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