Blepharoptosis, Causes, Symptoms, Diagnosis, Treatment

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Blepharoptosis/Ptosis is derived from the Greek word for falling and is the medical terminology describing a drooping or abnormal lowering of an anatomical area. When ptosis pertains to the eyelid it is more accurately described as blepharoptosis.Ptosis can either be present at birth (congenital) or appears later in life (acquired), following long-term contact lens wear, trauma, after cataract surgery or other eye operations. There are less common causes of a droopy eyelid, such as problems with the nerves or muscles.

Ptosis is a drooping or falling of the upper eyelid. The drooping may be worse after being awake longer when the individual's muscles are tired. This condition is sometimes called "lazy eye," but that term normally refers to the condition amblyopia. If severe enough and left untreated, the drooping eyelid can cause other conditions, such as amblyopia or astigmatism. This is why it is especially important for this disorder to be treated in children at a young age before it can interfere with vision development.

Anatomy

The opening located between the upper and lower eyelid is elliptical and termed the palpebral fissure. The upper eyelid curvature is maximum at a point just nasal to the mid pupillary point, which is an important point to mark before surgery to get the best cosmetic results. The upper lid covers 1 to 2mm of the superior limbus, while the lower lid lies at the lower limbs.

Structures forming the eyelid

  • Skin and subcutaneous tissue
  • Orbicularis oculi
  • Orbital septum
  • Preaponeurotic fat pad
  • Tarsal plate
  • Levator aponeurosis and Muller’s muscle
  • Conjunctiva

Skin

The eyelid skin is the thinnest layer of skin in the entire body. The eyelid crease forms due to the attachment of levator aponeurosis to the skin.

  • Orbicularis oculi - It is a circular muscle and consists of three parts namely: perceptual, pretarsal, and orbital orbicularis. Contraction is responsible for the gentle and forced closure of the eyelid.
  • Orbital septum - It is a multi-layered structure of thin fibrous connective tissue. The septum inserts superiorly to the periosteum over the superior orbital rim. Inferiorly it merges with the fibers of the levator aponeurosis above the superior tarsal border.In a ptosis surgery, the septum is opened to gain access to the levator muscle. Care should be taken to separate septal attachments to the levator muscle to avoid post-operative lid retraction.
  • Preaponeurotic fat pad - The fat pad is posterior to the septum and anterior to the elevator. This pad of fat can be identified intraoperatively by applying pressure over the globe which causes it to prolapse forward. It helps in identifying the levator muscle which lies immediately posterior to it.
  • Tarsal plate - Tarsal plate forms the structural skeleton of the eyelid and is made up of dense connective tissue. The vertical height is 10 to 12 mm in the upper eyelid and 4 to 5 mm in the lower lid. The tarsal plates contain the Meibomian glands, the orifices of which open posterior to the grey line.
  • Levator Palpebrae superioris muscle (LPS) - LPS is the main elevator of the eyelid. It originates at the orbital apex from the periosteum over the lesser wing of the sphenoid. It travels forward below the roof of the orbit. At the superior orbital rim, it changes direction from horizontal to vertical and forms the tendinous sheath called levator aponeurosis. At this transition, lies the Whitnall’s ligament which is seen as a dense white band of connective tissue and acts as a pulley. It lies 10 to 12 mm above the tarsal plate. Medially and laterally, it forms the medial and lateral horns which then insert into the posterior lacrimal crest and Whitnall’s tubercle along with the canthal tendons.The elevator receives nerve supply by the superior division of the oculomotor nerve.
  • Muller’s muscle - It is a sympathetically innervated smooth muscle. It originates from the under the surface of the levator aponeurosis at the level of the Whitnall ligament and inserts into the superior border of the tarsus. It contributes to 2 mm of eyelid elevation.
  • Conjunctiva - It is the innermost layer of the eyelid formed by non-keratinizing squamous epithelium. It continues over the anterior surface of the globe. It contains goblet cells, the secretions of which hour of utmost importance in keeping the eyes moist.

Types of Ptosis

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.
  • 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.
  • 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.
  • 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 elevator 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.
  • Neurotoxic ptosis - which is a classic symptom of envenomation[rx] by elapid snakes such as cobras,[rx] kraits,[rx] mambas and taipans. Bilateral ptosis is usually accompanied by diplopia, dysphagia and/or progressive muscular paralysis. Regardless, neurotoxic ptosis is a precursor to respiratory failure and eventual suffocation caused by complete paralysis of the thoracic diaphragm. It is, therefore, a medical emergency and immediate treatment is required. Similarly, ptosis may occur in victims of Botulism (caused by Botulinum toxin) and this is also regarded as a life-threatening symptom
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Pseudoptosis due to

  • Lack of lid support: empty socket or atrophic globe.
  • Higher lid position on the other side: as in lid retraction

Causes of Ptosis

  • Myasthenia gravis — Ptosis can be one of the first symptoms of myasthenia gravis, a rare disorder that affects the ways muscles respond to nerves. Myasthenia gravis can cause progressive muscle weakness, not only in the eyelids but also in the facial muscles, arms, legs and other parts of the body.
  • Muscle diseases — Ptosis can be a symptom of an inherited muscle disease called oculopharyngeal muscular dystrophy that affects eye motion and can cause difficulty swallowing. In younger adults, ptosis can be caused by a group of muscle illnesses called progressive external ophthalmoplegia, which cause ptosis in both eyes, problems with eye movement, and sometimes other muscle symptoms that involve the throat or heart muscle.
  • Nerve problems — Because the eye muscles are controlled by nerves that come from the brain, conditions that injure the brain or its cranial nerves sometimes can cause ptosis. These conditions include stroke, brain tumor, a brain aneurysm (a grapelike swelling on a blood vessel inside the brain), and nerve damage related to long-term diabetes. Another cause of ptosis is Horner's syndrome, which also can cause an abnormally small pupil and loss of the ability to sweat — on half the face. One particularly dangerous cause of Horner's syndrome is a cancerous tumor located at the top portion of the lungs.
  • Local eye problems — In some cases, an eyelid droops because of an infection or tumor of the eyelid, a tumor inside the eye socket, or a blow to the eye.
  • Ptosis may be caused - by damage to the muscle which raises the eyelid, damage to the superior cervical sympathetic ganglion or damage to the nerve (3rd cranial nerve (oculomotor nerve)) which controls this muscle. Such damage could be a sign or symptom of an underlying disease such as diabetes mellitus, a brain tumor, a Pancoast tumor (apex of the lung) and diseases which may cause weakness in muscles or nerve damage, such as myasthenia gravis or oculopharyngeal muscular dystrophy. Exposure to the toxins in some snake venoms, such as that of the black mamba, may also cause this effect.
  • The aponeurosis of the levator muscle - nerve abnormalities, trauma, inflammation or lesions of the lid or orbit.[Rx]Dysfunctions of the elevators may occur as a result of autoimmune antibodies attacking and eliminating the neurotransmitter.[rx]
  • Myogenic, neurogenic, aponeurotic -  mechanical or traumatic cause and it usually occurs isolated, but may be associated with various other conditions, like immunological, degenerative, or hereditary disorders, tumors, or infections [rx]
  • Acquired ptosis - is most commonly caused by aponeurotic ptosis. This can occur as a result of senescence, dehiscence or disinsertion of the levator aponeurosis. Moreover, chronic inflammation or intraocular surgery can lead to the same effect. Also, wearing contact lenses for long periods of time is thought to have a certain impact on the development of this condition.
  • Congenital neurogenic ptosis - is believed to be caused by the Horner syndrome.[rx] In this case, a mild ptosis may be associated with ipsilateral ptosis, iris, and areola hypopigmentation and anhidrosis due to the paresis of the Mueller's muscle. Acquired Horner syndrome may result after trauma, neoplastic insult, or even vascular disease.

Symptoms of Ptosis

  • Raising the eyebrows and eyelids
  • Tilting the head back to see better
  • Lifting the eyelid with a finger
  • Inflammation of Eyelid margin at the lash follicles
  • Moderate lid swelling along lash line
  • Lower Eyelid is usually more affected
  • Soft, oily Yellow SkinScaling
  • Pruritus
  • Local irritation and burning
  • Watery eyes
  • Red eyes
  • A gritty, burning or stinging sensation in the eyes
  • Eyelids that appear greasy
  • Itchy eyelids
  • Red, swollen eyelids
  • Flaking of the skin around the eyes
  • Crusted eyelashes upon awakening
  • Eyelid sticking
  • More frequent blinking
  • Sensitivity to light
  • Eyelashes that grow abnormally (misdirected eyelashes)
  • Loss of eyelashes
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Diagnosis of Ptosis

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.

  • 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.
  • 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

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.

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 elevator 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.

  • 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.
  • 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.
  • Assess the presence of lagophthalmos - and lid lag on downgaze which if present will worsen post-surgery.  Any brow ptosis or dermatochalasis if present should be documented. In involutional ptosis, blepharoplasty procedure is often combined with ptosis repair.
  • 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.
  • 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).
  • 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.
  • Hertel exophthalmometry - A Hertel reading helps rule out any proptosis or enophthalmos and thus excludes pseudoptosis.
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Treatment of Ptosis

Aponeurotic and congenital ptosis may require surgical correction if severe enough to interfere with vision or if cosmetics is a concern. Treatment depends on the type of ptosis and is usually performed by ophthalmic plastic and reconstructive surgeon, specializing in diseases and problems of the eyelid.

Surgical procedures include

  • Levator resection
  • Müller muscle resection
  • Frontalis sling operation (preferred option for oculopharyngeal muscular dystrophy)
  • Non-surgical modalities like the use of "crutch" glasses or Ptosis crutches or special scleral contact lenses to support the eyelid may also be used.

Ptosis that is caused by the disease may improve if the disease is treated successfully, although some related diseases, such as oculopharyngeal muscular dystrophy currently have no cures.

  • Topical antiseptics – Bibrocathol is an antiseptic drug for the treatment of acute eyelid diseases. Eye ointments containing 2 or 5 % bibrocathol are used to treat.
  • Topical steroids – Topical steroids may improve ptosis but frequent use is best avoided. All forms of ptosis may benefit from a course of treatment with topical corticosteroid drops to decrease inflammation in an acute exacerbation. Should applying drops several times daily, tapered to discontinuation over one to three weeks as it has significant adverse effects over the long-term such as increased intraocular pressure (IOP), posterior subcapsular cataract formation, and superinfection.
  • Oral antibiotics – In patients with posterior blepharitis oral tetracycline or doxycycline may is effective
  • Medications that affect the immune system – Topical cyclosporine (Restasis) is a calcineurin inhibitor that has been shown to offer relief of some signs and symptoms of blepharitis.
  • Treatments for underlying conditions – Ptosis caused by seborrheic dermatitis, rosacea or other diseases may be controlled by treating the underlying disease.
  • Eyedrops/ointments  Eye drops or ointments containing corticosteroids are frequently used in conjunction with antibiotics and can reduce eyelid inflammation.[rx][rx][rx]
  •  Hypochlorous acid products – Both over-the-counter and prescription products with hypochlorous acid (HOCl) can be used to alleviate the signs and symptoms of Ptosis. In a recent study, a saline hygiene solution with hypochlorous acid was shown to reduce the bacterial load significantly without altering the diversity of bacterial species on the eyelids. After 20 minutes of treatment, there was >99% reduction of the Staphylococci bacteria, which is a common cause of ptosis.[rx]
  • Cryotherapy  – with liquid nitrogen performed under the slit lamp was reported to be efficient by some authors. Some authors have proposed argon laser therapy as an effective treatment for phthiriasis palpebrarum. One session using a 200-micron beam, with a duration of 0.1 seconds, and a power of 0.2 W allowed the destruction of both lice and nits. However, this device necessitates strict eye protection and may not be available on a large scale.
  • Massage the eyes – After using the warm compress to loosen the sebum, massaging helps express the oily contents of the glands. With a finger or a cotton-tipped applicator or swab, massage the margin of the eyelid, where the eyelashes and glands are, using small circular motions.
  • Diet – There’s some evidence to suggest a diet high in omega-3 fats can help improve ptosis.

Surgery

Choice of the surgical technique according to the grade of ptosis and levator function

Technique Indication Levator function Complications
Fasanella–Servat Mild congenital ptosis >10 mm Dermatochalasis, undercorrection, overcorrection, bleeding, wound dehiscence, corneal abrasion, skin crease level defect
Aponeurosis surgery Mild ptosis >10 mm Asymmetric skin crease
Levator resection Mild ptosis >5 mm Corneal exposure, entropion, contour abnormality, conjunctival prolapse
Frontalis sling Amblyopia prevention Severe ptosis <2 mm Corneal exposure, infection, granulomas
Whitnall ligament sling Mild–severe ptosis >3 mm High risk of reintervention
Muller resection Mild ptosis >10 mm Corneal abrasion, undercorrection

 

References

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