Ptosis

Definition of eyelid ptosis: Abnormally low position of the upper eyelid margin caused by poor
function of the levator palpebrae superioris or Mllers muscle.
Paediatric ptosis see Chapter 23.
Actiology:
Involutional or aponeurotic (thinning or dehiscence of the anterior part of the levator, the
aponeurosis).There is good levator function (LF). Occurs commonly in the elderly.
Myogenic. LF is very poor. Includes the congenital dystrophic (fatty) levator muscles in
children and myopathies in adults, e.g. chronic progressive external ophthalmoplegia.
Neurogenic.The LF is usually poor, e.g. IIIrd nerve palsy.
Mechanical. Usually good LF. A tumour weighs the eyelid down, e.g. neurofibromatosis.
Traumatic ptosis may be aponeurotic, myogenic or neurogenic.
Symptoms
Cosmetically poor appearance.
Functionalreduces the visual field.
Management
Depends on the type of ptosis and LF. Normal or good LF is >10mm.Moderate LF is 5
10mm.Poor LF is <5mm.
Good and moderate LF: anterior levator resectiontuck / advance the levator
aponeurosis or muscle.
Poor LF: frontalis suspension with autogenous fascia lata.

Ptosis (drooping eyelid)

Congenital dystrophic.
Acquired third cranial nerve palsy (rare).
Inflammation, e.g. vernal keratoconjunctivitis.

Ptosis assessment
Measure the visual acuity, strength of the levator muscle in millimeters (levator function),
vertical palpebral aperture distance and skin crease height. Observe Bells phenomenon. Look for
aberrant eyelid movements with chewing and talking to detect Marcus Gunn jawwinking ptosis.
Check under the eyelid.
Type of surgery
This depends largely on the level of the levator function. If the levator function is very poor (<5
mm) the eyelid is internally suspended to the frontalis muscle (frontalis suspension). If there is
good levator function, the levator muscle is shortened (anterior levator resection, ALR). When
there is a risk of amblyopia in neonate because the lid covers the pupil most of the time, then
urgent frontalis suspension with a prolene suture is required. The child may later need
autogenous fascia lata frontalis suspension (where fascial strips are taken from their upper leg).
If the eyelid is only slightly drooping and the child can easily see out from beneath it by
adopting a small chin-up head position, there is less risk of amblyopia and the child can wait
until aged 4 years before having ptosis surgery.
6.1 Ptosis
Ptosis
Symptoms
Drooping upper eyelid, visual loss, especially with reading.
Signs
(See Figure 6.1.1.)
Critical. Drooping upper eyelid.
Other. Concerning associated signs include anisocoria, diplopia, ocular motility changes,
headache, or neck pain. See individual entities.
Etiology
Note
Although the vast majority of ptosis is of benign etiology, four entities must be ruled out in every
single case by careful examination:

Horner syndrome.

Third cranial nerve palsy (complete, partial, or aberrant III nerve regeneration).

Myasthenia gravis.

Superior eyelid or orbital malignancy.

Myogenic: Decreased levator function (<10mm), weak or absent eyelid crease.

Congenital levator dysgenesis may be associated with lagophthalmus in downgaze, poor
elevation in upgaze if associated with double elevator palsy; acquired myogenic ptosis
from localized or diffuse muscular dystrophy, chronic progressive external
ophthalmoplegia (CPEO), Kearns-Sayre syndrome, or oculopharyngeal dystrophy is
much less common.

Aponeurotic: High eyelid crease, moderate degree of ptosis (3 to 4 mm), good levator
function (10 to 15 mm), may worsen in downgaze. Levator stretching or dehiscence can
be due to normal aging changes, secondary to repetitive trauma in patients who rub eyes,
wear rigid contact lenses, or in patients with previous intraocular surgery.

Neurogenic: Third cranial nerve palsy (often complete ptosis, never an isolated
abnormality; congenital, compressive, vasculopathic, see 10.5, Isolated Third Nerve
Palsy); Horner syndrome (subtle upper and lower eyelid ptosis, see 10.2 Horner
Syndrome); myasthenia gravis (variable ptosis, worsens with fatigue, see 10.11,
Myasthenia Gravis); Marcus Gunn jaw winking syndrome (ptotic eyelid elevates with
jaw movement); ophthalmoplegic migraine; multiple sclerosis.

Mechanical: Retained contact lens in upper fornix; upper eyelid inflammation (chalazion,
giant papillary conjunctivitis, posttraumatic edema) or neoplasm.

Traumatic: History of eyelid laceration with levator transection, contusion injury to the
levator, tethering/ischemia within an orbital roof fracture, late dehiscence or cicatricial
changes.

Pseudoptosis: Contralateral eyelid retraction or proptosis, ipsilateral enophthalmos,

hypertropia, microphthalmus, phthisis bulbi,
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dermatochalasis, brow ptosis, chalazion or other eyelid tumor, eyelid edema,
blepharospasm, Duane syndrome.

Figure 6.1.1. Ptosis.

Work-Up

History: Determine onset and duration of ptosis. Present since birth? Acute onset? Old
photographs, a driver's license and family member's opinions are useful adjuncts to the
history. History of surgery in either eye? Orbital or eyelid trauma (orbital blow-out
fractures resulting in enophthalmos can mimic ptosis)? Variability with fatigue?
Associated with headache or neck pain? Any history of autoimmune disease (lupus,
Sjogren syndrome) or corneal abnormalities (which may predispose the patient to
postoperative exposure keratopathy).

Mandatory documentation: Must carefully check and document pupillary size and
extraocular motility, even if normal. If anisocoria is present, measurements should be
documented under light and dark conditions. Additional pharmacologic testing may be
indicated (see 10.1, Anisocoria). If extraocular muscle dysfunction is noted, additional
testing with prism bars may be indicated.

Complete orbital examination of both eyes: Measure and compare margin-reflex distance,
levator function (full upper eyelid excursion while preventing frontalis muscle
assistance), upper eyelid crease position of both eyes. Is there lagophthalmus? Associated
lower eyelid ptosis' (elevation of ipsilateral lower eyelid) is often seen in Horner
syndrome. Proptosis or eyelid lag may masquerade as contralateral ptosis. Exophthalmometry measurements are useful. Any sign of aberrant eyelid movements like jawwinking, variability and/or fatigue, orbicularis weakness, eyelid retraction with adduction
and/or infraduction? Palpate the superior orbit to rule out a mass or superior orbital rim
deformity.

Complete ocular examination: Determine if there are associated pupillary or extraocular

motility abnormalities. Flip upper eyelid to examine conjunctival surface and superior
fornix. Dilated fundus examination to look for pigmentary changes in adolescents and
young adults who present with ptosis, poor levator function, and external
ophthalmoplegia (i.e., possible Kearns-Sayre syndrome).

Corneal protective mechanisms. Document presence or absence of preoperative

lagophthalmos, orbicularis function, Bell phenomenon, and tear production. Check the
cornea carefully for any abnormalities or dystrophies which may predispose the patient to
postoperative keratopathy.

Other tests
o Ice test: Apply ice pack to ptotic eye for 2 minutes and reassess degree of
ptosis. Improvement with ice suggests myasthenia gravis.
o Neosynephrine test: Instill one drop 2.5% phenylephrine in the ptotic eye(s)
and reassess degree of ptosis. Patients with improvement of ptosis after 5 to 7
minutes may be good candidates for ptosis correction by an internal approach.
o Cocaine and hydroxyamphetamine tests. See 10.2, Horner Syndrome.

Imaging studies: In very select cases where a systemic or neurologic cause is suspected:
o Computed tomographic (CT) or magnetic resonance imaging (MRI) of orbit if
a superior orbital mass is suspected.
o Emergent CT/CTA or MRI/MRA of the head and neck if carotid artery
dissection is suspected in a patient with a painful Horner syndrome. Imaging of
the head alone is inadequate. See 10.2, Horner Syndrome.
o Emergent CT/CTA, MRI/MRA or conventional angiography if intracranial
aneurysm
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causing a third cranial nerve palsy with pupillary involvement or a partial third
cranial nerve palsy is suspected. See 10.5, Isolated Third Nerve Palsy.
o Chest CT if myasthenia gravis is suspected, to rule out thymoma. See 10.11,
Myasthenia Gravis.

Ancillary studies:
o Urgent EKG and cardiology consult if Kearns-Sayre syndrome is suspected.
Many of these patients can have a high degree heart block which can result in
sudden death.
o If myasthenia gravis is suspected, acetylcholine receptor antibody testing,
single fiber electromyography, and/or edrophonium chloride (e.g., Tensilon)
testing under monitored conditions may be indicated. See 10.11, Myasthenia
Gravis, and Pharmacopoeia.
o If severe dry-eye is found, consider an autoimmune work-up with serologies to
rule out lupus and Sjogren syndrome.

Treatment

Depends on underlying etiology (see 10.2, Horner Syndrome; 10.5, Isolated Third Nerve
Palsy; 10.11, Myasthenia Gravis).

Nonsurgical options: Observation. Taping and eyelid crutches attached to glasses in

neurogenic and myogenic ptosis. Management of chalazion with warm compresses
and/or excision, eyelid and/or orbital neoplasms with excision.

Surgical options: Based on initial evaluation and additional testing. Transcutaneous

levator advancement, transconjunctival levator advancement, frontalis muscle
suspension, Mller muscle resection.

Follow-Up

Congenital: Close follow-up is required to monitor for the presence of occlusion

amblyopia and exposure keratopathy

Traumatic: Observation for 6 months before considering surgical intervention.

Neurologic: Reevaluate based on particular entity.

Postoperative (after ptosis repair). Acute: monitor for infection and hemorrhage.
Subacute: Monitor for exposure keratopathy and for asymmetry that may require

postoperative readjustment. Mild lagophthalmos is common for 2 to 3 weeks after

surgical repair and usually resolves. Chronic: Monitor for ptosis recurrence and exposure
keratopathy.