Atypical Ocular Presentations of Rosai-Dorfman Disease

Purpose: To report atypical ocular findings of Rosai-Dorfman disease and to determine association with parvovirus 19. Design: The study is an observational case series of three patients that had atypical ocular presentations of Rosai-Dorfman disease. Methods: A multicenter, retrospective case series of 3 patients was evaluated for varied ocular complaints, including enlarging epibulbar masses and uveitis. Histologic specimens were examined retrospectively for parvovirus 19 antigen. Results: Patients presented with ocular findings prior to or concurrently with systemic findings, with and without associated lymphadenopathy. Two cases presented with epibulbar masses, one with the mass as the only sign of disease while the other exhibited multinodal involvement with bilateral epibulbar masses and anterior granulomatous inflammation. Parvovirus B19 antibody staining was negative in these cases. The final case exhibited bilateral anterior granulomatous inflammation and choroidal infiltrates. Conclusions: Ocular findings may be the sole or presenting complaint in Rosai-Dorfman disease prior to recognition of systemic disease and should be considered in the differential diagnosis of epibulbar masses as well as anterior and posterior granulomatous inflammation.     

Bilateral Iris Depigmentation and Ocular Hypotony as End-Stage Manifestations of Untreated Vogt–Koyanagi–Harada Disease

Purpose: To describe severe bilateral iris depigmentation and persistent ocular hypotony as end-stage manifestations of untreated Vogt–Koyanagi–Harada disease.

Methods: We present the clinical findings and diagnostic studies performed for three patients with bilateral iris depigmentation.

Results: Vogt–Koyanagi–Harada disease in late recurrent stage was diagnosed in three patients with bilateral severe iris depigmentation and persistent ocular hypotony.

Conclusions: Early diagnosis and treatment of inflammation are crucial factors in the clinical outcome of Vogt–Koyanagi–Harada disease. When left undiagnosed and untreated from early stages, severe iris depigmentation and ocular hypotony, uncommon manifestations of this disease, can develop.     

Topiramate-induced Bilateral Anterior Uveitis Associated with Hypopyon Formation

Purpose: To report a rare case of bilateral anterior uveitis with hypopyon formation following systemic topiramate use.

Materials and Methods: A 40-year-old woman with migraine headache who was under topiramate treatment referred with bilateral ocular pain and visual blurring. Physical examination disclosed shallow anterior chamber and high intraocular pressure in both eyes. Following discontinuation of topiramate a severe bilateral anterior uveitis with posterior synechiae and hypopyon developed.

Results: Ocular inflammation resolved with systemic and topical steroid. Because of severe cataract and synechiae formation she underwent phacoemulsification/posterior chamber intraocular lens implantation and visual acuity of both eyes improved to 20/25.

Conclusion: Topiramate should be added to the list of drugs that may cause anterior uveitis and hypopyon formation.     

Ocular Sarcoidosis in Southern Taiwan

Purpose: To assess the clinical features of ocular sarcoidosis in southern Taiwan.

Methods: A retrospective chart review of all patients diagnosed with sarcoidosis in Kaohsiung Veterans General Hospital between 1991 and 2008. Age, sex, clinical features, systemic manifestations, biopsy sites, ocular manifestations, and visual acuity were recorded.

Results: A total of 55 sarcoidosis patients were identified. Of these, 19 cases had ocular manifestations, including 5 males and 14 females. Of the total, 41 cases (75%) were confirmed by biopsy. Females were significantly older than males at onset. Posterior segment was involved in 16 cases. In 13 patients, initial presentations were blurred vision (n?=?11) and floaters (n?=?2).

Conclusions: Ocular symptoms are a major initial presentation in sarcoidosis. Posterior segment is commonly involved, and may lead to significant visual impairment if not managed properly. Ophthalmologists should be alert to detect these patients early.     

Clinical Features of Ocular Toxocariasis in Adult Korean Patients

Purpose: To investigate the clinical characteristics and treatment results of adult patients with ocular toxocariasis.

Methods: A total of 54 consecutive patients who were clinically and serologically diagnosed with ocular toxocariasis were retrospectively reviewed.

Results: Among patients, 66.7 and 77.3% showed increases in eosinophil cationic protein (ECP) and total Ig E in serum, respectively. Four eyes (7.2%) initially presented as neuroretinitis with subsequent motile retinal lesion. The recurrence rates in the combination treatment group with albendazole and corticosteroids were significantly lower than those in the steroid-alone group during the mean follow-up of 27.6 months (p?=?0.001).

Conclusions: The adjunctive test of serum total IgE level may be helpful for the diagnosis of ocular toxocariasis. Ocular toxocariasis should be considered in the differential diagnosis of unilateral neuroretinitis with subsequent motile retinal lesion. Combined treatment with albendazole and corticosteroids appeared to be effective for reducing the recurrence of intraocular inflammation.     

Bilateral Corneal Infiltrates and Uveitis in a Pediatric Patient with Presumed Ocular Sarcoidosis

Purpose: To report a pediatric case with presumed ocular sarcoidosis presented with bilateral corneal infiltrates and uveitis.

Design: Single case report.

Methods: A 9-year-old girl presented with a 1-month history of blurred vision and redness in both eyes. Ophthalmologic evaluation revealed midstromal infiltrates in cornea, granulomatous anterior uveitis, vitritis, snowball opacities, and localized perivenous exudates.

Results: She was treated with topical and oral steroids. During steroid tapering, oral cyclosporine A therapy was added.

Conclusion: In patients with corneal infiltrates and uveitis, sarcoidosis should be considered in the differential diagnosis.     

Fluocinolone Acetonide Intravitreal Implants in Vogt-Koyanagi-Harada Disease

Purpose: To describe the use of fluocinolone acetonide implants (Retisert) in Vogt-Koyanagi-Harada disease (VKH).

Design: Interventional case series.

Methods: Retrospective review of medical records.

Results: Two patients with VKH requiring high-dose systemic corticosteroid therapy to control their inflammation and bilateral serous retinal detachments received bilateral fluocinolone acetonide implants. Upon tapering of systemic corticosteroids, one patient had recurrent serous retinal detachments and the other patient’s anterior chamber and vitreous inflammation returned.

Conclusions: The authors’ experience with fluocinolone acetonide implants in VKH has been mixed with an inability to fully taper off of systemic corticosteroids.     

Management Dilemmas of Atypical Ocular Toxoplasmosis Aided by Wide-Field Fluorescein Angiography

Purpose: To report an atypical presentation of ocular toxoplasmosis and to demonstrate the utility of wide-field angiography in delineating peripheral retinal pathology.

Methods: Case Report.

Results: A 17-year-old male presented with acute unilateral papillitis and retinochoroiditis. Laboratory testing supported reactivation of congenitally acquired Toxoplasma gondii infection. Wide-field fluorescein angiography showed extensive retinal vasculitis. The patient was treated with sulfamethoxazole/trimethoprim. Ocular inflammation resolved over a two-week period. There was no documented visual loss.

Conclusion: Wide-field fluorescein angiography details ocular pathology otherwise unnoticed on traditional posterior pole fluorescein angiography. Documentation of extensive vasculitis associated with ocular toxoplasmosis can assist with management decisions.     

Risk Factors for the Development of Ocular Tuberculosis in Patients with Disseminated Tuberculosis

Aim: To identify risk factors for the development of ocular tuberculosis in patients with disseminated tuberculosis.

Methods: A retrospective case-control study. The authors studied all admitted patients with disseminated tuberculosis in two subgroups: patients who developed ocular tuberculosis and those who did not. The potential risk factors analyzed included age, sex, presence of diabetes mellitus, and preexisting immunosuppression.

Results: Of 47 patients, 26 patients (55.3%) developed ocular tuberculosis. Ocular tuberculosis was significantly associated with the presence of preexisting immunosuppression (p?=?.005) using a logistic regression model. Diabetes mellitus, age, and sex were not risk factors.

Conclusions: Preexisting immunosuppression is a risk factor.     

Intermediate Uveitis in a Pediatric Italian Population

Purpose: To investigate clinical data, outcome, and treatment of intermediate uveitis in children.

Methods: Retrospective cohort study, including 116 children affected by intermediate uveitis.

Results: Sixty-two percent of patients were males. Ocular involvement was bilateral in 84%. Mean age at uveitis onset was 10.3?±?3.6 years and 12.2?±?7 years at first visit. Ninety-seven percent were classified as having idiopathic uveitis. The incidence of ocular complications during follow-up was 0.131/eye-year. Macular edema or a marked vitreous haze at presentation may be risks factors for incident visual loss. The most frequent causes of visual loss were macular edema (64%) and cataract (21%).

Conclusions: Most cases are idiopathic (pars planitis); only few children presented associated systemic disease. Macular edema and severe vitritis at presentation are associated with increased risk of visual impairment. Cystoid macular edema is the main cause of visual impairment.     

Ocular Manifestations of Human Immunodeficiency Virus Infection at a Tertiary Referral Center in Taiwan

Purpose: To investigate the prevalence and characteristics of ocular manifestations of human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS) in patients treated at a tertiary referral center in Taiwan during a time of highly active antiretroviral therapy (HAART)

Materials and Methods: A retrospective cohort study in Taiwan was performed between January 2006 and July 2016. Ocular examination and systemic information were recorded from the HIV-infected patients.

Results: 1242 patients with HIV/AIDS were identified. Ninety patients had ophthalmic records, and HIV-related ocular manifestations were reported in 57 patients. The most prevalent ocular manifestations were cytomegalovirus (CMV) retinitis, ocular syphilis, and HIV microvasculopathy. Mean CD4 count was significantly lower in patients with HIV-related ocular manifestations compared to those without.

Conclusion: We found that lower CD4 count, especially <200 cells/μL, was a significant factor for detecting HIV-related ocular manifestations. Comprehensive ophthalmic screening in high-risk group is helpful for early diagnosis and prompt treatment of sight-threatening ocular complications.     

Intravitreal Injection of Sulfamethoxazole and Trimethoprim Associated with Dexamethasone as an Alternative Therapy for Ocular Toxoplasmosis

Purpose: To evaluate intravitreal injections of sulfamethoxazole/trimethoprim in association with dexamethasone for treating toxoplasmic retinochoroiditis.

Methods: Thirteen patients with active, recurrent ocular focal toxoplasmic retinochoroiditis and visual acuity worse than 20/63 in the affected eye were included. Ocular toxoplasmosis was diagnosed according to the classic clinical findings. The primary end point was the change in the final best-corrected visual acuity (BCVA).

Results: The intraocular inflammation decreased within 2 weeks after injection in all eyes and resolved in 8 (62%) eyes with only one injection after 30 days; the remaining eyes received two injections. In all eyes, the retinitis was inactive and no patient had decreased early treatment diabetic retinopathy study lines of BCVA at the final examination.

Conclusion: The combination of intravitreal trimethoprim/sulfamethoxazole and dexamethasone might be an alternative treatment strategy in patients with toxoplasmic retinochoroiditis.     

Development of Acute Retinal Necrosis in a Patient with Ocular Sarcoidosis: A Case Report

Purpose: To report a case of acute retinal necrosis (ARN) caused by varicella-zoster virus (VZV) in an elderly patient with ocular sarcoidosis after oral corticosteroid indication.

Methods: Retrospective case report.

Results: A 75-year-old male with a past history of ocular sarcoidosis came with blurred left vision. Ocular findings in the left eye were consistent with ocular sarcoidosis, while no inflammation in the right eye. On day 14, intraocular inflammation in the left eye resolved by topical corticosteroid, but inflammatory cells were found in the right eye. Suspecting recurrence of ocular sarcoidosis, systemic corticosteroid was initiated. On day 21, inflammation worsened, and the presence of extended yellowish white peripheral retinal lesion in the right eye suggested ARN. Polymerase chain reaction (PCR) testing using ocular fluid detected 3.0 × 107 copies/ml of VZV DNA.

Conclusions: In the case of poor response to immunosuppressive therapy in elderly uveitis, infection including ARN should be considered. Immediate PCR testing for pathogen screening is required.     

Infliximab and Adalimumab for Uveitis

Purpose: To describe the corticosteroid-sparing success in controlling chronic uveitis in patients treated with TNFα inhibitors.

Methods: Retrospective longitudinal case series of patients started on infliximab (n?=?31) or adalimumab (n?=?12) for chronic noninfectious uveitis at a tertiary referral center. The main outcome was corticosteroid-sparing success. Secondary outcomes were sustained control of inflammation regardless of corticosteroid-sparing effect, tapering of concurrent nonbiologic therapy, and discontinuation.

Results: Sustained control of inflammation with corticosteroid-sparing success on infliximab and adalimumab, respectively, was achieved in 33.3 and 37.5% at 3 months, 60.7 and 62.5% at 6 months, and 60.9 and 57.1% at 12 months. Median time to this outcome was 98 days for infliximab and 169 days for adalimumab. Six infliximab patients had adverse reactions.

Conclusions: Infliximab and adalimumab improve control of ocular inflammation and are successful corticosteroid-sparing agents. However, time to corticosteroid-sparing control of inflammation may take a few months with either agent, and adverse reactions may limit treatment.     

Clinical Characteristics of Ocular Sarcoidosis: A Population-Based Study 1976–2013

Purpose: To characterize the epidemiology and clinical characteristics of ocular involvement in patients with systemic sarcoidosis.

Methods: An inception cohort of patients with systemic sarcoidosis in 1976–2013 in Olmsted County, Minnesota, was identified based on comprehensive individual medical record review. Medical records of those patients were then reviewed for ocular involvement.

Results: A total of 345 incident cases of systemic sarcoidosis were identified. Ocular involvement occurred in 23 patients (7%). The most common ocular disease was uveitis (61%) followed by dry eye disease, conjunctival nodule, episcleritis, anterior scleritis, and conjunctivitis. Anterior uveitis was the most common type of uveitis (71%). The visual outcome of uveitis was favorable with only one patient lost three or more lines of VA during follow-up and had VA of less than 20/200 at last visit.

Conclusion: Ocular involvement occurred in 7% of sarcoidosis patients. Uveitis was the most common type of ocular disease.     

Ocular Whipple Disease: Report of Three Cases

Purpose: To emphasize the different manifestations of ocular involvement in Whipple disease with challenge in establishing the diagnosis as clinical, laboratory, and histological features could mimic other uveitis entities.

Methods: Case reports of three patients.

Results: The first patient was an African male suffering from a chronic bilateral keratoconjunctitivitis that was initially misdiagnosed as a chronic allergic conjunctivitis. The second patient was an Italian female who presented with bilateral vitritis, whereas the third patient was an Italian male suffering from a chronic bilateral panuveitis. The diagnosis of ocular Whipple in the first and third case was made by a positive T. whipplei PCR from the ocular specimen, and the second patient had detection of T. whipplei from extraocular sites.

Conclusions: Whipple disease can have protean manifestations in the eye including an isolated ocular surface involvement manifested as keratitis.     

Randomized Controlled Study to Evaluate the Efficacy of Adalimumab in Patients with Different Forms of Refractory Uveitis

Purpose: TNF alpha inhibitors have revolutionized the care of vision-threatening uveitis. This study evaluated the efficacy of adalimumab (ADA) for the treatment of refractory noninfectious uveitis.

Design: Randomized, prospective, controlled, two-center clinical trial

Methods: Patients with active uveitis despite combined oral low-dose prednisolone and immunosuppression were randomized for additional ADA with corticosteroids in a fixed tapering regime, or corticosteroids only. Primary outcome measure at three months was improved best-corrected visual acuity (BCVA; >2 lines). In case of treatment failure, switch to the other arm was possible.

Results: Twenty-five patients (10 ADA, 15 controls) were included. BCVA increased with ADA by > 2 lines in 6/10 patients (60%; mean increase of 0.23 logMAR), but in only 2/15 from controls (13%, mean increase of 0.04 logMAR, Fisher´s exact test p = 0.00221).

Conclusions: The results show superiority of ADA over controls in severe ocular inflammation including anterior uveitis.     

Chronic Anterior Uveitis Associated with Relapsing Polychondritis: A Case Report

ABSTRACT

Purpose: To report a case of chronic anterior uveitis associated with relapsing polychondritis (RP).

Case Report: A 43-year-old female patient presenting with bilateral progressive vision loss was diagnosed with bilateral anterior uveitis. According to her clinical signs and symptoms and biopsy results, RP was ultimately diagnosed. Her ocular inflammation was controlled with corticosteroids and systemic immunosuppressive treatment, and the symptoms related to RP improved.

Conclusion: Chronic uveitis is an inflammatory disease that may be associated with many systemic autoimmune diseases. RP should especially be considered in patients with bilateral chronic uveitis, and a relevant detailed history should be obtained for early and accurate diagnosis and treatment.     

Periocular dirofilariasis: A case series

Objective: To cite a series of patients presenting with complaints of a lid swelling associated with preseptal cellulitis.

Methods: Three patients on three different occasions came with complaints of a unilateral lid swelling associated with preseptal cellulitis. Examination showed palpable mass in the upper eyelid localized, firm in consistency and was freely mobile and occasionally disappeared during palpation. CT scan showed nonspecific preseptal inflammation. A trial of antibiotics was given in each of the cases with temporary reduction in the swelling but a consecutive flare up following which patient was subjected to excision biopsy. Each of the biopsies was subjected to histopathological and microbiological examination.

Results: On excision biopsy in all the three cases it was reported to be a parasitic infestation belonging to the genus Dirofilaria.Conclusions: Dirofilariaiasis is a common zoonotic infection among wild animals but rarely infects human beings with ocular involvement. Hence dirofilariasis should be considered as a differential diagnosis for migratory subcutaneous swellings and conjunctival nodules in the southern belt of India.     

Ophthalmic presentation of Wegener's granulomatosis on a background of polymyalgia rheumatica

We present a case of Wegener's granulomatosis (WG) in a 79‐year‐old man with limbitis and granulomatous conjunctivitis, on a background of polymyalgia rheumatica (PMR). The undifferentiated nature of ocular presentations of WG can be diagnostically challenging, especially in cases, such as this, where findings are initially inconclusive and evolve with time. This case highlights the significance of a history of PMR in patients with ocular inflammation. The systemic inflammatory systems of WG, including arthralgias, may mimic other conditions such as PMR. Patients with undifferentiated ocular inflammatory syndromes should be questioned regarding arthralgias, myalgias and stiffness. Such symptoms, or a background of PMR, should raise suspicion of WG.