Introduction
Ptosis may be due to disease of the:
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eyelid
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levator muscle
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neuromuscular junction
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third nerve
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brain
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neck
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upper chest
As ophthalmologists, we need to remember that serious disease can first present with ptosis and to have a practical plan for assessing patients with ptosis to avoid missing these potentially life-threatening causes.
The most common cause of either unilateral or bilateral ptosis is age-related dehiscence or disinsertion of the levator aponeurosis. Not infrequently, ptosis is the initial presentation of myasthenia gravis. Other less common but important causes include partial third nerve palsy due to aneurysm or tumor; or Horner syndrome from internal carotid artery (ICA) dissection or a tumor in the head, neck or upper chest.
The cause of ptosis can normally be diagnosed clinically (see management flowchart, p. 297). However, in some cases, further investigations are required, e.g. tests for myasthenia, neuro-imaging or muscle biopsy for genetic testing.
Examination checklist
Ptosis
Have you asked about, and looked for, all the following key features?
History
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the ptosis
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when did it start?
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speed of onset?
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development over time?
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variation through the day or from day to day? (possible myasthenia)
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getting better or worse or staying the same?
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other ophthalmic symptoms
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transient visual loss? (possible ICA dissection causing Horner syndrome)
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double vision? (possible myasthenia, or tumor/aneurysm causing partial third or Horner)
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pain?
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blurred vision?
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field loss?
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previous medical and surgical history
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recent head or neck trauma? (possible ICA dissection causing Horner syndrome)
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recent neck or chest surgery?
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cancer? (possible head, neck or chest primary or metastasis causing Horner syndrome or partial third nerve palsy)
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social history: smoker? (possible apical lung cancer causing Horner syndrome)
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if patient over 50: symptoms of giant cell arteritis (GCA)?
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system review questions: hemifacial, neck or arm pain? (possible ICA dissection causing Horner syndrome)
Examination
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visual acuity
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visual field defect to confrontation? (possible pituitary tumor causing partial third)
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limitation of eye movements (especially elevation)? (possible myasthenia, or tumor/aneurysm causing partial third nerve palsy)
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pupils
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is there anisocoria?
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smaller, normally reactive pupil on side of ptosis: possible Horner syndrome
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larger, poorly reactive pupil on side of ptosis: possible partial third nerve palsy
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relative afferent pupillary defect (RAPD)? (possible orbital apex tumor causing partial third nerve palsy)
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eyelids
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measure skin crease height and levator function (high skin crease and good levator function with otherwise normal examination: possible aponeurotic ptosis)
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does the ptosis worsen on 2 minutes’ sustained upgaze? (possible myasthenia; do ice test)
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is there a Cogan lid twitch? (possible myasthenia) ( )
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increase in ptosis on manual elevation of contralateral eyelid (possible myasthenia) ( )
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remember to check the strength of eyelid closure (strength of orbicularis oculi) in all patients with ptosis (possible myasthenia, myotonic dystrophy, chronic progressive external ophthalmoplegia [CPEO])
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orbits: proptosis, injection, chemosis?
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corneal and facial sensation to light touch: decreased sensation on side of ptosis? (possible cavernous sinus tumor)
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orbicularis and facial muscle power: (possible myasthenia or myopathy, if weak)
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if possible Horner syndrome
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examine the neck; are there any lumps? (neck tumor)
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examine the hands; is there finger abduction or adduction weakness? (cervical spinal cord or lung apex C8/T1 lesion)
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spoon test for facial sweating (see p. 285 )
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if patient over 50: palpate temporal arteries
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perimetry if: field defect to confrontation, decreased vision, RAPD, diplopia or motility defect
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full neurologic examination if: the patient has fatigable ptosis, diplopia or orbicularis or facial weakness; are there any signs of systemic muscle weakness or fatigability to support a diagnosis of myasthenia?
Management flowchart
Aponeurotic ptosis
Cause
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age-related dehiscence or disinsertion of the connective tissue aponeurosis connecting the levator muscle to the upper eyelid
Demographic
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middle-aged or elderly patients
Symptoms
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constant drooping of one or both upper lids
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no symptoms of myasthenia: no diplopia, no limb fatigability, no major change in the ptosis during the day or from day to day (note: some patients with aponeurotic ptosis do notice a slight worsening of the ptosis at night, due to fatigue of compensatory frontalis overaction)
Signs
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high skin crease (more than 7 mm from lid margin)
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high, deep superior sulcus
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thinned upper eyelid skin
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normal levator function ( Fig. 10.1 )