What is the recommended evaluation and management approach for a patient presenting with ptosis?

Evaluation and Management of Ptosis

The evaluation of ptosis must immediately distinguish life-threatening neurogenic causes (pupil-involving third nerve palsy from aneurysm, Horner's syndrome from carotid dissection) from benign etiologies through systematic pupillary examination, assessment of variability with fatigue, and measurement of levator function. 1

Immediate Triage: Rule Out Emergencies First

Pupillary Examination (Most Critical Step)

Pupil-involving ptosis with mydriasis requires emergent neuroimaging (MRI/MRA or CTA) within hours to exclude posterior communicating artery aneurysm, which can be fatal if ruptured. 1, 2

• Complete pupil-sparing (normal pupil) with complete ptosis AND complete ophthalmoplegia suggests microvascular third nerve palsy in patients with diabetes, hypertension, or hyperlipidemia 1
• However, incomplete ptosis or partial ophthalmoplegia with pupil-sparing cannot be assumed benign—compressive lesions (tumors, aneurysms) can present this way and require urgent MRI with gadolinium plus MRA or CTA 1
• Miosis with ptosis indicates Horner's syndrome, suggesting internal carotid dissection until proven otherwise—requires urgent vascular imaging 3, 1

Variability Assessment (Second Critical Step)

Variable, fatigable ptosis that worsens with prolonged upgaze and improves with rest is pathognomonic for myasthenia gravis. 1, 4

• Perform the ice pack test: apply ice over closed eyes for 2 minutes—reduction of ptosis by approximately 2 mm is highly specific for myasthenia gravis 1, 4, 2
• Look for Cogan lid-twitch sign (brief overshoot of eyelid on refixation from downgaze to primary position) and slow saccades 1, 4
• Strabismus pattern may change entirely during prolonged examination due to fatigue 4
• 50-80% of patients with ocular myasthenia develop life-threatening generalized myasthenia within a few years—refer to neurology for acetylcholine receptor antibodies (positive in 40-77% of ocular cases) and chest CT to screen for thymoma 4, 1

Systematic Clinical Examination

History

• Daily variation suggests myasthenia gravis 3
• Acute onset with headache suggests aneurysm or carotid dissection 1
• Trauma history indicates traumatic or aponeurotic disruption 5, 6
• Contact lens wear with lid swelling suggests giant papillary conjunctivitis 1
• Obesity and sleep apnea suggest floppy eyelid syndrome 1

Levator Function Measurement

Measure excursion of upper lid from extreme downgaze to extreme upgaze (normal >15 mm) 3, 7:

• Good function (>10 mm): suggests aponeurotic ptosis (most common, 60% of cases) 6
• Moderate function (5-10 mm): suggests congenital dysgenesis or chronic myopathy 7, 8
• Poor function (<5 mm): suggests severe congenital ptosis or chronic progressive external ophthalmoplegia 7, 8

Bell's Phenomenon

Absence of Bell's phenomenon (eye does not roll upward with forced lid closure) predicts postoperative corneal exposure—critical for surgical planning 3

Associated Findings

• Proptosis with ptosis: obtain MRI orbits with contrast to evaluate for orbital mass, thyroid eye disease, or vascular malformation 4, 1
• Strabismus with ptosis: consider thyroid eye disease (inferior/medial rectus restriction), myasthenia gravis (variable), or third nerve palsy 4
• Enlarged superior fornix with mucopurulent material in elderly women suggests giant fornix syndrome 1

Imaging Algorithm

Urgent Neuroimaging Indications

Order MRI brain/orbits with gadolinium PLUS MRA or CTA emergently for: 1, 9

• Any pupil involvement (mydriasis)
• Pupil-sparing with incomplete ptosis or partial ophthalmoplegia
• Horner's syndrome (miosis with ptosis)
• Acute onset with severe headache
• Progressive neurological deficits

Elective Imaging Indications

Order MRI orbits with contrast for: 4, 1

• Proptosis with ptosis
• Suspected orbital mass or thyroid eye disease
• Mechanical ptosis from unclear etiology

Isolated aponeurotic ptosis in elderly patients typically does not require neuroimaging. 9, 5

Classification and Management

Aponeurotic Ptosis (60% of cases)

• Most common in elderly patients 6
• Good levator function (>10 mm) 5
• Surgical repair: levator advancement or Müller's muscle-conjunctival resection 7

Neurogenic Ptosis

• Third nerve palsy: managed per algorithm above—microvascular cases typically resolve in 3-6 months with observation 1
• Horner's syndrome: requires evaluation for carotid dissection, apical lung tumor, or brainstem lesion 5

Myogenic Ptosis

• Myasthenia gravis: refer to neurology for immunosuppression, acetylcholinesterase inhibitors, and thymectomy consideration 4, 1
• Chronic progressive external ophthalmoplegia: frontalis suspension surgery 6

Congenital Ptosis

• Surgical correction between ages 3-5 years to prevent amblyopia 8, 9
• Levator resection if function >3 mm; frontalis suspension if function <5 mm 7, 8

Mechanical Ptosis

• Treat underlying cause (tumor resection, management of thyroid eye disease, treatment of giant papillary conjunctivitis) 1

Critical Pitfalls to Avoid

• Never assume pupil-sparing indicates benign disease when ptosis is incomplete or ophthalmoplegia is partial—these patients require urgent neuroimaging 1
• Missing myasthenia gravis due to variable presentation—always perform ice pack test when ptosis varies 1, 2
• Failing to screen for thymoma in myasthenia patients—10-15% have thymoma requiring surgical resection 1
• Delaying amblyopia treatment in children with congenital ptosis—leads to permanently poor vision 9, 7
• Operating on thyroid eye disease before orbital decompression—decompression changes alignment and may worsen strabismus 4