Lana M. Rifkin

A new OCT finding in tuberculous serpiginous-like choroidopathy

Abstract Purpose: To present a case of tubercular serpiginous-like choroiditis (SLC) with previously unreported choroidal findings on enhanced depth imaging OCT (EDI-OCT). Design: Case report. Methods: A 60-year-old female presented with decreased vision. Serpiginous choroidopathy was diagnosed. Laboratory workup revealed an infectious etiology. EDI-OCT revealed previously unreported choroidal findings. Results: Laboratory workup revealed nonreactive Treponema pallidum antibodies and positive QuantiFERON Gold. CT chest showed scars of prior granulomatous disease. OCT with EDI of active lesions demonstrated infiltration of the choroid, elevation of the RPE-Bruch’s membrane complex and focal increase of choroidal thickness. Conclusions: Choroidal infiltration with elevation of the RPE was demonstrated on EDI-OCT in active areas of tuberculous serpiginous-like choroiditis in this patient. This finding has not been described in imaging of patients with noninfectious serpiginous choroidopathy and may be a useful tool to differentiate serpiginous choroidopathy (SC) from serpiginous-like choroiditis (SLC). EDI-OCT may provide characterization of choroidal involvement.

H1N1-associated Acute Retinitis

Purpose: To present the first reported case of bilateral H1N1-associated acute retinitis and its successful treatment. Design: Interventional case report. Methods: A 41-year-old HIV-positive male presented with acute vision loss, panuveitis, and retinitis. A diagnostic and therapeutic vitrectomy with intravitreal injection of vancomycin and ganciclovir and endolaser was performed. One month later, the patient returned with similar symptoms in the fellow eye and underwent the same procedure. Results: ELISA immunoassay revealed H1N1 antibodies in both the vitreous and serum. PCR for herpes viruses included HSV, CMV, and VZV. Bacterial and fungal cultures were negative. On 1-year follow-up, the vision remained 20/20 in both eyes without evidence of recurrent inflammation. Conclusions: H1N1 should be included in the differential diagnosis of any patient with a history of recent influenza A (H1N1) infection and acute retinitis. H1N1 may carry a better prognosis than other viruses causing acute retinitis.

Sarcoidosis: Sex-Dependent Variations in Presentation and Management

Sarcoidosis is an inflammatory disease with a wide range of clinical presentations. The manifestations and prognosis in sarcoidosis are dependent upon not only organ involvement but also age and sex. The purpose of this review is to describe the systemic and ocular manifestations of sarcoidosis with a specific focus on sex-dependent difference in presentation and management. Sarcoidosis is more common in women, particularly in patients who present after age of 50 years. Women with sarcoidosis are more likely to develop cystoid macular edema and the mortality rate is higher than that of men.

Shortening ocular pain duration following intravitreal injections

Purpose. To determine ocular pain duration after routine in-office intravitreal injection and to determine whether topical eyedrops are beneficial in increasing patient comfort. Methods. Forty injection-naïve patients receiving routine intravitreal injections of bevacizumab for age-related macular degeneration were randomized into 3 groups: group 1 (control, no drops), group 2 (generic artificial tears), and group 3 (ketorolac tromethamine 0.4% eyedrops). Those who received topical medications were given a Visual Analog Pain score survey and asked to record their pain on a scale from 0 (no distress) to 10 (unbearable distress) daily until a score of 0 was achieved, at which point they were instructed to discontinue use of their given drops. Self-reported pain scores were assessed. Results. Pain after routine intravitreal injection lasts on average between 3 and 7 days. Patients receiving topical ketorolac eyedrops reported the fewest average number of pain days (2.25±1.22) vs patients receiving artificial tears (3.54±1.13) or those who received no postprocedure eyedrops (5.13±1.25); p<0.05. At most, patients receiving ketorolac eyedrops reported 3 days of recordable pain. Those who received artificial tears reported at most 5 days of recordable pain, and patients who did not receive any postprocedure eyedrops reported at most 7 days of recordable pain. Conclusions. Pain after intravitreal injection is generally mild, may be reduced by postinjection topical ketorolac eyedrops, and lasts less than 1 week.

Teenage acute macular neuroretinopathy.

Purpose To present the first case of acute macular neuroretinopathy in a teenager. Methods Case report, optical coherence tomography, fluorescein angiography, electroretinography. Results Optical coherence tomography, fluorescein angiography, and electroretinography were used to diagnose acute macular neuroretinopathy. Laboratory results were positive for HLA-B7. Conclusions The rare diagnosis of acute macular neuroretinopathy should be considered in girls on oral contraceptives, with a recent history of acute viral illness, who present with acute rapid onset of multiple, paracentral scotomas.

Utility of Leflunomide in the Treatment of Drug Resistant Cytomegalovirus Retinitis.

ABSTRACT Purpose: To describe leflunomide use in the treatment of drug resistant cytomegalovirus retinitis. Leflunomide has been shown to be effective in the treatment of systemic CMV viremia. Methods: Retrospective chart review of patients with CMV retinitis treated with leflunomide. Results: Two HIV-negative organ transplant recipients with UL 97 mutation resistant-genotype CMV were identified. Patient 1 developed CMV viremia post-kidney transplant and subsequently bilateral CMV retinitis. Retinitis progressed, despite intravitreal injection of ganciclovir and foscarnet, and IV foscarnet and oral valganciclovir. Retinitis control was achieved with the addition of oral leflunomide. Disease remained inactive for 22 months. Patient 2 developed CMV retinitis after lung transplant. Disease progressed despite intravitreal foscarnet injections and oral valganciclovir. Control of retinitis was achieved with addition of oral leflunomide, allowing cessation of intravitreal therapy. Disease remained inactive until his death. Conclusions: Leflunomide may be considered as a treatment option for resistant CMV retinitis.

Fundus autofluorescence findings in eyes with birdshot chorioretinitis

Purpose The purpose of this study was to describe fundus autofluorescence (FAF) findings in eyes with birdshot chorioretinitis (BSCR) and to compare findings to demographic, medical, and clinical characteristics. Methods In this multicenter, prospective, cross-sectional study, 172 eyes (86 patients) with BSCR were investigated. Participants underwent a standardized evaluation including collection of demographic data, ophthalmic and treatment history, and ophthalmologic examination. Using a standardized protocol, hypo- and hyperautofluorescence in macular and extramacular regions and specific patterns of abnormal FAF could be scored for 167 eyes. Images were scored by two independent, masked graders. Measures of visual function included best-corrected visual acuity (BCVA), contrast sensitivity (CS), color vision, and Humphrey visual field mean deviation (HVF-MD). Results Any abnormal FAF finding was observed in 132 eyes (79.0%); macular abnormalities were observed in 84 eyes (49.1%). The most common findings were peripapillary confluent hypoautofluorescence (122 eyes [73.1%]); extramacular granular hypoautofluorescence (100 eyes [59.9%]); and macular granular hypoautofluorescence (67 eyes [40.1%]). Confluent hypoautofluorescence was related to longer median disease duration (8.7 years) than granular hypoautofluorescence (7.9 years) or hyperautofluorescence (5.6 years). Macular confluent hypoautofluorescence was associated with BCVA ≤20/25 (odds ratio [OR] = 7.83, P = 0.007), BCVA ≤20/50 (OR = 4.94, P = 0.002), and abnormal CS (OR = 4.56, P = 0.009). Presence of macular or extramacular hypoautofluorescence was related to HVF-MD ≤-3 dB (OR = 2.43, P = 0.01 and OR = 2.89, P = 0.003, respectively). Conclusions In this large cohort, various FAF abnormalities were found, indicating that disorders of the retinal pigment epithelium are features of BSCR. Abnormal FAF is a marker of visual dysfunction in the disease.

Use of Retinal Optical Coherence Tomography to Detect Congenital Zika Syndrome

First identified in rhesus monkeys in Uganda in 1947, the Zika virus (ZIKV), a flavirus spread most commonly by the Aedes aegypti and Aedes albopictus mosquitos,1 was not implicated in human disease until 1953, when it was recognized in Nigeria to produce a mild, febrile illness. The virus, related to the pathogens that cause yellow fever, dengue, and the West Nile virus,1 has emerged recently as a serious health care threat resulting in widespread panic across the globe, particularly in the Pacific and the Americas. The recent outbreak began in Brazil in 2015, with over 1 million people now infected by ZIKV, and has since spread to North and South America, as well as Singapore. Most recently, the Florida Health Department has identified cases in several areas of Miami-Dade country in the United States.2 Recent data show that ZIKV is not only transmitted from mosquito to human but also from mother to fetus during pregnancy, as well as sexually transmitted from affected men to their partners.1 Researchers postulate that ZIKV may be shed in the tears of infected mice, which, if true for humans, could be a significant issue in ophthalmic practice.3 The incubation period is approximately 1 week, and common symptoms include a maculopapular pruritic rash, shortterm low-grade fever, arthritis or arthralgias, myalgias, headache, nonpurulent conjunctivitis, retro-orbital pain, edema, and vomiting.1 Zika virus has also been increasingly implicated in neurologic complications such as microcephaly and brain damage in infants of ZIKV-infected mothers, as well as Guillain-Barré syndrome. Ophthalmic manifestations of ZIKV infection are less well characterized. In January 2016, Ventura et al4 were the first to report ocular findings in infants with microcephaly and presumed ZIKV vertical transmission. They presented risk factors associated with ophthalmoscopic findings in infants with presumed congenital ZIKV infection and noted that 14 of 27 mothers who reported symptoms of rash, fever, headaches, and arthralgias in the first trimester of pregnancy had babies with abnormal ophthalmoscopic findings, including optic nerve hypoplasia, disc pallor, and an enlarged cup-disc ratio, as well as macular abnormalities including loss of the foveal reflex, pigment mottling, and chorioretinal atrophy. To date, a single case report of real-time polymerase chain reaction–proven ZIKV-associated uveitis by Furtado et al5 describes a middle-aged man with a 2-day history of rash and bilateral ocular hyperemia who developed anterior uveitis in 1 eye 8 days after onset of systemic symptoms, which were successfully treated with an antibiotic-steroid combination drop. Eight days after treatment, the fellow eye had developed similar symptoms of decreased vision, conjunctival hyperemia, nongranulomatous keratic precipitates, and anterior chamber cells and flare. This eye was also successfully treated with topical corticosteroids for 7 days, with a return of visual acuity to baseline and resolution of uveitis.5 Parke et al6 reported on a 64-year-old man who recently traveled to Haiti and who presented with a diffuse upper body rash, arthralgias, and unilateral decreased vision. His anterior chamber was quiet, but there were retinal pigment epithelial changes in the perifoveal area in a bull’s-eye pattern. Optical coherence tomography (OCT) revealed disruption of the outer retina and retinal pigment epithelial layers in the central macula. Fluorescein angiography revealed early blockage and late staining, and the patient received a diagnosis of unilateral acute idiopathic maculopathy until ZIKV serum plaque reduction neutralization technique assay values came back significantly elevated—strongly indicative of ZIKV infection. His visual acuity improved to 20/20 within 6 weeks without treatment, as did the retinal pigment epithelium and outer retina.6 The treatment of ZIKV infection is supportive only. Vaccines are being actively developed but are not yet available. PreventionofZIKVinfectionisaccomplishedbynottravelingtoendemic areas, with many countries issuing warnings about traveling to these areas, including in the United States, particularly to pregnant women or women who plan to become pregnant in the near future. Warnings are also in effect against unprotected sexual intercourse with potentially affected men. The central nervous system and tropic nature of ZIKV is obvious from the reported clinical presentation, but how and why neural cells bear the brunt of the damage is not yet clear. In the cross-sectional, consecutive case series published in this issue of JAMA Ophthalmology, a small piece of this puzzle is clarified by Ventura et al,7 who are the first to report the retinal and choroidal structural OCT findings in children with posterior segment effects of congenital Zika syndrome. Eight of 9 children had microcephaly, and 8 of 9 had confirmed ZIKV infection based on a positive enzymelinked immunosorbent assay of cerebrospinal fluid samples showing IgM for ZIKV. Optical coherence tomography was performed prior to 5 months of age in all cases. Eleven eyes of the 8 infants had retinal alterations detected by ophthalmoscopy, and OCT was performed on 9 of these 11 affected eyes. The most common OCT findings, found in all scanned eyes, were loss of the outer retina and retinal pigment epithelium, not dissimilar to the case report by Parke et al.6 One important finding is that the retinal effects of intrauterine ZIKV infection are not necessarily bilaterally symmetric. The most severe reported OCT findings consist of complete loss of the inner and outer retina and choriocapillaris. Clinically, this is seen as a “coloboma-like” excavation of the macular area. It was detected in about half of affected eyes. Related article Detecting Congenital Zika Syndrome With Optical Coherence Tomography Invited Commentary

TARANTULA HAIR ASSOCIATED PANUVEITIS TREATED WITH SUSTAINED-RELEASE INTRAVITREAL DEXAMETHASONE IMPLANT.

Purpose: To report a case of tarantula hair–induced panuveitis treated with sustained-release intravitreal dexamethasone implant and followed by sequential spectral domain optical coherence tomography imaging. Methods: Findings on clinical examination, anterior segment optical coherence tomography, corneal in vivo confocal microscopy, color fundus photos, fluorescein angiography, and retinal spectral domain optical coherence tomography are presented. Sequential optical coherence tomography images demonstrated the course of the chorioretinal lesions before and after sustained-release intravitreal dexamethasone implant. Results: A 19-year-old female presented with localized temporal episcleritis and scleritis that incompletely resolved despite multiple courses of topical and oral corticosteroids. She subsequently developed focal vitritis and chorioretinitis, and was found to have tarantula hair–induced panuveitis. Anterior segment optical coherence tomography and in vivo confocal microscopy confirmed the presence of tarantula hairs embedded in the anterior corneal stroma. There was only transient improvement with posterior sub-Tenons Kenalog injection. After treatment with sustained-release intravitreal dexamethasone implant, her symptoms improved and the chorioretinal lesions became inactive. Sequential spectral domain optical coherence tomography images revealed hyperreflective inner retina lesions that progressed to involve the outer retina, and then flattened to near resolution after therapy. Conclusion: This is the first case showing positive clinical outcomes supported by sequential retinal spectral domain optical coherence tomography using a sustained-release intravitreal dexamethasone implant to treat ophthalmia nodosa–induced by tarantula hairs.