Azzopardi Phenomenon in a Non-neoplastic Phthisical Eye: A Case Report

Objective: The Azzopardi phenomenon, known as the deoxyribonucleic acid deposition on various structures due to cellular necrosis, has never been reported in non-neoplastic eyes. Methods: We report a case of a 48-year-old man who had congenital nystagmus with poor vision in both eyes, presented with decreased vision and photophobia in his left eye. An exudative retinal detachment was found, which did not respond to systemic steroid treatment. Glaucoma due to occlusio pupillae was later developed. Laser iridotomy and anti-glaucoma medications decreased intraocular pressure to an acceptable level. Vision in the left eye gradually deteriorated during the 10-year clinical course. Evisceration was finally performed due to persistent dull aching ocular pain along with signs of ocular hypotony Results: Histopathological examination showed phthisis bulbi and focal nodular retinal gliosis. The Azzopardi phenomenon was found at the retinal vessel walls, within the retinal layers and along the internal limiting membrane. There was neither evidence of intraocular tumors nor foreign bodies. Conclusion: This case demonstrated that the Azzopardi phenomenon could be present in a non-neoplastic eye with a longstanding disease that proceeds to phthisis bulbi.

Coats Disease in 9 Patients: A Hispanic Case Series

Background/Aim To describe demographic and clinical characteristics, treatment, and visual prognosis of Coats disease in Hispanic patients. Methods A retrospective chart review was performed on nine patients (ten eyes) diagnosed with Coats disease in our two clinical centrers from 2004 – 2017. Results Mean age at diagnosis was 5.5 years (range 1 – 12 years) and mean follow-up time was 48 months (range 9 – 108 months). Eight patients (89%) were male and had unilateral disease and one (11%) female patient had bilateral disease. In 40% of the cases, patients were asymptomatic. Visual acuity at first presentation was worse than hand motion in 60% of the eyes. Half of the eyes (5/10 eyes, 50%) had exudative retinal detachment (≥ stage IIIA). Vascular ablation with cryotherapy combined with retinal photocoagulation was the most frequent therapeutic approach (40%). Despite anatomical success at 6 months in 100% of the treated eyes, visual outcome at 1 year of treatment was poor (worse than 20/200) in 70% of the cases. Conclusions In our case series, patients were mostly asymptomatic on presentation, with severe stages of Coats disease. Even with anatomical success after surgical treatment in all treated cases, long-term visual prognosis remained very limited.

Acute retinal necrosis with exudative retinal detachment in a child

Acute retinal necrosis (ARN) is a rare ocular emergency caused mainly by viral entities. ARN may be caused by Herpes zoster virus (HZV) and Herpes simplex virus (HSV), both HSV-1 and HSV-2. ARN mostly present in 20–60 years old immunocompetent adults. A 7-year-old girl presented to the eye clinic with complaints of left eye redness noted by her mother for 2–3 days. On examination with indirect ophthalmoscopy, no hypopyon was seen in either eye. In the left eye fundus view was hazy. Ultrasound B-scan performed showed exudative retinal detachment. PCR of ocular fluid was positive for HSV-1 DNA. The patient was started on topical steroids and antibiotics and systemic antivirals. In addition, she also received intravitreal ganciclovir 4 mg/0.1 mL three times under general anaesthesia. At her last follow-up, 3 years from her presentation, her right eye examination was within normal limits, and left eye showed thick vitreous bands with a posterior vitreous detachment, and left inferotemporal retinal scarring.

New Perspectives on the Immunopathogenesis and Treatment of Uveitis Associated With Vogt-Koyanagi-Harada Disease

Uveitis associated with Vogt-Koyanagi-Harada (VKH) disease is a bilateral, chronic, granulomatous autoimmune disease associated with vitiligo, poliosis, alopecia, and meningeal and auditory manifestations. The disease affects pigmented races with a predisposing genetic background. Evidence has been provided that the clinical manifestations are caused by a T-lymphocyte-mediated autoimmune response directed against antigens associated with melanocytes in the target organs. Alongside of T lymphocytes, autoreactive B cells play a central role in the development and propagation of several autoimmune diseases. The potential role of B lymphocytes in the pathogenesis of granulomatous uveitis associated with VKH disease is exemplified within several studies. The early initial-onset acute uveitic phase typically exhibits granulomatous choroiditis with secondary exudative retinal detachment and optic disc hyperemia and swelling, subsequently involving the anterior segment if not adequately treated. The disease eventually progresses to chronic recurrent granulomatous anterior uveitis with progressive posterior segment depigmentation resulting in “sunset glow fundus” appearance and chorioretinal atrophy if not properly controlled. Chronically evolving disease is more refractory to treatment and, consequently, vision-threatening complications have been recognized to occur in the chronic recurrent phase of the disease. Conventional treatment with early high-dose systemic corticosteroids is not sufficient to prevent chronic evolution. Addition of immunomodulatory therapy with mycophenolate mofetil as first-line therapy combined with systemic corticosteroids in patients with acute initial-onset disease prevents progression to chronic evolution, late complications, vitiligo, and poliosis. Furthermore, patients under such combined therapy were able to discontinue treatment without relapse of inflammation. These findings suggest that there is a therapeutic window of opportunity for highly successful treatment during the early initial-onset acute uveitic phases, likely because the underlying disease process is not fully matured. It is hypothesized that early and aggressive immunosuppressive therapy will prevent remnant epitope generation in the initiation of the autoimmune process, the so-called primary response. B cell depleting therapy with the anti-CD20 monoclonal antibody rituximab is effective in patients with refractory chronic recurrent granulomatous uveitis. The good response after rituximab therapy reinforces the idea of an important role of B cells in the pathogenesis or progression of chronic recurrent uveitis associated with VKH disease.

The Effectiveness of Propranolol in Managing Hemorrhagic Choroidal and Exudative Retinal Detachment following Ahmed Glaucoma Valve Implantation in Sturge-Weber Syndrome: Case Report and Literature Review

Sturge-Weber syndrome (SWS) is a congenital neurological disorder that is characterized by hamartomas involving the skin, brain, and eyes and marked by the presence of the facial port-wine stain, which consequently leads to various ocular complications. Among all ocular comorbidities, glaucoma is the most frequently witnessed in SWS patients with a prevalence of 30%–70%. If glaucoma is refractory to conventional medical management, surgical intervention can be considered. Common complications of glaucoma procedures in SWS are choroidal detachment and suprachoroidal hemorrhage. Moreover, we report a 6-year-old girl, known case of unilateral congenital glaucoma secondary to SWS. Despite being on maximal antiglaucoma drops and undergoing multiple surgical interventions, the patient had uncontrolled intraocular pressure of her right eye. A decision to proceed with Ahmed glaucoma valve implantation (AGVI) to the right eye was made. In the immediate postoperative period, the patient developed hemorrhagic choroidal detachment and exudative retinal detachment. A trial of oral propranolol (1.5–2 mg/kg/day) was then initiated for 4 months. After 30 days from oral propranolol course initiation, we started noticing a significant improvement of the hemorrhagic choroidal and exudative retinal detachment. Spontaneously, a marked reduction in subretinal fluid and suprachoroidal hemorrhage was also seen. Thus, the improvement was correlated with the propranolol therapy. Here, we report a significant improvement of the postoperative complications of AGVI in a patient with SWS, following 4 months of oral propranolol course (1.5–2 mg/kg/day). Further studies are needed to determine the dosage, duration, and optimal mechanism by which propranolol works in this situation.

Adolescent Plaque Brachytherapy for Large Choroidal Metastasis from Lung Carcinoid Tumor

The purpose of this publication is to report a child with choroidal metastasis from a primary lung carcinoid tumor treated with 125-iodine plaque brachytherapy while achieving complete tumor regression. A 15-year-old girl with a history of stage IV lung carcinoid tumor developed painless progressive visual loss in her right eye. Ophthalmological exam documented a unilateral solitary large choroidal mass with complete exudative retinal detachment. Planned palliative treatment with plaque brachytherapy was performed utilizing a total dose of 45 Gy to the tumor apex. No tumor regression was noted after 6 months of follow-up. Ten months after primary therapy, re-treatment with a total dose of 90 Gy to the tumor apex was performed due to tumor progression. Complete regression was achieved after secondary treatment without evidence of recurrence after 18 months of follow-up. In conclusion, high-dose plaque brachytherapy may induce complete tumor regression in select adolescent patients with choroidal metastasis from lung carcinoid tumor.

O28 A rare sight-threatening complication of a rare disease

Case report - Introduction Relapsing polychondritis (RP) is a rare autoimmune disorder characterised by inflammation of cartilaginous structures throughout the body. It usually presents in the fourth and sixth decade, and commonly affected areas include the nasal and respiratory tracts, external ears and joints. Ocular involvement is reported in around 65% of RP patients during their lifetime but is rarely sight-threatening. However, we present an unusual case of recurrent ocular inflammation due to RP that resulted in unilateral posterior scleritis with sub-retinal exudation, and posed a high risk of retinal detachment. Prompt escalation of immunosuppressive treatment was required to prevent this. Case report - Case description A 48-year-old man of south-east Asian descent presented to rheumatology in December 2020 with a typical history of new inflammatory arthritis of 4 weeks duration. He was known to have bilateral episcleritis and ocular hypertension for 3 years and took Brinzolamide and Latanoprost eye drops, as well as metformin for type 2 diabetes mellitus. The only other relevant history was treatment with antibiotics as an inpatient for bilateral pinna cellulitis 2 months prior, which was suspected to be related to his diabetes. Examination revealed mildly reduced hand grips but no definite synovitis. The most remarkable finding was bilateral painless red eyes. Both pinnae appeared inflamed on close inspection without obvious auricular lobe involvement. Blood tests showed raised C-Reactive Protein (38 mg/L), and Erythrocyte Sedimentation Rate (62 mm/hr), with normochromic normocytic anaemia but other blood counts were normal. Renal and liver function was normal. Detailed immunology was negative. Relapsing Polychondritis was suspected and confirmed following multidisciplinary team (MDT) discussions with the Ear Nose and Throat and ophthalmology teams. At the next visit, his disease progressed to florid polyarthritis in a rheumatoid distribution. This responded well to low-dose prednisolone and methotrexate. However, the patient attended eye casualty on multiple occasions over the next 6 weeks with alternating acute eye pain and redness, mildly reduced visual acuity and raised intraocular pressures. He developed sub-retinal fluid pockets suggestive of posterior scleritis in the right eye and retinal tomography demonstrated extensive pigment clumping especially in the macula with outer retinal disruption. Urgent pulses of intravenous methylprednisolone were given, followed by high-dose prednisolone tapering. The case was discussed with the regional uveitis MDT, and treatment was escalated to adalimumab. He is currently 1 month into treatment and has preserved visual acuity and stable intraocular pressures, with no other systemic involvement of his RP. Case report - Discussion The diagnosis of RP requires a high index of clinical suspicion given the lack of diagnostic markers. The clinical features are multisystem and might not occur simultaneously as in this case, where the eye disease preceded the ear and joint manifestations by a couple of years. Perichondritis might be more difficult to appreciate in patients with darker skin and can further delay diagnosis. The average time to diagnosis from the initial symptoms has been reported as 14 months from a case series of 158 patients with RP in China. Ocular complications are usually bilateral and the commonest are episcleritis and scleritis (over 50% of cases have one of these), followed by uveitis and retinopathy. Eyelid oedema, proptosis and optic neuritis can also occur but are rarer, and exudative retinal detachment has only been noted in a small number of case reports. Unfortunately, no current treatment guidelines exist for RP due to a lack of randomised controlled trials but treatment is usually guided by the severity of manifestations in conjunction with trends from case series and expert opinion. The aim is to halt or slow disease progression and glucocorticoids are often used first-line for moderate to severe disease, with or without the addition of steroid-sparing agents such as methotrexate, ciclosporin and azathioprine. A meta-analysis of biologics in RP conducted by Kemta et al. in 2012 has suggested anti-TNF (particularly Infliximab) might be beneficial as second-line treatment for severe or refractory cases of organ involvement due to RP such as central nervous system, nasal or pulmonary involvement. However, due to the extreme rarity of both RP and associated severe retinal disease, the optimum therapeutic choice in this setting is currently not known. Case report - Key learning points

A clinical case of bilateral exudative retinal detachment against the background of severe preeclampsia

This article describes a clinical case of a bilateral exudative retinal detachment development in a young woman with severe preeclampsia. The data of the ophthalmological examination, as well as those of the dynamic observation of the patient, are presented. The possible mechanisms of the formation of exudative retinal detachment on the background of preeclampsia are considered in detail. The described clinical case is of great interest, since this condition is a rare complication in preeclampsia. Exudative retinal detachment against the background of preeclampsia requires adherence to bed rest, normalization of blood pressure, control of proteinuria and, as a rule, does not require surgical intervention.