Ocular fundus examination is a critical part of the physical examination in patients with severely elevated blood pressure (BP), which is defined by the Seventh Report of the Joint National Committee on Prevention, Detection, Evaluation, and Treatment of High Blood Pressure (JNC7) as a BP exceeding 180/120 mm Hg. Indeed, the presence or absence of severe, grade III/IV hypertensive retinopathy helps differentiate hypertensive emergencies requiring intensive care from less severe hypertensive urgencies. As a secondary analysis in the Fundus photography versus Ophthalmoscopy Trial Outcomes in the Emergency Department (FOTO-ED) study, we sought to explore potential risk factors, in particular BP, for the presence of ocular fundus abnormalities relevant to the care of emergency department (ED) patients. We found evidence of acute end-organ ocular damage at lower blood pressures than the JNC7 criteria.
Objective: Non-mydriatic fundus photography by non-ophthalmic trained personnel has recently been shown to be a potential alternative to direct ophthalmoscopy in the emergency department (ED). We evaluated the reliability of a novel quality rating scale and applied this scale to non-mydriatic fundus photographs taken during routine ED patient encounters to determine factors associated with diminished photograph quality.
Design: Prospective, cross-sectional
Participants: 350 patients enrolled in the Fundus photography vs. Ophthalmoscopy Trials Outcomes in the Emergency Department (FOTO-ED) study were photographed by nurse practitioners after <30 minutes of training followed by supervision.
Methods: Photographs of both eyes were graded for quality on two occasions by two neuro-ophthalmologists. Four regions were independently evaluated for quality: optic disc, macula, superior and inferior vascular arcades. Quality as a function of the number of photographs taken was evaluated by Kaplan-Meier analysis. Mixed effects ordinal logistic regression was used to evaluate for predictors of image quality while accounting for the repeated measures design.
Main Outcome Measure: Overall photographic quality (1–5 scale, 5 best).
Results: We evaluated 1734 photographs. Inter- and intra-observer agreements between neuro-ophthalmologists were very good (weighted kappa:0.84–0.87). Quality of the optic disc area was better than those of other retinal areas (p<0.002). Kaplan-Meier analysis showed that if a high-quality photograph of an eye was not obtained by the third attempt it was unlikely that one would be obtained at all. A 10 second increase in the inter-photograph interval before a total of forty seconds increased the odds of a one unit higher quality rating by 1.81 times (95%CI: 1.68–1.98), and a ten year increase in age decreased the odds by 0.76 times (95%CI: 0.69–0.85). Black patients had 0.42 times (95%CI: 0.28–0.63) the odds of a one unit higher quality rating compared to whites.
Conclusions: Our 5-point scale is a reliable measure of non-mydriatic photograph quality. The region of interest, interphotograph interval, age, and race are significant predictors of image quality for non-mydriatic photographs taken by nurse practitioners in the ED. Addressing these factors may have a direct impact on the successful implementation of non-mydriatic fundus photography into the ED.
Idiopathic intracranial hypertension (IIH) is increasingly recognized as a cause of spontaneous cerebrospinal fluid (CSF) leak in the otolarnygological and neurosurgical literature. The diagnosis of IIH in patients with spontaneous CSF leaks typically is made a few weeks after surgical repair of the leak when symptoms and signs of elevated intracranial pressure (ICP) appear. Case reports and literature review. Two young obese women developed spontaneous CSF rhinorrhea related to an empty sella in one and a cribriform plate encephalocele in the other. Both patients underwent surgical repair of the CSF leak. A few weeks later, they developed chronic headaches and bilateral papilledema. Lumbar punctures showed elevated CSF opening pressures with normal CSF contents, with temporary improvement of headaches. A man with a 3-year history of untreated IIH developed spontaneous CSF rhinorrhea. He experienced improvement of his headaches and papilledema after a CSF shunting procedure, and the rhinorrhea resolved after endoscopic repair of the leak. These cases and the literature review confirm a definite association between IIH and spontaneous CSF leak based on: 1) similar demographics; 2) increased ICP in some patients with spontaneous CSF leak after leak repair; 3) higher rate of leak recurrence in patients with raised ICP; 4) patients with intracranial hypertension secondary to tumors may develop CSF leak, confirming that raised ICP from other causes than IIH can cause CSF leak. CSF leak occasionally may keep IIH patients symptom-free; however, classic symptoms and signs of intracranial hypertension may develop after a CSF leak is repaired, exposing these patients to a high risk of recurrence of the leak unless an ICP-lowering intervention is performed.
Mitochondrial diseases frequently manifest neuro-ophthalmologic symptoms and signs. Because of the predilection of mitochondrial disorders to involve the optic nerves, extraocular muscles, retina, and even the retrochiasmal visual pathways, the ophthalmologist is often the first physician to be consulted. Disorders caused by mitochondrial dysfunction can result from abnormalities in either the mitochondrial DNA or in nuclear genes which encode mitochondrial proteins. Inheritance of these mutations will follow patterns specific to their somatic or mitochondrial genetics. Genotype-phenotype correlations are inconstant, and considerable overlap may occur among these syndromes. The diagnostic approach to the patient with suspected mitochondrial disease entails a detailed personal and family history, careful ophthalmic, neurologic, and systemic examination, directed investigations, and attention to potentially life-threatening sequelae. Although curative treatments for mitochondrial disorders are currently lacking, exciting research advances are being made, particularly in the area of gene therapy. Leber hereditary optic neuropathy, with its window of opportunity for timely intervention and its accessibility to directed therapy, offers a unique model to study future therapeutic interventions. Most patients and their relatives benefit from informed genetic counseling.
Examination of the ocular fundus is imperative in the diagnosis and treatment of many acute medical and neurologic conditions, but direct ophthalmoscopy is underused and difficult to perform without pharmacologic pupillary dilation. We believe that nonmydriatic ocular fundus photography (i.e., performed without pupillary dilation) represents a promising alternative to direct ophthalmoscopy, particularly in the emergency department, where limited training in ophthalmoscopy, increased demands on physicians' time, and underappreciation of the prognostic value of ocular fundus examination can place patients at risk for poor outcomes and expose their caregivers to medicolegal liability. The FOTO-ED (Fundus Photography vs. Ophthalmoscopy Trial Outcomes in the Emergency Department) study hypothesized that the use of nonmydriatic fundus photography in the emergency department would result in increased detection of abnormalities in the ocular fundus relevant to emergency-department care, a majority of which would be missed during routine clinical practice in the department.
Idiopathic intracranial hypertension (IIH) is a condition of unknown etiology often encountered in neurologic practice. It produces non-localizing symptoms and signs of raised intracranial pressure and, when left untreated, can result in severe irreversible visual loss. It most commonly occurs in obese women of childbearing age, but it can also occur in children, men, non-obese adults, and older adults. While it is frequently associated with obesity, it can be associated with other conditions, such as obstructive sleep apnea and transverse cerebral venous sinus stenoses. Recent identification of subgroups at high risk for irreversible visual loss, including black patients, men, and patients with fulminant forms of IIH, help guide the optimal management and follow-up. Ongoing studies of venous anatomy and physiology in IIH patients, as well as a recently begun randomized clinical treatment trial, should provide more insights into this common yet poorly understood syndrome of isolated intracranial hypertension.
We present a unique case of a patient with deafness and blindness secondary to carcinomatous meningitis from colorectal adenocarcinoma with accompanying radiologic and pathologic images and a brief review of the relevant literature.
Objective
Transverse cerebral venous sinus stenosis (TSS) is common among patients with idiopathic intracranial hypertension. TSS likely also exists among individuals with normal ICP but the prevalence is unclear. The goal of this study was to identify patients with incidental TSS and normal ICP and describe their characteristics.
Methods
Among 240 adult patients who underwent brain magnetic resonance imaging (MRI) with magnetic resonance venography (MRV) with contrast at our institution between September 2009 and September 2011, 44 had isolated TSS without further substantial imaging abnormality. Medical records were reviewed for symptoms of increased ICP, papilledema, cerebrospinal fluid (CSF) constituents and opening pressure (OP), and reason for brain imaging. Of these, 37 were excluded for confirmed or possible idiopathic intracranial hypertension. Of the remainder,5 had CSF-OP ≤25 cmH2O without papilledema, and 2 did not have measured ICP, but had no papilledema or symptoms of increased ICP. Imaging was re-interpreted to assess for signs suggestive of elevated ICP and to characterize the TSS further.
Results
All patients were women (mean age: 41, mean BMI: 37.1). CSF contents were normal, but OPs were at the upper limit of normal (22 – 25 cmH2O). Indications for MRI/MRV included query pituitary abnormality (1), migraine (4), and anomalous-appearing optic nerves (2). All had bilateral TSS. Six had short TSS and an empty sella; one had long TSS and no empty sella; one had flattening of the posterior sclera; two had prominence of peri-optic nerve CSF.
Conclusion
Asymptomatic bilateral TSS exists in patients with ICP ≤25 cmH2O, but is likely uncommon. CSF-OP was at the upper limit of normal in our patients, who also had other radiologic signs suggestive (but not specific) of chronically-raised ICP. Findings of bilateral TSS on imaging should prompt funduscopic examination for papilledema.
Nonarteritic anterior ischemic optic neuropathy (NAION) is the most common clinical presentation of acute ischemic damage to the optic nerve. Most treatments proposed for NAION are empirical and include a wide range of agents presumed to act on thrombosis, on the blood vessels, or on the disc edema itself. Others are presumed to have a neuroprotective effect. Although there have been multiple therapies attempted, most have not been adequately studied, and animal models of NAION have only recently emerged. The Ischemic Optic Neuropathy Decompression Trial (IONDT), the only class I large multicenter prospective treatment trial for NAION, found no benefit from surgical intervention. One recent large, nonrandomized controlled study suggested that oral steroids might be helpful for acute NAION. Others recently proposed interventions are intravitreal injections of steroids or anti-vascular endothelial growth factor (anti-VEGF) agents. There are no class I studies showing benefit from either medical or surgical treatments. Most of the literature on the treatment of NAION consists of retrospective or prospective case series and anecdotal case reports. Similarly, therapies aimed at secondary prevention of fellow eye involvement in NAION remain of unproven benefit.