Optical coherence tomography-based consensus definition for lamellar macular hole.

BackgroundA consensus on an optical coherence tomography definition of lamellar macular hole (LMH) and similar conditions is needed.MethodsThe panel reviewed relevant peer-reviewed literature to reach an accord on LMH definition and to differentiate LMH from other similar conditions.ResultsThe panel reached a consensus on the definition of three clinical entities: LMH, epiretinal membrane (ERM) foveoschisis and macular pseudohole (MPH). LMH definition is based on three mandatory criteria and three optional anatomical features. The three mandatory criteria are the presence of irregular foveal contour, the presence of a foveal cavity with undermined edges and the apparent loss of foveal tissue. Optional anatomical features include the presence of epiretinal proliferation, the presence of a central foveal bump and the disruption of the ellipsoid zone. ERM foveoschisis definition is based on two mandatory criteria: the presence of ERM and the presence of schisis at the level of Henle's fibre layer. Three optional anatomical features can also be present: the presence of microcystoid spaces in the inner nuclear layer (INL), an increase of retinal thickness and the presence of retinal wrinkling. MPH definition is based on three mandatory criteria and two optional anatomical features. Mandatory criteria include the presence of a foveal sparing ERM, the presence of a steepened foveal profile and an increased central retinal thickness. Optional anatomical features are the presence of microcystoid spaces in the INL and a normal retinal thickness.ConclusionsThe use of the proposed definitions may provide uniform language for clinicians and future research

A consensus definition for lamellar macular hole

Background: A consensus on an optical coherence tomography (OCT) definition of lamellar macular hole (LMH) and similar conditions is needed. Methods: The panel reviewed relevant peer-reviewed literature to reach a consensus on LMH definition and to differentiate LMH from other similar conditions. Results: The panel reached a consensus on the definition of 3 clinical entities: LMH, epiretinal membrane (ERM) foveoschisis and macular pseudohole (MPH). LMH definition is based on 3 mandatory criteria and 3 optional anatomical features. The 3 mandatory criteria are the presence of irregular foveal contour, the presence of a foveal cavity with undermined edges, and the apparent loss of foveal tissue. Optional anatomical features include the presence of epiretinal proliferation, the presence of a foveal bump and the disruption of the ellipsoid line. ERM foveoschisis definition is based on 2 mandatory criteria: the presence of ERM and the presence of schisis at the level of Henle’s fiber layer. Three optional anatomical features can also be present: the presence of microcystoid spaces in the inner nuclear layer (INL), an increase of retinal thickness, and the presence of retinal wrinkling. MPH definition is based on 3 mandatory criteria and 2 optional anatomical features. Mandatory criteria include the presence of a foveal sparing ERM, the presence of a steepened foveal profile and an increased central retinal thickness. Optional anatomical features are the presence of microcystoid spaces in the INL and a normal retinal thickness. Conclusions: The use of the proposed definitions may provide uniform language for clinicians and future research

Autosomal-dominant familial hematuria with retinal arteriolar tortuosity and contractures: A novel syndrome

Autosomal-dominant familial hematuria with retinal arteriolar tortuosity and contractures: A novel syndrome.BackgroundAutosomal-dominant forms of hematuria have been mostly related to mutations in the COL4A3/COL4A4 genes. Patients with thin basement membrane (BM) disease do not have extrarenal manifestations, while those with Alport syndrome often present with hearing loss, anterior lenticonus, and dot-and-fleck retinopathy.MethodsWe performed a phenotypic study and a candidate gene approach in a four-generation family presenting with autosomal-dominant hematuria associated with extrarenal manifestations. Renal biopsy was analyzed for determination of BM thickness and expression of chains of type IV collagen. Linkage to 18 candidate genes/loci was investigated using polymorphic microsatellite markers.ResultsIn all affected patients, hematuria without proteinuria was associated with muscular contractures and retinal arterial tortuosities responsible for retinal hemorrhages. Cardiac arrythmia, Raynaud phenomena, and brain MRI abnormalities were also observed. Despite the presence of red cells in tubule sections, no glomerular abnormalities were found by electron microscopy. Expression of type IV collagen chains and glomerular BM thickness was normal. We searched for a molecular defect affecting either BM or angiogenesis. Linkage analyses of genes encoding BM components (COL4A3/COL4A4, COL6A1, COL6A2, COL6A3, FBLN1), and angiogenic factors or their receptors (VHL, ANPT1, ANPT2, TIE, TEK, NOTCH2, NOTCH3, NOTCH4, DLL4, JAG1, JAG2) and of the facio-sapulo-humeral dystrophy and 3q21 loci failed to show segregation of the disease with those gene loci.ConclusionWe have identified a new inherited hematuria syndrome associated with retinal vessel tortuosities and contractures. We recommend performing a fundus examination in patients with familial hematuria and episodes of visual impairment, as well as a urinary analysis in patients with retinal arterial tortuosity or congenital muscular contractures

SPIRONOLACTONE FOR NONRESOLVING CENTRAL SEROUS CHORIORETINOPATHY: A RANDOMIZED CONTROLLED CROSSOVER STUDY.

PURPOSE: To evaluate the effect of spironolactone, a mineralocorticoid receptor antagonist, for nonresolving central serous chorioretinopathy. METHODS: This is a prospective, randomized, double-blinded, placebo-controlled crossover study. Sixteen eyes of 16 patients with central serous chorioretinopathy and persistent subretinal fluid (SRF) for at least 3 months were enrolled. Patients were randomized to receive either spironolactone 50 mg or placebo once a day for 30 days, followed by a washout period of 1 week and then crossed over to either placebo or spironolactone for another 30 days. The primary outcome measure was the changes from baseline in SRF thickness at the apex of the serous retinal detachment. Secondary outcomes included subfoveal choroidal thickness and the ETDRS best-corrected visual acuity. RESULTS: The mean duration of central serous chorioretinopathy before enrollment in study eyes was 10 ± 16.9 months. Crossover data analysis showed a statistically significant reduction in SRF in spironolactone treated eyes as compared with the same eyes under placebo (P = 0.04). Secondary analysis on the first period (Day 0-Day 30) showed a significant reduction in subfoveal choroidal thickness in treated eyes as compared with placebo (P = 0.02). No significant changes were observed in the best-corrected visual acuity. There were no complications related to treatment observed. CONCLUSION: In eyes with persistent SRF due to central serous chorioretinopathy, spironolactone significantly reduced both the SRF and the subfoveal choroidal thickness as compared with placebo

Acute Lymphoblastic Leukemia Manifesting as Acute Vogt-Koyanagi-Harada Disease

We describe a case of bilateral exudative retinal detachment associated with prodromal symptoms simulating the presentation of acute Vogt-Koyanagi-Harada disease that was eventually diagnosed as acute lymphoblastic leukemia. A 42-year-old man presented with sudden visual loss in both eyes for two weeks. He complained of intermittent headache, neck stiffness and tinnitus for a month. His best-corrected visual acuities were 20/200 in both eyes. Fluorescein angiography, optical coherence topography and indocyanine green angiography featured bilateral serous retinal detachments. A clinical diagnosis of incomplete type Vogt-Koyanagi-Harada disease was considered. However, complete blood cell count showed a marked increase in the number of white blood cells and bone marrow examination revealed precursor B cell lymphoblastic leukemia. The patient started on induction chemotherapy. A week later, his best-corrected visual acuities were 20/25 and the serous retinal detachments were nearly absorbed in both eyes. Bilateral exudative retinal detachment associated with neurologic and auditory abnormalities may be a presenting sign of acute lymphoblastic leukemia. Clinicians should be aware of the possibility of leukemia in such patients

Parameter estimation to study the immediate impact of aortic cross-clamping using reduced order models

Aortic cross-clamping is a common strategy during vascular surgery, however, its instantaneous impact on hemodynamics is unknown. We, therefore, developed two numerical models to estimate the immediate impact of aortic clamping on the vascular properties. To assess the validity of the models, we recorded continuous invasive pressure signals during abdominal aneurysm repair surgery, immediately before and after clamping. The first model is a zero-dimensional (0D) three-element Windkessel model, which we coupled to a gradient-based parameter estimation algorithm to identify patient-specific parameters such as vascular resistance and compliance. We found a 10% increase in the total resistance and a 20% decrease in the total compliance after clamping. The second model is a nine-artery network corresponding to an average human body in which we solved the one-dimensional (1D) blood flow equations. With a similar parameter estimation method and using the results from the 0D model, we identified the resistance boundary conditions of the 1D network. Determining the patient-specific total resistance and the distribution of peripheral resistances through the parameter estimation process was sufficient for the 1D model to accurately reproduce the impact of clamping on the pressure waveform. Both models gave an accurate description of the pressure wave and had a high correlation (R2 >.95) with experimental blood pressure data.Fil: Ventre, Jeanne. Centre National de la Recherche Scientifique; FranciaFil: Politi, Teresa. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Fisiología y Biofísica Bernardo Houssay. Universidad de Buenos Aires. Facultad de Medicina. Instituto de Fisiología y Biofísica Bernardo Houssay; ArgentinaFil: Fernández, Juan M.. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Fisiología y Biofísica Bernardo Houssay. Universidad de Buenos Aires. Facultad de Medicina. Instituto de Fisiología y Biofísica Bernardo Houssay; ArgentinaFil: Ghigo, Arthur R.. Université de Toulouse; FranciaFil: Gaudric, Julien. Centre National de la Recherche Scientifique; Francia. Université de Toulouse; FranciaFil: Wray, Sandra. Universidad Favaloro; ArgentinaFil: Lagaert, Jean Baptiste. Université Paris Sud; FranciaFil: Armentano, Ricardo Luis. Universidad de la República; UruguayFil: Capurro, Claudia Graciela. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Fisiología y Biofísica Bernardo Houssay. Universidad de Buenos Aires. Facultad de Medicina. Instituto de Fisiología y Biofísica Bernardo Houssay; ArgentinaFil: Fullana, José Maria. Centre National de la Recherche Scientifique; FranciaFil: Lagrée, Pierre Yves. Centre National de la Recherche Scientifique; Franci

Macular Telangiectasia Type 2: A Classification System Using MultiModal Imaging MacTel Project Report Number 10

Purpose: To develop a severity classification for macular telangiectasia type 2 (MacTel) disease using multimodal imaging. Design: An algorithm was used on data from a prospective natural history study of MacTel for classification development. Subjects: A total of 1733 participants enrolled in an international natural history study of MacTel. Methods: The Classification and Regression Trees (CART), a predictive nonparametric algorithm used in machine learning, analyzed the features of the multimodal imaging important for the development of a classification, including reading center gradings of the following digital images: stereoscopic color and red-free fundus photographs, fluorescein angiographic images, fundus autofluorescence images, and spectral-domain (SD)-OCT images. Regression models that used least square method created a decision tree using features of the ocular images into different categories of disease severity. Main Outcome Measures: The primary target of interest for the algorithm development by CART was the change in best-corrected visual acuity (BCVA) at baseline for the right and left eyes. These analyses using the algorithm were repeated for the BCVA obtained at the last study visit of the natural history study for the right and left eyes. Results: The CART analyses demonstrated 3 important features from the multimodal imaging for the classification: OCT hyper-reflectivity, pigment, and ellipsoid zone loss. By combining these 3 features (as absent, present, noncentral involvement, and central involvement of the macula), a 7-step scale was created, ranging from excellent to poor visual acuity. At grade 0, 3 features are not present. At the most severe grade, pigment and exudative neovascularization are present. To further validate the classification, using the Generalized Estimating Equation regression models, analyses for the annual relative risk of progression over a period of 5 years for vision loss and for progression along the scale were performed. Conclusions: This analysis using the data from current imaging modalities in participants followed in the MacTel natural history study informed a classification for MacTel disease severity featuring variables from SD-OCT. This classification is designed to provide better communications to other clinicians, researchers, and patients. Financial Disclosure(s): Proprietary or commercial disclosure may be found after the references

Bilateral macular hole formation secondary to sclopetaria caused by shockwaves transmitted by a posterior vector: case report

<p>Abstract</p> <p>Background</p> <p>Sclopetaria is a rare ophthalmic finding in trauma</p> <p>Case Presentation</p> <p>This is a report of a patient who developed macular holes from sclopetaria induced by indirect trauma. A 22-year-old male, suffered a gunshot wound that passed behind his eyes, resulting in bilateral macular hole formation</p> <p>Conclusion</p> <p>To our knowledge, this is the first reported case in which trauma posterior to the globes caused bilateral macular hole formation</p