Clinical Review & Education

JAMA Ophthalmology Clinical Challenge

Serous Retinal Detachment Following Enucleation Sumayya Ahmad, MD; Connie J. Chen, MD; John Peter Campbell, MD, MPH

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Figure 1. A, Fluorescein late-phase angiogram shows multifocal areas of posterior choroidal leakage and diffuse retinal vascular leakage in the inferior (detached) retina. B, Optical coherence tomographic en face infrared image (left) and vertical line scan (right) through the fovea show subretinal fluid extending inferiorly with hyperreflective subretinal deposits.

A white man in his 30s with a remote history of right eye enucleation following globe trauma presented to our clinic in July 2014 with decreased visual acuity in the left eye. He described a history of initially intermittent (lasting hours to days) then constant (for the past 6-8 weeks) central and peripheral blurred vision in the left eye. He reported no headaches, numbness, weakness, eye pain, hearing loss, or other neurologic issues. Eight years earlier, he had sustained a fireworks injury to his right eye followed by globe repair and enucleation within 3 days. He had no other significant medical or ocular history. His right eye’s external appearance was normal, and the prosthesis fit well. Uncorrected visual acuity was Snellen 20/70−1 OS (no improvement with pinhole); intraocular pressure was 13 mm Hg. Slitlamp examination results of the anterior segment were normal. Ophthalmoscopy demonstrated 1+ cells in the anterior vitreous and a serous retinal detachment involving the macula and extending from the 2-o’clock to the 10-o’clock position with pigmentary and atrophic changes in the macula. No breaks were noted on Quiz at scleral depressed examination. Fluorescein angiograjamaophthalmology.com phy showed multifocal areas of early hyperfluorescence with late leakage and diffuse retinal vascular leakage predominantly in the inferior retina (Figure 1A). Optical coherence tomography (OCT) showed a thickened choroid and subretinal fluid throughout the macula (Figure 1B). Enhanced depth imaging showed an enlarged choroid throughout the posterior pole, and ultrasonography confirmed extensive inferior retinal detachment (not shown).

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WHAT WOULD YOU DO NEXT?

A. Systemic corticosteroid therapy B. Observation C. Photodynamic therapy D. Antivascular endothelial growth factor therapy (anti-VEGF)

(Reprinted) JAMA Ophthalmology June 2015 Volume 133, Number 6

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Clinical Review & Education JAMA Ophthalmology Clinical Challenge

Diagnosis Central serous chorioretinopathy

What To Do Next C. Photodynamic therapy

Discussion

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The 2 leading diagnoses under consideration were chronic central serous chorioretinopathy (CSR) and sympathetic ophthalmia (SO). There was an extensive serous retinal detachment with vitreous cell and diffuse vascular leakage in our patient with a history of eye trauma, which raised the possibility of SO. Many of the patient’s nonspecific visual symptoms could have been consistent with the early loss of accommodation that can be observed with mild SO. However, the pace of the disease, lack of anterior chamber inflammation, and overall angiographic features were more consistent with chronic CSR (the classic “starry sky” appearance of the choroiditis associated with SO was not present). In terms of the available therapeutic options, observation did not seem appropriate, and the role of anti-VEGF therapy, in the absence of clear evidence of choroidal neovascularization, is not clear. Thus, the decision was based on our ability to definitively differentiate SO from CSR. The classic clinical and angiographic presentations of each condition are different; however, the present case had features of both and was classic for neither. On imaging, both SO and CSR can present with exudative detachments, choroidal thickening on enhanced depth imaging OCT, elongated photoreceptor segments in detached retina, and multifocal hyperfluorescent angiographic lesions with late leakage.1-4 Considering the large serous detachment, widespread angiographic retinal vascular leakage, vitreous cell, and poor prognosis of untreated SO, we opted for a diagnostic (and possibly therapeutic) trial of high-dose corticosteroid therapy (intravenous methylprednisolone sodium succinate and oral prednisone). During the first 2 weeks of therapy, there were no appreciable clinical or angiographic changes. At the 3-week follow-up examination, careful review of macular OCT topography revealed gradually increased overall macular volume and subretinal fluid as well as a clinically worsening inferior serous retinal detachment. We believed that this finding confirmed an underlying diagnosis of CSR slowly worsening during

corticosteroid therapy. Multiple-spot photodynamic therapy was then performed, targeting the areas of leakage on fluorescein and indocyanine green angiography, and corticosteroid therapy was tapered to discontinuation across a few weeks.

Patient Outcome One month after photodynamic therapy, the patient’s macular OCT was markedly improved and his inferior serous retinal detachment had nearly resolved (Figure 2). At 3 months, there was complete resolution of the subretinal fluid and his visual acuity returned to 20/50 on Snellen examination, with mild intraretinal fluid. This atypical and dramatic presentation of chronic CSR was confounded by the patient’s history of traumatic injury and enucleation and by the evident intraocular “inflammation,” which made the diagnosis of SO higher on the differential than it might have been based solely on the imaging findings. Although the remoteness of the patient’s trauma and the early enucleation overall made SO less likely, both late onset and onset of SO after early enucleation have been reported,5-7 with as many as 10% of cases of SO occurring more than 1 year after the trauma. The clinical presentation of SO is variable, ranging from mild anterior-segment inflammation to severe granulomatous panuveitis with variable degrees of retinal involvement, and onset can be insidious or acute.5-7 In retrospect, we infer that the “vitritis” and diffuse vascular leakage were secondary to the chronic retinal detachment with subsequent breakdown of the blood-retinal barrier and were not a priori evidence of inflammatory disease (neither condition responded to prednisone therapy). We encourage continued advancement of multimodal imaging modalities to improve our ability to differentiate these pathophysiologically different diseases.

REFERENCES

Author Affiliations: Wilmer Eye Institute, the Johns Hopkins University School of Medicine, Baltimore, Maryland.

1. Gupta V, Gupta A, Dogra MR, Singh I. Reversible retinal changes in the acute stage of sympathetic ophthalmia seen on spectral domain optical coherence tomography. Int Ophthalmol. 2011;31(2): 105-110.

Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported.

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Figure 2. Optical coherence tomographic en face infrared image (left) and vertical line scan (right) through the fovea 1 month after photodynamic therapy show improvement in the subretinal fluid.

ARTICLE INFORMATION

Corresponding Author: John Peter Campbell, MD, MPH, Wilmer Eye Institute, the Johns Hopkins University School of Medicine, 600 N Wolfe St, Wilmer B-29, Baltimore, MD 21287 ([email protected]).

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2. Burkholder BM, Dunn JP. Multiple serous retinal detachments seen on wide-field imaging in a patient with sympathetic ophthalmia. JAMA Ophthalmol. 2014;132(10):1220. 3. Fleischman D, Say EA, Wright JD, Landers MB. Multimodality diagnostic imaging in a case of sympathetic ophthalmia. Ocul Immunol Inflamm. 2012;20(4):300-302.

4. Castiblanco C, Adelman RA. Imaging for sympathetic ophthalmia: impact on the diagnosis and management. Int Ophthalmol Clin. 2012;52(4): 173-181. 5. Chang GC, Young LH. Sympathetic ophthalmia. Semin Ophthalmol. 2011;26(4-5):316-320. 6. Lubin JR, Albert DM, Weinstein M. Sixty-five years of sympathetic ophthalmia. Ophthalmology. 1980;87(2):109-121. 7. Chu XK, Chan CC. Sympathetic ophthalmia. J Ophthalmic Inflamm Infect. 2013;3(1):49.

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Serous Retinal Detachment Following Enucleation.

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