Paediatric Cogan´s syndrome - review of literature, case report and practical approach to diagnosis and management.

BACKGROUND: Cogan´s syndrome is a rare, presumed autoimmune vasculitis of various vessels characterized by interstitial keratitis and vestibular impairment accompanied by sensorineural hearing loss. Due to the rarity of Cogan´s syndrome in children, therapeutic decision making may be challenging. Therefore, a literature search was performed to collect all published paediatric Cogan´s syndrome cases with their clinical characteristics, disease course, treatment modalities used and their outcome. The cohort was supplemented with our own patient. MAIN TEXT: Altogether, 55 paediatric Cogan´s syndrome patients aged median 12 years have been reported so far. These were identified in PubMed with the keywords "Cogan´s syndrome" and "children" or "childhood". All patients suffered from inflammatory ocular and vestibulo-auditory symptoms. In addition, 32/55 (58%) manifested systemic symptoms with musculoskeletal involvement being the most common with a prevalence of 45%, followed by neurological and skin manifestations. Aortitis was detected in 9/55 (16%). Regarding prognosis, remission in ocular symptoms was attained in 69%, whereas only 32% achieved a significant improvement in auditory function. Mortality was 2/55. Our patient was an 8 year old girl who presented with bilateral uveitis and a history of long standing hearing deficit. She also complained of intermittent vertigo, subfebrile temperatures, abdominal pain with diarrhoea, fatigue and recurrent epistaxis. The diagnosis was supported by bilateral labyrinthitis seen on contrast-enhanced magnetic resonance imaging. Treatment with topical and systemic steroids was started immediately. As the effect on auditory function was only transient, infliximab was added early in the disease course. This led to a remission of ocular and systemic symptoms and a normalization of hearing in the right ear. Her left ear remained deaf and the girl is currently evaluated for a unilateral cochlear implantation. CONCLUSIONS: This study presents an analysis of the largest cohort of paediatric Cogan´s syndrome patients. Based on the collected data, the first practical guide to a diagnostic work-up and treatment in children with Cogan´s syndrome is provided.

Chromatic Full-Field Stimulus Thresholds in Patients with Treatment-Naive Age-Related Macular Degeneration.

PURPOSE: To evaluate retinal sensitivity using chromatic full-field stimulus thresholds (FST) in patients with treatment naive exudative age-related macular degeneration (e-ARMD) before and during the first intravitreal anti-vascular endothelial growth factor (anti-VEGF) treatment. PATIENTS AND METHODS: After general ophthalmological examinations to diagnose patients with e-ARMD the FST, the central foveal thickness (FCt) and the visual acuity were assessed in 20 eyes of 20 patients during this prospective study. Examinations were performed before and during the first treatment series with three intravitreal anti-VEGF injections. Normal values for FST were assessed in 19 eyes of 19 healthy subjects. Results were analyzed using Student's two-tailed t-test and Pearson's correlation coefficients between all functional parameters. RESULTS: At baseline and before the 1st intravitreal anti-VEGF treatment, a moderately significant negative correlation between VA and FCt was found in the e-ARMD group (p = 0.02, r = -0.45 and p = 0.03, r = -0.45), respectively. After the 2nd intravitreal anti-VEGF injection, no significant correlation between VA and FCt was found (p = 0.12). However, a significant correlation between FCt and blue FST was evident (p = 0.04, r = 0.4). After the 3rd intravitreal anti-VEGF treatment, there was no correlation evident between VA and FCt (p = 0.31) but a high significant correlation between FCt and FST using red (p = 0.01, r = 0.53), green (p = 0.002, r = 0.6) and blue light (p = 0.007, r = 0.66). CONCLUSION: During anti-VEGF treatment in patients with e-ARMD, the FST test showed higher significant correlations with the morphology measured by FCt, as it is the case for VA. These findings support that the FST test might serve as a valuable diagnostic tool for monitoring patients with e-ARMD and enhance functional assessment of retinal function under treatment with anti-VEGF.

Influence of pupillary dynamics on the defocus curve of eyes implanted with diffractive multifocal lenses: a randomized study / Influência da dinâmica pupilar na curva de desfoco de olhos implantados com lentes multifocais difrativas: um estudo randomizado

ABSTRACT Purpose: To evaluate the influence of pupil dynamics on the defocus profile and area-of-focus of eyes implanted with a diffractive multifocal intraocular lens (IOL). Methods: This prospective randomized trial was conducted at the Department of Ophthalmology, School of Medicine of Ribeirão Preto, University of São Paulo, Brazil. Thirty-eight patients were randomly assigned to receive the multifocal SN6AD1 (n=20) or the aspheric monofocal SN60WF (aIOL) (n=18) IOLs bilaterally. Dynamic pupillometry, visual acuity for distance and near, corrected and uncorrected, and a defocus profile were assessed postoperatively. The area-of-focus was calculated using an empirical polynomial model of the defocus profile. Results: Sixteen patients (32 eyes) in the multifocal SN6AD1 group and 17 patients (34 eyes) in the aspheric monofocal SN60WF group completed the 1-year follow-up. There were no significant between-group differences in monocular uncorrected distance or near visual acuity. The defocus profiles of the mfIOL group showed a double peak, whereas those of the aspheric monofocal SN60WF group showed only one peak, which is typical for a monofocal intraocular lens. The area-of-focus of the aIOL group (4.66 ± 1.51 logMARxD) was significantly different from that of the multifocal SN6AD1 (1.99 ± 1.31 logMARxD). Pupil size at maximum contraction after exposure to a flash of 30 cd/m2 for 1 second was significantly correlated with a better area-of-focus in the multifocal SN6AD1 group (r=0.54; p=0.0017), whereas this was not the case in the aspheric monofocal SN60WF group. Conclusion: These findings indicate that in eyes implanted with an multifocal SN6AD1, the smaller the pupil size, the better is the area-of-focus and hence the better is the visual performance. This correlation was not found for the aspheric monofocal SN60WF.(AU)

RESUMO Objetivo: Avaliar a influência da dinâmica pupilar na curva de desfoco de olhos implantados com lente intraoculares multifocais difrativas. Métodos: Estudo prospectivo e randomizado realizado na Faculdade de Medicina de Ribeirão Preto - Universidade de São Paulo - Departamento de Oftalmologia. Trinta e oito pacientes foram aleatoriamente designados para receber bilateralmente lentes intraoculares SN6AD1 (n=20) (mfIOL) ou SN60WF (n=18) (aIOL). Além da acuidade visual para longe e perto, corrigida e não corrigida, e curva de desfoco, foi ainda realizada pupilometria dinâmica. A área sob a curva de desfoco foi calculada usando um modelo polinomial empírico. Resultados: Um total de 16 e 17 pacientes (n=32 e 34 olhos) completaram 1 ano de seguimento nos grupos mfIOL e aIOL, respectivamente. Não houve diferenças significativas entre grupos para as acuidades visuais seja para longe ou perto. As curvas de desfoco do grupo mfIOL mostraram um pico duplo; enquanto o SN60WF mostrou apenas um pico, típico para uma lente intraoculares monofocal. A média da área sob a curva de desfoco do grupo aIOL foi (4,66 ± 1,51 logMAR.dp), e essa é estatisticamente significante diferente da métrica do grupo mfIOL (1,99 ± 1,31 logMAR.dp). A pupila na contração máxima após a exposição a um flash de 30 cd/m2 por 1 segundo foi significativamente correlacionada com uma melhor área de foco no grupo mfIOL (r=0,54; p=0,0017), essa relação não foi observada para o grupo aIOL. Conclusão: Estes dados indicam que quanto menor a pupila durante contração, melhor é a área sob a curva de desfoco e, portanto, o desempenho visual dos olhos implantados com essa mfIOL. Esta correlação não foi encontrada para lentes intraoculares monofocais.(AU)

Influence of pupillary dynamics on the defocus curve of eyes implanted with diffractive multifocal lenses: a randomized study.

PURPOSE: To evaluate the influence of pupil dynamics on the defocus profile and area-of-focus of eyes implanted with a diffractive multifocal intraocular lens (IOL). METHODS: This prospective randomized trial was conducted at the Department of Ophthalmology, School of Medicine of Ribeirão Preto, University of São Paulo, Brazil. Thirty-eight patients were randomly assigned to receive the multifocal SN6AD1 (n=20) or the aspheric monofocal SN60WF (aIOL) (n=18) IOLs bilaterally. Dynamic pupillometry, visual acuity for distance and near, corrected and uncorrected, and a defocus profile were assessed postoperatively. The area-of-focus was calculated using an empirical polynomial model of the defocus profile. RESULTS: Sixteen patients (32 eyes) in the multifocal SN6AD1 group and 17 patients (34 eyes) in the aspheric monofocal SN60WF group completed the 1-year follow-up. There were no significant between-group differences in monocular uncorrected distance or near visual acuity. The defocus profiles of the mfIOL group showed a double peak, whereas those of the aspheric monofocal SN60WF group showed only one peak, which is typical for a monofocal intraocular lens. The area-of-focus of the aIOL group (4.66 ± 1.51 logMARxD) was significantly different from that of the multifocal SN6AD1 (1.99 ± 1.31 logMARxD). Pupil size at maximum contraction after exposure to a flash of 30 cd/m2 for 1 second was significantly correlated with a better area-of-focus in the multifocal SN6AD1 group (r=0.54; p=0.0017), whereas this was not the case in the aspheric monofocal SN60WF group. CONCLUSION: These findings indicate that in eyes implanted with an multifocal SN6AD1, the smaller the pupil size, the better is the area-of-focus and hence the better is the visual performance. This correlation was not found for the aspheric monofocal SN60WF.

Central mfERG amplitude ratio as a predictor for visual outcome of macular hole surgery.

PURPOSE: To investigate whether visual acuity improvement achieved after surgical treatment for macular hole (MH) can be predicted by preoperative multifocal ERG (mfERG) central/peripheral amplitude ratio. METHODS: Thirty patients with unilateral MH were included. Evaluations with comprehensive ophthalmological examination including best-corrected visual acuity (BCVA) were performed at baseline and 1, 3, 8, 24 and 48 weeks after surgery, while mfERG (Diagnosys LLC; 61 hexagons-30°) and spectral-domain optic coherence tomography (sOCT-Heidelberg Engineering) were performed at baseline and 2 months after surgery. mfERG results are shown by means of the ratio between the amplitudes' average from rings 1 and 2 (central) and rings 4 and 5 (peripheral): the P1 ratio. mfERG data from 20 normally sighted age-matched subjects was used for comparison. A macular hole index (MH index) was defined as the quotient between hole height and base measured on OCT. RESULTS: Twenty-six patients finished the 48-week follow-up. Mean ± SE (logMAR) preoperative BCVA was 0.93 ± 0.22 and improved in 0.25 ± 0.07 at 48 weeks. mfERG P1 ratio was reduced at baseline and increased significantly after surgery. A significant correlation was observed between preoperative P1 ratio and BCVA gain at week 8 (r = -0.42; P = 0.033). There was no significant correlation between preoperative MH index and postoperative BCVA (P > 0.05). CONCLUSION: Retinal function assessed using the ratio between central and peripheral mfERG responses might be used as predictor of visual acuity outcome after macular surgery for MH.

Decreased contrast sensitivity at high altitude.

BACKGROUND/AIMS: The aim of this study was to investigate a change in visual acuity and contrast sensitivity (CS) during high altitude exposure in healthy subjects due to the effects of hypobaric hypoxia. This study is related to the Tübingen High Altitude Ophthalmology study. METHODS: Visual acuity and Weber CS were tested monocularly using the Freiburger Visual Acuity and Contrast Test under standardised conditions in 14 healthy subjects at high altitude at the Capanna Margherita (4559 m, Italy) and compared with baseline measurements in Tübingen (341 m, Germany). Intraindividual differences between baseline and follow-up examinations were calculated by multivariate analysis of variance for repeated measures. Clinical parameters of peripheral oxygen saturation (SpO2) and heart rate (HR) as well as scores for acute mountain sickness (AMS) were correlated to psychophysical tests by Pearson's correlation coefficient. RESULTS: A significant decrease in CS with a mean effect size of -0.13 logCS was found for Weber CS (day 1=-0.16±0.22, p=0.01; day 2=-0.10±0.2, p=0.049; day 3=-0.12±0.19, p=0.03) at high altitude compared with baseline. Visual acuity remained unchanged. Decreased CS correlated with SpO2 (r=0.53, p=0.046) but not with HR (r=- 0.16, p=0.59) and occurred irrespective of AMS at high altitude. CONCLUSION: High altitude exposure leads to decreased CS. Changes occur independent of AMS. This finding is of clinical importance to trekkers and mountaineers exposed to high altitude as visual processing in particular under mesopic conditions at dusk and dawn is altered. Furthermore, it provides novel insight into hypoxia related changes in CS function.

Implantation, removal and replacement of subretinal electronic implants for restoration of vision in patients with retinitis pigmentosa.

PURPOSE OF REVIEW: The purpose of this review is to provide an update on the efforts to restore vision through subretinal implants in patients with degenerative retinal diseases. In addition to the current technique and its latest improvements, it will focus on the surgical technique of implantation as well as explantation and reimplantation. RECENT FINDINGS: The durability of the current subretinal implant RETINA IMPLANT Alpha AMS has increased substantially compared with the predecessor model RETINA IMPLANT Alpha IMS. According to validated examinations in the laboratory, a median lifetime of 4.7 years will be reached in clinical use; in similar examinations, the previous model has reached only 8 months. Visual function has slightly increased. The surgical technique for subretinal implants is complex and demanding for ophthalmic surgeons, as it is multifaceted and combines novel surgical steps in areas, which are not commonly entered such as the suprachoroidal and the subretinal space. The surgical approach for implantation has matured considerably and has led to successful implantation in 64 patient cases. Surgical challenges are now mainly encountered with the exact subfoveal positioning of the device. The explantation procedure is relatively straight-forward because the implant can be withdrawn in a reverse direction along the already existent subretinal path. Reimplantations, however, are more challenging because some degree of scar tissue may exist along the path of the chip and around the scleral trapdoor. Nevertheless, reimplantations have now been carried out successfully in four patients. SUMMARY: The new RETINA IMPLANT Alpha AMS shows significantly improved durability compared with the predecessor model RETINA IMPLANT Alpha IMS. The subretinal implant offers excellent visual results but requires experienced surgeons. Explantation of devices is straight-forward, and reimplantations are challenging but have been successful in four patients.

Impact of personality characteristics on patient satisfaction after multifocal intraocular lens implantation: results from the "happy patient study".

PURPOSE: This prospective multicenter study assessed personality characteristics that may influence patient satisfaction after implantation of multifocal intraocular lenses (MIOLs). METHODS: One hundred eighty-three patients who underwent bilateral implantation of different MIOLs were enrolled. Uncorrected and corrected distance visual acuity for distance and near (ie, UDVA, UNVA, CDVA, and CNVA) were assessed preoperatively and 3 and 6 months after implantation. Before surgery, personality characteristics were evaluated using a questionnaire based on the NEO Personality Inventory Test and the Compulsiveness Inventory Test. At the 3- and 6-month postoperative visits, patients answered a questionnaire concerning overall satisfaction, need for spectacles at different distances, visual function at different distances and lighting conditions, photic phenomena, and ease of performing daily activities. RESULTS: Three months after surgery (n = 163), mean CDVA was 0.03 ± 0.09 logMAR, UDVA was 0.05 ± 0.09 logMAR, and UNVA was 0.04 ± 0.11 logMAR. At the 6-month visit (n = 131), mean CDVA was 0.02 ± 0.07 logMAR, UDVA was 0.06 ± 0.09 logMAR, and UNVA was 0.05 ± 0.01 logMAR. Most patients (82.2%) would opt for an MIOL again, 3.7% would not, and 14.1% were uncertain. Overall satisfaction with the procedure was correlated to low astigmatism, good visual function, low spectacle dependence, and less halos or glare. The personality characteristics of compulsive checking, orderliness, competence, and dutifulness were statistically significantly correlated to subjective disturbance by glare and halos. CONCLUSIONS: Postoperative patient satisfaction after MIOL implantation is correlated to visual performance, spectacle independence, and less photic phenomena. Personality characteristics have an impact on subjective disturbance by photic phenomena and thus are important for patient satisfaction.

Exposure to high altitude alters tear film osmolarity and breakup time.

AIMS: High altitude provides environmental conditions with dry air and cold temperatures that may facilitate the development of dry eye symptoms. This study investigated, for the first time, the quality of the tear film during high altitude exposure in healthy subjects. This study is related to the Tübingen High Altitude Ophthalmology (THAO) study. METHODS: Tear film osmolarity (TFO), tear film breakup time (TBUT), and Schirmer I and II were used to assess tear film properties under standardized conditions in 14 healthy subjects on day 1, 2, and 4 during exposure to high altitude at the Capanna Margherita (CM; 4559 m, Italy) compared to baseline measurements in Tübingen (341 m, Germany) before (BL1) and after (BL2) exposure. RESULTS: Upon arrival at CM, a significant increase in intra-individual TFO (309.1 ± 19.3, 332.2 ± 24.1, 335.5 ± 28.7, 329.7 ± 19.0, and 308.5 ± 15.3 mOsms/L at BL1, day 1, 2, 4, and BL2, respectively) and a significant decrease of TBUT (11.2 ± 5.2, 7.3 ± 5.2, 7.2 ± 11.6, 4.5 ± 2.3, and 8.7 ± 4.6 seconds at BL1, day 1, 2, 4, and BL2, respectively) were found. Schirmer test changes at high altitude remained statistically nonsignificant compared to baseline. Comparisons of parameters between BL1 and BL2 showed no statistically significant differences and recordings of right and left eyes for TBUT and Schirmer did not differ significantly on any day measured. CONCLUSION: High altitude exposure leads to an altered tear film resulting in an increased TFO and a reduced TBUT. These changes were fully reversible after descent. This is of clinical importance to populations living in high altitude areas and to trekkers and mountaineers exposed to high altitude due to their ever-increasing number.