This article is more than 5 years old. Information may no longer be current.

Man referred for bilateral retinal detachments

Exam findings included mutton-fat keratic precipitates and dense vitritis.

Issue: May 10, 2015

ByJoseph Hoffman

ByJoseph Ho, MD

ByChristopher Robinson, MD

Add topic to email alerts

Receive an email when new articles are posted on

Please provide your email address to receive an email when new articles are posted on .

Subscribe

Added to email alerts

You've successfully added to your alerts. You will receive an email when new content is published.

Click Here to Manage Email Alerts

You've successfully added to your alerts. You will receive an email when new content is published.

Click Here to Manage Email Alerts

Back to Healio

We were unable to process your request. Please try again later. If you continue to have this issue please contact customerservice@slackinc.com.

Back to Healio

A 62-year-old Cambodian man was referred from an outside ophthalmologist with concern for bilateral macula-off retinal detachments. The patient reported progressively worsening vision over the past month, but particularly over the past week. He complained of mild photophobia and nonspecific ocular discomfort. He denied seeing any floaters or flashing lights. He denied any history of eye trauma. His medical and family histories were noncontributory. He denied tobacco, alcohol or illicit drug use.

Examination

On examination, best corrected visual acuity was light perception in the right eye and hand motions in the left. The pupils were minimally reactive bilaterally, and no relative afferent pupillary defect was noted. IOP was 19 mm Hg in the right eye and 20 mm Hg in the left. Extraocular movements were intact. The patient was unable to participate in confrontational visual field or color testing.

Anterior segment exam revealed bilateral conjunctival injection, hypopyon with 4+ anterior chamber cell, mutton-fat keratic precipitates and posterior synechiae (Figures 1a and 1b). The exam was also significant for moderate nuclear sclerotic cataracts in both eyes. Posterior segment exam revealed dense vitritis bilaterally with total bullous exudative retinal detachments in both eyes.

Figure 1. External photographs at time of presentation showing posterior synechiae in the right eye (a) and left eye (b). External photographs at 1-month follow-up showing poliosis (yellow arrows) in the right eye (c) and left eye (d).

Images: Ho J, Robinson C

What is your diagnosis?

Bilateral retinal detachments

The differential diagnosis for bilateral exudative retinal detachments with associated uveitis includes Vogt-Koyanagi-Harada disease, sympathetic ophthalmia, posterior scleritis, tuberculosis, sarcoidosis and syphilis.

Differential diagnosis

Vogt-Koyanagi-Harada (VKH) disease typically causes bilateral granulomatous uveitis. Anterior segment exam often reveals mutton-fat keratic precipitates. Posterior segment exam may show bilateral exudative retinal detachments and optic nerve hyperemia. Dalen-Fuchs nodules, which are elevated lesions between the retinal pigment epithelium (RPE) and Bruch’s membrane, may be seen. Fluorescein angiography classically shows a “starry sky” pattern with focal spots of expanding hyperfluorescence in the posterior pole. B-scan ultrasonography may show retinal detachment and choroidal thickening. Poliosis, perilimbal vitiligo and choroidal depigmentation resulting in a “sunset glow” fundus appearance may occur during the convalescent stage.

Sympathetic ophthalmia can present similarly to VKH. This rare form of bilateral granulomatous uveitis occurs in less than 1% of patients with penetrating eye trauma. Exam may show bilateral exudative retinal detachments with Dalen-Fuchs nodules. Poliosis and vitiligo may also be present. A history of penetrating eye trauma or intraocular surgery is usually elicited. The onset of disease can range from weeks to decades after the inciting event. Our patient did not have a history of penetrating eye trauma or ocular surgery.

Posterior scleritis may present with serous retinal detachments, and secondary uveitis can develop. Symptoms include blurred vision, deep eye pain and redness. B-scan ultrasonography may help to differentiate posterior scleritis from other causes of serous retinal detachments by the presence of an ultrasonic “T-sign,” which is thought to signify the presence of fluid in Tenon’s capsule. Pain associated with posterior scleritis is typically a deep, boring, severe pain, which our patient did not have, making this diagnosis less likely.

Tuberculosis, sarcoidosis and syphilis may all present with granulomatous uveitis with subretinal fluid and should be ruled out with serologic testing.

Diagnosis and management

B-scan ultrasonography showed bilateral retinal detachments, without ultrasonic T-sign, making posterior scleritis less likely (Figure 2). Fluorescein angiography was not obtained due to extensive posterior synechiae, precluding an adequate view. Chest X-ray, PPD, FTA-ABS, ACE and HLA-B27 were negative. Given his race, clinical presentation and negative serologic studies as above, the patient was diagnosed with VKH and started on 60 mg of oral prednisone, topical steroids and a cycloplegic agent in both eyes.

Two weeks later, he reported significant subjective improvement in vision, although his visual acuity remained light perception in the right eye and improved somewhat to finger counting at 4 feet in the left. A trace afferent pupillary defect was present in the right eye. IOPs remained normal, and the level of anterior segment inflammation and vitritis improved in both eyes. Fundus examination revealed persistent serous retinal detachments, although no Dalen-Fuchs nodules were seen. Spectral domain OCT showed foveal subretinal fluid in both eyes as well as areas of RPE irregularity (Figures 3a and 3b). HLA testing was positive for HLA-DR4 and HLA-DR53, which is consistent with a diagnosis of VKH. Despite a negative PPD, QuantiFERON-TB Gold test results returned positive. Oral prednisone was continued and treatment initiated for latent tuberculosis with isoniazid.

Figure 2. B-scan ultrasound image at time of presentation demonstrating bilateral serous retinal detachments in the right eye (a) and left eye (b).

Figure 3. SD-OCT of the right eye (a) and left eye (b) showing subretinal fluid and mild RPE irregularities. SD-OCT at 1 month showing a decrease in amount of subretinal fluid in the right eye (c) and left eye (d).

Figure 4. Color fundus photographs 1 month after presentation showing a sunset glow fundus appearance in the right eye (a) and left eye (b).

One month after presentation, the patient’s vision improved to hand motions in the right eye and remained stable at finger counting in the left. Anterior segment inflammation continued to improve, and poliosis was noted bilaterally (Figures 1c and 1d). Posterior segment exam revealed a “sunset glow” appearance with diffuse depigmentation bilaterally (Figure 4). Repeat SD-OCT showed decreased subretinal fluid (Figures 3c and 3d). Fluorescein angiography revealed bilateral areas of blockage secondary to RPE clumping as well as areas of hyperfluorescence signifying window defects in areas of atrophy (Figures 5a to 5c). Mild disc hyperfluorescence was present in mid and late phase fluorescein. ICG angiography was performed to assess for choroidal hypofluorescent dark dots, which have been associated with VKH and are thought to represent choroidal granulomas causing blockage (Figures 5d to 5f). No such lesions were identified in our patient.

Figure 5. Fundus fluorescein angiography photographs of right eye (a to c) 1 month after presentation. Note the blockage and mild disc hyperfluorescence. Photographs d to f show early, mid and late ICG angiography photographs of the left eye. There were no choroidal dark spots seen.

Discussion

VKH disease is a rare cause of uveitis. It is hypothesized to be a multisystemic disorder secondary to a T-cell-mediated autoimmune reaction against melanocytes. The average age of onset is in the fourth decade of life. It is most commonly seen in patients of Asian, Latin or Mediterranean descents and is more common in women. There is an association with multiple HLA alleles, including DR4 and DR53.

Classically, VKH has three main clinical stages. The prodromal stage lasts a few days and consists of fevers, headaches, meningismus and tinnitus. The acute uveitic phase lasts several weeks and is characterized by bilateral anterior chamber inflammation and/or vitritis and exudative retinal detachments. There may also be optic nerve hyperemia or edema. The convalescent stage consists of RPE depigmentation, perilimbal vitiligo and poliosis. The acute phase may recur, particularly if treatment is discontinued prematurely.

Useful ancillary testing includes fluorescein angiography, B-scan ultrasonography, OCT, ICG angiography and lumbar puncture. In the acute phase, fluorescein angiography shows multiple hyperfluorescent spots with late leakage, resulting in the classic “starry sky” appearance. This is followed by the convalescent stage during which fluorescein angiography typically shows blockage secondary to RPE clumping and window defects in areas of atrophy. B-scan ultrasonography may show serous retinal detachments and choroidal thickening. OCT may demonstrate subretinal fluid and/or Dalen-Fuchs nodules. Choroidal hypofluorescent dark dots, thought to be choroidal granulomas, may be seen during active disease using ICG angiography. ICG angiography has also proven to be useful in determining response to treatment (disappearance of dark dots) and in detecting subclinical recurrence (reappearance of dark dots). Lumbar puncture may reveal pleocytosis early in the course of the disease.

Conclusion

The mainstay of treatment is high-dose oral steroids, typically requiring at least 6 months of treatment. Shorter periods have been associated with recurrences. Immunosuppressants may be necessary if inflammation is not controlled with systemic steroids or if the patient is not able to tolerate side effects associated with high-dose prednisone use. Given the necessity for long-term immunosuppression, it is important to rule out tuberculosis before initiating treatment. With appropriate treatment, prognosis is generally good, with 60% of patients achieving at least 20/40 or better vision.