Ptosis

Functional anatomy• Levator palpebrae superioris (LPS):– is the primary muscle responsible for lid elevation. – It arises from the back of the orbit and extends forwards over the

cone of eye muscles. – It inserts into the eyelid and the tarsal plate, a fibrous

semicircular structure which gives the upper eyelid its shape. – The LPS is supplied by the superior division of the oculomotor

nerve. • Muller’s muscles:– The way that the LPS attaches to the tarsal plate is modified by

the underlying Müller's muscle. – This involuntary muscle, comprising sympathetically innervated

smooth muscle, – has the capacity to 'tighten' the attachment and so raise the lid a

few millimetres.

• Frontalis & orbicularis oculi:– frontalis muscle and the orbicularis oculi, both supplied

by the facial nerve.– Frontalis contraction helps to elevate the lid by acting

indirectly on the surrounding soft tissues, – while orbicularis oculi contraction depresses the eyelid.

DEFINITION

• Ptosis (from Greek Ptosis -to "fall") is a drooping or falling of the upper or lower eyelid.

CLASSIFICATION OF PTOSISA. CongenitalB. Acquired

1. Neurogenic2. Myogenic3. Aponeurotic4. Mechanical5. Neurotoxic

C. Pseudotosis

Congenital ptosis• It is associated with congenital weakness

(maldevelopment) of the levator palpebrae superioris (LPS). 1. Simple congenital ptosis– not associated with any other anomaly.

2. Congenital ptosis with associated weakness of superior rectus muscle.3. Blepharophimosis syndrome,

– which comprises congenital ptosis, blepharophimosis, telecanthus and epicanthus inversus .

4. Congenital synkinetic ptosis – (Marcus Gunn jaw winking ptosis). – In this condition there occurs retraction of the ptotic lid with jaw

movements i.e., with stimulation of ipsilateral pterygoid muscle.

Telecanthus

Epicanthus inversus

Blepharophimosis syndrome

• Rare congenital disorder

• Dominant inheritance

• Moderate to severe symmetrical ptosis• Short horizontal palpebral aperture• Telecanthus (lateral displacement of medial canthus)• Epicanthus inversus (lower lid fold larger than upper)• Lateral inferior ectropion• Poorly developed nasal bridge and hypoplasia of superior orbital rims

Congenital synkinetic ptosis • This condition is characterized as a synkinesis:

when two or more muscles that are independently innervated have either simultaneous or coordinated movements.

• In MARCUS GUNN PHENOMENON – The stimulation of the trigeminal nerve by

contraction of the pterygoid muscles of jaw results in the excitation of the branch of the oculomotor nerve that innervates the LPS ipsilaterally, so the patient will have rhythmic upward jerking of their upper eyelid.

• There are two major groups of trigemino-oculomotor synkineses:

1) External pterygoid-levator synkinesis:– is when the eyelid raises upon Jaw thrust to opposite

side (homolateral external pterygoid) Jaw is projected forward (bilateral external pterygoid) Mouth is opened widely

2) Internal pterygoid-levator synkinesis – is when the eyelid raises upon teeth clenching

• External pterygoid-levator synkinesis is the more common group.

Marcus Gunn jaw-winking syndrome• Accounts for about 5% of all cases of congenital ptosis• Retraction or ‘wink’ of ptotic lid in conjunction with stimulation of ipsilateral pterygoid muscles

Opening of mouth Contralateral movement of jaw

• Inverse Marcus Gunn phenomenon:– Synkinesia between CN V and the levator more rarely causes ptosis on mouth

opening . • Marin Amat syndrome:

– is a facial nerve aberrant innervation syndrome with levator inhibition with mouth opening.

• Ptosis in Lambert-Eaton syndrome :– may temporarily improve after a brief period of upgaze.

• Eyelid myotonia :– may cause transient difficulty opening the eyes after a forceful contraction or

transient lid retraction after looking up. • Blepharospasm :

– is a focal dystonia causing involuntary eye closure; – levator function is normal.

• In apraxia of lid opening, – the patient has difficulty in voluntarily initiating lid elevation although there is

no levator impairment or blepharospasm. • Rosenbach's sign:

– A fine tremor of the lid may occur in hyperthyroidism

ACQUIRED PTOSIS1. Neurogenic– Third nerve palsy– Third nerve misdirection– Horner syndrome

2. Myogenic3. Aponeurotic4. Mechanical5. Neurotoxic

A. Neurogenic ptosis

• It is caused by innervational defects such as third nerve palsy,

• 3rd nerve misdirection• Horner’s syndrome, • Ophthalmoplegic migraine • Cerebral ptosis• Multiple sclerosis.

Right third nerve misdirection• Rare, unilateral• Aberrant regeneration following acquired third nerve palsy• Pupil is occasionally involved• Bizarre movements of upper lid accompany eye movements

Right ptosis in primaryposition

Worse on right gaze Normal on left gaze

Horner syndromeCentral(first order neurone)

• Brainstem disease (vascular, demyelination)• Spinal cord disease (syringomyelia, tumours)

Pre-ganglionic (second order neurone)

• Intrathoracic lesions (Pancoast tumour, aneurysm)

• Neck lesions (glands, trauma)

Post-ganglionic (third order neurone)

• Internal carotid artery disease• Cavernous sinus mass

Posterior hypothalamus

Ciliospinal centre of Budge( C8 - T2 )

Superior cervicalganglion

Phenyl ephrine test

• Patients with minimal ptosis (2 mm or less) should have a phenylephrine test performed in the involved eye or eyes

•   Either 2.5 or 10% phenylephrine is instilled in the affected eye or eyes.  Usually two drops are placed and the patient is reexamined 5 minutes later. 

• The MRD1 is rechecked in the affected and unaffected eyes .

• A rise in the MRDl of 1.5 mm or greater is considered a positive test.  This indicates that Müller's muscle is viable

Ophthalmoplegic migraine• Ophthalmoplegic Migraine is a rare eye disorder, previously called

a “complicated migraine”, which is also recognized as cranial neuralgia by the International Classification of Headache Disorders (HIS II) .

• This disorder most commonly presents itself in early childhood or infancy.

• To date, there is no conclusive hypothesis as to the etiology of this disorder (3, 4).

• Ophthalmoplegic migraines are characterized by – Severe headaches – weakening of muscles around the eye. – these headaches commonly precede episodes of partial paralysis of one

or more ocular nerve (most commonly the third cranial nerve), – drooping of the eyelid, – double vision, – dilation of pupils

IHS diagnostic criteria:

• At least 2 attacks fulfilling criterion B• Migraine-like headache accompanied or

followed within 4 days of its onset by paresis of one or more of the third, fourth and/or sixth cranial nerves

• Parasellar, orbital fissure and posterior fossa lesions ruled out by appropriate investigations

Cerebral ptosis • is due to supranuclear lesions. • Unilateral cerebral ptosis occurs with lesions,

usually ischemic, of the opposite hemisphere, and is more common with right hemisphere lesions.

• Bilateral supranuclear ptosis may occur with unilateral or bilateral hemispheric lesions.

• Ptosis has been reported in as many as 37.5% of patients with hemispheric strokes.

B. MYOGENIC PTOSIS

• It is due to acquired disorders of the LPS muscle or of the myoneural junction.

1. Myasthenia gravis2. Myotonic dystrophy3. Ocular myopathies4. oculo-pharyngeal muscular dystrophy5. following trauma to the LPS muscle.

Myasthenia Gravis• The ptosis in MG is frequently asymmetric and may

be unilateral, though it will tend to shift from side to side

• It characteristically fluctuates from moment to moment and is worsened by prolonged upgaze (fatiguable ptosis).

• Cogan's lid twitch sign, – characteristic of myasthenia, consists of a brief

overshoot twitch of lid retraction following sudden return of the eyes to primary position after a period of downgaze.

• Curtain sign, seesaw ptosis:– When the ptosis is asymmetric, the driving discharges

attempting to keep the more ptotic eyelid open are also transmitted, per Hering's law, to the less ptotic eyelid.

– Manually raising the more ptotic lid causes relaxation and the eye with less ptosis, sometimes even no ptosis, may suddenly crash.

Myasthenia Gravis

• Edrophonium (Camiston) test

2. Investigations

• Medical - anticholinesterases, steroids and azathioprine

3. Treatment options

• Antibodies to acetylcholine receptors• CT or MRI for presence of thymoma

• Electromyography to confirm fatigue

• Thymectomy

Ocular myasthenia

• Insidious, bilateral but asymmetrical• Worse with fatigue and in upgaze

Ptosis

• Ptotic lid may show ‘twitch’ and ‘hop’ signs

• Intermittent and usually vertical

Diplopia

Edrophonium (Tensilon) Test:• Edrophonium chloride inhibits acetylcholinesterase, • thereby prolonging the presence of acetylcholine at the neuromuscular

junction. • This results in enhanced muscle strength. • In ptosis, a positive test is the elevation of eyelids in 2-5 minutes post

adminstration of Tensilon.• A negative response is no improvement within 3 minutes. • the Tensilon test has a relatively low sensitivity, approximately 60% for MG. • False positive results occur in patients with

– Lambert-Eaton Myasthenic Syndrome (LEMS), – Amyothrophic Lateral Sclerosis (ALS), and – localized intracranial mass lesions.

• Edrophonium chloride can cause overactivation of the parasympathetic system, and cause unwanted side effects like fainting, dizziness, involuntary defecation, severe bradycardia, apnea, and even cardiac arrest. It is important to always have atropine at hand if such side effects should occur

Edrophonium test

• Measure amount of ptosis or diplopia before injection

• Inject i.v. atropine 0.3 mg

• Inject i.v. test dose of edrophonium (0.2 ml-2 mg)• Inject remaining (0.8 ml-8 mg) if no hypersensitivity

Before injection Positive result

Myotonic dystrophyFacial weakness and ptosis

• Involvement of tongue and pharyngeal muscles

• Ophthalmoplegia - uncommon

• Muscle wasting • Hypogonadism• Frontal baldness in males• Intellectual deterioration• Presenile stellate cataracts

Release of grip difficult

Ocular myopathies

• Isolated• Oculopharyngeal dystrophy• Kearns-Sayre syndrome (pigmentary retinopathy)

• Ptosis - slowly progressive and symmetrical• Ophthalmoplegia - slowly progressive and symmetrical (no diplopia)

Clinical types Ocular features

C. APONEUROTIC PTOSIS • It develops due to defects of the levator

aponeurosis in the presence of a normal functioning muscle.

• It includes – involutional (senile) ptosis, – postoperative ptosis • Ptosis due to aponeurotic weakness associated with

blepharochalasis (Blepharochalasis is an inflammation of the eyelid that is characterized by exacerbations and remissions of eyelid edema, which results in a stretching and subsequent atrophy of the eyelid tissue resulting in redundant folds over the lid margins)

– Posttraumatic dehiscence or disinsertion of the aponeurosis.

Aponeurotic ptosis• Weakness of levator aponeurosis• Causes - involutional, postoperative and blepharochalasis

High upper lid crease Good levator function

Absent upper lid crease Deep sulcus

Mild

Severe

Senile ptosis• Senile or involutional ptosis is very common.

Asymmetric lids and redundant lid tissue in the elderly.

• The levator aponeurosis attaches the levator muscle to the tarsal plate, which forms the eyelid.

• Aging may cause levator dehiscencedisinsertion (LDD)—with stretching, thinning, or detachment of the aponeurosis.

• Normally, with the eyelids gently closed, the upper lid margin lies 5 mm to 7 mm below the upper lid fold (the skin fold at the upper part of the lid).

• An increase in this distance suggests LDD.

D. Mechanical ptosis

• Due to excessive weight on the upper lid– lid tumours, – multiple chalazia – lid oedema.

• Cicatricial Ptosis– ocular pemphigoid– trachoma.

Mechanical ptosisCauses

Dermatochalasis Large tumours

Severe lid oedema Anterior orbital lesions

E. NEUROTOXIC PTOSIS

• Envenomation by elapids such as cobras, or kraits.– Bilateral ptosis is usually accompanied by diplopia,

dysphagia and/or progressive muscular paralysis. – Regardless, neurotoxic ptosis is a precursor to

respiratory failure and eventual suffocation caused by complete paralysis of the thoracic diaphragm.

– It is therefore a medical emergency and immediate treatment is required.

PSEUDOPTOSIS • Pseudoptosis is the appearance of ptosis in the

absence of levator abnormality. • Exclude pseudoptosis (simulated ptosis) on

inspection. • Its common causes are: – microphthalmos,– anophthalmos, – enophthalmos – Phthisis bulbi.– Double elevator palsy– Blepharospasm– Contralateral proptosis

• Blepharochalasis (dermatochalasis) – refers to age-related lax, baggy skin around the

eyelids; – it can also simulate ptosis but levator function is

normal• Duane's syndrome – the palpebral fissure narrows on ocular adduction

because of globe retraction causing dynamic enophthalmos.

Causes of pseudoptosis

Ipsilateral hypotropia Brow ptosis - excessive eyebrow skin

Dermatochalasis - excessiveeyelid skin

Lack of lid support Contralateral lid retraction

EVALUATION OF PTOSIS

HISTORY• Ptosis – Age of onset– Duration – One/both eye– Diurnal variability

• Associated history : – Diplopia – Dysphagia – Muscle weakness

• Vision

• Association with– Jaw movements– Abnormal ocular movements– Abnormal head posture

• History of– Trauma or previous surgery– Poisoning– Use of steroid drops– Any reaction with anesthesia– Bleeding tendency

• Previous photographs may prove to be of great help.• Is there a family history of ptosis or of other muscle

weakness?

Ocular Examination:NORMAL POSITION OF EYELIDS• The normal upper eyelid in primary position – crosses the iris between the limbus (junction of the iris

and sclera) and the pupil, – usually 1 mm to 2 mm below the limbus; – the lower lid touches or crosses slightly above the

limbus. – Normally there is no sclera showing above the iris.

• The palpebral fissures:– are normally 9 mm to 12 mm from upper to lower lid

margin.

Ptosis • U/L or B/L• Complete /incomplete• Total unilateral ptosis – complete third nerve palsy.

• Mild to moderate unilateral ptosis – Horner's syndrome, – partial third nerve palsy.

• Mild to moderate bilateral ptosis – neuromuscular disorders, such as MG, – muscular dystrophy, – Ocular myopathy.

• Head Posture: – chin elevation as the ptosis is minimum in downgaze in a patient

with congenital ptosis is also one of the indications for surgery especially in the pediatric age group.

– In ptosis there will be superior altitudinal defect which is corrected by elevating the eyelids

• Ocular Motility: – Importance in myogenic ptosis, – To R/O 3rd nerve palsy– presence of strabismus, especially vertical strabismus entails that it

be corrected prior to the correction of the ptosis.• Visual acuity – Best-corrected visual acuity should be assessed to record any

amblyopia if present, especially in cases of congenital ptosis.• Pupillary Examination: – TO diagnosis Horner’s syndrome – Involvement in a case of third nerve palsy

Measurements

1. Margin reflex distance2. Vertical fissure height3. LPS action4. Lid crease level5. Lid level on down gaze

1. MARGIN REFLEX DISTANCE

• Margin-to-reflex distance 1 (MRD1) : – When light is thrown on the cornea a reflection

occurrs .the distance from the central pupillary light reflex to the upper eyelid margin with the eye in primary gaze.

• NORMAL : 4 - 5 mm.• If the margin is above the light reflex the MRD 1 is a

+ve value. • If the lid margin is below the corneal reflex in cases

of very severe ptosis the MRD 1 would be a –ve value. 

Marginal reflex distance• Distance between upper lid margin and cornal light reflex (MRD)

• Mild ptosis (2 mm of droop)

• Moderate ptosis (3 mm)

• Severe ptosis (4 mm or more)

2. Vertical fissure height

• The distance between the upper and lower eyelid in vertical alignment with the center of the pupil in primary gaze, with the patient’s brow relaxed.

• Normal – 9-10mm in primary gaze• Should be seen in up gaze, down gaze and

primary gaze• Amount of ptosis = difference in palpebral

apertures in unilateral ptosis or Difference from normal in bilateral ptosis

Grading of severity of ptosis

< or = 2mm : mild ptosis= 3 mm : moderate ptosis= or > 4 mm : severe ptosis

3. Levator function assesment• It is determined by the lid excursion caused by LPS

muscle (Burke’s method). – Patient is asked to look down, and thumb of one hand is

placed firmly against the eyebrow of the patient (to block the action of frontalis muscle) by the examiner.

– Then the patient is asked to look up and the amount of upper lid excursion is measured with a ruler held in the other hand by the examiner.

– Levator function is graded as follows:• Normal 15 mm• Good 8 mm or more• Fair 5-7 mm• Poor 4 mm or less

• Reflects levator function

• Normal (15 mm or more)

• Good (8 mm or more)

• Fair (5-7 mm)

Upper lid excursion- burke’s ,method

• Poor (4 mm or less)

Upper lid crease

• Distance between lid margin and lid crease in down-gaze• Normals - females 10 mm; males 8 mm• Absence in congenital ptosis indicates poor levator function• High crease suggests an aponeurotic defect

• Distance between lash line and skin fold in primary position of gaze

Pretarsal show

crease fold

Ice test

Investigation

• Serum acetylcholine receptor assay• Tensilon test• EMG• ECG• ERG• T3, T4, TSH

Surgical management

Mild ptosis

Phenylephrine +ve

mullerectomy

Phenylephrine - ve

Fasanella servat

Blepharoplasty

Levator resection

Surgical management

ptosis

moderate

Levator resesction

Levator advancement

severe

Frontalis sling