5. papilloedema

ophthalmology course

1. Papilloedema Dr / Reyad Abo Yossif ( MRCOphth ) Ophthalmology specialist Shebin Elkom ophthalmology hospital

2. Definitions - Optic disc oedema . Unilateral optic disc swelling. Bilateral optic disc swelling. Papilloedema . Pseudo- Papilloedema .

3. Optic disc oedema Optic disc edema refers to the ophthalmoscopic swelling of the optic disc with a concurrent increase in fluid within or surrounding the axons. 

4. Unilateral optic disc swelling Unilateral optic disc swelling with the most likely etiological categories are: - Non Arteritic Ischemic Optic Neuropathy - Arteritic Ischemic Optic Neuropathy - Inflammatory optic neuritis - Compressive optic nerve tumor - Central retinal vein occlusion

5. Unilateral optic disc swelling Optic nerve functions are affected: Visual Acuity diminution Pupillary affection ( + ve RAPD) Color vision affection

6. Bilateral optic disc swelling Bilateral optic disc swelling with the most likely etiological categories are: - Increased intracranial pressure (ICP) ( Papilloedema ) - Infectious/inflammatory - Demyelinating - Toxic-metabolic/nutritional, and Hypertensive emergency ( ie , malignant HTN). 

7. Bilateral optic disc swelling Optic nerve functions are normal - Normal Visual Acuity - Normal Pupillary response No Color vision problem (except late if optic atrophy occurred)

8. Papilloedema Bilateral optic disc swelling due to increased ICP can be caused by many disease processes including : - intracranial space occupying lesions , obstructions of the arachnoid villi, , CSF producing tumors and idiopathic intracranial hypertension (IIH).

9. Pseudo- Papilloedema Pseudo-papilledema is defined as anomalous elevation of one or both optic discs without edema of the retinal nerve fiber layer.

10. It is important to distinguish pseudo-papilledema from true papilledema, which can be the first sign of disease process with the potential for vision loss, neurological impairment, or death. Patients thought to have papilledema are often subjected to lumbar puncture, MRI, and extensive laboratory studies to find the underlying cause.

11. Papilloedema

12. Aetiology Papilledema is due to increased intracranial pressure from any cause. Normal ICP is typically less than 25 cm water when measured with a manometer in the recumbinat position. Papilledema results from orthograde axoplasmic flow stasis at the optic nerve head leading to edema of the nerve from the increased intracranial pressure pressing on the nerve behind the eye.

13. Increased intracranial pressure can be caused by : (1) a space occupying lesion such as a tumor or hemorrhage, (2) decreased CSF drainage due to: blockage (obstructive hydrocephalus) or decreased absorption (infection, hemmorhage , venous sinus thrombosis or venous outflow obstruction), (3) Idiopathic Intracranial Hypertenion ( IIH , also called pseudtumor cerebri ) , (4) increased CSF production such as from a choroid plexus tumor (rare), (5) decreased skull volume ( craniosynostosis ).  

14. Continued pressure can result in loss of axons and eventual optic atrophy secondary to intraneuronal ischemia ultimately causing vision loss. With optic atrophy there is little or no edema seen even with continuing increased intracranial pressure since there are no fibers to swell.

15. History Headache is typical and is often worse on waking or postural (worse on lying down and better on standing up). It is useful to ask about medicines that may cause increased intracranial pressure such as tetracyclines , vitamin A and derivatives, lithium, steroids - especially changes in the amount of steroids taken and withdrawal of steroids.

16. Physical examination In addition to the usual ophthalmic exam including visual fields , one should check blood pressure to rule out hypertensive emergency (i.e., “ malignant hypertension ”) which can mimic papilledema. Ocular motility exam may reveal a unilateral or bilateral 6th nerve palsy as a non-localizing signs of increased intracranial pressure.

17. Symptoms and signs of increased ICP Headache is common with increased intracranial pressure and often worse in the morning. Nausea and vomiting are noted with increased intracranial pressure, especially with an acute rise in pressure. This is made worse by straining, coughing or bending. Pulse synchronous tinnitus may be present from increased flow in stenotic cerebral venous sinuses.

18. Symptoms and signs of increased ICP Visual field loss (usually an increased size of the blind spot initially) can occur acutely (i.e., fulminant) or be slowly progressive. The patient may note short periods lasting seconds of losing vision which are called transient visual obscurations. These are often observed when raising to stand or changing position. A hyperopic shift may occur in the refraction due to axial length shortening.

19. Symptoms and signs of increased ICP Long standing papilledema may result in severe peripheral visual field loss and may progress to involve central vision if untreated. Increased intracranial pressure can also lead to cranial nerve palsies, usually an abducens palsy as a non-localizing finding. It is thought that the long course of the 6th cranial nerve is compressed by the increased CSF pressure.

20. Clinical diagnosis Papilledema Grading System ( Frisen Scale)

21. Papilledema Grading System ( Frisen Scale) in detail

22. Papilledema Grading System ( Frisen Scale) Stage 0 - Normal Optic Disc Blurring of nasal, superior and inferior poles in inverse proportion to disc diameter. Radial nerve fiber layer (NFL) without NFL tortuosity. Rare obscuration of a major blood vessel, usually on the upper pole.

23. Papilledema Grading System ( Frisen Scale) Stage 1 -Very Early Papilledema Obscuration of the nasal border of the disc. No elevation of the disc borders. Disruption of the normal radial NFL arrangement with grayish opacity accentuating nerve fiber layer bundles. Normal temporal disc margin. Subtle grayish halo with temporal gap (best seen with indirect ophthalmoscopy). Concentric or radial retrochoroidal folds.

24. Papilledema Grading System ( Frisen Scale) Stage 2 - Early Papilledema Obscuration of all borders. Elevation of the nasal border. Complete peripapillary halo.

25. Papilledema Grading System ( Frisen Scale) Stage 3 – Moderate Papilledema -Obscurations of all borders. -Increased diameter of optic nerve head. -Obscuration of one or more segments of major blood vessels leaving the disc. - Peripapillary halo-irregular outer fringe with finger-like extensions.

26. Papilledema Grading System ( Frisen Scale) Stage 4 - Marked Papilledema -Elevation of the entire nerve head. -Obscuration of all borders. - Peripapillary halo. -Total obscuration on the disc of a segment of a major blood vessel.

27. Papilledema Grading System ( Frisen Scale) Stage 5 - Severe Papilledema Dome-shaped protrusions representing anterior expansion of the optic nerve head. Peripapillary halo is narrow and smoothly demarcated. Total obscuration of a segment of a major blood vessel may or may not be present. Obliteration of the optic cup.

28. Papilledema Grading System ( Frisen Scale) in breif

29. Grade I papilledema is characterized by a C-shaped halo with a temporal gap

30. Grade II papilledema, the halo becomes circumferential

31. Grade III papilledema is characterized by loss of major vessels AS THEY LEAVE the disc (arrow)

32. Grade IV papilledema is characterized by loss of major vessels ON THE DISC.

33. Grade V papilledema has the criteria of grade IV plus partial or total obscuration of all vessels of the disc.

34. Diagnostic procedures -CNS imaging study (CT or MRI with contrast) to identify a central nervous system mass lesion. - MR venography(MRV) or CTV should also be considered to look for venous sinus thrombosis.

35. Diagnostic procedures Lumbar puncture (to be done after imaging demonstrates no risk for herniation) to document increased ICP and to look for neoplastic, infectious or inflammatory causes. At a minimum, opening pressure along with glucose, protein, cell count and differential, culture are obtained..

36. Diagnostic procedures Fluorescein angiography can detect disc leakage in true optic disc edema as opposed to disc staining without leakage in pseudopapilledema .

37. disc leakage in true optic disc edema

38. disc staining without leakage in pseudopapilledema

39. Diagnostic procedures Autofluorescence may  show pseudopapilledema (e.g., optic disc drusen ).

40. Diagnostic procedures B scan ultrasound may show pseudopapilledema (e.g., optic disc drusen ).

41. Dilated Optic Nerve Sheath with Papilledema with disc elevation , no calcification

42. Diagnostic procedures Successive fundus images may be helpful to follow the disc status over time .

43. Diagnostic procedures Automated perimetry commonly shows enlarged blind spots and nerve fiber layer type visual field defects. In severe chronic papilledema worsening peripheral field loss may be seen and can eventually involve central vision loss .

44. Management of bilateral optic disc swelling 1- Complete routine history taking and examination 2- Answer these questions: - Is it a unilateral or bilateral disc swelling ? What about optic nerve functions (VA , RAPD ,color) ? Differential diagnosis of bilateral disc oedema ? Blood pressure measurement to rule out malignant HTN (search for hypertensive changes during fundus examination) MRI and MRV with contrast to rule out space occupying lesions or venous sinus thrombosis Lumbar puncture to rule out IIH (Idiopathic Intracranial Hypertension)

45. Management of bilateral optic disc swelling Fundus photo for documentation and follow up Automated perimetry for follow up Think about other causes of bilateral disc oedema other than increased intracranial pressure as sarcoidosis , toxoplasmosis , VKH ,posterior scleritis , posterior uveitis causes Don’t forget to exclude pseudo- papilloema (optic disc drusen , crowded disc in hypermetropia , tilted disc in high myopia , optic disc hypoplasia)

46. Notes on workup When evaluating a patient for IIH, a complete ocular examination including a dilated fundus examination, visual field examination, and optic nerve photographs is required. Subsequently, neuroimaging is needed to exclude secondary causes of intracranial hypertension. Magnetic resonance imaging (MRI ) and MR venography (MRV) of the brain are usually the imaging modalities of choice.

47. Notes on workup Furthermore, a lumbar puncture is recommended for all patients who are suspected of having IIH. The diagnosis is based upon an elevated opening pressure greater than 25 cm H2O taken with the patient lying in the lateral decubitus position. Values (20 - 25 )cm H2O are considered equivocal. In obese or sedated children, pressures up to 28 cm H2O may be considered normal.

48. Notes on workup The CSF must also be studied to rule-out inflammation, tumor cells, and infection. Patients with IIH usually have normal or low protein level, normal glucose levels and a normal cell count

49. Notes on workup The primary cause of the increased intracranial pressure must be addressed. If a mass is present, primary therapy should be directed towards that lesion (e.g., surgery). If medications ( tetracyclines , vitamin A analogues, etc.) are felt to be causative they should be discontinued.

50. Notes on workup Patients with mild papilledema should be monitored for visual field defects by formal perimetry as the patients may not be able to recognize them.

51. Prognosis of papilloedema Chronic papilledema leads to axon loss with constriction of the visual field, loss of disc substance and in the end, loss of central acuity. Patients (especially those with IIH) need to be followed to prevent vision loss.

52. Pseudotumor cerebri (Idiopathic Intracranial Hypertension ) (IIH)

53. Pseudotumor cerebri (idiopathic intracranial hypertension) (IIH) It is a disorder characterized by increased intracranial pressure (ICP) of unknown cause that predominantly affects obese women of child-bearing age. Papilledema is the primary ocular finding and may progressively lead to optic atrophy and blindness if no treatment is provided.

54. Pseudotumor cerebri (idiopathic intracranial hypertension) IIH may be seen in any gender or age group but has a high predilection for females of childbearing age, especially when coupled with obesity. While males are less frequently affected, constituting less than 10% of adult IIH patients, the affected population also tend to be obese and are more likely to sustain worse visual prognosis compared to their female counterparts.

55. Pseudotumor cerebri (idiopathic intracranial hypertension) Interestingly , in the prepubertal age group, IIH has no particular predilection for obesity or female gender. Associated conditions include migraines and Down syndrome.

56. Pseudotumor cerebri (idiopathic intracranial hypertension) Certain systemic illnesses have been associated with IIH, including obstructive sleep apnea, hypothyroidism, anemia, Addison disease, systemic lupus erythematosus , Behçet's syndrome, polycystic ovary syndrome, coagulation disorders, and uremia. Certain medications have also been associated with IIH, including tetracyclines , vitamin A, lithium, anabolic steroids, oral contraceptive pills, nalidixic acid, and cyclosporine.

57. Diagnostic Criteria IIH is a diagnosis of exclusion. Nonetheless, diagnostic criteria were established by Dandy in 1937. However, over the years some modifications were provided and the new criteria are now known as the modified Dandy criteria: 1- Signs and symptoms of increased ICP (headaches, nausea, vomiting, transient visual obscurations, papilledema) 2- No localizing neurologic signs, except for unilateral or bilateral sixth cranial nerve palsy 3- CSF opening pressure >25 cm H2O with normal CSF composition 4- No evidence of hydrocephalus, mass, structural, or vascular lesion (including venous sinus thrombosis) on imaging 5- No other cause of increased ICP identified

58. Medical therapy Carbonic anhydrase inhibitors (acetazolamide) and weight loss (of about 5-10% ) are first line therapy for IIH but can be used in patients with elevated ICP from other etiologies. Recent clinical trials (e.g., Idiopathic Intracranial Hypertension Trial (IIHT)) have provided strong support for safe and effective use of acetazolamide combined with weight reduction in effectively treating IIH and improving papilledema in these patients with mild vision loss

59. Medical therapy Furosemide (Lasix) and topiramate are considered second line agents in IIH but may be useful in patients who are intolerant of acetazolamide. Topiramate , sold under the brand name  Topamax  , is a Carbonic anhydrase inhibitor medication used to treat  epilepsy and prevent  migraine)

60. In general however as steroids (especially with change in dosage or withdrawal) have also been causative of increased ICP we generally do not recommend steroids for IIH. Serial lumbar punctures are not typically recommended as a treatment for IIH but can be used to treat intracranial hypertension if the patient is not suitable for medical therapy or is pregnant or has cryptococcal meningitis related increased ICP. Medical therapy

61. Surgery Potential indications for surgery in patients with IIH : Development of a new visual-field defect Worsening of a previous visual field defect Severe visual loss at the time of presentation Anticipated hypotension induced by treatment of high blood pressure of renal dialysis Psychosocial reasons: non-compliance to medication, inability to perform visual field studies Refractory headache

62. Surgery The two most used procedures are CSF diversion via shunt and optic nerve sheath fenestration. The choice of the procedure is based on patients’ signs and symptoms.

63. Surgery Optic nerve sheath fenestration is the preferred surgical procedure for papilledema with associated severe vision loss but no or minimal ICP symptoms (such as headache). It has been shown to preserve or restore vision in 80-90% of patients. The procedure involves incisions in the abnormally bulbous anterior dural covering of the optic nerve sheath which creates an outlet for continuous CSF drainage. Consequently, the CSF no longer distends the sheath and axoplasmic flow in the optic nerve is restored. It is also considered to be the safest approach among patients with renal failure requiring hemodialysis and for vision loss occurring during pregnancy.

64. Surgery CSF shunting produces rapid reduction in ICP and is therefore most beneficial among patients with vision loss and symptoms of raised ICP. Two types are available: lumbo -peritoneal (LP) and ventriculo -peritoneal (VP). Although VP shunting is more difficult, as IIH patients do not have enlarged ventricles, it is the preferred method due to its lower complication rate. Complications of CSF shunting including shunt obstruction, shunt migration, intracranial hypotension, and tonsillar herniation. Sinclair et al. found that shunt revisions were needed in 51% of patients, with 30% requiring multiple revisions.

65. Surgery Venous sinus stenting (VSS) is an emerging procedure based on the findings of venous sinus stenosis in IIH patients. After identification of the stenotic area on MRV, further testing (e.g. digital subtraction venography and manometry ) is needed to establish a pressure gradient (typically ≥ 10mmHg) before proceeding with stent placement. VSS has been shown to decrease cerebral venous pressure, leading to increased CSF absorption and consequent decrease ICP. Some studies have demonstrated improvement in symptoms following this procedure. However, despite its possible beneficial outcomes, the procedure may be associated with serious complications such as stent migration, venous sinus perforation, in-stent thrombosis, subdural hemorrhage and recurrent stenosis formation proximal to the stent.

66. Prognosis Factors independently associated with a worse visual outcome : -Gender (male) -Race (Black) -Morbid obesity -Anemia -Obstructive sleep apnea -Acute onset of symptoms and signs of raised ICP (fulminant IIH) Recurrence may occur in 8 to 38% of patients weeks to years following recovery from initial presentation or a prolonged period of stability. Weight gain has been associated with disease recurrence.