Sight-Threatening Unilateral Posterior Scleritis With Positive Atypical p-ANCA As Early Manifestation of Lupus Spectrum Disease.

Antinuclear cytoplasmic antibody (ANCA)-related scleritis is a potentially sight-threatening inflammatory condition that may occur as a primary vasculitis disorder or as a secondary vasculitis in a variety of inflammatory conditions. While ANCA has been classically associated with primary vasculitis diseases such as granulomatosis with polyangiitis (GPA), microscopic polyarteritis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA), it is interesting that in cases of lupus spectrum disease (LSD), both ANCA and atypical p-ANCA have been observed as secondary autoantibodies. Scleritis is a rare ocular manifestation of lupus disease with an incidence of around 1%. This paper describes a case of sight-threatening posterior scleritis with positive atypical p-ANCA as an early manifestation of LSD. LSD is an acknowledged condition but frequently presents a diagnostic challenge or delay due to its ambiguous symptoms which may not fully align with the classification criteria of established systemic lupus erythematosus (SLE). Nonetheless, this condition should not be underestimated due to its potential impact on major organ involvement and its tendency to progress to established SLE. The diagnosis of LSD heavily relies on clinician suspicion, considering factors such as symptoms present in at least one organ system, positivity of antinuclear antibody (ANA), and clinical suspicion of future SLE development. Early identification allows for early treatment which would benefit high-risk patients. A middle-aged Chinese lady presented with bilaterally asymmetrical eye redness and swelling, which was worse on the right side. Clinical examination revealed right eye proptosis, conjunctival injection, chemosis, scleral redness and binocular diplopia in all gazes. Right eye fundoscopic examination displayed extensive choroidal folds with a positive T-sign on the B-scan. Apart from ocular symptoms, there was no significant medical history related to autoimmune or connective tissue disorders. Her p-ANCA and c-ANCA results were negative, however atypical p-ANCA titer was positive with a high antinuclear antibody (ANA) titer of 1:1280 with a homogenous pattern. Additionally, she has a family history of systemic lupus erythematosus in her daughter. A diagnosis of right eye posterior scleritis secondary to underlying LSD was made. The scleritis was successfully treated with a combination of corticosteroid and systemic immunosuppressants and the patient was initiated on oral hydroxychloroquine to manage underlying LSD. We aim to highlight to clinicians the diagnostic challenges associated with scleritis in LSD and emphasize the importance of prompt and timely multidisciplinary management in minimizing patient mortality and morbidity, as reflected in this case. This case of a positive atypical p-ANCA scleritis in LSD serves as an excellent example of effective management.

Double Seronegative Neuromyelitis Optica Spectrum Disorder with Longitudinally Extensive Transverse Myelitis and Optic Neuritis: A Challenging Case Report.

Neuromyelitis optica spectrum disorder (NMOSD) is a rare antibody-mediated neuroinflammatory disease of the central nervous system, typically manifesting in the optic nerves, spinal cord, and other regions of the central nervous system. We hereby report a case of a 16-year-old girl who presented with a six-month history of transverse myelitis with an acute episode of bilateral retrobulbar optic neuritis. MRI revealed patchy contrast enhancements over bilateral retrobulbar intraorbital optic nerves together with long-segment spinal cord hyperintensities (C2 to T2 level). Visual evoked potential testing during the acute presentation showed the absence of P100 bilaterally. However, both serum AQP4-IgG and MOG-IgG were reported to be negative. Despite remarkable improvement in bilateral optic nerve functions, she continued to have disabling bilateral lower limb spasticity, contractures, and loss of bilateral lower limb sensation after five cycles of plasma exchange. This case summarizes the challenges to diagnosing double seronegative NMOSD and its immediate therapeutic significance.

Various Ocular Manifestations in Systemic Lupus Erythematosus: A Case Series.

Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune disorder with various systemic and ocular clinical manifestations. In patients with SLE, central retinal vein and artery occlusion, choroidopathy, and occlusive vasculitis are among the most significant and clinically relevant ocular manifestations, although they do not commonly occur. We present a case series of three SLE patients of different races and genders who developed ocular-related clinical features of SLE during the course of their systemic disease. The clinical outcomes of each patient were different, affecting their vision in bilateral eyes, with some patients having better visual recovery while others having permanently poor vision. These outcomes were not significantly related to the patients' age, gender, or race.

Developing and evaluating the usability of a web-based Diabetic Retinopathy Health Education Program for patients with type 2 diabetes mellitus.

OBJECTIVES: To describe the development of a webpage based on the Intervention Mapping (IM) protocol and usability testing of the Diabetic Retinopathy Health Education Profram (DRHEP). METHODS: The mixed methods pilot feasibility study was carried out between April and September 2021, involving 16 patients with type 2 diabetes mellitus and 5 experts. The usability score was rated according to the System Usability Scale (SUS). RESULTS: The average SUS score by the experts was 88. The patients gave a higher score of 85 for SUS, with 58 as the lowest. The average SUS score was 72. The findings indicate that the webpage is acceptable, good, and highly usable for users. CONCLUSION: The outcomes of this study signify the relationship between effective health applications and how their design might hamper their effectiveness in changing patients' behavior.

COVID-19 and Crossed Eye: A Case Report and Literature Review.

This study aims to report a case of neuro-ophthalmic manifestation in a coronavirus disease 2019 (COVID-19) patient and a literature review of neuro-ophthalmological manifestation in COVID-19 patients. A 57-year-old male presented with headache, giddiness, and sudden onset of diplopia over two days after having a flu-like illness. Clinical examination revealed bilateral bizarre extraocular movement with right lower motor neuron facial nerve palsy. A polymerase chain reaction test for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) was positive. Computed tomography (CT) and contrast-enhanced CT of the brain and CT venography were unremarkable. Magnetic resonance imaging (MRI) of the brain, magnetic resonance angiography of the circle of Willis, and MRI of the internal auditory meatus revealed a subacute pontine infarction with no leptomeningeal or cranial nerve enhancement and a normal circle of Willis. The patient received a course of oral prednisolone and clinical symptoms improved gradually. Articles published between December 2019 and June 2022 were included. A total of 23 cases, with 14 male and nine female patients, were summarized. The mean age at presentation was 46.95 years (range = 9-71 years), with the most affected age group ranging from 31 to 70 years (17 of 23 cases, 73.91%). Neuro-ophthalmological symptoms and signs can be isolated or associated with neurological syndromes. The manifestations include optic neuritis, isolated or multiple cranial nerve palsies, acute vision loss, Miller Fisher syndrome, myasthenia gravis, acute disseminated encephalomyelitis, Guillain-Barré syndrome, internuclear ophthalmoplegia, and cerebrovascular events. Diagnosing neuro-ophthalmic complications secondary to SARS-CoV-2 infection is challenging, as there are no pathognomonic symptoms to detect the disease. High clinical suspicion aids in early diagnosis and initiation of treatment may help in relieving the symptoms.

Review of Risk Factors and Complications of Anterior Migration of Ozurdex Implant: Lessons Learnt from the Previous Reports.

Purpose: Ozurdex had shown promising anatomical and functional outcomes in managing refractory Irvine-Gass syndrome over the years. Burgeoning usage of Ozurdex has prompted the study of its related complications, particularly the anterior chamber migration of the implant. Methods: Literature reviews on the anterior chamber migration of the Ozurdex via PubMed, EBSCO, and TRIP databases were searched from 2012 to 2020. The predisposing factors, outcomes, and management of such cases were evaluated. Results: A total of 54 articles consisting of 105 cases of anterior migration of Ozurdex were included in this analysis. The vitrectomized eye and compromised posterior capsule were highly associated with this complication. About 81.9% of the cases had cornea edema upon presentation, with 31.4% of them ending up with cornea decompensation despite intervention. Although there was high intraocular pressure reported initially in 22 cases, only 2 cases required glaucoma filtration surgeries in which they had preexisting glaucoma. Numerous techniques of repositioning or surgical removal of the implant were described but they were challenging and the outcomes varied. Conclusions: A noninvasive method of manipulating the Ozurdex into the vitreous cavity via the "Trendelenburg position, external pressure with head positioning" maneuvers is safe yet achieves a favorable outcome. Precaution must be taken whenever offering Ozurdex to the high-risk eyes. Prompt repositioning or removal of the implant is crucial to deter cornea decompensation. Clinical Trial Registration number: NMRR-22-02092-S9X (from the Medical Research and Ethics Committee (MREC), Ministry of Health, Malaysia).

Purtscher's Retinopathy After Cardiopulmonary Resuscitation: A Literature Review.

Purtscher's retinopathy is a rare angiopathy reported in patients with a history of severe trauma and other systemic diseases. The diagnosis is made on clinical grounds, and the severity varies. A 41-year-old gentleman with underlying poorly controlled diabetes mellitus and dyslipidemia was referred to the ophthalmology department for diabetic retinopathy screening. He denied visual complaints. Ocular examination revealed a negative relative afferent pupillary defect with a visual acuity of 6/6 bilaterally. The anterior segment examination was unremarkable. Both eye (oculus uterque, OU) fundus revealed a pink disc with a cup-to-disc ratio of 0.4 and peripapillary flame-shaped hemorrhages. Right eye (oculus dexter, OD) also showed multiple cotton wool spots along the superotemporal arcade involving zones 1 and 2 of the retina, while left eye (oculus sinister, OS) showed a single cotton wool spot along the superotemporal arcade at zone 1 of the retina. Otherwise, there were no visible retinal emboli, dot hemorrhages, or hard exudates, and the macula was normal. The retinal features were not characteristic of diabetic retinopathy. It mimicked hypertensive retinopathy, but the patient was normotensive. The absence of inner retinal thickening and hyperreflectivity on optical coherence tomography of the macula ruled out retinal vein occlusion. This prompted us to elicit further history, and the patient disclosed a recent admission for myocardial infarction in which he received cardiopulmonary resuscitation with chest compressions for seven minutes. Hence, the diagnosis of OU Purtscher's retinopathy was made, and the patient was monitored closely in the clinic. Purtscher's retinopathy remains a diagnostic dilemma and should not be neglected in complex clinical contexts.

Effect of slow tempo music on markers of anxiety during cataract surgery: Randomized control trial.

PURPOSE: This study aims to objectively measure the effect of slow tempo music on various markers for anxiety. MATERIALS AND METHODS: This is a repeated measure randomized control trial of patients with age-related cataracts undergoing cataract extraction through phacoemulsification under local anesthesia. Patients were randomized into two groups: music group where a standardized piano music was played and control group. Salivary alpha-amylase (sAA) levels were measured at the beginning and at the end of surgery. Blood pressure and heart rate were also measured at 5 min before surgery and at four other standardized points during and after surgery. Visual Analog Scale for anxiety was also gauged: preoperatively, perioperatively, and 15 min postoperatively. RESULTS: Ninety-two patients were randomized equally to the music group and control group. Paired sample t-test showed a reduction in the level of sAA during surgery in the music group (P = 0.019). The odds ratio for a drop in sAA with music was 4.407 (P = 0.001). Lower systolic blood pressure was observed in the music group: at delivery of local anesthesia (P = 0.047), at first incision (P = 0.023), and during sculpting (P = 0.15). Similarly, diastolic blood pressure was lower at first incision (P = 0.019) in the music group. The visual analog scale for anxiety during surgery was found to be lower in the music group (P = 0.046). CONCLUSION: A slow tempo music during cataract surgery was shown to significantly reduce several indicators for anxiety at various points during cataract surgery.

Ophthalmic features of craniosynostosis: A Malaysian experience.

BACKGROUND: This study aims to collect local Malaysian data regarding the ophthalmic features and complications in craniosynostosis patients who attended the Combined Craniofacial Clinic (CFC) in University Malaya Medical Centre (UMMC). METHODS: Retrospective study of medical notes of craniosynostosis patients who attended the CFC in UMMC from 2014 to December 2020. RESULTS: Out of 37 patients, 29 had syndromic craniosynostosis, and 8 had non-syndromic craniosynostosis. Visual impairment was present in 32.1% of patients. Causes for visual impairment were as follows - amblyopia (25.0%), exposure keratopathy (3.6%), and optic atrophy (3.6%). Hypermetropia and myopia were each seen in 20.6% of patients. Astigmatism was seen in 47.1% of patients, and 29.1% had anisometropia. Proptosis was present in 78.6% and lagophthalmos in 53.3% of patients. Strabismus in primary position occurred in 51.7% of patients. Thirty-one percent of the patients had exposure keratopathy. Optic disc atrophy was seen in 13.7% of patients, and 8.3% had optic disc swelling. Optic disc swelling was resolved in all patients who underwent craniofacial surgery. CONCLUSION: Our experience in Malaysia was consistent with previously reported data on ophthalmic features of craniosynostosis patients. Additionally, we found that non-syndromic craniosynostosis patients are also at risk of ocular complications just as much as syndromic patients. Appropriate treatment of amblyogenic risk factors, ocular complications, and timely detection of papilledema, and prompt surgical intervention are crucial in preserving long-term visual function in these patients.

A case series with literature review on adjunctive usage of intravitreal ceftazidime in ocular syphilis with human immunodeficiency virus infection.

BACKGROUND: Penicillin is the conventional treatment for all stages of syphilis, including ocular and neurosyphilis, according to the recommendations by the Centre for Disease Control and Prevention Sexually Transmitted Disease. This case series highlighted three cases of ocular syphilis which showed prompt treatment response as early as 24 h after the adjunctive intravitreal ceftazidime injection. METHODS: Case Series. RESULTS: In case 1, there was significant improvement in the vision and vitritis after 24 h of a single intravitreal ceftazidime injection. In case 2, the patient achieved his best vision after a total of three intravitreal injections in his left eye and one in his right eye. In case 3, there was a further resolution of perivascular sheathing and retinal haemorrhages seen 1 week after a single intravitreal ceftazidime injection. CONCLUSION: The efficacy of intravitreal ceftazidime injection as a local adjunctive therapy in ocular syphilis treatment is noteworthy. It can be considered in cases which show suboptimal or slower treatment response despite early commencement of systemic penicillin to prevent devastating ocular sequelae.

Ocular surface disease in psoriatic patients in a developing country.

PURPOSE: To evaluate ocular surface changes among patients with psoriasis in Malaysia, a developing country in Southeast Asia. METHODS: An interdisciplinary case-control study (60 psoriasis patients and 40 control subjects) to look at the differences in ocular surface manifestations between patients with psoriasis and a group of age-, gender- and ethnicity-matched healthy controls. RESULTS: One hundred and twenty eyes of 60 patients with psoriasis and 80 eyes of 40 healthy controls without psoriasis were included in the study. Mild-to-moderate psoriasis was found in 42 patients (70%), while 18 patients (30%) had severe psoriasis. Psoriatic arthritis was found in 19 patients (32%). Of the 60 psoriatic patients, the prevalence of ocular involvement was 65% (39/60), in which 32% (19/60) had dry eyes, 27% (16/60) had lid margin abnormalities, 33% (20/60) had cataract, and one had history of anterior uveitis. Compared to controls, ocular surface of psoriatic patients showed more eyelid margin abnormalities, higher meibomian gland loss and lower tear film break-up time. The estimated odds ratio for dry eyes in the psoriasis group was 2.2 (95% CI: 0.8-6.9). CONCLUSION: Ocular surface disorders encompassing eyelid margin abnormalities, meibomian gland loss and tear dysfunction occur at an earlier and higher rate among psoriatic patients.

Effect of phacoemulsification on intraocular pressure and bleb function in eyes with previous trabeculectomy / 国际眼科杂志(Guoji Yanke Zazhi)

?AIM:To observe the intraocular pressure ( IOP) control and bleb function after phacoemulsification in patients with previous history of successful/qualified success mitomycin-C augmented trabeculectomy.?METHODS:This was a retrospective cohort study. Data of patients who had undergone trabeculectomy between 1st January 2013 to 31st Dec 2015 with subsequent cataract surgery were extracted from server. All patients had previous either success/qualified success trabeculectomy done. phacoemulsification via clear corneal incision. Postoperative follow-up, review of bleb and IOP readings using Goldmann tonometer were taken. Details that were investigated include postoperative IOP control, visual improvement, number of anti-glaucoma medications, as well as the timing from trabeculectomy to phacoemulsification. Comparison with control group was made.?RESULTS: Fifteen eyes from thirteen patients fulfilled the criteria for study and had undergone uncomplicated phacoemulsification on a post-trabeculectomy eye. Two of the eyes had secondary glaucoma while the other thirteen had primary glaucoma. All phacoemulsifications were done at least 6mo after trabeculectomy (mean 14.7± 4.3mo ) . There was only 1 eye ( 6. 7％) requiring anti-glaucoma medications before the cataract surgery. This number increased to 4 ( 26. 7％) at 1 - year post -phacoemulsification. The number further increased to eight ( 53. 3％) at 2y post - phacoemulsification. The number of anti-glaucoma drops needed after surgery at 2y ranged from 2-4. In all the 15 eyes, there was no statistically significant change in IOP control between pre-cataract surgery (mean 13.4±2.9 mmHg) compared to 1y (mean 14.1±3.2 mmHg, P=0.357) and 2y (mean 15.1±3.3 mmHg, P = 0. 212 ) post - phacoemulsification. Visual improvement after phacoemulsification however is significant, from a preoperative average visual acuity of LogMAR 1.52± 1.00 to a postoperative average visual acuity of 0.53± 0.54 ( P=0. 000, paired t-test) .?CONCLUSION: This analysis shows significant visual improvement following cataract surgery in post -trabeculectomy patients without compromising IOP control. However, a reduced bleb function is noted following the surgery evidenced by the increase in number of anti - glaucoma drops used after surgery especially two years after the cataract surgery. Patients should be counselled regarding the possibility of restarting on anti - glaucoma medications post -phacoemulsification. The timing and sequence of cataract and glaucoma surgery should be optimized for best outcome.

Role of Angiography in Systemic Lupus Erythematosus-Induced Choroiditis.

PURPOSE: To report a case of systemic lupus erythematosus-induced choroidal vasculitis. METHODS: A 34-year-old woman with a long-standing history of systemic lupus erythematosus had a sudden painless loss of vision in the right eye over 12 hours. Ocular examination revealed a visual acuity of counting fingers of 1 foot on the right eye and 20/20 on the left. There was a relative afferent pupillary defect on the right side with a pink, distinct optic disk margin. RESULTS: Optical coherence tomography of the macula and fundus fluorescein angiogram for the eyes were normal. The MRI brain and orbit with the cerebral MRA did not show signs of optic neuritis or occipital vasculitic changes. However, the indocyanine green angiography revealed patches of ill-defined areas of choroidal hypofluorescence in the early- to mid-phase in the macula region. CONCLUSION: ICGA becomes the crucial tool in unmasking the presence of choroidal vasculitis.

Diabetes and pachymetry changes in pregnancy.

BACKGROUND: Purpose of this study is to evaluate changes in the central corneal thickness (CCT) in patients during the third trimester and postpartum phases of normal pregnancy, pregestational diabetes mellitus (DM), and gestational diabetes mellitus (GDM). METHODS: This was a prospective study. Patients that fulfilled the inclusion criteria were recruited from the obstetric clinic. They were grouped into normal pregnancy, pregestational DM, and GDM. Ophthalmic assessment and haematological investigations were done during the third trimester (after 28 weeks of gestation) and in the postpartum phase (6 weeks postpartum). RESULTS: A total of 192 pregnant patients were recruited for this study. Out of the 192 patients, only 143 of them came back for their follow-up 6 weeks postpartum. A total of 70 (36.5%) normal pregnancy patients, 51 (26.6%) DM patients, and 71 (36.9%) GDM patients were included in this prospective study. Our study showed that the CCT decreased postpartum in all three groups. Patients in all three groups did not have significantly different CCT during the third trimester of pregnancy and postpartum phase. However, patients who had thicker CCT irrespective of the grouping during the third trimester also had a thicker CCT post-delivery (p value <0.001). However, these changes did not appear to affect refractive error and visual acuity. CONCLUSIONS: Diabetes mellitus during pregnancy did not appear to influence the CCT.

Soluble receptor for advanced glycation end-product (sRAGE)/pentosidine ratio: a potential risk factor determinant for type 2 diabetic retinopathy.

This study aims to investigate potential diabetic retinopathy (DR) risk factors by evaluating the circulating levels of pentosidine, soluble receptor for advanced glycation end-product (sRAGE), advanced oxidation protein product (AOPP) as well as glutathione peroxidase (GPx) and superoxide dismutase (SOD) activities in DR patients. A total of 235 healthy controls, 171 type 2 diabetic without retinopathy (DNR) and 200 diabetic retinopathy (DR) patients were recruited. Plasma was extracted for the estimation of pentosidine, sRAGE, AOPP levels and GPx activity whereas peripheral blood mononuclear cells were disrupted for SOD activity measurement. DNR and DR patients showed significantly higher levels of plasma pentosidine, sRAGE and AOPP but lower GPx and SOD activities when compared to healthy controls. The sRAGE/pentosidine ratio in DR patients was significantly lower than the ratio detected in DNR patients. Proliferative DR patients had significantly higher levels of plasma pentosidine, sRAGE, AOPP and sRAGE/pentosidine ratio than non-proliferative DR patients. High HbA1c level, long duration of diabetes and low sRAGE/pentosidine ratio were determined as the risk factors for DR. This study suggests that sRAGE/pentosidine ratio could serve as a risk factor determinant for type 2 DR as it has a positive correlation with the severity of DR.

Receptor for advanced glycation end-product (RAGE) gene polymorphism 2245G/A is associated with pro-inflammatory, oxidative-glycation markers and sRAGE in diabetic retinopathy.

BACKGROUND: Receptor for advanced glycation end-product (RAGE) gene polymorphism 2245G/A is associated with diabetic retinopathy (DR). However, the mechanism on how it affects the disease development is still unclear. AIM: This study aims to investigate the relationship between 2245G/A RAGE gene polymorphism and selected pro-inflammatory, oxidative-glycation markers in DR patients. METHODS: A total of 371 unrelated type 2 diabetic patients [200 with retinopathy, 171 without retinopathy (DNR)] and 235 healthy subjects were recruited. Genotyping was performed by polymerase chain reaction-restriction fragment length polymorphism method followed by DNA sequencing. The nuclear and cytosolic extracts from peripheral blood mononuclear cells were used for nuclear factor kappa B (NF-κB) p65 and superoxide dismutase activity measurement respectively. Plasma was used for glutathione peroxidase activity, advanced oxidation protein product (AOPP), monocyte chemoattractant protein (MCP)-1, pentosidine and soluble RAGE (sRAGE) measurements. RESULTS: DR patients with 2245GA genotype had significantly elevated levels of activated NF-κB p65, plasma MCP-1, AOPP and pentosidine but lower level of sRAGE when compared to DR patients with wild-type 2245GG. CONCLUSION: The RAGE gene polymorphism 2245G/A is associated with pro-inflammatory, oxidative-glycation markers and circulating sRAGE in DR patients. Patients with 2245GA RAGE genotype could aggravate DR possibly via NF-κB mediated inflammatory pathway.