Postoperative outcome and influencing factors of strabismus surgery in infants aged 1-6 years.

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.

Pre- and postsurgical measurements in patients with strabismus sursoadductorius a retrospective study.

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).

Patients with septo-optic dysplasia: General ophthalmologic assessment and retinal imaging.

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.

Management of cataract surgery in Lowe syndrome.

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.

Management of patients with dysthyroid optic neuropathy treated with intravenous corticosteroids and/or orbital decompression surgery.

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.

Microvascular Retinal Changes in Patients with Marfan Syndrome.

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.

Clinical characteristics and treatment of secondary glaucoma, glaucoma suspects and ocular hypertension after congenital cataract surgery.

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.

Clinical characteristics and surgical outcome of pediatric and early adulthood retinal detachment.

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.

Retinal vessel architecture in retinopathy of prematurity and healthy controls using swept-source optical coherence tomography angiography.

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.

Simultaneous Bilateral Pediatric and Juvenile Cataract Surgery Under General Anesthesia: Outcomes and Safety.

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.

Visual outcome and surgical results in children with Marfan syndrome.

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.

EFFECT OF PATIENTS' CLINICAL INFORMATION ON THE DIAGNOSIS OF AND DECISION TO TREAT RETINOPATHY OF PREMATURITY.

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.

Posterior capsule opacification and neodymium:YAG laser capsulotomy rates with 2 microincision intraocular lenses: Four-year results.

PURPOSE: To compare the development of posterior capsule opacification (PCO) and neodymium:YAG (Nd:YAG) capsulotomy rates between 2 microincision intraocular lenses (IOLs) 4 years after surgery. SETTING: Department of Ophthalmology, Medical University of Vienna, Vienna, Austria. DESIGN: Prospective randomized clinical trial. METHODS: Patients randomly received a Y-60H 3-piece hydrophobic IOL (hydrophobic group) in 1 eye and an MI60 1-piece hydrophilic IOL (hydrophilic group) in the contralateral eye during simultaneous bilateral cataract surgery. Eyes were examined 1 week, 20 months, and 4 years postoperatively. Digital retroilluminated images of each eye were evaluated using Automated Quantification of After-Cataract software. The Nd:YAG laser capsulotomy rate was recorded. RESULTS: Sixty patients were enrolled. Objective PCO was significantly higher in the hydrophobic group than in the hydrophilic group before capsulotomy 2 years after cataract surgery (mean score 2.1 ± 1.8 (SD) versus 1.2 ± 1.4) (P =.031). At the 4-year follow-up, the hydrophobic group had a statistically significantly higher Nd:YAG rate than the hydrophilic group (77% versus 50%) (P =.012). CONCLUSION: Comparison of 2 microincision IOLs indicated a statistically significant difference in PCO and Nd:YAG capsulotomy rates, with very high Nd:YAG rates 4 years after surgery.

Inter-expert and intra-expert agreement on the diagnosis and treatment of retinopathy of prematurity.

PURPOSE: To evaluate inter-expert and intra-expert agreement on the diagnosis and treatment of retinopathy of prematurity (ROP). DESIGN: Prospective intra- and inter-rater reliability analysis. METHODS: In this multicenter study, 260 wide-field digital photographs of 52 patients were presented to 7 recognized ROP experts on 2 consecutive assessment days 8 weeks apart. Experts were asked to assess the patients for ROP stage, presence of plus disease, presence of aggressive posterior ROP, necessity for treatment, and suggested treatment. Agreement levels were measured with Fleiss' kappa and Cohen's kappa. RESULTS: Inter-expert agreement was fair for the ROP stage (κ = 0.24), plus disease (κ = 0.32), and aggressive posterior ROP (κ = 0.35); moderate for the necessity for treatment (κ = 0.41); and fair for the kind of treatment (κ = 0.38). Perfect inter-expert agreement was found in 9.6% of all patients for ROP stage 0-5, 45.1% for ≥ stage 2 ROP, 17.3% for plus disease, 57.7% for aggressive posterior ROP, and 25% for the necessity for treatment. Intra-expert agreement was higher than inter-expert agreement and was moderate for the ROP stage (κ = 0.56) and plus disease (κ = 0.51), moderate to substantial for aggressive posterior ROP (κ = 0.60), moderate for the necessity for treatment (κ = 0.47), and substantial for the kind of treatment (κ = 0.63). CONCLUSIONS: ROP diagnosis and treatment decisions differ between experts and by 1 expert made on different days, indicating that the grading process is subjective and there is an observer bias when diagnosing ROP. These results could influence current practice in ROP assessment and training, and prompt further refinement of international ROP guidelines.

Identification and molecular characterisation of a homozygous missense mutation in the ADAMTS10 gene in a patient with Weill-Marchesani syndrome.

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.

Twin-twin transfusion syndrome as a possible risk factor for the development of retinopathy of prematurity.

PURPOSE: The objective of this study was to evaluate the correlation between twin-twin transfusion syndrome (TTTS) and the development of retinopathy of prematurity (ROP) in premature infants. METHODS: Fifty-one infants who were less than 32 postmenstrual gestational weeks at birth or with a birth weight less than 1,501grams were included in this longitudinal observational study. The infants were matched by gestational age and birth weight, and divided into three groups: multiples with TTTS, multiples without TTTS, and singletons. The primary outcome variable was the incidence of ROP in infants affected by TTTS versus infants not affected by TTTS. Secondary outcome variables were multiple pregnancy, gestational age, and birth weight. RESULTS: Infants affected by TTTS showed a significantly higher incidence of ROP than infants not affected by TTTS (p < 0.01). TTTS donors and TTTS recipients were both at greater risk of developing ROP. ROP occurred in infants with TTTS whose gestational age at birth was significantly higher than that of infants with ROP who were not affected by TTTS (p = 0.01). Multiple pregnancy itself was not a risk factor for ROP disease. CONCLUSIONS: Infants affected by TTTS during pregnancy are at high risk of developing ROP, even if they were born at an older gestational age. Special awareness in ROP screening is necessary for these infants.

Posterior capsular opacification and Nd:YAG capsulotomy rates with the iMics Y-60H and Micro AY intra-ocular lenses: 3-year results of a randomized clinical trial.

PURPOSE: To compare the incidence and intensity of posterior capsular opacification (PCO) and neodymium:yttrium-aluminium-garnet (Nd:YAG) capsulotomy rates between two microincision intra-ocular lenses (IOLs) 3 years after surgery. METHODS: Sixty-five patients randomly received a Y-60H IOL (HOYA Surgical Optics, Singapore) in one eye and a Micro AY IOL (PhysIOL, Liège, Belgium) in the contralateral eye during same-day bilateral cataract surgery. Eyes were examined 1 week, 20 months and 3 years after surgery. The amount of PCO (score: 0-10) was assessed subjectively at the slit lamp and objectively using automated image analysis software (aqua). Additionally, the Nd:YAG capsulotomy rate was noted. RESULTS: Three years postoperatively, the objective PCO score of Y-60H IOLs was 1.9 ± 1.7 compared to PCO score of 1.7 ± 2.2 for the Micro AY IOLs (p = 0.66). Thirty-four percentage of the Y-60H eyes had undergone Nd:YAG capsulotomy, compared to 49% of the Micro AY eyes (p = 0.04). Significantly, more capsular folds were observed in the Y-60H IOL group (p = 0.001). There was no significant difference in best-corrected visual acuity, rhexis/IOL overlap and anterior capsule opacification 3 years after surgery. CONCLUSION: Both microincision IOLs showed high YAG rates and comparable PCO scores 3 years after surgery. In the light of this unsatisfying PCO performance, the advantage of the present microincision IOLs over conventional IOLs must be questioned.

Impact of low versus high fluidic settings on the efficacy and safety of phacoemulsification.

PURPOSE: To compare intraoperative efficiency and postoperative outcomes of cataract surgery with low and high fluidic settings. METHODS: In this prospective, randomized, single-blinded study, 114 eyes of 57 patients were operated with low fluidic settings for one eye (group I) and high fluidic settings for the other eye (group II). Efficiency was judged as metred surgery time, effective phacoemulsification time (EPT) and the amount of balanced salt solution used. Visual outcome and endothelial cell count were determined 1 week and 18 months postoperatively. RESULTS: The overall effective phacoemulsification energy was statistically significantly lower (p = 0.003) in group II than in group I. Conquest of the nuclei was achieved with about two-thirds of the energy needed in group I, with 6.59 ± 4.79 effective ultrasound energy compared with 3.99 ± 3.18 (p = 0.001). Overall, about 12% more solution was used in group II than in group I. Median visual acuity was 1.0 for both groups 18 months after surgery. The mean endothelial cell loss was 5.0% in eyes in group I compared with 6.3% in eyes in group II (p > 0.5). CONCLUSION: Switching from low fluidic settings with a conventional coaxial 20G phacoemulsification tip to higher fluidic settings with a microcoaxial phaco tip statistically significantly decreases EPT. As only marginally more solution was used with the higher aspiration flow, occlusion must be accomplished more often with high than with low fluidics. Aspiration of the quadrants was therefore more efficient with high fluidic settings. The enhanced pump speed did not result in more tissue damage.