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5. Lueck Ptosis (Text only).ppt
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Transcript of 5. Lueck Ptosis (Text only).ppt
Ptosis
Dr. Christian LueckDepartment of Neurology
The Canberra Hospital andANU Medical SchoolCanberra, Australia
Ptosis
• abbreviation of βλέφαροπτώσις (“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 creaseB: upper marginal reflex distanceC: palpebral fissure
Eyelid Anatomy and Function• lid position determined by:
– levator palpebrae superioris (LPS):• oculomotor nerve (superior division)
– superior tarsal (Müller’s) muscle:• sympathetic
– surrounding muscles:• frontalis• orbicularis oculi
scapula.pl/anatomia/duze_rys/image894.gifcueflash.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 sec
Margin 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.jpghttp://neuromuscular.wustl.edu/pics/people/patients/CMSrapsynsm.jpg
http://bestpractice.bmj.com/best-practice/monograph/1168/resources/image/bp/9.htmlhttp://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 activity
http://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.gifcueflash.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.jpghttp://www.oculist.net/downaton502/prof/ebook/duanes/graphics/figures/v2/0400/003f.jpg
http://www.cancertreatment-wecareindia.com/other_condition/images/Eyelid%20Cancer.jpghttp://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– Duane’s 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 fracture Ahmad K et al. Practical Neurology 2011;11:332-340
Sargent JC. Nuclear and infranuclear ocular motility disorders. In Miller NR et al.Walsh & Hoyt’s Clinical Neuro-Ophthalmology, 6ed. Lippincott Williams Wilkins, 2005
Bilateral Duane’s 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 activity
Burde, RJ et al. Clinical Decisions in Neuor-Ophthalmology, 3e.Mosby, 2002
the sympathetic pathway
Horner’s syndrome
• partial ptosis (< 3mm)
• associated features:– small pupil– may be lower lid ptosis– anhidrosis– depigmented iris (congenital)
• pharmacological tests:– confirm presence of Horner’s
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 Horner’s 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):• Parinaud’s 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 afteradministration 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.fullhttp://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/PhotosUgarte M & Teimory M. Br J Ophthalmol 2007;91:854
http://bjo.bmj.com/content/suppl/2007/06/15/91.7.854.DC1/ugartefinalfast.mov
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.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;2:364-36
Management of Ptosis• history:
– how many eyes affected?– any associated symptoms?
• pain, malaise, visual disturbance, diplopia, dysphagia, or muscle weakness elsewhere?
– speed of onset, the duration and the extent of progression of the ptosis?• does the ptosis fluctuate?• are there any obvious relieving and exacerbating factors?
– does the patient have any co-morbidities? • vascular risk factors, a history of injury to head, neck or chest, a history of HIV
or other cause of immunosuppression, features of the metabolic syndrome, cancer or ocular disease
• any systemic features of giant cell arteritis?– history of trauma, ophthalmic surgery, or rubbing of the eyelid?
• does the patient wear contact lenses? – blepharoplasty in the past?– any medications, regular or new?– family history of ptosis or other muscle weakness?
Management of Ptosis• examination:
– general systemic and neurological examination:• N.B. pupils, visual acuity and fields, fundoscopy, extraocular and facial
movements and any evidence of other cranial nerve signs. – inspect eyelids:
• symmetry, visible lesions, thickening, discolouration and evidence of involuntary movement
• position of the skin crease, palpebral fissure, uRMD and eyelid excursion. – look for fatigability:
• ask patient to maintain fixation in upgaze for 60 seconds and remeasuring the palpebral fissure immediately afterwards
• ice or rest test may demonstrate reversibility of the ptosis in myasthenia• eye closure is frequently weak in ocular myasthenia
Management of Ptosis
• treatment:– depends on underlying cause– referral to oculoplastic surgeon:
• shortening of LPS• insertion of tendon slings
– ptosis props:• rarely effective
Ahmad K et al. Practical Neurology 2011;11:332-340