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AimsTo identify risk factors for rebubbling, and early graft failure after Descemet membrane endothelial keratoplasty (DMEK).MethodsIn this prospective registry study, all consecutive DMEK procedures registered in the Netherlands Organ Transplant Registry were assessed (n=752). Univariable and multivariable analysis was performed using logistic regression. The effect of rebubbling on endothelial cell density was analysed using a linear mixed model.Results144 of 752 (19%) eyes underwent rebubbling. Rebubbling was successful in 101 eyes (70%). In eyes that underwent rebubbling, the graft failure rate was significantly higher than eyes that did not undergo rebubbling (30% vs 9%, respectively; OR: 4.28, 95% CI 2.72 to 6.73, pConclusionsThis Dutch registry study identified independent risk factors for DMEK graft detachment leading to rebubbling, namely recipient age and surgical complication, and early graft failure, namely transplantation before 2016 and surgical complication. Rebubbling was associated with significantly higher endothelial cell loss in the first year after surgery.

We propose a simple and effective method to inject word-level information into character-aware neural language models. Unlike previous approaches which usually inject word-level information at the input of a long short-term memory (LSTM) network, we inject it into the softmax function. The resultant model can be seen as a combination of character-aware language model and simple word-level language model. Our injection method can also be used together with previous methods. Through the experiments on 14 typologically diverse languages, we empirically show that our injection method, when used together with the previous methods, works better than the previous methods, including a gating mechanism, averaging, and concatenation of word vectors. We also provide a comprehensive comparison of these injection methods.

Pure midbrain infarctions not involving surrounding structures are an uncommon clinical phenomenon. A midbrain infarction that results in isolated bilateral ptosis as the only neurological deficit is much rarer and an easy diagnosis to miss; therefore, potentially leading to further downstream complications. We describe the case of an elderly patient who presented with isolated bilateral ptosis, initially thought to be consequent to myasthenia gravis but subsequently identified to have a perforator infarct in the midbrain, resulting in his symptoms.

PURPOSE: To describe the incidence, prevalence, and clinical characteristics of presumed ocular histoplasmosis syndrome (POHS) in a Histoplasma endemic region. METHODS: The International Classification of Diseases, 9(th) and 10(th) Revision codes were used to search the Rochester Epidemiology Project, a record-linkage system for medical care provided in Olmsted County, MN. Medical records were reviewed to confirm POHS diagnoses in county residents from January 1, 2006 to December 31, 2015. Age- and sex-adjusted incidence rates were calculated and adjusted to the 2010 U.S. White population. RESULTS: There were 18 incident cases (30 eyes) and 87 prevalent cases (131 eyes). The incidence rate was 1.35 per 100,000 per year. The mid-study prevalence rate was 0.064%. Choroidal neovascularization (CNV) occurred in 17.4% of affected eyes. At last follow-up, 16.8% of affected eyes had POHS-related decreased VA (

Background/aimsFuchs’ uveitis syndrome (FUS) is one of the frequently misdiagnosed uveitis entities, which is partly due to the absence of internationally recognised diagnostic criteria. This study was performed to develop and evaluate a set of revised diagnostic criteria for FUS.MethodsThe clinical data of Chinese patients with FUS and patients with non-FUS were collected and analysed from a tertiary referral centre between April 2008 and December 2020. A total of 593 patients with FUS and 625 patients with non-FUS from northern China were enrolled for the development of diagnostic criteria for FUS. Three hundred and seventy-seven patients with FUS and 503 patients with non-FUS from southern China were used to validate the criteria. Clinical symptoms and ocular signs were collected from all patients with FUS and patients with non-FUS. Multivariate two-step cluster analysis, logistic regression and decision tree algorithms in combination with the clinical judgement of uveitis experts were used to revise diagnostic criteria for FUS.ResultsThree essential findings including diffuse iris depigmentation, absence of posterior synechiae, mild inflammation in the anterior chamber at presentation and five associated findings including mostly unilateral involvement, cataract, vitreous opacities, absence of acute symptoms and characteristic iris nodules were used in the development of FUS diagnostic criteria. All essential findings were required for the diagnosis of FUS, and the diagnosis was further strengthened by the presence of associated findings.ConclusionRevised diagnostic criteria for FUS were developed and validated by analysing data from Chinese patients and showed a high sensitivity (96.55%) and specificity (97.42%).

Erythema nodosum (EN) is a poly-etiological disease with an acute flow that is characterized by symmetric emergence of painful nodules often in pretibial areas. A twenty-month-old male child was admitted to hospital for evaluation of the eruptive skin changes in the lower extremities and forearms. The disease began 10 days before getting febricity and loose stools. The laboratory analysis showed an elevated erythrocyte sedimentation rate and leukocytosis. Blood cultures demonstrated the presence of coagulase-negative Staphylococcus, while Proteus vulgaris was isolated in urine cultures. After initiation of the empiric antibiotic therapy and then, the targeted antibiotic therapy according to the antibiogram, there was a significant improvement in a general condition and regression of cutaneous lesions. Erythema nodosum in the present case, is the result of staphylococcal bacteremia although Proteus vulgaris cannot be excluded as a cause.

To characterize the progression of structural and functional changes in the retinas of a small cohort of unrelated patients with early late-onset retinal degeneration and evaluate these changes as potential biomarkers for future treatment trials.Best-corrected visual acuity, contrast sensitivity, Goldman visual fields, retinal sensitivity measurement by mesopic microperimetry, extent of ellipsoid zone disruption using spectral domain optical coherence tomography, and fundus autofluorescence imaging were performed at each biennial visit.Three unrelated patients with molecularly confirmed late-onset retinal degeneration (S163R mutation in C1QTNF5 ) were prospectively followed for 4 years.The patient's ages were 44, 54, and 62 at baseline. Over the 4-year follow-up period, one patient demonstrated a significant reduction in best-corrected visual acuity (6 Early Treatment of Diabetic Retinopathy Study letters), whereas two patients suffered a significant reduction in contrast sensitivity. Early in the disease, there was a close relationship between ellipsoid zone disruption and a loss in retinal sensitivity. Later in the course of the disease, there were areas outside the zones of ellipsoid zone disruption that also suffered progressive loss of retinal sensitivity, suggesting that ellipsoid zone loss was not the only factor responsible for the loss of retinal sensitivity. Changes in fundus autofluorescence and Goldman visual field loss were not closely related to changes in ellipsoid zone disruption or retinal sensitivity loss.This study has found that the monitoring of the progression of ellipsoid zone disruption and changes in mesopic microperimetry may be useful biomarkers in future clinical trials in patients with late-onset retinal degeneration.

Introduction Endonasal and external dacryocystorhinostomy procedures have both been used for the treatment of post-saccular obstruction of the lacrimal system. Functional success of these surgeries depends on several factors. Objective To evaluate the status of the rhinostomy ostium with endonasal and external approaches in dacryocystorhinostomy operations and to determine the effect of ostium size on postoperative functional success. Methods The charts of the patients operated in our hospital between May 2017 and January 2019 were analyzed retrospectively (ethical approval number: 2018-12.04). The patients that were operated in the ophthalmology and otolaryngology departments were included in the study. Endoscopic rhinostomy ostium measurements, punctum lavage findings and complications were recorded at 8 weeks postoperative at the earliest. Results When the 64 patient charts were reviewed (76 operations), the mean ostium width was 1.85 ± 1.11 mm in the endonasal approach group and 3.60 ± 2.24 mm in the external approach group. The mean ostium areas in endonasal and external group were 14.61 ± 16.66 mm2 and 56.05 ± 60.41 mm2, respectively. The ostium was anatomically patent and punctum lavages were negative in 11 patients (6 patients in the endonasal approach group and 5 patients in the external approach group) and these cases were considered as functional failures. The rhinostomy ostium was significantly wider in the external approach group, but this was considered ineffective on functional outcomes. Conclusion Lacrimal duct stenosis can be successfully treated with endonasal and external methods. Tear drainage may be insufficient even in the presence of a patent ostium. Therefore, functional success should also be considered when evaluating the overall success of dacryocystorhinostomy. An anatomically patent ostium is definitely required, while it is believed that ostium size does not affect functional surgical success. Resumo Introdução Dacriocistorrinostomia endonasal e externa têm sido usados para o tratamento de obstrução pós‐sacular do sistema lacrimal. O sucesso funcional dessas cirurgias depende de vários fatores. Objetivo Avaliar o status do óstio da rinostomia nas abordagens endonasal e externa em dacriocistorrinostomias e a importância do tamanho do óstio no sucesso funcional pós‐operatório. Método Os prontuários de pacientes operados em nosso hospital entre maio de 2017 e janeiro de 2019 foram analisados retrospectivamente (número de aprovação ética: 2018-12,04). Foram incluídos no estudo os pacientes operados nos setores de oftalmologia e otorrinolaringologia. Medidas do óstio da rinostomia endoscópica, achados à irrigação do ponto lacrimal e complicações foram registrados a partir da 8ª semana de pós‐operatório. Resultados Foram avaliados prontuários de 64 pacientes (76 operações); a largura média do óstio era de 1,85 ± 1,11 mm no grupo endonasal e de 3,60 ± 2,24 mm no grupo externa. As áreas médias do óstio no grupo endonasal e externa foram 14,61 ± 16,66 mm2 e 56,05 ± 60,41 mm2, respectivamente. O óstio estava anatomicamente pérvio e as irrigações do ponto lacrimal foram negativas em 11 pacientes (6 pacientes no grupo endonasal e 5 pacientes no grupo externa) e esses casos foram considerados como falhas funcionais. O óstio da rinostomia foi significativamente maior no grupo externo, mas sem relação com a eficácia nos resultados funcionais. Conclusão A estenose do ducto lacrimal pode ser tratada com sucesso com métodos endonasais e externos. A drenagem lacrimal pode ser insuficiente mesmo na presença de óstio pérvio. Portanto, o sucesso funcional também deve ser considerado ao avaliar o sucesso do procedimento. Embora um óstio anatomicamente patente seja necessário, o tamanho do óstio não afeta o sucesso cirúrgico funcional.

Irreversible damage to the structure of axons and death of the retinal ganglion cell (RGC) soma in primary open-angle glaucoma (POAG) and Alzheimer’s disease (AD) develop against the background of the already existing clinical manifestation, which is preceded by a slow period of progressive loss of synapses and dendrites of the RGCs. Recent studies have shown that the integrity of the RGC’s dendritic branching can serve as both a target of neuroprotective therapy and a sensitive marker of retinal degeneration in AD and glaucoma. To develop methods of complex neuroprotective therapy, it is necessary to substantiate the targets and tactics of affecting the dendritic tree of the RGCs, the remodeling of which, according to modern concepts, can be closely and antagonistically related to the regeneration of the axon after its damage in trauma and neurodegenerative diseases. RGCs are highly capable of functional modification. Currently, it has been proven that the use of neuroprotective drugs and neurotrophins is promising for maintaining the adaptive plasticity of RGCs and restoring their synaptic contacts at the level of the retina and brain. Understanding the features of the adaptive plasticity of RGCs in AD and glaucoma will make it possible to use technologies to activate the internal potential of neuronal remodeling, including the modification of dendritic branching of RGCs and regeneration of their axons, in the preclinical stages of these diseases. Increasing knowledge about the sequence and mechanisms of early events in the retina’s inner plexiform layer will contribute to the development of targeted neuroprotective therapy and new technologies to detect early POAG, AD, and, possibly, other systemic and local neurodegenerative conditions.

Objective To correlate the histopathologic results of conjunctival specimens with clinical findings in patients with conjunctivochalasis and report the results of the paste-pinch-cut technique for management. Design Retrospective chart review. Methods SETTING: Single tertiary ophthalmological centre (Ocular Surface Diseases and Dry Eye Clinic, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Md.). Methods Twenty-five patients (32 eyes) with clinically significant conjunctivochalasis. All patients were referred for clinically significant dry eye without previous diagnosis of chalasis. Sixteen patients had an underlying inflammatory systemic condition. Intervention or Observation Procedure(s): Patients underwent surgery with paste-pinch-cut technique. Subjective dry eye symptoms and ocular surface staining scores (corneal and conjunctival staining using fluorescein and lissamine green respectively) were assessed at every visit. Main outcome measures Change in patient symptoms and ocular surface staining scores and histopathologic findings in conjunctival specimens. Results After surgery, significant improvement was achieved in dry-eye symptoms as well as both corneal and conjunctival staining scores in 29 eyes on reduced topical therapy. Only 3 eyes had persisting conjunctival lissamine staining. Light microscopic examination disclosed mild to moderate lymphoplasmocytic inflammation of the conjunctivae with areas of epithelial goblet cell loss, squamous metaplasia, stromal edema, and fibrosis. Conclusion Conjunctivochalasis appears to be associated with significant inflammation in the setting of dry eye and underlying inflammatory systemic conditions. Although topical anti-inflammatory treatment could be attempted in the initial management, surgical excision should be considered in the absence of clinical response.

OBJECTIVE In 2004, government-funded routine eye exams were discontinued for individuals aged 20-64 years in Ontario. We assessed whether this policy change reduced the rate of new glaucoma diagnoses. DESIGN Cohort-based time-series analysis. PARTICIPANTS Ontarians aged 20+ years in 2000-2014. METHODS Province-wide physician billing data were analyzed using segmented regression analysis. New glaucoma diagnoses were defined as the first glaucoma diagnostic billing code submitted by an ophthalmologist or optometrist among Ontarians who did not visit an ophthalmologist or an optometrist in the year prior to the study year. RESULTS Post- versus pre-2004, the rate of new glaucoma billings was reduced in all age groups: -2.7‰ (p < 0.0001) in the age group 20-39 years, -8.2‰ (p < 0.0001) in the age group 40-64 years (p < 0.0001), and -2.1‰ (p = 0.0003) in the age group 65+ years. This corresponds to a decreased number of individuals with a new glaucoma billing after 2004 versus before 2004: -8,800 (p < 0.001) in the age group 20-39 years, -32,234 (p < 0.0001) in the age group 40-64 years, and -3,255 (p = 0.0012) in the age group 65+ years. Reduced rates of new glaucoma diagnostic billings were seen in males, females and rural and urban residents among policy-affected and policy-unaffected age groups. Ontarians living in the wealthiest neighbourhood areas also had a significantly reduced rate after versus before 2004: -2.7‰ (p < 0.0001) for the age group 20-39 years, -9.0‰ (p < 0.0001) for the age group 40-64 years, and -2.3‰ (p < 0.001) for the age group 65+ years. CONCLUSIONS Discontinuation of government-funding for routine eye exams was associated with a significantly reduced rate of new glaucoma diagnostic billings irrespective of sociodemographics. More research is needed to understand the reduced glaucoma billings in unaffected seniors and those living in the wealthiest neighbourhood areas.

We present a case of a 53-year-old female with an 11 year history of myasthenia gravis (MG) with palpebral ptosis in the left eye which had become more marked over the previous year. Examination revealed a painless left orbital mass causing ptosis. The tumor was surgically removed and histopathology revealed deposits of a hyaline substance which when stained with Congo Red had an apple-green birefringence with polarized light, typical of amyloid. In the absence of amyloid deposits elsewhere in the body, amyloid tumor (AT) was diagnosed. No myelo- or lymphoproliferative syndromes, systemic involvement by amyloidosis or any autoimmune disease were found. The evolution and aesthetic results where satisfactory. Only two cases of orbital AT associated with MG have been described previously; however, in one of the cases, the symptoms of the AT had led to a false diagnosis of MG.

Purpose The authors describe a case of new bilateral epiretinal membranes and vitreous cells following intravitreal and subTenon's injections of bone marrow-derived stem cells. Methods Case report of a 43-year-old man with OPA-1 autosomal dominant optic atrophy who self-enrolled in a stem cell therapy clinical trial and received simultaneous bilateral intravitreal and subTenon's injections of bone marrow-derived stem cells. Results Within one month of receiving the injections, the patient developed epiretinal membranes and vitreous cells in both eyes, seen with optical coherence tomography. Conclusion Stem cell therapy has been gaining popularity as a potential intervention for progressive retinal and optic nerve diseases; however, the mechanism of action of bone marrow-derived stem cells is still not well understood and may result in unintended cell differentiation.

To present immunocytochemical characterization of a surgically excised central posterior lens capsule (PLC) that was transplanted to close a secondary refractory full-thickness macular hole (FTMH) as an epiretinal flap. For comparison, tissue of both an unaffected internal limiting membrane and unexposed PLC was processed.Clinical-pathological case report.We report of a 38-year-old patient who underwent pars plana vitrectomy (PPV) with PLC tissue for patching secondary FTMH and silicone oil tamponade after tractional retinal detachment. The PLC was peeled off during a vitrectomy 1 year after positioning. For immunocytochemistry, the removed PLC was prepared as flat mount and showed a positive immunofluorescence of the Müller cells marker glutamine synthetase and for vimentin. The microglia marker IBA and the neuroprotective neurotrophic marker glia cell-derived neurotrophic factor were tested positive too. There was no immunoreactivity of cellular retinaldehyde-binding protein and glial fibrillary acidic protein. In comparison, tissue of a control internal limiting membrane that was obtained during standard FTMH surgery showed few single cells that were likewise positive for glutamine synthetase, glia cell-derived neurotrophic factor, and IBA. The control specimen of unexposed PLC showed rarely cells that were without positive immunostaining for the tested markers.Our analysis revealed positive immunoreactivity of macroglia and microglia cells of the PLC tissue that was used to patch a refractory FTMH. Similar immunostaining of PLC material and internal limiting membrane suggests the PLC transplantation as an alternative treatment option for refractory FTMH.

Magnetic resonance imaging (MRI) is increasingly used by the orbital surgeon to aid in the diagnosis, surgical planning, and monitoring of orbital disease. MRI provides superior soft tissue detail compared with computed tomography or ultrasound, and advancing techniques enhance its ability to highlight abnormal orbital pathology. Diffusion-weighted imaging is a specialized technique that uses water molecule diffusion patterns in tissue to generate contrast signals and can help distinguish malignant from benign lesions. Steady-state free precession sequences such as Constructive Interference in Steady-State (CISS) and Fast Imaging Employing Steady-state Acquisition (FIESTA) generate highly detailed, 3-dimensional reconstructed images and are particularly useful in distinguishing structures adjacent to cerebral spinal fluid. Magnetic resonance angiography can be used to characterize vascular lesions within the orbit. New developments in magnetic field strength as well as the use of orbital surface coils achieve increasingly improved imaging resolution.

Objective To evaluate the safety of performing cataract surgery prior to eyelid (entropion/ectropion) surgery in patients with concurrent cataract and lower eyelid malposition. Design Retrospective case series. Participants and Methods Patients with concurrent lower eyelid malposition and cataract undergoing cataract surgery before lower eyelid repair from 2013 to 2020 were identified from two ophthalmologists (M.L.W., G.R.). Both surgeries were performed by the same ophthalmologist, with eyelid repair completed at least 1 month following cataract extraction. Data analysis was performed with two-parameter estimations. The primary outcome was the postoperative endophthalmitis rate in this cohort. Results 129 cases in 90 patients were found (86 involutional entropion and 43 involutional ectropion). No cases of endophthalmitis were encountered. Statistical analysis using the 95% Jeffreys interval for one-sample binomial proportion revealed an upper limit of 1.9%. The Agresti-Caffo interval of the proportional difference between the study procedure and historical incidence data of postoperative endophthalmitis following cataract surgery alone yielded an estimate of 0.8% with an upper confidence limit of 2.2%. Conclusion We present preliminary evidence on the endophthalmitis risk in patients with concurrent lower eyelid malposition and cataract who undergo cataract surgery prior to eyelid repair. We propose that this strategy may be a viable option to expedite vision restoration and reduce the risk of recurrent lower eyelid malposition in select patients. More data are required to reach statistically significant noninferiority and show that a malpositioned lower eyelid is not a risk factor for postoperative endophthalmitis following cataract surgery.

To compare primary selective laser trabeculoplasty (SLT) response in uveitic, steroid-induced, primary open-angle glaucoma (POAG) and pseudoexfoliative glaucoma (PEX).Single-centre retrospective case-control study.Patients with uveitic glaucoma, steroid-induced glaucoma, POAG, or PEX who had their first SLT.Eyes with POAG or PEX were in control groups. Eyes with steroid-induced or uveitic glaucoma were in experimental groups. Change in intraocular pressure from baseline, treatment failure, complication rates, and medication use were compared using rank-sum and log-rank tests.Six-hundred and eight eyes of 433 patients were enrolled. Steroid-induced glaucoma eyes had higher mean baseline pressure and a decrease in pressure at 3-8 weeks (27.6-17.4 mm Hg) than those with PEX (21.7-16.5 mm Hg; p0.001) or POAG (18.6-14.9 mm Hg; p ≤ 0.025). Failure rates after 2 years were lower in steroid-induced glaucoma (54%) than in PEX (84%; p = 0.01) or POAG (84%; p = 0.005). This survival benefit persisted when excluding patients with changes to their steroid dosing (p ≤ 0.03) but showed mixed results when compared with patients with a baseline pressure of 25mm Hg or greater (p = 0.020 vs PEX; p = 0.67 vs POAG). At 18 months, the steroid-induced group decreased ocular hypotensive medication use (3.5-1.9; p = 0.005); the uveitic group increased medication use (2.7-3.5; p = 0.02).SLT is an effective treatment for steroid-induced glaucoma, with greater response and a lower failure rate than in PEX and primary POAG, although high baseline intraocular pressure may be a confounder. Judicious use of SLT can be considered in uveitic glaucoma.

An association between race and formation of chalazion has yet to be objectively established. This study investigates race as a risk factor for chalazion and chalazion surgery. Understanding racial risk factors in formation of chalazion, recurrent chalazion, and chalazion requiring surgery (often with general anesthesia in children) informs decisions regarding eyelid hygiene, early topical medical therapy, and aggressiveness with oral antibiotic therapy for coexisting conditions such as blepharitis.Demographic data was collected for all pediatric visits to the University of Wisconsin-Madison ophthalmology department from 2012-2019. Retrospective chart review was performed for the subset with chalazion.Of 28 433 minors, 584 had 1088 chalazia, a 2% overall rate. Chalazion was seen in 1.8% of non-Hispanic/Latino participants and 3.8% of Hispanic/Latino participants (p value0.0001). Chalazion was seen in 1.7% of white participants, compared to 4.3% of American Indian or Alaska Native participants (p value0.0001) and 4.0% of Asian participants (p value0.0001). More than one chalazion was recorded in 31% of subjects without coexisting meibomian gland disease, blepharitis, or marginal keratitis, and in 56% (p 0.0001) with one of these conditions. Repeated diagnoses of chalazion on separate encounters were seen in 17% without these conditions and in 33% (p 0.0001) with one of these conditions.Hispanic/Latino, American Indian, and Asian participants developed chalazion at a rate higher than other racial/ethnic groups, whereas patients with meibomian gland disease or blepharitis are especially at risk for developing multiple chalazia on separate encounters. No group was more likely to require surgical intervention than any other.

To report a case of nonleaking cystoid macular edema (CME) associated with pentosan polysulfate sodium (PPS)-induced pigmentary maculopathy.Multimodal imaging, including optical coherence tomography, fundus photography, autofluorescence, and fluorescein angiography, was used to substantiate our diagnosis, further characterize the cystoid macular edema showed by our patient and to monitor the response to treatment.A 59-year-old woman was referred for decreased visual acuity and bilateral macular edema. She had been treated for interstitial cystitis with PPS for 10 years. Multimodal imaging showed the characteristic features of PPS-induced pigmentary maculopathy. Moreover, fluorescein angiogram showed nonleaking cystoid macular edema in both eyes. She was treated successfully with intravitreal injections of bevacizumab.To our knowledge, this report is the first to demonstrate that PPS-associated cystoid macular edema can be nonleaking on fluorescein angiography and responds well to intravitreal anti-vascular endothelial growth factor injections.

Purpose To describe a novel clinical and imaging finding in patients with tubercular posterior uveitis. Methods A retrospective review of 3 cases presented at a tertiary referral eye centre in North India between June 2016 to March 2019 was performed. All the patients had received an initial diagnosis of non-infective etiologies (sympathetic ophthalmia, necrotizing scleritis and lymphoma). Fundus photography, fluorescein angiography (FA), fundus autofluorescence (FAF), and enhanced-depth imaging optical coherence tomography (EDI-OCT) were reviewed. Results Three patients (all Asian Indian females: aged 18, 49 and 52 years) diagnosed with panuveitis were investigated for various etiologies based on the initial clinical suspicion. During the course of therapy, all the patients developed peripheral yellow sub-retinal pigment epithelim (RPE) deposits (YSRPE) which appeared hypo-autofluorescent on FAF, and initially hypofluorescent with late hyperfluorescence on FA. The patients were subjected to detailed systemic evaluation and laboratory tests. All the patients showed acid fast bacilli on invasive tissue biopsies. After initiation of anti-tubercular therapy, the lesions resolved in all eyes. Conclusions YSRPE deposits represent a novel and important diagnostic sign of tubercular posterior uveitis.

A 62-year-old woman presented with non-specific abdominal pain, elevated alkaline phosphatase levels and bilateral sequential visual loss. Visual acuity was counting fingers in right eye (RE) and 20/400 in left eye (LE). She was noted to have optic nerve pallor in RE and mildly elevated optic disc with signs of ocular inflammation in LE. After 2 weeks, vision deteriorated to light perception bilaterally with now extensive vitreal inflammation present in both eyes. Positive rapid plasma reagen and Treponema pallidum’s antibody tites confirmed syphilis infection. Unfortunately, as the diagnosis was delayed by many months, her visual acuity remained poor (hand motions in RE and 20/50 in LE) despite treatment with intravenous penicillin. This case reminds us of the re-emergence of this ‘great masquerader’ and highlights the importance of maintaining high suspicion for syphilis in patients with unexplained visual loss and systemic symptoms, even in older adults without identifiable risk factors.

Intraocular tuberculosis has protean clinical manifestations and remains an important etiological differential for uveitis in an endemic region. A 27-year-old male presented with visual acuity of counting fingers close to face in right (OD) and 20/25 in left eye (OS). Examination revealed a choroidal granuloma in OS and healed serpiginous-like choroiditis in OD. Antitubercular therapy was started with systemic corticosteroids. Granuloma resolved completely; however, the patient presented with neuroretinitis and posterior scleritis, as first and second recurrence, respectively, within a oneyear period. These were managed with systemic corticosteroids and immunosuppressive therapy was added, after second recurrence. The patient responded well and maintains remission. This case presented a clinical challenge with distinct recurrence patterns of tubercular posterior uveitis in the same eye, which has not been reported before. Successful management entailed use of antitubercular therapy, corticosteroids, and immunosuppressive therapy in a step-ladder approach, resulting in preservation of vision and achieving long-term remission.

Postoperative cystoid macular oedema (CMO) is a recognised complication of cataract surgery, occurring in around 1.5% of cases. It is generally managed with topical steroids or non-steroidal anti-inflammatory medications. We present a case of a patient who developed bilateral sequential CMO following bilateral sequential cataract surgery which was non-responsive to topical therapy and worsened following sub-Tenons administration of steroid. The patient took fingolimod for multiple sclerosis both prior to and during the period of cataract surgery which is known to result in the development of macular oedema in some patients. On fingolimod cessation, the oedema resolved over a period of 5 months with good visual recovery. We present this case to inform cataract surgeons of the risk of fingolimod-associated macular oedema in patients undergoing cataract surgery and to inform neurologists of the potential need to adjust treatment for patients undergoing cataract surgery.

This case report demostrates an unusual occurence of orbital metastasis along with liver metastasis 5 years following enucleation for ciliochoroidal melanoma with no extraocular extension. It exemplifies that metastasis of the tumour can occur in the ipsilateral orbit after enucleation. Importance of close clinical follow-up after enucleation for choroidal melanoma, careful socket examination, and regular systemic metastatic workup is demonstrated. To the best of our knowledge, this is the first case report of uveal melanoma with ipsilateral orbital metastasis.

A 35-year-old woman presented with a constellation of systemic symptoms: rashes, weight loss, arthralgia and mouth ulcers. Six months afterwards, she experienced bilateral and sequential reduction in vision, and was found to have bilateral vaso-occlusive retinopathy, with critical macular ischaemia in the left eye. Her serological markers were consistent with a diagnosis of lupus. A lymph node biopsy confirmed Kikuchi-Fujimoto disease, a benign condition of unknown cause characterised by fever, cervical and axillary lymphadenopathy. Given that this overlap syndrome was associated with a number of systemic features and had affected the eyes, an immunosuppressive regime with rituximab was considered prudent. This rendered her vasculitis stable and non-progressive, and there were signs of partial retinal microvasculature recovery on optical coherence tomography angiography. There is increasing evidence of an overlap between Kikuchi-Fujimoto disease and systemic lupus erythematosus, which is associated with vaso-occlusive retinopathy. In these instances, a multidisciplinary approach is warranted, with consideration of appropriate treatment in order to prevent harmful sequelae of vasculitis. Our treatment with rituximab abated the disease process, although close follow-up is paramount to monitor results and side-effects of treatment.

A 36-year-old man presented with proptosis and external ophthalmoplegia of the left globe following road traffic injury. Cerebral angiogram revealed moderate flow direct carotid cavernous fistula on left side for which coil embolisation was done repeatedly. Subsequently, the patient developed decreased vision in left eye and developed features of left-sided ocular ischaemic syndrome. The patient was treated conservatively with spontaneous reversal of ocular ischaemic syndrome and complete regain of visual function.

Idiopathic intracranial hypertension (IIH) is being diagnosed more often in the UK due to the rise in obesity. In fact, patients who present with bilateral optic disc swelling are habitually put on the papilloedema pathway, often without consideration of other diagnoses. We report the case of a middle-aged woman diagnosed with papilloedema and managed as IIH, until, cerebrospinal fluid (CSF) analysis revealed evidence of lymphocytic meningitis secondary to syphilis. The patient was treated successfully with intravenous antibiotics. Syphilis is the great masquerader and should be a diagnosis to consider in patients who have CSF findings incongruent with their clinical presentation.

PURPOSE: To describe the utility of fundus autofluorescence (FAF) for detection and monitoring of acute regional outer retinopathy in punctate inner choroidopathy (PIC). DESIGN: Multimodal imaging case series METHODS: Patients with PIC and acute regional hyperautofluorescence were identified. Multimodal imaging, treatment, and clinical course were reviewed. RESULTS: Six eyes of five patients were included (5 women, mean age 39 years, range 28–69). All endorsed new symptoms. Short-wave blue FAF revealed regional hyperautofluorescence in the symptomatic eye, corresponding to ellipsoid zone (EZ) loss on optical coherence tomography (OCT). All patients were treated with local or systemic corticosteroids or immunosuppression, with resolution of symptoms and acute imaging abnormalities. CONCLUSIONS: Symptomatic acute regional outer retinopathy in PIC, manifesting as hyperautofluorescence and EZ loss, is easily identifiable using widefield FAF imaging. Treatment resulted in resolution of symptoms and imaging findings in our cohort, and should be considered in this patient population.

(a). PURPOSE. A central neuropeptide mediator of ocular immune privilege is α-MSH, which can be used to therapeutically suppress experimental autoimmune uveitis (EAU). A part of α-MSH-regulation of immune activity is through its melanocortin 5 receptor (MC5r). One of the mechanisms of ocular immune privilege mediated by α-MSH is RPE suppression of phagolysosome activation associated with antigen presenting cell (APC) processing of antigen. Therefore, we examined the possible role of MC5r-expression in the recovery of RPE suppression of macrophage phagolysosome activation following α-MSH-treatment of EAU. (b). METHODS. The conditioned media of cultured in situ RPE-eyecup from α-MSH-treated EAU wild-type and MC5r((−/−)) mice were used to treat macrophages phagocytizing opsonized-pHrodoRed-bacterial bioparticles to assay for phagolysosome activation. In addition, the phagocytic activity of macrophages from MC5r((−/−)) mice was assayed. (c). RESULTS. The RPE from MC5r((−/−)) mice that have recovered from EAU after α-MSH-therapy do suppress phagosome maturation in wildtype macrophages; but do not suppress phagosome maturation in MC5r((−/−)) macrophages. In addition, α-MSH does not suppress phagolysosome activation in MC5r((−/−)) macrophages, and the macrophages are highly enhanced in their phagocytic activity. Along with the enhanced macrophage activity was observed an increase in damage of the EAU MC5r((−/−)) retinas. (d). CONCLUSION. The results demonstrated that treatment of EAU with α-MSH mediated recovery of RPE suppression of phagolysosome activation in macrophages and protected the retina from inflammatory damage. This was dependent on the expression of MC5r. Moreover, through MC5r the neuropeptide α-MSH potentially acts as a homeostatic moderator of phagosome-maturation within macrophages.

Antisynthetase syndrome (anti-SS) is a rare systemic autoimmune disease characterised by autoantibodies against aminoacyl-tRNA synthetases manifesting as one or more components of the classic triad: interstitial lung disease, arthritis and myositis. While it is well-recognised that autoimmune rheumatological disorders in general can contribute to multiple pregnancy complications, very little is known about how anti-SS itself affects pregnancy outcomes. Described here is the case of a 26-year-old pregnant woman with anti-SS whose pregnancy course was complicated by placental dysfunction and subsequent extremely premature delivery at 24 weeks’ gestation. This report presents a review of the literature to date and discusses potential pregnancy complications associated with anti-SS and their subsequent targeted management.

Cytomegalovirus (CMV) can cause recalcitrant recurrent keratouveitis and secondary glaucoma. We report a case of chronic recurrent anterior uveitis with secondary glaucoma presenting with acute visual loss and interface fluid 9 years after laser in situ keratomileusis. Based on clinical presentation, a viral aetiology was suspected. Aqueous tap was positive for CMV-DNA by real-time quantitative PCR of the aqueous humour. The patient was treated with systemic antivirals, topical corticosteroids and antiglaucoma medications. The interface fluid resorbed rapidly. The intraocular pressure (IOP) was controlled by trabeculectomy. There was no further corneal deterioration at 7-month follow-up and the IOP had also stabilised. We believe this is only the third reported case of CMV-related interface fluid syndrome. This case highlights the role of quantitative PCR analysis for establishing viral aetiology in recurrent unilateral hypertensive anterior uveitis and reports the unusual finding of interface fluid which resolved after starting systemic antiviral therapy.

A 47-year-old man presented with profound loss of vision in right eye and relative afferent pupillary defect. On fundus examination, posterior pole details were obscured due to dense vitreous haemorrhage. B-scan ultrasonography was performed that revealed a mushroom-shaped hyperechoic lesion with medium internal reflectivity on A-scan ultrasonography. After performing contrast-enhanced MRI of the orbit, a diagnosis of choroidal melanoma was established. Patient was managed using plaque brachytherapy based on multiplanar MRI. This was followed 10 months later by pars plana vitrectomy and cataract extraction. Vision postoperatively improved to 20/60. A systematic clinical assessment along with supportive ancillary investigations augments diagnostic accuracy and reduces delay in definitive management.

PURPOSE We describe a case of endophthalmitis caused by streptococcus salivarius presenting as a retinal vasculitis less than twenty-four hours after intravitreal injection. METHODS Case report RESULTS:: The patient progressed from a hemorrhagic retinal vasculitis to severe endophthalmitis with no view to the posterior segment within 24 hours. Visual acuity progressed from 20/20 pre-injection to light perception within less than 48 after injection. Vitreous tap and injection was performed within 24 hours of intravitreal aflibercept injection, followed by pars plana vitrectomy the next day. Final visual acuity was hand motions. CONCLUSIONS Streptococcus salivarius is a virulent organism that may cause an endophthalmitis early after intravitreal injection with an unusual presentation of hemorrhagic retinal vasculitis. A high index of suspicion for infectious endophthalmitis should be maintained for all patients presenting with ocular inflammation and worsening vision in the days following intravitreal injections.

To describe a patient with a choroidal osteoma treated with photodynamic therapy to prevent tumor growth in whom choroidal neovascularization (CNV) developed after being treated with photodynamic therapy.Case report.A 5-year-old Hispanic woman presented with an asymptomatic choroidal osteoma, temporal to the macula of her right eye. According to the patient's mother, her medical, surgical, and family history was unremarkable. At examination, best-corrected visual acuity was 20/30 in both eyes. After 11 months of follow-up, signs of tumor growth toward the fovea without any signs of CNV was noted. Photodynamic therapy was performed to prevent invasion of the foveola. Two months thereafter, the patient developed CNV in the macula region in the right eye, decreasing visual acuity to 20/200. The patient was treated with four total intravitreal injections of 1.25 mg of bevacizumab over 24 weeks, which resulted in inactivation of the CNV and improved visual acuity to 20/20. Choroidal neovascularization had been never reported in her past history and her follow-up visits over 7 years. In addition, no evidence of recurrent neovascular activity or tumor growth was reported.Choroidal osteoma is a benign tumor that can result in vision-threatening complications, caused by tumor growth and tumor decalcification. Photodynamic therapy is an effective modality in inducing choroidal osteoma decalcification and stabilization; however, CNV due to reperfusion following photodynamic therapy can be seen in the retina.

To report a sporadic case of a familial adenomatous polyposis (FAP) discovered in a patient with bilateral retinal pigment epithelial lesions.Case report.A 30-year-old Asian woman presented for evaluation of bilateral pigmented lesions at the level of the retinal pigment epithelium. She had no personal or family history of colonic polyps or colon cancer. Colonoscopy revealed innumerable adenomatous polyps and genetic testing revealed a mutation in the adenomatous polyposis coli gene consistent with FAP. She subsequently underwent prophylactic total colectomy.The pigmented retinal pigment epithelium lesions of FAP have a characteristic appearance and it is vital for the retinal specialist to be familiar with them. De novo mutations in the APC gene are responsible for 20% to 30% of FAP cases. In the presence of the characteristic retinal pigment epithelium lesions, it is important to send the patient for work up of FAP even in the absence of family history of FAP.

OBJECTIVE: To assess the effectiveness of a 3D-printed custom mask fitter in lieu of N95 respirators among ophthalmologists and other eye care professionals who may not be prioritized to receive N95 respirators amidst the coronavirus disease 2019 pandemic. METHODS: This was a proof-of-concept study from a tertiary eye care center in Oakville, Canada. All participants underwent the N95 Qualitative Fit Test with a custom mask fitter secured over an American Society for Testing and Materials Level 3 face mask. Participants answered a 10-point Likert scale questionnaire on comfort, ease of use, and feasibility of the custom mask fitter, as well as comfort of a regular face mask. RESULTS: Twenty participants were recruited. Of the 20 recruited, 18 (90%) successfully passed the fit test. The median scores for comfort, ease of use, and everyday feasibility for the custom mask fitter were 3.5, 4.5, and 3, respectively, whereas the median score for comfort of a regular face mask was 8.5. CONCLUSION: A reusable, low-cost, 3D-printed custom mask fitter is a potential effective alternative to an N95 respirator among eye care professionals but may require improvement in its design and comfort. This is especially relevant in the context of a limited supply of N95 respirators amidst a global pandemic.

The aim of this study was to evaluate the incidence, severity, and treatment modalities of retinopathy of prematurity (ROP) in moderate and late preterm infants with a gestational age (GA)31 + 6 weeks.ROP screening results of preterm infants with GA31 + 6 weeks to 36 + 6 weeks between March 2013 and January 2019 were evaluated retrospectively. Infants were divided into 2 groups according to GA as 32-33 + 6 weeks (moderate preterm) and 34-36 + 6 weeks (late preterm). In these groups, any ROP and severe ROP (requiring treatment) development rates and ROP types and treatment modalities were evaluated.A total of 4156 preterm infants, 1875 (45.1%) female and 2281 (54.9%) male, were included. Overall, 1466 (35.2%) of the infants were moderate preterm and 2690 (64.8%) were late preterm. The incidences of any ROP and severe ROP were 22% and 2.5%, respectively. The rate of severe ROP was 5.3% in moderate preterm infants and 0.9% in late preterm infants. Significant correlations were determined between duration of hospital stay, birth weight (BW), and GA with ROP development (r = +0.415, r = -0.258, r = -0.199, respectively; p0.001 for all). Of 102 patients (2.5%) requiring treatment, 64 (62.7%) had laser, 34 (33.3%) had intravitreal bevacizumab (IVB), 2 (1.9%) had sequential IVB and laser, and 2 (1.9%) had vitreoretinal surgery.ROP seems to still be an important health problem in moderate and late preterm infants in our country according to data from screening high-risk preterm infants with a GA31 + 6 weeks. In this cohort, ROP development correlates with GA, BW, and duration of hospitalization significantly.

Objective To characterize the association between prolonged supine postoperative positioning of patients undergoing Descemet-stripping automated endothelial keratoplasty (DSAEK) and graft dislocation rate. Methods In this retrospective cohort study, medical records of patients who underwent uncomplicated DSAEK surgery at Yitzhak Shamir Medical Center between 2010 and 2019 were reviewed. Nursing documentation of patients’ adherence to supine positioning during the postoperative hospitalization period was collected. A patient was considered compliant if he or she was documented as cooperative with supine positioning throughout the first 24 hours. Results A total of 170 eyes of 138 patients were found eligible. Main indications for surgery were pseudophakic bullous keratopathy (50.6%), previous graft failure (25.9%), and Fuch's endothelial dystrophy (FED; 20.6%). Twelve surgeries were combined with cataract extraction. Postoperative graft detachment occurred in 26 eyes (15.3%) after an average period of 1 day (range, 0–20 days). Compliance with supine positioning was documented in 84.1% (n = 143 patients). Noncompliance rates during the first 24 hours in the detached and nondetached groups were 26.9% (n = 7) and 14.4% (n = 20), respectively; after adjustment for possible confounders, the odds ratio (OR) was 1.44 (p = 0.249). Graft dislocation was observed in 13.3% (19 of 143) and 25.9% (7 of 27) of cooperative and noncooperative patients, respectively (p = 0.17). Subanalysis of 120 eyes with either BPK or FED for which it was the first transplantation demonstrated a protective effect of supine positioning (OR 3.42, confidence interval 1.095–10.700; p = 0.034). Findings for both groups remained unchanged in multivariate analysis. Conclusions We found a statistically significant protective effect of 24 hours of postoperative supine positioning against graft detachment after DSAEK in eyes with no prior transplantations.

Objective This study was designed to evaluate potential differences in circumpapillary retinal nerve fibre layer (cpRNFL) thickness and segmented macular retinal layers between dominant and nondominant eyes on spectral-domain optical coherence tomography in a pediatric population. Design Cross-sectional study. Participants 89 healthy children attending a general pediatric clinic. Methods Participants underwent sighting dominant testing and macular and cpRNFL spectral-domain optical coherence tomography. Segmented macular layer thicknesses and cpRNFL thickness were compared for individual patients based on their ocular dominance. Results Ocular dominance occurred particularly in the right eye (64.7%). Dominant and nondominant eyes did not differ significantly in axial length or spherical equivalent refraction; axial length: 22.99 ± 1.17 mm versus 22.98 ± 1.19 mm; p = 0.51 and spherical equivalent refraction: −0.09 ± 2.68 D versus 0.32 ± 2.93 D; p = 0.41. In the comparison of the macular ganglion layer the average thickness in the 1 mm central Early Treatment Diabetic Retinopathy Study area was significantly different between the dominant and nondominant eye (16.56 ± 6.02 μm vs 17.58 ± 8.32 μm; p = 0.02). However, when compensating with Bonferroni, this difference was no longer statistically significant. There were no differences in the analyses of average global and sectorial cpRNFL thickness in dominant and nondominant eyes. Conclusion Dominant eyes demonstrated no significantly thicker average macular retinal nerve fiber layer (mRNFL), Ganglion cell layer (GCL) thickness or cpRNFL thickness. No ocular characteristic was found to be associated with the relative dominance of an eye in eyes with low anisometropia.

A 63-year-old white man with hypertension, hyperlipidemia, adrenal insufficiency, and history of metastatic spindle cell melanoma previously treated with ipilimumab, presented with unilateral papillitis in the left eye. The disc edema evolved to neuroretinitis with additional MRI findings of perineuritis. Broad laboratory evaluation confirmed active B. henselae infection, and he confirmed exposure to a newly adopted kitten. His vision improved following a course of antibiotics and oral steroids with resolution of clinical findings. To the best of our knowledge, this is the first reported case of cat-scratch neuroretinitis with MRI findings of optic nerve and optic nerve sheath enhancement.

Objective To analyze the factors associated with response (control of ocular inflammation and corticosteroid sparing effect) to biologics (anti-TNF-α agents and tocilizumab) in patients with refractory uveitic macular edema. Design Multicenter retrospective observational study. Subjects Adult patients with uveitic macular edema refractory to systemic corticosteroids and/or disease modifying anti-rheumatic drugs. Methods Patients received anti-TNF-α agents [IFX 5 mg/kg at weeks 0, 2, 6 and every 4-6 weeks (n=69) and ADA 40 mg/14 days (n=80)] and tocilizumab [8 mg/kg every 4 weeks intravenously (n=39) and 162 mg/week subcutaneously (n=16)]. Main Outcome Measures Analysis of complete and partial response rates, relapse rate, low vision (visual acuity in at least one eye ≥ 1 LogMAR), corticosteroid sparing effect and adverse events at 6 months. Results 204 patients (median age of 40 years [28-58] with 42.2% of men) were included. Main etiologies of uveitis included Behcet’s disease (17.2%), birdshot chorioretinopathy (11.3%) and sarcoidosis (7.4%). The overall response rate at 6 months was of 46.2% (21.8% of complete response) with anti-TNF-α agents and 58.5% (35.8% of complete response) with tocilizumab. In multivariate analysis, treatment with tocilizumab (OR 2.10 [95% CI 1.06–4.06], p=0.03) was independently associated with complete response of uveitic macular edema, compared to anti-TNF-α agents. Anti-TNF-α agents and tocilizumab did not differ significantly in terms of relapse rate (HR=1.00 [0.31-3.18], p=0.99) or occurrence of low vision (OR=1.02 [0.51-2.07], p=0.95) or corticosteroid-sparing effect (p=0.29). Adverse events were reported in 20.6% of patients, including 10.8% of serious adverse events. Conclusions Tocilizumab seems to improve complete response of uveitic macular edema compared to anti-TNF-α agents.

This paper develops a model for a cold chain operated among a single product manufacturer and multiple retailers to minimize the total cost of the chain. A distinguishing feature of the cold chain is that the quality of the product degrades with time. The storage temperature and time influence the degradation rate. In this context, quality can be measured using multiple distinct indices. A global stability index is used to quantify the overall quality. The non-Arrhenius model is exclusively applied to measure the quality degradation during the transportation and at a retailer's storage as the product moves in the cold chain. Optimal replenishment times for the manufacturer and retailers are decision variables and the cold chain's total cost includes the cooling cost, carbon emission cost, and loss of value in addition to the traditional supply chain costs. It is found that for a given manufacturer's replenishment time, the total cost is convex on each retailer's replenishment time. Hence, an algorithm is developed based on this property to find the optimal replenishment times for the manufacturer and retailers. This paper also investigates how specific cold chain parameters influence optimal decisions and the total cost. The analyses show that the resulting costs will be significantly higher if quality degradation is ignored in a cold chain.

Purpose To develop a novel deep-learning approach that can describe the structural phenotype of the glaucomatous ONH and can be used as a robust glaucoma diagnosis tool. Design Retrospective, deep-learning approach diagnosis study. Method We trained a deep learning network to segment three neural-tissue and four connective-tissue layers of the ONH. The segmented OCT images were then processed by a customized autoencoder network with an additional parallel branch for binary classification. The encoder part of the autoencoder reduced the segmented OCT images into a low-dimensional latent space (LS); whereas the decoder and the classification branches reconstructed the images and classified them as glaucoma or non-glaucoma, respectively. We performed principal component analysis on the latent parameters and identified the principal components (PCs). Subsequently, the magnitude of each PC was altered in steps and reported how it impacted the morphology of the ONH. Results The image reconstruction quality and diagnostic accuracy increased with the size of the LS. With 54 parameters in the LS, the diagnostic accuracy was 92.0±2.3% with a sensitivity of 90.0±2.4% (at 95% specificity), and the corresponding Dice coefficient for the reconstructed images was 0.86±0.04. By changing the magnitudes of PC in steps, we were able to reveal how the morphology of the ONH changes as one transitions from a ‘non-glaucoma’ to a ‘glaucoma’ condition. Conclusions Our network was able to identify novel biomarkers of the ONH for glaucoma diagnosis. Specifically, the structural features identified by our algorithm were found to be related to clinical observations of glaucoma.

Purpose To report 3 cases of reversible epitheliopathy induced by A166—a human epidermal growth factor receptor (HER2)-targeted antibody-drug conjugate (ADC) therapy for resistant HER2 tumours. Methods Advanced HER2 tumour patients were enrolled in A166 phase I/II clinical trial using Bayesian logistic regression model dose escalation. Key exclusion criteria were ≥grade 2 (G2) corneal pathology, severe organ disease, and other cancer therapy within 4 weeks. Eye exams were performed at baseline, regularly scheduled intervals, and additionally upon A166-induced ocular symptoms. Topical therapy with autologous serum tears (ASTs) was implemented based on visual acuity, symptoms, and slit lamp exam. A166 was withheld if ≥G2 ocular toxicity developed; if status improved to ≤G1, A166 therapy was resumed. Visual acuity, corneal exam, and subjective comfort were recorded. Results After ≥2 cycles of A166, 6 eyes of 3/23 enrolled patients developed whorl pattern epitheliopathy suggestive of limbal stem cell (LSC) dysfunction requiring cessation of A166 despite positive tumour response. Patients 1 and 3 received 3.6 mg/kg A166 dose, and patient 2 received 3.0 mg/kg. Topical steroids (2/4 eyes) failed to improve epitheliopathy. Adding ASTs improved vision, ocular comfort, and whorl pattern epitheliopathy in 6/6 eyes within 3 weeks. Patient 1 continues to improve on ASTs; patient 2 withdrew from the study; and patient 3 resumed A166 therapy. Conclusion A166 precipitates LSC dysfunction-like epitheliopathy. Combination therapy including aggressive lubrication, withholding drug, and ASTs help reverse toxicity. Recognizing that ADC-induced epitheliopathy can respond to ocular management may enable cancer patients to continue lifesaving therapy.

Progressive familial intrahepatic cholestasis (PFIC) is a rare disease of impaired bile acid excretion which can lead to nutritional deficiencies. Vitamin deficiencies during pregnancy can result in adverse maternal and fetal outcomes. A 20-year-old primiparous woman at 30 4/7 weeks with PFIC type 2 presented with worsening cholestasis, coagulopathy and fat-soluble vitamin deficiency. She developed visual deficits and was found to have severe vitamin A deficiency. Her coagulopathy and visual deficits improved following vitamin K and A supplementation, respectively. She delivered at 32 2/7 weeks following preterm labour. This case highlights several unique aspects in the care of pregnant women with liver disease. These patients are at risk for fat-soluble vitamin deficiencies which can result in significant coagulopathy and rarely, visual deficits due to vitamin A deficiency. Prompt treatment is necessary to prevent permanent sequelae.

IMPORTANCE: This study identifies the diagnostic errors leading to misdiagnosis of 3(rd) nerve palsy in order to aid clinicians in making this diagnosis. OBJECTIVE: To determine the incidence of misdiagnosis of third cranial nerve palsy (3(rd) nerve palsy) amongst providers referring to a tertiary care neuro-ophthalmology clinic and to characterize diagnostic errors that led to an incorrect diagnosis. DESIGN: Retrospective clinic-based cross-sectional study of office encounters at two institutions from 1/2014 to 1/2017. All encounters with scheduling comments containing variations of “3(rd) nerve palsy” were reviewed. Patients with a documented referral diagnosis of new 3(rd) neve palsy were included in the study. Examination findings including extraocular movement exam, external lid exam, and pupil exam were collected. The final diagnosis was determined by a neuro-ophthalmologist. The Diagnosis Error Evaluation and Research (DEER) taxonomy tool was used to categorize the causes of misdiagnosis. SETTING: Multicenter study describing patients at two neuro-ophthalmology referral centers. PARTICIPANTS: 78 patients referred for a new diagnosis of 3(rd) nerve palsy. MAIN OUTCOMES AND MEASURES: The main outcome measure was the type of diagnostic error that led to incorrect diagnoses using the DEER criteria as determined by two independent reviewers. Secondary outcomes were rates of misdiagnosis, misdiagnosis rate by referring specialty, and examination findings associated with incorrect diagnoses. RESULTS: Out of 78 patients referred with a suspected diagnosis of 3(rd) nerve palsy, 21.8% were determined to have an alternate diagnosis. The most common error in misdiagnosed cases was failure to correctly interpret the physical exam. Ophthalmologists were the most common referring provider for third nerve palsy, and optometrists had the highest overdiagnosis rate of 3(rd) nerve palsy. CONCLUSIONS: Misdiagnosis of 3(rd) nerve palsy was common. Performance and interpretation of the physical exam were the most common factors leading to misdiagnosis of 3(rd) nerve palsy.

Single median maxillary central incisor (SMMCI) syndrome is rare. It is commonly associated with other midline defects. About 50% of children with SMMCI have short stature, associated with isolated growth hormone deficiency or panhypopituitarism.A 6-year-old girl presented to us with worsening convergent squint, slowing linear growth and a suspected pituitary macroadenoma on neuroimaging. The key findings on examination included a disproportionate short stature, SMMCI, congenital abduction defect and pseudohypertrophy of calf muscles with myopathy. The evaluation showed autoimmune thyroiditis with pituitary hyperplasia. Bone age corresponded to 3 years.Three months after initiation of thyroxine, her myopathy resolved, and the hormone profile and neuroimaging were normal. Autoimmune thyroiditis in association with SMMCI is not reported previously. This case study emphasises the importance of growth monitoring and the exclusion of common treatable conditions.