5. Lueck Ptosis (Text only).ppt

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Ptosis Dr. Christian Lueck Department of Neurology The Canberra Hospital and ANU Medical School Canberra, Australia Ptosis abbreviation of (blepharoptosis) from Greek meaning fallen eyelid definition: upper marginal reflex distance (uMRD) < 2 mm or asymmetry of 2 mm between two eyes Ahmad K et al. Practical Neurology 2011;11:332-340 A: eyelid crease B: upper marginal reflex distance C: palpebral fissure Eyelid Anatomy and Function lid position determined by: levator palpebrae superioris (LPS): oculomotor nerve (superior division) superior tarsal (Mllers) muscle: sympathetic surrounding muscles: frontalis orbicularis oculi scapula.pl/anatomia/duze_rys/image894.gif cueflash.com/cardimages/answers/thumbnails/6/6/3666695.jpg Eyelid Anatomy and Function LPS tonically active during wakefulness, punctuated by blinks lid typically covers top 20% of cornea, but affected by: vertical eye movements horizontal eye position state of arousal Assessment of Eyelid Function eyelid crease upper marginal reflex distance (uMRD): corneal light reflex to upper eyelid margin ptosis defined as uMRD < 2mm or asymmetry of 2 mm between two eyes palpebral fissure: typically 12-15 mm wide Ahmad K et al. Practical Neurology 2011;11:332-340 Assessment of Eyelid Function LPS function assessment: difference in eyelid margin position in upgaze and downgaze (while holding the eyebrow down to prevent frontalis activity) slow pursuit of an object from upgaze to downgaze in order to detect lid retraction or lid lag fatigability assessed by detecting any lowering of the eyelid during sustained upgaze for at least 60 secMargin of closed eyelid at 20mm. When eyelids fully elevated, increases to 33mm, i.e. eyelid excursion of 13mm (normal) Ahmad K et al. Practical Neurology 2011;11:332-340 Causes of Ptosis congenital: isolated congenital ptosis congenital myasthenic syndromes transient neonatal myasthenia (myasthenic mother) anomalous synkineses (e.g. jaw-winking) blepharophimosis and branchial arch syndromes structural: levator dehiscence other disorders of eyelid disorders of globe/orbit tissues above eye reduced sympathetic activity neurogenic/myogenic: neurogenic neuromuscular junction myogenic central causes: cerebral cortex basal ganglia excessive orbicularis activity http://eyepathologist.com/images/w2269.jpg http://neuromuscular.wustl.edu/pics/people/patients/CMSrapsynsm.jpg http://bestpractice.bmj.com/best-practice/monograph/1168/resources/image/bp/9.html http://bestpractice.bmj.com/best-practice/monograph/1168/resources/image/bp/11.html Congenital ptosis, congenital myasthenia (with facial dysmorphism), Marcus-Gunn (jaw-winking) phenomenon, blepharophimosis Causes of Ptosis congenital: isolated congenital ptosis congenital myasthenic syndromes transient neonatal myasthenia (myasthenic mother) anomalous synkineses (e.g. jaw-winking) blepharophimosis and branchial arch syndromes structural: levator dehiscence other disorders of eyelid disorders of globe/orbit tissues above eye reduced sympathetic activity neurogenic/myogenic: neurogenic neuromuscular junction myogenic central causes: cerebral cortex basal ganglia excessive orbicularis activityhttp://www.hessemer-augen.de/db_pics/content/lid-blepharochalasis.jpg blepharochalasis (not strictly ptosis) Structural Causes levator dehiscence (aponeurotic ptosis): middle-aged to elderly patients disinsertion of LPS tendon from tarsal plate may follow: trauma ophthalmic surgery (usually cataract) wearing hard contact lenses rubbing eyes clinical features: high skin crease (> 7 mm from margin) thinned eyelid normal range of movement Left levator dehiscence (photos courtesy of Dr. M. Wright, Edinburgh) scapula.pl/anatomia/duze_rys/image894.gif cueflash.com/cardimages/answers/thumbnails/6/6/3666695.jpg Structural Causes disorders of eyelid and surrounding structures: oedema infections tumours floppy eyelids http://images.paraorkut.com/img/health/images/a/angioneurotic_edema-321.jpg http://www.oculist.net/downaton502/prof/ebook/duanes/graphics/figures/v2/0400/003f.jpg http://www.cancertreatment-wecareindia.com/other_condition/images/Eyelid%20Cancer.jpg http://radiographics.rsna.org/content/26/1/157/F25.large.jpg angioedema hordeolum (stye) enlarged lacrimal gland plexiform neurofibromatosis Structural Causes floppy eyelid syndrome: first reported 1981 (Cuthbertson & Ostler) loose upper lid that readily everts soft, rubbery tarsus, easily folded chronic papillary conjunctival response typical in obese middle-aged men associated with obstructive sleep apnoea, hypertension and ischaemic heart disease Miyamoto C et al. Arq Bras Oftalmol 2011;74 Structural Causes retraction of globe due to: congenital microphthalmos Duanes retraction syndrome damage to orbital floor (e.g. #) scirrhous orbital secondary (may be elevation of lower eyelid) Left pseudoptosis due to enophthalmos following orbital floor fractureAhmad K et al. Practical Neurology 2011;11:332-340 Sargent JC. Nuclear and infranuclear ocular motility disorders. In Miller NR et al. Walsh & Hoyts Clinical Neuro-Ophthalmology, 6ed. Lippincott Williams Wilkins, 2005 Bilateral Duanes retraction syndrome Causes of Ptosis congenital: isolated congenital ptosis congenital myasthenic syndromes transient neonatal myasthenia (myasthenic mother) anomalous synkineses (e.g. jaw-winking) blepharophimosis and branchial arch syndromes structural: levator dehiscence other disorders of eyelid disorders of globe/orbit tissues above eye reduced sympathetic activity neurogenic/myogenic: neurogenic neuromuscular junction myogenic central causes: cerebral cortex basal ganglia excessive orbicularis activityBurde, RJ et al. Clinical Decisions in Neuor-Ophthalmology, 3e.Mosby, 2002 the sympathetic pathway Horners syndrome partial ptosis (< 3mm) associated features: small pupil may be lower lid ptosis anhidrosis depigmented iris (congenital) pharmacological tests: confirm presence of Horners syndrome help to localise lesion (1st, 2nd, or 3rd order neuron) Ahmad K et al. Practical Neurology 2011;11:332-340 two examples of right-sided Horners syndrome (photo courtesy of Dr. M. Wright) Causes of Ptosis congenital: isolated congenital ptosis congenital myasthenic syndromes transient neonatal myasthenia (myasthenic mother) anomalous synkineses (e.g. jaw-winking) blepharophimosis and branchial arch syndromes structural: levator dehiscence other disorders of eyelid disorders of globe/orbit tissues above eye reduced sympathetic activity neurogenic/myogenic: neurogenic neuromuscular junction myogenic central causes: cerebral cortex basal ganglia excessive orbicularis activity Left-sided oculomotor palsy Neurogenic oculomotor nerve palsy: nuclear (bilateral): Parinauds syndrome fascicular subarachnoid space cavernous sinus superior orbital fissure orbit non-localisable/diffuse Ahmad K et al. Practical Neurology 2011;11:332-340 Miller-Fisher syndrome demonstrating enhanced ptosis when other lid is lifted Left-sided oculomotor palsy Neuromuscular Junction myasthenia gravis: anti-ACh receptor anti-MuSK (congenital myasthenic syndromes) botulism (dilated pupils) Lambert-Eaton myasthenic syndrome very rare Ahmad K et al. Practical Neurology 2011;11:332-340 ocular myasthenia before and after administration of edrophonium (Tensiolon) Myogenic inherited: chronic progressive external ophthalmoplegia (CPEO): Kearns-Sayre syndrome mitochondrial dysfunction (MELAS, MNGIE) muscular dystrophies: dystrophia myotonica oculopharyngeal muscular dystrophy acquired: anti-retroviral therapy orbital myositis dysthyroid eye disease: ? Mechanical disruption of LPS ? Concomitant myasthenia chronic progressive external ophthalmoplegia http://pn.bmj.com/content/8/4/229.full http://jnnp.bmj.com/content/65/3/291.full oculopharyngeal muscular dystrophy Causes of Ptosis congenital: isolated congenital ptosis congenital myasthenic syndromes transient neonatal myasthenia (myasthenic mother) anomalous synkineses (e.g. jaw-winking) blepharophimosis and branchial arch syndromes structural: levator dehiscence other disorders of eyelid disorders of globe/orbit tissues above eye reduced sympathetic activity neurogenic/myogenic: neurogenic neuromuscular junction myogenic central causes: cerebral cortex basal ganglia excessive orbicularis activity Central Causes cerebral cortex: ptosis reported in context of stroke: usually non-dominant hemisphere lesions usually contralateral, but ipsilateral reported mechanism unclear basal ganglia: apraxia of eyelid opening in PSP (not true ptosis as LPS function normal) excessive orbicularis oculi activity: hemifacial spasm blepharospasm functional pseudoptosis www.doctorkraft.com/Photos Ugarte M & Teimory M. Br J Ophthalmol 2007;91:854 http://bjo.bmj.com/content/suppl/2007/06/15/91.7.854.DC1/ugartefinalfast.movCentral Causes cerebral cortex: ptosis reported in context of stroke: usually non-dominant hemisphere lesions usually contralateral, but ipsilateral reported mechanism unclear basal ganglia: apraxia of eyelid opening in PSP (not true ptosis as LPS function normal) excessive orbicularis oculi activity: hemifacial spasm blepharospasm functional pseudoptosis www.oculist.net/downaton502/prof/ebook/duanes/graphics/figures/v7/0370/007f.jpg Central Causes cerebral cortex: ptosis reported in context of stroke: usually non-dominant hemisphere lesions usually contralateral, but ipsilateral reported mechanism unclear basal ganglia: apraxia of eyelid opening in PSP (not true ptosis as LPS function normal) excessive orbicularis oculi activity: hemifacial spasm blepharospasm functional pseudoptosis Stone, J. Practical Neurology 2002;2:364-36Management of Ptosis history: how many eyes affected? any