Visual field defects after radiosurgery for mesial temporal lobe epilepsy.

PURPOSE: Gamma knife radiosurgery (RS) may be an alternative to open surgery for mesial temporal lobe epilepsy (MTLE), but morbidities and the anticonvulsant mechanisms of RS are unclear. Examination of visual field defects (VFDs) after RS may provide evidence of the extent of a postoperative fixed lesion. VFDs occur in 52-100% of patients following open surgery for MTLE. METHODS: This multicenter prospective trial of RS enrolled patients with unilateral hippocampal sclerosis and concordant video-electroencephalography (EEG) findings. Patients were randomized to low (20 Gy) or high (24 Gy) doses delivered to the amygdala, hippocampal head, and parahippocampal gyrus. Postoperative perimetry were obtained at 24 months after RS. Visual field defect ratios (VFDRs) were calculated to quantify the degree of VFDs. Results were contrasted with age, RS dose and 50% isodose volume, peak volume of radiation-induced change at the surgical target, quality of life measurements, and seizure remission. KEY FINDINGS: No patients reported visual changes and no patients had abnormal bedside visual field examinations. Fifteen (62.5%) of 24 patients had postoperative VFDs, all homonymous superior quadrantanopsias. None of the VFDs were consistent with injury to the optic nerve or chiasm. Clinical diagnosis of VFDs correlated significantly with VFDRs (p = 0.0005). Patients with seizure remission had smaller (more severe) VFDRs (p = 0.04). No other variables had significant correlations. SIGNIFICANCE: VFDs appeared after RS in proportions similar to historical comparisons from open surgery for MTLE. The nature of VFDs was consistent with lesions of the optic radiations. The findings support the hypothesis that the mechanism of RS involves some degree of tissue damage and is not confined entirely to functional changes in neuromodulation.

Orbital Surgery: Evolution and Revolution.

This article traces the development of orbital surgery and its subsequent modifications. It also points out the importance of defining one's goal before embarking on orbital surgery. Although generally considered part of ophthalmology, surgery on the orbit has been relatively neglected and not routinely practiced. This article reviews the history of development of orbital surgery, both the revolutionary ideas and the evolutionary changes. There are multiple orbital lesions that do not need to be treated with surgery at all. These days chemotherapy, radation therapy, or even immunotherapy may be more appropriate. The most common orbital pathology, that is thyroid orbitopathy, the physician needs to decide whether or not the orbit needs to be decompressed or whether there are problems related to motility that can be dealt with by eye muscle surgery.

Comparison of macular optical coherence tomography findings between postacute retinal artery occlusion and nonacute optic neuropathy.

PURPOSE: The purpose of this study was to report differentiating optical coherence tomography (OCT) findings between postacute retinal arterial occlusion (PARAO) and nonacute optic neuropathy (NAON). METHODS: A retrospective observational comparative study included 17 eyes with postacute permanent retinal arterial occlusion and 32 eyes with NAON whose assessment included a fast macular Status OCT. The macular OCT changes in the postacute phase of central retinal arterial occlusion and branch retinal arterial occlusion were, respectively, compared with those in diffuse and segmental NAON in an effort to establish differentiating features. The findings were used in the workup of four additional consecutive cases referred with suspected NAON. RESULTS: Three main features differentiating PARAO from NAON were identified on macular OCT. Complete inner retinal atrophy with loss of the normal stratification of the inner retinal layers, loss of the normal foveal depression, and marked thinning of the involved retina were characteristic findings of PARAO and were absent even in the most severe cases of NAON. The involved retina was significantly thinner in PARAO compared with that in NAON (P < 0.05). These OCT features helped establish the diagnosis of PARAO in four additional consecutive cases referred with the misdiagnosis of NAON. CONCLUSION: The extent and pattern of inner retinal atrophy differentiate PARAO from NAON and help guide the systemic workup.

Rarebit perimetry for bedside testing: comparison with standard automated perimetry.

BACKGROUND: Rarebit perimetry (RBP), a technique developed for the detection of early damage to the afferent visual system, has not been extensively tested at the bedside. This study was designed to test the feasibility of bedside testing with RBP in comparison with standard automated perimetry (SAP) performed in the clinic. METHODS: We tested 29 eyes of 15 subjects admitted with neurologic or neurosurgical diseases affecting the afferent visual system. RBP was performed on a laptop computer at the bedside. SAP (Humphrey field analyzer) testing was performed later in the clinic. Results were evaluated by a masked neuro-ophthalmologist. RESULTS: Visual fields corresponded between RBP and SAP in 21(72%) of the 29 tested eyes. RBP detected defects in 5 subjects who had normal visual field results on SAP. All subjects preferred RBP for convenience. CONCLUSION: RBP is a convenient method of bedside visual field testing and is no less sensitive to visual field defects in this role than SAP.

Doxycycline treatment of high-risk COVID-19-positive patients with comorbid pulmonary disease.

Infection with novel SARS-CoV-2 carries significant morbidity and mortality in patients with pulmonary compromise, such as lung cancer, autoimmune disease, and pneumonia. For early stages of mild to moderate disease, care is entirely supportive.Antiviral drugs such as remdesivir may be of some benefit but are reserved for severe cases given limited availability and potential toxicity. Repurposing of safer, established medications that may have antiviral activity is a possible approach for treatment of earlier-stage disease. Tetracycline and its derivatives (e.g. doxycycline and minocycline) are nontraditional antibiotics with a well-established safety profile, potential efficacy against viral pathogens such as dengue fever and chikungunya, and may regulate pathways important in initial infection, replication, and systemic response to SARS-CoV-2. We present a series of four high-risk, symptomatic, COVID-19+ patients, with known pulmonary disease, treated with doxycycline with subsequent rapid clinical improvement. No safety issues were noted with use of doxycycline.Doxycycline is an attractive candidate as a repurposed drug in the treatment of COVID-19 infection, with an established safety profile, strong preclinical rationale, and compelling initial clinical experience described here.The reviews of this paper are available via the supplemental material section.

Ocular neuromyotonia after gamma knife stereotactic radiation therapy.

Three patients who underwent multiple intracranial operations for recurrent nonsecreting pituitary adenomas followed by gamma knife stereotactic radiosurgery developed diplopia at 1, 5, and 6 years after the treatments. Examination disclosed features of ocular neuromyotonia, a phenomenon attributed to radiation damage to ocular motor cranial nerves. Amply reported after external beam radiotherapy, neuromyotonia has not been described after radiosurgery previously. These patients are, however, exceptional in that all had undergone multiple sellar region operations or received high doses of radiotherapy, or both.

Visual field defects after radiosurgery versus temporal lobectomy for mesial temporal lobe epilepsy: Findings of the ROSE trial.

PURPOSE: Stereotactic radiosurgery (SRS) may be an alternative to anterior temporal lobectomy (ATL) for mesial temporal lobe epilepsy (MTLE). Visual field defects (VFD) occur in 9-100% of patients following open surgery for MTLE. Postoperative VFD after minimally invasive versus open surgery may differ. METHODS: This prospective trial randomized patients with unilateral hippocampal sclerosis and concordant video-EEG findings to SRS versus ATL. Humphries perimetry was obtained at 24 m after surgery. VFD ratios (VFDR = proportion of missing homonymous hemifield with 0 = no VFD, 0.5 = complete superior quadrantanopsia) quantified VFD. Regressions of VFDR were evaluated against treatment arm and covariates. MRI evaluated effects of volume changes on VFDR. The relationships of VFDR with seizure remission and driving status 3 years after surgery were evaluated. RESULTS: No patients reported visual changes or had abnormal bedside examinations, but 49 of 54 (91%) of patients experienced VFD on formal perimetry. Neither incidence nor severity of VFDR differed significantly by treatment arm. VFDR severity was not associated with seizure remission or driving status. CONCLUSION: The nature of VFD was consistent with lesions of the optic radiations. Effective surgery (defined by seizure remission) of the mesial temporal lobe results in about a 90% incidence of typical VFD regardless of method.

Chiari malformations in patients with uncorrected sagittal synostosis.

BACKGROUND: Single suture craniosynostoses were long considered a benign condition with little risk of complications. In many cases, parents may forego surgical correction because of the inherent risks of surgical intervention. CASE DESCRIPTION: We report 2 cases of uncorrected sagittal synostosis associated with the development of Chiari malformations with syringomyelia later in life. To our knowledge, this is the first report of symptomatic Chiari malformations associated with uncorrected sagittal synostosis. We discuss mode of presentation and management in each case. We propose that these patients either have a pathophysiological predisposition to Chiari malformations based on the compensatory bone growth pattern in sagittal synostosis resulting in craniocephalic disproportion or that there may be an underlying genetic alteration accounting for the association of the 2 processes. CONCLUSIONS: Further investigations are warranted to investigate whether the incidence of Chiari malformations is decreased in patients who have undergone early correction of an isolated sagittal synostosis. We conclude that patients with sagittal synostosis should be evaluated for Chiari malformations before being discharged permanently from a neurosurgical practice.

Aberrant regeneration of the oculomotor nerve: implications for neurosurgeons.

Aberrant regeneration of cranial nerve III, otherwise known as oculomotor synkinesis, is an uncommon but well-described phenomenon most frequently resulting from trauma, tumors, and aneurysms. Its appearance usually follows an oculomotor palsy, but it can also occur primarily without any preceding nerve dysfunction. It is vital that neurosurgeons recognize this disorder because it may be the only sign of an underlying cavernous tumor or PCoA aneurysm. The tumor most often implicated is a cavernous or parasellar meningioma, but any tumor that causes compression or disruption along the course of the oculomotor nerve may cause primary or secondary misdirection. The most common clinical signs of oculomotor synkinesis consist of elevation of the upper eyelid on attempted downward gaze or adduction, adduction of the eye on attempted upward or downward gaze, and constriction of the pupil on attempted adduction. The authors present the largest series of patients with oculomotor synkinesis, including those in whom it developed after neurosurgical intervention, to illustrate various presentations. In addition, the various mechanisms that contribute to synkinesis are reviewed. Last, the treatment strategies for both oculomotor palsies and synkinesis are discussed.

Sparing the eye in craniofacial surgery for superior nasal vault malignant neoplasms: analysis of benefit.

OBJECTIVE: To evaluate orbital and ophthalmologic function following standardized eye-sparing protocol for treating superior nasal vault malignant neoplasms. DESIGN: A retrospective review of patients with sinonasal malignant neoplasms who underwent preoperative radiation therapy with or without chemotherapy and craniofacial resection (CFR) with eye preservation. Pretreatment and posttreatment ophthalmologic evaluations were performed. RESULTS: A total of 59 patients underwent eye-sparing therapy between 1983 and 2005; 36 had invasion of the lamina with or without periorbital resection (61%). The most common abnormality was esthesioneuroblastoma (37 cases; 63%), most of which were Kadish stage C (n = 26). The most common preoperative ophthalmologic findings were motility disturbances (18 cases; 31%), afferent pupillary dysfunction and change in acuity (17 cases; 29%), and proptosis (17 cases; 29%). A total of 36 patients were available for long-term follow-up (mean follow-up, 61 months); 35 of these retained functional vision (97%); and 1 patient had a nonfunctional eye. CONCLUSIONS: Standardized treatment for superior nasal vault malignant neoplasms using an eye-sparing CFR remains a sound approach both oncologically and ophthalmologically. Most patients with advanced disease present with ophthalmologic findings, and most posttreatment eye findings are notable only on detailed examination and either resolve spontaneously in the acute-care setting or do not require surgical repair.

Transorbital Approach for Endovascular Occlusion of Carotid-Cavernous Fistulas: Technical Note and Review of the Literature.

Carotid-cavernous fistulas (CCFs) pose an anatomically and physiologically challenging problem for clinicians. The most common method of treatment for these lesions is transvenous endovascular embolization via the inferior petrosal sinus or the facial vein. When transvenous access is not possible, an alternate approach must be devised. We describe a case example with bilateral Barrow Type B CCFs, which were inaccessible using the traditional transvenous approach. Hence, a direct transorbital approach, performed under fluoroscopic guidance, was employed to successfully obliterate the CCF. At five months follow-up, the patient was recovering without complications. This case delineates the technical aspects of transorbital CCF embolization and demonstrates that this approach is a viable alternative to conventional transvenous methods for appropriately selected CCF cases. We supplement our case example and technical note with a literature review of this approach.

Venous sinus stenting for reduction of intracranial pressure in IIH: a prospective pilot study.

OBJECTIVE Idiopathic intracranial hypertension (IIH) may cause blindness due to elevated intracranial pressure (ICP). Venous sinus stenosis has been identified in select patients, leading to stenting as a potential treatment, but its effects on global ICP have not been completely defined. The purpose of this pilot study was to assess the effects of venous sinus stenting on ICP in a small group of patients with IIH. METHODS Ten patients for whom medical therapy had failed were prospectively followed. Ophthalmological examinations were assessed, and patients with venous sinus stenosis on MR angiography proceeded to catheter angiography, venography with assessment of pressure gradient, and ICP monitoring. Patients with elevated ICP measurements and an elevated pressure gradient across the stenosis were treated with stent placement. RESULTS All patients had elevated venous pressure (mean 39.5 ± 14.9 mm Hg), an elevated gradient across the venous sinus stenosis (30.0 ± 13.2 mm Hg), and elevated ICP (42.2 ± 15.9 mm Hg). Following stent placement, all patients had resolution of the stenosis and gradient (1 ± 1 mm Hg). The ICP values showed an immediate decrease (to a mean of 17.0 ± 8.3 mm Hg), and further decreased overnight (to a mean of 8 ± 4.2 mm Hg). All patients had subjective and objective improvement, and all but one improved during follow-up (median 23.4 months; range 15.7-31.6 months). Two patients developed stent-adjacent stenosis; retreatment abolished the stenosis and gradient in both cases. Patients presenting with papilledema had resolution on follow-up funduscopic imaging and optical coherence tomography (OCT) and improvement on visual field testing. Patients presenting with optic atrophy had optic nerve thinning on follow-up OCT, but improved visual fields. CONCLUSIONS For selected patients with IIH and venous sinus stenosis with an elevated pressure gradient and elevated ICP, venous sinus stenting results in resolution of the venous pressure gradient, reduction in ICP, and functional, neurological, and ophthalmological improvement. As patients are at risk for stent-adjacent stenosis, further follow-up is necessary to determine long-term outcomes and gain an understanding of venous sinus stenosis as a primary or secondary pathological process behind elevated ICP.

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.

Esthesioneuroblastoma: continued follow-up of a single institution's experience.

OBJECTIVES: To analyze outcomes and to provide follow-up for our increasing patient cohort with esthesioneuroblastoma. DESIGN: Retrospective cohort analysis. SETTING: Patients were examined from September 1, 1976, to May 30, 2004, in a tertiary care academic hospital. PATIENTS: Fifty consecutive patients diagnosed as having esthesioneuroblastoma were treated with a standardized protocol during a 28-year period. Patients with tumors staged Kadish A or B received preoperative radiotherapy followed by craniofacial resection, while patients with Kadish stage C disease were treated with preoperative sequential chemotherapy and radiotherapy followed by a craniofacial resection. The mean follow-up is 93 months (range, 1-330 months). RESULTS: The disease-free survival was 86.5% and 82.6% at 5 and 15 years, respectively. There were 17 patients (34%) who developed recurrent disease, most of which was locoregional (12 patients [71%]). There was a long interval to relapse (mean, 6 years), with the longest time to regional recurrence being 10 years. Distant relapses occurred sooner, with poorer outcomes. Of these 17 patients, 7 (41%) underwent successful salvage surgery, while 3 remain alive with disease. CONCLUSIONS: Excellent outcomes for esthesioneuroblastoma are achievable. Long-term follow-up is necessary because of the extended interval for recurrent disease; unlike most sinonasal malignancies, surgical salvage is possible.

Development and validation of a computerized expert system for evaluation of automated visual fields from the Ischemic Optic Neuropathy Decompression Trial.

BACKGROUND: The objective of this report is to describe the methods used to develop and validate a computerized system to analyze Humphrey visual fields obtained from patients with non-arteritic anterior ischemic optic neuropathy (NAION) and enrolled in the Ischemic Optic Neuropathy Decompression Trial (IONDT). The IONDT was a multicenter study that included randomized and non-randomized patients with newly diagnosed NAION in the study eye. At baseline, randomized eyes had visual acuity of 20/64 or worse and non-randomized eyes had visual acuity of better than 20/64 or were associated with patients refusing randomization. Visual fields were measured before treatment using the Humphrey Field Analyzer with the 24-2 program, foveal threshold, and size III stimulus. METHODS: We used visual fields from 189 non-IONDT eyes with NAION to develop the computerized classification system. Six neuro-ophthalmologists ("expert panel") described definitions for visual field patterns defects using 19 visual fields representing a range of pattern defect types. The expert panel then used 120 visual fields, classified using these definitions, to refine the rules, generating revised definitions for 13 visual field pattern defects and 3 levels of severity. These definitions were incorporated into a rule-based computerized classification system run on Excel(R) software. The computerized classification system was used to categorize visual field defects for an additional 95 NAION visual fields, and the expert panel was asked to independently classify the new fields and subsequently whether they agreed with the computer classification. To account for test variability over time, we derived an adjustment factor from the pooled short term fluctuation. We examined change in defects with and without adjustment in visual fields of study participants who demonstrated a visual acuity decrease within 30 days of NAION onset (progressive NAION). RESULTS: Despite an agreed upon set of rules, there was not good agreement among the expert panel when their independent visual classifications were compared. A majority did concur with the computer classification for 91 of 95 visual fields. Remaining classification discrepancies could not be resolved without modifying existing definitions. Without using the adjustment factor, visual fields of 63.6% (14/22) patients with progressive NAION and no central defect, and all (7/7) patients with a paracentral defect, worsened within 30 days of NAION onset. After applying the adjustment factor, the visual fields of the same patients with no initial central defect and 5/7 of the patients with a paracentral defect were seen to worsen. CONCLUSION: The IONDT developed a rule-based computerized system that consistently defines pattern and severity of visual fields of NAION patients for use in a research setting.

Perineural Spread of Head and Neck Cancer: Ophthalmic Considerations.

Head and neck malignancies with orbital involvement present difficult decisions to the treating physician. When the spread is perineural, the challenges are greater due to the incipient nature of the spread and the fact that the orbit can also be involved by centrifugal spread from the non-ophthalmic branches of the trigeminal nerve. The disease is often misdiagnosed and the subsequent delay in treatment results in worse outcomes. This article discusses the evaluation of the eye and the many facets of orbital involvement by perineural spread of malignancy including the treatment of complications.

What's in a name?

A 51-year-old man with rheumatoid arthritis and diabetes mellitus presented with new onset left-sided hemiparesis, left-sided neglect, and left-sided incongruous, denser inferiorly, homonymous hemianopsia. Magnetic resonance image of the brain showed prominent swelling of the right frontal, parietal, and occipital lobes greater than on the left with significant change in the fluid-attenuated inversion recovery signal in the gray matter. Perinuclear antineutrophil cytoplasmic antibodies titers were elevated, and skin biopsy demonstrating leukocytoclastic vasculitis. He showed marked clinical and radiographic improvement in association with recovery of vascular abnormalities after the initiation of prednisone, pulse therapy with methylprednisolone, and methotrexate. Clinicians should be aware of the possibility of neuro-ophthalmic manifestations of rheumatoid arthritis, including rheumatoid arthritis-related vasculitis causing homonymous hemianopsia.

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guideline on Pretreatment Ophthalmology Evaluation in Patients With Suspected Nonfunctioning Pituitary Adenomas.

BACKGROUND: Nonfunctioning pituitary adenomas (NFPAs) are the most frequent pituitary tumors. Visual symptoms from NFPAs are common and include visual field defects, loss of central vision, and motility problems resulting in diplopia. OBJECTIVE: To create evidence-based guidelines in an attempt to formulate guidance for preoperative ophthalmologic evaluation of NFPA patients. METHODS: An extensive literature search was performed. Only clinical articles describing preoperative ophthalmologic evaluation of adult patients with NFPA were included. To ascertain the class of evidence for the posttreatment follow-up, the authors used the Clinical Assessment evidence-based classification. RESULTS: Six studies met the inclusion criteria with respect to the questions regarding the preoperative ophthalmologic evaluation of NFPA patients. Based on the studies located through the search, the authors formulated evidence-based recommendations as they pertain to the necessity of ophthalmologic evaluation before surgical treatment. CONCLUSION: Preoperative ophthalmologic evaluation is recommended. Such evaluation can provide prognostic factors for recovery and, when paired with postoperative evaluation, documents postoperative change. In addition to formal ophthalmologic examination, tests of value include automated static perimetry and optical coherence tomography. Older patients and patients with longer duration (>4 months) of vision loss should be counseled regarding the reduced chance of postoperative vision improvement. The full guidelines document for this chapter can be located at https://www.cns.org/guidelines/guidelines-management-patients-non-functioning-pituitary-adenomas/Chapter_4. ABBREVIATIONS: NFPA, nonfunctioning pituitary adenomaOCT, optical coherence tomography.

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines on the Management of Patients With Nonfunctioning Pituitary Adenomas: Executive Summary.

BACKGROUND: Nonfunctioning pituitary adenomas (NFPAs) are the most frequent pituitary tumors. OBJECTIVE: To create evidence-based guidelines for the initial management of NFPAs. METHODS: A multidisciplinary task force composed of physician volunteers and evidence-based medicine-trained methodologists conducted a systematic review of the literature relevant to the management of NFPAs. To ascertain the class of evidence for the posttreatment follow-ups, the task force used the Clinical Assessment evidence-based classification. RESULTS: Seven topics of importance were chosen for detailed evaluation. The topics addressed include preoperative evaluation, primary treatment, treatment options for residual tumors after surgery, and postoperative patient management. For preoperative patient evaluation, the guideline task force focused on preoperative imaging, preoperative laboratory evaluation, and preoperative ophthalmologic evaluation. For primary treatment, this guideline addresses surgical resection, medical therapy, radiation therapy, the natural history of untreated tumors, surgical methodologies, such as endoscopy, microscopy, or craniotomy, and intraoperative adjuncts like neuronavigation, cerebrospinal fluid diversion, or intraoperative imaging. For residual tumor treatment, the guideline task force evaluated radiation vs observation. Additional topics addressed in this guideline regarding postoperative patient management include the frequency of postoperative imaging, postoperative endocrine evaluation, and postoperative ophthalmologic evaluation. CONCLUSION: Although there is clearly a need for more randomized trials generating higher levels of evidence to help guide physicians managing NFPAs, the existing evidence provided valuable data upon which the guidelines described in the 7 articles generated from this effort are based. The full guidelines document can be located at https://www.cns.org/guidelines/guidelines-management-patients-non-functioning-pituitary-adenomas. ABBREVIATION: NFPA, nonfunctioning pituitary adenoma.