Congenital isolated unilateral third nerve palsy in children: the diagnostic contribution of high-resolution MR imaging.

PURPOSE: To describe the neuroanatomical correlates of unilateral congenital isolated oculomotor palsy by means of high-resolution MRI. METHODS: Children with a clinical diagnosis of congenital isolated oculomotr palsy and with a high-resolution MRI acquisition targeted on the orbits and cranial nerves were selected and included in the study. An experienced pediatric neuroradiologist evaluated all the exams, assessing the integrity and morphology of extraocular muscles, oculomotor, trochlear and abducens nerves as well as optic nerves and globes. Clinical data and ophthalmologic evaluations were also collected. RESULTS: Six children (age range: 1-16 years; males: 3) were selected. All patients showed, on the affected side (left:right = 5:1), anomalies of the III nerve and extraocular muscles innervated by the pathological nerve. One patient had complete nerve agenesis, two patients showed a diffuse thinning of the nerve, from the brainstem to the orbit and 3 patients showed a distal thinning of the oculomotor nerve, starting at the level of the cavernous sinus. In all cases atrophy of corresponding muscles was noticed, but the involvement of the affected muscles varied with the nervous pattern of injury. CONCLUSIONS: High-resolution MRI represents a valuable tool for the diagnosis of III nerve anomalies in unilateral congenital IOP, showing different patterns of nerve involvement and muscular atrophy.

Prevalence of dry eye in video display terminal users: a cross-sectional Caucasian study in Italy.

PURPOSE: To estimate the prevalence of dry eye among video-terminal (VDT) users and to assess risk factors for dry eye in this population. STUDY DESIGN: A single-centre, cross-sectional study was carried out on subjects employed as VDT workers and on a control group. METHODS: Demographic data, years spent working at a VDT, number of effective hours at VDT/day, number and hours of breaks/day were considered. All subjects underwent a complete ophthalmic examination and completed the Italian version of the computer vision symptom scale 17-item (CVSS17) questionnaire. Both groups were classified as definite, suspect and non-dry eye syndrome (DES). RESULTS: One-hundred and ninety four subjects completed the study; 70 (36.1%) of which represented the control group, and 124 (63.9%) represented the VDT group. Among VDT workers, 29 (23.4%) presented definite DES and 55 (44.4%) suspect DES, while among controls, only 2 (2.9%) presented definite DES and 37 (52.8%) suspect DES. In the univariate analysis, the DES group was older (p < 0.001), spent more time a day at VDT (p < 0.001), used VDT from more time (p < 0.001), instilled artificial tears (p = 0.031), and presented worst quality of life (p < 0.001). At the multivariate analysis, only age and time at VDT retained association with DES (OR 1.05; 95% CI 1.01-1.09; p = 0.01 and OR 1.57; 95% CI 1.07-2.02; p = 0.017, respectively). CONCLUSIONS: The global increase of VDT workers is accompanied by a higher frequency of ocular complaints. Older subjects and people spending more than 4 h a day at VDT are at major risk to develop DES and should take precautions to prevent the onset of the disease.

Macular staphyloma in patients affected by Joubert syndrome with retinal dystrophy: a new finding detected by SD-OCT.

PURPOSE: Joubert syndrome (JS) is an inherited autosomal recessive or X-lined disorder characterized by a congenital malformation of the mid-hindbrain and a large spectrum of clinical features. It is estimated that retinal dystrophy is present in association with the typical neurological findings in about one-third of the patients. The aim of this study is to better characterize the macular region in JS patients with and without retinal dystrophy. METHODS: We describe six individuals affected by JS as demonstrated by the presence of the typical "molar tooth sign" on MRI. The presence of retinal dystrophy was assessed by fundus examination and electrophysiology by means of full-field electroretinogram (ERG) and visual evoked potentials (VEP) at five spatial frequencies (300-15 min of arc). The macular region was examined with spectral domain optical coherence tomography (SD-OCT). All the exams were performed in awake conditions. All the patients underwent next-generation-sequencing analysis of known JS genes. RESULTS: Pathogenic biallelic variants in either the INPP5E gene or the AHI1 gene were detected in two pairs of siblings, all positive for retinal dystrophy. Genetic testing yielded no results in the remaining two patients, one with bilateral coloboma and retinal dystrophy and the other with normal fundus appearance. Decimal best-corrected visual acuity was between 0.1 and 1.0. In the two pairs of siblings, SD-OCT revealed a posterior staphyloma centred on the fovea, in one case associated with cystoid macular oedema. Macular morphology was just slightly altered in the fifth patient and completely normal in the last patient. Refractive error was between + 2.50 diopter sphere (DS) and - 8 DS and - 4 diopter cylinder ax 45°. ERG waves were markedly lower than the normal limits in both scotopic and photopic components in the two pairs of siblings and in the fifth subject, with VEP P100 latencies and amplitudes delayed and reduced in all spatial frequencies. ERG and VEP were within normal limits in the last patient. CONCLUSIONS: To our knowledge, macular staphyloma has not been described before in JS. Further work is warranted to assess the true prevalence of staphyloma in JS and its connection to retinal dystrophy.

Neuro-ophthalmological disorders in cerebral palsy: ophthalmological, oculomotor, and visual aspects.

AIM: Cerebral visual impairment (CVI) is a disorder caused by damage to the retrogeniculate visual pathways. Cerebral palsy (CP) and CVI share a common origin: 60 to 70% of children with CP also have CVI. We set out to describe visual dysfunction in children with CP. A further aim was to establish whether different types of CP are associated with different patterns of visual involvement. METHODS: A total of 129 patients (54 females, 75 males; mean age 4 y 6 mo, SD 3 y 5 mo; range 3 mo-15 y) with CP (51 with diplegia, 61 with tetraplegia, and 17 with hemiplegia; 62 [48%] of participants were able to walk) and CVI enrolled at the Centre of Child Neuro-ophthalmology (at the Department of Child Neurology and Psychiatry, IRCCS 'C. Mondino Institute of Neurology', University of Pavia) underwent an assessment protocol including neurological examination, developmental and/or cognitive assessment, neuro-ophthalmological evaluation including ophthalmological assessment, evaluation of visual acuity, contrast sensitivity, optokinetic nystagmus, visual field and stereopsis, and neuroradiological investigations. RESULTS: Visual dysfunction in diplegia was characterized mainly by refractive errors (75% of patients), strabismus (90%), abnormal saccadic movements (86%), and reduced visual acuity (82%). The participants with hemiplegia showed strabismus (71%) and refractive errors (88%); oculomotor involvement was less frequent (59%). This group had the largest percentage of patients with altered visual field (64%). Children with tetraplegia showed a severe neuro-ophthalmological profile, characterized by ocular abnormalities (98%), oculomotor dysfunction (100%), and reduced visual acuity (98%). INTERPRETATION: Neuro-ophthalmological disorders are one of the main symptoms in CP. Each clinical type of CP is associated with a distinct neuro-ophthalmological profile. Early and careful neuro-ophthalmological assessment of children with CP is essential for an accurate diagnosis and for personalized rehabilitation.

Effect of palmitoylethanolamide on inner retinal function in glaucoma: a randomized, single blind, crossover, clinical trial by pattern-electroretinogram.

Glaucoma is a neurodegenerative disease, our study aimed to evaluate the potential effects of Palmitoylethanolamide (PEA) supplementation on RGCs function by PERG examination, and to record effects on intraocular pressure, visual field and quality of life. It was a single centre, randomized, prospective, single blind, two treatment, two period crossover study on stable glaucoma patients on topical monotherapy comparing current topical therapy alone or additioned with PEA 600 mg one tablet a day. At baseline, at 4 and at 8 months, all patients underwent to complete ophthalmic examination, pattern electroretinogram, visual field, and quality of life evaluation. 40 patients completed the study: mean age 66.6 ± 7.6 years; 21 (52.5%) male; 35 POAG (87.5%). At baseline, most patients had an early visual field defect, the IOP was well controlled. At the end of the PEA 600 mg supplementation, a significantly higher (mean 0.56 µV, 95% CI 0.30-0.73, p < 0.001) in the P50-wave amplitude was observed; in the PEA period a significantly lower IOP (- 1.6 mmHg, 95% CI - 2 to 1.2, p < 0.001) and higher quality of life scores (+ 6.7, 95% CI 4-9.9, p < 0.001) were observed. Our study is the first to show promising effects of PEA on PERG and on quality of life in glaucoma patients.

Dry eye syndrome-related quality of life in glaucoma patients.

PURPOSE: To verify the presence of dry eye syndrome (DES) in treated patients with glaucoma and to analyze DES's impact on the patients' quality of life (QOL) versus the control group. METHODS: In this observational cross-sectional study, 61 patients were enrolled at a clinical practice. Patients were divided into three groups by number of glaucoma drops instilled per day (G1=1 drop/day, G2=2 drops/day, G3=3 drops/day). A control group of 20 subjects was also selected (G0). All subjects were submitted to a complete ocular examination (including tear function and ocular surface status) and completed the 25-item National Eye Institute Visual Function Questionnaire (NEI-VFQ), Glaucoma Symptom Scale (GSS) questionnaire, and Ocular Surface Disease Index (OSDI). DES was defined as presence of punctate keratitis and decreased break-up time. Statistical analysis was performed applying the Kruskal-Wallis analysis of variance and Mann-Whitney U tests (to compare median values between groups) as well as the chi2 and Fisher test (to verify significant differences). RESULTS: A total of 40% of G3 and 39% of G2 patients presented DES versus 11% of G1 and 5% of G0 (p=0.01). QOL was significantly influenced and altered (NEI-VFQ 25 total mean and GSS total mean and symptoms average: p=0.0085, p=0.006, and p=0.03, respectively). OSDI pointed out differences by group: 26% of G2 and 15% of G3 presented moderate OSDI and 15% of G3 and 8.7% of G2 severe OSDI (p>0.05). CONCLUSIONS: Patients with topically treated glaucoma present DES more often than a similar control group (p=0.01). The presence of DES negatively influences the patient's QOL. The patients with glaucoma's ocular surface status should be evaluated regularly to ensure the timely detection and treatment of pathologic signs on the ocular surface.

An in vivo confocal, prospective, masked, 36 months study on glaucoma patients medically treated with preservative-free or preserved monotherapy.

The aim of this study was to evaluate the in vivo effects at 3 years of preservative-free tafluprost on corneal health. It was a prospective, masked, study on consecutive patients with a new prescription of preservative-free (PF) tafluprost (naïve-N or switched-S, 44 and 14 patients), and preserved (P) bimatoprost 0.003% or travoprost 0.004% (P-group, 35 patients). A complete ophthalmic examination and an in vivo corneal confocal microscopy evaluation were performed at baseline and every 6 months for 3 years. Ninety-three patients were enrolled, clinical parameters were similar in the groups at baseline, apart from intraocular pressure (IOP) which was lower in the S-group (p = 0.012). Both at baseline and over time, confocal microscopy parameters had different trends. At baseline, keratocyte activation was similar in the three groups (p = 0.43) but over the next months naïve patients treated with PF-tafluprost presented a significant (p = 0.004) reduction in keratocyte activation. Sub-basal nerves tended to increase in patients switched to PF-tafluprost (p = 0.07) while were stable in the other two groups (p = 0.11 in PF and 0.40 in P group). Grade of tortuosity was stable over time in the three groups. Beading-like formations were stable over time for the P- and the PF-group, while significantly increased in the S-group (p = 0.027). Endothelial density values were statistically different at baseline (p = 0.007), they decreased both in PF-group and in S-group (p = 0.048 and 0.001, respectively), while increased in P-group (p = 0.006). Our study is the first to show that a PF-tafluprost formulation does not significantly alter the corneal structures as examined by confocal microscopy after 36 months of topical daily therapy, while improving corneal alterations due to chronic preserved therapies.

Clinical and molecular genetics of Leber's congenital amaurosis: a multicenter study of Italian patients.

PURPOSE: To identify the molecular basis of Leber's congenital amaurosis (LCA) in a cohort of Italian patients and to perform genotype-phenotype analysis. METHODS: DNA samples from 95 patients with LCA were analyzed by using a microarray chip containing disease-associated sequence variants in eight LCA genes. In addition, all patients in whom no mutations were identified by microarray were subjected to sequence analysis of the CEP290 gene. Patients with mutations identified underwent a detailed ophthalmic evaluation. RESULTS: Disease-causing mutations were identified in 28% of patients, and twelve novel variants were identified. Mutations occurred more frequently in the RPE65 (8.4%), CRB1 (7.4%), and GUCY2D (5.2%) genes. Mutations in CEP290 were found in only 4.2% of the patients analyzed. Clinical assessment of patients carrying RPE65 or CRB1 mutations revealed the presence of retained visual capabilities in the first decade of life. RPE65 mutations were almost always associated with normal macular thickness, as assessed by optical coherence tomography (OCT), whereas CRB1 mutations were associated with reduced retinal thickness and a coarsely laminated retina. Fundus autofluorescence was mostly observed in patients with RPE65 and GUCY2D mutations and was not elicitable in patients carrying CRB1. CONCLUSIONS: RPE65 gene mutations represented a significant cause of LCA in the Italian population, whereas GUCY2D and CEP290 mutations had a lower frequency than that found in other reports. This finding suggests that the genetic epidemiology of LCA in Italy is different from that reported in the United States and in northern European countries. Autofluorescence in patients with RPE65 mutations was more frequently associated with preserved retinal thickness, which suggests that these mutations are not associated with progression of retinal degeneration. Therefore, normal retinal thickness (identified with OCT) and fundus autofluorescence may be the means with which to identify patients with LCA who carry RPE65 mutations, which are expected to be a potential gene therapy target in the near future.

Spectrum of visual disorders in children with cerebral visual impairment.

Cerebral visual impairment is a visual function deficit caused by damage to the retrogeniculate visual pathways in the absence of any major ocular disease. It is the main visual deficit in children in the developed world. Preperinatal hypoxic-ischemic damage is the most frequent cause of cerebral visual impairment, but the etiology is variable. The authors set out to evaluate the presence of visual disorders not attributable to any major ocular pathology in a sample of children with central nervous system disease and to describe the clinical picture of cerebral visual impairment in this cohort. One hundred twenty-one patients with central nervous system damage and visual impairment underwent a protocol developed at the authors' center that included neurologic, neurophthalmologic, and neuroradiologic assessments (brain magnetic resonance imaging). Reduced visual acuity was found in 105 of 121 patients, reduced contrast sensitivity in 58, abnormal optokinetic nystagmus in 88, and visual field deficit in 7. Fixation was altered in 58 patients, smooth pursuit in 95, and saccadic movements in 41. Strabismus was present in 88 patients, and abnormal ocular movements were found in 43 patients. Of the 27 patients in whom they could be assessed, visual-perceptual abilities were found to be impaired in 24. Fundus oculi abnormalities and refractive errors were frequently associated findings. This study confirms that the clinical expression of cerebral visual impairment can be variable and that, in addition to already well-documented symptoms (such as reduced visual acuity, visual field deficits, reduced contrast sensitivity), the clinical picture can also be characterized by oculomotor or visual-cognitive disorders. Cerebral visual impairment is often associated with ophthalmologic abnormalities, and these should be carefully sought. Early and careful assessment, taking into account both the neurophthalmologic and the ophthalmologic aspects, is essential for a correct diagnosis and the development of personalized rehabilitation programs.

Changes in the optic disc excavation of children affected by cerebral visual impairment: a tomographic analysis.

PURPOSE: To obtain quantitative data on the optic disc excavation in children affected by cerebral visual impairment (CVI) by using the Heidelberg Retinal Tomograph (HRT)-II (Heidelberg Engineering, Heidelberg, Germany). METHODS: A total of 24 subjects affected by CVI (mean age, 7.28 years) were examined: 16 in alert conditions and 8 under general anesthesia. The following parameters of the optic nerve head were examined: disc area, cup area, rim area, cup volume, rim volume, cup-to-disc area ratio, mean cup depth, maximum cup depth, cup shape measure, and mean retinal nerve fiber layer (RNFL) thickness. The tomographic results in children with CVI were compared with those of 88 normal, alert subjects of similar age. RESULTS: The optic disc of patients with CVI appeared smaller than normal. Its excavation, however, was more pronounced. Several tomographic parameters were altered in CVI-affected subjects. Statistical analysis showed a highly significant probability in cup-to-disc area ratio (P < 0.01, both eyes), rim area (P < 0.01, both eyes), cup shape measure (P < 0.01, right eye; P < 0.01, left eye), and mean RNFL thickness (P < 0.01, right eye; P < 0.01, left eye). A novel observation was temporal atrophy of the optic nerve head in CVI. CONCLUSIONS: The data provide a tridimensional, objective evaluation of the anatomic alterations of the optic nerve head in children with CVI. Furthermore, tomographic standards for optic disc shape in normal children are set for the first time.

Long-term follow-up of anatomical and functional macular changes after a single intravitreal implant of dexamethasone 0.7 mg for radiation macular edema secondary to proton beam therapy for choroidal melanoma.

PURPOSE: To describe the efficacy and safety of a single intravitreal implant of dexamethasone in a patient affected by radiation maculopathy due to proton beam radiotherapy for choroidal melanoma. PATIENT AND METHODS: Retrospective data of a 46-year-old woman treated with a single intravitreal injection of dexamethasone for radiation maculopathy due to proton beam radiotherapy were collected. The main outcome measures were best-corrected visual acuity and central retinal thickness. Intraocular pressure, anterior segment evaluation with slit lamp, macular changes depicted with spectral domain optical coherence tomography, retinal perfusion studied with fundus fluorescein angiography, and grade of macular edema using the Horgan classification were also evaluated during a 16-month follow-up. RESULTS: Macular edema occurred 25 months after radiation treatment in the left eye. The patient underwent a single intravitreal implant of dexamethasone. Preinjection visual acuity and central retinal thickness were 6/12 and 502 µm, respectively. After 8 months, visual acuity was 6/6 and remained stable until 16 months. Central retinal thickness was 269 µm at 16 months. CONCLUSION: A single intravitreal implant of dexamethasone could effectively and stably improve visual acuity and central retinal thickness in some patients with radiation macular edema for 16 months after injection.

From benzalkonium chloride-preserved Latanoprost to Polyquad-preserved Travoprost: a 6-month study on ocular surface safety and tolerability.

BACKGROUND: To evaluate the safety and tolerability of Polyquad-preserved Travoprost (PQ-Travoprost) in patients previously treated with benzalkonium chloride (BAK)-preserved Latanoprost. METHODS: Cohort 6-month study on open-angle glaucoma or ocular hypertension patients. Complete ophthalmic examination, intraocular pressure (IOP) measurement and ocular surface status (tear film break-up time [TF-BUT], corneal staining and ocular surface disease index [OSDI]) were evaluated at baseline and 6 months later. RESULTS: A total of 44 patients were enrolled. Median (interquartile range [IQR]) baseline IOP was 18 (15.5 - 21) and 16 (14 - 17) mmHg (p < 0.0001) after 6 months. At baseline, 18 (40.9%) patients presented an IOP of < 18 mmHg, 11 (25%) < 16 mmHg, 2 (4.3%) < 14 mmHg and 1 (2.3%) < 12 mmHg; 6 months later the proportions were 36 (81.8%) (p < 0.0001), 21 (47.7%) (p = 0.00075), 8 (18.2%) (p = 0.0143) and 6 (13.6%) (p = 0.0253). Concerning safety, TF-BUT improved from 8 [IQR 6 - 10] to 10 [IQR 8 - 12] s (p < 0.0001). No eye developed corneal staining; punctate keratitis was absent in 13 (29.5%) patients at baseline and in 31 (70.4%) after 6 months (p < 0.001). OSDI changed from 16 (10 - 30) to 9 (2 - 20). CONCLUSIONS: No patient treated with PQ-Travoprost developed ocular surface disease after 6 months of monotherapy, whereas many patients reached a good IOP control with lower IOP values. Ocular surface status statistically improved when examined by TF-BUT and corneal staining.

Visual-perceptual impairment in children with periventricular leukomalacia.

We set out to define visuo-perceptual impairment related to periventricular leukomalacia (PVL) using the Developmental Test of Visual Perception (DTVP). Correlations were sought between visual-perceptual deficits and DTVP profile and neuroradiological and neurophthalmological findings. The DTVP was administered to 20 children (m/f: 10/10), aged between 5 and 8 years (mean: 6.95 years), presenting with: spastic diplegia; PVL documented by brain MRI; normal or mildly impaired visual acuity; mild-moderate upper limb functional impairment. The mean General Visual-Perceptual Quotient was impaired, showing a great variability among the patients. Despite this, an uneven DTPV profile, characterised by a significant difference between the VMIQ and the Non-Motor Visual-Perceptual Quotient (P < 0.001) and a poor result on the Closure subtest (identification of whole figures from incomplete visual information) was observed in all the subjects. This profile reflects a deficit in eye-hand coordination and in praxic-constructional abilities and could be the expression of malfunctioning of the occipital-parietal pathway of visual integration, the so-called 'dorsal stream,' a hypothesis reinforced by the emergence of a statistically significant correlation between the neuroradiological data and the presence of visual-perceptual impairment.

Visual acuity in the first two years of life in healthy term newborns: an experience with the teller acuity cards.

Teller Acuity Cards are a new "preferential looking" procedure for the evaluation of visual acuity in newborns and infants. We used this test to assess, longitudinally, visual acuity in 60 healthy term newborns followed up from birth to two years of age. In order to have a set of comparison parameters for use in studies of newborns at risk of developing visual system impairment, the relative maturational curve was plotted. The acuity values of our sample are in line with those reported by other authors in the literature and they represent the first set of such data referring to a group of healthy term newborns in Italy. This paper provides a visual acuity curve for the first two years of life obtained from healthy term newborns, a curve which could prove useful for reference when this technique is used on newborns at risk of developing neurological and especially visual problems.

Morphologic and functional analysis of the optic nerve in premature and term children with OCT, HRT, and pVEP: a 10-year resurvey.

PURPOSE: Most studies on premature newborns have focused on infants of less than 28 weeks of gestational age (GA) due to their increased risk of developing diseases, such as premature retinopathy. Studies on premature infants born between 28 and 35 weeks GA with normal development are less frequent. The aim of our study was to identify subclinical morphologic or functional defects in these children. METHODS: We evaluated 14 premature newborns at birth (mean gestational age, 33.45 weeks) with a neuro-ophthalmologic examination and patterned visual evoked potentials (pVEP). The same subjects were surveyed when they were young children (mean age, 7.5 ± 0.2 years) using Heidelberg retinal tomography (HRT) and optical coherence tomography (Stratus OCT). The pVEP studies were performed as transient (temporal frequency, 1.96 Hz) and steady-state (7.5-Hz temporal frequency). A complete ophthalmic examination also was performed. The data were compared to those from 15 term newborns who were examined in the same manner (mean age, 9.8 ± 0.3 years). RESULTS: A statistically significant thickening of the macular temporal and inferior nerve fibers was found on OCT in premature newborns. The thickness of the superior and inferior retinal nerve fiber layer (RFNL) also was reduced. A difference also was found in rim area thickness based on HRT. Multiple significant P values were found in the VEP P100 peak time and steady-state amplitudes at the time of birth, but not at the time of morphologic analysis. CONCLUSIONS: Healthy, premature newborns may have morphologic abnormalities of the optic nerve. These abnormalities do not cause visual acuity or functional decreases.

Efficacy and safety of azithromycin 1.5% eye drops in paediatric population with purulent bacterial conjunctivitis.

OBJECTIVE: To determine the efficacy and safety of azithromycin 1.5% eye drops in a paediatric population with purulent bacterial conjunctivitis. PATIENTS AND METHODS: This was a multicentre, international, randomised, investigator-masked study in 286 children with purulent discharge and bulbar conjunctival injection. Patients received either azithromycin 1.5% eye drops (twice daily for 3 days) or tobramycin 0.3% eye drops (every 2 h for 2 days, then four times daily for 5 days). Clinical signs were evaluated on day (D) 0, 3 and 7, and cultures on D0 and D7. The primary variable was the clinical cure (absence of bulbar conjunctival injection and discharge) on D3 in the worse eye for patients with positive cultures on D0. RESULTS: 286 patients (mean age 3.2 years; range 1 day-17 years) were included; 203 had positive cultures on D0. Azithromycin was superior to tobramycin in clinical cure rate on D3 (47.1% vs 28.7%, p=0.013) and was non-inferior to tobramycin on D7 (89.2% vs 78.2%, respectively). Azithromycin treatment eradicated causative pathogens, including resistant species, with a similar resolution rate to tobramycin (89.8% vs 87.2%, respectively). These results were confirmed in a subgroup of patients younger than 24 months old. CONCLUSIONS: Azithromycin 1.5% eye drops provided a more rapid clinical cure than tobramycin 0.3% eye drops in the treatment of purulent bacterial conjunctivitis in children, with a more convenient twice-a-day dosing regimen.

Ocular surface disease and glaucoma: how to evaluate impact on quality of life.

PURPOSE: To record signs and symptoms of ocular surface disease (OSD) in patients treated with Intra Ocular Pressure (IOP)-lowering medications; to evaluate the relationship between signs and symptoms; and to identify how to diagnose and follow OSD and its impact on the quality of life in such patients. METHODS: A prospective observational study of enrolled consecutive topically treated open-angle glaucoma or ocular hypertension patients: patients presenting systemic or ocular conditions that could interfere with ocular surface status were excluded. Enrolled patients underwent a complete ophthalmic examination comprehensive of evaluation of tear film break-up time (TF-BUT) and fluorescein corneal staining (keratitis punctatae) and who completed the Italian version of both the National Eye Institute-Visual Function Questionnaire (NEI-VFQ) 25 and the Glaucoma Symptom Scale (GSS) questionnaires. RESULTS: 233 patients adhered to a study protocol. Punctatae keratitis was detected in 70 (30%) eyes; abnormal TF-BUT in 67 (28.8%) patients: 97 patients (42.1%) presented an OSD. The abnormal values were gender-independent, keratitis was statistically related to age (P=0.01) and number of instillations/die (P=0.0007). TF-BUT was related to the IOP value (P<0.0001). The NEI ocular pain subscale was statistically related to TF-BUT (P=0.017); GSS was both related to TF-BUT and punctatae keratitis (P<0.00001). CONCLUSIONS: Many patients present an OSD related to therapy, and this affects their quality of life. The use of fixed combinations to reduce surface exposition and of benzalkonium chloride-free formulations should be encouraged to reduce and contain the onset or worsening of this secondary condition in glaucoma patients. The GSS has shown a good relation to signs and should be routinely used to evaluate the impact of OSD on the quality of life.

Risk factors to develop ocular surface disease in treated glaucoma or ocular hypertension patients.

PURPOSE: To identify risk factors for developing ocular surface disease (OSD), to verify the prevalence of OSD, and to record efficacy of questionnaires in identifying symptoms' impact on patients' quality of life.
 METHODS: . This was an observational, cross-sectional study of patients with topically treated glaucoma. Tear film break-up time (TFBUT) and punctate keratitis were evaluated; 2 quality-of-life questionnaires (National Eye Institute-Visual Function Questionnaire 25 and Glaucoma Symptom Scale) were submitted to all patients. Class of previous and current intraocular pressure (IOP)-lowering drugs, number of drugs, number of drops/day, and total and current benzalkonium chloride (BAK) exposure were collected.
 RESULTS: . A total of 233 patients completed the study. TFBUT was abnormal in 71 (30.5%) eyes; punctate keratitis was present in 74 (31.7%). Keratitis was more frequent with increasing number of eyedrops (p=0.008) and number of instillations per day (p=0.009). Ocular surface disease was present in 97 (41.6%) patients and was statistically related to number of medications used (p=0.026). The univariate analysis pointed out that patients with OSD were older (p=0.04), had lower IOP values (p=0.03), were topically treated for more time (p<0.0001), had assumed more BAK (p<0.0001), and presented worst quality of life (p<0.01). The multivariate analysis found that OSD was related to number of medications used (p=0.002), prolonged use of preserved medications (p=0.005), and total BAK exposure (p<0.001).
 CONCLUSIONS: There is clinical evidence that the number of medications, their prolonged use, and the total BAK exposure are risk factors to develop OSD in patients with glaucoma. To prevent OSD onset, BAK exposure and the number of topical medications should be reduced.

The Italian version of the Glaucoma Symptom Scale Questionnaire: translation, validation, and reliability.

PURPOSE: To validate the Italian version of the Glaucoma Symptom Scale (GSS) Questionnaire and its symptoms and function subscales. METHODS: This transversal validation study enrolled nonhospitalized patients with glaucoma, and a reference sample of patients without eye diseases. Eligible participants had to be cognitively able to respond to a health status interview. The Italian self-administered versions of the 25-item National Eye Institute-Visual Function Questionnaire and the GSS Questionnaire were administered to all participants. Reliability and validity of the Italian translation of the GSS Questionnaire were tested using standard statistical methods for questionnaire validation. RESULTS: Ninety-seven patients were enrolled. Cronbach α coefficient ranged from 0.72 to 0.92 across subscales and eyes. Test-retest stability was >85% for each subscale and eye. The control group of participants had better scale scores across all dimensions of vision-targeted health-related quality of life captured by the GSS Questionnaire (P<0.05) and there were good correlations between responses GSS Questionnaire subscales and analogous domains of the 25-item National Eye Institute-Visual Function Questionnaire. CONCLUSIONS: The Italian version of the GSS Questionnaire has good validity, discriminatory power, internal consistence and reliability, showing psychometric properties comparable with those of the English version, and can therefore be used in clinical research as a specific measure of vision-related quality of life in Italian-speaking patients with ocular hypertension or glaucoma.