Ophthalmology – Ptosis

Basics

Description

Ptosis (blepharoptosis) is abnormal drooping of the upper eyelid caused by dysfunction of the eyelid-elevating apparatus.

It may be:

• Congenital
• Acquired
• Unilateral or bilateral

Ptosis can cause:

• Superior visual field loss
• Reduced central vision if severe
• Astigmatism
• Abnormal head posture
• Amblyopia in children

The key clinical task is to determine:

Is the ptosis aponeurotic, myogenic, neurogenic, mechanical, traumatic, or pseudoptosis?

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Eyelid Elevators

The upper eyelid is elevated primarily by:

Levator Palpebrae Superioris

• Innervated by CN III
• Provides most upper eyelid elevation

Müller Muscle

• Sympathetically innervated
• Contributes approximately 1–2 mm of elevation

Frontalis Muscle

• Innervated by CN VII
• Can compensate by elevating the eyebrow

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Classification

The major categories are:

• Aponeurotic
• Myogenic
• Neurogenic
• Mechanical
• Traumatic
• Congenital developmental
• Pseudoptosis

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Aponeurotic Ptosis

Aponeurotic/involutional ptosis is the most common acquired form in adults.

It results from:

• Stretching
• Thinning
• Dehiscence
• Disinsertion

of the levator aponeurosis.

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Risk Factors for Aponeurotic Ptosis

Associations include:

• Aging
• Previous intraocular surgery
• Long-term contact lens wear
• Chronic eye rubbing
• Repeated eyelid manipulation
• Trauma

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Typical Aponeurotic Examination

Classic findings include:

• Mild–moderate ptosis
• Good levator function
• High or absent upper lid crease
• Deep superior sulcus
• Lid lag may be absent
• Ptosis may appear more pronounced in downgaze

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Congenital Ptosis

Most simple congenital ptosis results from:

Levator muscle dysgenesis

with replacement of normal muscle by:

• Fibrous tissue
• Fatty tissue

This causes both:

• Reduced elevation
• Reduced relaxation

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Congenital Examination

Typical findings include:

• Ptosis present from birth or infancy
• Poor levator function
• Weak or absent eyelid crease
• Lid lag in downgaze
• Possible lagophthalmos
• Compensatory brow elevation
• Chin-up head posture

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Pediatric Importance

Congenital ptosis can cause amblyopia through:

• Visual-axis occlusion
• Induced astigmatism
• Anisometropia
• Associated strabismus

Every child with ptosis requires:

• Cycloplegic refraction
• Amblyopia assessment
• Strabismus examination
• Visual-axis assessment

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Timing of Congenital Ptosis Surgery

If the eyelid obstructs the visual axis or produces significant abnormal head posture:

Early surgery may be necessary to prevent amblyopia.

If there is no amblyopia risk, surgery can often be delayed until:

• Later preschool years

when measurements and postoperative cooperation are easier.

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Myogenic Ptosis

Myogenic ptosis results from intrinsic dysfunction of the levator or related skeletal muscle.

Causes include:

• Myasthenia gravis
• Chronic progressive external ophthalmoplegia
• Oculopharyngeal muscular dystrophy
• Myotonic dystrophy
• Other mitochondrial or muscular disorders

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Myasthenia Gravis

Myasthenia is an essential cause of:

Variable or fluctuating ptosis

Typical features include:

• Ptosis worsens with fatigue
• Improvement after rest
• Variable diplopia
• Orbicularis weakness
• Cogan lid twitch
• Enhancement of ptosis
• Pupils remain normal

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Myasthenia Pearl

A pupil abnormality should make isolated ocular myasthenia:

Much less likely

because the autonomic pupil is typically spared.

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Ice-Pack Test

An ice-pack test may support ocular myasthenia.

After several minutes of cooling the eyelid:

• Improvement in ptosis of approximately 2 mm or more

supports the diagnosis.

It is:

• Simple
• Noninvasive
• Particularly useful in ptosis-predominant disease

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Laboratory Testing for Myasthenia

Consider:

• AChR antibodies
• MuSK antibodies in selected seronegative generalized cases
• Other antibody testing depending on clinical context

Electrophysiology may include:

• Repetitive nerve stimulation
• Single-fiber EMG, which is highly sensitive

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Important Modern Correction – Edrophonium

The historical:

Edrophonium (Tensilon) test

is now rarely used because:

• Availability is limited
• Cardiac/cholinergic adverse effects are possible
• Safer diagnostic alternatives exist

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Chronic Progressive External Ophthalmoplegia

CPEO typically causes:

• Slowly progressive bilateral ptosis
• Symmetric ophthalmoplegia
• Little diplopia despite marked motility restriction

because progression is gradual and symmetric.

It is often associated with:

Mitochondrial disease.

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Oculopharyngeal Muscular Dystrophy

Typically presents in later adulthood with:

• Bilateral ptosis
• Dysphagia

Family history may be present.

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Myotonic Dystrophy

May produce:

• Bilateral ptosis
• Orbicularis weakness
• Ophthalmoplegia
• Christmas-tree cataract
• Systemic myotonia

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Neurogenic Ptosis

Major causes include:

• Third cranial nerve palsy
• Horner syndrome
• Rare central neurologic disease

These are especially important in acute ptosis.

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Third Nerve Palsy

CN III innervates:

• Levator palpebrae
• Superior rectus
• Inferior rectus
• Medial rectus
• Inferior oblique
• Parasympathetic pupillary fibers

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Classic Third Nerve Palsy

Findings may include:

• Marked or complete ptosis
• Eye positioned “down and out”
• Adduction deficit
• Elevation deficit
• Depression deficit
• Diplopia
• Possible dilated pupil

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Pupil-Involving Third Nerve Palsy

An acute third nerve palsy with:

• Mydriasis
• Pain
• Partial ophthalmoplegia

must raise concern for:

Posterior communicating artery aneurysm

and requires urgent vascular imaging.

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Modern Imaging Principle for Third Nerve Palsy

Because aneurysms can occasionally present atypically:

Acute acquired third nerve palsy generally warrants urgent neurovascular imaging, particularly if:

• Pupil is involved
• Palsy is partial
• Severe headache/pain is present
• Patient is young
• Pattern is atypical

Preferred studies include:

• CTA
• MRA

with catheter angiography reserved for selected cases.

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Horner Syndrome

Horner syndrome results from interruption of the sympathetic pathway.

Classic findings include:

• Mild upper eyelid ptosis
• Miosis
• Lower eyelid elevation (“reverse ptosis”)
• Apparent enophthalmos
• Possible facial anhidrosis depending on lesion location

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Horner Ptosis

Because Müller muscle contributes only a small amount of lid elevation, Horner ptosis is usually:

Mild

rather than complete.

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Congenital Horner Syndrome

Congenital or long-standing early childhood Horner syndrome may produce:

Iris heterochromia

with the affected iris appearing lighter.

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Painful Horner Syndrome

Acute Horner syndrome associated with:

• Ipsilateral neck pain
• Facial pain
• Headache

should be considered:

Internal carotid artery dissection until proven otherwise.

Urgent:

• CTA head/neck
• MRA head/neck

is indicated.

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Pharmacologic Testing for Horner Syndrome

Modern confirmation usually uses:

Apraclonidine

because denervation supersensitivity produces:

• Dilation of the affected pupil
• Improvement of mild ptosis

The anisocoria may reverse after testing.

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Important Modern Correction – Cocaine/Hydroxyamphetamine

Older testing used:

• Cocaine
• Hydroxyamphetamine

These are now much less commonly used because:

• Availability is limited
• Apraclonidine is simpler
• Localization is increasingly performed with imaging rather than pharmacologic hydroxyamphetamine testing

Use caution with apraclonidine in:

Very young infants

because systemic CNS and cardiovascular adverse effects can occur.

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Marcus Gunn Jaw-Winking Syndrome

This congenital synkinesis causes:

Upper eyelid elevation with jaw movement

such as:

• Chewing
• Sucking
• Moving jaw to opposite side

It results from aberrant innervation between:

• Trigeminal motor pathways
• Levator palpebrae

It is often associated with congenital ptosis.

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Blepharophimosis Syndrome

The classic BPES phenotype includes:

• Bilateral ptosis
• Blepharophimosis
• Epicanthus inversus
• Telecanthus

It is commonly associated with:

FOXL2

mutations and follows an autosomal dominant pattern.

Some forms are associated with:

• Premature ovarian insufficiency

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Mechanical Ptosis

Mechanical ptosis occurs when excess weight or structural abnormality pulls the lid downward.

Causes include:

• Eyelid tumor
• Chalazion
• Eyelid edema
• Amyloid deposition
• Neurofibroma
• Scar
• Severe dermatochalasis
• Giant papillary conjunctivitis

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Traumatic Ptosis

Trauma can damage:

• Levator muscle
• Levator aponeurosis
• CN III
• Sympathetic fibers

Mechanism may include:

• Laceration
• Contusion
• Orbital injury

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Post-Traumatic Observation

Some blunt-trauma ptosis improves spontaneously as:

• Edema resolves
• Nerve function recovers
• Muscle injury heals

Definitive surgery is often delayed when reasonable, but:

A fixed six-month waiting period is not required in every case.

Repair may be earlier when there is:

• Clear levator transection
• Significant laceration
• Visual-axis obstruction
• Little expectation of spontaneous recovery

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Reactive Ptosis

Painful ocular disease may produce temporary ptosis from:

• Reflex orbicularis activation
• Inflammation
• Swelling

Examples include:

• Corneal abrasion
• Uveitis
• Orbital inflammation

Treating the underlying disorder usually improves the lid position.

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Pseudoptosis

Not all apparent ptosis represents true upper eyelid elevator dysfunction.

Causes of pseudoptosis include:

• Dermatochalasis
• Brow ptosis
• Contralateral upper lid retraction
• Enophthalmos
• Microphthalmos
• Phthisis bulbi
• Hypotropia
• Small or recessed globe

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History

Important questions include:

• Congenital or acquired?
• Acute or gradual?
• Constant or fluctuating?
• Worse late in day?
• Associated diplopia?
• Pupil change?
• Headache or neck pain?
• Previous ocular surgery?
• Trauma?
• Contact lens wear?
• Difficulty swallowing or generalized weakness?
• Family history?

Old photographs are particularly useful for determining:

Chronicity.

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Examination – Core Measurements

A formal ptosis examination should document:

• MRD1
• MRD2
• Palpebral fissure height
• Levator function
• Upper lid crease height
• Brow position
• Lagophthalmos
• Bell phenomenon

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Margin Reflex Distance 1

MRD1 is the distance between:

• Central corneal light reflex
• Upper eyelid margin

in primary gaze.

Normal MRD1 is approximately:

4–5 mm

although normal values vary.

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Ptosis Severity by MRD1

Approximate clinical description:

• Mild: ~2 mm droop
• Moderate: ~3 mm
• Severe: ≥4 mm or pupil covered

The actual surgical decision depends on:

• Levator function
• Etiology
• Visual function

not simply droop magnitude.

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Levator Function

Measured by:

• Stabilizing the brow to eliminate frontalis action
• Measuring upper lid excursion from downgaze to upgaze

Approximate interpretation:

• Good: ≥12 mm
• Fair: ~5–11 mm
• Poor: ≤4 mm

These categories guide surgical choice.

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Lid Crease

A:

High lid crease + good levator function

strongly suggests:

Aponeurotic ptosis.

A weak or absent crease with poor levator function favors:

Congenital myogenic ptosis.

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Brow Examination

Patients may compensate for ptosis by:

• Elevating eyebrows
• Wrinkling forehead

The brow should therefore be manually relaxed when evaluating true eyelid position.

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Pupils

Pupil examination is mandatory in:

Any new ptosis.

Look for:

• Miosis → Horner syndrome
• Mydriasis → CN III palsy
• Anisocoria pattern in light vs dark

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Ocular Motility

Assess:

• Ductions
• Versions
• Alignment
• Diplopia

Ptosis associated with ophthalmoplegia strongly suggests:

• CN III palsy
• Myasthenia
• CPEO
• Orbital disease

rather than simple aponeurotic ptosis.

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Bell Phenomenon

Assess Bell phenomenon before surgery.

Poor Bell phenomenon increases the risk of:

Postoperative exposure keratopathy

especially after aggressive elevation.

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Corneal Sensation and Ocular Surface

Assess:

• Tear film
• Corneal sensation
• Exposure
• Dry eye
• Lagophthalmos

because ptosis correction may worsen:

• Exposure keratopathy
• Dry eye symptoms

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Upper Lid Eversion

Evert the upper lid when mechanical disease is possible.

Look for:

• Foreign body
• Giant papillary conjunctivitis
• Mass
• Scar

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Hering’s Law

Elevation of the upper eyelids is bilaterally linked through central innervation.

In unilateral ptosis, the brain may increase levator drive to both eyes.

After lifting the ptotic lid, the fellow lid may:

Drop

revealing previously masked bilateral ptosis.

This is the:

Hering phenomenon

and is important for surgical planning.

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Phenylephrine Test

Topical phenylephrine stimulates Müller muscle.

Improvement of ptosis can help identify patients who may respond to:

Müller muscle–conjunctival resection (MMCR)

It also helps estimate postoperative eyelid position in selected cases.

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Visual Field Testing

Functional visual fields may document:

• Superior field loss

from ptosis.

Testing may be performed:

• With eyelid in natural position
• With lid taped/elevated

to demonstrate functional improvement.

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Clinical Photography

Standardized external photographs are useful for:

• Baseline documentation
• Surgical planning
• Insurance/functional documentation
• Postoperative comparison

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Imaging

Imaging is not required for typical chronic aponeurotic or congenital ptosis.

Obtain imaging when the history or examination suggests:

• Orbital mass
• Neurologic lesion
• CN III palsy
• Horner syndrome
• Trauma

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Orbital Mass

Consider:

• CT orbit
• MRI orbit

when there is:

• Proptosis
• Globe displacement
• Palpable mass
• Progressive unilateral mechanical ptosis
• Motility restriction

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Differential Diagnosis

Important differential diagnoses include:

• Dermatochalasis
• Brow ptosis
• Contralateral lid retraction
• Enophthalmos
• Hypotropia
• Microphthalmos
• Phthisis bulbi
• Eyelid edema

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Treatment Principles

Treatment depends on:

• Etiology
• Severity
• Visual impairment
• Levator function
• Ocular surface status
• Patient goals

Before surgery:

Treat the underlying neurologic, muscular, inflammatory, or mechanical cause whenever possible.

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Observation

Observation is appropriate for:

• Mild stable ptosis
• No visual field impairment
• No amblyopia risk
• Acceptable cosmesis

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Medical Treatment – Myasthenia

Treatment may include:

• Pyridostigmine
• Corticosteroids
• Steroid-sparing immunosuppressants
• IVIG or plasma exchange in selected severe disease
• Targeted biologic therapy in appropriate generalized disease

Ptosis surgery is usually avoided until ocular myasthenia is:

Stable and medically optimized.

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Acquired Ptosis Medication

In selected adults with acquired ptosis, topical:

Oxymetazoline 0.1%

can temporarily elevate the upper eyelid by stimulating Müller muscle.

It is most useful in:

• Mild acquired ptosis

It does not correct:

• Severe levator dysfunction
• Mechanical ptosis
• Major neurologic disease

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Eyelid Crutch

A spectacle-mounted eyelid crutch may be considered in patients who:

• Are poor surgical candidates
• Have neuromuscular disease
• Need temporary mechanical elevation

Potential problems include:

• Dry eye
• Exposure
• Discomfort

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Surgical Treatment

Main surgical approaches include:

• External levator advancement/resection
• Müller muscle–conjunctival resection
• Frontalis suspension

Choice depends primarily on:

• Etiology
• Levator function
• Degree of ptosis

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External Levator Advancement

Best suited for:

Aponeurotic ptosis with good levator function

The levator aponeurosis is:

• Reattached
• Advanced

onto the tarsal plate.

This is a standard procedure for involutional ptosis.

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Levator Resection

Levator resection may be used in:

• Congenital ptosis
• Fair to good levator function

The amount of resection is adjusted according to:

• Ptosis severity
• Levator function

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Müller Muscle–Conjunctival Resection

MMCR is particularly useful for:

• Mild–moderate ptosis
• Good levator function
• Good response to phenylephrine

Advantages include:

• Posterior approach
• No external skin incision
• Predictable contour in selected patients

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Fasanella-Servat Procedure

Historical posterior lamellar shortening procedures remain available but are:

Less commonly emphasized than modern MMCR techniques.

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Frontalis Sling

Frontalis suspension is usually used when levator function is:

Poor

especially in:

• Severe congenital ptosis
• Selected neuromuscular disorders

The eyelid is linked to the frontalis muscle so brow elevation raises the lid.

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Frontalis Sling Materials

Options include:

• Autologous fascia lata
• Silicone rod
• Other synthetic materials

Selection depends on:

• Age
• Etiology
• Surgeon preference

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Exposure Risk After Surgery

All ptosis surgery carries a risk of:

• Lagophthalmos
• Exposure keratopathy

Risk is particularly important with:

• Poor Bell phenomenon
• Reduced corneal sensation
• Severe dry eye
• CPEO
• Aggressive frontalis sling

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Postoperative Complications

Potential complications include:

• Undercorrection
• Overcorrection
• Asymmetry
• Abnormal contour
• Lagophthalmos
• Exposure keratopathy
• Dry eye
• Infection
• Hemorrhage
• Recurrence
• Need for revision

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Congenital Ptosis Prognosis

Visual prognosis is good when:

• Amblyopia is prevented
• Refractive error is corrected
• Strabismus is treated
• Visual axis remains clear

Cosmetic symmetry can often be significantly improved but may not be perfect.

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Acquired Aponeurotic Ptosis Prognosis

Surgical prognosis is generally:

Excellent

with appropriate patient selection.

Some patients develop:

• Recurrent aponeurotic stretching
• Contralateral ptosis
• Residual asymmetry

over time.

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Emergency Ptosis Red Flags

Urgent evaluation is required for:

• Acute ptosis + dilated pupil
• Acute ptosis + ophthalmoplegia
• Painful acute Horner syndrome
• Acute ptosis with severe headache
• Ptosis with new neurologic deficits
• Rapidly progressive orbital signs
• Ptosis with generalized weakness or respiratory symptoms suggesting myasthenic crisis

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Ophthalmology Pearls

• Ptosis is classified as aponeurotic, myogenic, neurogenic, mechanical, traumatic, congenital, or pseudoptosis.
• Involutional/aponeurotic ptosis is the most common acquired form and typically shows good levator function with a high lid crease.
• Simple congenital ptosis usually reflects levator dysgenesis, causing poor levator function and lid lag in downgaze.
• Children with ptosis must be evaluated for amblyopia, astigmatism, anisometropia, strabismus, and visual-axis occlusion.
• Always examine pupils and ocular motility in any acute ptosis.
• Acute CN III palsy, especially with mydriasis, pain, or partial ophthalmoplegia, requires urgent vascular imaging for aneurysm.
• Painful acute Horner syndrome should be considered carotid dissection until proven otherwise.
• Horner syndrome produces mild ptosis + miosis, while CN III palsy may produce severe ptosis with a “down-and-out” eye and possible mydriasis.
• Variable fatigable ptosis with normal pupils suggests myasthenia gravis; the ice-pack test and AChR antibody testing are useful modern investigations.
• Edrophonium/Tensilon testing is now largely historical.
• Apraclonidine is the common modern pharmacologic confirmation test for Horner syndrome, although caution is required in infants.
• Measure MRD1, levator function, lid crease, brow position, Bell phenomenon, and lagophthalmos before planning surgery.
• A high lid crease with good levator function favors aponeurotic ptosis.
• Poor levator function generally favors a frontalis sling, whereas aponeurotic ptosis with good function is usually treated with levator advancement.
• MMCR is useful for selected mild–moderate ptosis with good levator function and a favorable phenylephrine response.
• Consider Hering’s law: correcting one ptotic lid may reveal previously masked ptosis of the fellow eye.
• The major postoperative concern is exposure keratopathy, particularly in patients with poor Bell phenomenon or ocular surface disease.