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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.

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.

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.

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.

1. Fisherman GA, Birch DG, Holder GE, Brigell MG. Ophthalmology Monograph 2. Electrophysiologic Testing in Disorders of the Retina, Optic Nerve, and Visual Pathway. 2nd ed. San Francisco: American Academy of Ophthalmology; 2001. 2. Gitlits VM, Toh BH, Sentry JW. Disease association, origin, and clinical relevance of autoantibodies to the glycolytic enzyme enolase. J Investig Med. 2001;49:138-45. 3. Adamus G, Reng G, Weleber RG. Autoantibodies against retinal proteins in paraneoplastic and autoimmune retinopathy. BMC Ophthalmol. 2004;4:5-14. 4. Ares-Luque A, Garcia-Tunon LA, Saiz A, et al. Isolated paraneoplastic optic neuropathy associated with small-cell lung cancer and anti-CV2 antibodies. J Neurol. 2007;254:1131-2.

Nonarteritic ischemic optic neuropathy (NAION) is one of the most prevalent optic nerve disorders seen in ophthalmic practice. The role of corticosteroid therapy in NAION remains a highly controversial area of debate in ophthalmology. This brief review will provide an overview of the current clinical evidence on this topic as well as some comment on the medical debate.

To provide a clinical update of the neuro-ophthalmology literature over the last twelve months.This is an annual review of current literature from August 1, 2011 to August 1, 2012.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.We reviewed the literature over the past year in neuro-ophthalmology of potential interest and relevance to the comprehensive ophthalmologist.This annual review provides a brief update on a number of neuroophthalmic conditions that might be of interest to the practicing clinical ophthalmologist.

A 70-year-old woman presented with a one-month history of progressive, painful loss of vision in the right eye (OD), a droopy right upper eyelid, and horizontal binocular diplopia. Past medical history was significant for hypertension, diabetes mellitus type II, and breast cancer (status post-mastectomy and -chemotherapy). Review of systems was significant for nausea, vomiting, and malaise. On examination, visual acuity was light perception OD and 20/25 in the left eye (OS). The right pupil measured 5 mm in the dark and was non-reactive to light or accommodation with a relative afferent pupillary defect (RAPD) OD (by reverse testing). The left pupil measured 4 mm in the dark and 3 mm in the light with no light-near dissociation. Ocular motility revealed complete ophthalmoplegia OD but full extraocular movements OS (Fig. 13.1). Anterior segment examination and intraocular pressure measurements were normal in both eyes (OU). External exam showed complete ptosis OD. Hertel exophthalmometry showed 3mm of proptosis OD (23mm OD compared with 20mm OS). Dilated fundus exam revealed diffuse optic disc pallor OD and a normal fundus OS. Magnetic resonance imaging (MRI) of the brain and orbits with and without contrast showed a dural-based extra-axial lesion located within the sella and extending into the right-sided cavernous sinus, superior orbital fissure, planum sphenoidale, and infundibulum (Fig. 13.2). Trans-sphenoidal biopsy revealed diffuse large B-cell lymphoma. The patient underwent staging and was treated with systemic chemotherapy and radiotherapy.

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.