Amina Malik

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).

Hyperbaric oxygen therapy in the treatment of radiation optic neuropathy

Radiation optic neuropathy (RON) is characterized by the rapid onset of painless irreversible vision loss in one or both eyes. We report 4 consecutive patients who presented with unilateral or asymmetric bilateral RON. All had bilateral optic nerve enhancement on MRI and were treated with hyperbaric oxygen (HBO) and oral corticosteroids. In the less affected eye, 2 of the 4 patients had preservation of vision, while in the other 2 patients, vision declined. No patient showed improvement in the more symptomatic eye, and in 2 patients, there was a significant decline in visual acuity. Our findings suggest that prompt treatment with HBO and oral corticosteroids may result in visual preservation in the less affected eye despite the presence of optic nerve enhancement on MRI.

Optic Neuropathy Due to Chronic Lymphocytic Leukemia Proven With Optic Nerve Sheath Biopsy.

Central nervous system involvement from chronic lymphocytic leukemia (CLL) occurs infrequently, and manifestations include cognitive and cerebellar dysfunction and cranial nerve palsies. We report a 45-year-old man with CLL believed to be in clinical remission, who presented with vision loss and bilateral optic disc edema. His optic neuropathy due to CLL was proven by optic nerve sheath biopsy, and he experienced visual recovery after treatment with ibrutinib and intrathecal methotrexate.

Treatment options for atypical optic neuritis.

Context: Optic neuritis (ON) is defined as inflammation of the optic nerve and can have various etiologies. The most common presentation in the US is demyelinating, or “typical” ON, usually associated with multiple sclerosis. This is in contrast to “atypical” causes of ON, which differ in their clinical presentation, management, and prognosis. These atypical cases are characterized by lack of eye pain, exudates, and hemorrhages on exam, very severe, bilateral or progressive visual loss, or with failure to recover vision. Aims: The aim was to describe the clinical presentations of atypical ON and their treatments. Settings and Design: Review article. Materials and Methods: Literature review. Results: Types of atypical ON identified include neuromyelitis optica, autoimmune optic neuropathy, chronic relapsing inflammatory optic neuropathy, idiopathic recurrent neuroretinitis, and optic neuropathy associated with systemic diseases. Atypical ON usually requires corticosteroid treatment and often will require aggressive immunosuppression. Conclusions: Unlike demyelinating ON, atypical ON requires treatment to preserve vision.

Cerebrospinal fluid diversion procedures in the treatment of patients with idiopathic intracranial hypertension.

Idiopathic intracranial hypertension (IIH) is a disease that usually affects obese women of childbearing age, though it can occur in children, older adults, and individuals of either sex. It has been reported to have an estimated prevalence in the general American population of 0.9 to 1.0 per 100,000. The primary problem in IIH is elevated intracranial pressure (ICP), often manifesting with papilledema, headaches, or transient visual obscurations. The diagnostic criteria as set forth by Dandy in 1937 and later modified by Smith in 1995 include: (1) signs and symptoms of elevated ICP, (2) no localizing neurological signs (with exception of sixth nerve palsy), (3) lumbar puncture done in lateral decubitus position with cerebrospinal fluid (CSF) opening pressure of >25 cm H2O and with normal cytologic and chemical composition, and (5) normal neurological imaging to exclude other causes of increased ICP (usually including MRI/MRV to exclude venous sinus thrombosis). Treatment options for IIH vary according to the clinical course, with the primary goals of treatment being prevention of vision loss and alleviation of symptoms of increased ICP, including headache, nausea, vomiting, memory loss, pulsatile tinnitus, and diplopia from sixth cranial nerve palsy. Most IIH patients do well with medical management (primarily carbonic anhydrase inhibitors), including weight loss. Only a modest degree of weight loss (approximately 5% to 10% of total body weight) usually is required for improvement in signs and symptoms. When IIH is associated with a triggering factor (including certain medications or untreated obstructive sleep apnea), stopping the medication or treating the associated disease can cause rapid improvement. In patients with rapid loss of vision at presentation or in whom medical

Idiopathic Orbital Inflammation Associated with Relapsing Polychondritis

The authors describe a patient with recurrent idiopathic orbital inflammatory disease as an unusual presentation of relapsing polychondritis. There are very few reported cases in the literature of relapsing polychondritis associated with idiopathic orbital inflammation. Clinicians should be aware of the orbital and ophthalmic presentations of relapsing polychondritis.

Necrotizing Fasciitis of the Periorbital Region Complicated by Combined Central Retinal Artery Occlusion, Central Retinal Vein Occlusion, and Posterior Ciliary Occlusion.

A 50 year-old man on immunosuppressive agents presented with left eye vision loss, periorbital swelling, pain, and ophthalmoplegia. The patient was clinically found to have a central retinal artery and vein occlusion. A CT scan was performed which demonstrated intraorbital fat stranding, however the patient lacked sinus disease. The etiology of the orbital infection was held in question. The area was debrided in the operating room, and the specimen demonstrated group A streptococcal species consistent with necrotizing fasciitis. Periorbital necrotizing fasciitis should be suspected in patients with rapidly progressive orbital symptoms without sinus disease as lack of surgical intervention can result in poor outcomes. The unusual aspect to this case is the mechanism of vision loss, as the authors hypothesize that there was vascular infiltration of the infection resulting in the central retinal artery occlusion and central retinal vein occlusion which have not been previously reported secondary to necrotizing fasciitis of the orbit.

Stabilization of Visual Function After Optic Nerve Sheath Fenestration for Optic Nerve Meningocele

A 10-year-old boy with bilateral colobomatous cavitary disc anomalies presented with a 3-month history of vision loss in his right eye. MRI of the head and orbit revealed bilateral tubular cystic enlargement of the optic nerve/optic sheath complex with thickening of the optic nerves without inflammation or neoplasm, suggestive of bilateral optic nerve meningocele. An optic nerve sheath fenestration was performed OD, and he experienced an improvement and stabilization of vision in his right eye during a 1-year follow-up period. The authors recommend that surgical decompression, particularly optic nerve sheath fenestration, should be considered in cases with progressive vision loss due to optic nerve meningocele.