AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

INTRODUCTION

Section 2 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

Background

Blepharoptosis, also referred to as ptosis, is defined as an abnormal low-lying upper eyelid margin with the eye in primary gaze. The normal adult upper lid lies 1.5 mm below the superior corneal limbus and is highest just nasal to the pupil.

Blepharoptosis can be classified as congenital or acquired. This differentiation is based on age. A more comprehensive classification is based on etiology and includes myogenic, aponeurotic, neurogenic, mechanical, traumatic, and pseudoptotic. The most common cause of congenital ptosis is myogenic due to the improper development of the levator muscle.

Most cases of acquired blepharoptosis are secondary to aponeurotic causes, such as involutional changes, a disinsertion, or a dehiscence. Identification of the underlying pathophysiologic mechanism is paramount in instituting optimal treatment.

Pathophysiology

Blepharoptosis, which is a droopy upper eyelid, is the result of dysfunctioning of one or both upper eyelid elevator muscles. These elevator muscles are the levator palpebrae superioris and its aponeurosis and the Mueller muscle.

The levator palpebrae superioris is a striated muscle that is innervated by the superior division of the oculomotor nerve (cranial nerve III). This muscle is about 40 mm long and originates from the lesser wing of the sphenoid. It continues anteriorly, and at the Whitnall ligament, it travels inferiorly as an aponeurosis. This aponeurosis is 14-20 mm long and inserts into the anterior aspect of the tarsal plate. It also sends attachments to the skin, forming the upper eyelid crease. The levator muscle and aponeurosis is the major elevator of the upper eyelid.

The Mueller muscle, a sympathetically innervated smooth muscle, has its origins from the undersurface of the levator superioris. Approximately 12 mm long, it inserts superiorly on the tarsal border and elevates the upper eyelid by approximately 2 mm.

Mortality/Morbidity

• The associated mortality is usually due to anesthetic complications from surgery. Kearns-Sayre disease, a subtype of chronic progressive external ophthalmoplegia, is a syndrome with associated myogenic ptosis, retinal pigmentary changes, and cardiac conduction abnormalities that can cause death.
• Morbidity is associated with blockage of the visual axis in the severely ptotic eyelid. Congenital cases can obstruct vision and lead to amblyopia. Even without visual axis obstruction, the eyelid may induce refractive errors, especially astigmatism resulting in amblyopia.
• In adults, the morbidity is associated with constriction of the superior visual fields. Patients may complain that they tire easily when reading and experience frontal headaches as they lift their eyebrows in an effort to keep the eyelids open. Patients may state they are dissatisfied with their appearance.

Race

No racial predilection has been described.

Sex

No sexual predilection has been described.

Age

Acquired ptosis can occur at any age, but it is commonly seen in older adults. Congenital ptosis occurs at birth.

CLINICAL

Section 3 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

History

As with any patient, obtain a thorough medical and ophthalmic history.

• More specifically, the onset of ptosis, alleviating or aggravating factors, family history of ptosis, and history of trauma or ocular surgery are important clues to the etiology.
• Patients usually complain of a bedroom-eye appearance, always appearing sleepy or tired, and constriction of the visual fields.

Physical

If the patient has not been under the care of an ophthalmologist, a complete ocular examination is required.

Quantification and qualification of the blepharoptosis is essential for proper diagnosis and treatment. All quantitative eyelid and eyebrow measurements should be taken before the use of dilating drops.

• The palpebral fissure is the distance between the upper and lower eyelid in vertical alignment with the center of the pupil.
• The marginal reflex distance-1 (MRD-1) is the distance between the center of the pupillary light reflex and the upper eyelid margin with the eye in primary gaze. A measurement of greater than 2.5 mm is considered normal.
• The marginal reflex distance-2 (MRD-2) is the distance between the center of the pupillary light reflex and the lower eyelid margin with the eye in primary gaze. A measurement greater than 5 mm is considered normal.
• The margin crease distance is the distance from the upper eyelid margin to the lid crease. In white women, a central measurement of 10-11 mm is considered normal, and in white men, 8-10 mm is considered normal.
• Levator function is the distance the eyelid travel from downgaze to upgaze while the frontalis muscle is held inactive at the brow. A measurement of greater than 10 mm is considered excellent, whereas 0-5 mm is considered poor.
• The presence of proptosis, lagophthalmos, tear dysfunction, absence of a Bell response, and lower eyelid laxity or scleral show should be appreciated and may alter the amount of ptosis repair.
• Pseudoptosis can result from microphthalmos, enophthalmos or anophthalmos, acquired hypotropia after a blowout fracture (orbital floor fracture), superior sulcus deformity, or contralateral vertical lid retraction.
• • Eyelid retraction may warrant thyroid function studies and the consideration of dysthyroid orbitopathy.
  • Parinaud syndrome should be considered if convergence-retraction nystagmus and pupillary light-near disassociation is found in conjunction with eyelid retraction; neuroimaging should be obtained.
• The margin fold distance is the distance from the upper eyelid margin to the fold of skin.

Causes

Ptosis can be caused by problems with the elevator muscles of the eyelid, the aponeurosis of the levator, nerve abnormalities either central or peripheral, trauma, inflammation, or lesions of the lid or orbit.

• Aponeurotic ptosis is the most common cause of acquired ptosis.
• • Senescence, involutional changes, dehiscence, or disinsertion of the levator aponeurosis are common.
  • Chronic inflammation or intraocular surgery (eg, cataract surgery) can incite stretching of the levator aponeurosis and dehiscence from the anterior surface of the tarsal plate.
  • Long-term use of contact lenses has also been implicated. Patients maintain normal or near-normal levator function, with a high upper eyelid crease. The attachments from the levator to the skin remain intact, and this forms the crease.
• Neurogenic blepharoptosis may be congenital or acquired in origin. Congenital neurogenic ptosis is usually due to Horner syndrome or a third nerve palsy. Acquired neurogenic ptosis causes include Horner syndrome, third nerve palsy, or myasthenia gravis.
• • Congenital Horner syndrome can result in mild ptosis associated with ipsilateral miosis, iris and areola hypopigmentation, and anhidrosis. The cause is paresis of the Mueller muscle, secondary to an embryologic lesion of the sympathetic pathway.
  • Congenital third nerve palsy has a variety of causes. Patients can present with aberrant regeneration and a small pupil. Often, parents believe that this is secondary to birth trauma.
  • Acquired Horner syndrome can be secondary to trauma, neoplastic insult, or vascular disease of the sympathetic pathway. All stigmata of congenital Horner syndrome, excluding iris and areola hypopigmentation, are present. Raeder paratrigeminal syndrome occurs in middle-aged men with daily ipsilateral headaches and the stigmata of acquired Horner syndrome.
  • Dysfunction of the third cranial nerve can result from a myriad of acquired insults. Trauma, multiple sclerosis, vasculopathy, and infection are all potential etiologies. Extraocular muscle dysfunction, pupillary abnormalities, and the presence of aberrant regeneration may aid in establishing the correct diagnosis.
  • Synkinetic neurogenic ptosis is the product of innervational anomalies. Marcus-Gunn jaw winking and posttraumatic ptosis are 2 examples of this interesting etiology. Microvascular diabetic neuropathies never result in synkinetic neurogenic ptosis.
• Myogenic blepharoptosis usually is congenital, but it can be associated with acquired disease processes.
• • Congenital myogenic ptosis is secondary to levator dysgenesis.
  • Acquired myogenic ptosis can be found in myasthenia gravis, chronic progressive external ophthalmoplegia, oculopharyngeal dystrophy, and myotonic dystrophy.
• Traumatic blepharoptosis can ensue after an eyelid laceration with transection of the upper eyelid elevators or disruption of the neural input.
• Mechanical ptosis can stem from the presence of eyelid neoplasms, for example, neurofibromas or hemangiomas or cicatrization secondary to inflammation or surgery.

DIFFERENTIALS

Section 4 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

Other Problems to be Considered

Craniofacial syndromes
Socket contraction
Poor-fitting ocular prosthesis
Hemifacial spasm
Blepharophimosis
Blepharochalasia
Double elevator palsy
Orbital and lid tumors
Cavernous sinus syndrome
Superior orbital fissure syndrome
Malingering

WORKUP

Section 5 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

Lab Studies

• If myasthenia gravis is suspected, a serum assay for acetylcholine receptor antibodies and an edrophonium chloride (Tensilon) test or single-fiber electromyography may be needed.
• CSF analysis can aid in the diagnosis of multiple sclerosis. Mild lymphocytosis or increased protein levels in the CSF levels may be present. In addition, elevated immunoglobulin G (IgG) levels and oligoclonal bands often are found.
• In patients with chronic progressive external ophthalmoplegia, an electrocardiogram, electroretinogram, electromyography, and mitochondrial assay should be considered.
• Patients with suspected thyroid abnormalities should undergo tests for thyroid function, including triiodothyronine (T3), thyroxine (T4), and thyroid-stimulating hormone (TSH).

Imaging Studies

• MRI of the brain with gadolinium enhancement is the imaging modality of choice if multiple sclerosis is suspected.
• If blepharoptosis is present with other neurologic deficits, imaging of the brain, orbits, or cerebrovascular system should be performed.
• CT scanning can be used to evaluate dysthyroid orbitopathy.
• In acquired Horner syndrome, MRI or CT of the brain, CT or radiography of the spine, and CT or radiography of the chest (especially of the apex of the lung) are warranted.

Other Tests

• Sympathomimetic agents can be used to stimulate the Mueller muscle, as follows:
• • 2.5% phenylephrine
  • 10% phenylephrine: Be aware of cardiac complications.
  • 0.5% apraclonidine (Iopidine): This is an alpha-adrenergic agonist.
  • 1.0% apraclonidine (Iopidine): This is an alpha-adrenergic agonist.
• Instill 2 drops on the eye under the eyelid (have the patient look down), wait 5 minutes, and assess any change in the palpebral fissure and the marginal reflex distance.
• • If no response is observed or if elevation is not adequate, external levator resection or advancement may be needed to correct the blepharoptosis.
  • If a good response is observed, the ptosis can be repaired by advancing the internal levator (Mueller muscle–conjunctival resection).

TREATMENT

Section 6 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical Care

If myasthenia gravis is diagnosed, treatment may involve the use of pyridostigmine (Mestinon).

In certain cases, a patient may not want to undergo surgery. Glasses can be made with a crutch attachment that can hold up the lid.

Surgical Care

Many surgical techniques have been well described for blepharoptosis correction. A surgeon may prefer one technique to another. This brief discussion is merely a guide and not dogma for approaching ptosis correction.

• If levator function is poor (<4 mm) or absent, the use of frontalis slings can achieve desirable postoperative results.
• • Many materials, both autogeneic and allogeneic, have been used with great success.
  • Fascia lata and frontalis muscle flaps are examples of autogeneic tissue, whereas Gore-Tex suture, frozen dura mater, silicone, and Alloderm are useful allogeneic materials.
  • Whether autogeneic or allogeneic material is chosen, the goal is to suspend the upper eyelid from the eyebrow or frontalis muscle.
  • With elevation of the eyebrow, the eye opens, and the orbicularis is used to close the eye.
• A levator advancement or resection is a technique that results in shortening of the levator aponeurosis and muscle, depending on the amount of correction needed. The levator can be approached from an anterior or posterior direction.
• • In the anterior approach, an external eyelid incision is made by using the natural lid crease, if present, to allow for direct visualization of the aponeurosis. Once the levator aponeurosis is identified, it is disinserted from the tarsus, advanced and/or resected, and reattached. The amount of advancement depends on the degree of blepharoptosis being treated. The aponeurosis also is attached to the skin to reform the crease.
  • In posterior levator resection, the eyelid is everted, and the conjunctiva is separated from the Mueller muscle and the levator aponeurosis. Double-armed sutures are placed in the conjunctiva. The Mueller muscle and levator are separated from the septum and clamped. Then, the preplaced sutures in the conjunctiva are passed through the levator, and the excess tissue is excised. The sutures are passed through the skin with 1 arm of the double-armed suture taken a bit through the tarsus, and these sutures are tied reforming the eyelid crease.
• If the levator is disinserted or dehisced, the anterior or posterior approach can be used, and the dehiscence or disinsertion repaired.
• In the Fasanella-Servat ptosis procedure, the conjunctiva and tarsus and the Mueller muscle are resected. Two hemostats are placed across the superior tarsal border. The tissue below the hemostats is sutured, and then the tissue is resected.
• The internal levator advancement, known more commonly as the Mueller muscle–conjunctival resection, is performed on the underside of the lid, as in a Fasanella-Servat procedure.
• • This surgery is chosen if the eyelid has had a good response to phenylephrine.
  • The conjunctiva and the Mueller muscle are marked off, clamped with a specialized clamp, sutured, the tissues are resected.
  • The conjunctival layer is then closed.
  • This procedure is believed to advance the levator aponeurosis, thereby elevating the ptotic lid.

Consultations

If a specific etiology of blepharoptosis is identified and has related systemic manifestations, consultation with other specialists is necessary.

• If myasthenia gravis or multiple sclerosis is diagnosed, appropriate follow-up care with a neurologist is warranted.
• If dysthyroid orbitopathy is found, an endocrinologist should be consulted to address the thyroidopathy.
• Patients with Kearns-Sayre disease can have cardiac conduction abnormalities that should be managed by an internist or a cardiologist.
• If the etiology of the ptosis is unclear and associated with ophthalmoplegia, consultation with a neuro-ophthalmic specialist is prudent.

FOLLOW-UP

Section 7 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

Further Outpatient Care

• If surgical correction of blepharoptosis is undertaken, the patient should be observed on days 1-7 after surgery.

In/Out Patient Meds

• After blepharoptosis surgery, a topical antibiotic ointment (with or without a steroid) should be applied twice daily for 5-7 days.
• An oral antibiotic, that is, a penicillin derivative or a cephalosporin, may be given for 5-7 days as well.

Complications

• Uncorrected congenital ptosis can result in amblyopia secondary to deprivation or uncorrected astigmatism.
• • An abnormal eyelid position can have negative psychosocial effects, especially in young children and teenagers.
  • Ostracism can lead to poor academic performance, loss of self-esteem, and alienation.
• In some cases, uncorrected acquired blepharoptosis results in decreased field of vision and frontal headaches.
• • The decreased visual field can affect one's ability to perform activities of daily life.
  • Driving, reading, and navigating a flight of steps can be particularly difficult.
• If correction of blepharoptosis is undertaken, complications related to the surgery can ensue.
• • Because most ptosis surgery is performed with the patient under local anesthesia and with monitored anesthesia care, reactions to anesthetic agents are possible complications.
  • Bleeding and poor response to anesthetic agents are potential intraoperative complications.
  • Bleeding and infection can be devastating complications in the early postoperative period. Prolonged bruising, edema, undercorrection or overcorrection of the ptosis, eyelid asymmetry, and corneal foreign body sensation can be later complications.

Patient Education

• Inform patients that symmetry is difficult, if not impossible, to achieve (see Medical/Legal Pitfalls).

MISCELLANEOUS

Section 8 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical/Legal Pitfalls

• Correction of blepharoptosis without an appropriate examination or exclusion of medically treatable etiologies can result in poor outcomes.
• Aesthetic and functional complications can lead to dissatisfied patients, a reduced referral base, and litigation.
• Patients must be informed that symmetry is difficult, if not impossible, to achieve.
• • If a patient presents with unilateral ptosis, the other eyelid must be evaluated to ensure that contralateral ptosis is not present.
  • Even if contralateral ptosis is not discovered on examination, informing the patient that the uninvolved side might manifest ptosis after surgery may be prudent.
  • Also, when an eyelid is lifted, the amount of dermatochalasia may appear to be increased. The patient should be forewarned of this outcome and of the need for possible blepharoplasty.

MULTIMEDIA

Section 9 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

Media file 1:  Patient with bilateral ptosis before surgery. Note the high lid creases.
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Media file 2:  Same patient as in Media file 1 after bilateral internal levator advancement. No skin incision was made, and no crease reformation was performed.
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Media file 3:  Anterior approach to the levator. White band is the levator aponeurosis (arrow).
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Media file 4:  Left ptosis. Lid crease is absent on the left. The crease is up in the sulcus. Superior sulcus deformity is present on the left and right, and the patient is elevating her brows. The right upper lid should be checked for an underlying or masked ptosis. If the right lid is ptotic, lifting the left lid causes the right lid to droop.
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Media file 5:  Visual field shows functional blockage of superior visual field due to a ptotic lid. Hashed line represents the superior extent of the seen visual field with the lid lifted. Solid line is with the lid in its natural, ptotic position.
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Media file 6:  Congenital ptosis on right. Note the presence of a lid crease.
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Media file 7:  Glasses with a crutch attached (arrow) that can be used to lift the lid if the patient does not desire surgery.
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Media file 8:  Patient with myasthenia gravis. Right lid is more ptotic than the left lid.
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Media file 9:  Same patient as in Media file 8, 3 months later. Note how the ptosis has changed and is more on the left than the right.
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Media file 10:  Patient with bilateral ptosis before surgery.
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Media file 11:  Same patient as in Media file 10 after internal levator advancement. Patient has excessive skin (dermatochalasia) after the lid was lifted, with a pseudoptotic effect more on the left than the right. The dermatochalasia was present before surgery but is more significant afterward. Patient also has brow ptosis.
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REFERENCES

Section 10 of 10

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

• Arslan E, Demirkan F, Unal S, et al. Enhanced frontalis sling with double-fixed, solvent-dehydrated cadaveric fascia lata allograft in the management of eye ptosis. J Craniofac Surg. Nov 2004;15(6):960-4; discussion 965-6. [Medline].
• Beard C. Types of ptosis. In: Beard C, ed. Ptosis. 3^rd ed. St. Louis: Mosby; 1981:39-76.
• Carter SR, Meecham WJ, Seiff SR. Silicone frontalis slings for the correction of blepharoptosis: indications and efficacy. Ophthalmology. Apr 1996;103(4):623-30. [Medline].
• Collin JRO. Ptosis. In: Manual of Systematic Eyelid Surgery. Oxford, England: Butterworth-Heinemann; 1999:41-72.
• Dinges WL, Witherspoon SR, Itani KM, Garg A, Peterson DM. Blepharoptosis and external ophthalmoplegia associated with long-term antiretroviral therapy. Clin Infect Dis. Sep 15 2008;47(6):845-52. [Medline].
• Dutton JJ. Atlas of Clinical and Surgical Orbital Anatomy. Philadelphia: WB Saunders; 1994:120-5.
• Emsen IM. A new ptosis correction technique: a modification of levator aponeurosis advancement. J Craniofac Surg. May 2008;19(3):669-74. [Medline].
• Frueh BR, Musch DC, McDonald H. Efficacy and efficiency of a new involutional ptosis correction procedure compared to a traditional aponeurotic approach. Trans Am Ophthalmol Soc. 2004;102:199-206; discussion 206-7. [Medline].
• Frueh BR, Musch DC, McDonald HM. Efficacy and efficiency of a small-incision, minimal dissection procedure versus a traditional approach for correcting aponeurotic ptosis. Ophthalmology. Dec 2004;111(12):2158-63. [Medline].
• Goldey SH, Baylis HI, Goldberg RA, et al. Frontalis muscle flap advancement for correction of blepharoptosis. Ophthal Plast Reconstr Surg. Mar 2000;16(2):83-93. [Medline].
• Levine MR. Manual of Oculoplastic Surgery. Oxford, England: Butterworth-Heinemann; 1996:75-105.
• Park DH, Baik BS. Advancement of the Müller muscle-levator aponeurosis composite flap for correction of blepharoptosis. Plast Reconstr Surg. Jul 2008;122(1):140-2. [Medline].
• Putterman AM. Cosmetic Oculoplastic Surgery Eyelid, Forehead, and Facial Techniques. London: WB Saunders; 1999:137-59.
• Sakol PJ, Mannor G, Massaro BM. Congenital and acquired blepharoptosis. Curr Opin Ophthalmol. Oct 1999;10(5):335-9. [Medline].
• Tsa CC, Li TM, La CS, et al. Use of orbicularis oculi muscle flap for undercorrected blepharoptosis with previous frontalis suspension. Br J Plast Surg. Sep 2000;53(6):473-6. [Medline].

Article Last Updated: Aug 29, 2008