paulFloppy Eyelid Syndrome: A Diagnostic Challenge

By: Paul M. Karpecki, OD, FAAO
Clinical Director – PECAA

Patients who present with floppy eyelid syndrome frequently are misdiagnosed. Here’s why.
Floppy eyelid syndrome (FES) is a relatively rare condition that often masquerades as dry eye disease. Patients may exhibit clinical signs of chronic superficial punctate keratopathy (SPK) and blepharitis, as well as more acute symptoms of ocular dryness and grittiness. In this month’s column, we’ll examine the most effective diagnostic and management strategies for FES and its associated ophthalmic complications.

Findings

image1The lack of tight eyelid closure in patients with FES yields chronic corneal and conjunctival inflammation. This persistent irritation often leads to edema, chemosis, staining and occasional epithelial abrasions.1
Everting the upper eyelids frequently reveals an extremely elastic eyelid, and patients may even complain of spontaneous lid eversion. The upper eyelid also may reveal the presence of papillae secondary to chronic trauma.2
Many patients with FES may have a genetic or developmental problem that reduces the inherent structural rigidity of collagen or elastin tissues.3,4 Multiple studies have indicated that patients with obstructive sleep apnea (OSA) experience a partial collapse of the pharynx when breathing in while sleeping, resulting in loud snoring and gasping for air. Some authors have theorized that, like the upper tarsal plate, the pharynx may lack the necessary structural integrity for proper function.5,6 Other researchers cite that a high percentage of matrix metalloproteinase enzymes located on the ocular surface and eyelids may, in fact, damage the elastic fibers present in the eyelid.7
Further, patients with FES are more likely to develop keratoconus secondary to a potential structural integrity defect or repetitive nocturnal eye rubbing.8

Diagnosis

FES frequently is under-diagnosed or misdiagnosed by eye care providers because patients often present with concurrent SPK and advanced MGD. So, you must carefully evaluate the individual for more unique signs and symptoms of FES to help confirm the diagnosis.
For example, be sure to look for a stringy mucous discharge or evidence of severe ocular itch. FES patients also may report a history of corneal abrasions in the complete absence of traumatic insult. Additionally, because of the strong association between FES and OSA, remember to ask the patient about symptoms of fatigue, headache, and sleeping or breathing problems.8,9

Management

Treating the symptoms associated with FES may work for mild cases but, as the condition advances, eyelid-tightening surgery typically is required. Initial treatments for the ocular surface inflammation and SPK may include ample lubrication with artificial tears. For more severe presentations, topical difluprednate 0.5% BID and loteprednol 0.5% QID could be used to control the initial inflammation. Be sure, however, to taper the loteprednol to BID in cases that require long-term treatment and monitor IOP.
If a corneal abrasion is present, you must treat it with ample lubrication and topical antibiotics before initiating anti-inflammatory therapy. In some instances, the patient may require a bandage lens or overnight patching to help heal the abrasion. Patients with corneal abrasions also may benefit from the use of bland ointments at night. Additionally, antibiotic ointments (i.e., erythromycin) or corticosteroid ointments (i.e., loteprednol or fluoromethalone) may facilitate rapid healing in highly inflamed cases that exhibit significant papillae.
In severe or non-responsive cases of FES, eyelid tightening surgery often is the only effective treatment option.10 Following the procedure, you must maintain the best possible ocular surface conditions via artificial tears, punctal plugs and therapeutics for chronic SPK.
Finally, don’t forget to manage the associated systemic disease components, including sleep apnea. Refer patients with OSA to a sleep specialist or pulmonologist.11 Interestingly, OSA management actually may actually help reverse or eliminate the signs and symptoms of FES.12 It is also important to be aware of serious ocular findings that are more common in patients with both FES and OSA, including glaucoma and non- arteritic anterior ischemic optic neuropathy.13
FES is a rare condition––and typically, it is these less common conditions that are often overlooked or misdiagnosed. Because the symptoms of FES are similar to those associated with ocular surface disease, it makes the diagnosis significantly more difficult to confirm. But, with cautious observation and a thorough differential, you can help identify and manage patients FES patients early in the disease process and minimize their discomfort.

1. Moscato EE, Jian-Amadi A. Floppy eyelid syndrome. Compr Ophthalmol Update. 2007 Mar-Apr;8(2):59-65.
2. Kestelyn P, van Liefferinge T, Goes F, et al. A rare case of chronic papillary conjunctivitis diagnosed after several years of evolution. Clinical and pathological findings. Bull Soc Belge Ophtalmol. 1993;248:61-6.
3. Karger RA, White WA, Park WC, et al. Prevalence of floppy eyelid syndrome in obstructive sleep apnea-hypopnea syndrome. Ophthalmology. 2006;113(9):1669-74.
4. Lee WJ, Kim JC, Shyn KH. Clinical evaluation of corneal diseases associated with floppy eyelid syndrome. Korean J Ophthalmol. 1996 Dec;10(2):116-21.
5. Netland PA, Sugrue SP, Albert DM, et al. Histopathologic features of the floppy eyelid syndrome. Involvement of tarsal elastin. Ophthalmology. 1994 Oct;10(1)1:174-81.
6. Ezra DG, Beaconsfield M, Collin R. Floppy eyelid syndrome: stretching the limits. Surv Ophthalmol. 2010;55(1):35-46.
7. Schlötzer-Schrehardt U, Stojkovic M, Hofmann-Rummelt C, et al. The pathogenesis of floppy eyelid syndrome: involvement of matrix metalloproteinases in elastic fiber degradation. Ophthalmology. 2005 Apr;112(4):694-704.
8. Ezra DG, Beaconsfield M, Sira M et al. The associations of floppy eyelid syndrome: a case control study Ophthalmology. 2010 Apr;117(4):831-8.
9. Waller EA, Bendel RE Kaplan J. Sleep disorders and the eye. Mayo Clin Proc. 2008 Nov;83(11):1251-61.
10. Ezra DG, Beaconsfield M, Sira M et al. Long-term outcomes of surgical approaches to the treatment of floppy eyelid syndrome. Ophthalmology. 2010 Apr;117(4):839-46.
11. Leibovitch I, Selva D. Floppy eyelid syndrome: clinical features and the association with obstructive sleep apnea. Sleep Med. 2006 Mar;7(2):117-22.
12. McNab AA. Reversal of floppy eyelid syndrome with treatment of obstructive sleep apnoea. Clin Experiment Ophthalmol. 2000;28(2):125-6.
13. Abdal H, Pizzimenti JJ, Purvis CC. The eye in sleep apnea syndrome. Sleep Med. 2006 Mar;7(2):107-15.

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