Abstract: Pediatric neuro-ophthalmology is a subspecialty within neuro-ophthalmology. Pediatric neuro-ophthalmic diseases must be considered separate from their adult counterparts, due to the distinctive nature of the examination, clinical presentations, and management choices. This manuscript will highlight four common pediatric neuro-ophthalmic disorders by describing common clinical presentations, recommended management, and highlighting recent developments. Diseases discussed include pediatric idiopathic intracranial hypertension (IIH), pseudopapilledema, optic neuritis (ON) and optic pathway gliomas (OPG). The demographics, diagnosis and management of common pediatric neuro-ophthalmic disease require a working knowledge of the current research presented herein. Special attention should be placed on the differences between pediatric and adult entities such that children can be appropriately diagnosed and treated.

Abstract: To present spectral domain optical coherence tomography (OCT) findings during treatment in a case of acute isolated cilioretinal artery occlusion (CLRAO) reversed with intravenous systemic administration of mannitol and carbogen inhalation. Close monitoring with OCT thickness topographic map and cross section scans, every 12 hours, during treatment and till complete reversal of retinal nerve fiber layer edema. Fundus photography and fluorescein angiography (FFA) were used to illustrate occlusion and recanalization. After 72 hours of therapy, visual acuity improved from counting fingers (CF) to 7/10, Snellen’s chart. Consecutively OCT scans showed that the initial macular edema was gradually restored to typical 72 hours of treatment initiation. FFA performed after treatment confirmed recanalization of the cilioretinal artery. Early intervention with the combined intravenous administration of mannitol and carbogen inhalation can reverse acute onset loss of vision due to CLRAO. The reflectivity of retinal layers differs significantly regarding stages of acute CLRAO. In our case report increased reflectivity of the innermost layers of the retina was illustrated and a corresponding reduction in the outer retina and the retinal pigment epithelium and choriocapillaris layers. Macular thickness follow-up data recorded the course of intracellular edema to normal.

Abstract: In this review, recent studies regarding riboflavin-ultraviolet A (UVA) collagen cross-linking for the treatment of acanthamoeba keratitis (AK) were reviewed. English written studies about acanthamoeba, keratitis, riboflavin and collagen cross-linking were retrieved from PubMed search engine (www.ncbi.nlm.nih.gov/pubmed). Although there were significant numbers of cases reporting the effectiveness of riboflavin-UVA collagen cross-linking in AK, experimental studies (in vivo and in vitro) failed to verify amoebicidal or cysticidal effect of riboflavin-UVA collagen cross-linking. In conclusion, the efficacy of riboflavin-UVA collagen cross-linking for the treatment of AK is still debatable. It is necessary to conduct a prospective case-control study for clear guidance for clinicians.

Abstract: Autoimmune retinopathy (AIR) refers to both paraneoplastic and non-paraneoplastic forms of a rare, acquired retinal degeneration thought to be mediated by the production of antiretinal antibodies. However, the mechanisms underlying AIR pathogenesis are incompletely understood, and it remains a diagnosis of exclusion given the lack of definitive testing as well as its protean clinical presentation. This review summarizes the current literature on the epidemiology, diagnosis, and management of AIR, with a focus on non-paraneoplastic disease and the potential role of immunomodulatory therapy. A recent expert consensus statement on diagnosis and management of non-paraneoplastic AIR served as a framework for interpreting the limited data available, a process that was complicated by the small sample sizes, heterogeneity, and retrospective nature of these studies. Additional work is needed to characterize AIR patients on the basis of cytokine and immunogenetic profiling; to establish the pathogenicity of antiretinal antibodies; and to standardize treatment regimens as well as assessment of clinical outcomes.

Abstract: Acute retinal necrosis (ARN) is a devastating syndrome characterized by panuveitis, retinal necrosis, and a high rate of retinal detachment that may result in poor visual outcomes if not promptly diagnosed and treated. ARN is most commonly caused by viruses within the herpesvirus family. Etiologies include varicella-zoster virus, herpes simplex virus, and cytomegalovirus, and may be promptly diagnosed by polymerase chain reaction testing of aqueous or vitreous fluid. The true incidence of ARN is not known due to its rarity; as a result, clinical treatment is often guided by retrospective case series, case reports, and expert opinion. Standard of care has evolved over time but currently includes a combination of systemic and intravitreal antiviral in conjunction with topical or oral steroids and surgical therapy as needed. Combination therapy may reduce the rate of severe vision loss and increase the rate of visual acuity gain, although further studies are needed in this area. In particular for patients with mild to moderate disease, combination therapy may reduce the rate of retinal detachment. Adjunctive therapies including oral corticosteroid and prophylactic laser barricade are incompletely studied, but corticosteroid in particular, may reduce inflammation, which also is involved in the severe disease pathogenesis observed in ARN. This review discusses the advances in diagnosis and treatment of ARN, including management with combination antiviral medication and surgical interventions.

Abstract: Cavernous hemangioma is the most primary benign orbital tumor in adults, and majority of cases could be easily settled by surgical treatment. However, cavernous hemangioma lodged deep in the orbital apex remained a challenge because the surgery may pose a high risk of injury to the optic nerve and significant visual loss. This presentation would report a case of cavernous hemangioma located in orbital apex who presented superonasal and inferotemporal peripheral vision defect. The patient received fully transnasal endoscopic surgery, and a 2 cm × 1.5 cm tumor was successfully removed from the left orbital apex. The treatment results were satisfactory, with no after-effects and adverse reactions during follow-up. This case highlighted that transnasal endoscopic surgery is a promising technique for cavernous hemangiomas that are located deep in orbital apex. This approach provides direct pathway to tumor with limiting morbidity, maximal surgical field and ample illumination. The procedure represents a safe and less invasive management.

Abstract: Optical coherence tomography angiography (OCTA) is a fast, non-invasive imaging modality that provides detailed information on retinal and choroidal vascular flow and macular structure. OCTA offers an accurate three-dimensional view of the individual retinal vascular plexuses and the choriocapillaris which facilitates the detection of the microvascular abnormalities in a variety of macular diseases. The perfusion indices (vessel density and flow index) are valuable parameters evaluated by OCTA that allow a quantitative interpretation of changes in the retinal vasculature that can reflect the severity of disease. Crystalline retinopathy encompasses a group of conditions whose distinctive feature is the presence of retinal crystals often located in the posterior pole. Select crystalline retinopathies also demonstrate retinal vascular abnormalities as well. Considering that the OCTA is a novel imaging modality and crystalline retinopathies are relatively rare conditions, there are currently few reports of OCTA findings associated with crystalline retinopathy. The advent of OCTA allows visualization of vascular and structural changes in crystalline retinopathies that are unique and cannot be appreciated on other imaging modalities, including fluorescein angiography (FA). This article reviews novel OCTA findings which provide new insights in the pathogenesis of crystalline retinopathies, including Bietti crystalline retinopathy, talc retinopathy, macular telangiectasia type 2, tamoxifen retinopathy, and Sj?gren-Larsson Syndrome maculopathy.