Ophthalmology – Floppy Eyelid Syndrome

Basics

Description

Floppy eyelid syndrome (FES) is characterized by an abnormally loose, lax, and easily everted upper eyelid, typically associated with chronic papillary conjunctivitis. The upper eyelid may spontaneously evert during sleep, exposing the conjunctiva and ocular surface to repeated mechanical irritation.

The disorder may be unilateral or bilateral and has a particularly strong association with obstructive sleep apnea–hypopnea syndrome (OSAHS).

Epidemiology

Floppy eyelid syndrome occurs more commonly in males and is strongly associated with obesity and a high body mass index. Either one or both eyes may be affected.

The condition is particularly important because a large proportion of affected patients also have obstructive sleep apnea.

Risk Factors

The major risk factor is obstructive sleep apnea. Obesity and recurrent mechanical trauma to the eyelids, particularly from sleeping face-down or on one side and repeatedly rubbing the eyes, also contribute.

Patients often sleep preferentially on the side of the more severely affected eye.

Prevention

Recognition and treatment of associated obstructive sleep apnea are important. Treatment with continuous positive airway pressure (CPAP) may improve both the sleep apnea and manifestations of floppy eyelid syndrome.

Reducing chronic eye rubbing and mechanical trauma to the eyelids may also be beneficial.

Pathophysiology

The upper eyelid becomes abnormally lax because of structural abnormalities involving the tarsal plate and elastic tissue.

Repeated mechanical trauma during sleep and recurrent ischemia-reperfusion injury may increase the activity of matrix metalloproteinases, enzymes that degrade elastin. Progressive loss and alteration of elastic fibers weaken the eyelid and allow it to evert easily.

Once the eyelid begins everting during sleep, the exposed conjunctiva and cornea undergo repeated mechanical irritation, creating a cycle of inflammation, ocular surface exposure, and further eyelid damage.

Etiology

The precise cause is not completely understood. Important contributing mechanisms include elongation and weakening of the tarsal plate, chronic mechanical trauma, repeated eye rubbing, and abnormalities of elastic tissue.

Obstructive sleep apnea is strongly associated with the condition and may contribute through intermittent hypoxia and ischemia-reperfusion injury.

Associated Conditions

The most important association is obstructive sleep apnea–hypopnea syndrome, reported in a very high proportion of patients with classic floppy eyelid syndrome.

Other associations include obesity, keratoconus, chronic ocular surface disease, ptosis, and eyelid malposition.

Keratoconus may be particularly prominent on the side on which the patient habitually sleeps or rubs the eye.

OSA itself has important systemic associations, including cardiovascular disease, stroke, metabolic syndrome, cognitive and emotional disturbances, and excessive daytime sleepiness.

Diagnosis

History

Patients commonly report chronic ocular irritation, foreign-body sensation, redness, tearing, and mucous discharge. Symptoms are often particularly severe upon awakening in the morning.

Some patients are aware that their upper eyelid spontaneously turns outward while sleeping. They may report sleeping predominantly on the affected side of the face.

A history of loud snoring, witnessed apneic episodes, daytime somnolence, obesity, or previously diagnosed sleep apnea should be specifically sought.

Smoking and habitual eye rubbing should also be documented.

Physical Examination

The characteristic finding is an extremely lax upper eyelid that can be everted easily with minimal manipulation.

The upper tarsal plate often feels unusually soft and rubbery, and significant horizontal eyelid laxity may be present. The lower eyelid may also demonstrate increased laxity.

The eyelashes may point downward, producing lash ptosis.

Eversion of the upper eyelid commonly reveals chronic papillary conjunctivitis.

Other findings may include ptosis and lagophthalmos. Incomplete eyelid closure and nocturnal exposure may produce punctate epithelial keratitis, particularly when ocular surface disease is advanced.

Diagnostic Testing

Patients suspected of having associated obstructive sleep apnea should undergo appropriate evaluation, commonly including a sleep study or polysomnography.

This aspect of the evaluation is particularly important because untreated sleep apnea carries significant systemic morbidity.

The ophthalmic examination should carefully assess the cornea for punctate epithelial erosions, abrasions, ulceration, thinning, scarring, or other evidence of exposure-related disease.

Differential Diagnosis

Conditions that may resemble floppy eyelid syndrome include ptosis, chronic blepharoconjunctivitis, ectropion, vernal keratoconjunctivitis, giant papillary conjunctivitis, and superior limbic keratoconjunctivitis.

The combination of marked upper eyelid laxity, easy spontaneous eversion, chronic papillary conjunctivitis, and symptoms that are worse after sleep strongly favors floppy eyelid syndrome.

Treatment

First-Line Treatment

Initial treatment is directed toward protecting and lubricating the ocular surface.

Artificial tears may be used during the day, while more viscous gels or ophthalmic ointments provide longer-lasting protection and are particularly useful before sleep.

At night, the eyelids may be gently taped closed or protected with an eye shield to prevent spontaneous eversion and reduce mechanical trauma to the ocular surface.

Treatment of Obstructive Sleep Apnea

Associated obstructive sleep apnea should be actively treated. CPAP therapy is a central treatment for appropriate patients and may improve the eyelid disorder in addition to reducing the systemic consequences of sleep apnea.

Depending on the severity and cause of OSA, additional management may include behavioral measures, weight management, mandibular advancement devices, or selected surgical procedures.

Additional Medical Treatment

In selected patients with significant inflammatory or meibomian gland-related disease, systemic therapy such as doxycycline may be considered by the treating clinician.

Treatment should also address associated ocular surface inflammation, blepharitis, keratoconus, or exposure keratopathy when present.

Surgical Treatment

Surgery is considered when conservative treatment and management of associated sleep apnea fail to provide adequate control.

When possible, significant obstructive sleep apnea should be addressed before definitive eyelid surgery.

Surgical options primarily aim to restore normal eyelid tension. These include horizontal eyelid shortening or canthal tightening procedures and, in selected cases, full-thickness eyelid resection.

Surgical treatment may simultaneously improve associated ptosis in some patients.

Recurrence can occur, and some patients eventually require additional eyelid procedures.

Follow-Up

Patients with active ocular surface disease require relatively close ophthalmologic follow-up, particularly when there is corneal involvement.

The cornea should be monitored for epithelial breakdown, exposure keratopathy, ulceration, infection, thinning, and scarring.

Long-term follow-up should also assess the effectiveness of sleep apnea treatment and the persistence or recurrence of eyelid laxity.

Patient Education

Patients should understand the particularly important relationship between floppy eyelid syndrome and obstructive sleep apnea.

Symptoms such as loud snoring, witnessed apnea, morning headaches, excessive daytime sleepiness, or unexplained fatigue should prompt appropriate sleep evaluation.

Patients should also be instructed to minimize eye rubbing and nocturnal mechanical trauma and to use prescribed lubrication and nighttime eyelid protection consistently.

Prognosis

The prognosis is generally favorable when both the ocular surface disease and associated sleep apnea are appropriately treated.

Treatment of obstructive sleep apnea with CPAP may improve floppy eyelid syndrome in some patients and, more importantly, reduces the substantial systemic risks associated with untreated sleep apnea.

Persistent severe eyelid laxity may require surgical correction, and recurrence after surgery is possible.

Complications

Chronic exposure and mechanical trauma can lead to corneal abrasion, epithelial breakdown, ulceration, infection, scarring, thinning, and, in severe cases, corneal perforation.

Floppy eyelid syndrome and its strong association with obstructive sleep apnea have also been reported alongside important ophthalmic disorders such as keratoconus, normal-tension glaucoma, nonarteritic ischemic optic neuropathy, and papilledema.