The Translaminar Pressure Gradient: Papilledema After Trabeculectomy Treated With Optic Nerve Sheath Fenestration.

INTRODUCTION: Trans-lamina cribrosa pressure has been postulated to be a contributor in the development of a glaucomatous optic nerve versus optic nerve edema, depending on the pressure gradient. Uncertainty remains in the therapeutic outcome of adjusting this gradient. CASE REPORT: We discuss a unique case of idiopathic intracranial hypertension presenting as asymmetric optic disc edema following trabeculectomy. It was treated via optic nerve fenestration due to the patient remaining symptomatic on maximum tolerated acetazolamide. Intraocular pressure stabilized into target range and the optic nerve edema resolved. CONCLUSIONS: Rarely, intraocular pressure reduction can unmask elevated intracranial pressure, leading to optic nerve edema. Optic nerve sheath fenestration is a practical therapeutic modality to consider when treating this occurrence.

Perioperative Vision Loss in Cervical Spinal Surgery.

STUDY DESIGN: Retrospective multicenter case series. OBJECTIVE: To assess the rate of perioperative vision loss following cervical spinal surgery. METHODS: Medical records for 17 625 patients from 21 high-volume surgical centers from the AOSpine North America Clinical Research Network who received cervical spine surgery (levels from C2 to C7) between January 1, 2005, and December 31, 2011, inclusive, were reviewed to identify occurrences of vision loss following surgery. RESULTS: Of the 17 625 patients in the registry, there were 13 946 patients assessed for the complication of blindness. There were 9591 cases that involved only anterior surgical approaches; the remaining 4355 cases were posterior and/or circumferential fusions. There were no cases of blindness or vision loss in the postoperative period reported during the sampling period. CONCLUSIONS: Perioperative vision loss following cervical spinal surgery is exceedingly rare.

Improvement in Visual Fields After Treatment of Intracranial Meningioma With Bevacizumab.

High-grade (World Health Organization [WHO] Grade II and III) meningiomas constitute a minority of all meningioma cases but are associated with significant morbidity and mortality, due to more aggressive tumor behavior and a tendency to recur despite standard therapy with resection and radiotherapy. They display a higher degree of vascularity than WHO Grade I meningiomas and produce angiogenic and growth factors, including vascular endothelial growth factor (VEGF). Bevacizumab, a humanized monoclonal antibody against VEGF-A, has been used in the treatment of recurrent or progressive meningiomas resistant to standard therapy. We report a patient with a recurrent left frontotemporal meningioma and associated-vision loss who experienced substantial visual field recovery after 3 cycles of bevacizumab. In addition, we provide a review of the literature regarding the efficacy of bevacizumab in the treatment of recurrent meningiomas.

Concomitant presentation of three rare mesencephalic syndromes: case report.

We describe a unique case of concomitant presentation of three rare mesencephalic syndromes. A 48-year-old man with an acute stoke was found to have an unusual combination of three rare mesencephalic syndromes after detailed neuro-ophthalmic evaluation: the plus-minus lid syndrome, the vertical one-and-a-half syndrome, and Claude's syndrome. We discuss the clinical and anatomical localization of these syndromes. This was corroborated by magnetic resonance imaging (MRI) which revealed areas of infarction at the thalamo-mesencephalic junction and the right rostral midbrain involving the third nerve fascicle and the red nucleus. Our case highlights the importance of a careful ocular motility examination as a tool which has a highly localizing value in the diagnosis of stroke.

Progression of visual field defects in leber hereditary optic neuropathy: experience of the LHON treatment trial.

PURPOSE: To describe the visual fields of patients with Leber hereditary optic neuropathy (LHON), a maternally inherited disorder characterized by bilateral, often sequential vision loss, before and during progressive visual deterioration. DESIGN: Prospective longitudinal follow-up of serial visual fields in patients enrolled onto an open-label, nonrandomized pilot study of topical brimonidine purite as prophylactic treatment after first eye involvement in LHON. METHODS: Nine molecularly confirmed primary mutation patients with LHON with monocular vision loss for less than six months and normal visual function in the other eye were followed prospectively for up to two years. Visual fields were performed on automated perimetry at baseline and on many follow-up visits. RESULTS: Despite normal visual acuity at baseline in all patients, seven patients had some minimal changes in the central visual field of the second eye. All patients had subsequent deterioration of visual acuity, mean deviation, and foveal sensitivity in their second eye. The earliest pattern of abnormality was typically a cecocentral defect enlarging to become a central defect, often with a superior or inferior predilection. The visual field defects in the two eyes of any given patient were remarkably similar. CONCLUSIONS: LHON may be a bilateral condition at onset more frequently than appreciated. Automated static perimetry of the "normal" eye may reveal subclinical findings that typically worsen rapidly over weeks to months to similar central scotomatous damage. Quantitative automated static perimetry is helpful in elucidating the natural history of LHON and in understanding the underlying pathology and pathophysiology of this disease.

Axonal neuropathy with optic atrophy is caused by mutations in mitofusin 2.

OBJECTIVE: Charcot-Marie-Tooth (CMT) neuropathy with visual impairment due to optic atrophy has been designated as hereditary motor and sensory neuropathy type VI (HMSN VI). Reports of affected families have indicated autosomal dominant and recessive forms, but the genetic cause of this disease has remained elusive. METHODS: Here, we describe six HMSN VI families with a subacute onset of optic atrophy and subsequent slow recovery of visual acuity in 60% of the patients. Detailed clinical and genetic studies were performed. RESULTS: In each pedigree, we identified a unique mutation in the gene mitofusin 2 (MFN2). In three families, the MFN2 mutation occurred de novo; in two families the mutation was subsequently transmitted from father to son indicating autosomal dominant inheritance. INTERPRETATION: MFN2 is a mitochondrial membrane protein that was recently reported to cause axonal CMT type 2A. It is intriguing that MFN2 shows functional overlap with optic atrophy 1 (OPA1), the protein underlying the most common form of autosomal dominant optic atrophy, and mitochondrial encoded oxidative phosphorylation components as seen in Leber's hereditary optic atrophy. We conclude that autosomal dominant HMSN VI is caused by mutations in MFN2, emphasizing the important role of mitochondrial function for both optic atrophies and peripheral neuropathies.

Prophylaxis for second eye involvement in leber hereditary optic neuropathy: an open-labeled, nonrandomized multicenter trial of topical brimonidine purite.

PURPOSE: To test a topical agent with purported antiapoptotic properties as prophylactic treatment after first eye involvement in Leber hereditary optic neuropathy (LHON), a maternally-inherited disorder characterized by bilateral, often sequential, visual loss. DESIGN: Open labeled, nonrandomized prospective pilot study. METHODS: Nine primary mutation molecularly confirmed LHON patients with one eye vision loss for less than 6 months and normal visual function in the fellow eye were treated with brimonidine purite 0.15% (Alphagan P) 4 times daily in the unaffected eye for up to 2 years. Visual acuity was the primary efficacy outcome. Secondary measures included changes on automated perimetry and quantification of the relative afferent pupillary defect. RESULTS: There were 8 men and 1 woman enrolled, aged 13 to 54 years (mean 32 years), eight with the 11778 mitochondrial DNA (mtDNA) mutation, and one with the 3460 mutation. Despite normal visual acuity at baseline in all patients, 7 patients had some minimal changes in the central visual field of the study eye. All patients had deterioration of vision in their second eye. In 1 of the 2 patients who had treatment initiated within 16 days after first eye involvement, good visual acuity was maintained in the study eye at 15 month followup, despite a mildly abnormal study eye baseline visual field. CONCLUSIONS: LHON may be a bilateral condition at onset more frequently than appreciated, with asymmetric severity at presentation. Topical brimonidine purite in this dosage was unsuccessful in preventing second eye involvement in recently monocularly-symptomatic LHON.