Introduction
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
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.
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References
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Further Reading
Keywords
adult ptosis, blepharoptosis, droopy lid, droopy eyelid, drooping eyelid, upper eyelid ptosis, lazy eye, bedroom eyes
Overview: Ptosis, Adult