27 nystagmus hubli
TRANSCRIPT
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Management of Nystagmus the
Ophthalmologists perspectiveDr. R.R.Battu
Consultant Pediatric Ophthalmologist
Narayana NethralayaBangalore
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Historically
What is the presenting feature? Informant:::
Nystagmus -Wobblyeyes
Anomalous HeadPosture
Poor vision Photophobia
Night blindness Oscillopsia
Vertigo
Diplopia
Head nodding
Many times a combination of the above !!
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Historically
Family history Poor vision
Nystagmus Neurological disease
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Historically
When did this start? At birth or shortly thereafter [ Congenital or
infantile nystagmus ] Congenital sensory or motor nystagmus Congenital neurological nystagmus Rare variants
PAN
Spasmus nutans
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Historically
MedicationAnticonvulsants
Sedatives Psychiatric medications
Occupation [ - and hobbies? ] Epilepsy Head Trauma Neurological abnormalities.. Craniofacial anomalies
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Is there a visual defect?
If so, qualify and quantify Is this likely to be an Ocular nystagmus
Sensory defect nystagmus [ SDN ]
Latent nystagmus [ LN/ MLN ]
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Observe
One time observation
Multiple session observation Usually required in children
Tired adults
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What to Observe
The eye The alignment
The nystagmusAnomalous Head position
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The Eye
Evaluate refractive error Evaluate the anterior segment
Evaluate the posterior segment
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Visual Acuity
Behaviour
Eye poking
Pre verbal child or infant Fix and followOther techniques
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Special problems with Latentnystagmus - Infantile Esotropia
Fogging
Polarised glasses Vectograph
Neutral density filter
Remote occlusion
The Spielman Occluder
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The Eye
Microphthalmos Obvious malformations
AFFERENT PUPILLARY DEFECT
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The Eye
Iris Obvious or subtle transillumination defects
Ocular or oculocutaneous albinism is usually astraightforward diagnosis. The anteriorsegment clues you onto the typical posteriorsegment abnormalities
The lens Cataract
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The Eye
Optic nerve abnormalities Hypoplasia
Atrophy
Coloboma
Retinal abnormalities Albinism
Macular hypoplasia
Cicatricial ROP
Dysplasia
Coloboma
Pigmentary retinopathy
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The Alignment
Ortho, Eso or Exo?In an infant:
Eso - Infantile esotropia with LN/MLN
Nystagmus Compensation Syndrome
Exo Infantile exo,
many times with neuro-developmental issues
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The Nystagmus
Pendular or Jerk Direction Frequency and Amplitude
Variation with gaze
Variation with convergence Variation with monocular
occlusion
Binocular symmetric
Binocular asymmetric Monocular
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How long to observe ?
Single concentrated effort of observation ofat least 3 minutes !!!
Periodic Alternating Nystagmus
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Serious neurological disease?
Asymmetricnystagmus
Monocular nystagmusVisual pathwaydisorders !
Vertical nystagmus
Purely torsionalnystagmus
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Evaluation
Asymmetric nystagmus
INO
Spasmus nutans
Rarely Congenital nystagmus
Parasellar tumours
Restrictive or paralytic ocular muscular disorders
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Congenital Idiopathic Nystagmus
Observation Most commonly horizontal
Pendular or jerk
Horizontal nystagmus invertical gaze positions [Uniplanar ]
Null position Eccentric oron near gaze
Usually symmetric
Fulcrum of rotation inapparently asymmetricnystagmus.
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Congenital Idiopathic Nystagmus
Typically 3 phases of development [ Dr. RobertReinecke]
Phase 1- Broad triangular wave form [ 3-6 mths]
Phase 2- low amp pendular waveform [6-24 months]
Phase 3-Typical jerk nystagmus [24-36 months]
Historically:
No oscillopsia Invariably improves with age
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Spasmus nutans
Head noddingAnomalous head position
Monocular/asymmetric nystagmus Shimmering
RULE OUT CNS TUMOUR [ glioma ]
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Latent nystagmus/ Manifest LatentNystagmus
Probably the only cause of Infantile nystagmus
which does not need Electrophysiologic study orNeuro imaging
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Latent nystagmus
Beats away from thecovered eye [ towardsthe fixing eye ]
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Anomalous Head Position
Null point Beware PAN
Wandering Null point Usually in an eccentric gaze position Head is positioned AWAY from the null
point i.e. Null point to left, face turn to right
Mostly lateral turn, occasionally verticaland cyclovertical head turns
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Electrophysiology
ERG, EOG and VER Would probably be indicated in most
situations as an initial workup May allow to avoid neuroimaging
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Neuro imaging
Again, would probably be required as aninitial workup, unless there is
unequivocally ophthalmic cause ofnystagmus evident on examination andElectrophysiology
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TREATMENT
Drug treatment Optical treatment
Chemodenervation Surgical treatment
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Drug Therapy - Specific
Pendular Nystagmus Gabapentin andMemantine
PAN Baclofen
Superior Oblique MyokymiaCarbemazipine, Gabapentin
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Drug Therapy Less specific
Pendular Valproate, Trihexyphenidyl,Isoniazid, Cannabis
Downbeat nystagmus 3,4diaminopyridine, 4 aminopyridine,gabapentin, clonazepam, baclofen
Any form of Nystagmus Clonazepam,baclofen
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Optical treatment
CORRECT REFRACTIVE ERROR
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Refraction in nystagmus
1. Binocular UCVA in forced pp2. Binocular UCVA in preferred AHP
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Refraction in nystagmus
1. Binocular retinoscopy with patient fixing eitherin AHP or forced PP
1. Put the lenses in front of both eyes, fog one eye by1-3 lines
2. Subjectively refract other eye
3. Repeat on the other side
4. If there is no strabismus ( orthophoric), then addupto 7pd BO prism and -1.0DS to the prescription,observe nystagmus and check binocular acuity
5. Repeat all steps with cycloplegia
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Factors which can be improved
Visual acuityVA, contrast sensitivity, colour, motion sensitivity,
gaze angle
Anomalous Head Position Congenital nystagmus, acquired nystagmus,convergence damping, adduction null in LN/MLN
OscillopsiaAcquired nystagmus, decompensated congenital
nystagmus Hypo accommodation Photophobia
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Refractive Correction
In children upto 10 years, full cycloplegicrefraction
In adults, subjective, try to push over timeif there is a difference in sub and objrefraction
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Amblyopia therapy
May significantly decrease or eliminateMLN LN
Periods of occlusion have to be veryprolonged in patients with LN
Alternatively fogging or penalisation may
have to be used
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Optical treatment
To direct the null point centrally Prisms placed with apex directed towards the
null point. Large power prisms may have to be used.
Fresnels
May degrade vision
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Optical treatment
To stabilize visual image on the retina High plus spectacle with high minus contact
lens[ -58 & +32 ] Entire 30 deg field focussed to centre of eye,
and CL refocuses to the retina.
Image remains stable irrespective of eyemovement !!
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Optical treatment
To induce convergence Base out prisms bilaterally
Induce a convergence Useful only if there is a convergence null
May have to compensate with a -1.0 sph for
induced accommodation
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Chemodenervation
Botox
2.5 5 units into all horizontal recti
Retrobulbar injection of 25 30 units
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Chemodenervation
Useful to reduce amplitude of nystagmus Has been shown to improve foveation
time and improve visual acuity slightly. More useful in neurological acquired
nystagmus, particularly in oculopalatal
myoclonus RB injection effect lasts for several weeks
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Chemodenervation
Complications include Ptosis
Diplopia Filamentary keratitis
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Electronystagmography
Nystagmovideography
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Surgical principles
Decrease the amplitude of nystagmus Maximal recession of horizontal muscles
Tenotomy
Increase foveation time
Tenotomy Broaden the null zone
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Rotate the null zoneAnderson Goto
Kestenbaum Parks modification of KestenbaumAugmented Kestenbaum
40% 60%
Induce an attempt to convergeArtificial divergence surgery
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Surgery to correct AHP
Face turns - horizontal
Anderson advocated bilateral recession Eg. Null zone to left, weaken levo-verters
Kestenbaum advocated recess-recess [pull and push]
Parks modification of Kestenbaums 5-6-7-8 rule [both eyes get 13 mm ]Very rarely corrects more than 10 -15 degrees
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Surgery to correct AHP
Augmented K-A procedure Classic + 40% - For > 30 deg of face turn
Classic +60% - for > 45 deg of face turn
Problems
Intractable diplopia
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Surgery to correct AHP
Vertical AHP
Chin up
IR recess SR resect Chin down
IR resect SR recess
Anteriorisation of IO
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Patient with right horizontal gaze palsy and head turn of approximately 20 to the right (a); the same patient 1 year after
recession of right medial rectus and left lateral rectus muscles (b). Note: the patient can use his glasses more effectively.
Patient with acquired nystagmus equilibrium in upward gaze; CHP with chin-down is present (c); the same patient 1 year
after surgical weakening of both superior rectus muscles (d).
E C Campos1, C Schiavi1 and C Bellusci1.
Surgical management of anomalous head posture because of horizontal gaze palsy or acquired vertical nystagmus
Eye (2003) 17, 587
592. doi:10.1038/sj.eye.6700431
http://www.nature.com/eye/journal/v17/n5/full/6700431a.htmlhttp://www.nature.com/eye/journal/v17/n5/full/6700431a.htmlhttp://www.nature.com/eye/journal/v17/n5/full/6700431a.htmlhttp://www.nature.com/eye/journal/v17/n5/full/6700431a.htmlhttp://www.nature.com/eye/journal/v17/n5/full/6700431a.htmlhttp://www.nature.com/eye/journal/v17/n5/full/6700431a.html -
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Surgery to correct AHP
Cyclovertical AHP
As an adaptation to torsional nystagmus Surgery to recreate the torsional directioncreated by the patients head tilt
Several methods Strengthen or weaken obliques Slanting recti insertions
Vertical recti slanting
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Surgery
Other problems Management of co existent strabismus with
nystagmusAcquiring of a new head position - PAN
Creating a new strabismus
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Surgery primarily designed toimprove vision
Artificial divergence Bimedial recession
Unilateral recess-resect to XT 4 muscle retro equatorial recession
10 mm MR and 12 mm LR
Ideal for PAN May induce an exotropia
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DellOsso & Hertle
Based on the principle of enthesialproprioceptive input to nystagmus at the
insertion of the horizontal recti Dell'Osso LF. Extraocular muscle tenotomy, dissection, and suture: A hypothetical therapy for
congenital nystagmus. J Pediatr Ophthalmol Strab 1998; 35:232-3.
Hertle RW, Dell'Osso LF, FitzGibbon EJ, Thompson D, Yang D, Mellow SD. Horizontal rectustenotomy in patients with congenital nystagmus. Results in 10 adults. Ophthalmology 2003;110:2097-105.
Hertle RW, Dell'Osso LF, FitzGibbon EJ, Thompson D, Yang D, Mellow SD. Horizontal rectusmuscle tenotomy in patients with infantile nystagmus syndrome: a pilot study. JAAPOS 2004;8:539-48.
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Summary
Evaluation of nystagmus ismultidisciplinary
However, it is possible to improve thequality of life with drugs/opticaldevices/surgical procedures
No single procedure has shown to beconsistently predictive of success
This does not mean we cannot try.
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Thank you