Multiple Evanescent White Dot Syndrome Following Intradermal Influenza Vaccination

Purpose: To present a case report of MEWDS following an intradermal influenza vaccination.

Methods: Review of the clinical, laboratory, photographic, and angiographic records of a patient with MEWDS.

Results: 27-year-old female that presented to our clinic with photopsias OD. These symptoms began 2 weeks following an influenza intradermal vaccination.

Fundus examination and diagnostic testing were consistent with MEWDS.

Conclusion: While rare, MEWDS can occur following influenza vaccination.     

Total Corneal Melt in Patient with Porphyria Cutanea Tarda in Presence of Another Risk Factor

Purpose: To report a case of two siblings affected by porphyria cutanea tarda (PCT) presenting with varying severity of ocular disease and evaluation of difference in the presence of risk factors.

Methods: Case report

Result: A 42-year-old Indian male diagnosed with PCT and history of daily intake of alcohol, presented with sudden decrease in vision of right eye. Examination showed perforated cornea surrounded by rim of 360-degree avascular necrotic sclera, with no evidence of infection.

Similar systemic history was present in his elder brother. He did not consume alcohol and presented with mere localized scleral thinning. Since the patient’s sibling acted as a natural control for the evaluation of alcohol as an independent risk factor, the difference in severity of disease can be explained on the basis of difference in alcohol intake.

Conclusion: In the presence of progression of the disease, other risk factors like intake of alcohol should be evaluated.     

Evidence of retinal degeneration in Wolfram syndrome

Purpose: Wolfram syndrome is characterized by early onset diabetes mellitus, diabetes insipidus, deafness, and optic atrophy, but retinal degeneration has not been described as a major component of the phenotype. We present two cases with Wolfram syndrome and evidence of retinal degeneration.

Materials and Methods: Observational case series. Patients underwent complete ocular examinations as well as retinal imaging and electroretinography.

Results: Both patients had electroretinographic evidence of retinal dysfunction/degeneration in addition to optic atrophy with an otherwise normal-appearing retina.

Conclusions: Some patients with Wolfram syndrome have a mild retinal degeneration that may be a manifestation of the neuronal involvement that is present in this condition.     

Ocular Complications in Influenza Virus Infection

Purpose: To describe a case series of ocular complications associated with upper respiratory tract infections.

Methods: Four patients aged 21–61 years (three females, one male) had confirmed ocular complications connected with a general upper respiratory tract infection with myalgia and fever. Ophthalmological examination, including a visual acuity test, a slit-lamp exam, intraocular pressure measurements, fluorescein and indocyanine green angiography, optical coherence tomography (OCT), and diagnostic tests for influenza were performed in the patients (RT-PCR, HAI).

Results: Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) was diagnosed in three patients and serous macular detachment (SME) in one. Influenza virus infection was confirmed by molecular biological methods (RT-PCR) or the hemagglutination inhibition test (HAI) in two patients. All patients were treated with systemic prednisone.

Conclusion: A coincidence between APMPPE and SME epitheliopathy and influenza virus infection was observed in different months of a given epidemic season.     

Amniotic Membrane Transplant for Bullous Keratopathy: Confocal Microscopy & Anterior Segment Optical Coherence Tomography

Purpose: To assess amniotic membrane retention after amniotic membrane transplant in bullous keratopathy patients and whether there were any corneal structural changes that may hinder further penetrating keratoplasty

Methods: A retrospective study including 22 patients who have undergone amniotic membrane transplant from 1 Jan 1998 till 30 Jun 2016. Confocal microscopy and anterior segment optical coherence tomography (ASOCT) were performed to assess the retention of amniotic membrane and to detect any corneal structural changes. The comparison was made with 5 controls who had bullous keratopathy awaiting endothelial keratoplasty.

Results: Patients had a mean follow-up of 61 ± 33.7 months. Pain reduction was significant (p < .001) although it did not significantly correlate with the regularity of the superficial, intermediate or basal epithelial layers, nor with the retention of the amniotic membrane. No long-term structural changes that may hinder future penetrating keratoplasty were detected.

Conclusion: This procedure is a safe and effective long-term treatment for symptomatic bullous keratopathy patients.     

A giant dermoid cyst of the orbit

To describe a unique case of a non-germinomatous germ cell tumor, a giant orbital dermoid, present in adulthood.

A 31-year-old male presented in our clinic with gradual, painless, progressive proptosis in the left eye since 28 years associated with gradual loss of vision. The left eye showed gross proptosis. Ocular structures could not be made out. Computed tomography scan showed a well-circumscribed oval heterogeneous mixed solid and cystic lesion completely filling the left orbit with calcification. The differential diagnosis was that of either teratoma or optic nerve glioma.

The patient underwent excision of the lesion. Histopathology revealed a capsulated tumor with multiple cystic components filled with blood and eosinophilic material suggestive of a dermoid cyst.

Non-germinomatous germ cell tumors may present atypically in adults and neglected benign dermoid cysts can attain massive size mimicking malignant lesions.     

Quality of Life and Psychological Aspects in Patients with Visual Impairment Secondary to Uveitis: A Clinical Study in a Tertiary Care Hospital in Brazil

Purpose: To evaluate the quality of life in individuals with bilateral visual impairment secondary to uveitis and its association with sociodemographic, clinical and psychosocial aspects.

Methods: Data were collected through a structured interviews, using four forms; the SF-12v2, HADS, NEI-VFQ-25 and a specific form developed to collect clinical and sociodemographic data.

Results: 80 patients were included with a mean age of 42.8 years; 63.8% female; 57.5% with permanent reduction of familiar income. The average number of medical visits was 15.2 ±10.7/year. In terms of health-related quality of life (HRQol), the mean of VFQ-25 score was 36.6 ± 15.1 and the mean of SF-12 physical and mental components scores were 43.3 ± 9.9 and 44.9 ± 12.2. Anxiety and depression symptoms were found in 65.1% and 32.5% of patients, respectively.

Conclusions: Visual impairment has impact on several scales of HRQoL leading to productivity loss and consumption of health resources.     

The Role of IFN-alpha in Experimental and Clinical Uveitis

Purpose: IFN-α is the only treatment capable of inducing long-term remission in some patients with ocular Behçet’s disease. In this review, we focus on immune mechanisms of IFN-α in animal models and patients and compare the outcome of different clinical studies.

Methods: Review of literature using PubMed and Google and original data from rat models with monophasic/chronic or relapsing experimental autoimmune uveitis treated with IFN-α.

Results: The role of IFN-α and its effect on various cell types were investigated, in some cases with contradictory results. Some patients respond very well to IFN-α treatment, while others are non-responders, which was reflected in the uveitis rat models: relapsing uveitis was ameliorated, the monophasic/chronic disease even aggravated.

Conclusions: Despite intensive investigations in patients and animal models, the immune mechanisms explaining the therapeutic effect of IFN-α in ocular Behçet’s disease are not yet fully understood and need further investigation.     

Systematic Review of the Yield of Temporal Artery Biopsy for Suspected Giant Cell Arteritis

Purpose: To determine the positive yield (utility rate) of temporal artery biopsy (TAB) in patients with suspected giant cell arteritis (GCA).

Study Design: Systematic review (CRD42017078508) and meta-regression.

Materials and Methods: All articles concerning TAB for suspected GCA with English language abstracts from 1998 to 2017 were retrieved. Articles were excluded if they exclusively reported positive TAB, or only cases of known GCA. Where available, the pre-specified predictors of age, sex, vision symptoms, jaw claudication, duration of steroid treatment prior to TAB, specimen length, bilateral TAB, and use of ultrasound/MRI (imaging) were recorded for meta-regression.

Results: One hundred and thirteen articles met eligibility criteria. The I² was 92%, and with such high heterogeneity, meta-analysis is unsuitable. The median yield of TAB was 0.25 (95% confidence interval 0.21 to 0.27), with interquartile range 0.17 to 0.34. On univariate meta-regression age (coefficient 0.012, p = 0.025) was the only statistically significant patient factor associated with TAB yield.

Conclusions: Systematic review revealed high heterogeneity in the yield of TAB. The median utility rate of 25% and its interquartile range provides a benchmark for decisions regarding the under/overutilization of TAB and aids in the evaluation of non-invasive alternatives for the investigation of GCA.     

Challenging Treatment of Ocular Surface Squamous Neoplasia in Patients with Atopic Disease

Purpose: Few studies have described ocular surface squamous neoplasia (OSSN) and its association with atopic diseases and there is no consensus on the course of OSSN in atopic patients. We thereby report three patients with atopy and OSSN.

Methods: Retrospective case series.

Results: Three male patients with mean age of 73 presented with OSSN and history of atopy treated with immunosuppressant therapy. Their histories included atopic dermatitis and keratoconjunctivitis. All patients had treatment complicated by multiple surgeries, recurrences, or advanced disease. The patients initially received medical treatment with topical interferon-alpha-2b (IFNα2b). However, all the patients had recurrences and required modification of treatment including topical 5-fluorouracil (5-FU).

Conclusion: We report on three patients with a history of atopy whose OSSN presentation and course was challenging. Overall, our cases responded better to topical 5-fluorouracil compared to topical interferon-alpha-2b, but recurrences were common. These patients may benefit from more aggressive and long-term treatment.     

Ultra-Widefield Fluorescein Angiography in Intermediate Uveitis

Purpose: To examine associations between pattern of vascular leakage on ultrawide-field fluorescein angiography (UWFFA) and visual acuity, cystoid macular edema (CME), and inflammatory activity in intermediate uveitis.

Methods: Single center cross-sectional, retrospective review of medical records, spectral domain optical coherence tomography (SD-OCT) and angiographic images of intermediate uveitis patients who underwent UWFFA over a 12-month period.

Results: Forty-one eyes from 24 patients were included. Twelve eyes (29%) exhibited peripheral leakage, 26 eyes (64%) had diffuse leakage and three eyes (7%) had no leakage. Diffuse leakage was associated with 0.2 logMAR worse visual acuity than peripheral leakage (p = 0.02). There was no statistically significant difference in the odds of having CME when diffuse leakage was compared to peripheral leakage.

Conclusion: UWFFA identifies retinal vascular pathology in intermediate uveitis not present on clinical examination. Diffuse retinal vascular leakage was associated with worse visual acuity when compared to peripheral and no leakage patterns.     

Enhanced Vitreous Imaging in Uveitis

Purpose: To study the structural visibility of the posterior vitreous cavity and vitreoretinal interface using Spectral Domain–Optical Coherence Tomography in patients with uveitis using conventional, Enhanced Depth Imaging (EDI), Combined Depth Imaging (CDI), and Enhanced Vitreous Imaging (EVI) techniques.

Methods: It was an observational cross-sectional study of 59 eyes of 33 patients between age group 12–72 years (Median 43) diagnosed as uveitis with posterior segment manifestations underwent OCT using conventional, EDI, CDI, and EVI techniques. The visibility of posterior vitreous was graded in all the techniques.

Results: A statistically significant difference was seen in posterior vitreous visibility on comparing all four techniques. EVI showed better visibility of posterior vitreous cavity compared to all other techniques (P < 0.001).

Conclusions: EVI technique provides better structural visibility of posterior vitreous compared to conventional, EDI, and CDI techniques.     

Clinical Profile of Scleritis in Children

Purpose: To describe the clinical pattern of scleritis in patients ≤16 years of age at a tertiary care eye hospital in India.

Methods: The medical records of 24 eyes of 20 children with scleritis were included in this retrospective study. Collected data included age, subsets of uveitis, best corrected visual acuities (BCVA), detailed laboratory investigations and treatment.

Results: Mean age at presentation was 12.2 ± 2.5 years. Scleritis was unilateral in 80% patients and an equal gender distribution was observed. Posterior scleritis (41.7%) was the most common subtypes of scleritis and nodular scleritis was the most common type of anterior scleritis (33.3%). Necrotizing scleritis was seen in 16.7% eyes. Tuberculosis was the only systemic association and found in three (15%) patients.

Conclusions: Clinical profile of scleritis in children can be different from that of adults.     

Clinical Profile,Ultra-Wide-Field Fluorescence Angiography Findings,and Long-Term Prognosis of Uveitis in Tubulointerstitial Nephritis and Uveitis Syndrome at One Tertiary Medical Institute in China

Purpose: To analyze the clinical features and long-term prognosis of uveitis in tubulointerstitial nephritis and uveitis (TINU) syndrome in a cohort of Chinese patients.

Methods: A total of 32 patients with TINU syndrome between 2000 - 2016 were analyzed retrospectively.

Results: Mild anterior inflammation was observed in all cases. Ultra-wide-field fluorescence angiography (UWFA) was conducted on 13 patients, and peripheral vascular leakage was observed in 22/26 eyes (84.62%) compared with active anterior chamber inflammation in 13/26 eyes (50%). Three patients received increased corticosteroid dosage therapy on their first episode of uveitis guided by UWFA had stopped steroids without recurrence.

Conclusion: The uveitis in TINU syndrome was mild. Corticosteroids were efficient in most cases, but a slower tapering and long-term treatment were required. UWFA is sensitive in detecting the activity of uveitis and might be useful in monitoring disease progression. Complete withdrawal of medication was difficult to achieve in some cases.     

Biologic Treatment Options for Retinal Neovascularization in Behçet’s Disease

Purpose: Relapsing ocular inflammation occurs in about 70% of patients with Behçet’s disease (BD) and can lead to permanent loss of vision. Neovascularization of the optic disc (NVD) or elsewhere in the retina (NVE) is a relatively uncommon but severe complication that lacks standardized treatment.

Methods: We report on the therapeutic use of anti-TNF monoclonal antibodies for BD-associated NVD and NVE in one pediatric patient (subcutaneous adalimumab) and one young man (intravenous infliximab). Also, we review the previously published experience on biologic therapeutic options, namely anti-TNF agents and interferon-alpha in a total of three and eight patients, respectively.

Results: A fast-onset therapeutic effect was observed in both patients leading to complete regression of neovascularizations.

Conclusions: Both options may lead to regression of neovascularization, thus preventing loss of vision, but comparative studies need to determine the optimal treatment for this sight-threatening complication of BD.     

Polymerase Chain Reaction and Goldmann-Witmer Coefficient to Examine the Role of Epstein-Barr Virus in Uveitis

Purpose: To use polymerase chain reaction (PCR) and Goldmann-Witmer Coefficient (GWC) calculation to search for evidence that Epstein-Barr virus (EBV) causes uveitis.

Methods: A prospective cross-sectional study where participants with positive multiplex EBV PCR results were further investigated by: 1) real-time PCR for EBV viral loads (VL) and 2) EBV GWC.

Results: Eleven of 106 consecutive uveitis patients (10.4%) had positive multiplex PCR for EBV on aqueous humor sampling and 7/11 (63.6%) were HIV-positive. Only 4/10 (40%) cases had detectable intraocular EBV VLs which were always lower than the blood or plasma VL. EBV GWC was negative in all 10 cases tested. In 9/11 (81.8%) of these cases an alternative, more plausible cause of uveitis was identified.

Conclusion: We found no evidence of active intraocular replication or antibody production to prove that EBV caused uveitis in these cases. In most cases an alternative treatable cause of uveitis was identified.     

Induction of Vogt-Koyanagi-Harada Disease by Interferon-Alpha and Ribavirin Treatment in Patients with Hepatitis C: A Case Report and Review of the Literature

Purpose: To describe a case of Vogt-Koyanagi-Harada disease (VKH) presenting in a hepatitis C patient after interferon-alpha (IFN-α) and ribavirin treatment.

Methods: A retrospective review of our patient and a review of the published literature.

Results: A 59 year-old man developed VKH after the four months of IFN-α and ribavirin therapy for hepatitis C. The patient’s VKH was controlled by systemic corticosteroids. The relationship between VKH and IFN-α is discussed based on the published literature.

Conclusions: VKH is a rare autoimmune complication triggered by interferon-alpha therapy; the T-cell modulatory properties of IFN-α possibly contribute to this association. Early diagnosis of VKH and aggressive systemic corticosteroid intervention are essential for this type of IFN-α -related autoimmune complication.     

Multiple Evanescent White Dot Syndrome: A Multimodal Imaging Study of Foveal Granularity

Purpose: To describe multiple evanescent white dot syndrome (MEWDS)-related foveal granularity features on different imaging modalities.

Methods: Clinical and multi-imaging files from five patients affected by MEWDS were reviewed. Each image set included: blue fundus autofluorescence (BAF), near-infrared fundus autofluorescence (NIR-FAF), spectral-domain optical coherence tomography (SD-OCT), fluorescein (FA), and indocyanine green (ICGA) angiography. Foveal granularity features were analyzed and described for each technique at baseline and follow-up.

Results: At baseline, 4/5 patients did not show white dots at funduscopic examination, but all subjects demonstrated hypo-fluorescent lesions on ICGA and areas of increased BAF. In all patients, foveal granularity was detectable on NIR-FAF as an irregular hypo-fluorescent area, persisting during follow-up visits. The corresponding SD-OCT scans revealed outer retinal layers’ disruption resolving overtime.

Conclusions: Foveal granularity can be the sole presenting sign of MEWDS. NIR-FAF and SD-OCT should be considered as non-invasive investigations in the detection of MEWDS-related foveal granularity.     

Conjunctival Flap with Biodegradable Collagen Matrix Implantation for the Treatment of Scleromalacia after Periocular Surgery

Purpose: To report a new surgical procedure using biodegradable collagen matrix (Ologen) implantation with conjunctival flap for reconstruction of scleromalacia after periocular surgery.

Methods: A total of 26 areas of 25 eyes in 25 patients were included. We divided all eyes according to the preoperative extent of calcified plaque or scleral defect as follows: small, large, and large and severe. Morphologic outcomes were graded from 0 to 7, with higher scores indicating worse cosmetic outcomes.

Results: Mean follow-up period was 8.3 ± 5.6 months. No eyes showed recurrence or specific complications related to Ologen such as implant extrusion or allergic reaction. Minor complications such as flap vessel engorgement and flap hypertrophy were easily treated. Mean morphologic outcome scores were 1.8, 2.4, and 5.3 in groups 1, 2, and 3, respectively.

Discussion: The combined surgery may be an effective and safe procedure for the treatment of scleromalacia after periocular surgeries.     

Orbital Inflammatory Syndrome Post Group A Streptococcal Infection — Case Series and Review of Literature

Objective: To report new cases of orbital inflammatory syndrome (OIS) post group A streptococcal (GAS) infection and review the literature.

Methods: A retrospective case series of patients with OIS post GAS infection seen in our clinic (from January 2015 to December 2016) and review of additional patients from the literature.

Results: A total of nine patients with OIS post GAS infection were included in the study (four patients seen in our clinic and five from previous literature); seven females and two males, three children (under the age of 16), and six adults. Eight patients (89%) presented with a single rectus muscle (5 medial, 3 lateral) myositis, and one (11%) presented with posterior scleritis and diffuse OIS.

Conclusions: Although there are very few reports on OIS post GAS infection, it may be more common than previously thought and should be considered in the differential diagnosis of patients with orbital inflammation.