Telemedicine in neuro-ophthalmology.

PURPOSE OF REVIEW: The COVID-19 public health emergency accelerated the adoption of telemedicine in neuro-ophthalmology. This review surveys the current telemedicine landscape and discusses associated limitations and opportunities. RECENT FINDINGS: Logistic considerations and regulatory changes related to the COVID-19 pandemic have spurred a proliferation of tele-neuro-ophthalmic practice. One significant benefit of telemedicine is increased access to the limited number of neuro-ophthalmologists in the country. Certain elements of the neuro-ophthalmic examination can be ascertained during a video visit or through the use of mobile applications. However, data quality can be limited and more direct evaluation of the fundus currently requires the implementation of imaging techniques, such as fundus photography and/or optical coherence tomography. For cases that require it, a 'hybrid' model can be adopted in which patients physically present to designated locations for testing and subsequently participate in televisit with the physician to discuss the assessment and plan. SUMMARY: Telemedicine provides an alternate pathway for patients to access the limited resource of neuro-ophthalmic care, and it will likely persist beyond the current COVID-19 pandemic. It has some limitations currently but continued progress in technologic, legal, and reimbursement strategies will hopefully facilitate further adoption.

Magnetic Resonance Imaging Features and Clinical Findings in Pediatric Idiopathic Intracranial Hypertension: A Case-Control Study.

The purpose of this study is to identify salient magnetic resonance imaging (MRI) findings of pediatric IIH, to determine the relevance of these findings with regard to disease pathogenesis, and to relate these findings to the clinical presentation towards identification of risk factors of disease. A retrospective, a case-control study of 38 pediatric patients with and 24 pediatric patients without IIH from the ophthalmology department at a tertiary care center was performed. Clinical data, including ophthalmic findings and lumbar puncture results, were recorded. Neuroimaging, including both MRI and magnetic resonance venography (MRV), was evaluated for perioptic subarachnoid space diameter enlargement, posterior globe flattening, optic nerve head protrusion, empty or partially empty sella turcica, dural venous sinus abnormalities, skull base crowding, and prominent arachnoid granulations. Compared with controls, IIH patients had larger perioptic subarachnoid space diameters, higher incidences of posterior globe flattening, protrusion of the optic nerve heads, an empty sella turcica, and dural venous sinus abnormalities. A perioptic subarachnoid space diameter of ≥5.2 mm was identified as an independent predictor of IIH (p < 0.001) with sensitivity of 87% and specificity of 67%. Several significant MRI findings in pediatric IIH were identified. Using a model that uniquely incorporated clinical and MRI findings at presentation, we provide a framework for risk stratification for the diagnosis of pediatric IIH which may be utilized to facilitate diagnosis. Future prospective work is needed to further validate the model developed in this study.

Site of Origin of the Ophthalmic Artery Influences the Risk for Retinal Versus Cerebral Embolic Events.

BACKGROUND: Embolic events leading to retinal ischemia or cerebral ischemia share common risk factors; however, it has been well documented that the rate of concurrent cerebral infarction is higher in patients with a history of transient ischemic attack (TIA) than in those with monocular vision loss (MVL) due to retinal ischemia. Despite the fact that emboli to the ophthalmic artery (OA) and middle cerebral artery share the internal carotid artery (ICA) as a common origin or transit for emboli, the asymmetry in their final destination has not been fully explained. We hypothesize that the anatomic location of the OA takeoff from the ICA may contribute to the differential flow of small emboli to the retinal circulation vs the cerebral circulation. METHODS: We report a retrospective, comparative, case-control study on 28 patients with retinal ischemia and 26 patients with TIA or cerebral infarction caused by embolic events. All subjects underwent either computed tomography angiography or MRA. The location of the ipsilateral OA origin off the ICA was then graded in a blinded fashion and compared between cohorts. Vascular risk factors were collected for all patients, including age, sex, hypertension, hyperlipidemia, arrhythmia, diabetes, coronary artery disease, and smoking. RESULTS: We find that in patients with retinal ischemia of embolic etiology, the ipsilateral OA takeoff from the ICA is more proximal than in patients with cerebral infarcts or TIA (P = 0.0002). We found no statistically significant differences in demographic, vascular, or systemic risk factors. CONCLUSIONS: We find that the mean anatomical location of the OA takeoff from the ICA is significantly more proximal in patients with MVL due to retinal ischemia compared with patients with TIA or cerebral ischemia. This finding contributes significantly to our understanding of a long observed but poorly understood phenomenon that patients with MVL are less likely to have concurrent cerebral ischemia than are patients with TIA.

Glioblastoma multiforme mimicking optic neuritis.

PURPOSE: To present a case of glioblastoma multiforme which initially presented with only ophthalmic manifestations. OBSERVATIONS: A 48-year-old man presented with decreased vision and pain with eye movements of the right eye. MRI of the brain showed increased T2/FLAIR signal involving the right optic nerve with no other identified abnormalities. He was treated with intravenous steroids for presumed optic neuritis. His visual acuity then rapidly worsened to no light perception, with new orbital apex symptoms including central retinal artery and vein occlusions and inferior division third and fourth nerve palsies. Repeat MRI with contrast showed perineural enhancement surrounding the right optic nerve and markedly reduced diffusion along its entire course. After an unrevealing initial workup and then a 7 month period during which the patient refused follow up, he re-presented with left sided weakness, headache, and confusion. Repeat brain MRI revealed a large mass involving the right optic nerve, optic chiasm, basal ganglia, corpus callosum and brainstem. Biopsy led to a diagnosis of WHO grade IV glioblastoma multiforme. The disease was poorly responsive to temozolomide, bevacizumab and external beam radiation, and the patient passed away 5 months later. CONCLUSIONS AND IMPORTANCE: Malignant optic glioma of adulthood is a challenging diagnosis with a poor prognosis. This rare case highlights the importance of maintaining neoplasm in the differential for optic neuritis masqueraders.

Older age and larger cyst size in children with spontaneous rupture of periorbital dermoid cysts.

We analyzed clinical and histopathologic data of 97 pediatric patients who underwent excision of dermoid cysts. On review, 16.5% of the sample population demonstrated localized chronic inflammatory changes, including the presence of giant cells and epithelial disruption. These features were considered indicative of prior cyst rupture. Age at time of initial presentation was significantly older and cyst size was significantly larger in patients with histopathologic signs of previous rupture. Longer time to presentation and time to excision were associated with increased odds of spontaneous rupture.

Spontaneous cerebrospinal fluid leak and idiopathic intracranial hypertension.

PURPOSE OF REVIEW: To review the most recent literature on the relationship of spontaneous cerebrospinal fluid (CSF) leak with idiopathic intracranial hypertension (IIH) and considerations in management of these conditions. RECENT FINDINGS: A link has been proposed between spontaneous CSF leak and IIH based on similar demographics, radiologic, and clinical findings in these patients, and on a plausible mechanism of skull base erosion in the setting of high CSF pressure over time. IIH patients with CSF leak may not present with classic IIH signs and symptoms as the leak can alleviate excess pressure; however, they may develop these after a leak is repaired. There may also be a higher risk of leak recurrence if intracranial hypertension is not treated postoperatively. SUMMARY: A growing body of evidence supports an association between IIH and spontaneous CSF leak. However, this relationship is still not fully elucidated, and there is no current agreement on how to incorporate screening, management, or counseling guidelines for CSF leak into the care of IIH patients. There are also no specific guidelines for evaluation/management of IIH in patients with spontaneous CSF leak. Further interdisciplinary research is needed to explore this connection and to establish screening, evaluation, and management guidelines.

Neuro-Ophthalmic Disorders in Pregnancy.

The physiologic changes that accompany pregnancy can have important implications for neuro-ophthalmic disease. This article discusses pregnancy-related considerations for meningioma, pituitary disorders, demyelinating disease, myasthenia gravis, thyroid eye disease, idiopathic intracranial hypertension, cerebral venous sinus thrombosis, stroke, migraine, and cranial neuropathies. The article also details the potential neuro-ophthalmic complications of preeclampsia and eclampsia and reviews the use of common diagnostic studies during pregnancy.

Complications of Optic Nerve Sheath Fenestration as a Treatment for Idiopathic Intracranial Hypertension.

There are a number of surgical options for treatment of idiopathic intracranial hypertension (IIH) when it is refractory to medical treatment and weight loss. Optic nerve sheath fenestration (ONSF) is one of these options. Use of this procedure varies among centers due to experience with the procedure and concern for associated complications that can result in severe loss of vision. This review summarizes the literature concerning post-surgical complications of ONSF for IIH.

Perioperative Vision Loss after Non-Ocular Surgery.

Perioperative vision loss (POVL) may cause devastating visual morbidity. A prompt anatomical and etiologic diagnosis is paramount to guide management and assess prognosis. Where possible, steps should be undertaken to minimize risk of POVL for vulnerable patients undergoing high-risk procedures. We review the specific risk factors, pathophysiology, and management and prevention strategies for various etiologies of POVL.

Duane syndrome with prominent oculo-auricular phenomenon.

Duane syndrome is a congenital cranial dysinnervation disorder involving absent or anomalous innervation of the lateral and medial rectus muscles that is sometimes associated with other manifestations of dysinnervation. We describe a patient with right esotropic Duane syndrome with a long-standing retroauricular tugging sensation in right gaze who was noted to have prominent ipsilateral oculo-auricular phenomenon, representing either abnormal enhancement of existing innervation or an uncommon dysinnervation. After successful strabismus surgery the tugging sensation improved but the phenomenon could still be elicited.

Treatment of Amblyopia and Amblyopia Risk Factors Based on Current Evidence.

PURPOSE: Amblyopia is a leading cause of low vision and warrants timely management during childhood. We performed a literature review of the management of amblyopia and potential risk factors for amblyopia. METHODS: Literature review of the management of amblyopia and risk factors for amblyopia. RESULTS: Common amblyopia risk factors include anisometropic or high refractive error, strabismus, cataract, and ptosis. Often a conservative approach with spectacles is enough to prevent amblyopia. However, surgery may be necessary to clear the visual axis or align the eyes. CONCLUSION: Amblyopia risk factors should be managed early. Though amblyopia treatment is more likely to be successful at a younger age, those who are older but treatment-naïve may still respond to treatment. Promoting binocular or dichoptic experiences may be the future direction of amblyopia management.

Update on the evaluation of pediatric idiopathic intracranial hypertension.

PURPOSE OF REVIEW: Papilledema associated with idiopathic intracranial hypertension (IIH) may result in irreversible, progressive visual loss. The development of tools for the evaluation of pediatric patients with IIH is particularly relevant as many patients may not be able to comply with the detailed clinical evaluation utilized in adults for the treatment and management of this disease. The purpose of this review is to summarize relevant articles on the diagnostic tools used in evaluation and management of pediatric IIH. RECENT FINDINGS: Studies suggest that characteristic pediatric IIH MRI findings include empty sella turcica, decreased pituitary gland size, optic nerve tortuosity, perioptic subarachnoid space enlargement, posterior globe flattering, and intraocular protrusion of the optic nerve head. On optical coherence tomography (OCT), increased retinal nerve fiber layer and macular thickness may be observed in children with IIH compared with controls. The retinal nerve fiber layer thickness seems to coincide with the severity of papilledema and may be more sensitive than funduscopy for detecting optic nerve head elevation. Research on ultrasound of the optic nerve shows increased size of the optic nerve sheath diameter in pediatric IIH patients, and this may correlate with increased opening pressure on lumbar puncture. SUMMARY: There appears to be characteristic findings on MRI, OCT, and ultrasound studies in pediatric IIH patients. Although ultrasound is rarely used for monitoring these patients nowadays, MRI and OCT can be useful in the evaluation and management of these individuals.

Keeping an eye on dialysis: the association of hemodialysis with intraocular hypertension.

Intraocular hypertension is common during hemodialysis. Dialysis disequilibrium syndrome and intraocular hypertension occur via similar pathophysiologic mechanisms. These mechanisms may contribute to the development of glaucoma and cataracts in a patient population already at high risk for ocular abnormalities, given the common risk factors for chronic kidney disease and impaired aqueous humor outflow. We describe a patient with complicated diabetes mellitus, end-stage renal disease, and recent cataract surgery who developed severe intraocular hypertension during hemodialysis. We recommend increased awareness of the symptoms of intraocular hypertension and subsequent ophthalmologic surveillance in order to prevent long-term visual complications.

Correlation of clinical profile and specific histopathological features of temporal artery biopsies.

BACKGROUND: This study sought to correlate the clinical features of patients with giant cell arteritis (GCA) who present with ophthalmic symptoms and signs, with 2 specific histopathological findings-the presence of giant cells and arterial wall neoangiogenesis. The goal was to assess if these pathological features might be useful in guiding the approach to patient management. METHODS: Medical charts were retrospectively reviewed from 58 patients who underwent a temporal artery biopsy at a single institution. Detailed information was collected about the clinical presentation and course, with an emphasis on visual function. Histopathological and immunohistochemical techniques were used to examine temporal artery biopsies for evidence of inflammation. Correlations were made between the clinical data and the presence of giant cells and neoangiogenesis. RESULTS: Twenty-one (34%) biopsies were positive for inflammation consistent with GCA. Although the percentage of positive biopsies with giant cells was high, neither the presence of giant cells nor neoangiogenesis was predictive of a patient's presenting visual symptoms, severity and bilaterality of vision loss, other ophthalmic manifestations of GCA, presence of headache or jaw claudication, or erythrocyte sedimentation rate. Giant cells were more common in patients with recent weight loss. Immunohistochemistry confirmed diagnoses but did not alter the clinical course or treatment plan. CONCLUSIONS: There was no correlation between the clinical, specifically visual, features of GCA and the presence or absence of giant cells or neoangiogenesis in temporal artery biopsy specimens. Although the presence of neoangiogenesis may be important in the pathogenesis of GCA, our study showed no correlation between this finding and the clinical course.

A comparison of retrokeratoprosthetic membrane and conjunctival inflammatory responses to silicone oil.

Silicone oil continues to be an important aid in retinal detachment surgery. We report a case in which disparate responses to silicone oil were noted in the conjunctiva and intraocularly. Intraocularly, the oil permeated a fibrous membrane that formed behind a keratoprosthesis, the first example of this phenomenon. We detail the histological response to the oil at this site as well as a distinctly different reaction present to oil in the conjunctiva of the same eye. The divergence of histological responses provides a demonstration of the eye's apparent retained capacity to protect against intraocular inflammation, despite multiple previous surgeries.

Blepharospasm in a patient with pontine capillary telangiectasia.

Blepharospasm is rarely due to an identifiable etiology. In the majority of cases, imaging fails to reveal any structural lesion. Here we describe an otherwise healthy patient with blepharospasm who was found to have pontine capillary telangiectasia. We propose a potential association between blepharospasm and pontine capillary telangiectasia.