) Decreased colour vision and contrast sensitivity Visual field defects: classically central/centrocaecal scotoma but other defects can also occur Fundus changes 1. Papillitis: edema, hyperemia, blurred margins, dilated tortuous vs, few exudates and vitreous haze 2. Retrobulbar neuritis: normal 3. Neuroretinitis: macular star with exudates VEP-Delayed latency and decreased amplitude FAG to differentiate from other causes-dilated and telangiectatic vs with leak from capillaries

CINICAL FEATURES Field defects in optic neuritis Papillitis Neuroretinitis

INVESTIGATIONS To determine cause for optic neuritis 1.Complete Hemogram 2. CRP, ESR, Mantoux 3. VDRL 4. Serology-ANA, Toxoplasma, Lymes 5. PNS X-ray, chest x ray(sarcoidosis) 6. X ray skull, CT 7. MRI(demyleinating plaques-2 or more predictive of deveplopment of MS) 8. Lumbar puncture-CSF pleocytosis and oligoclonal bands

MRI scan showing demyelinating optic neuritis

TREATMENT 1. ONTT Regimen - Intravenous methylprednisolone 250mg q 6 h for 3 days followed by Oral prednisolone 1 mg/kg/day for 11 days, tapered with 20mg on 15th day and 10mg on 16th and 18th day 2. Posterior sub-tenon injection of triamcinolone 3. Vitamin B12 4. Treatment of identifiable cause

Ischemic optic neuropathy Infarction of prelaminar or laminar portions of optic nerve caused by occlusion of posterior ciliary artery. Seen in >50 yrs. H/s/o giant cell arteritis or predisposing factors like DM/HT Pale swollen disc with splinter hemorrhages Altitudinal scotoma Classified as Arteritic & Non-Arteritic

Clinical Features Features Arteritic AION Non Arteritic AION Age >60yrs 40-60yrs Sex Ratio F>M F=M Vision loss Severe Moderate (>6/60) Laterality Fellow eye affected in 95% within days to wks Fellow eye affected in <30% in months or yrs Optic disc Pale edema, may be sectoral Hyperemic or pale edema Assoc. Signs Scalp tenderness, palpable tender, non-pulsatile temporal artery Assoc. HT – 40%, DM – 24% Shock, nocturnal hypotension ESR >40 mm in 1st hr 20-40mm in 1st hr FAG Disc and choroidal filling delay Disc filling delay Treatment IV methylprednisolone ? Levadopa-carbidopa Prognosis Poor Improvement in upto 43%

THANK YOU