BASICS

DESCRIPTION

• Drooping of the upper eyelid

– May be congenital or acquired

– May cause blurred vision or loss of visual field

EPIDEMIOLOGY

Unknown

RISK FACTORS

See associated conditions

Genetics

• Most commonly idiopathic

• May be inherited as autosomal dominant trait

• Familial occurrences suggest that genetic or chromosomal defects are likely, although yet unknown.

PATHOPHYSIOLOGY

• Aponeurotic: levator stretching or dehiscence (1)

• Myogenic: primary or secondary levator and/or Muller’s muscle abnormality

• Neurogenic: damage to nerves innervating the levator and/or Muller’s muscle

• Mechanical

• Traumatic: damage to eyelid retractors

ETIOLOGY

• Congenital:

– Myogenic: levator dysgenesis

– Blepharophimosis

– Neurogenic: synkinetic, Horner’s syndrome, cranial nerve (CN) III palsy

• Acquired:

– Aponeurotic: normal aging changes, repetitive trauma in patients who rub eyes, contact lens wearers, patients with previous intraocular surgery

– Myogenic: myasthenia gravis, chronic progressive external ophthalmoplegia (CPEO), myotonic dystrophy, oculopharyngeal dystrophy

– Neurogenic: CN III palsy, Horner’s syndrome

– Mechanical

– Traumatic: history of eyelid laceration with levator transection, contusion injury to the levator

– Reactive: due to ocular or orbital inflammation

COMMONLY ASSOCIATED CONDITIONS

• CN III palsy

– Congenital, compressive, vasculopathic, traumatic

• Horner’s syndrome

• Myasthenia gravis

• Other: Marcus Gunn jaw winking syndrome, ophthalmoplegic migraine, multiple sclerosis

DIAGNOSIS

HISTORY

• Determine the onset and duration of ptosis.

• History of surgery in either eye?

• Symptoms of ocular irritation?

• Orbital or eyelid trauma?

• Variability with fatigue?

• Associated with headache or neck pain?

• Associated diplopia?

• History of autoimmune disease (lupus, Sjögren’s syndrome)?

PHYSICAL EXAM

• Visual acuity

• External

– Position of upper and lower eyelid margins (primary, up and down gaze)

– Levator function

– Presence and position of eyelid crease

– Amount of dermatochalasis, position of eyebrows

– Preexisting eyelid scars

– Evert upper eyelid

– Orbital exam: palpable masses or proptosis?

• Pupils

– Irregular, miosis or mydriasis

• Motility

– Ductions and versions

– Bell’s phenomenon

– Strabismus or diplopia

• Anterior segment

– Conjunctival inflammation, filtering blebs

– Tear film

– Keratopathy

– Anterior segment inflammation

• Retina

• Pigmentary changes

DIAGNOSTIC TESTS & INTERPRETATION

Lab

Acetylcholine receptor antibody testing and/or edrophonium chloride (Tensilon) testing under monitored conditions are recommended in cases of suspected myasthenia gravis.

Imaging

• Suspected orbital mass: orbital CT or MRI

• Painful Horner’s syndrome: rule out carotid artery dissection with emergent CT/CT angiography (CTA) or MRI/magnetic resonance angiography (MRA) of the head/neck.

• CN III palsy (partial or pupil involving): rule out intracranial aneurysm with emergent CT/CTA, MRI/MRA, or angiography.

Diagnostic Procedures/Other

• Visual fields to determine functional impact

• Clinical photographs

• Ice test

– Improvement of ptosis with ice suggests myasthenia gravis.

• Cocaine and/or hydroxyamphetamine tests

– In cases of suspected Horner’s syndrome

• Single fiber electromyography (EMG)

– In cases of suspected myasthenia gravis

Pathological Findings

• Congenital ptosis

– Underdeveloped levator muscle, may be infiltrated by fatty tissue

• Aponeurotic ptosis

– Thinning, lengthening, and/or disinsertion of the levator aponeurosis from the tarsal plate

• Myogenic ptosis

– Ragged red fibers in CPEO

DIFFERENTIAL DIAGNOSIS

• Contralateral upper lid retraction

• Ipsilateral exophthalmos

• Small globe: microphthalmos, phthisis bulbi

• Dermatochalasis

• Brow ptosis

• Eyelid edema

TREATMENT

MEDICATION

• Myasthenia gravis: treatment of primary disorder will improve ocular symptoms

– Pyridostigmine, prednisone, intravenous immunoglobulin (IVIG), steroid-sparing agents

ADDITIONAL TREATMENT

General Measures

• Observation

• Management of chalazion and eyelid and/or orbital neoplasms with excision

• Treatment of ocular irritation in reactive ptosis

• Traumatic ptosis should be observed for spontaneous improvement for at least 6 months.

Issues for Referral

• Congenital ptosis

– Referral to pediatric ophthalmologist for monitoring/management of possible amblyopia and/or strabismus

• Neurogenic ptosis

– Referral to neuro-ophthalmologist, neurologist, and/or neurosurgeon

COMPLEMENTARY & ALTERNATIVE THERAPIES

Taping and eyelid crutches attached to glasses

SURGERY/OTHER PROCEDURES

• External levator resection/advancement

• Internal levator resection

• Frontalis sling procedure: for patients with poor levator function

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

See issues for referral.

Patient Monitoring

Congenital ptosis: see issues for referral.

PROGNOSIS

Dependent of etiology

COMPLICATIONS

• After surgical repair:

– Orbital hemorrhage

– Under-/overcorrections

– Eyelid asymmetry

– Infection

– Dry eye syndrome

– Corneal exposure/lagophthalmos

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