Patient reports cloudy vision out of a red left eye

Sara Weidmayer

A 59-year-old white man presented with a chief complaint of cloudy vision out of a red left eye. These symptoms started 2 days prior, after sanding wood the night before. He added that the cloudy vision also had a “film of squiggly black lines” that seemed to float with eye movement.

Despite minimal pain (2 out of 10), he was concerned about a possible foreign body. Appropriately, he had flushed his eye with saline, but his symptoms did not improve. He reported mild left eye soreness, irritation and photophobia, but denied any discharge.

He had no history of ocular injury or surgery. The patient disclosed a long history of sinus problems. His medical history was also remarkable for thrombocytopenia, chronic lower back pain, allergic rhinitis, spondylosis (a degenerative osteoarthritis of the joints between the vertebrae, usually related to repetitive strain), hyperlipidemia and tobacco use.

The patient had no known drug allergies, and his active medications were hydrocodone 500 mg tablet orally, three times a day as needed; simvastatin 40 mg half a tablet orally at bedtime; and verdenafil HCl 20 mg tablet, half a tablet as needed.

Entering visual acuity was 20/25- OD and 20/200- OS, pinholed to 20/150- OS wearing his habitual correction. His extraocular muscle excursions were full and smooth and confrontation visual fields were full in both eyes. He had anisocoria, with pupils measuring 4 mm OD and 2 mm OS. The left eye was only minimally reactive, but no afferent pupillary defect was elicited.

Left eye, fibrin debris with synechiae. Left eye, corneal edema. Images: Weidmayer S

Anterior segment biomicroscopy of the right eye was remarkable only for 1+ pigment cells and two pigment clumps on the anterior capsule of the lens.

Findings in the left eye included 3+ diffuse conjunctival injection, moderate corneal edema with Descemet’s folds, 3+ anterior chamber cells and flare, posterior synechiae nearly 360°, and fibrin and pigmentary debris on the anterior capsule of the lens. The patient’s corneal epithelium was intact, and there was no other indication of trauma or foreign body.

Intraocular pressure measured 16 mm Hg OD and 18 mm Hg OS at 9:55 a.m. A posterior segment evaluation was attempted, but was not visualized due to the densely clouded media. Ocular coherence tomography was not feasible due to the patient’s clouded media and initially dense fibrin collection on the anterior capsule of the lens.

What is your diagnosis?

The patient was assessed as having severe nongranulomatous anterior uveitis in the left eye. In an attempt to break the posterior synechiae, several drops of 10% phenylephrine and 1% tropicamide were administered. The patient was educated and was prescribed 5% homatropine twice per day and 1% prednisolone acetate every hour in the left eye; he was to return to the clinic the following day.

The patient was seen over the course of several weeks, where his symptoms gradually lessened and vision gradually improved as his topical medications were tapered, then discontinued. A fundus exam was conducted as soon as the media cleared, revealing unremarkable posterior ocular health.

Left eye, corneal edema, 1 week after initial visit.

While there was no active ocular inflammation apparent in the right eye at any of the patient’s exams, the mild pigment clumping on the anterior capsule of the lens suggests a previous bout of anterior uveitis in that eye, as well. It is certainly possible, and even quite likely, given the severity of the discussed case of uveitis and relatively few symptoms, that the patient has a history of a more chronic course of low-grade uveitis in both eyes, with the discussed episode being an exacerbation.

Click here for a table of the patient's exam summary.

Clinical picture

Acute anterior uveitis typically presents unilaterally with a painful or aching red eye, blurred vision, photophobia and tearing. Few of these symptoms usually present in cases of chronic anterior uveitis. Visual acuity may be decreased proportional to the severity of inflammation. The affected pupil may appear miotic or minimally reactive, related to posterior synechiae.

Anterior chamber cells and flare are characteristic clinical signs of this disease; white and/or red blood cells suspended within the anterior chamber’s aqueous humor and an increased content of protein within the aqueous, known as flare, result from increased permeability of the inflamed uveal vessels. Other signs include perilimbal injection, corneal edema, keratic precipitates (KPs), iris nodules and posterior synechiae.

KP, coalesced white blood cells that adhere to the corneal endothelium, may vary in size. The size is used to classify the uveitis as granulomatous (large, “mutton-fat” KP) or nongranulomatous (small to medium KP). Granulomatous anterior uveitis may also present with iris nodules (Koeppe or Busacca nodules). IOP may be low in the acute inflammatory phases of uveitis, but may elevate as the inflammation resolves or as a side effect of topical steroid use.

Left eye, fibrin debris resolved, 1 week after initial visit.

Unfortunate ocular sequelae may result secondary to uveitis; namely, elevated IOP from the development of posterior synechiae and pupillary block, inflammation or topical steroid use can lead to subsequent optic nerve damage or central retinal artery occlusion. Posterior subcapsular cataract is associated with both chronic anterior uveitis and topical steroid use. Chronic anterior uveitis may also produce band keratopathy or cystoid macular edema.

Systemic associations

According to the literature, the incidence of uveitis is about 8 to 15 per 100,000 people in the U.S. per year and demonstrates no sex predilection. In my duration of practice, the number of patients I have treated for anterior uveitis suggests that this statistic may be low. However, the variability may be related to the regional demographics and patient base, as well.

Most affected patients are 20 to 50 years of age, according to Tsang and the AOA Clinical Practice Guideline. Idiopathic anterior uveitis comprises about 50% of anterior uveitis cases; however, acute nongranulomatous anterior uveitis is often coupled with diseases associated with human leukocyte antigen B27 (HLA-B27), such as ankylosing spondylitis, inflammatory bowel disease, psoriatic arthritis, reactive arthritis (also known as Reiter’s syndrome) and Behçet disease.

Source: Tsang and Wittenberg

Recurrent bilateral alternating anterior uveitis is characteristic of HLA-B27-associated uveitis. According to Zamecki and colleagues, patients with recurrent unilateral or unilateral alternating acute anterior uveitis are 80% likely to be HLA-B27 positive, and being HLA-B27 positive carries a 15% relative risk of developing anterior uveitis. Trauma, herpes simplex, herpes zoster, cytomegalovirus, Lyme disease, masquerade syndromes (lymphoma, leukemia, etc.) and less common viral etiology such as rubella and Epstein-Barr virus may also be linked to acute nongranulomatous anterior uveitis. Traumatic anterior uveitis is common and usually follows a history of blunt trauma to the eye or adnexa, but may also follow foreign bodies, corneal abrasions or ocular burns. Hyphema may present in addition to the classic uveitic signs with traumatic anterior uveitis.

Acute granulomatous anterior uveitis is seen with sarcoidosis, syphilis and tuberculosis; such cases may also present with posterior uveitis, as is the case with toxoplasmosis, toxocariasis, ocular histoplasmosis or cytomegaloviral infections. Chronic cases of anterior uveitis, lasting longer than 6 weeks, are associated with juvenile rheumatoid arthritis and Fuch’s heterochromatic iridocyclitis, in addition to the diseases seen with acute granulomatous anterior uveitis.

Treatment, management

Once appropriately diagnosed, topical steroids and cycloplegics are the mainstays for anterior uveitis treatment. Steroids reduce inflammation by suppressing the migration of polymorphonuclear leukocytes, reducing exudation, inhibiting lysozyme release and reversing the increased capillary permeability found in uveitis by stabilizing cell membranes.

Prednisolone acetate 1% is the agent of choice and is dosed every hour to 6 hours, depending on the severity of uveitis. I would recommend branded over generic; the concentration achieved in the aqueous with generics is questionable. Lower-potency steroids should not be used initially, also due to the limited concentration obtained in the aqueous, according to Wittenberg.

Left eye, pigmentary remnants, 1 month after initial visit.

Durezol (difluprednate ophthalmic emulsion 0.05%, Alcon) would be a great first-line agent, certainly for more severe or recalcitrant cases. Due to formulary restrictions at the VA medical center where I practice, I have limited experience with the drug; however, I have heard of much success with this powerful drug’s use. It is important to note that IOP spikes from steroid responders may occur more quickly and may be more pronounced.

Oral steroids are typically reserved for cases of chronic or posterior uveitis; likewise, periocular subtenon corticosteroid injections or sustained-release intravitreal steroid implants, such as Retisert (fluocinolone acetonide intravitreal implant, Bausch + Lomb) or Ozurdex (dexamethasone 0.7 mg intravitreal implant, Allergan), have also found a niche in the treatment of recalcitrant or posterior uveitis, according to Jap and Chee and Taylor and colleagues.

Blocking both the nerve impulses to and contraction of the iris sphincter and ciliary body muscle with cycloplegics stabilizes the blood-aqueous barrier and reduces both pain and photophobia associated with anterior uveitis. Additionally, dilation prevents or breaks the formation of posterior synechiae, reducing the likelihood of secondary IOP spikes. Homatropine 2% to 5%, two to three times per day works well; it induces cycloplegia in 30 minutes to 90 minutes and mydriasis in 10 minutes to 30 minutes. Its cycloplegic effect lasts for 10 hours to 48 hours, and its mydriatic effect lasts from 6 hours to 4 days, according to Tsang. While phenylephrine causes dilation, it is not anti-inflammatory, nor does it induce cycloplegia, so it is not used therapeutically; however, it may be used to help break posterior synechiae, according to the AOA Clinical Practice Guideline.

If the patient is a steroid responder, resulting in elevated IOP, the course of action is controversial. Many practitioners may choose to remove the topical steroid that induced the IOP spike; however, the inflammation from the anterior uveitis must be controlled. Therefore, it is recommended that the topical steroids be cautiously continued with the addition of IOP-lowering medication. Prostaglandin analogs should be avoided due to their association with inflammation, advised Wittenberg.

While being treated, the patient should be examined every 1 day to 7 days with visual acuity, slit lamp biomicroscopy and IOP measurements. A posterior segment evaluation should be performed when possible. The median duration of anterior uveitis attack due to HLA-B27 associated disease is 6 weeks; therefore, treatment of such an attack should be prolonged for at least that length of time to prevent exacerbation when topical treatment is stopped. The steroids and cycloplegics are tapered judiciously, according to the degree of resolution.

Ancillary laboratory testing

Appropriate laboratory testing should be ordered according to the patient’s history or if a certain etiology is suspected, and it may help direct the clinician regarding the necessity of referrals to other health care providers. When a patient has bilateral, granulomatous or recurrent anterior uveitis, a nonspecific laboratory workup is indicated; however, sources vary as to which laboratory tests should be used routinely. A nonspecific workup is shown in the accompanying chart.

References:

• Brent LH. Ankylosing spondylitis and undifferentiated spondyloarthropathy. EMedicine. WebMD. Available at: http://emedicine.medscape.com/article/332945-overview. Accessed July 1, 2011.
• Care of the patient with anterior uveitis. Optometric Clinical Practice Guideline. American Optometric Association, 2004. Available at: http://www.aoa.org/documents/CPG-7.pdf. Accessed July 1, 2011.
• Ehlers JP, Shah CP. Uveitis. The Wills Eye Manual. 5th ed. Philadelphia: Lippincott Williams & Wilkins. 2008:334-338.
• Jap A, Chee SP. Emerging forms of viral uveitis in the developing world. International Ophthalmology Clinics. 2010;50(2):155-171.
• Taylor SRJ, Isa H, Joshi L, Lightman S. New developments in corticosteroid therapy for uveitis. Ed. Cunha-Vaz J. Ophthalmologica: The International Journal of Ophthalmology. 2010;224(suppl. 1):46-453. Available at: http://content.karger.com/produktedb/produkte.asp?tpy=fulltext&file=000318021. Accessed July 1, 2011.
• Tsang K. Iritis and uveitis. EMedicine Emergency Medicine. WebMD. Available at: http://emedicine.medscape.com/article/798323-overview. Accessed July 1, 2011.
• Uveitis. Wikipedia. Available at: http://en.wikipedia.org/wiki/Uveitis. Accessed July 1, 2011.
• Wittenberg S. Ten clinical pearls for treating uveitis. American Academy of Ophthalmology. Available at: http://www.aao.org/yo/newsletter/200808/article05.cfm. Accessed July 1, 2011.
• Zamecki KJ, Jabs DA. HLA typing in uveitis: use and misuse. Am J Ophthalmol. 2010;149(2):189-193. Available at: http://www.ncbi.nlm.nih.gov/pubmed/20103052. Accessed July 1, 2011.

• Sara Weidmayer, OD, practices with a small group of optometrists in Adrian and Brooklyn, Mich. She can be reached at Eye Center of Lenawee; saraweidmayer@gmail.com.
• Edited by Leo P. Semes, OD, a professor of optometry, University of Alabama at Birmingham and a member of the Primary Care Optometry News Editorial Board. He may be contacted at (205) 934-6773; fax: (205) 934-6758; lsemes@uab.edu.
• Disclosure: Dr. Weidmayer has no direct financial interest in the products mentioned in this article, nor is she a paid consultant for any companies mentioned.