Is it Papilloedema?Papilloedema?

John Ross Ainsworth Orthoptic staff Birmingham Children’s Hospital Birmingham and Midland Eye Centre Unive rsit y of Bi rmingham AimsAims

 Children/young people

 A bit about hypoplasia/ NFL

 Why is it a problem?

 Background

 Mild optic disc changes

 Marked disc changes

 Testinggg for pppppapilloedema Optic nerve hypoplasia

Failure of development of optititic nerve OROR Abnormal loss of neurones earlypgy in pregnanc y

Cupped optic discs (non -- gg)laucomatous)

Late prenatal or perinatal cause : expanded occipital horns, loss of white/white/graygray matter posteriorly. “Cupped” or atrophic optic discs) Optic atrophy Look at nerve fibre layer – biomicrosocopypypy and fundus ppgpyhotography Why do we have a problem?

Pseudopapilloedema and headache are common Raised ICP in children is unusual Poor evidence base for investigation No singggggle gold standard test Many cases left with various degrees of uncertainty AnxietyAnxiety

 DoctorDoctor

 OtOptomet titrist

 FamilyFamily Opportunistic screening without evidence base. Background

Papilloedema a sign of raised intracranial pressurepressure

 Idiopathic intracranial hypertension

 Obstructive

 SpaceSpace--occupyingoccupyingoccupying lesionlesion

 Communicating

 EdEndocri iEdine

 DrugsDrugs

 Systemic disorder C a u s e s o f Causes of ↑ ↑ ICPICP Endocrine disorder Hyperthyroidism DrugsDrugs Hypothyroidism Vitamin D deficiency Tetracycline Hypoparathyroidism Corticosteroid withdrawal Adrenal insufficiencyyy  Inc topical for eczema Nitrofurantion hyperadrenalism NalidixicNalidixicacidacid Head trauma Oral contraceptive SystemicSystemicSystemic IsoretinoinIsoretinoin InfectionsInfections Thyroid replacement  Otitis media/mastoiditis media/mastoiditis Growth hormone replacement  SinusitisSinusitis Vasopressin Venous sinus thrombosis 11°°,2,2°° PhenytoinPhenytoin Systemic disorders Indomethacin SLESLE Cyclosporin Protein malnutrition GuillainGuillain--BarreBarresyndrome Iron deficiency anaemia LeukaemiaLeukaemia Hypercoaguable states Symptoms of ↑↑ICPICP HeadacheHeadache

 Characteristic Vomiting without nausea Visual disturbance

 Characteristic obscurations

 DiplopiaDiplopia

 less specific : photopsias/ photophobia

 Vision losslossVision Back painBack pain TinnitusTinnitus

 pulsatilepulsatile ICPICP standing

on standing ↑↑ on

ofof of better loss,not on standing

, lying

on

worse worse on lying better on standing ––

SymptomsSymptoms Symptoms obscurations if profound disc swelling, unequivocal atrophy postural –– VI palsy present if unequivocal manifest nausea) – VI palsy present unequivocal manifest if oss ll l useful unless characteristic (too common) without nausea) Especially postural Worse on wakening, less during the day Opposite of tension headache / migraine bilateral Momentary bilateral complete vision loss,not complete vision up (this is postural hypotension)    

UsefulUseful NotNot Characteristic obscurations Vision DiplopiaDiplopia Vision and/or haemorrhages/exudates     HeadacheHeadache (Vomiting without Visual disturbance Idiopppathic intracranial hypertension

 Raised ICP with

 Normal imaging

 Normal CSF composition

 Normal consciousness

 Normal neurology

 Except papilloedema/VI palsy

 >70% not obese in children Intracranial pressure

Upper limit of normal

 20cm CSF –– older childolder child

 13.5 cm H13.5 H22O < 5 years old

 7.5 cm HH7.5 22O < 2 years old

Most under GA or sedated

 Effect on ICP not studied.

X mmHg = 1.36X cmH 22OOO X cmHcmHX 22O = 0.736X mmHg Papppilloedema Raised ICP and papilloedema in aastronautstronaut after prolonged weightlessness

In papilloedema there is an expanded subarachnoid space just behind the globe Is it MILD Papppilloedemailloedema?? Papppilloedema or ppppseudopapilloedemailloedema?? Is it MILD Papppillodemaillodema??

Chklhklhecklist Optic disc edema Pseudopapilledema with buried drusen Disc vasculature obscured at disc Disc vasculature remains visible at disc margins margins Elevation extends into peripapillary Elevation confined to optic disc retina Graying and muddying of peripapillary Sharp peripapillary nerve fiber nerve fibre layer Venous congestion No venous congestion +/– Exudates / NFL haemorrhage No exudates, NFL hge rare Loss of optic cup only in moderate to Small cupless disc severe disc edema Normal configuration of disc vasculature Increased major retinal vessels with despite venous congestion early branching No circumpapillary light reflex Crescentic circumpapillary light reflex Absence of spontaneous venous Spontaneous venous pulsations may be pulsations present or absent

Taylor DSI. Paediatric Ophthalmology Papppilloedema or ppppseudopapilloedemailloedema??

True

PseudoPseudo

TrueTrue PseudoPseudo Spontaneous venous pulsation

Video SVP : Direct ophthalmoscopy

Very useful for SVP as greater magnification than biomicroscopy, but not done like this, and here child is too young except for red reflex check! Absent SVP in ppppseudopapilloedema 25% vs 75% in normals

Bilateral pseudopapilloedema : Ekdawi, Brodsky Rochester BJO 2011 elevated discs but no surroundign oedema NonNon--mydriaticmydriatic fundus camera : children >3y.o. find very easy, allows assessment and communication

Orthoptist or technician using non-mydriatic (no drops needed) fundus camera OCTOCT

 OCT of optic disc not useful

 Too much variability in population

 RNFLARNFLARNFLA –– retinal nerve fibre layer analysis

 Useful in diagnosis and followfollow--upup

 Measures oedema around disc

 Use same ppgrogramme as g laucoma, but looking for ↑↑ not ↓ thickness Resolving papilloedema following treatment Developing ppv apilloeddp ema Causes of pseudopapilloedema

Hypermetropia Small discsdiscsSmall Disc drusen Dysplastic discs

Exposed disc drusen DiscDiscDisc drusendrusen Buried drusen

Daughter anddd FatherFatherFather

ExposedExposed drusendrusen With ageWithageage Tests for Disc DrusenDrusen?? Tests for Disc Drusen

Priel E.E. J J Ophth PhotoPhoto 2007 2007 Disc drusen: Red free with Autofluorescence

Fong Bristol Arch Dis Child 2010Child 2010 FAF : autofluoresence with cSLO

488nm excitation Barrier/emission >500>500--520nm520nm

Disc drusen

 emission 520--520nm520nm DiscDiscDisc DrusenDrusen

U/S set to low gain confirms buried Disc drusen(here, in both eyes surprisingly) Is it SEVERE papilloedema? Optic disc edema Pseudopapilledema with buried drusen Disc vasculature obscured at disc Disc vasculature remains visible at disc margins margins Elevation extends into peripapillary Elevation confined to optic disc retina Graying and muddying of peripapillary Sharp peripapillary nerve fiber nerve fibre layer Venous congestion No venous congestion +/– Exudates / NFL haemorrhage No exudates, NFL hge rare Loss of optic cup only in moderate to Small cupless disc severe disc edema Normal configuration of disc vasculature Increased major retinal vessels with despite venous congestion early branching No circumpapillary light reflex Crescentic circumpapillary light reflex Absence of spontaneous venous Spontaneous venous pulsations may be pulsations present or absent Taylor DSI. Paediatric Ophthalmology Papilloedema Severe Papilloedema : PRATsPRATs

Focal arrest in axonal transport : same as cotton wool spot Severe Papilloedema : Haemorrhage Severe Papilloedema : Haemorrhage

Macular star pointing to disc Severe Papilloedema : Haemorrhage

Haemorrhages and axonal tttilidtransport arrest imply rapid ongoing permanent damage tiitiito vision

There is usually a reversible element to vision loss if treated urgently Vessel obscuration on disc Different questions depending on how obvious are the sigggns

Mild signsMild signs Obvious signs

 Is it normal or not?  Could it be something

 Is it pseudopapilloedemapseudopapilloedema?? differentdifferent??  EEEg iihschemi c opti c neuropath th?y? “It can’t be papilloedema” ”It can’t be papilloedema”papilloedema”

Vision loss without papilloedema, due to prepre--existentexistent obvious optic atrophy, ititithkin patients with known pas thitfiditilt history of raised intracranial pressure an dilldd papilloedema. CommentComment Most important diagnostic test is fundus examination

 Use photo as ancillary and help communication

 Direct ophthalmoscope for SVP

 ChecklistChecklist Next most imppyortant is history

 SymptomsSymptoms

 NeurologyNeurology ReferralReferral ReferralReferral

If there is papilloedema an urgent referral (I.E. SPEAK WITH THE HOSPITAL THAT DAY) is required is required ReferralReferral

 intended to be guidance about which conditions reqqquire emergency or urgent referral.

 You should follow relevant local ppprotocols for referral.

 If a patient presents with a condition requiring an emergency reeeferr al you m ay wi sh toseeto seek advice from the on-on-callcall ophthalmologist Headaches in general – common and worth learning about them IHSIHS

 Need good source of information about headaches

 Eg International HHeadache society websitewebsite

 PS nonPSnonnon--specific headaches are very common in children (and adults)