Mismatch in Supply and Demand for Neuro-Ophthalmic Care.

BACKGROUND: Previous research suggests the number of neuro-ophthalmologists in the United States may be below a level that provides sufficient access to neuro-ophthalmic care in much of the United States. However, national estimates of the amount of clinical time spent on neuro-ophthalmology are lacking. METHODS: The North American Neuro-Ophthalmology Society administered a survey on professional time allocation to its active members. Survey response was 95%. The survey characterized the hours each week each respondent allocated to overall work, clinical work, clinical work in ophthalmology/neurology, and clinical work in neuro-ophthalmology specifically. The survey additionally collected information regarding demographics, current wait times to be seen for new patients, and the difference in clinical time spent in neuro-ophthalmology spent between the current day compared with that shortly after completing clinical training. Linear regression was used to identify potential relationships between the above and average wait time. RESULTS: On average, responding physicians spent 70% of their clinical time on neuro-ophthalmology. In 6 states, there were no reported practicing neuro-ophthalmologists, and in only 8 states was the clinical full-time equivalent to population ratio below the suggested threshold of 1 for every 1.2 million. The median wait time for a new patient was 6 weeks. This wait time was associated with the fraction of clinical time spent in neuro-ophthalmology (0.2 weeks longer wait for a 10 percentage point increase in the fraction of time spent in neuro-ophthalmology; P = 0.02), and suggestively associated with training (training in ophthalmology was associated with 1.0 week shorter wait time; P = 0.06). CONCLUSION: The survey suggests that neuro-ophthalmologists are unable to see patients in a timely manner and a decreasing number of clinicians are entering the field. Future interventions should be considered to incentivize neuro-ophthalmology training in ophthalmology and neurology residents such that the United States population is able to appropriately access neuro-ophthalmic care.

Clinical and Magnetic Resonance Imaging Characteristics of Postfenestration Optic Nerve Sheath Pseudomeningoceles.

PURPOSE: The clinical significance of postoperative pseudomeningocele formation following optic nerve sheath fenestration (ONSF) has not been fully characterized. A literature review identifies 9 previously published cases the authors believe demonstrate pseudomeningocele formation and approximately 19 other similar findings that were either transient or less defined blebs. This study was undertaken to more clearly define the clinical, radiographic, and histopathologic features associated with this entity. METHODS: Sixteen-year, single-center, retrospective chart review of all ONSF cases performed by 2 surgeons. Clinical data, intracranial pressure, radiographic imaging, and histopathology of clinically detected pseudomeningoceles after ONSF were reviewed. RESULTS: Eighty-six eyes in 57 patients underwent ONSF (28 unilateral, 12 bilateral sequential, 17 bilateral simultaneous). Forty-nine of 57 patients had elevated intracranial pressure preoperatively (41 idiopathic intracranial hypertension, 4 venous thrombosis, 2 meningitis, 1 arteriovenous malformation, and 1 sarcoid). In 32 patients undergoing postoperative imaging, 4 eyes (4.7%) in 4 patients developed well-defined pseudomeningoceles, of which 3 were symptomatic and 2 required surgical revision. Each pseudomeningocele developed in the setting of elevated preoperative intracranial pressure (350, 360, 430, 500 mm H20). Magnetic resonance imaging and/or computed tomography revealed sharply demarcated fluid-filled sacs adjacent to the optic nerve. The contents of these sacs were hypointense on T1-weighted imaging, hyperintense on T2-weighting, variably enhanced with contrast, and hypointense on fluid attenuated inversion recovery, and were thus consistent with cerebrospinal fluid. Histopathologic analysis of one of these outpouchings demonstrated an acellular, fibrocollagenized lining consistent with pseudomeningocele. Three eyes in 3 additional patients had less well-defined findings on imaging interpreted as bleb-like or cyst-like change. CONCLUSIONS: Pseudomeningoceles following ONSF may be asymptomatic or may cause symptomatic orbital mass effect and rarely visual loss, amendable to surgical excision. Post-ONSF pseudomeningoceles are identified on computed tomography or magnetic resonance imaging to occur at the locations of fenestration sites and contain cerebrospinal fluid communicating with the subdural space that may act as a "filtration" bleb in some cases. Imaging findings may represent a spectrum spanning intraorbital cerebrospinal fluid leakage, partial walling off of bleb, or fully developed cysts. Resection of optic nerve pseudomeningoceles is considered in symptomatic cysts or eyes with papilledema that fails to improve.

A Profile of Neuro-Ophthalmic Practice Around the World.

BACKGROUND: To compare contrast neuro-ophthalmic practice in various countries, an 18-question survey was sent to the international North American Neuro-Ophthalmology Society (NANOS) members in the spring of 2016. METHODS: At least 1 NANOS member was contacted for each non-US nation in the NANOS membership roster. If there were multiple NANOS members from 1 country, multiple were contacted. If responses were received from more than 1 person from a single country, the first response received was used as the source data. The survey (in English) was emailed to 47 NANOS members from 31 countries. Twenty responses were received representing members from 15 nations. RESULTS: In all 15 nations, at least half of the neuro-ophthalmologists were trained as ophthalmologists. In 60% of nations, at least half of the neuro-ophthalmologists were trained internally, whereas in 33% of countries, at least half were trained in the United States. The number of physicians who practiced a significant amount of neuro-ophthalmology ranged from low (0.08/million, India) to high (3.10/million, Israel). Countries having the highest percentage of neuro-ophthalmologists exclusively practicing neuro-ophthalmology also were those with better patient access to neuro-ophthalmic care. Requirement of approval to see a neuro-ophthalmologist or for imaging studies requested by neuro-ophthalmologists was not typical. In most nations, academic neuro-ophthalmologists were paid a straight salary. In no nation were neuro-ophthalmologists paid more than another ophthalmic subspecialty. CONCLUSIONS: Individual national health care system designs and compensation models have had a profound influence on the rewards and challenges that face neuro-ophthalmologists. There seems to have been a connection between recognition of the discipline, financial rewards of neuro-ophthalmic practice, conditions that permit full-time neuro-ophthalmic practice, and patient access to care. A higher percentage of gross national product for health care did not seem to insure an adequate supply of neuro-ophthalmologists.

Neuro-Ophthalmology: Transitioning From Old to New Models of Health Care Delivery.

In contradiction to fundamental laws of supply and demand, 2 decades of payment policies have led to some medical specialties experiencing declines in both manpower and reimbursement. This paradox has resulted in increasingly long wait times to see some specialists, some specialties becoming less attractive to potential trainees, and a dearth of new trainees entering these fields. Evolving models of health care delivery hold the promise of increasing patient access to most providers and may diminish costs and improve outcomes for most patients/conditions. However, patients who need care in understaffed fields may, in the future, be unable to quickly access a specialist with the requisite expertise. Impeding the sickest and most complex patients from seeing physicians with appropriate expertise may lead to increased costs and deleterious outcomes-consequences contrary to the goals of health care reform. To ensure appropriate access for these patients requires 2 conditions: 1. Compensation models that do not discourage trainees from pursuing nonprocedural specialties, and 2. A care delivery model that expediently identifies and routes these patients to the appropriate specialist.

18F-FDG Uptake in Neurosarcoid Dural Plaque on PET/CT.

While the imaging modality of choice to diagnose neurosarcoidosis is gadolinium-enhanced MRI, F-FDG PET/CT maybe used to stage the disease or target the optimal biopsy site. Few cases have described intense F-FDG uptake at the sites of active neurosarcoidosis in the midbrain and pituitary gland, cerebellar hemispheres, and temporal lobes. Here, we present a case of neurosarcoidosis whose PET/CT examination demonstrated F-FDG avidity in a dural plaque.

Reversal of Visual Impairment Associated with Vasospasm After Resection of Sphenoclinoidocavernous Meningioma with Intra-Arterial Verapamil.

BACKGROUND: Visual loss can be encountered in the immediate, early, or even delayed postoperative period after resection of skull base meningiomas involving the optic apparatus. Various mechanisms for visual loss can include mechanical injury, vascular insult, optic nerve and chiasm edema, and vasospasm of the blood supply to the visual apparatus. CASE DESCRIPTION: In this article, we describe a patient who developed unilateral visual worsening in the early postoperative period after skull base resection of a sphenoclinoidocavernous meningioma that was compressing the ipsilateral optic nerve. After implementing hyperdynamic therapy and high-dose corticosteroids, catheter angiography showed severe vasospasm of the ipsilateral ophthalmic artery with delayed filling of the choroidal blush. Intra-arterial chemical angioplasty with verapamil was administered that resulted in angiographic improvement and clinical restoration of vision back to preoperative baseline. Vision remained stable in the postoperative course and continued to improve after discharge from the hospital. CONCLUSIONS: To our knowledge, this is the first report of successful reversal of postoperative unilateral visual loss related to vasospasm after resection of a skull base sphenoclinoidocavernous meningioma. This may be a useful strategy to diagnose and treat possible vasospasm-induced visual loss after resection of skull base meningiomas involving the optic apparatus.

New developments in giant cell arteritis.

Giant cell arteritis (GCA) is a medium-to-large vessel vasculitis with potentially sight- and life- threatening complications. Our understanding of the pathogenesis, diagnosis, and treatment of GCA has advanced rapidly in recent times. The validity of using the American College of Rheumatology guidelines for diagnosis of GCA in a clinical setting has been robustly challenged. Erythrocyte sedimentation rate, an important marker of inflammation, is lowered by the use of statins and nonsteroidal anti-inflammatory drugs. Conversely, it may be falsely elevated with a low hematocrit. Despite the emergence of new diagnostic modalities, temporal artery biopsy remains the gold standard. Evidence suggests that shorter biopsy lengths and biopsies done weeks to months after initiation of steroid therapy are still useful. New imaging techniques such as positron emission tomography have shown that vascular inflammation in GCA is more widespread than originally thought. GCA, Takayasu arteritis, and polymyalgia rheumatica are no longer thought to exist as distinct entities and are more likely parts of a spectrum of disease. A range of immunosuppressive drugs have been used in conjunction with corticosteroids to treat GCA. In particular, interleukin-6 inhibitors are showing promise as a therapy.

Treatment of neuro-ophthalmic sarcoidosis.

BACKGROUND: Because of the rarity of neuro-ophthalmic sarcoidosis, there are no therapeutic guidelines based on evidence-based medicine for this disorder. EVIDENCE ACQUISITION: Review of literature combined with personal experience. RESULTS: Corticosteroids are the preferred initial therapy for neuro-ophthalmic sarcoidosis. If patients cannot tolerate the requisite dose of corticosteroid needed to control their disease, or if corticosteroids fail to adequately control the disease process, the choices of a second agent are based on the consideration of rapidity of clinical response and the safety profile. CONCLUSIONS: Although methotrexate and mycophenolate mofetil are the medications that are often selected after corticosteroid failure, more rapidly acting agents that have been used are infliximab and intravenous cyclophosphamide.

Postirradiation periocular granuloma faciale associated with uveitis.

Granuloma faciale is a rare dermatopathologic condition that presents as brown-red plaques, nodules, or papules primarily on the face, with the potential for extrafacial and mucous membrane involvement. A case of an 83-year-old woman with periocular granuloma faciale accompanied by a marked anterior uveitis is presented; an association of periocular granuloma faciale with anterior uveitis has not been previously reported.

Lacrimal gland uptake of (67)Ga-gallium citrate correlates with biopsy results in patients with suspected sarcoidosis.

PURPOSE: To investigate whether lacrimal gland uptake on (67)Ga-gallium citrate scintigraphy correlates with histopathologic evidence of sarcoidosis. METHODS: A retrospective, pilot study of 31 patients with suspected sarcoidosis who underwent gallium scintigraphy and lacrimal gland biopsy. Lacrimal gland gallium uptake was assessed by subjective visual scoring (SVS) and lacrimal uptake ratio (LUR). RESULTS: Eleven (36%) patients had lacrimal gland biopsies containing noncaseating granulomas. A statistically significant correlation was found between lacrimal gland gallium uptake and biopsy positivity using SVS (p = 0.03) or LUR (p = 0.01). Using SVS, biopsy positivity rate increased from 0 to 50% in patients with mild to intense uptake. Using LUR, biopsy positivity rate increased linearly as the ratio increased from 13% (LUR < 4) to 100% (LUR > 8). CONCLUSIONS: Lacrimal biopsy positivity rate significantly correlated with gallium uptake on scintigraphy. Both SVS and LUR methods appear to correlate with histologic results and may potentially aid in patient selection for biopsy.

A curious case of glaucoma?

An 84-year-old woman diagnosed with primary open-angle glaucoma was referred because her optic nerve appearance did not account for her visual field deficits. Further evaluation showed loss of color vision and rapid progression of visual field defects. Electroretinography revealed abnormal scotopic and photopic responses. Blood samples were positive for anti-retinal antibodies, but a malignancy work-up was negative, consistent with non-paraneoplastic autoimmune retinopathy.

Blind runner.

Bilateral ocular ischemic syndrome and ischemic optic neuropathy have rarely been reported as initial manifestations of Takayasu arteritis (TA). Appearance of ocular symptoms in TA is related to the extent and severity of involvement of the aorta and its major branches. We report a case of bilateral ocular ischemic syndrome with unilateral ischemic optic neuropathy secondary to TA in a 42-year-old Pakistani man who had severe ocular and cerebral ischemia.

Clinical course and prognosis of trochlear nerve schwannomas.

PURPOSE: To delineate the disease course and prognosis of patients with mass lesions of the fourth nerve presumed to be schwannomas. DESIGN: Nonrandomized retrospective case series. PARTICIPANTS: Thirty-seven consecutive cases of presumed trochlear nerve schwannoma from 9 tertiary university neuro-ophthalmology centers. METHODS: Cases were collected, and their clinical characteristics on presentation and follow-up are described. Inclusion criteria were brain magnetic resonance imaging (MRI) with a lesion suggestive of a schwannoma along the course of the fourth nerve. Exclusion criteria were other causes of fourth nerve palsy, such as congenital, traumatic or microvascular; normal (or lack of) initial brain MRI; lack of adequate clinical information; and disappearance of the lesion on subsequent follow-up brain MRI. MAIN OUTCOME MEASURES: Demographics of patients, presence of neurofibromatosis, symptoms on presentation, vertical deviation, lesion size (on presentation and follow-up), length of follow-up, and outcomes of treatment for lesions or diplopia. RESULTS: Seven patients were excluded and of the 30 patients included in our series, patients were predominantly male (77%) with a mean age of 51 years (range 9-102 years). In contrast with prior case reports, almost all of our cases had a fourth nerve palsy on presentation (29/30), often isolated. Mean follow-up was 3.1 years (range 0.2 months to 11.1 years). There was no significant difference between initial and follow-up lesion size (4.4 vs. 5 mm) for patients who did not receive treatment of lesions (P = 0.36). Only 3 patients underwent neurosurgical resection and an additional patient received gamma-knife radiotherapy. The majority of patients (24/30) did not pursue strabismus surgery for vertical diplopia. CONCLUSIONS: Patients with isolated fourth nerve palsy and small lesions of the fourth nerve have a good prognosis and should be followed with serial MRI scans without neurosurgical intervention unless they develop signs of brain stem compression. Most patients with diplopia and benign fourth nerve lesions typical of trochlear nerve schwannoma can adapt with either prism spectacles or no treatment at all, although strabismus surgery can be successful.

The human resource crisis in neuro-ophthalmology.

Neuro-ophthalmology is facing a serious human resource issue. Few are entering the subspecialty, which is perceived as being poorly compensated compared with other subspecialties of ophthalmology. The low compensation comes from the fact that 1) non-procedural encounters remain undervalued, 2) efforts that benefit other medical specialists are not counted, and 3) the relatively low expenses of neuro-ophthalmologists are not factored into compensation formulas. Mission-based budgeting, which forces academic departments to be financially accountable without the expectation of fiscal relief from medical schools or practice plans, has exacerbated the compensation issue. Solutions must come from within neuro-ophthalmology, academic departments, medical schools, and medical practice plans. They include 1) providing educational resources so that neuro-ophthalmologists need not spend so much time teaching the basics, 2) factoring into compensation the impact of neuro-ophthalmologists in teaching and on revenue generation by procedure-based specialists, 3) improving the efficiency of neuro-ophthalmologists in their consultative practices by providing ample clerical support and other measures, 4) providing contractual salary compensation by departments such as neurosurgery to recognize the contributions made by neuro-ophthalmologists, and 5) reorganizing the academic clinical effort as multidisciplinary rather than departmental.

Use of whole-body FDG PET-CT to aid in the diagnosis of occult sarcoidosis.

PURPOSE: To report a case where combined whole-body Fluorine-18 fluorodeoxyglucose (FDG) PET-CT scanning was used to aid in the diagnosis of a patient with occult sarcoidosis. DESIGN: Case report. METHODS: FDG PET-CT scanning was performed in a patient who presented with persistent bilateral panuveitis after cataract surgery and had undergone an extensive negative workup. RESULTS: FDG PET-CT scanning demonstrated extensive mediastinal adenopathy. Biopsy showed a non-caseating granuloma with associated giant cell formation consistent with a diagnosis of sarcoidosis. CONCLUSIONS: FDG PET-CT scanning generates tomographic scans with excellent sensitivity, spatial resolution, and anatomical landmark identification and may be useful in the workup of idiopathic uveitis.

Idiopathic hypertrophic pachymeningitis mimicking lymphoplasmacyte-rich meningioma.

A 28-year-old woman with a 6-year history of optic neuropathy and 8 years of hearing loss had enhancing dural lesions around the brain stem and in both internal auditory canals on MRI. Histopathology from cranial procedures performed in 1990 and 1993 was originally interpreted as inflammatory meningioma, now known as lymphoplasmacyte-rich meningioma (LRM). Because the clinical course was more consistent with a relapsing process, the original surgical specimens were restudied with additional immunocytochemical stains. The review led to a pathologic diagnosis of idiopathic hypertrophic pachymeningitis (IHP). IHP and LRM can be confused on both imaging and histopathologic grounds.