Extensive Cerebral Venous Sinus Thrombosis Due to Medroxyprogesterone Acetate.

This retrospective patient report describes a unique case of cerebral venous sinus thrombosis arising from hormonal contraceptive use, and the management of said thrombosis and its secondary ophthalmic manifestations. The patient initially presented with significant visual decline, headaches, florid disc oedema, and vessel tortuosity, due to extensive sinus thromboses that were causing increased intracranial pressure. It was determined that the root cause of the thrombosis was the use of injectable medroxyprogesterone acetate, leading to a hypercoagulable state. Optic nerve sheath fenestration was performed for this patient as an early intervention. The patient, though ultimately experiencing some vision loss, was able to recover and retain limited unilateral central vision, despite a protracted hospital course. We hope to propose that optic nerve sheath fenestration, in conjunction with anti-coagulation therapy, may be a consideration for patients with similar presentations on an individualised basis, in order to prevent vision loss from cerebral venous sinus thrombosis. Though studies are limited, we believe early intervention may be what allowed our patient to retain some central vision, and suggest more studies be done into the utility of this procedure for patients with this clinical vignette.

Deep Learning and Glaucoma Specialists: The Relative Importance of Optic Disc Features to Predict Glaucoma Referral in Fundus Photographs.

PURPOSE: To develop and validate a deep learning (DL) algorithm that predicts referable glaucomatous optic neuropathy (GON) and optic nerve head (ONH) features from color fundus images, to determine the relative importance of these features in referral decisions by glaucoma specialists (GSs) and the algorithm, and to compare the performance of the algorithm with eye care providers. DESIGN: Development and validation of an algorithm. PARTICIPANTS: Fundus images from screening programs, studies, and a glaucoma clinic. METHODS: A DL algorithm was trained using a retrospective dataset of 86 618 images, assessed for glaucomatous ONH features and referable GON (defined as ONH appearance worrisome enough to justify referral for comprehensive examination) by 43 graders. The algorithm was validated using 3 datasets: dataset A (1205 images, 1 image/patient; 18.1% referable), images adjudicated by panels of GSs; dataset B (9642 images, 1 image/patient; 9.2% referable), images from a diabetic teleretinal screening program; and dataset C (346 images, 1 image/patient; 81.7% referable), images from a glaucoma clinic. MAIN OUTCOME MEASURES: The algorithm was evaluated using the area under the receiver operating characteristic curve (AUC), sensitivity, and specificity for referable GON and glaucomatous ONH features. RESULTS: The algorithm's AUC for referable GON was 0.945 (95% confidence interval [CI], 0.929-0.960) in dataset A, 0.855 (95% CI, 0.841-0.870) in dataset B, and 0.881 (95% CI, 0.838-0.918) in dataset C. Algorithm AUCs ranged between 0.661 and 0.973 for glaucomatous ONH features. The algorithm showed significantly higher sensitivity than 7 of 10 graders not involved in determining the reference standard, including 2 of 3 GSs, and showed higher specificity than 3 graders (including 1 GS), while remaining comparable to others. For both GSs and the algorithm, the most crucial features related to referable GON were: presence of vertical cup-to-disc ratio of 0.7 or more, neuroretinal rim notching, retinal nerve fiber layer defect, and bared circumlinear vessels. CONCLUSIONS: A DL algorithm trained on fundus images alone can detect referable GON with higher sensitivity than and comparable specificity to eye care providers. The algorithm maintained good performance on an independent dataset with diagnoses based on a full glaucoma workup.

Bilateral Adie's Tonic Pupil and Hyperopic Shift in Idiopathic Sclerochoroidal Calcification.

Sclerochoroidal calcification is a rare but recognised ophthalmic manifestation seen mostly in elderly Caucasian individuals. The lesions, often bilateral, appear as yellow-white irregular subretinal lesions usually found along the mid-peripheral fundus. Though typically asymptomatic, sclerochoroidal calcification has rarely been associated with parafoveal involvement, choroidal neovascularisation, and serous detachment of the calcifications. Visual involvement is typically minimal, and neovascularisation is often visually insignificant. We present a rare case of sclerochoroidal calcification in a 64-year-old Caucasian female who presented with painless progressive bilateral vision loss and a hyperoptic shift with subsequent development of bilateral sequential Adie's tonic pupil. To the best of our knowledge, this is the first such report in the English language literature.

Surgical and endovascular interventions in idiopathic intracranial hypertension.

PURPOSE OF REVIEW: Idiopathic intracranial hypertension (IIH) is a potentially blinding disease and may require surgical management when maximal medical treatment has failed. The purpose of this review is to discuss the current literature on surgical and endovascular treatments for IIH. RECENT FINDINGS: The most commonly performed surgical treatments for IIH are cerebrospinal fluid diversion procedures (e.g. ventriculo- and lumbo-peritoneal shunts) and optic nerve sheath fenestration. Controversy still exists about which is the preferred initial surgical treatment for IIH. Emerging procedures include venous sinus stenting in cases with venous sinus stenosis, and bariatric surgery for weight loss. Cranial (suboccipital or subtemporal) decompression was a more popular surgical procedure in the past, but can still have a role in selected cases with impaired cerebrospinal flow dynamics (e.g. Chiari malformation) or after multiple failed conventional surgical procedures. SUMMARY: This review compares and contrasts the surgical management options for IIH.

Transverse and Sigmoid Sinus Dural Arteriovenous Fistula Mimicking Idiopathic Intracranial Hypertension and Carotid Cavernous Fistula.

Intracranial dural arteriovenous fistulas (dAVFs) can produce a variety of symptoms depending on fistula location, size, and venous drainage. Although cavernous sinus fistulas (CCFs) classically present with symptoms of orbital venous congestion due to retrograde venous drainage into the superior ophthalmic vein (i.e. an arterialised "red eye") (Miller NR. Neurosurg Focus 2007;23:1--15), dAVFs not localised to the cavernous sinus rarely present with a "red eye" and instead produce increased intracranial pressure, which can mimic idiopathic intracranial hypertension (IIH). The authors present a unique case of an intracranial dAVF with clinical features suggestive of both CCF and IIH. Clinicians should be aware of this possibility to avoid delayed diagnosis of the intracranial dAVF.

Neuro-ophthalmology as a career.

This essay was written to discuss the reasoning behind the personal decisions made by 2 current neuro-ophthalmology fellows to pursue neuro-ophthalmology as a career. It is meant to enlighten the reader about what role neuro-ophthalmologists play in clinical practice, what makes neuro-ophthalmology unique to all other sub-specialties, and how this contributes to making neuro-ophthalmology not only one of the most medically interesting, yet rewarding sub-specialties in ophthalmology.

Controversies: Optic nerve sheath fenestration versus shunt placement for the treatment of idiopathic intracranial hypertension.

BACKGROUND: Idiopathic intracranial hypertension (IIH) has been increasing in prevalence in the past decade, following the obesity epidemic. When medical treatment fails, surgical treatment options must be considered. However, controversy remains as to which surgical procedure is the preferred surgical option - optic nerve sheath fenestration (ONSF) or cerebrospinal fluid (CSF) shunting - for the long-term treatment of this syndrome. PURPOSE: To provide a clinical update of the pros and cons of ONSF versus shunt placement for the treatment of IIH. DESIGN: This was a retrospective review of the current literature in the English language indexed in PubMed. METHODS: The authors conducted a PubMed search using the following terms: Idiopathic IIH, pseudotumor cerebri, ONSF, CSF shunts, vetriculo-peritoneal shunting, and lumbo-peritoneal shunting. The authors included pertinent and significant original articles, review articles, and case reports, which revealed the new aspects and updates in these topics. RESULTS: The treatment of IIH remains controversial and lacks randomized controlled clinical trial data. Treatment of IIH rests with the determination of the severity of IIH-related visual loss and headache. CONCLUSION: The decision for ONSF versus shunting is somewhat institution and surgeon dependent. ONSF is preferred for patients with visual symptoms whereas shunting is reserved for patients with headache. There are positive and negative aspects of both procedures, and a prospective, randomized, controlled trial is needed (currently underway). This article will hopefully be helpful in allowing the reader to make a more informed decision until that time.

Neuro-ophthalmology Annual Review.

PURPOSE: To provide a clinical update of the neuro-ophthalmology literature over the last twelve months. DESIGN: This is an annual review of current literature from August 1, 2011 to August 1, 2012. METHODS: The authors conducted a one year English language neuro-ophthalmology literature search using PubMed from August 1, 2011 to August 1, 2012 using the following search terms: pupil abnormalities, eye movements, diseases of muscle and musculoskeletal junction, optic nerve disorders, optic neuritis and multiple sclerosis, chiasm and posterior primary visual pathway lesions, increased intracranial pressure and related entities, tumors (e.g., meningioma) and aneurysm affecting the visual pathways, vascular diseases, higher visual functions, advances in neuroimaging, and miscellaneous topics in neuro-ophthalmology. The authors included original articles, review articles, and case reports, which revealed the new aspects and updates in neuro-ophthalmology. Letters to the editor, unpublished work, and abstracts were not included in this annual literature review. We propose to update the practicing clinical ophthalmologist on the most clinically relevant literature from the past year. However, this review is not meant to be all-inclusive and highlights only the literature most applicable to the practicing clinical ophthalmologist. RESULTS: We reviewed the literature over the past year in neuro-ophthalmology of potential interest and relevance to the comprehensive ophthalmologist. CONCLUSION: This annual review provides a brief update on a number of neuroophthalmic conditions that might be of interest to the practicing clinical ophthalmologist.

Unfolded protein response in fuchs endothelial corneal dystrophy: a unifying pathogenic pathway?

PURPOSE: To assess for activation of the unfolded protein response in corneal endothelium of Fuchs endothelial corneal dystrophy patients. DESIGN: Retrospective, comparative case series of laboratory specimens. METHODS: Corneal specimens of patients with Fuchs dystrophy and controls with corneal pathologic features other than Fuchs dystrophy were evaluated by transmission electron microscopy (TEM) to evaluate for structural changes of the rough endoplasmic reticulum in corneal endothelium. TEM images were evaluated for alterations of rough endoplasmic reticulum as a sign of unfolded protein response. Normal autopsy eyes, Fuchs dystrophy corneas, and keratoconus corneas were used for immunohistochemistry. Immunohistochemistry was performed on formalin-fixed, paraffin-embedded sections of patient corneas for 3 unfolded protein response markers (GRP78, the alpha subunit of eukaryotic initiation factor 2, C/EBP homologous protein) and 2 apoptosis markers (caspase 3 and 9). Immunohistochemistry signal quantitation of corneal endothelium for evaluation of marker expression was performed using automated software. Corneal sections were assessed quantitatively for levels of immunohistochemistry marker expression. RESULTS: TEM showed enlargement of rough endoplasmic reticulum in corneal endothelium of all Fuchs dystrophy specimens. Immunohistochemistry quantitation demonstrated a significant increase in mean signal in corneal endothelium from Fuchs dystrophy patients for markers GRP78, the alpha subunit of eukaryotic initiation factor 2, C/EBP homologous protein, and caspase 9 compared with non-Fuchs dystrophy corneas (P < .05). CONCLUSIONS: Results of both TEM and immunohistochemistry indicate activation of unfolded protein response in Fuchs dystrophy. Unfolded protein response activation leads to endothelial cell apoptosis in Fuchs dystrophy and may play a central pathogenic role in this disease.