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Dr Clare Fraser
 Swelling of the optic disc occurs when there is
hold up of axonal transport at the level of the
lamina cribrosa
 = appearance is identical in apparently
different pathological processes
 True vs pseudo
 Unilateral vs bilateral
 Normal vs abnormal vision
The first question to ask
 True
 Blurred disc margin
 Obscured vessels
 Hyperaemic
 Loss SVP (20% normal
patients)
 Pseudo
 Vessels visible
crossing margin
 Anomalous branching
 No micro-vascular
congestion
 Drusen
 Hypermetropia
 Crowded discs
 Hyaloid remnants
 Myelinated nerve fiber layer
Less helpful but worth considering
UNILATERAL
 Local
 Optic neuritis
 Vascular occlusion
 Infiltrative
BILATERAL
 Systemic
 Disc drusen
 Infectious optic neuritis
 Papilledema
 Malignant hypertension
 Toxicity
The final question to ask
 Papilloedema
 preserved visual function
 Papillitis secondary to local causes
 Poor visual function
 Ischaemia
 visual loss is determined by the occurrence of disc
infarction
 Optic nerve
 Optic neuritis
 Ischaemic ON
▪ arteritic AION
▪ NA-AION
 Compressive orbital
 Infiltrative
 LHON
 Vascular
 CRVO
 malignant hypertension
 Ocular
 Uveitis / posterior
scleritis
 Hypotony
Characteristic Patients
Female
Caucasian
Age
Ocular pain
Pain on eye m'ment
Optic disc
Normal
Swollen
No retinal or disc Hb
Vision
6/6 or better
6/6 - 6/12
6/15 – 6/240
CF or LP
NPL
77%
85%
32
92%
87%
65%
35%
85%
11%
25%
49%
13%
3%
 90% will have 6/12 vision or better
 75% will have 6/9 vision or better
 40% develop Multiple Sclerosis in 10 years
 30% have a relapse of optic neuritis in 5 years
 Leukaemia
 Lymphoma
 Papilledema
 brain tumour
 thrombosis
 meningitis
 malignant hypertension
 pseudo-tumor cerebri
 IIH
 Optic nerve
 perineuritis
 diabetic papillopathy
 optic neuritis (early)*
 Vascular
 CRVO
 There are no clinically diagnosable causes of
optic disc swelling with normal vision
 All require some form of investigation
 Urgency depends of speed of symptom onset
 Simple things first
 Blood pressure
 Temperature (meningitis)
 Urine analysis (haematuria in vasculitis)
 Systemic hypertension
 Space occupying lesion
 Dural venous sinus thrombosis
 Meningitis
 Pseudotumour cerebri / IIH
 MRI and MRV with contrast
 CT and CTV is sufficient
 Referral for lumbar puncture
Pseudotumour cerebri
 Headache
 Pulsatile tinnitus
 Transient visual obscurations
 Double vision
 Incidental finding at an optom visit
 Daily diffuse non-pulsating
 Any location
 Worse lying flat or straining
 Wakes up with headache, clears after 30-60
minutes
 Can sound migrainous – pulsating, unilateral
 Increased ICP worsens all other types of
headache tendencies
 Signs and symptoms of increased ICP
 Headaches, nausea, vomiting,TVO, papilledema
 No localising focal neurological signs
 Except CNVI palsies
 CSF opening pressure >25cm H20
 Normal constituents
 Normal neuroimaging
 Exclude mass lesion, venous sinus thrombosis
 No other underlying cause identifiable
 “No other underlying cause identifiable”
 So I prefer to use the term
 “pseudotumour cerebri”
 Especially if the patient has BMI <25
 Until I am happy there is no underlying cause
 As a reminder to keep looking for one
 Drugs
 Vit A, tetracyclines, steroids, some NSAIDS,
cyclosporin, OCP
 Diseases
 COPD, sleep apnea, renal failure, anaemia
 Need urgent attention if
 rapid increase in symptoms
 acuity loss
 Obese women
 Dose relationship between BMI and risk
 Lower body (gynaecoid) adiposity
 Childbearing age
 Increased risk of IIH with 5-15% weight gain1
 even if BMI remains <30
 A return to a BMI similar to the time of first
presentation = risk for IIH recurrence2
 even a 6% weight gain is a risk of recurrence
 BMI associated with more severe visual loss3
 Even a 10% weight loss can be sufficient
1. Daniels et al. Profiles of obesity in IIH. AmJO 2007;143.
2. Ko M et al.Weight gain in IIH. Neurology 2011;76.
3. Szweka A et al. IIH obesity vs vision. JNO. July 2012
 Seen in 80-100% of
adult IIH patients
 There are no studies in
children
 Alleviate symptoms
 Headache
 Preserve vision
 While the patient loses weight
Based on
 Duration of symptoms
 Speed of symptom onset (?fulminant)
 Evaluation of visual function
 Patient characteristics
 Male
 Black race
 Morbid obesity
 Anemia
 OSA
 1-2g daily in divided dose
 Decreases CSF production
 Side effects
 Parasthesia, altered taste, lethargy
 Low K+
 If not tolerated
 topiramate
 bendroflumethiazide
 Optic nerve sheath fenestration
 Fulminant onset
 Other treatments failing to prevent progressive
vision loss
 Depends on local resources
 Produces a rapid reduction in pressure on the
optic nerve head
 Reduces papilledema
 Improves vision in operated eye
 +/- fellow eye
 Does not decrease ICP
 ? Causes local fistula formation
 Acute and rapidly progressive vision loss
 Headache, no response to other therapy
 Lumbar drain (transient)
 Ventriculo-peritoneal shunt
 Lumbar-peritoneal shunt
 If increased pressure across stenosis
 Reduces cerebral venous pressure
 Reduces ICP and improves symptoms
 Still not widely accepted in international
community, frequently done in Sydney
 IIH is a diagnosis of exclusion
 Pathophysiology is still not understood
 There are no RCTs to guide management
 Seen by an optometrist, patchy HVF changes
 24 year old with reduced vision one eye
 34 yo presents with bilateral vision loss to
6/36 right and 6/12 left
 Referred ?disc swelling
 13 yo girl, 3 week history of severe headaches,
nausea and vision loss to HMs in each eye
 After lumbar puncture, optic nerve sheath
fenestration and medical treatment, 6/36 OU
Elevated optic discs - Are they ever benign?

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Elevated optic discs - Are they ever benign?

  • 2.  Swelling of the optic disc occurs when there is hold up of axonal transport at the level of the lamina cribrosa  = appearance is identical in apparently different pathological processes
  • 3.  True vs pseudo  Unilateral vs bilateral  Normal vs abnormal vision
  • 5.  True  Blurred disc margin  Obscured vessels  Hyperaemic  Loss SVP (20% normal patients)  Pseudo  Vessels visible crossing margin  Anomalous branching  No micro-vascular congestion
  • 6.  Drusen  Hypermetropia  Crowded discs  Hyaloid remnants  Myelinated nerve fiber layer
  • 7.
  • 8.
  • 9.
  • 10.
  • 11.
  • 12.
  • 13.
  • 14. Less helpful but worth considering
  • 15. UNILATERAL  Local  Optic neuritis  Vascular occlusion  Infiltrative BILATERAL  Systemic  Disc drusen  Infectious optic neuritis  Papilledema  Malignant hypertension  Toxicity
  • 17.  Papilloedema  preserved visual function  Papillitis secondary to local causes  Poor visual function  Ischaemia  visual loss is determined by the occurrence of disc infarction
  • 18.  Optic nerve  Optic neuritis  Ischaemic ON ▪ arteritic AION ▪ NA-AION  Compressive orbital  Infiltrative  LHON  Vascular  CRVO  malignant hypertension  Ocular  Uveitis / posterior scleritis  Hypotony
  • 19. Characteristic Patients Female Caucasian Age Ocular pain Pain on eye m'ment Optic disc Normal Swollen No retinal or disc Hb Vision 6/6 or better 6/6 - 6/12 6/15 – 6/240 CF or LP NPL 77% 85% 32 92% 87% 65% 35% 85% 11% 25% 49% 13% 3%
  • 20.  90% will have 6/12 vision or better  75% will have 6/9 vision or better  40% develop Multiple Sclerosis in 10 years  30% have a relapse of optic neuritis in 5 years
  • 21.
  • 22.
  • 23.
  • 24.
  • 25.
  • 27.
  • 28.
  • 29.  Papilledema  brain tumour  thrombosis  meningitis  malignant hypertension  pseudo-tumor cerebri  IIH  Optic nerve  perineuritis  diabetic papillopathy  optic neuritis (early)*  Vascular  CRVO
  • 30.
  • 31.
  • 32.
  • 33.
  • 34.
  • 35.
  • 36.
  • 37.  There are no clinically diagnosable causes of optic disc swelling with normal vision  All require some form of investigation  Urgency depends of speed of symptom onset  Simple things first  Blood pressure  Temperature (meningitis)  Urine analysis (haematuria in vasculitis)
  • 38.  Systemic hypertension  Space occupying lesion  Dural venous sinus thrombosis  Meningitis  Pseudotumour cerebri / IIH
  • 39.  MRI and MRV with contrast  CT and CTV is sufficient  Referral for lumbar puncture
  • 41.  Headache  Pulsatile tinnitus  Transient visual obscurations  Double vision  Incidental finding at an optom visit
  • 42.  Daily diffuse non-pulsating  Any location  Worse lying flat or straining  Wakes up with headache, clears after 30-60 minutes  Can sound migrainous – pulsating, unilateral  Increased ICP worsens all other types of headache tendencies
  • 43.  Signs and symptoms of increased ICP  Headaches, nausea, vomiting,TVO, papilledema  No localising focal neurological signs  Except CNVI palsies  CSF opening pressure >25cm H20  Normal constituents  Normal neuroimaging  Exclude mass lesion, venous sinus thrombosis  No other underlying cause identifiable
  • 44.  “No other underlying cause identifiable”  So I prefer to use the term  “pseudotumour cerebri”  Especially if the patient has BMI <25  Until I am happy there is no underlying cause  As a reminder to keep looking for one
  • 45.  Drugs  Vit A, tetracyclines, steroids, some NSAIDS, cyclosporin, OCP  Diseases  COPD, sleep apnea, renal failure, anaemia
  • 46.  Need urgent attention if  rapid increase in symptoms  acuity loss
  • 47.  Obese women  Dose relationship between BMI and risk  Lower body (gynaecoid) adiposity  Childbearing age
  • 48.  Increased risk of IIH with 5-15% weight gain1  even if BMI remains <30  A return to a BMI similar to the time of first presentation = risk for IIH recurrence2  even a 6% weight gain is a risk of recurrence  BMI associated with more severe visual loss3  Even a 10% weight loss can be sufficient 1. Daniels et al. Profiles of obesity in IIH. AmJO 2007;143. 2. Ko M et al.Weight gain in IIH. Neurology 2011;76. 3. Szweka A et al. IIH obesity vs vision. JNO. July 2012
  • 49.  Seen in 80-100% of adult IIH patients  There are no studies in children
  • 50.  Alleviate symptoms  Headache  Preserve vision  While the patient loses weight
  • 51. Based on  Duration of symptoms  Speed of symptom onset (?fulminant)  Evaluation of visual function  Patient characteristics  Male  Black race  Morbid obesity  Anemia  OSA
  • 52.  1-2g daily in divided dose  Decreases CSF production  Side effects  Parasthesia, altered taste, lethargy  Low K+  If not tolerated  topiramate  bendroflumethiazide
  • 53.  Optic nerve sheath fenestration  Fulminant onset  Other treatments failing to prevent progressive vision loss  Depends on local resources
  • 54.  Produces a rapid reduction in pressure on the optic nerve head  Reduces papilledema  Improves vision in operated eye  +/- fellow eye  Does not decrease ICP  ? Causes local fistula formation
  • 55.  Acute and rapidly progressive vision loss  Headache, no response to other therapy  Lumbar drain (transient)  Ventriculo-peritoneal shunt  Lumbar-peritoneal shunt
  • 56.  If increased pressure across stenosis  Reduces cerebral venous pressure  Reduces ICP and improves symptoms  Still not widely accepted in international community, frequently done in Sydney
  • 57.
  • 58.  IIH is a diagnosis of exclusion  Pathophysiology is still not understood  There are no RCTs to guide management
  • 59.
  • 60.  Seen by an optometrist, patchy HVF changes
  • 61.  24 year old with reduced vision one eye
  • 62.  34 yo presents with bilateral vision loss to 6/36 right and 6/12 left
  • 63.  Referred ?disc swelling
  • 64.  13 yo girl, 3 week history of severe headaches, nausea and vision loss to HMs in each eye
  • 65.  After lumbar puncture, optic nerve sheath fenestration and medical treatment, 6/36 OU

Editor's Notes

  1. a normal optic nerve (A), optic nerve head drusen with moderate (B) and marked (C) elevation, and optic disc edema with mild (D), moderate (E), and marked (F) elevation, based on the papilledema grading scale of Johnson et al. The “lumpy-bumpy” internal contour of the optic nerve head in optic nerve head drusen (B), the recumbent “lazy V” pattern of the subretinal hyporeflective space in optic disc edema (D), and the subretinal hyporeflective space measurement at radii 0.75 mm, 1.5 mm, and 2.0 mm from the optic disc center (E) are depicted.
  2. Syphilis +, resolved with penicillin
  3. On minocycline, rapid progression