Julie Falardeau

Dural venous sinus angioplasty and stenting for the treatment of idiopathic intracranial hypertension

Background Lumboperitoneal shunt (LPS), ventriculoperitoneal shunt (VPS) and optic nerve sheath fenestration (ONSF) are accepted surgical therapies for medically refractory idiopathic intracranial hypertension (IIH). In the subset of patients with IIH and venous sinus stenosis, dural venous sinus stenting has emerged as an alternative surgical approach. Methods All cases of dural stents for IIH at our institution were retrospectively reviewed. Eligibility criteria included medically refractory IIH with documented papilledema and dural venous sinus stenosis of the dominant venous outflow system (gradient ≥10 mm Hg). Results Fifteen cases (all women) of mean age 34 years were identified. All had failed medical therapy and six had failed surgical intervention. Technical success was achieved in all patients without major periprocedural complications. The mean preprocedural gradient across the venous stenosis was reduced from 24 mm Hg before the procedure to 4 mm Hg after the procedure. Headache resolved or improved in 10 patients. Papilledema resolved in all patients and visual acuity stabilized or improved in 14 patients. There were no instances of restenosis among the 14 patients with follow-up imaging. Conclusion In this small case series, dural sinus stenting for IIH was performed safely with a high degree of technical success and with excellent clinical outcomes. These results suggest that angioplasty and stenting for the treatment of medically refractory IIH in patients with dural sinus stenosis warrants further investigation as an alternative to LPS, VPS and ONSF.

The use of acetazolamide during pregnancy in intracranial hypertension patients.

Background:Acetazolamide is the mainstay of medical therapy for idiopathic intracranial hypertension (IIH). Its use in pregnant women has not been recommended because of reported teratogenic effects in rodents and rabbits. However, the safety of acetazolamide use during human pregnancy remains unclear. We report the pregnancy and offspring outcomes in women with intracranial hypertension (IH) treated with acetazolamide during pregnancy. Methods:Data were collected through questionnaires sent to patients with IH and their physicians. The questionnaires focused on IH diagnosis, obstetric history and outcomes, and pediatric outcomes. Results:A total of 101 women with IH were consented (total of 158 pregnancies) and acetazolamide usage before 13 weeks of gestation was reported in 50 pregnancies. The risk of spontaneous abortion was similar to the control group and no major complication was identified in the offspring of women treated with acetazolamide. Conclusion:There is no convincing evidence for an adverse effect for acetazolamide use in human pregnancy, even when prescribed prior to the 13th week of gestation. While the liberal use of acetazolamide should be avoided during pregnancy, this medication should remain a treatment option in pregnant women when clinically indicated.

Risk factors for poor visual outcome in patients with idiopathic intracranial hypertension

Objectives: Determine potential risk factors for progressive visual field loss in the Idiopathic Intracranial Hypertension Treatment Trial, a randomized placebo-controlled trial of acetazolamide in patients with idiopathic intracranial hypertension and mild visual loss concurrently receiving a low sodium, weight reduction diet. Methods: Logistic regression and classification tree analyses were used to evaluate potential risk factors for protocol-defined treatment failure (>2 dB perimetric mean deviation [PMD] change in patients with baseline PMD −2 to −3.5 dB or >3 dB PMD change with baseline PMD −3.5 to −7 dB). Results: Seven participants (6 on diet plus placebo) met criteria for treatment failure. The odds ratio for patients with grades III to V papilledema vs those with grades I and II was 8.66 (95% confidence interval [CI] 1.65–∞, p = 0.025). A 1-unit decrease in the number of letters correct on the ETDRS (Early Treatment Diabetic Retinopathy Study) chart at baseline was associated with an increase in the odds of treatment failure by a factor of 1.16 (95% CI 1.04–1.30, p = 0.005). Compared with female participants, the odds ratio for male participants was 26.21 (95% CI 1.61–433.00, p = 0.02). The odds of treatment failure were 10.59 times higher (95% CI 1.63–116.83, p = 0.010) for patients with >30 transient visual obscurations per month vs those with ≤30 per month. Conclusions: Male patients, those with high-grade papilledema, and those with decreased visual acuity at baseline were more likely to experience treatment failure. All but one of these patients were treated with diet alone. These patients should be monitored closely and be considered for aggressive treatment of their idiopathic intracranial hypertension.

VITREO-RETINAL TRACTION AND ANASTROZOLE USE

Purpose This study tested a prediction stemming from the hypothesis that anastrozole users experience heightened vitreo-retinal traction. This hypothesis was based on the knowledge that menopause increases the risk of intraocular tractional events such as posterior vitreous detachments (PVDs). Methods Retinal thickness was measured for 3 groups of amenorrheic women: (1) anastrozole users and (2) tamoxifen users undergoing adjuvant therapy for early-stage breast cancer, and (3) control subjects not using hormonal medication. Foveal shape indices were derived for subjects without PVDs. Results For anastrozole users, the distance to the temporal side of the fovea became less than the distance to the nasal side at a sufficient height above the foveal base. This effect did not exist for control subjects; the between-group difference was appreciable. Results concerning tamoxifen users were inconclusive. Conclusions The foveas of women using anastrozole appear to be subjected to more tractional force than are the foveas of women not using any hormonal medication.

Retinal hemorrhages in anastrozole users.

Purpose. To determine whether women using anastrozole (an aromatase inhibitor, which suppresses estrogen synthesis) are likely to have retinal hemorrhages. Methods. Fundus photographs were obtained for a study comparing effects on vision and the eye of anastrozole (1 mg/d) and tamoxifen (20 mg/d) for women receiving adjuvant endocrine therapy for early-stage breast cancer. Photographs (2 eyes per subject) were evaluated for hemorrhages by an ophthalmologist masked from identifying subject information. The presence of hemorrhages was assessed for 35 anastrozole users, 38 amenorrheic tamoxifen users, and 53 amenorrheic (peri- or postmenopausal) control subjects not using any endocrine medication. The age range was 43 to 69 years; only subjects reporting normal vision and healthy eyes were recruited. Photographs from 36 tamoxifen users from a previous study also were assessed. Views of the fundus extended appreciably beyond the posterior pole for all tamoxifen and anastrozole users, and for 38 of the control subjects. Photographic assessments were compared with medical history and blood pressure data, and optical coherence tomography was used for quantifying aspects of foveal shape that might be affected by vitreoretinal traction. Results. Four anastrozole users and no contemporaneous tamoxifen users but one previously tested tamoxifen user had retinal hemorrhages, in each case within the posterior pole. Two anastrozole users had a flame hemorrhage (in the retinal nerve fiber layer), and two had a blot hemorrhage (deeper in the retina). No control subjects were observed to have hemorrhages. Conclusions. Anastrozole use appears to be associated with an increased prevalence of retinal hemorrhages. The hemorrhages may mark systemic vascular compromise resulting from estrogen depletion, and/or they may be consequences of vitreoretinal traction resulting from estrogen depletion. They may also depend on the use of medications for controlling common anastrozole-induced side effects. Prospective studies need to be conducted with larger numbers of subjects.

Giant Cell Arteritis

Giant cell arteritis is a systemic vasculitis with a wide clinical spectrum, and it represents a medical emergency. Visual loss is the most feared complication, and when it happens, it tends to be profound and permanent. Prompt diagnosis and treatment are imperative to minimize potentially devastating visual loss and neurologic deficits. A temporal artery biopsy should be performed on every patient in whom the diagnosis is suspected. The mainstay of therapy remains corticosteroids.

Atypical Optic Neuritis

Classic demyelinative optic neuritis is associated with multiple sclerosis and typically carries a good prognosis for visual recovery. This disorder is well characterized with respect to its presentation and clinical features by baseline data obtained through the optic neuritis treatment trial and numerous other studies. Atypical optic neuritis entails clinical manifestations that deviate from this classic pattern of features. Clinical signs and symptoms that deviate from the typical presentation should prompt consideration of less common etiologies. Atypical features to consider include lack of pain, simultaneous or near-simultaneous onset, lack of response to or relapse upon tapering from corticosteroids, or optic nerve head or peripapillary hemorrhages. The most important alternative etiologies to consider and the steps towards their respective diagnostic evaluations are suggested for these atypical features.

Optic disc cupping associated with neurosyphilis

CASE REPORT A 52-year-old woman with a history of neurosyphilis and chorioretinitis was referred for evaluation of optic disc cupping. Since treatment with an intramuscular injection of penicillin-G 4 years ago, the chorioretinitis has remained quiescent, and the free treponemal antibody test has been normal. She uses atenolol for high blood pressure. She complains of poor vision in the right eye and “worsening vision” in the left eye. The visual acuity is 20/400 in the right eye and 20/30 in the left eye. The intraocular pressure (IOP) is 11mm Hg in both eyes, and central corneal thickness measures 581 and 588 microns in the right and left eyes, respectively. She has a prominent relative afferent pupillary defect in the right eye, a right exotropia, and a normal anterior chamber in both eyes without corneal scarring, uveitis, iris, or lens abnormalities. The drainage angles are open in both eyes without synechiae. She has a clear, wellplaced posterior chamber lens OU. The right eye has optic disc cupping without pallor (Fig. 1, left side) and chorioretinal scars in the macula and periphery. The left optic nerve shows cupping and excavation of the neuroretinal rim without pallor (Fig. 1, right side) and focal areas of chorioretinal scarring in the peripheral retina. Goldmann visual field testing of the right eye demonstrates a small temporal island of vision, and achromatic automated visual field testing of the left eye shows a dense superior and inferior arcuate defect with a central island of vision (Fig. 2). A review of prior records indicates the maximum IOP to be 15mm Hg, and a diurnal tension curve demonstrated IOP values between 11 and 14mm Hg. Spectral domain ocular coherence tomography shows a flattened nerve fiber layer contour with reduced thickness measurements outside normal limits in both eyes (Fig. 3).

Homonymous hemianopia due to Erdheim-Chester disease.

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis typically affecting multiple organ systems. We report 2 patients who presented with homonymous hemianopia and were ultimately diagnosed with biopsy-confirmed ECD. We review the spectrum of ECD and its treatment as well as histopathological and immunohistochemical differentiation from other histiocytic disorders.

The unfaithful neuritis

A 55-year-old man with a history of mantle cell lymphoma reported acute blurred vision in the right eye. Although initially diagnosed with acute retrobulbar optic neuritis, 3 weeks later retinal infiltrates and vitritis developed, but vitrectomy resulted negative for vitreoretinal lymphoma. Further investigation revealed a serology positive for syphilis. This case highlights the role of the ophthalmologist in the diagnosis of syphilis, which is able to mimick multiple eye disorders, optic neuritis, and vitreoretinal lymphoma among others.