Blepharoptosis

An ophthalmological condition known as blepharoptosis causes the upper eyelid to hang lower than it should. The upper eyelid generally covers the cornea by 1 to 2 millimeters. Blepharoptosis causes the eyelid to close even further, which impairs eyesight. To see clearly, a person with this issue may need to manually elevate their eyelid.

Blepharoptosis Definition

When the eye is in the primary gaze, blepharoptosis refers to an abnormally low-lying upper eyelid edge. Normally, the superior section of the cornea is covered by 1-2 mm of the upper eyelid. Dermatochalasis, or upper lid skin redundancy, is a distinct feature that may coexist with blepharoptosis. Because the surgical therapy for dermatochalasis and blepharoptosis is different, they should be identified.

Blepharoptosis Causes

Both congenital and acquired blepharoptosis are possible.

Congenital Blepharoptosis

The levator palpebrae superioris's isolated localized myogenic dysgenesis is typically the cause of congenital ptosis. A very tiny percentage of congenital blepharoptosis cases may be caused by chromosomal or genetic abnormalities, as well as neurological dysfunction.

Telecanthus, congenital ptosis, small palpebral fissures, and epicanthus inversus are all hallmarks of blepharophimosis syndrome.
Congenital third cranial nerve palsy.
Congenital Horner syndrome is manifested by minor ptosis, miosis, anhidrosis, and heterochromia.
Due to improper innervation of the ipsilateral levator muscle by the motor nerve to the external pterygoid muscle, Marcus Gunn jaw-winking syndrome develops. Patients experience eyelid elevation during chewing or after shifting their jaw to the opposite side.

Acquired Blepharoptosis

The aponeurotic variant of acquired blepharoptosis predominates. Stretching, dehiscence or disinsertion of the levator aponeurosis can cause aponeurotic blepharoptosis. When patients have aponeurotic blepharoptosis, it is also referred to as involutional ptosis because the anatomic alterations are age-related. Less often occurring causes of acquired blepharoptosis include myogenic, neurogenic, traumatic, and mechanical factors.

Myasthenia gravis, chronic progressive external ophthalmoplegia, oculopharyngeal muscular dystrophy, and myotonic dystrophy are among the conditions that can cause myogenic blepharoptosis.
Horner's syndrome, a form of third nerve palsy, may cause neurogenic blepharoptosis.
After an eyelid laceration, there may be transection of the upper eyelid elevators or interruption of the neural circuit, both of which can result in traumatic blepharoptosis.
A mass on the eyelid, such as a neurofibroma or hemangioma, or cicatrization as a result of an infection or surgery can cause mechanical ptosis.

Blepharoptosis Pathophysiology

Superior Levator Palpebrae and Mueller Muscle are the muscles that lift the upper eyelids. The levator palpebrae superioris arises from the orbital apex, continues anteriorly, and travels as aponeurosis inferiorly at the Whitnall ligament. The anterior portion of the tarsal plate receives aponeurosis. The upper eyelid crease is formed by attachments it sends to the skin. A striated muscle, the levator palpebrae superioris, is innervated by the superior subdivision of the oculomotor nerve. This muscle serves as the upper eyelid's primary elevator. The underside of the levator superioris is the origin of the Mueller muscle, a smooth muscle with sympathetic innervation. It extends to a length of about 12 mm, attaches superiorly on the tarsal border, and raises the upper eyelid by about 2 mm. Failure of either one or both of the upper eyelid elevator muscles causes blepharoptosis.

Blepharoptosis Symptoms

History

Patients typically report the affected eye being undersized, looking exhausted, having a limited range of vision, and having headaches. Any age can experience acquired blepharoptosis, but older persons are more likely to experience it. From birth, congenital blepharoptosis manifests. Blepharoptosis does not have a predilection for any one race or gender. Additional information is required regarding the beginning of ptosis, aggravating or alleviating factors, ptosis in the family, recent botulinum toxin injections, and history of trauma or ocular surgery. History typically offers extremely strong information about the causes of blepharoptosis. To prevent potential complications after surgery, it is crucial to inquire about any anticoagulant usage or bleeding, a family history of malignant hyperthermia, and heart disease.

Blepharoptosis Signs

Documentation of refractive error and visual acuity is necessary. The position of the eyebrows and any excess skin on the eyelids should be observed. It is important to do a thorough external examination and palpate the orbital rim and eyelids. A lid mass may add weight to the lid, which will trigger blepharoptosis. The following measures should be conducted before applying topical eye drops:

The space between the upper and lower eyelids in vertical alignment with the pupil's center is known as the palpebral fissure.
The distance between the upper eyelid margin and the pupillary light reflex center with the eye in primary gaze is known as the marginal reflex distance-1 (MRD-1).
The distance between the lower eyelid line and the pupillary light reflex center with the eye in primary gaze is known as the marginal reflex distance-2 (MRD-2).
When the frontalis muscle is held passive at the brow, the levator function is the distance that the eyelids travel from the downgaze to the upgaze position. A measurement of more than 10 mm is regarded as excellent, whilst a measurement of 0-5 mm is regarded as poor.
Presence of lid crease and its height

When a patient has unilateral blepharoptosis, the affected eyelid should be manually raised, and the contralateral eyelid should be evaluated for any Hering phenomena or masked blepharoptosis.

Examining the patient is necessary to check for lagophthalmos, proptosis or enophthalmos, and the presence of the Bell phenomenon. As certain patients with blepharoptosis may have restricted ocular motility, such as in myasthenia gravis and chronic progressive external ophthalmoplegia, ocular motility needs to be assessed. It's important to check patients with congenital blepharoptosis for Marcus Gunn jaw-winking syndrome. For Horner's syndrome, the discrepancies in iris and pupillary size between the eyes should be investigated.

It is crucial to check the cornea, tear meniscus, and tear break-up time with fluorescein stain to see if dry eye is present. The Mueller muscle can be stimulated using sympathomimetic eyedrops. If a positive response is seen, Mueller muscle-conjunctival resection may be used to treat the ptosis.

Blepharoptosis Diagnosis

For the majority of patients, a clinical examination is sufficient. Visual fields are typically requested to demonstrate the effects of blepharoptosis on peripheral vision. Finding the source of the blepharoptosis requires laboratory and imaging testing in a small number of cases.

Laboratory test

Patients suspected of having myasthenia gravis may have a single-fiber electromyography, an edrophonium chloride (Tensilon) test, and a serum analysis for acetylcholine receptor antibodies ordered.

Electrocardiograms, electroretinograms, electromyograms, and mitochondrial assays should all be taken into account in patients with persistent progressive external ophthalmoplegia.

Diagnostic Imaging

Patients with blepharoptosis and neurologic impairments should have imaging tests for the brain, orbits, or cerebrovascular system. Patients who experience blepharoptosis and are thought to have an inflammatory or infiltrative orbital disease should undergo an orbital CT or MRI scan. It is necessary to image the head and neck for acquired Horner's syndrome.

Blepharoptosis Treatment

Surgery is the main method of blepharoptosis treatment. Only in mild cases of congenital ptosis, where amblyopia, strabismus, and aberrant head posture are absent, is observation recommended. Surgery is performed as soon as feasible if there is a chance of amblyopia, strabismus, or a significant aberrant head posture. You can have blepharoptosis surgically corrected at any moment to improve your field of vision or your appearance.

Blepharoptosis should be reported to the relevant doctor for further treatment if it indicates a systemic illness such as myasthenia gravis or Kearns-Sayre disease. Before trying surgical repair, it is recommended to collect external photographic documentation.

Medical Treatment

Myasthenia gravis patients may get better with medical care. For some patients, sympathomimetic topical eye drops such as apraclonidine and phenylephrine provide a brief, transitory lift of the upper eyelid. In July 2020, the FDA approved the use of topical oxymetazoline hydrochloride (0.1%) for blepharoptosis.

Blepharoptosis Surgery

Depending on the severity of the condition, congenital ptosis can be surgically corrected at any age. Early intervention is necessary if there is a chance that amblyopia or a severe aberrant head position would develop. There are various surgical methods available to correct blepharoptosis. The best procedure is selected based on the purpose of the treatment, the underlying diagnosis, the surgeon's preferences, and the level of levator function. Patients should understand that symmetry is challenging to achieve. Patients with dry eyes decreased corneal sensitivity, absent Bell phenomenon, double elevator palsy, or progressive external ophthalmoplegia should be treated with extreme caution to prevent exposure keratopathy after surgery. The patient's strabismus must be treated first if they also have blepharoptosis.

Mueller muscle-conjunctival resection

Patients with mild to moderate aponeurotic blepharoptosis (these patients have very good levator function) can benefit from this approach. Even with strong levator function, this procedure doesn't work too well for patients with congenital blepharoptosis since the levator palpebrae superioris muscle is abnormal. A positive response to topical sympathomimetic eye drops serves as a reliable predictor of the success of this kind of surgery. Compared to other surgical methods, this one has the lowest likelihood of changing the form of the eyelid.

For a 1 mm correction of blepharoptosis from the tip of the upper tarsus, the conjunctiva and Mueller muscle are defined on the conjunctival side, and the defined conjunctiva and Mueller muscle area is clamped. To remove the tissues above the clamp, a continuous suture is inserted beneath the clamp and externalized through the skin at both ends of the defined area.

The Fasanella-Servat ptosis correction method is similar. However, the conjunctiva, Mueller muscle, and a portion of the upper tarsus must be removed. A stronger elevation is produced by including a portion of the tarsal plate in the resection. Tarsus should not be removed excessively since this could compromise the structural integrity of the eyelid.

Levator advancement or resection

Based on the degree of blepharoptosis, this procedure entails shortening the levator aponeurosis. Patients with good and acceptable levator function (>5mm) can benefit from it.

An incision in the crease of the eyelid is used for the procedure. Preaponeroutic fat is pulled away from the levator aponeurosis as the orbital septum is opened. The levator aponeurosis is separated from the tarsus once the muscle has been located, and dissection may then continue between the levator aponeurosis and Mueller’s muscle. After that, the levator aponeurosis is advanced and/or removed, and it is temporarily attached to the tarsus with one to three sutures and partial thickness bites. The degree of blepharoptosis determines the amount of advancement and/or removal. At this point, if the patient is conscious, the lid height and shape are inspected. The tarsal lamellar bites are then permanently tightened after a suitable height and shape are reached. To restore the crease, the skin is closed by inserting a portion of the levator aponeurosis.

Frontalis suspension

Patients with inadequate (4 mm) or absent levator function undergo this treatment. Numerous autogenous and allogeneic materials can be utilized as slings. Frontalis muscle and fascia lata flaps, preserved fascia lata (from a tissue bank), autologous temporalis fascia, silicone, Alloderm, and Gore-Tex sutures have all been used. The most successful surgical outcome was discovered to be achieved with autogenous fascia lata. The sling joins the eyelid and brow, and when the brow is raised, the eye opens. After surgery, it may take patients several months before they can close their eyelids while they sleep. During this time, significant lubrication is required.

The greatest cosmetic result for patients with unilateral severe blepharoptosis is achieved by bilateral frontalis sling. However, convincing the patient and their family to have surgery on the healthy contralateral levator muscle is frequently challenging.

The surgical repair method of jaw-winking blepharoptosis for patients with Marcus Gunn syndrome is debatable. Depending on the degree of the ptosis and levator function, blepharoptosis repair alone (with levator advancement or frontalis sling) may be adequate if the jaw-winking is minor. Extirpation of the levator muscle and the implantation of a frontalis sling may be required if the jaw-winking is severe.

Blepharoptosis Follow-Up

Blepharoptosis surgery is typically performed as an outpatient operation. To lessen swelling and bruising, apply cold compresses to the patient's eyes while they are awake for 20 minutes every 1-2 hours for 2-3 days. Patients are given a topical antibiotic ointment (with or without a steroid) to apply twice daily for 5-7 days to the incision site and the eye. Excessive lubrication is required for patients whose surgical lagophthalmos is expected. Following surgery, patients are often seen one to two weeks later. The patients are assessed for over- and under-correction, infection, granuloma formation, exposure keratopathy, and other eye conditions. Amblyopia patients must continue receiving treatment.

Blepharoptosis Complications

Amblyopia due to deficiency or untreated astigmatism may result from congenital ptosis. Frontal headaches and a restricted field of vision are effects of acquired blepharoptosis. Significant psychosocial impacts of blepharoptosis can result in poor academic and professional performance.

Bleeding, infection, edema, under- or overcorrecting the ptosis, asymmetry of the eyelids, granuloma formation, corneal foreign body sensation, and exposure keratopathy can all complicate the surgical correction of blepharoptosis. Most of these issues are relatively manageable if identified early and treated appropriately.

Blepharoptosis Prognosis

The available medical and surgical methods for treating blepharoptosis typically produce positive results. Over time, recurrence is not rare. This outcome can require multiple surgeries, particularly in cases of congenital ptosis.

Conclusion

Patients seeking oculoplastic surgery frequently have blepharoptosis of the upper eyelid. Although there are many different kinds of ptosis, simple congenital ptosis in young patients and senile ptosis in adults are the two most common clinical occurrences. It is crucial to differentiate between these and other, less common kinds of ptosis, such as those that are neurogenic, myogenic, and posttraumatic, through patient examination. These latter conditions can call for particular therapeutic approaches. Surgery is usually an effective way to treat ptosis.