Floppy eyelid syndrome

From EyeWiki


Disease

Floppy eyelid syndrome (FES) is an under-diagnosed entity characterized by chronic papillary conjunctivitis in upper palpebral conjunctiva that is poorly respondent to topical lubrication and steroids.

Etiology

The extensive lid laxity can be attributed to significant decrease in elastin content when was tested by special stains, immunohistochemistry, and electron microscopy. Collagen content was not noted to be decreased in FES patients. This was noticed to cause spontaneous eversion of the eyelid leading to chronic irritation and inflammation of the lid and palpebral conjunctiva and being mechanically irritated by constant rubbing of the palpebral conjunctiva with the pillow. The theory was further confirmed by the fact that patients who sleep on one side more than the other side tend to have more severe changes on that side. This finding suggests mechanical injury as the primary cause of the papillary conjunctivitis. Other postulated pathophysiology suggests that the cause of the chronic conjunctivitis is poor apposition of the lax upper eyelid to the globe with inadequate spreading of the tear film. This condition leads to corneal and conjunctival compromise, rather than direct mechanical irritation.

Association

FES is known to be associated with obesity, obstructive sleep apnea, Down syndrome, and keratoconus. Keratoconus can be linked to frequent rubbing and mechanical effect on the cornea.

History

Patients initially present with non-specific symptoms including eye irritation and a long history of unilateral or bilateral ocular redness and discharge. They may describe the eyelids spontaneously "flipping over" when they sleep due to rubbing on the pillow. Some patients may have a history of preexisting obstructive sleep apnea and/or history of heavy snoring. These symptoms in association with increased body mass index and a diagnosis of obstructive sleep apnea should alert the ophthalmologist about the high likelihood of having floppy eyelid syndrome. However, many reports have also documented the symptoms and signs of floppy eyelid syndrome in patients of both genders and without a history of obesity of sleep disorders.

Physical examination

Eversion (Image courtesy of Cat Burkat, MD FACS)

Easy eversion of the eyelid without excess manipulation or even spontaneous eversion is an important examination mark for FES with subsequent increased horizontal laxity and redundancy of the lid (See Figure). The lateral upper eyelid may appear elongated and imbricate (overlap) over the edge of the lower lid margin. Chronic severe papillary conjunctivitis with whitish mucus discharge.

Superficial punctate erosions, corneal, abrasions and microbial keratitis,and eyelashes ptosis are common clinical findings. Dry eyes with low tear meniscus.

May be associated with keratoconus.

Diagnostic procedures

It should be directed to address possible associated conditions including obstructive sleep apnea and keratoconus and other morbidity consequences of obesity and obstructive sleep apnea. Patient should be managed in cooperation with internal medical team and sleep disorder specialist.

Differential diagnosis

  • Blepharitis
  • Allergic conjunctivitis
  • Atopic keratoconjunctivitis
  • Superior limbic keratoconjunctivitis
  • Ptosis
  • Giant papillary conjunctivitis
  • Contact lens complication
  • Dermatochalasis
  • Ectropion

Management

Supportive measures such as ocular lubrication and temporary antihistamine in addition to prevention of the upper lid from everting during sleep by taping the eye or eye shield can be effective in solving the patient's complains. If minimal response to medical treatment is achieved, surgical procedures such as horizontal eyelid shortening can help to relieve ocular symptoms and provide good functional and cosmetic results.

If medial upper lid laxity is predominant, excision of this area may stabilize the upper eyelid in an anatomic fashion.

General treatment

Obstructive sleep apnea is a potentially fatal disorder. Frequent episodes of apnea and hypopnea can lead to systemic and pulmonary hypertension and, ultimately, congestive cardiomyopathy. Patients need to be managed by a sleep specialist to prevent these potentially fatal conditions.

Additional Resources

References

  1. Orbit, Eyelids and Lacrimal System, Section 7. Basic and Clinical Science Course, AAO, 2011-2012.
  2. Burkat CN, Lemke BN. Acquired lax eyelid syndrome: an unrecognized cause of the chronically irritated eye. Ophthal Plast Reconstr Surg. 2005 Jan;21(1):52-8.
  3. Fowler AM, Dutton JJ Floppy eyelid syndrome as a subset of lax eyelid conditions: relationships and clinical relevance. Ophthal Plast Reconstr Surg. 2010;26(3):195-204.
  4. Valenzuela AA, Sullivan TJ. Medial upper eyelid shortening to correct medial eyelid laxity in floppy eyelid syndrome: a new surgical approach. Ophthal Plast Reconstr Surg. 2005;21(4):259-63.
  5. Chambe J, Laib S, Hubbard J, Erhardt C, Ruppert E, Schroder C, et al. Floppy eyelid syndrome is associated with obstructive sleep apnea: a prospective study on 127 patients. J Sleep Res. Oct 11 2011.
  6. Periman LM, Sires BS. Floppy eyelid syndrome: a modified surgical technique. Ophthal Plast Reconstr Surg.2002;(18)5:370-2.