Recommended data elements for health registries: a survey from a German funding initiative.

BACKGROUND: The selection of data elements is a decisive task within the development of a health registry. Having the right metadata is crucial for answering the particular research questions. Furthermore, the set of data elements determines the registries' readiness of interoperability and data reusability to a major extent. Six health registries shared and published their metadata within a German funding initiative. As one step in the direction of a common set of data elements, a selection of those metadata was evaluated with regard to their appropriateness for a broader usage. METHODS: Each registry was asked to contribute a 10%-selection of their data elements to an evaluation sample. The survey was set up with the online survey tool "LimeSurvey Cloud". The registries and an accompanying project participated in the survey with one vote for each project. The data elements were offered in content groups along with the question of whether the data element is appropriate for health registries on a broader scale. The question could be answered using a Likert scale with five options. Furthermore, "no answer" was allowed. The level of agreement was assessed using weighted Cohen's kappa and Kendall's coefficient of concordance. RESULTS: The evaluation sample consisted of 269 data elements. With a grade of "perhaps recommendable" or higher in the mean, 169 data elements were selected. These data elements belong preferably to groups' demography, education/occupation, medication, and nutrition. Half of the registries lost significance compared with their percentage of data elements in the evaluation sample, one remained stable. The level of concordance was adequate. CONCLUSIONS: The survey revealed a set of 169 data elements recommended for health registries. When developing a registry, this set could be valuable help in selecting the metadata appropriate to answer the registry's research questions. However, due to the high specificity of research questions, data elements beyond this set will be needed to cover the whole range of interests of a register. A broader discussion and subsequent surveys are needed to establish a common set of data elements on an international scale.

[Intermediate and Posterior Uveitis - Disease entities]. / Intermediäre und posteriore Uveitis ­ Teil 2.

Intermediate and posterior uveitis can have multiple infectious and noninfectious causes, and posterior uveitis in particular is clinically multifaceted. Some entities require prompt initiation of therapy to ensure visual prognosis. This article presents typical characteristics of intermediate and posterior uveitides and explains special features of their treatment.

[Intermediate and Posterior Uveitis - Classification, diagnostics, complications, and therapeutic algorithms]. / Intermediäre und posteriore Uveitis ­ Teil 1.

Intermediate and posterior uveitis describes a broad variety of different types of intraocular inflammation. Before starting treatment of intermediate or posterior uveitis, a differentiation between infectious or non-infectious uveitis must always be made. Pathognomonic symptoms do not exist, visual loss and vitreous floaters are the most common symptoms. The indication for therapy is influenced by the anatomical localization, the degree of inflammation, an association, complications and the activity of the inflammation. In addition to clinical ophthalmological standard examination, angiography and OCT are the most important investigations to classify and assess the course of inflammation. Macular edema is the most common complication of intermediate or posterior uveitis and should be treated at first onset, recurrence, or worsening. Oral, intravenous, or intravitreal corticosteroids are usually the primary therapy for intermediate or posterior uveitis. Systemic immunosuppression is indicated after steroid failure in non-infectious uveitis.

Structural Endpoints and Outcome Measures in Uveitis.

Most uveitis entities are rare diseases but, taken together, are responsible for 5-10% of worldwide visual impairment which largely affects persons of working age. As with many rare diseases, there is a lack of high-level evidence regarding its clinical management, partly due to a dearth of reliable and objective quantitative endpoints for clinical trials. This review provides an overview of available structural outcome measures for uveitis disease activity and damage in an anatomical order from the anterior to the posterior segment of the eye. While there is a multitude of available structural outcome measures, not all might qualify as endpoints for clinical uveitis trials, and thorough testing of applicability is warranted. Furthermore, a consensus on endpoint definition, standardization, and "core outcomes" is required. As stipulated by regulatory agencies, endpoints should be precisely defined, clinically important, internally consistent, reliable, responsive to treatment, and relevant for the respective subtype of uveitis. Out of all modalities used for assessment of the reviewed structural outcome measures, optical coherence tomography, color fundus photography, fundus autofluorescence, and fluorescein/indocyanine green angiography represent current "core modalities" for reliable and objective quantification of uveitis outcome measures, based on their practical availability and the evidence provided so far.

[The Treatment Exit Options for Uveitis (TOFU) Registry: Involving Patients in the Generation of Evidence]. / Das Treatment Exit Options for Uveitis (TOFU) Register: Einbindung von Patienten in die Evidenzgenerierung.

Uveitis comprises a group of rare diseases characterised by intraocular inflammation which may cause vision impairment and blindness and mostly affects people of working age. Non-infectious uveitis involving the posterior pole or the entire eye is often treated with different immunomodulating or disease-modifying anti-rheumatic drugs (DMARDs). However, the evidence on long-term management strategies and reduction/termination of treatment is limited. To help develop treatment exit strategies for patients with quiescent uveitis on long-term DMARD treatment, the Treatment Exit Options for Non-infectious Uveitis registry was initiated by the German ophthalmological society. A key aspect of the registry is active participation of patients (patient-reported outcomes, PROs). In a pilot study involving members of patient organizations, a combination of questionnaires covering vision- and general health-related quality of life, adherence to treatment, productivity and effects of treatment were evaluated. As the pilot study showed coverage of relevant patient-related aspects of the disease and its effect on daily life, the evaluated questionnaires were implemented in the registry's patient module. The registry including the patient module uses the electronic data capture (EDC) software REDCap (Version 9, Vanderbilt University, USA). By involving patients in both conceptualization and ongoing data collection, the TOFU registry emphasizes the patients' perspectives, and the inclusion of patient-relevant evidence for such as the development of guidelines and treatment recommendations is ensured.

[Imaging in Uveitis]. / Bildgebung bei Uveitis.

Imaging is one of the key elements in the diagnosis and treatment of eye diseases. This is especially true for intraocular inflammation. In this article, the current imaging modalities such as optical coherence tomography (OCT), OCT angiography, fluorescein and indocyanine green angiography, fundus autofluorescence and wide-field imaging modalities are discussed. The use of these examination methods is illustrated, depending on the focus of intraocular inflammation. In addition, current and future options are presented for optimised, objective monitoring for these clinical entities.

[Trabeculectomy with Collagen Matrix Implant as Bevacizumab Depot: Clinical Results on Efficacy and Safety]. / Trabekulektomie mit Kollagen-Matrix-Implantat als Bevacizumabdepot: klinische Ergebnisse zur Effektivität und Sicherheit.

BACKGROUND: Trabeculectomy for the surgical reduction of internal eye pressure is an established procedure, but the principle limitations are in postoperative wound healing. Various antimetabolites are regularly used to avoid postoperative subconjunctival scarring. The present study investigates whether the combination of an Ologen implant with bevacizumab can modify the rates of success and/or complications during trabeculectomy. METHODS: Three different groups of patients were evaluated in which trabeculectomy was performed with mitomycin C, either alone (group 1) or in combination with an Ologen implant (group 2) or with an Ologen implant with bevacizumab (group 3). The success and failure rates, changes in tension, postoperative complications and postoperative revision for pressure regulation were evaluated. The follow-up was 12 months for all eyes. RESULTS: A total of 130 eyes in 130 patients (mean age 67.74 ± 12.16 years). The number of substances applied preoperatively to reduce intraocular pressure was 2.89 ± 0.98 and the internal ocular pressure was 21.74 ± 5.49 mmHg. Twelve months postoperatively; the mean pressure was 13.14 ± 3.71 mmHg. The greatest absolute success rate (= IOD ≤ 15 mmHg without antiglaucoma medication) was in the group with exclusive goniotrepanation + mitomycin C (MMC; 72.5%), followed by the groups with Ologen (67.5%) and Ologen + bevacizumab (63.6%). The greatest failure rate (= IOD > 15 mmHg) was in the Ologen bevacizumab group (17.3%), followed by the pure Ologen group with 22.5% and the pure trabeculectomy group (12.3%). There were no significant differences in the success and failure rates. There were no serious intra- or postoperative complications in any group. The number of patients for whom it was necessary to loosen the traction thread was significantly lower in the sole Ologen group (p = 0.02). There were also no significant differences between the groups with respect to revisions. CONCLUSION: Using Ologen as drug depot for bevacizumab in a trabeculectomy (TE) with MMC is a safe and active surgical method without an increased risk of complications in comparison to pure TE with MMC or TE with MMC and Ologen implant. After 12 months, this treatment exhibited no significant advantages.

[Does the Foveal Avascular Zone Change in Glaucoma?] / Verändert sich die foveale avaskuläre Zone beim Glaukom?

BACKGROUND: The foveal avascular zone (FAZ) - with perimeter and acircularity index (AI) and surrounding vessel density - are measured in glaucomatous eyes in comparison to healthy eyes - with additional subdivision into visual field defects in different locations (central vs. peripheral). MATERIALS AND METHODS: The data from 128 eyes were evaluated. Firstly, glaucomatous eyes (n = 74) were compared with healthy eyes (n = 54). In addition, glaucomatous eyes with a central visual field defect (ZGD, n = 19) and eyes with peripheral visual field defect (PGD, n = 34) were differentiated. The FAZ area, its perimeter and roundness, as well as the parafoveal vessel density (VD), were calculated by means of optical coherence tomography angiography in the superficial and deep vascular plexus. RESULTS: Glaucomatous eyes have no significant change in FAZ, its perimeter or the AI compared to healthy eyes. However, dividing the glaucomatous eye by visual field defect reveals that the eyes with a central defect have a significantly larger FAZ than those with a peripheral defect and the perimeter is also significantly larger. The AI shows no significant difference depending on the localisation of visual field defects. The macular/foveal VD is significantly lower in the ZDG group. CONCLUSIONS: In glaucoma, the FAZ enlarges when the visual field defect is centralised and VD decreases significantly in the area of fovea.

Transcriptomic and proteomic analysis of iris tissue and aqueous humor in juvenile idiopathic arthritis-associated uveitis.

Gene and protein expression profiles of iris biopsies, aqueous humor (AqH), and sera in patients with juvenile idiopathic arthritis-associated uveitis (JIAU) in comparison to control patients with primary open-angle glaucoma (POAG) and HLA-B27-positive acute anterior uveitis (AAU) were investigated. Via RNA Sequencing (RNA-Seq) and mass spectrometry-based protein expression analyses 136 genes and 56 proteins could be identified as being significantly differentially expressed (DE) between the JIAU and POAG group. Gene expression of different immunoglobulin (Ig) components as well as of the B cell-associated factors ID3, ID1, and EBF1 was significantly upregulated in the JIAU group as compared to POAG patients. qRT-PCR analysis showed a significantly higher gene expression of the B cell-related genes CD19, CD20, CD27, CD138, and MZB1 in the JIAU group. At the protein level, a significantly higher expression of Ig components in JIAU than in POAG was confirmed. The B cell-associated protein MZB1 showed a higher expression in JIAU patients than in POAG which was confirmed by western blot analysis. Using bead-based immunoassay analysis we were able to detect a significantly higher concentration of the B cell-activating and survival factors BAFF, APRIL, and IL-6 in the AqH of JIAU and AAU patients than in POAG patients. The intraocularly upregulated B cell-specific genes and proteins in iris tissue suggest that B cells participate in the immunopathology of JIAU. The intracameral environment in JIAU may facilitate local effector and survival functions of B cells, leading to disease course typical for anterior uveitis.

Peripheral blood monocytes reveal an activated phenotype in pediatric uveitis.

OBJECTIVE: To characterize peripheral blood monocytes in uveitis associated with juvenile idiopathic arthritis (JIAU). METHODS: Peripheral blood monocytes from children with JIA (either with (n = 18) or without uveitis (n = 11)), idiopathic anterior uveitis (IAU; n = 12) and healthy controls (n = 11) were analyzed by flow cytometry. RESULTS: Percentage of CD14 + CD86+ monocytes and CD86 expression on single cell level were significantly higher in all patient groups than in controls, whereas no major differences existed between patient groups. Frequency of CD39+ (p < 0.05 all groups) and CD73+ monocytes (p = 0.03 JIAU vs controls) was elevated in patients. Disease activity did not influence monocyte phenotypes, but in methotrexate-treated JIAU patients numbers of CCR2+ monocytes were reduced and numbers of CD86+ and CD39+ cells increased. CONCLUSION: Children with arthritis or uveitis display a distinct monocytic phenotype when compared to cells from healthy children. Phenotypic changes seem to be neither arthritis- nor uveitis-dependent, but may be modified by treatment.

The effect of methotrexate and sulfasalazine on the course of HLA-B27-positive anterior uveitis: results from a retrospective cohort study.

PURPOSE: To investigate the effect of methotrexate (MTX) or sulfasalazine (SSZ) on the course of HLA-B27-positive, remitting acute anterior uveitis (AAU). METHODS: Forty-six patients with HLA-B27-positive AAU with or without associated systemic rheumatic disease either receiving MTX (n = 20), SSZ (n = 13), or no systemic immunomodulating treatment (Ctrl; n = 13) were studied retrospectively. Best-corrected visual acuity (BCVA), AAU relapse rate, and occurrence of uveitis-related ocular complications were analyzed at baseline (BL) and at 12-month follow-up (FU). RESULTS: Groups did not differ regarding age, gender, and presence of associated systemic diseases. BCVA at baseline was significantly worse in patients receiving MTX (logMAR 0.39 ± 0.4) than in those treated with SSZ (0.17 ± 0.2; P = 0.05) or in controls (Ctrl; 0.14 ± 0.2; P = 0.009). At the 12-month endpoint, MTX treatment was associated with significantly improved BCVA (0.18 ± 0.4 logMAR; P = 0.004). In contrast, BCVA did not significantly change in patients treated with SSZ (0.17 ± 0.3 logMAR) or in the controls (0.11 ± 0.2 logMAR). The annual uveitis relapse rate significantly decreased with MTX (BL 3.6 ± 2.4 relapses to FU 0.7 ± 0.8; P = 0.0001) and SSZ (BL 3.6 ± 1.9 to FU 1.8 ± 2.4, P < 0.01), but not in the controls (BL 1.9 ± 1.4 vs 1.9 ± 1.7 FU). The complication rate was slightly reduced with MTX (BL 1.75 ± 1.2 complications present versus FU 1.3 ± 1.2, P = 0.09) but not with SSZ (BL 0.9 ± 0.8 to FU 1.3 ± 1.4; P = 0.4) or in the controls (BL and FU 1.0 ± 0.95; P = 0.7). CONCLUSIONS: MTX and SSZ reduced the uveitis relapse rate in HLA-B27-positive AAU patients, with MTX showing a beneficial effect on AAU-related macular edema.

[Macular Edema in Uveitis - Steroids or VEGF Inhibitors?] / Makulaödem bei Uveitis ­ Steroide oder VEGF-Inhibitoren?

Macular edema in uveitis patients is certainly the most frequent complication leading to a permanent and irreversible reduction in vision during the course of the disease. Thanks to optical coherence tomography (OCT) technology and fluorescein angiography (FAG), significantly more macular edemas are detected. Macular edema can be found in various uveitis varieties and can show different clinical patterns. All macular edema should be treated. Macular edema with active inflammation usually reacts very well to general uveitis treatment. In the case of eyes without visible inflammation, however, the response to such therapy is usually less effective. According to the latest treatment recommendations, dexamethasone implants should be used as the first intravitreal therapy. Vascular endothelial growth factor inhibitors (VEGF inhibitors) are second-line treatment regimens. The choice of therapy is, therefore, primarily based on the degree of inflammation and the individual complications, such as glaucoma, lens situation or previous increase in IOP after steroid administration. These individual complications may allow using VEGF inhibitors as first line treatment. An improvement in the macular edema can be achieved with both groups of active substances.

[Uveitic Secondary Glaucoma]. / Sekundärglaukom bei Uveitis.

An intraocular pressure increase with development of glaucomatous damage is a common complication of uveitis. The prevalence has a wide range depending on various factors such as the underlying uveitis type and the duration of the disease. Pathogenetically, a distinction must be made between a secondary angle closure component and the more frequently occurring open-angle glaucoma. In diagnostics, in addition to the clinical optic nerve head assessment, perimetry and tonometry, the use of imaging examination equipment, such as OCT and HRT, are recommended. In the context of uveitic glaucoma, it must be considered in the evaluation, because the glaucoma-typical changes are generally less pronounced or can be concealed by retinal swelling in comparison with other forms of glaucoma. Therapeutically, drug therapy in the form of eye drops continues to be a first-line recommendation, with the use of topical carbonic anhydrase inhibitors or beta-blockers primarily preferred, depending on the contraindications. An operative therapy follows after unsuccessful or inadequate conservative therapy: the adequate surgical technique depends on the respective finding and includes actually techniques such as filtering procedures and glaucoma drainage devices.

Discontinuation of long-term adalimumab treatment in patients with juvenile idiopathic arthritis-associated uveitis.

PURPOSE: The purpose of this study was to evaluate the discontinuation of adalimumab (ADA) treatment in patients with juvenile idiopathic arthritis-associated uveitis (JIAU). METHODS: Patients in whom ADA treatment was initiated for JIAU were included in this retrospective analysis. Reasons for discontinuing ADA treatment in patients with primary treatment response were analysed. RESULTS: Within a group of 387 JIAU patients, 59 of 68 patients who were treated with ADA achieved a sufficient response to treatment within 6 months. Here, 39 patients (66.1 %) were still on therapy at their last follow-up visit (mean treatment duration of 38.3 months, range 12-91). In another 20 patients, ADA had been discontinued after 1 or 2 years or later, in 10 % (n = 2), 45 % (n = 9) and 45 % (n = 9) of patients, respectively (mean 30.6 months; range 10-65). Reasons for discontinuing ADA were reactivation of uveitis (n = 8, 3.93 per 100 patient-years) or arthritis (n = 4; 1.97 per 100 patient-years), or ≥2 years of complete disease inactivity (n = 3, 1.47 per 100 patient-years), adverse events (n = 4; 1.89 per 100 patient-years), or other (n = 1; 0.47 per 100 patient-years). CONCLUSIONS: The data show a good primary response to ADA in patients with refractory JIAU. Due to the increasing rate of adalimumab failure or adverse events during long-term treatment, further treatment options may be required.

Phenotypic changes of peripheral blood mononuclear cells upon corticosteroid treatment in idiopathic intermediate uveitis.

We analyzed phenotype and function of peripheral blood mononuclear cells in 9 patients with active idiopathic intermediate uveitis (IIU) before and after 6 and 12weeks of systemic corticosteroid (CS) treatment and compared to 28 healthy individuals. Monocytes from IIU patients showed increased MHCII expression compared with controls (p=0.09). Treatment reduced expression of MHCII, CD86, CD39 and CD124 (all p<0.05), whereas the percentage of CD121b-expressing monocytes was increased by week 6 (p=0.039). Patients showed alterations in T cell polarization (Th1/Th2 ratio: patients 5.2 versus controls 3.1, p=0.054; Th17/Treg ratio: 3.0 versus 1.7, p=0.027). S100A12 serum levels were higher in active IIU (p=0.057). Phagocytosis, oxidative burst and serum cytokine levels did not differ between patients and controls, and were not altered by treatment. In conclusion, monocytes from patients with active IIU show increased co-stimulatory capacities, which are modulated by systemic CS treatment, whereas innate immune cell functions are not altered.

Influence of optic disc leakage on objective optic nerve head assessment in patients with uveitis.

PURPOSE: Secondary glaucoma is a common complication in patients with uveitis. Heidelberg Retina Tomography (HRT) and retinal nerve fiber layer (RNFL) thickness on optical coherence tomography (OCT) are widely used for examining optic nerve head changes. We evaluated these parameters in patients with uveitis and secondary glaucoma and with inflammatory papillary leakage on fluorescein angiography. METHODS: Prospective single-center analysis of patients with uveitis, evaluating the impact of optic disc leakage on objective optic disc imaging parameters. RESULTS: Overall, 96 eyes of 59 patients were included. Papillary leakage was found in 42 eyes (43.8 %), and secondary glaucoma was found in 41 eyes (42.7 %). Glaucoma and papillary leakage were present in 12 (29 %) eyes with leakage and in 29 (54 %) eyes without leakage (p = 0.023). Neuroretinal rim area (p = 0.004), rim volume on HRT (p = 0.004), and RNFL thickness on OCT (p = 0.0008) were significantly increased in eyes with papillary leakage, while RNFL on HRT was unchanged (p = 0.255). When only eyes with normal IOP were examined, all objective parameters on OCT and HRT were significantly increased, whereas in eyes with secondary glaucoma, there was only a trend in the same direction, which did not reach significance. A comparison of eyes with secondary glaucoma and optic disc leakage to normal eyes with no glaucoma or leakage revealed no difference in any of the parameters. CONCLUSIONS: The objective parameters of optic nerve head imaging tools are significantly influenced by papillary leakage. In patients with secondary glaucoma and papillary leakage, these techniques are unable to detect and monitor glaucomatous damage.

Canaloplasty in patients with uveitic glaucoma: a pilot study.

PURPOSE: Glaucoma is a common vision-threatening complication of uveitis. We investigated the outcome of canaloplasty in patients with chronic uveitis and uncontrolled secondary glaucoma. METHODS: This was a retrospective study of 12 patients with medically uncontrolled secondary glaucoma who underwent canaloplasty (14 treated eyes), with follow-up of ≥ 24 months. The primary outcome measure was complete and qualified (requirement for anti-glaucomatous medication) surgical success rates, as determined by a reduction in intraocular pressure (IOP) and the need for anti-glaucomatous medication. Secondary outcome measures were uveitis activity, best-corrected visual acuity (BCVA), vision-threatening complications, and secondary glaucoma surgery. RESULTS: Canaloplasty resulted in a significant IOP reduction, from a mean preoperative baseline level of 27.1 ± 12.3 mmHg to a mean of 14.5 ± 4.3 mmHg (p = 0.01) at 24 months. Complete success (limit of 15 mmHg) was achieved in six patients and qualified success in one patient. However, failure was noted in five patients, two of whom required additional glaucoma surgery. Topical anti-glaucomatous medications were reduced from 2.7 ± 1.2 (mean ± SD) at baseline to 0.6 ± 1.2 at 24 months (p = 0.007). Uveitis activity did not increase after surgery. BCVA was not reduced; cataract progressed in two patients. CONCLUSION: At 2-year follow-up, overall success rate was 58 % (IOP ≤ 15 with or without medication), and surgery failed in 5 eyes, 2 eyes requiring additional glaucoma surgery. No harmful complications or worsening of uveitis activity were noted.

Clinical manifestation of Fuchs uveitis syndrome in childhood.

PURPOSE: The aim of this study was to describe clinical signs and complications of Fuchs uveitis syndrome (FUS) with onset in childhood. METHODS: Ophthalmologic findings and complications in patients with FUS becoming manifest before the age of 16 years were analyzed in a retrospective study at a tertiary referral uveitis center. Inclusion criteria were the presence of pathognomonic FUS findings at any time point and exclusion of any systemic immune-mediated or infectious disease. RESULTS: A total of 23 patients (male = 16, female = 7) with juvenile FUS (unilateral n = 20, bilateral n = 3 patients) were included in the study. Mean ages at uveitis and FUS diagnosis were 12.0 ± 4.2 and 22.7 ± 10.7 years, respectively. In six patients, inflammation was noted at age ≤ 7 years. The following inflammatory signs were observed in a total of 26 eyes: ≤ 1+ anterior chamber cell grade (n = 26), vitreous cells (n = 24), fine keratic precipitates (KPs; n = 23), stellate KPs (n = 11), mutton-fat KPs (n = 23), diffuse (n = 24) or inferior (n = 8) distribution of KPs, Koeppe nodules (n = 10), and iris heterochromia (n = 14). A representative subgroup of patients (n = 5) is shown who presented with non-specific clinical signs in the beginning and in whom typical FUS signs became manifest only at a later stage. Secondary complications such as cataract (n = 19), ocular hypertension (n = 3), or glaucomatous disc damage (n = 2) were found after a mean uveitis duration of 11.6, 19.5, and 20.3 years, respectively. CONCLUSION: FUS may begin in early childhood, and the characteristic findings may not be present at onset of disease. The diagnosis is often delayed for years, occasionally with the consequence of overtreatment with anti-inflammatory drugs.

Fluocinolone acetonide 0.2 µg/day intravitreal implant in non-infectious uveitis affecting the posterior segment: EU expert user panel consensus-based clinical recommendations.

BACKGROUND: Non-infectious uveitis affecting the posterior segment of the eye (NIU-PS) is an inflammatory disease, which can significantly impair visual acuity if not adequately treated. Fluocinolone-acetonide sustained-release-0.2 µg/day intravitreal (FAc) implants are indicated for prevention of relapse in recurrent NIU-PS. The aim here was to provide treating clinicians with some consensus-based-recommendations for the clinical management of patients with NIU-PS with 0.2 µg/day FAc implants. METHODS: A European-clinical-expert-group agreed to develop a consensus report on different issues related to the use of FAc implants in patients with NIU-PS. RESULTS: The Clinical-expert-panel provided specific recommendations focusing on clinical presentation (unilateral/bilateral) of the NIU-PS; systemic involvement of NIU-PS and the lens status. Treatment algorithms were developed; one that refers to the management of patients with NIU-PS in clinical practice and another that establishes the best clinical scenarios for the use of FAc implants, both as monotherapy and as adjuvant therapy. Additionally, the Clinical-expert-panel has provided recommendations about the use of the FAc implants in a clinical-setting. The Clinical-expert-panel also considered the safety profile of FAc implants and their possible implications in the daily practice. CONCLUSIONS: As more clinical experience has been gained using FAc implants, it was necessary to update the clinical recommendations that guide patient management in the clinic. The current consensus document addresses relevant issues related to the use of FAc implants on different types of patients with various etiologies of NIU-PS, and was conducted to standardize approaches to help specialists obtain better clinical outcomes.