Recurrent Optic Neuritis in Paediatric Anti-N-Methyl-D-Aspartate Receptor (Anti-NMDAR) Encephalitis: Case Report and Literature Review.

Optic neuritis associated with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis poses a novel challenge and is recognised as being part of overlapping antibody-associated demyelinating syndromes. We report a case of a 10-year-old girl who first presented with anti-NMDAR encephalitis and later developed four episodes of left optic neuritis. Blurring of vision, pain on eye movement, and headache were the striking features in all episodes of optic neuritis. For each recurrent episode of optic neuritis she was treated with intravenous methylprednisolone, following which visual acuity, colour vision, and visual field improved to normal. Neuroimaging was unremarkable. Myelin oligodendrocyte glycoprotein and aquaporin-4 antibody tests were negative. She developed a relapse of anti-NMDAR encephalitis after recurrent episodes of optic neuritis despite being on azathioprine. Intravenous immunoglobulin and cycles of cyclophosphamide were started, and she has been free from optic neuritis and encephalitis for more than one year since. Optic neuritis and anti-NMDAR encephalitis are reversible with intensive immunotherapy. Early and accurate diagnosis can lead to better management of this condition.

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.

A Simple Bone Cyst Requiring Exenteration: A Case Report With Literature Review.

Simple bone cyst is a benign osteolytic lesion, found primarily in the mandible, occasionally in the maxilla, and rarely in the zygoma. A 17-year-old male patient with 6-month history of left facial mass presented with worsening proptosis and displacement of left eyeball associated with reduced OS vision. The lesion was painless to start with, but with time, he reported OS pain. There was no history of any trauma. CT scan and MRI of orbit performed revealed cystic bony lesion involving greater wing of sphenoid. He subsequently had excision of the left facial mass with exenteration of OS due to poor visual prognosis, followed by reconstruction with osteomyocutaneous flap from left scapular region. The histopathologic features were consistent with a diagnosis of simple bone cyst of zygomatic arch.

Subfoveal Choroidal Thickness in Pre-eclampsia.

SIGNIFICANCE: To our knowledge, this is the first time a study looking at the association between subfoveal choroidal thickness (SFCT) and the parameters affecting the severity of pre-eclampsia has been reported. We provide evidence that SFCT is thicker in pre-eclamptic (PE) patients and speculate on the possible causes. PURPOSE: The purposes of this study were to compare SFCT in PE, normal pregnant, and nonpregnant women using spectral domain optical coherence tomography and to correlate SFCT with severity of pre-eclampsia. METHODS: A cross-sectional, observational study was performed. A total of 150 participants were divided into three groups: group 1 (50 PE women), group 2 (50 normal pregnant women), and group 3 (50 nonpregnant healthy women). Subfoveal choroidal thickness was measured using spectral domain optical coherence tomography. Other parameters including mean arterial blood pressure (MABP), central corneal thickness, macular thickness, IOP, ocular perfusion pressure (OPP), and urine protein-to-creatinine ratio were also measured. ANOVA and Pearson correlation analysis were used to look at differences between the groups. P < .05 was considered as statistically significant. RESULTS: The MABP was higher in group 1 than in groups 2 and 3 (103.0 ± 12.9 vs. 83.2 ± 9.8 vs. 89.5 ± 7.2 mmHg, respectively; all P < .001). The SFCT of the PE group was higher than in groups 2 and 3 (370.7 ± 23.8 vs. 344.5 ± 30.8 vs. 315.8 ± 49.9 µm, respectively; all P < .001). There were no statistically significant differences in central corneal thickness, macular thickness, or IOP between the PE and healthy pregnant groups (all P > .05). The OPP was greater in PE patients (52.8 ± 8.5 vs. 41.9 ± 6.9 vs. 43.4 ± 5.2 mmHg, respectively; both P < .001). The SFCT was positively correlated with MABP (r = 0.464, P < .001), OPP (r = 0.495, P < .001), and urine protein-to-creatinine ratio (r = 0.635, P < .001) in the PE group. CONCLUSIONS: Subfoveal choroidal thickness is higher in pre-eclampsia and is proportional to established markers of severity of the condition. This parameter might serve as a novel predictive marker for the severity of pre-eclampsia.

Complete recovery of filler-induced visual loss following subcutaneous hyaluronidase injection.

The rise in popularity of hyaluronic acid (HA) dermal filler injection has caused an exceptional increase in the number of cases of reported irreversible blindness. Here, we reported a case of ischemic optic neuropathy and ophthalmoplegia following subcutaneous HA filler injection with complete visual recovery. A 31-year-old Chinese woman presented with sudden onset of right monocular visual impairment associated with diplopia. Patient had received a hyaluronic acid-containing filler injection for nasal dorsum augmentation twelve hours prior to presentation. Visual acuity of the right eye was counting finger. A right relative afferent pupillary defect was demonstrated with ophthalmoplegia. Humphrey visual field test disclosed a right inferior altitudinal field defect with impairment of colour vision. Computed tomography of the orbit revealed mild enlargement of the right medial and inferior recti muscles. Our patient showed a tremendous improvement of vision after a subcutaneous hyaluronidase injection with complete visual recovery within 2 weeks.

Orbitofacial dermatofibrosarcoma protuberans with intranasal extension.

A 25-year-old Chinese woman presented with recurrent painless swelling over the left medial canthus region for 3 months and intranasal mass for an indeterminate duration. Initial incision biopsy of the mass was reported as nodular fasciitis but the lesion recurred 3 weeks later. Intraoperative findings during repeat biopsy showed a mass extending from the deep dermal tissue into the anterior orbit and polyp-like nasal mass. Histopathology findings were that of dermatofibrosarcoma protuberans (DFSP). The mass recurred 4 months later without orbital or intranasal recurrence. Wide excision biopsy under frozen section guidance was attempted however; clear surgical margins could not be achieved despite extensive resection. She was subsequently referred for adjuvant radiotherapy. We report an exceptionally rare case of local recurrence of DFSP in an unusual anatomic location. This case was surgically challenging in achieving negative margins, and thus neoadjuvant therapy may improve overall outcome to prevent local relapse.

Refractive outcomes comparison between the Lenstar LS 900® optical biometry and immersion A-scan ultrasound.

To determine the accuracy of intraocular lens (IOL) calculations in eyes undergoing phacoemulsification cataract surgery with IOL implantation using immersion A-scan ultrasound (US) and Lenstar LS 900(®) biometry. In this prospective study, 200 eyes of 200 patients were randomized to undergo either Lenstar LS 900(®) or immersion A-scan US biometry to determine the IOL dioptric power prior to phacoemulsification cataract surgery. Post-operative refractive outcomes of these two groups of patients were compared. The result showed no significant difference between the target spherical equivalent (SE) and the post-operative SE value by the Lenstar LS 900(®) (p value = 0.632) or immersion A-scan US biometry (p value = 0.438) devices. The magnitude of difference between the two biometric devices were not significantly different (p value = 0.868). There was no significant difference in the predicted post-operative refractive outcome between immersion A-scan US biometry and Lenstar LS 900(®). Based on the results, the immersion A-scan US technique is as accurate as Lenstar LS 900(®) in the hands of an experienced operator.

Diagnostic challenge: Diffuse large B-cell lymphoma masquerading as orbital cellulitis.

We report a clinical case of orbital lymphoma masquerading as orbital cellulitis. Orbital lymphoma and lymphoma of the orbital adnexa are relatively rare types of lymphoma. They represent the malignant end of a spectrum of lymphoproliferative lesions that occur in the orbit, conjunctiva and eyelid. Diffuse large B-cell lymphomas (DLBCL) with variable clinical presentations have a relatively poor prognosis with rapid visual loss. Therefore, thorough evaluation and assessment with a multi-specialty effort are warranted for earlier diagnosis and initiation of treatment to save life and prevent sight loss.

Anophthalmic socket in retinoblastoma: Exploring complications and risk factors in a tertiary centre in Malaysia.

PURPOSE: To evaluate the complications of anophthalmic socket in retinoblastoma patients at a tertiary centre in Malaysia. DESIGN: Retrospective study. METHODS: Patients who underwent enucleation for retinoblastoma were reviewed from 2004-2020. Details were recorded, including demographics, diagnosis, surgical techniques, implant types, additional therapies, and complications. RESULTS: Of 250 patients with retinoblastoma managed over the period, the anophthalmic sockets of 160 eyes who underwent enucleation were analysed. The mean age at enucleation was 2.03 years (26 days to 9.18 years). The follow-up periods after enucleation range from 5 days to 16.83 years. Porous polyethylene (Medpor) implants were used in 135 patients (84.4%), as were Bioceramic in 9, glass balls in 7, acrylic in 7, dermis fat grafts in 1, and silicone implants (Aurosphere) in 1. The overall complications in our study were 28.8%. Complications seen in the study included implant exposure (12.5%), shallow inferior fornix (10.6%), granuloma formation (3.1%), discharge (2.5%), implant migration (1.9%), ptosis (0.6%), and orbital dystopia (0.6%). Implant exposure is solely found in Medpor, more common in those with donor sclera caps, and exposure times range from 28 days to 11.42 years. The suturing of the Tenon and conjunctiva in separate layers significantly reduced the rate of implant exposure. Six out of seven radiation patients had shallow inferior fornixes. CONCLUSIONS: Long-term post-enucleation complications were not uncommon. Luckily, most had good outcomes, with a few needing surgical intervention. Meticulous suturing technique on the Tenon and conjunctival layer is essential to prevent implant exposure.

Isolated third nerve palsy: Lessons from the literature and 4 case studies.

A patient's age, clinical presentation, medical history, and circumstances at time of palsy onset suggest likely underlying causes and help prioritize choice of imaging.

Ocular Manifestations of Ectrodactyly-Ectodermal Dysplasia-Cleft Palate (EEC) Syndrome: A Case Report.

Ocular manifestations are common associations of ectrodactyly-Ectodermal dysplasia-cleft palate (EEC) syndrome. We would like to report a case of a 48-year-old patient with EEC syndrome who manifested ocular and extraocular signs and symptoms. The ophthalmic findings in this patient included chronic blepharitis and absence of meibomian gland. There was also a presence of hazy cornea with vascularized corneal stroma and symblepharon involving the lower lid. Systemic conditions showed generalized dry and scaly skin with hand-foot split deformity. Therefore, ophthalmologists should be alert to spot and diagnose this condition as prompt treatment should be commenced considering this can be sight-threatening.

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.

Co-occurrence of acute ophthalmoplegia (without ataxia) and idiopathic intracranial hypertension.

BACKGROUND: Acute ophthalmoparesis without ataxia was designated as 'atypical Miller Fisher syndrome' as it presents with progressive, relatively symmetrical ophthalmoplegia, but without ataxia nor limb weakness, in the presence of anti-GQ1b antibody. Idiopathic intracranial hypertension is characterized by signs of raised intracranial pressure occurring in the absence of cerebral pathology, with normal composition of cerebrospinal fluid and a raised opening pressure of more than 20 cmH2O during lumbar puncture. We aim to report a rare case of acute ophthalmoplegia with co-occurrence of raised intracranial pressure. CASE DESCRIPTION: A 28-year-old gentleman with body mass index of 34.3 was referred to us for management of double vision of 2 weeks duration. His symptom started after a brief episode of upper respiratory tract infection. His best corrected visual acuity was 6/6 OU. He had bilateral sixth nerve palsy worse on the left eye and bilateral hypometric saccade. His deep tendon reflexes were found to be hyporeflexic in all four limbs. No sensory or motor power deficit was detected, and his gait was normal. Plantar reflexes were downwards bilaterally and cerebellar examination was normal. Both optic discs developed hyperaemia and swelling. Magnetic resonance imaging of brain was normal and lumbar puncture revealed an opening pressure of 50 cmH2O. Anti-GQ1b IgG and anti-GT1a IgG antibody were tested positive. CONCLUSION: Acute ophthalmoparesis without ataxia can present with co-occurrence of raised intracranial pressure. It is important to have a full fundoscopic assessment to look for papilloedema in patients presenting with Miller Fisher syndrome or acute ophthalmoparesis without ataxia.

Baseline Central Visual Field Defect as a Risk Factor For NTG Progression: A 5-Year Prospective Study.

PRECIS: This 5-year follow-up study on normal-tension glaucoma (NTG) patients demonstrated that those with baseline central visual field (VF) defect progress at a more increased rate compared with those with peripheral field defect. PURPOSE: The purpose of this study was to investigate the clinical characteristics, including 24-hour ocular perfusion pressure and risk of progression in patients with baseline central VF defect, as compared with those with peripheral VF defect in NTG. DESIGN: This was a prospective, longitudinal study. METHODS: A total of 65 NTG patients who completed 5 years of follow-up were included in this study. All the enrolled patients underwent baseline 24-hour intraocular pressure and blood pressure monitoring via 2-hourly measurements in their habitual position and had ≥5 reliable VF tests during the 5-year follow-up. Patients were assigned to two groups on the basis of VF defect locations at baseline, the central 10 degrees, and the peripheral 10- to 24-degree area. Modified Anderson criteria were used to assess global VF progression over 5 years. Kaplan-Meier analyses were used to compare the elapsed time of confirmed VF progression in the two groups. Hazard ratios for the association between clinical risk factors and VF progression were obtained by using Cox proportional hazards models. RESULTS: There were no significant differences between the patients with baseline central and peripheral VF defects in terms of demography, clinical, ocular and systemic hemodynamic factors. Eyes with baseline defects involving the central fields progressed faster (difference: ßcentral=-0.78 dB/y, 95% confidence interval=-0.22 to -1.33, P=0.007) and have 3.56 times higher hazard of progressing (95% confidence interval=1.17-10.82, P=0.025) than those with only peripheral defects. CONCLUSION: NTG patients with baseline central VF involvement are at increased risk of progression compared with those with peripheral VF defect.

Endoscopic transnasal removal of an intraconal foreign body using an image-guided surgical system.

Foreign bodies lodged in the intraconal space of the orbit pose a surgical challenge due to its deep location behind the globe. Image-guided endoscopic transnasal surgery facilitates the localization of the metallic foreign bodies and enables its safe removal with minimal surrounding tissue damage and optic nerve injury.

Association of glycemic control with progression of diabetic retinopathy in type 2 diabetes mellitus patients in Malaysia

Abstract The risk of having microvascular complication is high among Type 2 Diabetes Mellitus (T2DM) patients. However, factors associated with the glycemic control and progression of diabetic retinopathy (DR) in T2DM patients is limited. This study aims to determine association between anti-diabetic agents, glycemic control and progression of diabetic retinopathy in a Malaysian population. A retrospective study conducted in a tertiary teaching hospital in Malaysia, from January 2009 until March 2014. This study enrolled 104 patients aged 40-84 years, with a mean age 63.12 ± 9.18 years. patients had non-proliferative diabetic retinopathy (NPDR, 77%) and 35% had proliferative diabetic retinopathy (PDR). Diabetic macula edema (DME) was present in 20% of NPDR patients, compared with 7% in PDR. Alpha-glucosidase inhibitor (p=0.012), age (p=0.014) and number of antidiabetic agents used (p=0.015) were significantly associated with stages of diabetic retinopathy. Family history of T2DM (p=0.039) was associated with DME. Identifying factors influencing the progression of diabetic retinopathy may aid in optimizing the therapeutic effects of anti-diabetic agents in T2DM patients.