The term “ptosis” is derived from the Greek word falling and refers to drooping of a body part. Blepharoptosis is upper eyelid drooping with the eyes in the primary position of gaze.
The shape of one's eyes along with the position of the eyelids, shape, and position of the eyebrow determines one's identity. Hence, drooping of the eyelids may produce a functional or a cosmetic deficit.
Ptosis can occur in all age groups and is the result of various factors. One must remember that when a patient presents with complaints of drooping, it is a mere symptom and not the diagnosis. A thorough evaluation is of utmost importance to determine the cause.
Ptosis can classify as true ptosis or pseudoptosis. True ptosis is further classified based on the age of presentation into congenital ptosis and acquired ptosis.
Acquired adult ptosis is further classified based upon the etiological factors as
Aponeurotic ptosis
Neurogenic ptosis
Myogenic ptosis
Mechanical ptosis
Traumatic ptosis
Aponeurotic ptosis
Aponeurotic ptosis is the most prevalent form of adult ptosis and usually presents in the 5th or 6th decade of life. It is also known as involutional ptosis. However, it can occur in young individuals following trauma, recent eyelid swelling, ocular surgery or prolonged use of contact lenses. The pathogenesis of aponeurotic ptosis is most often due to dehiscence or disinsertion of the levator aponeurosis. In involutional cases, true dehiscence is sometimes absent, and ptosis occurs due to stretching or thinning of the aponeurosis. Rarely the levator muscle shows fatty infiltration.
Characteristic features of this type of ptosis are that patients have a good levator function with a high lid crease, affected eyelid appears lower on down gaze and a thin upper eyelid with redundant skin.
Neurogenic ptosis
Neurogenic ptosis results from any condition which disrupts the innervation of either the levator muscle or muller’s muscle. The varieties most commonly encountered by an ophthalmologist are 3rd cranial nerve palsy and Horner syndrome.
Third cranial nerve palsy
Lesions along the oculomotor nerve present with ptosis and restriction of adduction, elevation and depression movements of the eyeball. Pupillary involvement may or may not be present. Bell's phenomenon is usually poor. Pupil-involving third nerve palsy is considered a neurological as it is most often due to a posterior communicating artery aneurysm compressing the nerve. Pupil-sparing third nerve palsy is most often due to an ischemic vascular cause and usually resolves spontaneously in 3 months. Other causes include inflammation, trauma or tumors along the course of the nerve. Lesions of the superior orbital fissure, orbital apex, or cavernous sinus, present in combination with other cranial nerve palsies.
Treatment is challenging as the patients have a poor or absent Bell’s phenomenon placing them at high risk of developing exposure keratopathy post-surgery. Ideally, strabismus surgery is done first to correct the deviation followed by ptosis correction via the frontalis sling technique with planned under-correction.
Horner syndrome (Oculosympathetic paresis)
Horner syndrome consists of mild ptosis, pupillary miosis, apparent enophthalmos, and anhidrosis. It occurs due to interruption of the sympathetic nerve supply to the muller’s muscle and dilator pupillae muscle.
Pupillary anisocoria can be well demonstrated in dim illumination. Patients with Horner’s syndrome occurring during childhood also have iris heterochromia due to decreased melanin production in melanocytes which is controlled by the sympathetic pathway.
The diagnosis of Horner syndrome is often made clinically. Pharmacological tests using 4% cocaine, 1% hydroxyamphetamine or 2.5% phenylephrine help confirm the diagnosis.
Myogenic ptosis
Myogenic ptosis arises due to an abnormality in the levator muscle itself. These patients usually present with reduced levator action along with restricted extraocular motility and facial expression.
Myasthenia gravis
Myasthenia gravis is an autoimmune disorder characterized by the presence of antibodies to acetylcholine receptors located at the neuromuscular endplates of voluntary muscles. This leads to decreased action of acetylcholine which results in muscle weakness and fatigue. Myasthenia may be generalized or localized to the eye (ocular myasthenia). The most common presenting feature is variable ptosis associated with diplopia. Symptoms may be unilateral or bilateral. Patients with myasthenia initially have a good levator function. Prolonged upgaze will cause a worsening of ptosis in these patients due to muscle fatigue.
Cogan lid twitch sign: Rapid saccadic eye movements from downgaze to primary position results in rapid upshoot of the lid followed by a gradual drop to the primary position.
Other tests which help confirm the diagnosis include ice test, serum acetylcholine receptor antibody assay, single fiber electromyography, and repetitive nerve stimulation test.
Treatment of such patients involves administration of acetylcholinesterase drugs, oral steroids or immunosuppressants. In patients with severe ptosis, ptosis correction with planned under-correction may be an option.
Myotonic dystrophy
Myotonic dystrophy is an autosomal dominant disorder which presents with gradually progressing ptosis and external ophthalmoplegia. The pathologic process is a failure of muscle to relax after contraction. It also involves muscles of facial expression, neck, and limbs.
Ocular examination in these patients also shows pupillary light-near dissociation, chromatic cataracts (Christmas tree cataract), and retinal pigmentary degeneration. Males develop a frontal pattern of balding and testicular atrophy.
Chronic progressive external ophthalmoplegia (CPEO)
CPEO is a mitochondrial myopathy causing bilateral symmetrical involvement of the extraocular muscles. Manifestations begin in childhood or adolescent age and progress slowly during adulthood. Bilateral symmetrical involvement is the first symptom followed by bilateral ophthalmoplegia. Due to the symmetric involvement of extraocular muscles, patients often do not complain of diplopia. As the muscles of facial expression are involved, patients develop an expressionless face (Hutchinson's face)
Diagnosis is confirmed by muscle biopsy which shows ragged red fibers due to enlarged mitochondria.
Kearns-Sayre syndrome: A variant of CPEO which shows retinal pigmentary degeneration, cardiac conduction defects, complete heart block, ataxia, neuropathy, endocrine dysfunction, and occurs in young adults.
There is no treatment to date for CPEO. Ptosis surgery to clear the visual axis can be done in severe cases keeping in mind the high risk of exposure keratopathy.
Oculopharyngeal muscular dystrophy
This autosomal dominant disorder manifests in the 4 to 5 decade of life with bilateral ptosis, progressive external ophthalmoplegia, dysphagia, dysarthria, facial muscle weakness, and proximal limb weakness.
Mechanical ptosis
Ptosis secondary to any tumor producing an increased weight on the lids, cicatrization or scarring of the conjunctiva, and blepharochalasis.
Traumatic ptosis
Ptosis occurs due to direct or indirect trauma to the levator muscle. Penetrating injuries involving the levator can be repaired immediately. However, ptosis secondary to blunt trauma may resolve spontaneously over time. Ptosis which does not improve after 6 months can have surgical repair.
Pseudoptosis
It is not true ptosis but apparent ptosis due to abnormalities in structures other than the levator muscle. Causes include dermatochalasis, brow ptosis, hypotropia, microphthalmos, anophthalmos, phthisis bulbi, and contralateral eyelid retraction.
It is very important to distinguish true ptosis from a pseudoptosis before embarking upon any surgical correction for drooping.
Clinical presentation
Patients usually complain of
Drooping of eyelids
Feeling of heaviness in the eyes
Visual obscuration due to drooping
Cosmetic complaints
Assessment
A thorough history taking and clinical examination help determine the etiology of ptosis and plan appropriate treatment.
History
History taking should include the age of onset of ptosis, progression, duration, and any aggravating or relieving factors. Any associated symptoms such as diplopia, diurnal variation, pain, lid swelling, dysphagia or muscle weakness help provide a provisional diagnosis.
Predisposing factors such as trauma, ocular or eyelid surgery, contact lens use, and botulinum toxin injection should be carefully ruled out. A family history of ptosis should be looked for to rule out hereditary disorders. In patients where the history is inconclusive, assessment of old photographs gives an idea about the time of onset.
Any systemic illness, mental health issues, and medication history require documentation. Patients on blood thinners such as aspirin should be advised to stop medications 1 week before surgery.
Clinical examination
Clinical examination starts from the moment the patient walks into the doctor's clinic. It is essential to look for any facial asymmetry, frontalis overaction, chin up or head tilt posture.
Ocular examination
Visual acuity and refraction
Cover test to look for any hypotropia and rule out any component of pseudoptosis
Extraocular motility disturbance and any aberrant eyelid movements
Pupillary examination to look for Horner syndrome or 3rd cranial nerve palsy
Examination to look for any giant papillary conjunctivitis or symblepharon
Corneal sensation and dry eye evaluation as they can predispose to post-operative keratopathy.
Fundus examination for features of retinal pigmentary degeneration
Specific examination of ptosis
Lid measurements should be done positioning the face in the frontal plane, negating the action of frontalis muscle with the thumb, and eyes in the primary position of gaze. The examiner should be seated at the eye level of the patient to avoid parallax error.
1. Palpebral fissure height (PFH): It is the vertical palpebral aperture height between the upper and lower eyelid margin in the pupillary plane with eyes in the primary position of gaze. Average PFH is around 10mm.
2. Marginal reflex distance 1 (MRD 1): MRD 1 is the distance between the upper lid margin and the corneal light reflex. Normal MRD 1 is 4-5mm. The difference in MRD 1 between the two eyes helps classify ptosis as mild, moderate or severe in patients presenting with unilateral ptosis.
The difference in MRD 1 between two eyes:
2mm – Mild ptosis
3mm – Moderate ptosis
4mm – Severe ptosis
3. Marginal reflex distance 2 (MRD 2): MRD 2 is the distance between the corneal light reflex and lower eyelid margin. Normally MRD 1 + MRD 2 = PFH.
4. Levator action: It is the amount of excursion measured with a millimeter scale when the eyelid moves from extreme downgaze to extreme upgaze with frontalis action negated. Normal levator action is greater than 15mm. It is the single most important measurement in a patient with ptosis as its value determines the choice of surgical procedure.
Grading of levator action
Less than 4 mm – Poor
5 to 9 mm – Fair
9 to 11 mm – Good
Greater than 12 mm – Excellent
In patients with poor levator action (less than 4mm), frontalis sling surgery is the preferred procedure.
5. Margin crease distance (MCD): It is the distance between the lid margin and skin crease in downgaze. Normal MCD is 7 to 8mm in men and 8 to 10 mm in women. In congenital ptosis, MCD is usually absent or faint, whereas in aponeurotic ptosis MCD is higher than normal.
During surgery, it is very important to reform the crease identical to the contralateral eye to maintain symmetry and achieve good cosmesis.
6. Bell’s phenomenon: This is another very important factor to be considered before ptosis correction. The patient is asked to close the eyes gently, and an attempt is made to open them. In patients with poor bell’s, ptosis correction should be avoided or undercorrected to avoid the risk of post-operative exposure keratopathy.
7. Assess presence of lagophthalmos and lid lag on downgaze which if present will worsen post-surgery.
8. Any brow ptosis or dermatochalasis if present should be documented. In involutional ptosis, blepharoplasty procedure is often combined with ptosis repair.
9. Hering test: In patients with unilateral ptosis, the ptotic lid is gently elevated manually, and the contralateral eyelid observed. Due to Hering's law of equal innervation, the contralateral eyelid may drop (See-saw effect). It is important to demonstrate this to the patient preoperatively and warn them about the possibility of requiring ptosis surgery in the contralateral eye. In such cases, a planned under-correction may be the treatment.
10. Phenylephrine test: It is a useful test in patients with mild ptosis or ptosis due to Horner syndrome; instill 2.5% phenylephrine drops in the superior fornix. Ptosis measurements are repeated after 10 minutes. Patients in whom the ptotic lid elevates due to stimulation of Muller's muscle are ideal candidates for posterior approach ptosis correction (conjunctival – mullerectomy surgery).
11. Tests to rule out myasthenia gravis:
Fatigue test: The patient maintains fixation in upgaze for 30 seconds. In patients with myasthenia, the eyelid gradually drops down due to muscle fatigue.
Ice test: An ice pack is placed over the closed ptotic eyelid for 2 minutes. Ptotic measurements are repeated after 2 minutes. Improvement in PFH by 2mm or more is considered positive for myasthenia. This is because cooling improves neuromuscular transmission.
12. Hertel exophthalmometry: A Hertel reading helps rule out any proptosis or enophthalmos and thus excludes pseudoptosis.
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