A Rare Case of von Willebrand Disease Presenting as Hemolacria and Literature Review.

Hemolacria is a rare condition that causes a person to produce tears that are partially composed of blood. It can be a presenting feature of certain ocular and systemic conditions. Here, the authors describe an interesting case of a 12-year-old boy with an underlying beta-thalassemia trait, who presented with a 2-day history of bilateral blood-stained tears, and an episode of epistaxis. Ocular examination was normal, and syringing showed no nasolacrimal duct blockage. Systemic examination was unremarkable. Laboratory investigations confirmed type 2 von Willebrand disease. Management of hemolacria remains a clinical challenge given the rare occurrence of the disease. In this case report, the authors discuss the differential diagnosis and management approach to hemolacria.

Sickle Cell Maculopathy: Prevalence, Associations and Impact on Visual Acuity.

AIMS: To investigate the prevalence of sickle cell maculopathy (SCM), and associations with age, sex, genotype, proliferative sickle cell retinopathy (PSR) stage, and the impact on visual acuity. METHODS: Age, sex, and visual acuity were recorded and spectral domain OCT and ultra-wide-field images of the macula and retina were reviewed in a consecutive series of 74 adults with sickle cell disease. RESULTS: The median age was 37 years (range 19-73 years) and 36 cases (48.6%) were male. SCM was present in at least 1 eye of 40 cases (54.1%) or in 67 of all eyes (42.3%). SCM prevalence was 54.8%, 62.5%, and 25% for the HbSS, HbSC, and HbS/BThal or other genotypes, respectively. SCM was observed in 41 (39.4%) of the eyes with PSR stages 0, 1, and 2, and in 21 (51.2%) of the eyes with PSR stages 3, 4, and 5, respectively. Mild visual impairment or worse was present in 3 eyes (4.8%) with SCM but this was secondary to other pathology. CONCLUSION: SCM is a frequent finding in the eyes of adults with sickle cell disease. The prevalence is similar for the HbSS and HbSC genotypes and is not related to the PSR stage. High-contrast distance visual acuity is typically preserved.

Treatment of uveitis and scleritis patients in Malaysia.

AIM: To determine the common causes and visual outcome after treatment among uveitis and scleritis patients. METHODS: This is a retrospective cohort observational study. All consecutive clinical records of patients with newly diagnosed uveitis and scleritis over a 4-year period, from Jan. 1, 2017 to Dec. 31, 2020, were analysed. Data was collected at the presentation and included a follow-up period of one year. RESULTS: A total of 288 patients were recruited during the study period. Anterior uveitis was the most common anatomical diagnosis (50.0%) followed by panuveitis (25.0%), scleritis (13.5%), posterior uveitis (6.9%), and intermediate uveitis (4.5%). Viral Herpes was the most common cause of infectious cases, while Vogt-Koyanagi-Harada (VKH) disease and human leucocyte antigen (HLA) B27 spondyloarthropathy were the leading causes of identifiable non-infectious cases. Majority of patients presented with unilateral, non-granulomatous uveitis with an absence of hypopyon. Anatomical locations like posterior uveitis and panuveitis, and visual acuity worse than 3/60 at presentation were the factors associated with poor visual outcomes (P<0.05). About 60% of patients had an identifiable cause for the uveitis and scleritis, with nearly equal distribution of infectious (n=85, 29.5%) and non-infectious causes (n=84, 29.2%). About 14.5% of patients were clinically blind at 1y of follow-up. The most common complication in our uveitis patients was glaucoma (47.5%), followed by cystoid macula oedema (18.9%) and cataract (13.9%). CONCLUSION: Uveitis and scleritis are important causes of ocular morbidity. They are potentially blinding diseases which can have a good outcome if diagnosed and treated early.

Rationale and protocol paper for the Asia Pacific Network for inherited eye diseases.

PURPOSE: There are major gaps in our knowledge of hereditary ocular conditions in the Asia-Pacific population, which comprises approximately 60% of the world's population. Therefore, a concerted regional effort is urgently needed to close this critical knowledge gap and apply precision medicine technology to improve the quality of lives of these patients in the Asia-Pacific region. DESIGN: Multi-national, multi-center collaborative network. METHODS: The Research Standing Committee of the Asia-Pacific Academy of Ophthalmology and the Asia-Pacific Society of Eye Genetics fostered this research collaboration, which brings together renowned institutions and experts for inherited eye diseases in the Asia-Pacific region. The immediate priority of the network will be inherited retinal diseases (IRDs), where there is a lack of detailed characterization of these conditions and in the number of established registries. RESULTS: The network comprises 55 members from 35 centers, spanning 12 countries and regions, including Australia, China, India, Indonesia, Japan, South Korea, Malaysia, Nepal, Philippines, Singapore, Taiwan, and Thailand. The steering committee comprises ophthalmologists with experience in consortia for eye diseases in the Asia-Pacific region, leading ophthalmologists and vision scientists in the field of IRDs internationally, and ophthalmic geneticists. CONCLUSIONS: The Asia Pacific Inherited Eye Disease (APIED) network aims to (1) improve genotyping capabilities and expertise to increase early and accurate genetic diagnosis of IRDs, (2) harmonise deep phenotyping practices and utilization of ontological terms, and (3) establish high-quality, multi-user, federated disease registries that will facilitate patient care, genetic counseling, and research of IRDs regionally and internationally.

Diagnostic Challenges in an Atypical Case of IRVAN Syndrome: Literature Review.

A 60-year-old female with known seropositive rheumatoid arthritis and other co-morbids, presented with right eye hypertensive retinopathy and exudative macroaneurysms. Over the years, she developed vitreous haemorrhage, macula oedema and full thickness macula hole. Fluorescein angiography showed macroaneurysms and ischaemic retinal vasculitis. Initial diagnosis was thought to be hypertensive retinopathy with macroaneurysms and retinal vasculitis secondary to rheumatoid arthritis. Laboratory investigations did not support other causes for macroaneurysms and vasculitis. Hence, delayed diagnosis of IRVAN syndrome was made after detailed review of clinical findings, investigations, and angiographic evidence. Our understanding of IRVAN continues to evolve in the face of challenging presentations. To our knowledge, this is the first reported case of IRVAN associated with rheumatoid arthritis.

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.

Unusual presentation of infectious crystalline keratopathy: A case report with literature review.

INTRODUCTION: Infectious crystalline keratopathy (ICK) is a rare corneal disease. ICK has been recognised in patients with immunocompromised cornea or post penetrating keratoplasty. Here we report a case of ICK in an apparently healthy cornea. CASE DESCRIPTION: A 25-years old Chinese female, with no history of systemic or ocular disease, presented to the eye clinic with one-month history of right eye (RE) blurring of vision with foreign body sensation. On examination, there were dense white crystalline needle-like projections over inferior paracentral corneal stroma with intact epithelium. There was also presence of lower eyelid epiblepharon with lashes rubbing against the diseased area. Corneal scraping cultures were suggestive of bacterial infection. Patient responded well with corneal epithelium debridement, intensive topical antibiotics and epiblepharon correction to prevent further microtrauma. CONCLUSIONS: The only contributing factor for ICK in our patient was trichiasis from epiblepharon. Repetitive microtrauma caused by the eyelashes lead to direct penetration and inoculation of normal ocular flora into the corneal stroma. Clinicians need to be vigilant in ruling out other possible causes such as lid abnormalities when managing an ICK patient without apparent risk factors.

Ocular Jarisch-Herxheimer Reaction in the Treatment of Ocular Syphilis: A Case Report and Review of the Literature.

Jarisch-Herxheimer reaction (JHR) is a transient clinical phenomenon in patients with syphilis who receive antibiotic treatment. A 31-year-old man with an underlying HIV infection presented with worsening vision in the right eye two days after being treated with oral doxycycline for presumed left-eye neuroretinitis. Prior history revealed two episodes of penile discharge and ulcers that were not investigated. Examination showed bilateral optic disc swelling with right eye placoid chorioretinitis around the macula. Optical coherence tomography (OCT) demonstrated right macular edema and left macular thinning. Blood investigations confirmed syphilis infection. Subsequently, the patient was scheduled for a contrasted brain CT with oral steroid coverage due to underlying allergies. His vision incidentally improved soon after the short course of steroids. Repeated OCT demonstrated marked improvement of right macular edema, which we believe was secondary to JHR initiated by the earlier doxycycline treatment. Following oral steroid addition, improvement in vision and ocular findings were seen. At six-month post-treatment, there was right macular atrophy as a sequela of the macular edema. Ophthalmologists should be aware of ocular-related JHR complications, particularly in potentiating macular atrophy following macular edema upon initiating antibiotic treatment in syphilitic disease.

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.

Oculomotor and trochlear nerve neuritis following botulinum toxin injection for masseter hypertrophy - a case report with literature review.

OBJECTIVE: To report a rare complication of oculomotor and trochlear nerve neuritis following botulinum toxin injection for masseter hypertrophy. CASE PRESENTATION: A previously healthy 31-year-old man presented with a two-week history of left eye (OS) ptosis and diplopia, following botulinum toxin injection over the masseter area for masseter hypertrophy at an aesthetic centre. He had no proptosis or facial asymmetry. Visual acuity was 6/6 in the right eye (OD) and 6/9 in the OS. There was anisocoria, with pupils measuring 3 mm in the OD and 5 mm in the OS but no relative afferent pupillary defect. OS appeared hypertropic in primary gaze with impaired intorsion. Extraocular movement of the OS was restricted in all gazes, except for laevoversion; that of the OD was normal. This was associated with diplopia in all gazes except on laevoversion. Both eyes' anterior and posterior segment examinations were otherwise unremarkable. Besides the oculomotor and trochlear nerve, the other cranial nerves and neurological examinations were normal. Investigations including blood and cerebrospinal fluid, magnetic resonance imaging and angiography of the brain, were normal. Our impression was left oculomotor and trochlear nerve neuritis secondary to botulinum toxin injection. He was started on oral prednisolone 1 mg/kg daily and tapered by 5 mg per week. His condition improved gradually with no residual ptosis or anisocoria after three months. Extraocular movements normalised except for minimal residual restriction on depression. CONCLUSION: Oculomotor and trochlear nerve neuritis can occur following botulinum toxin injection over the masseter area. Healthcare professionals should be aware of this potential complication before offering the injection.

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).

Prevalence of Dark without Pressure and Angioid Streaks in Sickle Cell Disease.

BACKGROUND AND OBJECTIVES: To investigate the prevalence of areas of dark without pressure (DWOP) and angioid streaks (AS) in patients with sickle cell disease (SCD). PATIENTS AND METHODS: This was a consecutive series of 77 adults with SCD. RESULTS: DWOP appeared as multiple patches in 35 of the affected eyes and as a single lesion in 3 eyes. OCT finding demonstrated the ellipsoid layer was hyporeflective in DWOP. AS were identified in six cases (3.9%) and were bilateral in five cases. The prevalence of AS was higher with increasing age, being 67% in the patients older than age 45 years. CONCLUSION: The prevalence of DWOP in adults with SCD is 25% in this study, which is higher than previously reported, and the prevalence of AS is around 4%, which is midway between prior estimates. Recognition of the clinical examination and imaging features of DWOP reduce the need for additional investigation. [Ophthalmic Surg Lasers Imaging Retina. 2021;52:620-622.].

"Mummy, my eyelids are heavy": A case series of juvenile myasthenia gravis.

PURPOSE: To report three cases of juvenile myasthenia gravis aged between 18 and 24 months with ocular symptoms as their first presentation. METHOD: A case series. RESULTS: We present a case series of juvenile myasthenia gravis in a tertiary centre in Malaysia. Two of the three cases consist of a pair of twins who presented with ptosis of bilateral eyes; the first twin presented 4 months later than the second twin. These two cases were positive for anti-acetylcholine receptor antibodies and had generalized myasthenia gravis, whereas the other case was negative for receptor antibodies and was purely ocular myasthenia gravis. CONCLUSION: Juvenile myasthenia gravis is relatively rare in toddlers. Early diagnosis and commencement of treatment is important to slow the progression of the disease and avoiding life-threatening events.

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.

Ointment tacrolimus for steroid resistant adenoviral nummular keratitis

@#A 33-year-old man presented with a four-day history of redness and blurring of vision of the right eye. A clinical diagnosis of adenoviral keratitis was made with a differential of microsporidia epithelial keratitis. The patient subsequently developed nummular keratitis which was resistant to topical steroids. He continued to develop multiple recurrences of the condition. Treatment with tacrolimus ointment was started as the patient had an elevated intraocular pressure due to prolonged steroid use. Tacrolimus ointment showed a favourable outcome in the management of recurrent nummular keratitis.