Diagnostic performance of neuroimaging in suspected idiopathic intracranial hypertension.

The diagnostic utility of neuroradiologic signs associated with idiopathic intracranial hypertension (IIH) for the evaluation of patients presenting with papilloedema remains yet to be elucidated. This multicentre retrospective cohort study assessed consecutive patients presenting with suspected papilloedema to Auckland District Health Board (NZ) and Stanford University Medical Centre (US), between 2005 and 2019, undergoing magnetic resonance imaging and venography (MRI/MRV) or computed tomography and venography (CT/CTV) prior to lumbar puncture assessment for diagnostic suspicion of IIH. Data were collected regarding demographic, clinical, radiologic, and lumbar puncture parameters, and the diagnosis of IIH was determined according to the Friedman criteria for primary pseudotumor cerebri syndrome. A total of 204 participants (174 females; mean ± SD age 29.9 ± 12.2 years) were included, and 156 (76.5%) participants fulfilled the diagnostic criteria for IIH. The presence of any IIH-associated radiologic sign on MRI/MRV demonstrated a sensitivity (95% CI) of 74.8% (65.8%-82.0%) and specificity (95% CI) of 94.7% (82.7%-98.5%), while radiologic signs on CT/CTV exhibited a sensitivity (95% CI) of 61.0% (49.9%-71.2%) and specificity (95% CI) of 100.0% (83.2%-100.0%). In summary, the modest sensitivities of radiologic signs of IIH would support the routine use of lumbar puncture assessment following neuroimaging to secure the diagnosis. However, the high specificities might lend limited support for the judicious deferment of lumbar puncture assessment among typical IIH demographic patients who consent to the inherent small risk of missed pathology, which has been proposed by some clinicians.

Long term outcomes of idiopathic intracranial hypertension: Observational study and literature review.

BACKGROUND: Idiopathic intracranial hypertension (IIH) is an unexplained increase in intracranial pressure often associated with obesity. The aim of this study was to conduct a retrospective observational study of the long term clinical, visual, and treatment outcomes in IIH patients. METHODS: A retrospective observational study of patients diagnosed with IIH over a 12-year period at a single centre was completed via database review. Demographic data, symptoms at baseline and last visit, treatments undertaken, and duration of follow-up were included. Visual outcomes, including visual acuity, colour vision, 30-2 Humphrey automated perimetry data, and retinal nerve fibre layer thickness (RNFL), were collected at baseline and last visit. RESULTS: IIH was diagnosed in 132 patients (90.9 % female) with a median of 2.8 years (range: 0-9.1) follow-up. Mean BMI was 35.9 ± 7.9 kg/m2. Symptoms at presentation were headache (87.6 %), pulsatile tinnitus (27.2 %) and transient visual obscurations (27.2 %). First-line management was acetazolamide in 86.4 %, with 34.2 % of these patients ceasing treatment because of adverse events. Visual field measures and RNFL at last follow-up improved when compared to baseline (median MD: - 1.99 dB (IQR -3.6 to -0.9) to -0.85 (-2.1 to 0.0) (p < 0.001), median RNFL: 132 µm (IQR 116 - 183) to 103 (92 - 113) (p < 0.001)). Some patients (6.1 %) required surgery for more severe IIH. CONCLUSIONS: Long-term symptomatic and visual prognosis in IIH patients is excellent. However, a subset of patients with more severe disease require surgical intervention. Adverse events of treatment lead to high medication discontinuation rates.

Cicatricial ectropion surgery: a prospective study of long-term symptom control, patient satisfaction and anatomical success.

IMPORTANCE: Cicatricial ectropion repair is effective and has a low complication rate. BACKGROUND: To evaluate the effectiveness and long-term functional outcomes of surgical repair of lower lid cicatricial ectropion. DESIGN: Prospective consecutive case series. PARTICIPANTS: Forty-four consecutive operations for cicatricial ectropion repair. METHODS: Consecutive cases of cicatricial ectropion repair completed during 2007-2011 in Waikato Hospital and Hamilton Eye Clinic, New Zealand, were enrolled in the study following formal ethics approval. Ectropion repair was completed using inferior retractor repositioning, horizontal lid tightening and full-thickness skin grafting. MAIN OUTCOME MEASURES: Patient-reported symptoms and satisfaction, ectropion recurrence and punctal ectropion. RESULTS: Forty-four eyes of 40 patients (30 males and 10 female) were included in the study. The average age at surgery was 75 years. Watering and poor appearance were the most common presenting symptoms. Postoperative follow-up was 1-6 years (mean = 4 years). There were no major perioperative complications, one patient developed trichiasis postoperatively. There were five reoperations (11%) during the study period. Two patients underwent medial spindle procedures for symptomatic medial ectropion and three patients required additional horizontal lid tightening. Seventy percent of patients reported satisfaction with their long-term results despite the partial recurrence of symptoms in 45%. Ectropion recurrence in <1 year occurred in three patients. CONCLUSION AND RELEVANCE: In this series, satisfactory appearance and symptom control were reported by 68% of patients at long-term follow-up. Preoperative marked ectropion with marked lid laxity is associated with early treatment failure and may be regarded as a relative contraindication to surgical correction with this technique. Punctal ectropion is an unreliable indicator of functional success.

Optical coherence tomography predicts visual outcome for pituitary tumors.

We evaluate if the relationship between optical coherence tomography (OCT) of the retinal nerve fibre layer (RNFL) and visual outcome continued over long-term visual recovery in 107 patients undergoing pituitary decompression. Recently, it has been recognized that OCT of the RNFL has prognostic value in predicting visual outcomes after surgery for chiasmal compression caused by pituitary tumours. Patients were followed up at three time points: pre-operative (visit 1), 6-10 weeks post-operative (visit 2) and 9-15 months follow-up (visit 3). We found that patients with thin pre-operative RNFL had more severe visual field defects (mean deviation [MD] -9.22 versus -3.96 decibels [dB]; p = 0.001), but pre-operative visual acuity (VA) was good in both normal and thin RNFL groups (Snellen VA 6/5 and 6/4; p = 0.039). For those with thin RNFL the greatest improvement was between visit 2 and 3 (MD -7.1 dB versus -3.4 dB, respectively; p < 0.001) compared with pre-operative -9.8 dB. Normal RNFL patients showed greatest improvement between visits 1 and 2 (pre-operative -4.8 dB, visit 2 -2.0 dB, visit 3 -0.9 dB; p = 0.001). For long-term follow-up, 81.0% of eyes with normal RNFL, compared to 37.1% with thin RNFL (p < 0.001), achieved an MD of -2.00 dB (final visit). At final follow-up, 97.5% of normal RNFL eyes achieved VA of 6/12 or better compared with 88.2% with thin RNFL (p = 0.034). Our results indicate that long-term visual recovery after surgical decompression of pituitary lesions is predicted by pre-operative OCT RNFL. Patients with normal RNFL thickness show an increased propensity for visual recovery. This effect continues after long-term follow-up, however, most visual recovery occurs within the first 6-10 weeks.

Macular thickness predictive of visual field sensitivity in ischaemic optic neuropathy.

PURPOSE: To investigate the ability of optical coherence tomography (OCT) parameters of macular thickness (MT) and peripapillary retinal nerve fibre layer (RNFL) thickness to differentiate eyes with nonarteritic anterior ischaemic optic neuropathy (NAION) from uninvolved eyes and to identify the relationship between macular and RNFL parameters and visual field sensitivity (VFS). METHODS: Thirty patients with unilateral NAION participated in a prospective observational cross-sectional study. Patients underwent Humphrey visual field (SITA Standard 24-2, HVF) testing and OCT to measure MT and RNFL. The contralateral uninvolved eye was used as controls. Areas under the receiver operating characteristic curves (AUROCs) of MT and RNFL for discriminating NAION from control eyes were also determined. The prespecified outcome measure was the correlation between RNFL, MT and mean deviation (MD). RESULTS: Average RNFL and MT were thinner in NAION eyes: 72.8 µm versus 98.9 µm (p<0.0001) and 231.9 µm (SD, 21.4) vs. 251.1 µm (SD, 14.8; p=0.0001), respectively. The largest AUROCs were for average MT (0.87) and average RNFL thickness (0.88). Overall, macular parameters showed stronger correlation with VFS than RNFL parameters. The highest correlation was average MT (0.71; p<0.0001) followed by RNFL parameter nasal quadrant RNFL (0.40; p=0.030). CONCLUSION: Both MT and RNFL show strong correlations with level of VFS in NAION. Macular thickness showed more robust correlations with VF and provides strong surrogate marker of the level of damage in NAION.

Optic nerve atrophy in adrenoleukodystrophy detectable by optic coherence tomography.

The adrenoleukodystrophies (ALDs) are a group of metabolic disorders characterised by the accumulation of very long-chain fatty acids in all tissues. The two most frequent ALD phenotypes are adult-onset adrenomyeloneuropathy (AMN) and childhood cerebral ALD. Visual system involvement in the adult phenotype is well described as impairment of visual function and optic disc pallor on clinical examination accompanied by demyelination of the optic nerves seen on MRI. Thinning of the retinal nerve fiber layer and ganglion cell death has been described in a neonatal form of ALD. Our patient provides evidence, through ocular coherence tomography scanning of the retina, that such degenerative changes also underlie the visual dysfunction seen in the AMN phenotype.

Medically controlled glaucoma patients show greater increase in intraocular pressure than surgically controlled patients with the water drinking test.

PURPOSE: To evaluate whether patients who have undergone trabeculectomy with mitomycin C have a different intraocular pressure (IOP) response profile after the water drinking test (WDT) from that of patients who are medically managed with a similar baseline IOP and level of visual field (VF) damage. DESIGN: Prospective observational study. PARTICIPANTS: Thirty glaucoma patients with IOP controlled by trabeculectomy and 30 with medically treated glaucoma matched for level of VF damage and IOP at baseline (7-14 mmHg). METHODS: All patients underwent the WDT, which involved drinking 1000 ml of water in 15 minutes. MAIN OUTCOME MEASURES: The IOP was measured before the WDT and subsequently at 15-minute intervals for 1 hour. The peak IOP with the WDT was compared between both groups using Tukey post hoc multiple comparison and paired t tests. RESULTS: Baseline IOPs were 10.4+/-2.3 mmHg in surgically treated and 11.1+/-1.8 mmHg (P = 0.07) in medically treated eyes. After the WDT, mean IOPs in the surgically and medically treated groups were 10.7+/-2.3 mmHg and 14.6+/-2.2 mmHg. Mean maximum IOPs were 11.7+/-2.6 mmHg and 17.3+/-2.7 mmHg in the surgically and medically treated groups, respectively (P<0.0001), increases of 12.5% and 56%. Ranges of IOP during the WDT were 2.2+/-1.3 mmHg and 5.6+/-1.9 mmHg in the surgically and medically treated patients (P<0.0001). CONCLUSIONS: Patients with advanced glaucoma who are medically controlled show greater IOP elevation and peak IOP after the WDT than eyes that have undergone trabeculectomy.

In vivo retinal nerve fiber layer thickness measured by optical coherence tomography predicts visual recovery after surgery for parachiasmal tumors.

PURPOSE: Restoration of visual function after neurosurgery for parachiasmal tumors is variable and unpredictable. The current study was conducted to determine whether in vivo retinal nerve fiber layer (RNFL) thickness measurements predict the visual recovery of such patients. METHODS: Forty patients undergoing surgical resection of parachiasmal lesions were prospectively assessed before surgery with a neuro-ophthalmic examination, involving standard automated visual field (VF) testing and optical coherence tomography (OCT) measurements of RNFL thickness, which was the prespecified marker for axonal loss. Tests were repeated within 6 weeks after surgery. RESULTS: Thinner preoperative RNFL thickness was associated with worse visual acuity (VA) and VF mean deviation (MD). Patients with normal preoperative RNFL had significant improvement in mean VA after surgery, from 20/40 to 20/25 (P = 0.028), whereas patients with thin RNFL did not improve (20/80 to 20/60, P = 0.177). Eyes with normal RNFL showed improvement in MD (-7.0 dB before surgery, -3.5 dB after surgery, P = 0.0007) unlike eyes with thin RNFLs, which had no significant improvement after surgery (-15.3 dB before and -13.3 dB after surgery, P = 0.191). RNFL thickness increased by 1% after surgery among all eyes (P = 0.04). Eyes with severe VF defects (MD

Brightness sensitivity and color perception as predictors of relative afferent pupillary defect.

PURPOSE: To characterize the relationship between brightness sensitivity and color perception and relative afferent pupillary defect (RAPD) in patients with optic neuropathy. METHODS: The "swinging flashlight test" was used to diagnose RAPD, the degree of which was quantified by neutral density filters, in 325 consecutive patients in a case-control study. A separate examiner, masked to the pupillary findings, then assessed participants for Ishihara color plate reading, brightness sense, and red perception. The latter two were quantified by asking the patient to score (out of 100%) brightness (of a light source) or redness (of an object) of the two eyes relative to each other. Pearson correlation coefficients and receiver operating characteristic (ROC) curves were calculated. RESULTS: Brightness sense (r = -0.79; 95% confidence interval [CI], -0.84 to -0.73; P < 0.0001), red perception (r = -0.73; 95% CI, -0.79 to -0.65; P < 0.0001), and Ishihara color plate reading (r = -0.68; 95% CI, -0.79 to -0.66; P < 0.0001) were each strongly and highly significantly correlated with the diagnosis and degree of RAPD. Brightness sense and red perception were each able to discriminate almost all the area under ROC for the diagnosis of RAPD (area of 0.99; 95% CI, 0.98-1.00; P < 0.0001; area of 0.93; 95% CI, 0.90-0.96; P < 0.0001, respectively). Sensitivity and specificity of brightness sense in detection of RAPD were 99% (95% CI, 0.97-1.00) and 95% (95% CI, 0.91-0.98), respectively. The red perception test was only slightly less accurate. CONCLUSIONS: Rapid, simple assessments of brightness sense and color perception provide accurate methods to facilitate the diagnosis of optic neuropathy and may prove to be valuable in screening for optic neuropathy or alternatives to the swinging flashlight test.

Regional correlation of structure and function in glaucoma, using the Disc Damage Likelihood Scale, Heidelberg Retina Tomograph, and visual fields.

PURPOSE: To evaluate relationships between the Disc Damage Likelihood Scale (DDLS), global and sectoral structural parameters provided by the Heidelberg Retina Tomograph (HRT), and global and regional functional loss in visual field (VF) testing. DESIGN: Consecutive observational case series. PARTICIPANTS: One hundred ten eyes from 110 patients categorized as glaucoma, glaucoma suspect, or normal. METHODS: Participants were examined clinically to grade the DDLS score and were tested with HRT and Swedish Interactive Threshold Algorithm standard 24-2 VF tests. All tests were performed within 6 months of each other by examiners masked to the other findings. For each patient, the eye with the worse mean deviation (MD) of the VF test was enrolled in the study. Each field was divided into 6 sectors based on a published scheme, and the MD for each sector was calculated. The relationships among clinical DDLS score, HRT parameters, and VF indexes were analyzed by correlation coefficients and linear regression analysis. MAIN OUTCOME MEASURES: The relationship between the DDLS score, global and sectoral optic disc (HRT) parameters, and global and sectoral VF MDs was evaluated. RESULTS: The DDLS showed significant correlation with all global and sectoral VF indexes (r = -0.39 to -0.62, all Ps < 0.0001) and with sectoral rim area HRT measurements (r = -0.27 to -0.51, all Ps < 0.006). The DDLS correlated most strongly with superior and inferior regional data from HRT and VF, and less well with temporal and nasal data. Heidelberg Retina Tomograph rim area and rim volume were the only HRT parameters to correlate moderately with global VF MD (r = 0.30, P = 0.0018, and r = 0.28, P = 0.0030, respectively). Sectoral HRT rim area correlated moderately strongly with the corresponding sectoral VF MDs in the superior and inferior sectors (r = 0.35-0.46, P = <0.0001-0.04). CONCLUSION: Clinical disc assessment and laser tomographic data that determine the state of the neuroretinal rim are associated with sensitivity loss in VF testing in corresponding regions.