Disc oedema

OPHTHALMOLOGY PRESENTATIONS

1. Disc oedema Dr. sidesh Hendavitharana (Registrar-ophthalmology)

2. Means swelling of the optic disc. It includes all causes of active or passive oedematous swelling of the optic disc other than the causes of papillioedema What do you mean by disc edema?

3. Disc hyperemia Blurred disc margins Nerve fiber layer swelling Disc elevation Tortuous vessels You can notice,

4. Causes for pseudodisc edema, Optic nerve head drusen Myelinated nerve fibres Morning glory syndrome Tilted disc Small hypermetropic disc Optic disc dysplasia Bergmeister’s papilla Is it a true edema or not,

5. Composed of hyaline like calcific material within the substance of the optic nerve head. Present in 0.3% of population Are often bilateral Associations include, Retinitis pigmentosa Angioid streak Alagille syndrome Complications-usually rare, include CNV,CRVO,CRAO Imaging, FA-shows progressive hyperfluorescence due to staining but absence of leakage US-most reliable method because of its ability to detect calcific deposits that show high acoustic reflectivity Optic disc drusen

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7. In normal eyes , optic nerve myelination stops at the cribriform plate. In eyes with myelinated nerve fibres the ganglion cells retain a myelin sheath. Most often unilateral. Ocular associations include high myopia,anisometropia and amblyopia. Systemic associations include NF1 and Gorlin syndrome Myelinated nerve fibres

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9. Very rare usually unilateral sporadic condition. Signs, VA-may be normal or impaired to a variable extent. Large disc with funnel shaped excavation surrounded by an annulus of chorioretinal disturbance. Blood vessels emerge from the rim of the excavation in a radial pattern like the spokes of a wheel. Complications, Serous retinal detachment develops in about 30% of cases. CNV is less common and develop adjecent to the lesion Morning glory syndrome

10. Frontonasal dysplasia -mid facial anomalies consisting of hypertelorism,flat nasal bridge and occasionally a mid line notch in the upper lip and a midline cleft in the soft palate. Midline brain malformations such as absent corpus callosum,hypoplastic cerebellar vermis,small optic chiasm,malformed occipital lobe,pituitary deficiency. NF2 less common. PHASE syndrome-posterior fossa brain malformations,large facial haemangiomas and cardiovascular anomalies. Systemic associations,

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12. Is common usually bilateral anomaly caused by an oblique entry of the optic nerve into the globe. Signs, Small oval or D shaped disc Disc margin is indistinct where the retinal nerve fibres are elevated. Associated finding includes inferonasal chorioretinal thining and myopic astigmatic refractive error. Perimetry - superotemporal defects that do not respect vertical midline. Complications- uncommon,include CNV and sensory macular detachment. Tilted disc

13. Unilateral or bilateral Characterized by a diminished number of nerve fibres . Commonly involving the midline structures of the brain. Predisposition, Agents taken by mother during gestation. LSD,quinine,anticonvulsants,excess alcohol Superior segmental hypoplasia may be associated with maternal diabetes. Optic nerve hypoplasia

14. Severe bilateral cases present with blindness in early infancy. Less severe bilateral involvement may cause minor minor visual defects or squint at any time in childhood. Unilateral cases usually present with squint. Signs, VA may be normal or impaired to a variable degree,even to NPL. Double ring sign-small gray disc surrounded by a yellow halo of hypopigmentation caused by concentric chorioretinal atrophy. Distance from the fovea to the temporal border of the optic disc often equals or exceeds 3 times the disc diameter Retinal vessels are normal in caliber but tortous presentation

15. Depends on the severity Includes astigmatism,field defect , dyschromatopsia , APD,foveal hypoplasia,aniridia,microphthalmos,strabismus , nystagmus Systemic associations, Wide variety of midline developmental brain defects-most common De Morsier syndrome( septo -optic dysplasia) Characterized by absence of septum pellucidum agenesis of corpus callosum,hypopituitarism Bilateral optic nerve hypoplasia may be a marker for potential endocrine dysfunction. Other features

16. Inflammatory- papillitis , neuroretinitis , papillophlebitis and uveitis Ocular hypotony -due to any cause Vascular- CRVO,diabetic papillopathy , uremia, AION Orbital causes- tumors,Graves ’ orbitopathy , orbital cellulitis Infiltrative conditions- leukaemias,lymphomas Causes of true disc edema

17. Is hydrostatic non inflammatory edema of optic disc due to raised intracranial pressure. Is almost always bilateral although it may be asymetrical . Etiology- Intracranial causes, congenital- Aqueductal stenosis craniosynostosis papilloedema

18. Brain tumors-commonly tumors arising in posterior fossa- cerebellum,midbrain,parieto - ocipital region which obstruct aqueduct of sylvius . Abscess,tuberculoma,gumma,subdural hematoma,aneurysms,hydrocephalus Intracranial infections- meningitis,encephalitis IC h’age -SAH,SDH Intracranial space occupying lesions

19. Direct blockage of villi by blood and protein Cavernous sinus thrombosis Damaged arachnoid villi Hypersecretion of CSF Tumors of spinal cord Idiopathic intracranial hypertension- pseudotumor cerebri,due to drugs like tetracycline,vit.A,N.acid,contraceptives , corticosteroids Obstruction to CSF absorption by arachnoid villi

20. Systemic conditions Malignant hypertension Pregnancy induced hypertension Cardiopulmonary insufficiency Blood dyscrasias Nephritis Diffuse cerebral edema-due to blunt trauma to head. Metabolic disorders Addison’s diseases,diabetic ketoaccidosis , hypoparathyroidism .

21. Tumors Orbital abscess Orbital cellulitis Aneurysm of ophthalmic artery Pseudotumor Endocrinal exophthalmos Orbital causes

22. Marked ocular hypotony Acutely raised intraocular tension CRVO AION uveitis Ocular causes

23. Pathogenesis( hayreh’s theory )

24. Usually bilateral(in case of raised ICP) but may be unilateral(in case oforbital or ocular causes) General features, Features of raised ICP Headache,worsen on coughing,sneezing or straining Projectile vomiting(without nausea) Diplopia Focal neurological deficit with changes in level of consciousness. Clinical features

25. Early(incipient)papilledema Symptoms Usually absent Normal VA Signs Normal pupillary reactions Fundus picture Obscuration of disc margin-1 st sign Absence of spontaneous venous pulsation at disc Mild hyperemia of disc dilated retinal capillaries splinter h’ages in peripapillary region Visual field-normal Stages of ocular manifestation

26. Symptoms , Amaurosis fugax -recurrent attacks of transient blackout of vision in one or both eyes lasting few seconds,after standing Normal VA -until optic atrophy sets in, Painless progressive gradual loss of vision Signs, Normal pupillary reactions(until optic atrophy sets in) Established(fully developed) papilledema

27. Blurring of disc margins-1 st sign, Markedly hyperemic disc Obliteration of physiological cup Bending of vessels over elevated optic disc Apparent optic disc edema evident by forward elevation up to 1-2mm of disc above the retina. Visual field, Enlargement of blind spot Progressive contraction of visual field Fundus picture

28. Symptoms, Variably reduced VA Signs, Normal pupillary reactions Fundus picture Resolving acute h’age and exudates Resorption of peripapillary edema Disc appears like dome of champagne cork Obliteration of central cup Deposition of small drusen like crystal(corpora amylacea )and opto-ciliary shunts on disc surface Visual fields, Enlarged blind spot Constriction of peripheral visual field. Chronic or long standing papilledema

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30. Papillitis , Unilateral Sudden diminution of vision Marcus G unn pupil Ocular tenderness at insertion of superior and medial rectus Pain on ocular movements Pulfrich phenomenon Hazy media Central or centrocaecal scotoma To differentiate from papillitis ,

31. Hypermetropia , Small porus opticus Blurring of disc margins(due to heaped up optic nerve fibers) Swelling never more than 2D Absence of venous engorgement,h’ages and exudates No enlargement of blindspot No leakage on FFA To differentiate from hypermetropic disc

32. Neurological emergency warranting urgent hospitalization Treatment of underlying cause Surgical decompression of optic nerve before field changes occur. Treatment

33. Thank you