Maffucci syndrome and intracranial chondrosarcomas: a case report featuring spontaneous resolution of sixth nerve palsy.

Maffucci syndrome is a rare disease process characterized by enchondromatosis with cutaneous hemangiomatosis. We report a 20-year-old woman with Maffucci syndrome with a 5-day history of diplopia. She was found to have a left sixth nerve palsy due to a parasellar chondrosarcoma. Three weeks later, the patient's diplopia spontaneously resolved. This unusual clinical course prompted us to review frequency of sixth nerve palsy with skull base neoplasms and the phenomenon of spontaneous resolution of diplopia.

EIGHTY-TWO-YEAR-OLD MONOCULAR FEMALE WITH ACUTE DECREASED CENTRAL VISION.

PURPOSE: To present a case of suspected sympathetic ophthalmia in an 82-year-old monocular woman. METHODS: Case report. RESULTS: Here, we present an 82-year-old woman, status postcataract extraction with lens subluxation followed by a complicated course ultimately requiring enucleation because of a blind and painful eye in 2020, who developed contralateral choroidal lesions 6 months postenucleation along with vitritis and anterior uveitis. The lesions were suspicious for an infectious versus autoimmune etiology. Thorough systemic work-up and multimodal imaging suggest an autoimmune case. The findings in this patient suggest early identified sympathetic ophthalmia with multifocal choroiditis. This patient responded well to treatment of the acute episode with systemic corticosteroids and ultimately required steroid-sparing immunosuppression. CONCLUSION: Sympathetic ophthalmia is a rare entity classically observed after intraocular surgery and trauma. It may mimic many infectious and noninfectious uveitis entities. In this patient, sympathetic is a primary concern given her history and age of presentation with consideration for other uveitic entities.

Intravitreal anti-vascular endothelial growth factor for the treatment of chronic central serous retinopathy: a meta-analysis of the literature.

OBJECTIVE: The purpose of this study was to evaluate the role of anti-vascular endothelial growth factor (anti-VEGF) treatment on the functional and structural parameters of chronic central serous retinopathy (CSR). METHODS: PubMed was used to systematically review literature published from 1 January 2009 to 1 July 2022. Studies were included if patients in their cohort had symptoms for more than 3 months, anti-VEGF treatment was provided and the following outcomes were reported: best-corrected visual acuity (BCVA), central macular thickness (CMT) and proportion of subretinal fluid (SRF) resolution. RESULTS: 339 eyes met inclusion criteria with a mean patient age of 45.8±4.9 years. The weighted mean baseline BCVA for the 20 studies was 0.39±0.23 logMAR, which improved to 0.28±0.24 after treatment with anti-VEGF injections (p=0.069). The weighted baseline CMT for the 20 studies decreased from 395.2±52.0 µm to 243.0±41.9 µm (p<0.001). The weighted overall percentage of SRF resolution was 68.4%. CONCLUSION: Anti-VEGF treatment demonstrated significantly decreased macular thickness and resolution of SRF in the treatment of chronic CSR without any reported adverse effects. However, BCVA did not significantly improve with pharmacotherapy.

Radiation Retinopathy and Benign Lymphoproliferative Orbital Tumor Presenting 47 Years Following Treatment of Hereditary Retinoblastoma.

PURPOSE: To better understand onset of radiation retinopathy and secondary orbital tumors in the setting of retinoblastoma treated with radiation and chemotherapy. METHODS: Case report. RESULTS: Here we present a 48-year-old female with a history of bilateral hereditary retinoblastoma status-post enucleation of the left eye and radiation therapy to the right eye, along with systemic chemotherapy. She underwent bladder leiomyosarcoma resection at age 24. In 2020, she presented with significantly delayed radiation retinopathy complicated by cystoid macular edema, and bevacizumab injections were initiated. An incidental benign lymphoproliferative tumor in the right lacrimal gland was found on B-scan ultrasound and was successfully excised. CONCLUSION: It is rare for radiation retinopathy to present with significant delay after local radiation treatment, with only 2 other cases found in the literature describing a similar late onset. In addition, there have been no other published cases of a secondary benign lymphoproliferative tumor in the setting of retinoblastoma treated with radiation and chemotherapy.

Open globe injury and intraocular foreign body following crossbow-related penetrating ocular trauma.

PURPOSE: To describe a case of a penetrating ocular trauma and plastic intraocular foreign body (IOFB), undetected on preoperative imaging. OBSERVATIONS: We present the findings of a 40-year-old male who sustained an open globe injury and IOFB composed of plastic following crossbow-related trauma. Preoperative detection of the IOFB was unsuccessful on clinical exam, computed tomography (CT) and ultrasonography. During extraction of the traumatic cataract, an intralenticular IOFB was discovered and removed through an enlarged limbal incision. Postoperative review revealed that a fragmented plastic "nock", from the crossbow arrow bolt, was the likely IOFB source. The bolt was produced by injection molding which may lead to trapped gas within the plastic, causing radiolucency on CT. CONCLUSIONS AND IMPORTANCE: Radiolucent plastic warrants consideration on the differential diagnosis when intraocular gas is noted on computed tomography following penetrating ocular trauma. Multimodal imaging should be considered if IOFB is suspected and not detected by CT.

Automated Region of Interest Selection Improves Deep Learning-Based Segmentation of Hyper-Reflective Foci in Optical Coherence Tomography Images.

Hyperreflective foci (HRF) have been associated with retinal disease progression and demonstrated as a negative prognostic biomarker for visual function. Automated segmentation of HRF in retinal optical coherence tomography (OCT) scans can be beneficial to identify the formation and movement of the HRF biomarker as a retinal disease progresses and can serve as the first step in understanding the nature and severity of the disease. In this paper, we propose a fully automated deep neural network based HRF segmentation model in OCT images. We enhance the model's performance by using a patch-based strategy that increases the model's compute on the HRF pixels. The patch-based strategy is evaluated against state of the art HRF segmentation pipelines on clinical retinal image data. Our results shows that the patch-based approach demonstrates a high precision score and intersection over union (IOU) using a ResNet34 segmentation model with Binary Cross Entropy loss function. The HRF segmentation pipeline can be used for analyzing HRF biomarkers for different retinopathies.

Cytomegalovirus Retinitis in HIV and Non-HIV Individuals.

Cytomegalovirus retinitis (CMVR) is a severe, vision-threatening disease that primarily affects immunosuppressed patients. CMVR is the most common ocular opportunistic infection in human immunodeficiency virus (HIV) infected patients and is the leading cause of blindness in this group; however, the incidence of CMVR in HIV patients has dramatically decreased with antiretroviral therapy. Other causes of immunosuppression, including organ transplantation, hematologic malignancies, and iatrogenic immunosuppression, can also lead to the development of CMVR. Herein, we describe the pathogenesis of CMVR and compare clinical features, epidemiology, and risk factors in HIV and non-HIV infected individuals with CMVR.

Cilioretinal artery hypoperfusion and its association with paracentral acute middle maculopathy.

BACKGROUND/AIMS: To study the multimodal imaging findings of a large series of eyes with cilioretinal artery obstruction (CILRAO) and describe the systemic associations. METHODS: Multicentre, retrospective chart review from 12 different retina clinics worldwide of eyes with CILRAO, defined as acute retinal whitening in the distribution of the cilioretinal artery, were identified. The clinical, systemic information and multimodal retinal imaging findings were collected and analysed. RESULTS: A total of 53 eyes of 53 patients with CILRAO were included in the study. In 100% of eyes, fundus photography illustrated deep retinal whitening corresponding to the course of the cilioretinal artery. Twenty-eight patients (52.8%) presented with isolated CILRAO (baseline best-corrected visual acuity (BCVA) 20/50, final BCVA 20/25) associated with nocturnal hypotension, 23 patients (43.4%) with CILRAO secondary to central retinal vein occlusion (CRVO) (baseline BCVA 20/40, final BCVA 20/20) and two patients with CILRAO due to biopsy-proven giant cell arteritis (GCA) (baseline BCVA 20/175, final BCVA 20/75). With spectral domain optical coherence tomography (SD-OCT), a hyper-reflective band involving the inner nuclear layer (ie, paracentral acute middle maculopathy or PAMM) was noted in 51 eyes (28/28 eyes with isolated CILRAO and 23/23 eyes with CILRAO+CRVO) corresponding to the retinal whitening. In the two eyes with CILRAO+GCA, SD-OCT illustrated hyper-reflective ischaemia of both the middle and inner retina. CONCLUSIONS: Isolated CILRAO and CILRAO secondary to CRVO are the result of hypoperfusion or insufficiency, rather than occlusion, of the cilioretinal artery and are associated with PAMM or selective infarction of the the inner nuclear layer. With GCA, there is complete occlusion of the cilioretinal artery producing ischaemia involving both the middle and inner retina associated with worse visual outcomes.

Lid splinting eyelid retraction technique: a minimised sterile approach for intravitreal injections.

BACKGROUND/AIMS: To describe an alternative technique for avoiding contact with the lids and lashes, without the use of a lid speculum, during intravitreal anti-vascular endothelial growth factor injections. METHODS: Retrospective case series of all patients undergoing intravitreal injections of bevacizumab and ranibizumab, with the lid splinting retraction technique from January 2010 to December 2015. Injections performed by six vitreoretinal specialists were included. The key preinjection ocular surface preparation includes topical anaesthetic, 5% povidone-iodine and a subconjunctival injection of 2% lidocaine with epinephrine. A second instillation of 5% povidone-iodine is given and the intravitreal injection is then performed. No lid speculum is used. A search of the electronic medical records identified patients diagnosed with postinjection endophthalmitis and charts were reviewed to ensure inclusion criteria were met. The main outcome measure was incidence of postinjection endophthalmitis. RESULTS: A total of 78 009 consecutive intravitreal injections were performed, of which 22 207 were bevacizumab and 55 802 were ranibizumab. In this cohort of patients (n=6320), 12 cases of endophthalmitis developed, corresponding to a rate of 0.015%. CONCLUSIONS: The technique of eyelid retraction for intravitreal injection has a low rate of endophthalmitis, similar to the reported rates using a metal lid speculum. This is beneficial for both the physician and the patient as it minimises patient discomfort as well as the duration of the procedure. To our knowledge, this is one of the largest studies performed to date evaluating intravitreal injection-related endophthalmitis.

The other CNVM: a review of myopic choroidal neovascularization treatment in the age of anti-vascular endothelial growth factor agents.

Choroidal neovascular membranes (CNVM) associated with pathological myopia (PM) can result in significant vision loss and legal blindness. These membranes usually occur subfoveally and are a major complication of PM, developing in approximately 5-10% of such eyes. PM is the second most common cause of choroidal neovascularization after age-related macular degeneration (AMD), and accounts for nearly 60% of CNVM cases in patients younger than age 50. Vascular endothelial growth factor-A has been implicated as the major angiogenic stimulus responsible for choroidal neovascularization secondary to AMD and several major studies have proved the benefits of anti-VEGF treatment for AMD-related CNVM. Benefits have also been observed in a number of prospective and retrospective studies evaluating PM CNVM. Despite the small differences in molecular properties of ranibizumab and bevacizumab, both drugs showed similar therapeutic effects for CNVM associated with PM. Many studies also highlighted that patient age, previous photodynamic therapy treatment, axial length, and visual acuity prior to treatment may affect treatment prognosis. Although there is a paucity of large randomized controlled trials, this systematic review highlights the large numbers of individual trials that demonstrate a significant improvement in VA. The inferior long-term results of alternative therapies, combined with an excellent safety profile from anti-VEGF treatment, make anti-VEGF the current recommended first-line therapy.

Documenting the subjective patient experience of first versus second cataract surgery.

PURPOSE: To examine the subjective patient experience after cataract surgery. SETTING: Single multisurgeon cataract facility. DESIGN: Prospective intraindividual observational study. METHODS: Patients completed a questionnaire immediately after cataract extraction performed in their second eye. All patients had second-eye surgery within 6 months of first-eye surgery. Cases longer than 30 minutes were excluded. RESULTS: Of the 292 patients who completed the questionnaire, 12 were excluded based on surgical time. The response rate varied per question. The surgery was rated as taking longer or being more painful in the second eye by 127 patients (45.4%) and in the first eye by 38 patients (13.5%) (P < .05); 115 patients (41.1%) reported no difference. Patients (47.83%) who rated the second eye as the generally more negative experience thought their vision would be better and 3.48% worse (P < .05); 48.70% thought it would be the same. No difference was noted in length of surgery (P = .3) or sedation used (P = .96). CONCLUSIONS: Of 125 patients who rated second-eye surgery as the generally more unpleasant procedure, 90 (72.0%) were similarly or more relaxed during the second procedure. Second-eye cataract surgery was perceived as being a longer and/or more painful procedure by a significant number of patients (45.4%), and only 3.48% thought that vision in the second eye would be worse. These results can help surgeons when counseling patients regarding expectations for second surgery. FINANCIAL DISCLOSURE: No author has a financial or proprietary interest in any material or method mentioned.

Treatment of ocular metastasis with anti-VEGF: a literature review and case report.

PURPOSE: This is the first case report where 1.25mg intravitreal bevacizumab (IVB) correlated with choroidal mass resolution from metastatic breast cancer given concurrently with chemotherapy demonstrating, at best, disease stability in other organs. STUDY DESIGN: Case report. METHODS: Upon confirmation of choroidal, liver and bone metastasis from breast carcinoma, a 72-year-old female received four intravitreal bevacizumab 1.25mg injections based on the presence of subretinal and intraretinal fluid. Visual outcomes were analyzed by ophthalmologic evaluation, B-scan, fluorescein angiography, and optical coherence tomography. RESULTS: After 3 treatments of 1.25mg intravitreal bevacizumab, visual acuity improved from 20/125 OD to 20/30 OD. These results were maintained for 5 months, after which a 4th IVB injection was given to try to further improve visual outcomes. Following this, complete resolution of the mass was observed with remaining pigmentary changes and vision improved to 20/25 one month following this. IVB was administered concurrently to systemic chemotherapy that demonstrated at best disease stability in metastases in other organs. CONCLUSION: In this case 1.25mg intravitreal bevacizumab proved to be a safe, effective and relatively easy treatment for choroidal metastasis from breast cancer. An important benefit of intravitreal bevacizumab therapy for choroidal metastasis is the ease of administration and minimal time commitment required as compared to other therapies. Further studies should be conducted to confirm the appropriate dosing and long-term outcomes of intravitreal bevacizumab to treat choroidal metastasis.

Two cases of spontaneous filtering blebs, one idiopathic and one associated with Terrien marginal degeneration.

PURPOSE: To report a case of an idiopathic unilateral filtering bleb in an otherwise well patient and a case of bilateral spontaneous filtering blebs in association with Terrien marginal degeneration. METHODS: In this retrospective observational study of 2 cases, clinical notes, ultrasound biomicroscopy and gonioscopy images were reviewed. RESULTS: Case 1: A 32-year-old woman presented with a 1-year history of a gradually growing cyst in her right eye. No changes in vision were reported. The patient had no previous ocular or systemic history, trauma, or surgical interventions. Slit-lamp examination revealed a multilobulated cyst at the supranasal limbus OD. Diagnosis was an idiopathic spontaneous limbal filtering bleb of unclear etiology because there was no inciting event or obvious cause. Case 2: A 31-year-old man, with 2 past episodes of spontaneous filtering bleb development, affecting the left, then the right, eye, presented with his third event in May 2012. The visual acuity was decreased to 20/400 OD. The right eye was hypotonous and ultrasound biomicroscopy confirmed a shallow anterior chamber, a superior peripheral corneal defect, and an echolucent space suggestive of subconjunctival fluid. Limbal thinning was noted bilaterally, consistent with Terrien marginal degeneration. CONCLUSIONS: This is a rare case of an idiopathic filtering bleb occurring in a healthy patient with no precipitating cause, other ocular abnormalities, or physical malformations, and the only reported case of bilateral sequential filtering blebs associated with an atypical presentation of Terrien marginal degeneration.