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.     

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.     

A novel missense variant in IDH3A causes autosomal recessive retinitis pigmentosa

Background: Inherited retinal degenerations (IRDs) encompass a wide spectrum of genetic ocular diseases characterized by considerable genetic and clinical heterogeneity.

Methods: Complete ophthalmic examination and next-generation sequencing.

Results: We describe a patient with no family history of vision loss, who at the age of 28 years developed visual impairment consistent with a severe form of retinitis pigmentosa. Genetic testing by means of whole exome sequencing identified a homozygous variant in the gene IDH3A. To date, only three papers have reported mutations in IDH3A, in families with early-onset retinal degeneration with or without the presence of macular pseudocoloboma.

Conclusion: This study highlights the importance of including this rarely-mutated gene in the molecular diagnostic set-ups for IRDs, and further delineates the phenotypic spectrum elicited by mutations in IDH3A.     

Paradoxical response to carbonic anhydrase inhibitors in patients with intraretinal cystoid spaces

Background: Intraretinal cystoid spaces (IRCS) are fluid-filled spaces seen in some retinal dystrophies and often treated with carbonic anhydrase inhibitors. The purpose of this study is to report an unexpected bilateral improvement in the IRCS after discontinuation of therapy.

Material and Methods: We identified from our records 23 patients with retinal dystrophy and IRCS who had been treated with topical and/or oral carbonic anhydrase inhibitors. All subjects had regular follow-up with OCT and previous genetic testing.

Results: We identified four (17%) patients who experienced a bilateral and symmetrical paradoxical improvement in IRCS size and visual acuity after discontinuation of carbonic anhydrase inhibitors. Two were mutations in RS1, one in CLN3 and another in NR2E3. All patients were followed for at least three years (range 39–63 months). None had systemic abnormalities.

Conclusions: Patients with IRCS may exhibit a paradoxical response after discontinuation of carbonic anhydrase inhibitors. Although the pathophysiology of these phenomena is unclear, stopping treatment may be an option in patients who cease to improve or get worse on treatment.     

Retinal Pigment Epithelium Detachment in Acute Vogt–Koyanagi–Harada Disease: An Unusual Finding at Presentation

Purpose: To report the cases of two patients who presented with acute Vogt–Koyanagi–Harada (VKH) disease and associated retinal pigment epithelium detachment (PED).

Methods: 2 case reports. Case 1: A 22-year-old male patient complained of acute bilateral decrease in vision. Initial best corrected visual acuity (BCVA) was 20/63, OU. There was 1+ vitreous cells, OU, exudative retinal detachment (ERD) in the right eye (RE), and a macular deep yellow lesion suggestive of PED in the left eye (LE). Case 2: A 40-year-old female patient presented with bilateral vision blurring. BCVA was 20/40 in the RE and 20/32 in the LE. There was 1+ vitreous cells OU, bilateral optic disc swelling and retinal striae.

Results: Multimodal imaging showed features of acute VKH disease with associated PED in the two patients. ERD and PED resolved under oral prednisone and cyclosporine in both cases.

Conclusion: PED may rarely occur in acute VKH disease at presentation.     

Involutional entropion repair combining the modified Bick quick strip procedure with Quickert rotational sutures

Purpose: The purpose of the study was to determine the results of involutional entropion repair by combining the modified Bick strip procedure with Quickert rotational sutures.

Methods: A retrospective review of consecutive patients with involutional entropion who underwent repair by combined Bick and Quickert technique from January 2013 to December 2017 was performed.

Results: A total of 43 cases of involutional entropion repair were preformed in this time period. Fourteen eyelids of 12 patients had the combined procedure. There were no failures with a median follow-up of 29 months. Mean operative time, recorded in 10 patients, was 15 ± 2.2 minutes. Complications were minimal.

Conclusions: The combination of the modified Bick quick strip procedure and Quickert sutures requires minimal surgical dissection and provides excellent results for the treatment of involutional entropion.     

Anterior and Intermediate Uveitis Following Yellow Fever Vaccination with Fractional Dose: Case Reports

Purpose: To describe two cases of anterior and intermediate uveitis following yellow fever vaccination with fractional dose.

Methods: Case report.

Results: Case 1: A 35 year-old healthy woman presented with unilateral anterior uveitis 10 days after the yellow fever vaccination. Testing excluded infectious and rheumatic diseases and the episode was fully recovered after a short course of topical treatment. Case 2: A 21 year-old previously healthy woman presented with blurred vision in the left eye (OS) 14 days after the yellow fever vaccination. The ophthalmic examination of the OS revealed intermediated uveitis. Testing excluded infectious and neurological diseases. After six weeks of treatment with oral prednisone, the ocular inflammation had resolved.

Conclusion: Physicians should be aware of the possibility of eye inflammation following the yellow fever vaccination.     

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.     

Time Course of Subretinal Proliferation after Scleral Buckling for Rhegmatogenous Retinal Detachment Repair

Purpose: To observe course of subretinal proliferation (SRP) after scleral buckling (SB) for the rhegmatogenous retinal detachment.

Methods: Fundus photographs and serial optical coherence tomographies (OCTs) were retrospectively analyzed in 10 cases from 9 patients.

Results: Preoperative SRP locations were beneath the retina in six cases, intraretinal in one, and between the retina and retinal pigment epithelium (RPE) in one. One demonstrated SRP heterogeneity. Final SRP locations were intraretinal in two, on the RPE in seven, and beneath the retina in one. In two, the SRP invaginated into the retina while being absorbed.

Conclusions: Detached retina can be reattached following retinal break occlusion by SB, although the remaining SRP can disturb the reattachment. OCT images of SRP demonstrated various features. Before surgery, the SRPs were typically strand-like types located beneath the retina. Postoperatively, they were located on the RPE. In some cases, they invaginated into the retina.     

Horizontal gaze palsy and progressive scoliosis with two novel ROBO3 gene mutations in two Jordanian families

Background: Horizontal gaze palsy and progressive scoliosis (HGPPS) is a rare autosomal recessive disorder due to mutations in ROBO3 gene. Patients have characteristic clinical and imaging findings. We report six patients from two families with this disorder with two novel mutations.

Materials and methods: One patient from a non-consanguineous family and five patients from extended consanguineous families were clinically and radiologically examined. Blood samples from the patients and their parents were obtained and all the coding exons and flanking intronic sequences of the ROBO3 gene were amplified and subjected to bidirectional DNA sequencing.

Results: All six patients had the characteristic clinical and radiological findings of HGPPS. Genetic testing showed two novel mutations including frame-shift and nonsense.

Conclusion: Two novel mutations in the ROBO3 gene were identified in two Jordanian families with six affected individuals. To our knowledge, this is the first molecular study of HGPPS in Jordan.     

Temporomandibular Disorder: An Important Cause of Temporal Headache in Patients Unlikely to Have Giant Cell Arteritis

Introduction: Temporal headache often results in an Ophthalmology referral to rule out giant cell arteritis (GCA). When clinical suspicion of GCA is low, alternative diagnoses should be considered. Temporomandibular disorder (TMD) commonly causes a temporal headache.

Materials and Methods: Patients referred with a temporal headache attending the Friday emergency ophthalmology clinic from February to July 2017 were assessed for GCA using the American College of Rheumatology (ACR) diagnostic criteria and TMD using a validated screening questionnaire.

Results: Ten symptomatic patients presented and were screened for TMD during this period. Four had mild TMD, two moderate, and three severe. The mean TMD score in patients with less than 3 ACR GCA criteria was 14.75 (SD 4.03) versus 6.8 (SD 3.06) in patients with 3 or more criteria (p = 0.0075).

Conclusion: TMD is an important cause of a temporal headache in patients unlikely to have GCA. Onward referral to dentistry for further management may be merited.     

The ‘over-the-top’ modified Cutler–Beard procedure for complete upper eyelid defect reconstruction

Purpose: To describe the ‘Over-the-Top’ Modified Cutler Beard Procedure (OTTMCB) for complete upper eyelid defect reconstruction

Methods: This is retrospective case note review of four patients that underwent the OTTMCB procedure. This two-stage procedure comprises the replacement of the posterior lamella with a free tarsal graft from the contralateral upper eyelid, and the anterior lamella with a lower lid skin flap over the lower eyelid margin which is divided 2–4 weeks later.

Results: The procedure was undertaken for four patients with 75–90% upper eyelid defects from tumour excision surgery. One patient had post-operative dehiscence requiring debridement and resuturing and further reconstructive surgery 18 months later to improve the cosmesis and lagophthalmos. The other three patients had good functional and cosmetic outcomes.

Conclusions: The OTTMCB procedure replaces the anterior and posterior lamellae of the upper eyelid with ‘like-for-like’ tissues. It avoids some of the drawbacks of the original and other variations of the Cutler–Beard procedure and achieves a good cosmetic outcome.     

Role of OCT Angiography in the Detection of Retinal Vascular and Macular Abnormalities in Subjects with Asteroid Hyalosis

Purpose: To study the role of Optical coherence tomography (OCT) Angiography (OCTA) in detecting retinal vascular and macular abnormalities as compared to Fundus Fluorescein Angiography (FFA) in subjects with Asteroid hyalosis (AH).

Methods: In a prospective study, patients with AH underwent OCTA and FFA. AH graded as Grade 1 in 8 (optic disc, second order vessels visible), Grade 2 in 4 (optic disc, first-order vessels visible), Grade 3 in 11 (hazy view of optic disc) and Grade 4 (no view of fundus) in 2 eyes.

Results: A total of 20 patients (25 eyes) with AH were included. In comparison to FFA, OCTA was able to similarly detect Diabetic Retinopathy changes such as Neovascularization, microaneurysms, capillary dropout, and foveal avascular zone extent in vascular occlusion in all grades of AH.

Conclusion: OCTA, a noninvasive imaging tool, could detect various retinal vascular and macular abnormalities in patients with all grades of AH in comparison to FFA.     

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.     

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.     

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.     

Calcitriol,the Active Metabolite of Vitamin D3, Inhibits Dry Eye Related Corneal Inflammation In Vivo and In Vitro

Purpose: To examine the influence of topical administration of calcitriol on dry eye (DE) related corneal inflammation.

Methods: Benzalkonium chloride (BAC, 0.2%) was applied to induce DE. Then rats were treated topically with calcitriol (10^?6μM). Tear break-up time (TBUT), fluorescein staining score, inflammatory index, and tear volume were measured. Corneal epithelium damage and corneal inflammation were examined by H&E staining or RT-qPCR. In vitro, human corneal epithelial cells (iHCEC) were cultured in hyperosmotic medium (450 mOsM) with various concentrations of calcitriol. Levels of pro-inflammatory mediators were measured by RT-qPCR or ELISA. NF-κB activation was examined by Western blotting and immunofluorescence staining.

Results: Calcitriol significantly ameliorated DE symptoms, attenuating corneal inflammation. In vitro studies showed that calcitriol significantly decreased the expression of pro-inflammatory mediators in iHCECs under hyperosmotic stress, probably through inhibiting NF-κB activation.

Conclusion: The results suggest that calcitriol might be a potential therapeutic agent for DE.     

Revision eDCR using a superior pedicled mucosal flap

Background: Endoscopic dacryocystorhinostomies (eDCRs) show patency rates between 81% and 94%. However, dacryocystorhinostomy (DCR) failure and the need for revision remain a significant challenge. One of the principal challenges in revision eDCR is the need to surgically identify the correct osteotomy site and maintain long-term patency in the setting of previously instrumented and potentially scarred tissue. At the same time, the surgeon must assume that the blood supply to the commonly described anterior and posteriorly pedicled flaps has been compromised.

Objective: The objective of the study is to describe a novel flap technique for revision eDCR.

Methods: The superior based mucosal flap is a novel technique that provides a vascularized mucosa preserving technique in revision eDCR despite previous instrumentation of the lacrimal system. This technique provides wide exposure of the revision osteotomy site while simultaneously allowing a viable mucosal flap to be replaced at the conclusion of the procedure, thereby minimizing bone exposure and cicatricial restenosis.

Results: The authors have utilized this technique in 13 procedures with 100% positive identification of the lacrimal sac, a 0% complication rate, and a 100% success rate after a mean follow-up of 26.93 ± 10.33 months (range 6–35 months).

Conclusion: The eDCR using the superior pedicled mucosal flap provides excellent exposure of the maxillary bone and the lacrimal sac. This method preserves vascularity of the flap using a superiorly based pedicle which is typically inviolate during both open and endoscopic primary DCR. The mucosal flap can then be replaced, thereby minimizing bone exposure and optimizing patency.     

Phenotypic high myopia in X-linked retinitis pigmentosa secondary to a novel mutation in the RPGR gene

Background: Retinitis pigmentosa (RP) is a clinically and genetically heterogeneous disease causing progressive degeneration of retinal photoreceptor cells. The most severe form of this disease is X-linked RP (XLRP), in which photoreceptor degeneration begins in early childhood and complete blindness often occurs by the fourth decade of life. Two genes commonly associated with XLRP have been previously identified.

Material and methods: One Spanish family with confirmed XLRP was studied for mutations using direct sequencing. A genotype-phenotype correlation with pathologic myopia (PM) is detailed.

Results: A new pathogenic mutation in the third exon of the RP GTPase regulator (RPGR) was identified: a variant c212C>G (pSER71*). This mutation appears as a hemizygous variant in the male proband with RP, and as heterozygous variant in the females of this pedigree who invariably exhibit symmetrical PM in both eyes.

Conclusion: A complete family history allowed determination of the inheritance pattern providing genetic counseling for patients and their families. The geno-phenotypic attributes of this heterozygosity suggest a correlation between RP and PM. This novel mutation would expand the mutation spectrum of RP2 and RPGR, and help to study molecular pathogenesis of RP.