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Young female complains of blurred vision, headache, redness
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A 24-year-old African-American female presented to our office complaining of blurred vision in both eyes. She had been seen earlier that day by a fellow optometrist who made a same-day referral to our practice for management.
The patient stated that the blurriness began 3 weeks prior and had gradually worsened. She described a “white fog over everything” that was more debilitating in the left compared with the right eye. She also reported pain in the left eye; headache; and redness, flashes and floaters in both eyes. The headache was located toward the front of the head and around the eyes and had been occurring once a day, although the patient was unsure of the exact duration. Oral over-the-counter pain medication provided mild relief.
The patient’s medical, ocular and surgical histories were unremarkable, and she was taking ibuprofen for her headache as needed. She was also using Visine (McNeil) topically as needed in both eyes. She was not a smoker, denied drug or alcohol use, and had no known drug allergies.
The patient did not wear vision correction. Uncorrected visual acuity was 20/30 OD and 20/50-1 OS with pinhole acuity of 20/30+1 OD and 20/30-2 OS. No refraction was performed at this visit. Pupils were round and equal with no relative afferent pupillary defect. Intraocular pressure was 17 mm Hg OD and 18 mm Hg OS measured with Tonopen (Reichert).
Images: Fulmer P
Anterior segment evaluation was remarkable for trace+ injection in the right eye and trace injection in the left eye of the bulbar conjunctiva, dense granulomatous keratic precipitates (KPs) on the corneal endothelium greater in the right eye than the left, grade 4 cell and grade 2 flare in the anterior chamber of the right eye, grade 3+ cell and grade 1+ flare in the anterior chamber of the left eye, and posterior synechia in both eyes. Anterior segment photos were obtained prior to dilation in both eyes. Using 10% phenylephrine, all synechia except at 6:00 OD were broken in office.
Dilated fundus exam was attempted but difficult secondary to anterior segment findings. Retinal red reflex was present in both eyes. Cup-to-disc ratios were unable to be assessed in the right eye and 0.3/0.3 OS. Marked syneresis was present in the vitreous of each eye, but vitritis was not able to be appreciated at this visit secondary to difficult views. Macular contour was unable to be assessed in either eye. No retinal breaks or detachments were present in the peripheral retina of the right or left eye, and no snow banking was seen in either eye. Although difficult to obtain, macular optical coherence tomography scans were performed using a Cirrus HD-OCT (Carl Zeiss Meditec), and results demonstrated no edema or change in foveal contour in either eye.
The most likely differential diagnoses in the context of this presentation include nongranulomatous anterior uveitis (nontraumatic and traumatic), intermediate uveitis, posterior uveitis, Posner-Schlossman syndrome, sclerouveitis, keratouveitis, infectious endophthalmitis and granulomatous uveitis. When a diagnosis of uveitis is made, the location(s) affected must be specified and include: anterior, intermediate, posterior and panuveitis. In addition, the clinician should attempt to establish an etiology.
Nongranulomatous, anterior uveitis
Nongranulomatous anterior uveitis in patients without a history of trauma is most commonly unilateral. These patients typically present with acute pain and photophobia that is greater in one eye than the other, and fine KPs are often noted diffusely dusting the corneal endothelium. The retina, choroid and posterior vitreous are not involved, although some inflammatory cells may spill over into the anterior vitreous in severe presentation. The etiology in these cases is most frequently idiopathic.
Traumatic uveitis is also nongranulomatous, can be unilateral or bilateral, and is a result of trauma to one or both eyes. Pain, photophobia and/or tearing presents within 3 days of the traumatic event in most patients.
This was not the diagnosis in this case due to the bilateral presentation and granulomatous nature of the KPs as well as the absence of trauma.
Intermediate uveitis
Intermediate uveitis is usually bilateral with its primary locations of inflammation in the vitreous, pars plana and peripheral retina. Patients are most often between 15 and 40 years old and commonly report floaters and cloudy vision without pain, photophobia or external inflammation.
While this patient was in the appropriate age range and did present with floaters and vision complaints in line with this diagnosis, she did experience pain, and her most affected site of inflammation was the anterior chamber instead of the intermediate locations. Therefore, intermediate uveitis was ruled out.
Posterior uveitis
Posterior uveitis is typically painless and has primary sites of inflammation in the anterior and posterior vitreous, retina and choroid and along the retinal vessels. Blurred vision and floaters are noted complaints from these patients, as well as occasional redness.
Again, the patient’s pain and primary site of inflammation excluded this option.
Posner-Schlossman syndrome
Posner-Schlossman syndrome is most often unilateral in young to middle-aged patients and presents with recurrent episodes of elevated intraocular pressure and mild inflammation. Decreased vision, mild pain and observation of a rainbow appearance around lights are commonly described symptoms.
Being a self-limiting condition, this diagnosis is often overlooked unless the patient is symptomatic and attends for care. The patient’s IOP was normal, making this diagnosis unlikely.
Sclerouveitis
Sclerouveitis occurs secondary to scleritis and results in inflammation in the anterior uveal tract. Patients present with moderate to profound pain that is often described as boring. Presence of the condition often indicates poor ocular prognosis including possible progressive vision loss.
There was no concurrent scleritis in this patient’s case, and sclerouveitis was eliminated as a diagnosis.
Keratouveitis
Keratouveitis is a condition that couples keratitis with anterior chamber inflammation and can result from trauma, contact lens over wear or infection. Corneal infiltrates will be present in infectious cases.
Corneal findings in this patient were remarkable for KPs only on the endothelium of both eyes. No keratitis or infiltrates were noted, so keratouveitis was ruled out.
Infectious endophthalmitis
Infectious endophthalmitis is most frequently associated with recent intraocular surgery, although the condition can be traumatic or endogenous in nature. Patients typically present with acute onset of marked, increasing pain and decreased vision. Fibrin, vitritis, hypopyon and severe anterior chamber reaction are commonly found upon examination.
There was no history of surgery, intraocular penetration, fibrin or hypopyon in this case, so infectious endophthalmitis was unlikely.
Granulomatous uveitis
Granulomatous uveitis presents bilaterally more frequently than does nongranulomatous uveitis. Symptoms are often more gradual or chronic in nature. KPs formed are large and sticky with a mutton-fat appearance, and inflammation may be present in primarily the anterior segment, intermediate structures, posterior segment or a combination of these locations. Unlike nongranulomatous uveitis, granulomatous uveitis is most often a manifestation of an underlying, systemic condition. The most common causes of this condition are sarcoidosis, herpes simplex or zoster, tuberculosis, Lyme disease and syphilis.
This patient’s management
In this case, the condition was bilateral and included large, mutton-fat KPs in both eyes. Therefore, the patient was diagnosed with bilateral granulomatous uveitis. Initially, the location was designated as anterior, but that description was later amended to panuveitis due to snow banking and retinal inflammatory lesions that were observed on subsequent follow-up visits.
Granulomatous uveitis is responsive to treatment in most cases, though it is important to remember that any form of uveitis can lead to sight-threatening complications such as glaucoma, cystoid macular edema, cataracts and band keratopathy. Determining the underlying cause of the condition is imperative in reaching full resolution and preventing recurrences. Bloodwork should be ordered for any patient presenting with granulomatous uveitis, and orders should include tests specific for the most common etiologies.
We evaluated this patient for sarcoidosis and syphilis through a bloodwork panel including angiotensin converting enzyme (ACE) and both rapid plasma reagin (RPR) and fluorescent treponemal antibody absorption (FTA-ABS) testing. A purified protein derivative (PPD) skin test was also ordered to determine the presence or absence of tuberculosis. There were no signs of herpes simplex or zoster. The patient’s history did not suggest Lyme disease, so testing was not ordered in this particular case. However, enzyme-linked immunosorbent assay (ELISA) testing can be added to the panel to evaluate for this condition.
Results of the patient’s lab work were remarkable for positive ACE, elevated C-reactive protein (CRP) and elevated erythrocyte sedimentation rate (ESR). The latter two tests confirmed systemic inflammation.
Topical steroids are the mainstay of treatment for all forms of uveitis, and topical cycloplegic agents are useful in decreasing pain, stabilizing the blood-aqueous barrier, breaking current synechia and preventing the formation of future synechia. This patient was prescribed Durezol (difluprednate ophthalmic emulsion 0.05%, Alcon) every 2 hours in both eyes and atropine 1% solution twice daily in both eyes.
Oral steroids are sometimes necessary in severe cases or when topical steroids are minimally effective. This patient’s ocular inflammation did not significantly improve with topical treatment alone, so an oral prednisone taper of 60 mg daily for 1 week, then 40 mg daily for 1 week, then 20 mg daily for 1 week, then 20 mg every other day for 1 week was added to her management. Her Durezol was also tapered as improvement was appreciated.
The condition responded very well, and full resolution with visual acuity of 20/20 OU was reached within 5 weeks. Due to the positive ACE lab finding, a chest X-ray was ordered, which returned negative. The patient was referred to a rheumatologist to further evaluate for underlying systemic conditions, particularly sarcoidosis. However, she did not keep that appointment and has not rescheduled to date.
Granulomatous uveitis that is a result of a systemic problem will recur in most cases without proper management of the causative factor. This patient was educated to that fact and, unfortunately, did experience a recurrence 4.5 months later. The same treatment approach was used, and resolution was again obtained.
In cases such as this one, maintenance therapy with topical steroids can be beneficial in preventing flare-ups. The patient is currently on a successful maintenance dose of one drop of prednisolone acetate 1% suspension in both eyes every other day, with follow-up visits every 3 months.
- References:
- Agrawal RV, et al. Ind J Ophthalmol. 2010;58(1):11–19. doi: 10.4103/0301-4738.58468.
- Alexander KL, et al. Care of the Patient with Anterior Uveitis. Optometric Clinical Practice Guideline. http://www.aoa.org/documents/optometrists/CPG-7.pdf. Reviewed 2004. Accessed July 14, 2015.
- Dahl AA. Uveitis, Anterior, Granulomatous. http://emedicine.medscape.com/article/1209505-overview. Updated September 11, 2014. Accessed July 14, 2015.
- Forster DJ. General approach to the uveitis patient and treatment strategies. In: Yanoff M, Duker JS, eds. Ophthalmology. 3rd ed. Mosby Elsevier; 2009:783-787.
- Sainz de la Maza M, et al. Ophthalmology. 1997;104(1):58-63.
- Wills Eye Hospital. Uveitis. In: Kunimoto DY, Kanitkar KD, Makar M, eds. The Wills Eye Manual. 4th ed. Lippincott Williams & Wilkins; 2004:290-314.
- For more information:
- Patricia Fulmer, OD, completed an ocular disease and primary care residency at the Thomas E. Creek VA Medical Center in Amarillo, Texas. She is currently the center director at Vision-America of Huntsville in Huntsville, Ala. She can be reached at patricia.fulmer@eyehealthpartners.com. Edited by Leo P. Semes, OD, FAAO, a professor of optometry, University of Alabama at Birmingham and a member of the Primary Care Optometry News Editorial Board. He may be reached at lsemes@uab.edu.
Disclosure: Fulmer has no relevant financial disclosures.