Ptosis, also referred to as blepharoptosis, 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.[1] See the images below.
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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 pat....
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Visual field shows functional blockage of superior visual field due to a ptotic lid. Hashed line represents the superior extent of the seen visual fie....
Ptosis can be classified as congenital, as shown below, or acquired.[2] 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.[3]
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Congenital ptosis on right. Note the presence of a lid crease.
Most cases of acquired ptosis are secondary to aponeurotic causes, such as involutional changes, a disinsertion, or a dehiscence. Identification of the underlying pathophysiologic mechanism is paramount to institute proper treatment.
Ptosis is the result of dysfunctioning of one or both upper eyelid elevator muscles. These elevator muscles are the levator palpebrae superioris and the Mueller muscle.
The levator palpebrae superioris is a striated muscle 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. The 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, originates 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 associated with ptosis usually results from 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 be dissatisfied with their appearance.
Race
No racial predilection has been described for ptosis.
Sex
No sexual predilection has been described for ptosis.
Age
Acquired ptosis can occur at any age, but it is commonly seen in older adults. Congenital ptosis occurs at birth.
Obtain a thorough medical and ophthalmic history in patients with ptosis.
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 their visual fields.
If the patient has not been under the care of an ophthalmologist, a complete ocular examination is required.
Quantification and qualification of the ptosis is needed 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 of 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 may affect the amount of ptosis repair.
Pseudoptosis can result from dermatochalasis, 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 to exclude 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.
Ptosis can be caused by problems with elevator muscles of the eyelid or the levator aponeurosis; central or peripheral nerve abnormalities, trauma, inflammation, or lesions of the lid or orbit.[4]
Aponeurotic ptosis
Aponeurotic ptosis is the most common cause of acquired ptosis.
Involutional changes, dehiscence, or disinsertion of the levator aponeurosis are common causes.
Chronic inflammation or intraocular surgery (eg, cataract surgery) necessitating speculum use can stretch the levator aponeurosis, causing dehiscence of the levator 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 ptosis
Neurogenic ptosis may be congenital or acquired. Congenital neurogenic ptosis is usually due to Horner syndrome or third nerve dysfunction. Acquired neurogenic ptosis causes include Horner syndrome, third nerve dysfunction, 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, neoplasms, 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 who experience daily headaches and have 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 stems from innervational anomalies. Marcus-Gunn jaw winking and posttraumatic ptosis are 2 examples of this interesting etiology. Importantly, microvascular diabetic neuropathies never result in synkinetic neurogenic ptosis.
Myogenic ptosis
Myogenic ptosis usually is congenital, but 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 ptosis
Traumatic ptosis can occur after eyelid laceration with transection of the upper eyelid elevators or disruption of neural input.
Mechanical ptosis
Mechanical ptosis can stem from the presence of eyelid neoplasms, for example, neurofibromas or hemangiomas or from cicatrization secondary to inflammation or surgery.
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 ptosis 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 ptosis is undertaken, complications can occur. 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 occur in the early postoperative period. Prolonged bruising, edema, undercorrection or overcorrection of the ptosis, eyelid asymmetry and abnormal shape (i.e. peaking), and corneal foreign body sensation can be later complications.
If myasthenia gravis is suspected in a patient with ptosis, perform a serum assay for acetylcholine receptor antibodies and antistriated muscle antibody. Muscle-specific tyrosine kinase (MUSK) levels can be obtained when acetylcholine receptor antibodies levels are negative and the suspicion for generalized myasthenia is high.
The ice test has been shown to improve ptosis in patients with myasthenia.[5]
An edrophonium chloride (Tensilon) test or single-fiber electromyography may be needed to definitely exclude the diagnosis.
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 thyroid function studies.
If ptosis is present with other neurologic deficits, imaging of the brain, orbits, or cerebrovascular system should be performed. An emergent CT angiography of the brain is necessary when ptosis is accompanied by other signs of a third nerve palsy. MRI of the brain with and without gadolinium is the imaging modality of choice to exclude structural cerebral etiologies, including middle cranial fossa neoplasia and demyelinating disorders.
CT scanning without contrast can be used to exclude dysthyroid orbitopathy and intraorbital neoplasia.
In acquired Horner syndrome, MRI or CT scan of the brain, MRA of neck, CT scan or X-ray of the spine, and CT scan or X-ray of the chest (especially of the apex of the lung) are needed to exclude a structural lesion along the sympathetic pathway.
Sympathomimetic agents can be used to stimulate the Mueller muscle, as follows:
2.5% phenylephrine
0.5% apraclonidine (Iopidine)
1.0% apraclonidine (Iopidine)
Ten percent phenylephrine has not be shown to have increased clinical efficacy in predicting the outcome of Muller muscle–conjunctival resection. Therefore, the authors do not feel the use of 10% phenylephrine is necessary.[6]
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 ptosis. If a good response is observed, the ptosis can be repaired by advancing the internal levator (Mueller muscle–conjunctival resection).
Many surgical techniques have been described for ptosis correction.[7, 8, 9, 4] A surgeon may prefer one technique to another.
This brief discussion is a guide for approaching ptosis correction using the most common surgical techniques.[10, 11, 12]
Frontalis sling
If levator function is poor (< 4 mm) or absent, the use of frontalis slings can achieve desirable postoperative results.[13, 14, 15]
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 frontalis muscle.
With elevation of the eyebrow, the eye opens, and the orbicularis oculi is used to close the eye.
Levator advancement
A levator advancement or resection results in shortening of the levator aponeurosis and muscle. The levator can be approached from an anterior or posterior direction.[16, 17]
In the anterior approach (see the image below), 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 ptosis being treated. The aponeurosis also is attached to the skin to reform the crease.
Small paracentral incision techniques to access the levator and/or aponeurosis have also been used. This minimally invasive approach allows for less disruption of the fascia attachments but also provides less visualization. Often, a single-suture technique is used to reestablish the connection between the levator and tarsus.[18]
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Anterior approach to the levator. White band is the levator aponeurosis (arrow).
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.
Fasanella-Servat ptosis procedure
In the Fasanella-Servat ptosis procedure, the conjunctiva, 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.
Mueller muscle–conjunctival resection
The internal levator advancement (see the images below), known more commonly as the Mueller muscle–conjunctival resection, is performed on the underside of the lid, as in a Fasanella-Servat procedure.[19]
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.
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Patient with bilateral ptosis before surgery. Note the high lid creases.
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Same patient as in the previous image after bilateral internal levator advancement. No skin incision was made, and no crease reformation was performed....
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Patient with bilateral ptosis before surgery.
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Same patient as in the previous image after internal levator advancement. Patient has excessive skin (dermatochalasia) after the lid was lifted, with ....
Full-thickness resection
A full-thickness resection can be used in combination with an external levator advancement. After a blepharotomy is performed, the superior tarsus can be resected for the length of the eyelid. Remember that aggressive tarsal resection can result in eyelid instability. Therefore, the resection should be limited to a height of 4 mm.
After ptosis surgery, a topical antibiotic ointment (with or without a steroid) can be applied twice daily for 5-7 days. The authors do not routinely use ointments to dress wounds.
Perioperative IV antibiotics can be given or an oral antibiotic prescribed for 5-7 days as well. The authors do not routinely prescribe antibiotics.
Pain is usual minimal in the post-operative period but Tylenol #3 or Vicodin can be prescribed if necessary.
Adam J Cohen, MD, Physician/CEO, Eyelid and Facial Plastic Surgery and MediSpa
Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Triad Inc.<br/>Serve(d) as a speaker or a member of a speakers bureau for: Mimedx.
Coauthor(s)
Michael Mercandetti, MD, MBA, FACS, Private Practice
Disclosure: Nothing to disclose.
Specialty Editors
Simon K Law, MD, PharmD, Clinical Professor of Health Sciences, Department of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine
Disclosure: Nothing to disclose.
J James Rowsey, MD, Former Director of Corneal Services, St Luke's Cataract and Laser Institute
Disclosure: Nothing to disclose.
Chief Editor
Hampton Roy, Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Disclosure: Nothing to disclose.
Additional Contributors
Ron W Pelton, MD, PhD, Private Practice, Colorado Springs, Colorado
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.
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.
Congenital ptosis on right. Note the presence of a lid crease.
Patient with myasthenia gravis. Right lid is more ptotic than the left lid.
Same patient as in the previous image, 3 months later. Note how the ptosis has changed and is more on the left than the right.
Glasses with a crutch attached (arrow) that can be used to lift the lid if the patient does not desire surgery.
Anterior approach to the levator. White band is the levator aponeurosis (arrow).
Patient with bilateral ptosis before surgery. Note the high lid creases.
Same patient as in the previous image after bilateral internal levator advancement. No skin incision was made, and no crease reformation was performed.
Patient with bilateral ptosis before surgery.
Same patient as in the previous image 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.
Patient with bilateral ptosis before surgery. Note the high lid creases.
Same patient as in the previous image after bilateral internal levator advancement. No skin incision was made, and no crease reformation was performed.
Anterior approach to the levator. White band is the levator aponeurosis (arrow).
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.
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.
Congenital ptosis on right. Note the presence of a lid crease.
Glasses with a crutch attached (arrow) that can be used to lift the lid if the patient does not desire surgery.
Patient with myasthenia gravis. Right lid is more ptotic than the left lid.
Same patient as in the previous image, 3 months later. Note how the ptosis has changed and is more on the left than the right.
Patient with bilateral ptosis before surgery.
Same patient as in the previous image 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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