Long-term efficacy of fractionated conformal radiotherapy for the management of primary optic nerve sheath meningioma.

BACKGROUND/AIM: Fractionated conformal radiotherapy (FCRT) is now used to treat vision-threatening optic nerve sheath meningioma (ONSM), but long-term efficacy and safety data are lacking; the purpose of this study was to assess these key data. METHODS: This is a retrospective chart review with prospective follow-up of adult patients treated with FCRT for primary ONSM at four academic medical centres between 1995 and 2007 with ≥10 years of follow-up after treatment. RESULTS: 16 patients were identified with a mean post-treatment follow-up of 14.6 years (range: 10.5-20.7 years). The mean age at symptom onset was 47.6 years (range: 36-60 years). FCRT was performed at a mean of 2.3 years after symptom onset (range: 0.2-14.0 years). At last follow-up, visual acuity had improved or stabilised in 14 of the 16 (88%) patients, and 11 (69%) had retained or achieved ≥20/40. The mean deviation on automated perimetry remained stable (-14.5 dB pretreatment vs -12.2 dB at last follow-up; p=0.68, n=10). Two (11%) patients had persistent pain, proptosis or diplopia, compared with six (38%) pretreatment (p=0.11). Two (13%) patients developed radiation retinopathy more than 6 months after completion of therapy, one (50%) of whom had worse visual acuity compared with pretreatment. No patient developed tumour involvement or radiation damage in the fellow eye. CONCLUSION: FCRT stabilises or improves visual function in patients with primary ONSM and is associated with a low risk of significant ocular sequelae. This treatment should be considered instead of surgery in patients with primary ONSM who require intervention due to loss of visual sensory and/or ocular motor function.

Risk of Acute Ischemic Stroke in Patients With Monocular Vision Loss of Vascular Etiology.

BACKGROUND: To evaluate the risk of concurrent acute ischemic stroke and monocular vision loss (MVL) of vascular etiology. DESIGN: Retrospective, cross-sectional study. SUBJECTS: Patients aged 18 or older diagnosed with MVL of suspected or confirmed vascular etiology who had no other neurologic deficits and who received brain MRI within 7 days of onset of visual symptoms were included. METHODS: A medical record review was performed from 2013 to 2016 at Yale New Haven Hospital. Patients were included if vision loss was unilateral and due to transient monocular vision loss (TMVL), central retinal artery occlusion (CRAO), or branch retinal artery occlusion (BRAO). Any patients with neurologic deficits other than vision loss were excluded. Other exclusion criteria were positive visual phenomena, nonvascular intraocular pathology, and intracranial pathology other than ischemic stroke. MAIN OUTCOME MEASURES: The presence or absence of acute stroke on diffusion-weighted imaging (DWI) on brain MRI. RESULTS: A total of 641 records were reviewed, with 293 patients found to have MVL. After excluding those with focal neurologic deficits, there were 41 patients who met the inclusion criteria and received a brain MRI. Eight of the 41 subjects (19.5%) were found to have findings on brain MRI positive for acute cortical strokes. The proportion of lesion positive MRI was 1/23 (4.3%) in TMVL subjects, 4/12 (33.3%) in CRAO subjects, and 2/5 (40%) in BRAO subjects. Brain computed tomography (CT) scans were not able to identify the majority of acute stroke lesions in this study. CONCLUSIONS: Patients with MVL of vascular etiology such as TMVL, CRAO, or BRAO may have up to 19.5% risk of concurrent ischemic stroke, even when there are no other neurologic deficits. These strokes were detected acutely with brain MRI using DWI but were missed on CT.

Photopsia and a temporal visual field defect.

A 30-year-old woman presented with intermittent photopsia, a temporal visual field defect below the horizontal in her left eye, and flu-like symptoms. Slit-lamp and fundus examinations were unremarkable. Humphrey 30-2 threshold perimetry and 120-point screening visual field demonstrated blind spot enlargement of the left eye and a normal field in the right eye. Fundus autofluorescence, optical coherence tomography of the macula, full-field electroretinogram, electrooculogram, and multifocal electroretinogram were normal. Swept-source optical coherence tomography scan of the left optic nerve showed an intact outer retina, a remarkably thinned nerve fiber layer nasally, and peripapillary vitreous traction. Goldmann kinetic perimetry revealed a sector-shaped dense defect breaking out from the blind spot to the temporal periphery just below the horizontal in the left eye. The patient had nasal hypoplasia of the optic nerve and peripapillary vitreous traction.

Vision Loss With Sexual Activity.

A 51-year-old white man presented with multiple episodes of transient painless unilateral vision loss precipitated by sexual intercourse. Examination was significant for closed angles bilaterally. His visual symptoms completely resolved following treatment with laser peripheral iridotomies.

Antiphospholipid Antibody Syndrome: Raised Intracranial Pressure Without Cerebral Venous Sinus Thrombosis.

Antiphospholipid antibody syndrome (APS) has been reported to cause elevated intracranial pressure, but usually this is due to cerebral venous sinus thrombosis (CVST). We present a 36-year old man with APS with elevated intracranial pressure with neuro-ophthalmic, renal and hematological involvement without identifiable CVST.

Vision loss with bending over.

A 66-year-old African American man presented with episodic transient visual loss triggered by bending forward. The initial examination did not suggest intraocular pathology and the patient was nearly sent for vascular evaluation given his cardiovascular risk factors. Fundus photographs taken during an episode of visual loss suggested an intraocular process, however. Gonioscopy revealed a microhyphema causing a "snow globe" effect in the anterior chamber, most likely related to recent bleb manipulation in the affected eye.

Metastasis of uveal melanoma millimeter-by-millimeter in 8033 consecutive eyes.

OBJECTIVE: To determine the rate of metastasis of uveal melanoma on the basis of tumor thickness in millimeters. METHODS: Retrospective medical record review. RESULTS: The mean (median) patient age was 58 (59) years. A total of 8033 eyes were examined. Of the 285 eyes with iris melanoma, the mean tumor thickness was 2.7 mm and metastasis occurred in 0.5%, 4%, and 7% at 3, 5, and 10 years, respectively. Of the 492 eyes with ciliary body melanoma, the mean tumor thickness was 6.6 mm and metastasis occurred in 12%, 19%, and 33% at 3, 5, and 10 years, respectively. Of the 7256 eyes with choroidal melanoma, the mean tumor thickness was 5.5 mm and metastasis occurred in 8%, 15%, and 25% at 3, 5, and 10 years, respectively. For all uveal melanoma, metastasis at 5, 10, and 20 years was 6%, 12%, and 20% for small melanoma (0-3.0 mm thickness), 14%, 26%, and 37% for medium melanoma (3.1-8.0 mm), and 35%, 49%, and 67% for large melanoma (>8.0 mm). More specifically, metastasis per millimeter increment at 10 years was 6% (0-1.0 mm thickness), 12% (1.1-2.0 mm), 12% (2.1-3.0 mm), 16% (3.1-4.0 mm), 27% (4.1-5.0 mm), 28% (5.1-6.0 mm), 29% (6.1-7.0 mm), 41% (7.1-8.0 mm), 50% (8.1-9.0 mm), 44% (9.1-10.0 mm), and 51% (>10.0 mm). Clinical factors predictive of metastasis by multivariate analysis included increasing patient age, ciliary body location, increasing tumor diameter, increasing tumor thickness, having a brown tumor, and the presence of subretinal fluid, intraocular hemorrhage, or extraocular extension. CONCLUSION: Increasing millimeter thickness of uveal melanoma is associated with increasing risk for metastasis.

Recurrent vision loss after endoscopic sinus surgery managed with palmar pressure.

A 41-year-old woman underwent endoscopic sinus surgery and 24 hours later, she developed acute orbital emphysema with marked vision loss. CT showed disruption of the inferior aspect of the medial wall of the orbit with fat herniation. She was immediately treated with the application of bedside palmar pressure to the globe with complete return of her vision without the need for a needle aspiration or orbital decompression. Weeks later, the patient experienced 2 more episodes of transient monocular visual loss that were again successfully treated with the same palmar pressure. Bedside digital decompression may be a potentially helpful method of restoring vision in cases of orbital emphysema with acute vision loss. We advise attempting this simple bedside procedure before committing the patient to a more invasive needle aspiration or surgical decompressive procedure.