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BackgroundTo evaluate the clinical outcomes of subfoveal haemorrhages secondary to neovascular age-related macular degeneration (AMD), which were treated with intravitreal recombinant tissue plasminogen activator (rTPA)/gas and anti-vascular endothelial growth factor (anti-VEGF) drug or with an intravitreal anti-VEGF monotherapy.MethodsThis is a retrospective pilot study. Patients who received intravitreal rTPA/gas and anti-VEGF injections (n=20, bevacizumab or ranibizumab) were included in group A. Patients who refused prone positioning after rTPA/gas injections and were treated with an anti-VEGF monotherapy (bevacizumab) alone were included into group B (n=10). Changes in baseline visual acuity (VA, Snellen), central retinal thickness (CRT) and haemorrhage size were analysed.ResultsMean baseline VA was 0.15±0.2 and 0.25±0.17 in groups A and B, respectively. At month 4, significant improvement in mean VA was observed in group A (mean difference: +0.1±0.14; P=0.003), and a stabilization in group B (mean difference: +0.008±0.2; P=0.94). CRT decreased significantly by 70 μm in group A (P=0.001) and by 84 μm in group B (P=0.03). The mean size of subfoveal haemorrhage in groups A and B was 20.2 mm2 and 19.1 mm2 at baseline and 0.0 mm2 and 2.0 mm2 at month 4, respectively. The anti-VEGF treatmentrate was 1.6 in group A and 3.0 in group B.ConclusionIn patients with extensive subfoveal haemorrhage secondary to neovascular AMD, the combination therapy of rTPA/pneumatic displacement and anti-VEGF results in mean improvement of VA and stabilization of morphological parameters. If rTPA and pneumatic displacement combination is contraindicated, an anti-VEGF monotherapy may be performed to prevent further visual loss.Eye (2009) 23, 1404–1410; doi:10.1038/eye.2008.267; published online 29 August 2008 [ABSTRACT FROM AUTHOR]

Retinopathy of prematurity (ROP) remains a major cause of childhood blindness. Its pooled prevalence worldwide is 31.9%, and that of severe ROP is 7.5% among prematurely born babies. Investigating risk factors is essential for improving early detection and treatment outcomes. Purpose : To determine the frequency and stages of ROP cases and evaluate the treatment methods for premature infants at the Medical University of Vienna. Methods : In this retrospective study, 352 children who underwent ROP screening between 2018 and 2021 with a gestational age (GA) ≤ 32 weeks and/or a birth weight (BW) ≤ 1500 g were included. Results : ROP was found in 144 (40.9%) of the 352 screened premature infants, with 17 (4.8%) requiring treatment. Significant risk factors included GA and BW, while sex and pregnancy type were not significant. The mean GA was 27.7 ± 2.5 weeks, and the mean BW was 989.1 ± 359.7 g. Infants with ROP had a lower GA (25.9 ± 1.7 weeks) and BW (778.6 ± 262.4 g) than those without ROP (28.9 ± 2.2 weeks; 1134.9 ± 345.9 g). GA and BW were significantly lower in infants developing ROP ( p < 0.001). Stage 2 ROP was the most common severity in 74 children (51.4%). Laser therapy was the most common first-line treatment, used in 11 infants (64.7%), followed by anti-VEGF therapy, used in 6 infants (35.3%). Children were treated within 1.0 ± 0.6 days on average. Of the 17 infants treated, 14 (82.4%) showed initial regression. Three infants (17.6%) required re-treatment: two with initial anti-VEGF therapy and one after laser therapy. Conclusions : The findings provide insights into ROP's prevalence and treatment preferences at a university tertiary care center. GA and BW were confirmed to be significant predictors, aiding in early detection and informing treatment decisions. These insights will enable comparisons with similar studies and contribute to improved patient care.

Objectives: This study aims to provide a comprehensive analysis of ocular biometric parameters in pediatric patients with cataracts to optimize surgical outcomes. By evaluating various biometric data, we seek to enhance the decision-making process for intraocular lens (IOL) placement, particularly with advanced technologies like femtosecond lasers. Methods: This retrospective comparative study included pediatric patients with cataracts who underwent ocular biometric measurements and cataract extraction with anterior vitrectomy at the Medical University of Vienna between January 2019 and December 2021. Parameters measured included corneal diameter (CD), axial length (AL), corneal thickness (CT) and flat and steep keratometry (Kf and Ks). The study explored the correlations between these parameters and IOL placement. Results: A total of 136 eyes from 68 pediatric patients were included in the study. Significant positive correlations were found between corneal diameter, age and AL. The mean CD was 11.4 mm, mean AL was 19.5 mm, CT was 581.2 ± 51.8 µm, Kf was 7.76 ± 0.55 mm and Ks 7.41 ± 0.59 mm, respectively. Older pediatric patients with larger corneal diameters and longer ALs were more likely to receive in-the-bag IOL implantation. Conversely, younger patients often required alternative IOL placements or remained aphakic. Our data indicated that over 95% of the study population and all patients aged one year and older had a corneal diameter of 10 mm or larger. Conclusions: Detailed ocular biometric analysis is crucial for optimizing both surgical outcomes and postoperative care in pediatric cataract patients. The positive correlations between CD, age and AL underline the importance of individualized surgical planning tailored to each patient's unique anatomical features. Additionally, our findings suggest that the use of a femtosecond laser is both feasible and safe for pediatric patients aged one year and older, potentially offering enhanced surgical precision and improved outcomes.

Purpose: To evaluate the postoperative outcome of strabismus surgery performed in children aged 1-6 years by investigating the change of the preoperative angle of deviation (AOD), elevation in adduction, best-corrected visual acuity (BCVA) and refractive error., Methods: Retrospective chart review of 62 children who received strabismus surgery between January 2018 and December 2021 at the Department of Ophthalmology and Optometry of the Medical University of Vienna. Age, sex, type of strabismus, AOD, BCVA, refractive error and visual acuity were evaluated with respect to the postoperative outcome., Results: Mean follow-up was 13.55 ± 11.38 months with a mean age of 3.94 ± 1.97 years (range: 1.0-6.0) at time of surgery. 74.19% of patients (n = 46) had isolated or combined esotropia, 12.90% (n = 8) had isolated or combined exotropia and 12.90% (n = 8) had isolated strabismus sursoadductorius. Mean preoperative AOD of 15.69 ± 16.91°/15.02 ± 14.88° (near/distance) decreased to 4.00 ± 9.18°/4.83 ± 7.32° (near/distance) at final follow-up (p < 0.001). BCVA improved from 0.26 ± 0.26/0.25 ± 0.23 (left/right) to 0.21 ± 0.25/0.20 ± 0.23 (left/right) (p = 0.038). There was no significant change regarding refractive error (p = 0.109) or elevation in adduction (p = 0.212). Success rate which was defined as a residual AOD of less than 10° was 74.19% (n = 46). In 3.23% (n = 2) retreatment was necessary., Conclusion: Strabismus surgery in infants was shown to have a satisfactory outcome with a low retreatment rate. Surgical success rate was not linked to age, sex, type of strabismus or the preoperative parameters AOD, refractive error and visual acuity in this study., (© 2024. The Author(s).)

Background: To evaluate the functional development and, retinal and optic disc morphology using OCT in patients with septo-optic dysplasia (SOD)., Methods: This retrospective case series included patients diagnosed with SOD between 2007 and 2020. Ophthalmologic assessment included visual acuity (VA) and funduscopy at the initial and last presentation. Retinal imaging included OCT of the macula analyzing the retinal morphology, central retinal thickness volume (CRT) and ganglion cell layer (GCL). Also, scans of the optic nerve head were taken to evaluate the retinal nerve fiber layer (RNFL) and global value., Results: 38 eyes of 19 children with a mean age 6.3 ± 5.3 years were included. 31.6% showed all 3 characteristics of SOD, whereof ONH, midline defects and endocrine dysfunctions were found in 94.7%, 89.5% and 47.4% respectively. The mean VA was 0.70 ± 0.66logMar in the right eye (RE) and 0.40 ± 0.55logMar in the left eye (LE) at the initial presentation. No change of vision (RE: 0.69 ± 0.71logMar; LE: 0.31 ± 0.57logMar) was found after a follow-up period of 6.3 ± 4.5years. Funduscopy showed an ONH in 79% (n = 30/38), tortuous retinal vessels in 36.8% (n = 14/38) and a double-ring sign in 15.8% (n = 6/38). Retinal imaging showed variable morphology. 6 eyes of 4 patients showed temporal retinal thinning with corresponding GCL attenuation. The optic nerve head appearance varied between no changes, sectoral and hemispherical reduction., Conclusions: Patients suffering from SOD show diverse expression of retinal changes such as retinal, GCL and RNFL thinning in OCT. Furthermore, visual function remained stable during follow-up examinations, indicating no further alteration due to underlying pathology.

Purpose: Strabismus sursoadductorius is the most common strabismus of the oblique eye muscles. The changes in squint angle, elevation in adduction, abnormal head posture and binocular vision were collected to get an overview of the results at the largest clinic in Austria., Methods: This study is a retrospective study. The orthoptic and ophthalmologic parameters of 102 patients who were treated in the Department of Ophthalmology and Optometry between January 2017 und September 2020 were collected and statistically analysed., Results: The mean age was 12.2 ± 14.8 years. The squint angle changed from 18.3 ± 6.3 presurgical to 13.4 ± 9.7 (p = 0.09) with Hirschberg measurement. The squint angle changed from 12.5 ± 7.7 to 6.7 ± 4.9 (p < 0.01) at distance and from 12.5 ± 7.6 to 6.1 ± 4.5 (p < 0.01) at near with the prism covertest. Elevation in adduction showed a highly significant change (p < 0.01) from 15 ± 5.6 to 1.4 ± 2.8 degrees on average postsurgical. In binocular vision at distance there was also a highly significant (p < 0.01) change with 21.6% to postsurgical 37.3% positive tests. At near, the positives also increased significantly (p = 0.01) from 32.4% to 39.2%. There was no significant change in stereo vision (p = 1.0). Presurgical 52.9% of the sample had an abnormal head posture, postsurgical it was 38.2%, this change is also highly significant (p < 0.01)., Conclusion: The change was significant to highly significant for most parameters (i.e., squint angle, elevation in adduction, and abnormal head posture).

Purpose: To assess the characteristics and long-term outcomes of adult patients with dysthyroid optic neuropathy (DON) who underwent orbital decompression surgery and/or received intravenous (IV) methylprednisolone., Methods: Retrospective chart review of 98 eyes of 49 patients who were diagnosed and treated with bilateral DON between 2007 and 2018 at the Department of Ophthalmology and Optometry and Oral and Maxillofacial Surgery of the Medical University of Vienna., Results: The mean follow-up period was 4.1 ± 2.7 years. The most common presenting symptoms were eyelid and periorbital swelling (45%) representing active inflammation. Upgaze restriction was the most common clinical finding (73%). At time of diagnosis, the mean clinical activity score was 4 ± 1/4 ± 1 (right/left eye, respectively). Sixty-three percent (31/49) of the patients were treated both with IV methylprednisolone and underwent orbital decompression surgery, 22% (11/49) were treated with IV methylprednisolone alone and 14% (7/49) underwent surgical decompression only. Seventy-one percent (30/42) of the patients underwent 3-wall decompression. The mean reduction of proptosis in patients treated with both IV methylprednisolone and orbital decompression surgery was 4/5 mm. Mean of reduction in proptosis in patients receiving IV methylprednisolone only was 1/0 mm and in patients with surgical decompression only was 5/5 mm. Mean VA was 0.1 ± 0.5/0.1 ± 0.5 logMAR at baseline and 0.05 ± 0.7/0.05 ± 0.7 at final follow-up. In 92% (45/49), VA was preserved or improved at final follow-up., Conclusions: The majority of patients with DON were treated both with IV corticosteroids and 3-wall decompression surgery. Vision could be successfully preserved in most cases and reduction of proptosis was achieved, especially after orbital decompression surgery., (© 2022. The Author(s).)

Purpose: To determine microvascular changes in patients with genetically proven Marfan syndrome., Methods: In a cross-sectional study, 32 eyes of 16 patients with genetically proven Marfan syndrome were evaluated using swept-source optical coherence tomography angiography (SS-OCTA). Patients were analyzed regarding lens status and systemic vascular disease. The foveal avascular zone (FAZ) and vessel density (VD) of the superficial and deep vascular plexus and central retinal thickness (CRT) were evaluated on SS-OCTA., Results: 44/56% patients presented without/with subluxation of the lens. 69% of patients had presence of mitral valve insufficiency, aortic dilatation or aneurysm of the aortic root. In patients with Marfan syndrome the mean area of the FAZ was 0.2 ± 0.1 mm and the average VD of the superficial/deep vascular plexus was 36 ± 5%/22 ± 7%. In patients with subluxation of the lens FAZ area and perimeter were larger when compared to patients without subluxation of the lens (0.18 ± 0.08/0.28 ± 0.10 mm and 1.7 ± 0.4/2.3 ± 0.8; p  = 0.02). VD of the superficial vascular plexus was reduced in patients with subluxation of the lens (on average 39 ± 3/33 ± 8; p  = 0.01) together with an increased CRT in the inner segments of the ETDRS grid when compared to patients without subluxation of the lens. In patients with systemic vascular disease a larger FAZ area (0.19 ± 0.06/0.25 ± 0.1 mm; p  = 0.04) and reduced VD of the superficial vascular plexus in the central ETDRS grid (28 ± 7/21 ± 6; p  = 0.02) was observed in comparison to patients without systemic vascular changes., Conclusions: In patients with Marfan syndrome SS-OCTA imaging revealed microvascular differences in patients with lens subluxation and/or systemic vascular disease.

Aim: To evaluate the ophthalmic and anesthesiologic management of cataract surgery in children with Lowe syndrome receiving lens removal, the development and management of secondary glaucoma., Methods: This retrospective case series included 12 eyes of 6 children with genetically verified Lowe syndrome receiving cataract removal. Information regarding the type and duration of surgery and total anesthesia time were recorded. Additionally, intra- and postoperative complications were noted as well as clinical examinations such as visual acuity and funduscopy., Results: All children received simultaneous bilateral cataract surgery at the mean age of 8.98±3.58wk. Lensectomy combined with posterior capsulotomy and anterior vitrectomy was performed in all children. The mean time for cataract surgery per eye was 35.83±8.86min, whereas the total time of surgery was 153.33±22.11min. The mean extubation time and duration at recovery room was 42.33±22.60min and 130.00±64.37min, respectively. During surgery, a decrease of oxygen saturation below 93% was found in only one child. During the postoperative follow-up, nystagmus (6 children) and strabismus (5 children) was commonly found in contrast to no case of visual axis opacification. Secondary glaucoma developed in five eyes of three children, which was treated with topical eye drops in only one child. A trabeculectomy was performed in both eyes of one child, whereas removal of syechia and an iridectomy in one eye of one child., Conclusion: Bilateral simultaneous cataract surgery under general anesthesia is a safe surgical procedure in Lowe syndrome children. The glaucoma screening with intraocular pressure measurements is crucial in the postoperative management of Lowe syndrome patients to avoid additional visual impairment., (International Journal of Ophthalmology Press.)

Objective: To evaluate changes in intraocular pressure after congenital cataract surgery in a real-world setting., Methods: This retrospective case series included all children aged 0-2 years undergoing lens extraction due to congenital cataract. Development of an elevated intraocular pressure was divided into three groups: secG, suspG and OHT. Further, risk factors for IOP changes, the therapeutic approach and functional outcome were assessed during follow-up., Results: One hundred and sixty-one eyes of 110 patients aged 0-2 years were included, whereof 29 eyes of 17 children developed secondary glaucoma (secG; 11 eyes/8 patients), glaucoma suspect (suspG; three eyes/three patients) or ocular hypertension (OHT; 15 eyes/10 patients). No difference in surgrical procedure ( p  = 0.62) was found, but age at cataract surgery differed significantly ( p  = 0.048), with the secG group (1.74 ± 1.01 months) being the youngest (suspG: 3.93 ± 1.80 months; OHT group: 5.91 ± 5.36 months).Secondary surgical intervention was significantly higher in the secG (4.64 ± 3.41) followed by the suspG (2.00 ± 2.65) and OHT groups (0.40 ± 0.74; p  < 0.001). Postoperative complications including nystagmus ( p  = 0.81), strabismus ( p  = 0.98) and amblyopia ( p  = 0.73) showed no difference, in contrast to visual axis obscuration which was more common in the secG group ( p  = 0.036)., Conclusion: Initial lensectomy and anterior vitrectomy procedure together with or without IOL implantation seems to have no influence for the development of IOP changes after pediatric cataract surgery. However, children who developed secondary glaucoma had cataract surgery significantly earlier, within the first 2-3 months of life. Glaucoma surgery was required to achieve final IOP control in most eyes. The development of secondary glaucoma was also associated with a significant increase in surgical re-treatments.

Objective: The aim of this study was to evaluate and compare the underlying pathologies, demographic and retinal detachment characteristics in pediatric and early adulthood retinal detachment., Methods: Patients with rhegmatogenous, serous, or tractional retinal detachment aged 0-26 years were retrospectively reviewed. The preschool group (n = 4) comprised children aged 0-6 years, the pediatric group (n = 19) comprised children aged 7-16 years, and the early adulthood group (n = 13) aged 17-26 years. Demographic information and retinal detachment characteristics, type of surgery, and intraocular tamponade were analyzed. Postoperatively, the functional outcome, anatomic success, and ocular adverse events were evaluated. Due to the low patient number in the preschool group, statistical analysis was performed for pediatric group and early adulthood group only., Results: All causes of retinal detachment were present in the pediatric group, but only rhegmatogenous retinal detachment in the early adulthood group. In both groups, the main type of surgical intervention was pars plana vitrectomy (pediatric group: 52%, early adulthood group: 38%; p = 0.36). The type of intraocular tamponade varied statistically significantly between the groups (p = 0.014). Silicone oil was the main intraocular tamponade in the pediatric group (48%), whereas no tamponade (54%) followed by gas tamponade (46%) in the early adulthood group. Final attachment rate was similar in both groups (pediatric group: 89%, early adulthood group: 100%; p = 0.35). Re-detachment occurred significantly sooner in the pediatric group (1.3 ± 0.3 months) than in the early adulthood group (4.3 ± 1.4 months; p = 0.03)., Conclusion: In pediatric and early adulthood retinal detachment, pars plana vitrectomy appeared as a successful surgical intervention. Re-attachment rate and re-treatment were similar in both groups with a better functional outcome observed in cases of retinal detachment in early adulthood and poorer results in young children.

Purpose: To determine microvascular changes in children with a history of retinopathy of prematurity (ROP) and in a control group of full-term children., Methods: In a cross-sectional study, 30 eyes of 15 children aged 6-8 years with a history of ROP were evaluated with swept-source optical coherence tomography angiography (SS-OCTA). Twenty-eight eyes of 22 age-matched full-term children served as a healthy control group. The foveal avascular zone (FAZ), vessel density (VD) and choroidal vascular flow area (VFA) were evaluated on OCTA and correlated with central retinal thickness (CRT), visual acuity (VA), birth weight (BW), gestational age (GA) and ROP stages., Results: Twenty-two eyes of 14 children with a history of ROP (stage 1-3) and 25 eyes of 19 full-term children were available for evaluation. In the ROP group, the gestational age was 27 ± 2 weeks and birth weight was 781 ± 164 g. In the ROP group, CRT was higher in the central ETDRS segment (mean difference [95% CI]: 32.8 µm [18.7; 47.0], p = 0.0002) compared to the controls. Smaller mean FAZ area (-0.12 [-0.19; -0.04], p = 0.004) and perimeter (-662 [-1228; -96], p = 0.03) was found in comparison to the control group. An oval shape of the FAZ was observed among patients with a history of ROP. The mean central VD of the superficial plexus was 28 ± 8/23 ± 8% and of the deep plexus 7 ± 7/3 ± 5% (ROP group/control group; p > 0.05). No statistically significant difference was found regarding the choroidal VFA. Only weak correlation of FAZ and VD with function was observed., Conclusions: Swept-source optical coherence tomography angiography imaging revealed significant microvascular anomalies in children with a history of ROP indicating disturbance of early morphological development of the central retina., (© 2020 The Authors. Acta Ophthalmologica published by John Wiley & Sons Ltd on behalf of Acta Ophthalmologica Scandinavica Foundation.)

A severe episode of high and abnormal mortality was observed in the population of Cyprinus carpio of Lake Caldaro (South Tyrol, Italy) in summer 2016. The diagnostic investigation carried out led to the identification of Koi Herpesvirus (KHV) as the etiologic agent. Following this disease outbreak and its socio-economic consequences, the local authorities, in agreement with the local Fishing Association, decided to implement a surveillance program for the achievement of KHV-free health status (Category I) in the Province, in accordance to the Implementing Decision (EU) 2015/1554. The selected area was a defined geographical compartment (the Monticolo lakes compartment, South Tyrol, Italy), which is located near Lake Caldaro, where the Koi Herpesvirus disease (KHVD) outbreak had occurred. This area is of particular interest because it supplies other water bodies with juvenile C. carpio individuals; with the achievement of a KHV-free health status, South Tyrol could possibly become independent in the breeding of this fish species. Suitable samples were collected and processed during a two-year period in order to detect the presence/absence of KHV. The same samples were tested for other viruses that can affect carp, namely spring viraemia of carp (SVCV) and carp edema virus (CEV). According to the results, the authors conclude that the Monticolo lakes area should be classified as KHV-free, as no sample has tested positively for the presence of this specific virus (KHV)., (Copyright © 2020 The Authors. Published by Elsevier B.V. All rights reserved.)

Purpose: To evaluate the surgical approach, outcome, and safety of bilateral simultaneous cataract surgery (BS-Cat) compared with unilateral cataract surgery (US-Cat) and bilateral 2-timed cataract surgery (BT-Cat) in children., Design: Retrospective, interventional case series., Methods: Setting: Department of Ophthalmology, Medical University, Vienna, Austria., Participants: Children aged 0-18 years who received cataract extraction owing to a unilateral or bilateral cataract between January 2003 and December 2018 were included. Main Outcomes and Measures: Information regarding the type and duration of surgery and total anesthesia time were recorded. Additionally, intraoperative and postoperative complications including retreatments were evaluated., Results: A total of 220 eyes of 147 patients were included in this analysis (US-Cat: n = 74 patients; BS-Cat: n = 63 patients; BT-Cat: n = 10 patients). The mean age at surgery was 15.94 ± 27.10 months in the US-Cat group, 33.47 ± 58.20 months in the BS-Cat group, and 41.91 ± 55.09 months in the BT-Cat group. The main surgical approach was lensectomy combined with anterior vitrectomy ± IOL implantation (US-Cat: 98.65%; BS-Cat: 95.24%; BT-Cat: 100%; P = .08). Initial intraocular lens implantation was not commonly performed in all groups (US-Cat: 27.03%; BS-Cat: 23.02%; BT-Cat: 50.00%). The mean anesthesia time was shortest in the US-Cat (91.62 ± 26.12 min), followed by the BS-Cat (123.81 ± 30.11 min) and BT-Cat groups (186.00 ± 42.34 min; P < .001), contrary to the duration of surgery (US-Cat: 37.56 ± 15.69 min; BS-Cat: 32.33 ± 17.31 min; BT-Cat: 37.50 ± 18.67; P = .087). An accidental oxygen decrease below 93% was rare in all groups (US-Cat: 6.76%; BS-Cat: 17.46%; BT-Cat: 10.00%). Intraoperative surgical complications occurred rarely (P = .95) and involved mainly the iris. The number of postoperative complications (P = .17) and interventions (P = .10) was similar in all groups. Visual axis obscuration (US-Cat: 28.38%; BS-Cat: 23.81%; BT-Cat: 20.00%; P = .67) and glaucoma (US-Cat: 6.76%; BS-Cat: 15.87%; BT-Cat: 15.00%; P = .20) showed no difference between the groups. However, nystagmus was more pronounced in the BS-Cat group (US-Cat: 12.16%; BS-Cat: 49.21%; BT-Cat: 20.00%; P < .001), whereas strabismus was more pronounced in the US-Cat group (US-Cat: 68.92%; BS-Cat: 33.33%; BT-Cat: 40.00%; P < .001)., Conclusion: Simultaneous removal of bilateral cataract in children showed no statistically significant differences regarding intraoperative and postoperative complications when compared to unilateral and 2-timed bilateral cataract surgery. Anesthesia time was longer in simultaneous bilateral than in unilateral cataract surgery, but only by the surgery time of the second eye. However, prolonged anesthesia time was not accompanied by a decrease of oxygen saturation., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Importance: To determine visual and surgical results in children with Marfan syndrome., Background: Marfan syndrome involves ocular complications which can lead to visual disturbance and amblyopia. Data about the visual and surgical results in children with Marfan syndrome is vital for the clinical management of these patients., Design: Retrospective data analysis., Participants: Eighty-two eyes of 41 patients with a genetically proved diagnosis of Marfan syndrome., Methods: Medical records of patients with Marfan syndrome were reviewed between 2007 and 2017. Ocular complications, visual acuity (VA) of patients with/without lensectomy and surgical method were evaluated., Main Outcome Measures: VA outcomes of patients with Marfan syndrome with/without surgical repair of ectopia lentis., Results: In 27 (66%) of the 41 patients a bilateral subluxation of the lens was visible and 14 (52%) patients received lensectomy. The mean age at initial presentation was 12.3 ± 9 years and mean follow-up was 3 years (range 1-7). VA varied from 1.2 to -0.1 logMAR at first examination. At initial presentation, mean VA was 0.1 ± 0.7 logMAR in patients with a normal lens status (n = 14) and 0.3 ± 0.5 logMAR in patients with subluxation of the lens (n = 27) (P < .01). VA improved from 0.2 ± 0.5 logMAR to 0.1 ± 0.5 logMAR (P = .06) in children with subluxation of the lens who did not need a lensectomy (n = 12) and from 0.5 ± 0.5 logMAR to 0.2 ± 0.5 logMAR (P = .02) in patients who were referred to lensectomy with/without secondary lens implantation., Conclusions and Relevance: In Marfan syndrome subluxation of the lens was found in the majority of children. Improvement of VA resulted in children with subluxation of the lens and following lensectomy., (© 2019 The Authors. Medical University of Vienna. Clinical & Experimental Ophthalmology published by John Wiley & Sons Australia, Ltd on behalf of Royal Australian and New Zealand College of Ophthalmologists.)

Purpose: To evaluate the effect of patients' clinical information on experts' diagnoses of retinopathy of prematurity (ROP) and decisions to treat., Methods: Seven experts assessed wide-field fundus photographs of eyes of 52 premature infants of ≤30 weeks' gestational age or ≤1,500 g birthweight (BW) for ROP diagnosis (stage, plus disease, and aggressive posterior ROP) and the necessity for treatment for 2 days. On Day 1, they were masked to all patient data. On Day 2, they were given information on gestational age and BW., Results: A significant shift in the experts' ratings toward a less aggressive ROP grading stage (P = 0.006) and less frequent decision for intervention (P = 0.021) was observed after receipt of patients' clinical information. This was truer for heavier/less premature infants (gestational age ≥ 28 0/7 weeks or BW ≥ 900 g) than those with very low BWs/high prematurity (gestational age < 24 0/7 weeks or BW < 600 g) (ROP stage P = 0.009 vs. P = 0.399, treatment decision P = 0.022 vs. P = 0.648)., Conclusion: These results suggest knowledge of patients' clinical information influences the grading of ROP disease and decision for treatment. Retinopathy of prematurity staging seemed to be set at a lower level and the decision for treatment at a higher threshold for heavier/less premature babies. Our findings may have implications for further refinements in ROP assessment.

Small ruminant lentivirus infections in goats affect both production and animal welfare. This represents a threat to the qualitative and quantitative growth of goat farming, recently observed in mountainous regions such as the Autonomous Province of Bolzano - South Tyrol (Italy). To monitor and eradicate the caprine arthritis encephalitis virus in this goat population, a compulsory eradication campaign was launched, based on a strict census of small ruminants and yearly serological testing of all animals, followed by the consequent culling of seropositive individuals. The campaign succeeded in completely eliminating cases of clinical disease in goats, while drastically reducing the seroprevalence at the herd as well as individual animal level. The serological outcome of the introduced control measures was determined using commercially available ELISA kits, demonstrating their suitability for use in this type of campaign, aimed at reducing seroprevalence as well as clinical manifestations of these infections. However, this clear success is diminished by the failure to achieve a complete eradication of these viruses. The reasons leading to the observed tailing phenomenon and the occurrence of new infections in already sanitised flocks are discussed and implementation of further measures are proposed., Competing Interests: Competing interests: None declared., (© British Veterinary Association (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Weill-Marchesani syndrome is a rare disorder of the connective tissue. Functional variants in ADAMTS10 are associated with Weill-Marchesani syndrome-1. We identified a homozygous missense mutation, c.41T>A, of the ADAMTS10 gene in a 19-year-old female with typical symptoms of WMS1: proportionate short stature, brachydactyly, joint stiffness, and microspherophakia. The ADAMTS10 missense mutation was analysed in silico, with conflicting results as to its effects on protein function, but it was predicted to affect the leader sequence. Molecular characterisation in HEK293 Ebna cells revealed an intracellular mis-targeting of the ADAMTS10 protein with a reduced concentration of the polypeptide in the endoplasmic reticulum. A large reduction in glycosylation of the cytoplasmic fraction of the mutant ADAMTS10 protein versus the wild-type protein and a lack of secretion of the mutant protein are also evident in our results.In conclusion, we identified a novel missense mutation of the ADAMTS10 gene and confirmed the functional consequences suggested by the in silico analysis by conducting molecular studies.