Treatment plan for ptosis

1. Assessment

• Measure: margin-reflex distance 1 (MRD1), levator function (excursion), upper lid crease position, and any lid lag or jaw-winking.
• Check for amblyopia and abnormal head posture in children. The Wills Eye Manual advises observing only if the ptosis is mild, there is no amblyopia, and no abnormal head position.
• Look for a neurologic or systemic cause:
  • Ice test: improvement suggests myasthenia gravis.
  • Phenylephrine 2.5% test: improvement after 5 to 7 minutes suggests the patient is a good candidate for Müller muscle-conjunctival resection.
  • Apraclonidine test: used for Horner syndrome.
• Imaging (Wills Eye Manual):
  • Horner syndrome: CT/CTA or MRI/MRA of the head and neck, done emergently if carotid dissection is suspected. Add a chest CT for an apical lung mass.
  • Third nerve palsy: emergent vascular imaging to exclude a posterior communicating artery aneurysm.
  • Myasthenia gravis: chest CT to look for thymoma.
  • Kearns-Sayre syndrome: urgent ECG and cardiology consult because of the risk of heart block.

2. Treat the underlying cause first

• Myasthenia gravis, Horner syndrome, third nerve palsy, orbital or lid mass, chalazion: manage the primary condition.
• Mechanical ptosis from dermatochalasis, tumour, scar or oedema is treated by removing the cause.
• Neurogenic and myogenic ptosis, nonsurgical: observation, taping the upper lids open, or eyelid crutches attached to glasses (Wills Eye Manual).

3. Surgical choice by levator function (Kanski, 10th ed.)

4. Treatment by type

• Simple congenital ptosis: frontalis suspension if levator function is poor, or levator resection if it is moderate or normal. Reassess every 3 to 12 months while observing, to watch for amblyopia.
• Blepharophimosis syndrome: the ptosis must be repaired, using frontalis suspension. Severe telecanthus may need separate surgery.
• Marcus Gunn jaw-winking:
  • Mild cases need no treatment, and the winking often improves around school age.
  • Mild cases with levator function of at least 5 mm can have unilateral levator advancement.
  • Moderate cases need levator disinsertion plus brow suspension, bilateral if a symmetrical result is wanted.
  • Plain levator resection increases the winking.
• Third nerve misdirection and aberrant facial nerve regeneration: levator disinsertion with brow suspension.
• Involutional (aponeurotic) ptosis: this is the most common acquired type. It shows good levator function, a high lid crease and a thin lid above the tarsus. Treat with levator advancement or reinsertion, or anterior levator repair. It can worsen toward the end of the day, so it can be confused with myasthenia gravis.
• Adults, general: the Family Medicine textbook describes congenital ptosis as corrected with a brow suspension (fascia lata) or levator resection when levator function is good, and adult ptosis as treated surgically by cause.

5. Follow-up

Recent evidence

Approach to ptosis, with and without associated anomalies

Step 1: Confirm true ptosis and exclude pseudoptosis

• Lack of globe support: artificial eye, microphthalmos, phthisis, enophthalmos.
• Contralateral lid retraction.
• Ipsilateral hypotropia. The ptosis disappears when the hypotropic eye fixates after you cover the normal eye.
• Brow ptosis or seventh nerve palsy. Lift the brow manually to check.
• Dermatochalasis.

Step 2: History

• Onset: congenital or acquired. Old photographs (for example a driver's license) help.
• Variability: worse with fatigue or at the end of the day suggests myasthenia gravis. Involutional ptosis can also worsen late in the day.
• Associated symptoms: diplopia, headache, neck pain, dysphagia or breathing trouble.
• Past events: trauma, previous eye surgery, long-term rigid contact lens wear, eye rubbing, autoimmune disease, family history.

Step 3: Examination

• Pupil size and extraocular motility, even if normal.
• Globe position (Hertel), lagophthalmos, orbicularis function, Bell phenomenon, tear production, and corneal health.
• Jaw-winking, fatigability, lid retraction on adduction or downgaze, and orbital palpation for a mass.
• Evert the lid and examine the fornix.
• Dilated fundus examination in young patients with ptosis, poor levator function and ophthalmoplegia, to look for pigmentary changes of CPEO or Kearns-Sayre syndrome.

Step 4: Classify

Management without associated anomalies

• If the ptosis is mild with no amblyopia and no chin-up head position, observe and re-examine every 3 to 12 months.
• Correct refractive error and treat amblyopia.
• Operate early if the visual axis is covered or there is a chin-up posture.
• For surgery, use levator resection if levator function is moderate or normal. Use frontalis suspension if it is poor (Wills Eye).

• Involutional (aponeurotic): levator advancement or reinsertion, or anterior levator repair (Kanski).
• Mild ptosis with good levator function (10 mm or more), positive phenylephrine test, or Horner syndrome: conjunctiva-Müller resection, which lifts the lid 2 to 3 mm.
• Levator function of at least 5 mm, any cause: levator advancement or resection.
• Severe ptosis (over 4 mm) with levator function under 4 mm: frontalis suspension.
• Mechanical: remove the cause, such as a mass or chalazion, or perform blepharoplasty for dermatochalasis.

Management with associated anomalies

• Always test the superior rectus in congenital ptosis.
• A poor Bell phenomenon with upgaze limitation and lagophthalmos in downgaze suggests double elevator palsy (monocular elevation deficiency).
• Correcting the hypotropia first may improve the ptosis.
• Do not do aggressive ptosis surgery in a patient with a poor Bell phenomenon. Use a conservative correction because of corneal exposure risk.
• Look for a subtle or partial third nerve paresis.

• Levator function is poor, so the ptosis is repaired by frontalis suspension.
• Telecanthus often improves as the nasal bridge grows. Surgery is reserved for severe telecanthus.
• Medial canthal and epicanthus surgery is usually done before or in stages with the ptosis repair.
• BPES is autosomal dominant (FOXL2). Female patients should have endocrine and fertility counselling for premature ovarian insufficiency. This comes from my own knowledge, not the library excerpts I read.

• Mild cases need no treatment, and the winking often improves around school age.
• Levator resection alone increases the winking.
• Mild cases with levator function of at least 5 mm and little winking can have unilateral levator advancement.
• Moderate or severe cases need levator disinsertion plus ipsilateral brow suspension, so the lid is lifted only by the frontalis. Bilateral surgery gives symmetry.
• Check for amblyopia and strabismus, which are common.

• Third nerve palsy needs urgent imaging to exclude a posterior communicating artery aneurysm.
• Misdirection syndromes, and ptosis after aberrant facial nerve regeneration, are treated with levator disinsertion plus brow suspension.
• Strabismus surgery is done before the ptosis repair. A complete palsy gives a severe ptosis that guards against diplopia, so correct it cautiously.

• Mild ptosis of about 2 mm with lower lid elevation, plus miosis and anhidrosis.
• In children, congenital Horner syndrome needs a pediatric workup for neuroblastoma, per Wills Eye (see its Horner syndrome chapter).
• In adults, urgent head and neck vascular imaging looks for carotid dissection, and chest CT for an apical lung lesion.
• Once the cause is treated, any residual ptosis is managed with conjunctiva-Müller resection.

• Ice test, acetylcholine receptor antibodies, and single-fiber EMG.
• Chest CT for thymoma.
• Treat medically first. Do not operate until the disease is stable. Taping the lid or crutches can be used meanwhile.

• Ptosis with poor levator function and ophthalmoplegia, with or without retinal pigmentary changes.
• Get an urgent ECG and cardiology consult because of the risk of heart block and sudden death.
• Surgery is by frontalis suspension, with crutches as an alternative. Protect the cornea carefully, since Bell phenomenon and orbicularis function are often poor.

• Look for congenital cataract, epicanthus, punctal abnormalities, refractive error and anisometropia, strabismus and amblyopia, and skeletal or CNS anomalies.
• Congenital fibrosis of the extraocular muscles is a myogenic cause. Treat with frontalis suspension and corneal protection.

Principles before surgery

• Do a dry eye and corneal assessment first. Poor Bell phenomenon, lagophthalmos or poor orbicularis function means under-correct.
• In children, amblyopia, strabismus and refractive error management come before or alongside surgery.
• Postoperative problems to watch for: exposure keratopathy, lagophthalmos, under- or overcorrection, and asymmetry.

Recent evidence