PURPOSE: To evaluate the immediate alterations in the thickness of the macular ganglion cell-inner plexiform layer (mGCIPL), peripapillary retinal nerve fiber layer (RNFL), inner retinal layer (IRL), and outer retinal layer (ORL) using spectral domain optical coherence tomography (SD-OCT) subsequent to strabismus surgery in pediatric patients diagnosed with horizontal esotropia. METHODS: Twenty-eight eyes from twenty-one child patients who had undergone uncomplicated horizontal rectus muscle surgery due to strabismus were included. Measurements of RNFL, mGCL-IPL, IRL, and ORL using structural OCT were conducted both before the surgery and one month after the surgical procedure. Importantly, a control group comprising 14 healthy eyes, matched for age and significant refractive error (<3.00 diopters), was included in the current analysis. RESULTS: Our analysis indicated no significant disparity before and after surgery in terms of best-corrected visual acuity (BCVA), RNFL, IRL, and ORL. Conversely, concerning the macular ganglion cell layer-inner plexiform layer analysis, a substantial increase in mGCL-IPL was observed following the surgical intervention. The mean mGCL-IPL measured 60.8 ± 9.2 µm at baseline and 66.1 ± 13.2 µm one month after the surgery (p = 0.026). Notably, comparison between the strabismus group at baseline and the healthy group revealed a significant reduction in mGCL-IPL in the strabismus group (60.8 ± 9.2) compared to the healthy control group (68.3 ± 7.2; p = 0.014). CONCLUSIONS: Following strabismus surgery, our observations pointed towards a thickening of the mGCL-IPL layer, which is likely attributable to transient local inflammation. Additionally, we identified a significant differentiation in the mGCL-IPL complex between the pediatric patient group with strabismus and the control group.
PURPOSE: To assess the success of the short-term location of the heavy silicone oil (Densiron 68, HSO) as endotamponade after pars plana vitrectomy (PPV) for rhegmatogenous complex retinal re-detachment (RRD). METHODS: Consecutive, retrospective, nonrandomized, pilot study was conducted. Twenty-two eyes of 22 patients with complex inferior RRD previously tamponade with gas (SF6 or C3F8) or 1000cSt silicon oil (SSO) were selected. All were treated with heavy silicone oil HSO endotamponade, and its removal was performed after 1-month. The main outcomes were best corrected visual acuity (BCVA) and postoperative complications after the heavy silicone oil removal. RESULTS: Of the 22 eyes, 10 were SSO endotamponade, 3 with fluorinated gas 14% C3F8, and 9 with 20% SF6 at first surgery. In all cases, a complex inferior RRD was observed after the first surgery, in 1-3 months after silicone oil removal or gas endotamponade introduction.In ten cases the proliferative vitreoretinopathy PVR (grade B or C 1-3) was found. The main BCVA before HSO removal was 0.55± 0.20 logMAR (range 0.4-0.7) and after the HSO removal, it was 0.32± 0.29 logMAR (0.1-0.4).Among the postoperative complications, only in 4 eyes the macular edema was found (medically resolved), in 4 eyes an increase of intraocular pressure (IOP), and none of these developed the epiretinal membrane (ERM). CONCLUSION: The main purpose of this study is to establish a short-term heavy silicone oil endotamponade in eyes with complex retinal detachment recurrences, reducing the possible postoperative complications and having a better prognosis for visual acuity outcomes.
PURPOSE: To investigate outcomes after Brolucizumab injection in naïve treatment or non-responder patients with exudative age-related macular degeneration (AMD). METHODS: It is a retrospective, comparative, cohort study conducted at the tertiary referral center of the University Hospital Polyclinic of Bari, for 5 years, from November 2017 until May 2022. 41 eyes with wet-AMD (w-AMD) were included, undergoing anti-vascular endothelial growth factor (VEGF) intravitreal injections. The sample was divided into two groups, the Bro-Switch group, and the Bro-Naïve group. The Bro-Switch group previously received a slot of other anti-VEGF intravitreal drugs. The Bro-Naïve group received Brolucizumab (Bro) as the first treatment. The pigment epithelium detachment (PED) and the best-corrected visual acuity (BCVA) outcomes before and after Bro-injection were evaluated. RESULTS: A significant reduction in PED measurement was registered in all eyes treated with Bro-injection (p = 0.35). The Bro-Naïve group improved better in PED measurement (mean difference: 297.92 ± 72,32) as compared to the Bro-Switch group (mean difference: 185.06 ± 11.07). On the contrary, no significant reduction in BCVA in the two groups was recorded (p = 0.66). CONCLUSION: We suggest Bro-injection for w-AMD as effective anatomical outcomes in PED flattening, but not similar in visual results. Although this study evaluated short-term outcomes, the hopeful results can lead to interesting medium-long time effects.
Chronic diseases are a growing problem for global health due to the large number of people they involve, the repercussions they have on the mental and physical well-being of those affected, and the costs to society. Particularly, chronic illnesses of childhood have important psychological implications, not only for affected children but also for their parents. Among these pathologies, neurodevelopmental disorders (NDDs) and uveitis associated with juvenile idiopathic arthritis (JIA-U) may affect mental and physical health, emotions, memory, learning, and socializing. This study evaluates the psychological and behavioral/emotional impact of NDDs and JIA-U on children and parents. Specifically, 30 children with active JIA-U and 30 children with NDDs and their parents completed the Child Behavior Checklist (CBCL) and Parent Stress Index-Short Form (PSI) questionnaires. Children with NDDs have statistically significant differences in all the emotional and behavioral variables compared to JIA-U children, and parents of children with NDDs experience an increased stress load compared to parents of children with JIA-U. This study emphasizes the wide range of emotional and behavioral challenges that parents face with NDDs. This study emphasizes that parents of children with NDDs not only experience higher levels of stress compared to parents of normally developing children but also experience higher levels of stress compared to parents of children with potentially debilitating chronic diseases such as JIA-U.
Introduction: Inverted Internal Limiting Membrane (ILM)-flap technique demonstrated its effectiveness, in terms of anatomical closure rate and visual acuity recovery for high myopic macular holes. We evaluated macular function after a successful inverted ILM-flap for macular holes in high myopic eyes (hMMH) using microperimetry to predict visual prognosis. Methods: A retrospective study on 23 eyes of 23 patients after surgical closure of hMMH, was performed. All patients underwent inverted ILM-flap and gas tamponade. Cataract surgery was performed in phakic eyes. Study outcomes including best-corrected visual acuity (BCVA), retinal sensitivity (RS) at central 12°, central retinal sensitivity (CRS) at central 4° and mean deviation (MD), and fixation behavior as bivariate contour ellipse area (BCEA, degrees2) measured by microperimetry, were evaluated over 6 months. A mixed-effects model was used to evaluate and compare the repeated measurements of outcomes between phakic and pseudophakic eyes. A regression model was performed to assess the relationship between BCVA at 6 months and independent variables. Results: Overall mean BCVA improved from 0.98 ± 0.21 logMAR at baseline to 0.47 ± 0.31 logMAR at the last follow-up (p < 0.001). Over 6 months, overall sensitivity measurements improved (RS, p = 0.001; CRS, p < 0.0001; MD, p = 0.03), and the BCEA decreased in dimension, although not significantly (p ≥ 0.05). The mixed model revealed a significantly better effect of inverted ILM-flap combined with cataract surgery on BCVA and CRS in phakic eyes than inverted ILM-flap alone in pseudophakic ones. The regression model revealed a relationship of 6-month BCVA with pre-operative BCVA (ß = 0.60, p = 0.02) and RS (ß = -0.03, p = 0.01). Conclusion: The inverted ILM-flap technique significantly improved visual acuity and retinal sensitivity after the hMMH closure, particularly when combined with cataract extraction. Pre-operative visual acuity and retinal sensitivity at central 12° may predict post-surgical visual acuity.
BACKGROUND: Microperimetric biofeedback training improved visual acuity and fixation stability in patients who previously underwent macular surgery. We aimed to compare the functional results of biofeedback training with the standard of care in patients who underwent successful inverted Internal Limiting Membrane (ILM)-flap technique for high myopic macular holes (hMMH). METHODS: This was a retrospective, comparative, cohort study. Patients with hMMH after surgical hole closure underwent microperimetric biofeedback using structured light stimulus plus acoustic tone (n = 12; Biofeedback) or standard of care with scheduled visits (n = 11; Control). Best-corrected visual acuity, retinal sensitivity at central 12° (RS) and 4° (CRS) with a mean deviation at central 12° (MD), and fixation stability as bivariate contour ellipse area (BCEA 68%, 95%, and 99%) were assessed at baseline and month 1, 3, 6, and 12. The Mann-Whitney test was used to test the difference between the groups. RESULTS: Baseline functional parameters were not significantly different among the groups. BCVA significantly improved in each group (Biofeedback, p = 0.002; Control, p ≤ 0.02) at all follow-up visits. CRS significantly improved at 6 (p = 0.03) and 12 (p = 0.01) months in the Biofeedback group and at month 12 (p = 0.01) in the Control group. RS (p = 0.001) and MD (p = 0.005) improved at the last follow-up only in the trained group. After training, BCEA 68% and 95% significantly improved (6 and 12 months, p < 0.05). The Biofeedback group had better results in RS (p ≤ 0.02), CRS (p ≤ 0.02), and BCEA 68%, 95%, and 99% (p ≤ 0.01) compared to the Control at all follow-ups. BCVA and MD were better in the Biofeedback group at month 3 (p = 0.01), and month 3 (p = 0.01) and 12 (p = 0.003), respectively. CONCLUSIONS: Microperimetric biofeedback can increase retinal sensitivity and stabilize fixation better than the standard care over months after a successful inverted ILM-flap for hMMH.
Purpose: We present a rare case of Citrobacter koseri culture-positive endophthalmitis in a postvitrectomy silicone oil-filled eye. Case report. A 64-year-old male patient presented to our ophthalmology emergency room with representative symptoms of acute endophthalmitis. He underwent a plana vitrectomy oil-filled tamponade previously. Preoperative and postoperative findings of the case were reported. Results: Culture tests of aqueous, silicone oil, and vitreous taps were positive for Citrobacter koseri. Conclusions: Culture-positive endophthalmitis in a silicone oil-filled eye has very rarely been in the literature. The described cases were caused by acute inflammatory reactions to silicone oil and were culture-negative. The postvitrectomy culture-positive endophthalmitis caused by Citrobacter is a very rare condition, and its management is not so smooth. Approaching with silicone oil removal, intraoperative intravitreal antibiotic injection, and silicone oil reinjection was performed in our case with good outcomes.
PURPOSE: Spectral-domain optical coherence tomography (SD-OCT) was used to evaluate, in patients with multiple sclerosis without a history of optic neuritis (MSNON), the proportion of the different macular ganglion cell-inner plexiform layer complex (mGCIP) defect patterns. The results were compared with those of healthy controls (HCs). METHODS: In this cross-sectional case-control study, 34 eyes of 34 individuals, 17 with MSNON and 17 HCs, were evaluated. All participants underwent mGCIP thickness measurement using SD-OCT (Zeiss Cirrus HD-OCT 4000, macular cube protocol). The mGCIP defect patterns were classified in nine types (minimal, inner, outer, diffuse mild, diffuse severe inferior confined, inferior dominant, superior confined, and superior dominant), according to the shape derived by the deviation map of the instrument, and the proportion of each type was assessed. RESULTS: A mGCIP defect pattern was detected in 70.5% of MSNON eyes, with an inner type as the most frequent pattern (47%), followed by the outer type (11.7%) and the inferior confined type (11.7%). No defect was found in Hcs. CONCLUSIONS: A significant thinning of the mGCIP with the frequent presence of an inner defect was seen in MSNON patients. The presence of this defect may serve as a biomarker of subclinical optic nerve involvement in MS patients.
Multiple Sclerosis , Optic Neuritis , Humans , Multiple Sclerosis/complications , Multiple Sclerosis/diagnosis , Tomography, Optical Coherence/methods , Pilot Projects , Retinal Ganglion Cells , Cross-Sectional Studies , Case-Control Studies , Optic Neuritis/diagnosis
PURPOSE: To propose optical coherence tomography angiography (OCT-A) for the follow-up of neovascular age-related macular degeneration (nAMD) treated with a treat-and-extend (T&E) aflibercept regimen to avoid overtreatment. METHODS: Retrospective, cohort, pilot study. We analysed 16 consecutive-treatment naïve nAMD eyes following up 2-years at the Eye Clinic, Bari, Italy. Intravitreal aflibercept injections in the T&E regimen for no less than 12 months, during which the macula was dry without any sign of intraretinal or subretinal fluid (SRF) at each visit, were performed. Parametric data were evaluated using an analysis of variance (ANOVA); any non-parametric statistical calculations were performed using the Wilcoxon and Mann-Whitney test. RESULTS: The average number of injections during follow-up was: 8.8 ± 1. Treatment regimen adjustments were 4 weeks (W), 8W (4 + 4), 10W (8 + 2), 12W (8 + 4, or 10 + 2). No significant CNV size change from 4 to 8W (-0.027 ± 0.22â mm², p = 0.088), and from 8 to 12W (-0.04 ± 0.11â mm², p = 0.065) were found. Likewise, no significant decrease in choriocapillaris flow (CF) was detected (p = 0.056). CONCLUSION: We suggest that OCT-A may be useful in the evaluation of dry macula to decide the best approach for perchance adjusting injection intervals based on changes of CNV size.
Angiogenesis Inhibitors , Choroidal Neovascularization , Humans , Angiogenesis Inhibitors/therapeutic use , Follow-Up Studies , Tomography, Optical Coherence/methods , Retrospective Studies , Pilot Projects , Choroidal Neovascularization/diagnosis , Choroidal Neovascularization/drug therapy , Receptors, Vascular Endothelial Growth Factor/therapeutic use , Recombinant Fusion Proteins/therapeutic use , Fluorescein Angiography/methods , Overtreatment , Intravitreal Injections
Juvenile idiopathic arthritis (JIA) is a chronic inflammatory disease common in children and young adults. Uveitis is the most frequent serious extra-articular JIA manifestation and can lead to severe ocular complications, vision loss, and permanent blindness. This study aims to evaluate the psychological condition and the quality of life of children affected by JIA associated with uveitis (JIA-U) and the repercussion of this condition on parents. Thirty children and adolescents with active uveitis (Uveitis group) and comorbid joint symptoms of JIA were referred to the Unit of Ophthalmology, Giovanni XXIII Hospital of Bari, and 30 age-matched healthy controls (Healthy group) were enrolled with their parents. Four questionnaires were administered: Child Behaviour Checklist (CBCL), Parent Stress Index in Short Form (PSI), Pediatric Quality of Life Inventory (PedsQL), and Coping Inventory for Stressful Situations (CISS). The data were collected from February 2021 to December 2021. No significant differences between the two groups in CBCL, PSI, or CISS tests were shown (p > 0.05). Conversely, significant differences between the two groups were observed in the PedsQL (p < 0.05). This study shows how several ocular complications, recurrent eye examinations, and the rigor of long-term treatment may negatively influence health-related quality of life in children with JIA-U.
BACKGROUND: Posterior uveitis represents the second most frequent type of uveitis (15-30% of all uveitis). Noninfectious posterior uveitis complicated with secondary cystoid macular edema (CME) affects the visual prognosis negatively. The objective of the current study is to detect possible microvascular changes causing relapsing uveitis-related CME using optical coherence tomography angiography (OCTA). METHODS: This is an interventional, observational, retrospective study with 1 year follow-up. Patients with noninfectious, posterior uveitis-related CME undergoing dexamethasone (DEX) implant were evaluated. Following the DEX-implant were carried out control visits after 1 month, 2-months, 4-months, 6-months, and for up 1-year. A total of 76 eyes of 38 consecutive patients with noninfectious posterior uveitis were enrolled (consecutive sample). Complicated noninfectious posterior uveitis with secondary CME was diagnosed in 56 eyes of uveitis patients (73.7%) and reviewed. RESULTS: Our investigation showed (1) a reduction in superficial vessel plexus (SVP) measurements within 2-month (84%), reaching 96.4% for up 1-year, (2) an irregular profile of SVP in 69.6% of cases, persisting for up 1-year; relapsing uveitis-related CME eyes with irregular superficial foveal avascular zone (FAZ) profile were in 51%, while the SVP measurements reestablished in 100% of cases. Conversely, (3) the deep vascular plexus (DVP) parameters restored in a lower number of eyes within the 2-month (39.3%), remaining abnormal in 46.4% of cases for up 1-year; despite DVP restored in 53.6% of cases for up 1 year, (4) a capillary rarefaction ring around the FAZ appeared in 80.4% of cases; the relapsing uveitis-related CME eyes with abnormal DVP parameters were present in 41% of cases, of which 92.1% showed a rarefaction ring had abnormal DVP. CONCLUSIONS: The use of OCTA enabled the evaluation in detail of retinal microvascular changes. We suggested that the possibility of the recurrence of the uveitis-related CME depends on the persistence of modifications of the superficial and deep layers. In this regard, we propose to implement the current imaging armamentarium with OCTA for the follow-up of patients with noninfectious uveitis-related CME.
Macular Edema , Uveitis, Posterior , Uveitis , Humans , Macular Edema/etiology , Macular Edema/complications , Tomography, Optical Coherence/methods , Fluorescein Angiography/methods , Retinal Vessels , Retrospective Studies , Visual Acuity , Neoplasm Recurrence, Local , Uveitis, Posterior/complications , Uveitis/complications , Uveitis/diagnosis , Dexamethasone
INTRODUCTION: The aim of this paper is to point out the design, development and deployment of the AutoInflammatory Disease Alliance (AIDA) International Registry for paediatric and adult patients with non-infectious uveitis (NIU). METHODS: This is a physician-driven, population- and electronic-based registry implemented for both retrospective and prospective collection of real-world demographics, clinical, laboratory, instrumental and socioeconomic data of patients with uveitis and other non-infectious inflammatory ocular diseases recruited through the AIDA Network. Data recruitment, based on the Research Electronic Data Capture (REDCap) tool, is thought to collect standardised information for real-life research and has been developed to change over time according to future scientific acquisitions and potentially communicate with other similar instruments. Security, data quality and data governance are cornerstones of this platform. RESULTS: Ninety-five centres have been involved from 19 countries and four continents from 24 March to 16 November 2021. Forty-eight out of 95 have already obtained the approval from their local ethics committees. At present, the platform counts 259 users (95 principal investigators, 160 site investigators, 2 lead investigators, and 2 data managers). The AIDA Registry collects baseline and follow-up data using 3943 fields organised into 13 instruments, including patient's demographics, history, symptoms, trigger/risk factors, therapies and healthcare utilization for patients with NIU. CONCLUSIONS: The development of the AIDA Registry for patients with NIU will facilitate the collection of standardised data leading to real-world evidence and enabling international multicentre collaborative research through inclusion of patients and their families worldwide.
INTRODUCTION: This article points out the design, methods, development and deployment of the international registry promoted by the AutoInflammatory Disease Alliance (AIDA) Network with the aim to define and assess paediatric and adult patients with immune-mediated scleritis. METHODS: This registry collects both retrospective and prospective real-world data from patients with non-infectious scleritis through the Research Electronic Data Capture (REDCap) tool and aims to promote knowledge and real-life evidence from patients enrolled worldwide; the registry also allows the collection of standardised data, ensuring the highest levels of security and anonymity of patients' data and flexibility to change according to scientific acquisitions over time. The communication with other similar registries has been also ensured in order to pursue the sustainability of the project with respect to the adaptation of collected data to the most diverse research projects. RESULTS: Since the launch of the registry, 99 centres have been involved from 20 countries and four continents. Forty-eight of the centres have already obtained a formal approval from their local ethics committees. At present, the platform counts 259 users (95 principal investigators, 160 site investigators, 2 lead investigators, and 2 data managers); the platform collects baseline and follow-up data using 3683 fields organised into 13 instruments, including patient's demographics, history, symptoms, trigger or risk factors, therapies and healthcare utilization. CONCLUSIONS: The development of the AIDA International Registry for patients with non-infectious scleritis will allow solid research on this rare condition. Real-world evidence resulting from standardised real-life data will lead to the optimisation of routine clinical and therapeutic management, which are currently limited by the rarity of this ocular inflammatory condition.
BACKGROUND: The prospective DIabetes and CATaract Study II (DICAT II) was performed to characterise the risks of cataract surgery to the retinae of patients with early diabetic macular oedema (E-DMO). METHODS: DICAT II was a prospective, comparative, multicentre, observational study involving six Italian clinics. Patients were aged ≥55 years, had type 1 or 2 diabetes with spectral-domain optical coherence tomography evidence of ESASO classification Early DMO. Group 1 eyes (78 eyes, 78 patients) underwent phacoemulsification-based cataract surgery. Group 2 eyes (65 eyes, 65 patients) had E-DMO and either clear media or had undergone uncomplicated cataract surgery ≥1 year previously. Central subfield thickness (CST) and best-corrected visual acuity (BCVA) were assessed in both groups. RESULTS: The negative impact of surgery on CST was evident after the first postoperative week; CST peaked during the first month, then rapidly decreased. CST worsening ≥10 µm was observed in 63/78 eyes (80.7%) and 29/65 eyes (44.6%) in Groups 1 and 2, respectively (p < 0.0001). CST worsening of ≥50 µm was observed in 51 eyes (65.4%) and 10 eyes (15.4%) in Groups 1 and 2, respectively (p < 0.0001). Mean CST worsening was lower in Group 2 than in Group 1 (38.6 ± 30.4 µm vs 85.5 ± 55.3 µm, p < 0.0001) with a lower BCVA loss (-2.6 ± 3.5 letters vs -8.2 ± 6.2 letters, p < 0.0001). Higher glycaemic levels and HBA1c levels were significantly associated with the risk of >50 µm CST worsening in eyes from both groups. CONCLUSION: Early DMO is associated with poorer outcomes after cataract surgery and requires close pre- and postoperative monitoring.
Cataract , Diabetes Mellitus , Diabetic Retinopathy , Macular Edema , Cataract/complications , Diabetic Retinopathy/complications , Diabetic Retinopathy/surgery , Humans , Prospective Studies , Tomography, Optical Coherence/methods , Visual Acuity
Endogenous Endophthalmitis (EE) is a rare cause of blindness in the pediatric age group and this may account for the paucity of management guidelines in the literature. In this report, we describe our experience with a 10-year-old immunocompetent female who developed EE and became blind because of rapidly progressive and destructive inflammatory changes in her eye in spite of seemingly timely treatment.
Endophthalmitis , Eye Infections, Fungal , Blindness , Child , Endophthalmitis/diagnosis , Endophthalmitis/drug therapy , Eye Infections, Fungal/diagnosis , Eye Infections, Fungal/drug therapy , Female , Fungi , Humans
BACKGROUND/PURPOSE: Full-thickness macular hole is a complication of idiopathic macular telangiectasia type 2 with a low surgical closure rate and a poor functional recovery. We analyze morphologic and functional outcomes after an inverted internal limiting membrane-flap technique for full-thickness macular hole complicating idiopathic macular telangiectasia type 2 using optical coherence tomography and microperimetry. CASE REPORT: A 77-year-old man presented us complaining visual impairment in the left eye (20/40) due to a full-thickness macular hole complicating idiopathic macular telangiectasia type 2. The patient underwent vitrectomy with an inverted internal limiting membrane-flap technique and injection of 22% SF6 gas. Optical coherence tomography and functional analysis, including visual acuity test and microperimetry, were performed before surgery and over 3 months. Optical coherence tomography scans revealed macular hole closure at 1-month follow-up. Visual acuity and retinal sensitivity improved from 20/40 to 20/20 and from 13.1 to 14.9 dB, respectively. Fixation stability (bivariate contour ellipse area) improved from 2.02 to 1.58 deg2, from 5.44 to 4.24 deg2 and from 9.72 to 7.58 deg2, at 68%, 95%, and 99% of fixation points, respectively. CONCLUSION: Inverted internal limiting membrane-flap technique may be an alternative approach in patients with full-thickness macular hole complicating diopathic macular telangiectasia type 2, and microperimetry may be a useful tool to analyze related functional changes.
Basement Membrane/surgery , Retinal Perforations/surgery , Retinal Telangiectasis/complications , Surgical Flaps , Aged , Endotamponade , Humans , Male , Recovery of Function , Retinal Perforations/diagnostic imaging , Retinal Perforations/etiology , Retinal Telangiectasis/diagnostic imaging , Tomography, Optical Coherence/methods , Visual Acuity/physiology , Visual Field Tests , Visual Fields/physiology , Vitrectomy/methods
PURPOSE: We investigated the molecular causes of an unusual pigmented and ulcerated iris lesion detected in a patient diagnosed with neurofibromatosis type 1 (NF1). CASE REPORT: A 52-year-old man was referred to our clinic with a non-traumatic ulcer in his left eye. Hyphema reabsorption disclosed a pigmented iris mass, thus ultrasound biomicroscopy and anterior segment fluorescein angiography were performed to investigate for the presence of a malignant lesion. Upon angiography, the lesion appeared highly vascularized but prevented posterior iris examination. Therefore, a gonioscopy was executed revealing extension of the lesion into the peripheral iris. Histopathology of the excisional iris biopsy revealed iris melanoma over a dysplastic nevus. NF1 is an autosomal dominant disorder characterized by pigmented cutaneous lesions, multiple skin tumors, and spinal and cranial nerve tumors. Uveal melanoma is the most common primary intraocular malignancy in adults. Up to 92% of cutaneous melanomas occur in patients with dysplastic nevus syndrome. Skin melanomas have been found in 0.1%-5.4% of NF1 patients. In literature, only 18 reports of uveal melanoma have been documented in association with NF1, including three cases of iris melanoma. RESULTS: NF1 gene testing identified a causative mutation in the germline but no loss of the wild-type allele in the iris melanoma. CONCLUSIONS: Occurrence of both diseases in one patient is extremely rare, but the common origin of Schwann cells and melanoblasts suggests a non-casual association. Therefore, we propose that NF1 patients should be screened for nevi, both cutaneous and uveal, for better patients' management.
Dysplastic Nevus Syndrome , Melanoma , Neurofibromatosis 1 , Skin Neoplasms , Dysplastic Nevus Syndrome/diagnosis , Dysplastic Nevus Syndrome/genetics , Humans , Iris/diagnostic imaging , Male , Melanoma/diagnosis , Middle Aged , Neurofibromatosis 1/complications , Neurofibromatosis 1/diagnosis
Background: Despite the high closure rate of large macular hole (LMH) after surgery, visual recovery is often worse than expected. Microperimetric biofeedback can improve visual function in macular pathologies. We evaluated the efficacy of biofeedback on macular function after successful inverted flap technique for LMH. Methods: In this prospective comparative study, 26 patients after LMH surgical closure were enrolled. The whole sample was equally divided into two groups. In Group 1 (trained), patients underwent a double cycle of microperimetric biofeedback, using structured light stimulus plus acoustic tone; in Group 2 (control), patients underwent scheduled visits. We analyzed visual acuity, retinal sensitivity at central 12° (macular sensitivity, MS) and 4° (central macular sensitivity, CMS), and fixation stability over twelve months. Results: Visual acuity improved mainly in the trained group, without any significant differences between the groups (p > 0.05). Only after training did MS significantly improve (p = 0.01). CMS more significantly improved in the trained (p < 0.001) than the control group (p < 0.01) (Group 1 vs. 2, p = 0.004). Only in the trained group did fixation significantly improve (3 months, p ≤ 0.03; 12 months, p ≤ 0.01). An equality test on matched data confirmed a greater significant improvement of CMS (p ≤ 0.02) at all follow-up and fixation (p ≤ 0.02) at last follow-up after training. Conclusion: Microperimetric biofeedback consolidates and increases the improvement of retinal sensitivity and fixation gained after successful inverted flap technique.
BACKGROUND: Inverted Internal Limiting Membrane (ILM)-flap technique would seem to lead to higher closure rate and better visual acuity than traditional procedure with ILM peeling for the treatment of large macular hole (LMH). Visual acuity recovery does not reveal many other functional changes related to surgical approach. Our purpose was to evaluate macular function and morphology over a 1-year follow-up after inverted ILM-flap technique for LMH by using microperimetry in order to predict visual prognosis. METHODS: This study was a prospective unrandomized single-center study. 23 eyes of 22 patients with idiopathic LMH, with a minimum diameter ranging from 400 to 1000 µm, were included. All patients underwent vitrectomy with inverted ILM-flap technique and gas tamponade. We analyzed macular hole closure rate and functional outcomes including best-corrected visual acuity (BCVA), macular sensitivity (MS) at central 12° and central macular sensitivity (CMS) at central 4°, and fixation behavior as bivariate contour ellipse area (BCEA, degrees2) at 68%, 95%, and 99% of fixation points measured by microperimeter, over a follow-up of 12 months. RESULTS: The macular hole closure rate was 98%. The BCVA improved from 20/230 (Logmar, 1.06 ± 0.34) to 20/59 (logMar, 0.47 ± 0.45) at last follow-up (p < 0.001). Retinal sensitivity and BCEA significantly improved (MS, p = 0.001; CMS, p < 0.0001; BCEA: 68%, p < 0.01; 95%, p < 0.01; 99%, p = 0.001). Multiple stepwise regression analysis showed the final BCVA was significantly associated with macular hole size (ß = 0.002, p = 0.03), preoperative MS (ß = - 0.06, p = 0.001) and BCEA at 95% and 99% of fixation points (ß = - 0.12, p = 0.01; ß = 0.06, p = 0.01). CONCLUSIONS: Inverted ILM-flap technique for LMH results in good morphologic and functional outcomes. Macular hole size and microperimetric parameters as preoperative MS and BCEA have a predictive role on post-surgical visual acuity.
