Godtfredsen syndrome - recurrent clival chondrosarcoma with 6 years follow up: a case report and literature review.

BACKGROUND: We report a rare case of Godtfredsen syndrome caused by clival chondrosarcoma and perform a review of literatures. This article also explains the clinico-anatomical correlation of this rare neurological syndrome. CASE PRESENTATION: A 22-year-old gentleman presented with binocular diplopia. Clinical examination revealed an isolated right abducent nerve and right hypoglossal nerve palsy, with other cranial nerves intact. Neuroimaging revealed a right clival mass. Supraorbital craniotomy and tumour debulking were done in the same year. Histopathological examination showed low-grade chondrosarcoma. After 5-years of default, he came back with the tumour enlarged. He underwent a right orbitozygomatic craniotomy and tumour excision with 33 cycles of radiotherapy. Despite two surgeries and radiotherapy, the abducent nerve and hypoglossal nerve did not improve throughout 6 years of follow-up. Cranial nerve VI palsy is not always a false localizing sign, in Godtfredsen syndrome it serves as a localizing sign. CONCLUSION: To the best of our knowledge, this is the first case report of Godtfredsen Syndrome secondary to clival chondrosarcoma. Cranial nerve VI and XII palsy with no involvement of other cranial nerves, most likely the pathology is located at the clivus.

Enriching traditional didactic teaching in undergraduate ophthalmology with lateral thinking method: a prospective study.

PURPOSE: To evaluate undergraduate medical students' perspective on lateral thinking pedagogy in teaching clinical ocular anatomy in correlation to developing differential diagnoses and recognising red flags in managing common eye conditions. METHODS: The prospective study compared the lateral thinking method (LTM) versus the traditional didactic method in teaching clinical ocular anatomy. Two hundred seventy-two medical students who underwent ophthalmology clerkships were recruited over 3 years. They were randomised into two groups, the LTM and regular didactic groups. Students participated in pre and post-tests to assess their theoretical clinical ophthalmic knowledge, and their responses to respective teaching methods were measured via the self-assessment questionnaire (SAQ), which incorporated a five-point Likert-type scale. RESULTS: Overall, the LTM group scored significantly higher than the control group, and they found the innovative teaching method improved their confidence and awareness in theoretical knowledge in generating differential diagnoses, managing common eye conditions and recognising potential signs that could be sight and/or life-threatening compared to the regular teaching group (P < 0.05). However, all students were neutral towards both lectures regarding changing their negative perception of the current ophthalmic curriculum. CONCLUSION: From the student's perspective, LTM is an effective tool in enriching regular teaching. The method encouraged versatile thinking patterns while enhancing the effectiveness of learning experience in time and resource-limited undergraduate ophthalmic teaching.

A rare case of unilateral Cogan's anterior internuclear ophthalmoplegia, upgaze palsy and ataxia caused by dorsal tegmentum lesion at pontomesencephalic junction.

BACKGROUND: Cogan's anterior internuclear ophthalmoplegia (INO) is characterized by INO with inability to converge and commonly thought to be due to rostral midbrain lesion. A lesion outside midbrain that causes unilateral Cogan's anterior INO combined with upgaze palsy and ataxia are rarely described. CASE PRESENTATION: A 67-year old male presented with left Cogan's anterior internuclear ophthalmoplegia (INO), left appendicular ataxia and bilateral upgaze palsy. A Magnetic Resonance Imaging (MRI) and Magnetic Resonance Angiography (MRA) brain showed a left dorsal tegmental infarct at the level of pontomesencephalic junction. CONCLUSIONS: This case highlights the clinical importance of Cogan's anterior INO in combination with upgaze palsy and ataxia, and report possible site of lesion in patients with such constellation. Clinicians should consider looking for cerebellar signs in cases of Cogan's anterior INO, apart from just considering localizing the lesion at the midbrain.

The Effect of Socio-Economic Status on Severity of Periocular Basal Cell Carcinoma at Presentation.

PURPOSE: To evaluate the influence of socio-economic factors on size of periocular basal cell carcinoma at presentation. METHODS: All periocular basal cell carcinoma cases receiving treatment from the oculoplastics team in South Glasgow Hospitals NHS Trust, Glasgow, between 1999 and 2009, were identified retrospectively. Information collected included demographic details of patients, side and site of lesions, type of lesions, and size of lesions. The size of lesion was defined as small for any dimension not exceeding 5 mm, medium for dimensions between 6 mm and 10 mm, and large for dimensions exceeding 11 mm. Home address was used to determine the Scottish Index of Multiple Deprivation rank. The demographics, size of lesion, and Scottish Index of Multiple Deprivation rank were investigated using the general linear regression modelling. RESULTS: Of the 67 cases, 24 were men and 43 were women. The mean age was 71.5 years. There were a total of 67 identified cases, of which 38 presented with small-size lesions, 24 with medium-size lesions, and 5 with large-size lesions. Scottish Index of Multiple Deprivation is related to the presenting incidence of basal cell carcinoma, with the lower ranks presenting more frequently. CONCLUSIONS: Socio-economic deprivation is associated with larger and more frequent presentation of periocular basal cell carcinoma. This highlights the importance of raising awareness among populations of the more deprived areas of the significance of lumps and bumps within the periocular regions.

A review of drug-induced acute angle closure glaucoma for non-ophthalmologists.

Acute angle closure glaucoma is an ophthalmic emergency and can lead to blindness if left untreated. Several types of drugs have the potential to precipitate acute angle closure glaucoma. These include adrenergic, cholinergic and anticholinergic, antidepressants, anticoagulants and sulfa-based agents. This article provides a basic overview of the risk factors and pathophysiologic mechanisms involved in angle closure glaucoma and focuses on drug-induced angle closure glaucoma for the non-ophthalmologist. A PubMed search limited to the English language was conducted to find relevant literature for the purpose of this article. Most attacks occur in subjects unaware that they are at risk due to innately narrow iridocorneal angles. Clinicians should always review medications in patients presenting with symptoms of acute angle closure glaucoma. The aim of this article is to bring this ophthalmic condition to the attention of clinicians, particularly those outside the field of ophthalmology who commonly prescribe these medications or see these patients prior to referring to ophthalmologists.

Development of 3D-printed universal adapter in enhancing retinal imaging accessibility.

BACKGROUND: The revolutionary technology of smartphone-based retinal imaging has been consistently improving over the years. Smartphone-based retinal image acquisition devices are designed to be portable, easy to use, and cost-efficient, which enables eye care to be more widely accessible especially in geographically remote areas. This enables early disease detection for those who are in low- and middle- income population or just in general has very limited access to eye care. This study investigates the limitation of smartphone compatibility of existing smartphone-based retinal image acquisition devices. Additionally, this study aims to propose a universal adapter design that is usable with an existing smartphone-based retinal image acquisition device known as the PanOptic ophthalmoscope. This study also aims to simulate the reliability, validity, and performance overall of the developed prototype. METHODS: A literature review has been conducted that identifies the limitation of smartphone compatibility among existing smartphone-based retinal image acquisition devices. Designing and modeling of proposed adapter were performed using the software AutoCAD 3D. For the proposed performance evaluation, finite element analysis (FEA) in the software Autodesk Inventor and 5-point scale method were demonstrated. RESULTS: Published studies demonstrate that most of the existing smartphone-based retinal imaging devices have compatibility limited to specific older smartphone models. This highlights the benefit of a universal adapter in broadening the usability of existing smartphone-based retinal image acquisition devices. A functional universal adapter design has been developed that demonstrates its compatibility with a variety of smartphones regardless of the smartphone dimension or the position of the smartphone's camera lens. The proposed performance evaluation method generates an efficient stress analysis of the proposed adapter design. The end-user survey results show a positive overall performance of the developed universal adapter. However, a significant difference between the expert's views on the developed adapter and the quality of images is observed. CONCLUSION: The compatibility of existing smartphone-based retinal imaging devices is still mostly limited to specific smartphone models. Besides this, the concept of a universal and suitable adapter for retinal imaging using the PanOptic ophthalmoscope was presented and validated in this paper. This work provides a platform for future development of smartphone-based ophthalmoscope that is universal.

Eight-and-a-half syndrome as the first presentation of multiple sclerosis in an Asian male: a case report.

BACKGROUND: Multiple sclerosis is a diffuse chronic demyelinating disease of the central nervous system. It is relatively uncommon in the Asian population and even more so in males. Despite the usual involvement of the brainstem, eight-and-a-half syndrome remains a rare first presentation in multiple sclerosis. Only a few cases have been reported previously, but none involving the Asian population. Eight-and-a-half syndrome, a neuro-ophthalmological condition, is characterized by one-and-a-half syndrome with ipsilateral lower facial nerve palsy, which localizes lesions to the pontine tegmentum. This case report demonstrates the first case of eight-and-a-half syndrome as the first presentation of multiple sclerosis in an Asian male. CASE PRESENTATION: A healthy 23-year-old Asian man presented with sudden onset of diplopia followed by left-sided facial asymmetry for 3 days. Assessment of extraocular movement revealed left conjugate horizontal gaze palsy. On right gaze, there was limited left eye adduction and horizontal nystagmus of the right eye. These findings were consistent with a left-sided one-and-a-half syndrome. Prism cover test revealed left esotropia of 30 prism diopters. Cranial nerve examination showed left lower motor neuron facial nerve palsy, while other neurological examination was normal. Magnetic resonance imaging brain showed multifocal T2 fluid attenuated inversion recovery hyperintense lesions, involving bilateral periventricular, juxtacortical, and infratentorial regions. A focal gadolinium contrast-enhanced lesion with open ring sign on T1 sequence was seen at the left frontal juxtacortical region. Multiple sclerosis was diagnosed on the basis of the clinical and radiological evidence, which fulfilled the 2017 McDonald criteria. Positive oligoclonal bands in cerebrospinal fluid analysis further confirmed our diagnosis. He had a complete resolution of symptoms 1 month after a course of pulsed corticosteroid therapy, and was subsequently placed on maintenance therapy with interferon beta-1a. CONCLUSION: This case illustrates eight-and-a-half syndrome as the first presentation of a diffuse central nervous system pathology. A wide range of differential diagnoses needs to be considered in such a presentation as based on the patient's demographics and risk factors.

Traumatic Orbital Emphysema Following Blunt Trauma and Nose Blowing.

Orbital emphysema commonly resolves with no morbidity. However, sight-threatening complications, such as central retinal artery occlusion and ischemic optic neuropathy, may occur, which can result in poor visual outcomes. Plain skull X-ray, which is widely available, is a useful tool in identifying orbital emphysema. We report a case of a 29-year-old gentleman with underlying allergic rhinitis who presented with a painless, progressively increasing periorbital swelling of the right eye, which was aggravated by nose blowing. He had a history of blunt trauma one day prior to the presentation. Visual acuity was unaffected and optic nerve function tests were unremarkable. There was right upper lid swelling with crepitations, right hypoglobus with restricted upward gaze movement, and right conjunctival injection. Intraocular pressure was within normal limits. The posterior segment examination was unremarkable. A plain skull radiograph revealed a "black eyebrow sign" over the right orbit with no obvious orbital wall fracture. Computed tomography of the orbit showed focal indentation over the right lamina papyracea with superior orbito-palpebral emphysema. Systemic antibiotics, steroid nasal spray, and oral antihistamines were initiated with the prohibition of nose blowing. On post-trauma day five, he made an uneventful recovery. High clinical suspicion and thorough clinical examination with the aid of a plain skull radiograph can diagnose orbital emphysema in order for prompt referral to be undertaken to prevent morbidity. Clinicians should consider orbital emphysema as a differential diagnosis for periorbital swelling, especially if there was a preceding trauma.

Preferred cataract surgery practices in Malaysia: a survey based study.

BACKGROUND: To describe the preferred cataract surgery practices among Malaysian ophthalmologists and medical officers in comparison with the recommended practices. METHODS: An online questionnaire was sent out in April 2021, to Malaysian Ophthalmologists and medical officers who perform cataract surgeries. The questions were focused on the preferred cataract surgery practices of the participants. All data obtained were collected, tabulated and analysed. RESULTS: A total of 173 participants responded to the online questionnaire. 55% of the participants were within 31-40 years of age. 56.1% preferred peristaltic pump over venturi system. 91.3% of participants practised povidone iodine instillation to the conjunctival sac. With regards to the main wound incision, more than half of the surgeons (50.3%) preferred fixed superior incision and 72.3% of them preferred 2.75 mm microkeratome blade. Most of the participants (63%) were inclined towards C-Loop clear intraocular lens (IOL) with a single-handed push preloaded system. 78.6% of the surgeons routinely use carbachol in their cataract surgery. CONCLUSIONS: This survey provides some insight into the current practices among Malaysian ophthalmologists. Most of the practices are in line with international guidelines for preventing postoperative endophthalmitis. This article could help trainees and ophthalmologists benchmark and observe the common cataract surgery practices among their seniors and peers in Malaysia.

Diplopia following sub-tenon's anaesthesia: an unusual complication.

Diplopia is a rare but well recognised complication following retrobulbar and peribulbar local anaesthesia but it has not been widely reported following sub-tenon's local anaesthesia (STLA). We report on a 76-year-old woman who developed vertical diplopia after left phacoemulsification. She had received a STLA. She had left hypotropia measuring 30 prism diopters for near and distance. She was managed with occlusion but there was no improvement in her findings over 6 months. Ocular motility opinion was then sought and a presumptive diagnosis of inferior rectus fibrosis was made. She subsequently underwent a left inferior rectus recession using adjustable sutures. Postoperatively she had a residual left hypotropia measuring 8 prism dioptres and single vision. Possible causes of inferior rectus fibrosis include muscle damage during traumatic sub-tenon's block or myotoxicity due to local anaesthetic agents. This case highlights the importance of close supervision of inexperienced staff administering regional anaesthetics.

Familial exudative vitreoretinopathy in a 4 generations family of South-East Asian Descendent with FZD4 mutation (c.1501_1502del).

BACKGROUND: Familial Exudative Vitreoretinopathy (FEVR) is a hereditary disorder characterized by peripheral avascular retina with neovascularization. Although FEVR has been thoroughly described in multiple literature publications from different countries, there are currently limited articles describing the phenotypes of FEVR among South-East Asian Descendent. This paper describes the clinical phenotype of the FZD4 gene with c.1501_1502 deletion in a 4-generation case series of a South East Asian family. METHODS: We reviewed a 4-generation case series of a South-East Asian descendent family consisting of 27 family members with 10 members diagnosed with FEVR. We observed the clinical phenotype of these series of patients, including some of the family members who underwent whole-exome sequencing, PCR amplification and DNA sequencing techniques to identify the mutated gene. RESULTS: Frameshift mutation (c.1501_1502del) were found in FZD4 gene in this series of patients with the age ranging from 1 month old to 69 years old. There was a 100% (4/4) of our paediatric patients being diagnosed within 21 days of life. It was also found that 75% of patients (6/8) less than 40 years old exhibited disease asymmetry of 2 stages or more and 80% (8/10) had a history of vitreoretinal surgery or diode laser photocoagulation, with a further 50% of the adult patients identified as legally blind; the mean age of blindness was 18-years-old. CONCLUSIONS: Phenotypic manifestation of FZD4 gene with c.1501_1502del mutation can be identified within the neonatal period. They have relatively greater clinical asymmetry of 2 stages or more compared to other mutations. Without treatment, most of them will have bilateral severe visual impairment around the adolescent age group.

Acute pulmonary histoplasmosis in a patient with uveitis after infliximab therapy.

The purpose of this study is to report a case of disseminated histoplasmosis in a patient with uveitis, after treatment with infliximab. The method employed in this study is single case report. Infliximab can be useful in controlling idiopathic uveitis, but can give rise to disseminated histoplamosis, especially in patients living in geographic areas where histoplasmosis is endemic. Clinicians should be aware of the possibility of rapid onset histoplasmosis in patients receiving anti-tumor necrosis factor agents. In such cases, these agents should be immediately stopped, investigations performed, and appropriate treatment started.

Update on surgical management of complex macular holes: a review.

Modern surgical interventions effectively treat macular holes (MHs) more than 90%. Current surgical treatment for MHs is pars plana vitrectomy with epiretinal membrane, internal limiting membrane (ILM) peeling, gas endotamponade, and prone posturing postoperatively. However, a small subset of MHs imposes challenges to surgeons and frustrations on patients. A narrative review was performed on the surgical treatment of challenging MHs including large and extra-large MHs, myopic MHs with or without retinal detachment, and chronic and refractory MHs. There are robust data supporting inverted ILM flap as the first-line treatment for large idiopathic MHs and certain secondary MHs including myopic MHs. In addition, several studies had shown that ILM flap manipulations in combination with surgical adjuncts increase surgical success, especially in difficult MHs. Even in eyes with limited ILM, surgical options included autologous retinal graft, human amniotic membrane, and creation of a distal ILM flap that can assist in MH closure even though the functional outcome may be affected by the MH chronicity. Despite relative success anatomically and visually after each technique, most techniques require a long-term study to analyze their safety profile and to establish any morphological changes of the MH plug in the closed MHs.

Human coronaviruses: ophthalmic manifestations.

The 2019 novel coronavirus which causes severe acute respiratory syndrome (SARS) known as SARS-CoV-2 still remains as a global pandemic since its discovery and continues to spread across the world, given how highly contagious the virus is. We reviewed various articles that explore eye involvement in COVID-19 and other human coronaviruses, its human manifestations in comparison to animal studies and potential mechanism of viral entry into the eye surface. Evidence of animal studies depicted various complications of coronaviruses infection into the eyes, in both anterior and posterior segments of the eye. Conjunctival inflammation remains uncommon in association with COVID-19, with other ophthalmic findings. The risk of transmission via the ocular surface remains likely low, though it is inarguably present based on preliminary finding of viral load in ocular samples and expression of ACE2 on the ocular surface. Testing the tears sample for diagnosing SARS-CoV-2 was unreliable due to limitations of the testing kits and conflicting evidence of the viral titre in the ocular samples. Further larger, more precise and specific studies are required to allow us to better understand the pattern of virulence underlying the associations of SARS-CoV-2 in the eye despite its rare occurrence. This review article aims to enhance better awareness among clinicians regarding ocular manifestations associated with COVID-19 and necessary precautions should be implemented to minimise the risk of person-to-person especially in the nosocomial setting.

Iatrogenic intralenticular broken steroid implant: a case report.

BACKGROUND: This is a case report of an iatrogenic intralenticular broken steroid (Ozurdex™) implant in a patient with uveitis. There are only a few case reports on broken Ozurdex™ implants in the vitreous cavity, with none of them involving the crystalline lens. A few authors have described the accidental injection of an Ozurdex™ implant into the crystalline lens, but all of the implants remained in one piece in the lens and none of them were broken. We report an unusual case of an Ozurdex™ implant which was injected inadvertently into the crystalline lens, resulting in a broken Ozurdex™ implant with an entry and exit wound through the posterior capsule of the lens. CASE PRESENTATION: An ophthalmic trainee performed an Ozurdex™ intravitreal injection into a 48-year-old Asian man's right eye under aseptic conditions. This patient was then followed up for further management. On day 7 post-procedure, a slit lamp examination revealed that the Ozurdex™ implant was injected into the intralenticular structure of his right eye and had fractured into two pieces. The posterior capsule of the right lens was breached, with one half of the Ozurdex™ implant stuck at the entry and the other stuck at the exit wound of the posterior capsule. This patient underwent right eye cataract extraction and repositioning of the fractured implant; he made an uneventful recovery. CONCLUSIONS: Ophthalmologists should be aware of the potential risk of injecting an Ozurdex™ implant into an anatomical structure other than the vitreous cavity. Adequate training and careful administration of the Ozurdex™ implant are necessary to avoid such a complication, which fortunately is rare.

Comparison of Icare Rebound Tonometer and Perkins Applanation Tonometer in Community Eye Screening.

PURPOSE: To compare the measurement of intraocular pressure (IOP) of Icare rebound tonometer and Perkins applanation tonometer (PAT) during community eye screening and to assess the agreement between these 2 instruments. DESIGN: A cross-sectional, non-interventional study. METHODS: The IOP measurements by handheld Icare rebound tonometer (Finland) were first performed by a primary care physician. Then the IOP was measured using Perkins Mk3 applanation tonometer (Haag-Streit, UK) by an ophthalmologist who was masked to previous readings from the Icare rebound tonometer. The mean IOP measured by each tonometer was compared. Pearson correlation coefficient was used to explore the correlation between the IOP measurements of the 2 instruments. The level of agreement between them was assessed using the Bland and Altman method. RESULTS: A total of 420 left eyes were examined. The mean age of subjects was 38.6 ± 18.2 years. Approximately 67% of subjects were female. The mean IOP was 16.3 ± 4.0 mm Hg using Icare and 13.4 ± 2.3 mm Hg using PAT. Pearson correlation coefficient showed a moderate positive correlation between the 2 methods (r = +0.524, P < 0.001). Linear regression analysis revealed a slope of 0.28 with R² of 0.255. The mean difference between the 2 methods was 2.90 ± 3.5 mm Hg and the sample t-test revealed a statistically significant mean difference from 0 (P < 0.001). The 95% limits of agreement between the 2 methods were between -9.73 and 3.93 mm Hg. CONCLUSIONS: The handheld Icare rebound tonometer is a reasonably acceptable screening tool in community practices. However, Icare overestimated IOP with a mean of 2.90 mm Hg higher than the PAT. Thus, using Goldmann applanation tonometer as a confirmatory measurement tool of IOP is suggested.

The incidence of acute angle closure in Scotland: a prospective surveillance study.

PURPOSE: To estimate the incidence, and describe the clinical features and short-term clinical outcomes of acute angle closure (AAC). METHODS: Patients with newly diagnosed AAC were identified prospectively over a 12-month period (November 2011 to October 2012) by active surveillance through the Scottish Ophthalmic Surveillance Unit reporting system. Data were collected at case identification and at 6 months follow-up. RESULTS: There were 114 cases (108 patients) reported, giving an annual incidence of 2.2 cases (95% CI 1.8 to 2.6) or 2 patients (95% CI 1.7 to 2.4) per 1 00 000 in the whole population in Scotland. Precipitating factors were identified in 40% of cases. Almost one in five cases was associated with topical dilating drops. Best-corrected visual acuity (BCVA) at presentation ranged from 6/6 to perception of light. The mean presenting intraocular pressure (IOP) was 52 mm Hg (SD 11). Almost 30% cases had a delayed presentation of 3 or more days. At 6 months follow-up, 75% had BCVA of 6/12 or better and 30% were found to have glaucoma at follow-up. Delayed presentation (≥3 days) was associated with higher rate of glaucoma at follow-up (22.6% vs 60.8%, p<0.001), worse VA (0.34 vs 0.74 LogMAR, p<0.0001) and need for more topical medication (0.52 vs 1.2, p=0.003) to control IOP. CONCLUSION: The incidence of AAC in Scotland is relatively low compared with the Far East countries, but in line with previous European data. Almost one in five cases were associated with pupil dilation for retinal examination.