Arielle Spitze

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.

Optic nerve sheath fenestration vs cerebrospinal diversion procedures: what is the preferred surgical procedure for the treatment of idiopathic intracranial hypertension failing maximum medical therapy?

Although most patients with idiopathic intracranial hypertension (IIH) can be effectively treated with conservative measures, such as lumbar puncture, weight loss, acetazolamide, medical treatment of headaches, surgery is sometimes necessary, particularly in patients with visual loss secondary to chronic papilledema (1–4). Recently, endovascular venous stenting of a stenosed dominant intracranial transverse venous sinus has been proposed as a possible treatment (5); however, cerebrospinal fluid (CSF) shunting and optic nerve sheath fenestration (ONSF) remain among the most commonly used surgical procedures to treat IIH in the United States (6). In the absence of any prospective, randomized clinical trials comparing these procedures for the treatment of IIH, opinions vary greatly between ONSF and CSF shunting procedures as the most appropriate recommended surgical treatment (7,8). The decision to use one or the other is often based on local preferences and the availability of specific surgeons; some centers always perform ONSF as a first-line treatment, some use both procedures based on patient’s symptoms and signs (ONSF for visual loss and shunts for headaches), while others exclusively recommend lumboperitoneal shunt (LPS) or ventriculoperitoneal shunt (VPS) when surgery is necessary (9).

Pallid disc edema and choroidal perfusion delay in posthemodialysis nonarteritic ischemic optic neuropathy

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.

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.

Systemic corticosteroids in nonarteritic ischemic optic neuropathy

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.

Fourth nerve palsy

What is fourth nerve palsy? There are 12 pairs of nerves (cranial nerves) that control most of the functions of the head and neck. These functions include eye movements, swallowing, facial sensation, and other facial movements. One of those nerves is the fourth cranial nerve. The fourth cranial nerve controls a specific eye movement. If this nerve is not functioning properly on one or both sides of the head, it is called a “palsy.” You may experience double vision and the need to tilt your head. A fourth nerve palsy usually only affects one eye, but it can affect both eyes.

Orbital apex syndrome

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.

Non-arteritic anterior ischemic optic neuropathy (NAION)

A 65-year-old male presented with acute, painless vision loss in the lower portion of his left eye (OS) for one day. Past medical history was significant for hypertension, hyperlipidemia, and coronary artery disease with previous coronary artery stenting. Significant medications included aspirin and clopidogrel (Plavix). He smoked one pack of cigarettes per day for many years. He denied jaw claudication, scalp pain, headache, fever, chills, or malaise and had no history of obstructive sleep apnea. He was not using any phosphodiesterase inhibitors (erectile dysfunction medications). External examination was negative for temporal nodularity or scalp tenderness. Visual acuity was 20/20 in the right eye (OD) and 20/400 OS. The pupils measured 4 mm in the dark and 2 mm in the light in both eyes (OU), but there was a relative afferent pupillary defect (RAPD) OS. The remainder of the anterior segment examination was within normal limits. Automated visual field testing (Humphrey 24-2) was normal OD but showed a dense inferior arcuate field defect OS (Fig. 6.1). Dilated fundus examination OD was normal and showed a cup-to-disc ratio of 0.1 (Fig. 6.2). There was superior (sectoral) disc edema OS (Fig. 6.3) that resolved over time to residual sectoral optic atrophy OS. Optical coherence tomography (OCT) of the optic nerve OS showed a thinned superior nerve fiber layer (Fig. 6.4) six months later. Laboratory workup at the time of

Transverse and sigmoid sinus dural arteriovenous fistula mimicking idiopathic intracranial hypertension and carotid cavernous fistula

Abstract 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.