CHORIORETINAL CHANGES IN A GENETICALLY CONFIRMED CASE OF BOUCHER-NEUHÄUSER SYNDROME.

PURPOSE: To describe the retinal findings in a 25-year-old white woman in whom a diagnosis of Boucher-Neuhäuser Syndrome (BNS) was supported by genetic testing, which identified a missense and novel nonsense mutation in the PNPLA6 gene. METHODS: Observational case report of a 25-year-old woman who presented with primary amenorrhea, cerebellar ataxia, and mild retinal pigmentary abnormalities. Neurologic, endocrine, and genetic evaluations established a diagnosis of BNS. RESULTS: Clinical examination and multimodal imaging documented focal outer retinal and retinal pigment epithelium changes including bilateral foveal stippling and a circular area of hypopigmentation in the superior macula of the left eye. Optical coherence tomography showed a linear area of outer retinal attenuation superonasal to the fovea and multiple foci of pinpoint outer retinal defects in the temporal macula of the left eye. Humphrey visual field 24-2 testing showed nonspecific defects in both eyes. Full-field electroretinography showed no evidence of a generalized retinal dysfunction. CONCLUSION: Recognition that the chorioretinal abnormalities occurring in BNS can be rather subtle is essential because the diagnosis of BNS may depend on their detection. To the best of our knowledge, this is the first report in the ophthalmic literature of mild chorioretinal changes in a patient with BNS testing positive for a mutation in the PNPLA6 gene.

Clinical course of acute zonal occult outer retinopathy complicated by choroidal neovascularization.

PURPOSE: To report the clinical course and multimodal imaging features of acute zonal occult outer retinopathy (AZOOR) complicated by choroidal neovascularization (CNV) treated with anti-vascular endothelial growth factor (VEGF) treatment or photodynamic therapy (PDT). METHODS: Observational case series. Retrospective analysis of patients presenting to different institutions with evidence of AZOOR and neovascular lesions. Diagnosis of AZOOR was made on the basis of clinical presentation and multimodal imaging. All patients underwent a comprehensive ophthalmic evaluation and multimodal retinal imaging, including color fundus photos, fundus autofluorescence, fundus fluorescein angiography and spectral-domain optical coherence tomography. RESULTS: Four patients (three males, mean age 53.5 years) were included in the study. Mean follow-up was 5.1 years. Presentation of AZOOR was unilateral in two patients and bilateral in the remainder two patients. One of the patients presenting with unilateral AZOOR developed zonal lesions in the fellow eye during follow-up. All patients presented with unilateral type 2 (subretinal) CNV. Three patients underwent intravitreal anti-VEGF injections and one patient underwent a single PDT. Multimodal retinal imaging showed zonal or multizonal progression during treatment. After treatment, visual acuity and CNV stabilization was observed in all patients. CONCLUSIONS: The presence of CNV expands the clinical spectrum of AZOOR. CNV complicating AZOOR may be effectively treated with intravitreal injections of anti-VEGF, despite progression of the zonal lesions. Further studies are required to define the role of treatment in the progression of the zonal lesions.

Objective and subjective outcomes of strabismus surgery in Graves' orbitopathy: a prospective multicentre study.

PURPOSE: To assess the change and interrelationship of the field of binocular single vision (BSV) and the quality of life (QoL), tested with two different tools, after one or two strabismus surgeries in patients with Graves' orbitopathy (GO). METHODS: Prospectively, consecutive patients with GO who were scheduled for their first strabismus surgery were recruited from five centres specialized in the treatment of GO. One week preoperatively and 3 months after the last operation, a full ophthalmic and orthoptic examination was performed. Change in field of BSV, GO-QoL and thyroid eye disease-QoL (TED-QoL) was recorded. RESULTS: A total of 59 met all the eligibility criteria of whom 15 underwent two strabismus operations. The median (interquartile range) preoperative score of the field of BSV was 0 (0-0), which improved to 73 (53-85) after the correction(s) (p < 0.001). After the first surgery, a significant higher score of the field of BSV was found in the patients who underwent one operation (76; 60-86) compared with those who underwent two 0 (0-63) operations (p < 0.001). After the second surgery, this score increased to 62 (40-76; p = 0.05). A moderate correlation was found between the score of the field of BSV and the GO-QoL visual functioning (VF) questionnaires (r = 0.485; p < 0.001). Both the GO-QoL and TED-QoL for VF and appearance (AP) showed significantly higher scores after the treatment (p < 0.001). CONCLUSION: After strabismus surgery in GO patients, both the field of BSV and quality of life questionnaires increase. In approximately 30% of the patients, an additional strabismus surgery is performed. This second surgery significantly expands the field of BSV and the quality of life.

OCULAR MANIFESTATIONS OF MONOCLONAL IMMUNOGLOBULIN LIGHT CHAIN DEPOSITION DISEASE.

PURPOSE: To demonstrate unusual retinal findings in a patient with progressive renal failure due to idiopathic monoclonal immunoglobulin light chain deposition disease, using multimodal imaging. METHODS: Observational case report of a 43-year-old white man with renal failure due to light chain deposition disease. His course over 6 years was documented with multimodal imaging including fundus photography, fundus autofluorescence, fluorescein angiography, and spectral domain optical coherence tomography. Additional evaluations included ocular ultrasound, electroretinography, positron emission tomography, serum protein electrophoreses, skeletal surveys to detect osteolytic lesions, and renal, liver, and rectal biopsies in search of amyloid. RESULTS: The patient's ocular course mirrored the severity of his renal dysfunction for which he required a renal transplant. Changes observed in the native kidney recurred in the transplant 2 years later, as evidenced by immunohistochemistry, revealing thick linear deposits of kappa chains, with no complement, overlying the glomerular basement membrane. The systemic workup was negative for amyloid but showed an overwhelming ratio of kappa to lambda light chains on serum protein electrophoreses and no clinical signs of plasma cell dyscrasias, all consistent with idiopathic light chain deposition disease. The patient presented with a generalized, bilateral "leopard-spot" fundus appearance on fundus autofluorescence, striking globular subretinal deposits on spectral domain optical coherence tomography, and subfoveal subretinal fluid without retinal pigment epithelium detachment or choroidal effusions. The subfoveal fluid did not respond to intravitreal injections of antiangiogenic agents or steroids but resolved after renal transplantation. A temporary posttransplant visual improvement was associated with lessening of the subretinal drusenoid deposits demonstrated by multimodal imaging. The terminal vision deterioration was associated with amorphous, vitelliform-like material deposition and atrophic changes. CONCLUSION: This case may illustrate a resemblance in the renal glomerulus basement membrane and retinal pigment epithelium-Bruch membrane complex, because the authors observed deposits of excess monoclonal kappa chains manifesting as extracellular, proteinaceous aggregates on the basement membrane of the glomerulus, and striking, globular subretinal deposits that overlay a thickened retinal pigment epithelium-Bruch membrane complex. The ocular lesions' refractoriness to intravitreal treatments could be attributed to the fact that they represent proteinaceous aggregates similar to those documented in the glomeruli. This is the first report of generalized, large, subretinal drusenoid deposits and their course, as documented through multimodal imaging, paralleling the chronology of systemic changes in a patient with light chain deposition disease.

PURTSCHER RETINOPATHY AS A MANIFESTATION OF HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS.

PURPOSE: The authors describe a woman diagnosed with hemophagocytic lymphohistiocytosis and found to have retinal examination findings consistent with Purtscher retinopathy. METHODS: A 52-year-old woman underwent multimodal imaging, including color fundus photography and spectral-domain optical coherence tomography, to confirm the diagnosis. RESULTS: The ophthalmic examination and imaging confirmed the findings of Purtscher retinopathy with significant inner retinal thickening on spectral-domain optical coherence tomography. Throughout a hospital course complicated by multi-organ failure, she continued to have profoundly limited visual acuity, likely resulting from inner retinal ischemia affecting the posterior pole of both eyes. CONCLUSION: The authors describe a patient with hemophagocytic lymphohistiocytosis, a disease characterized by disruption of normal natural killer cell activity with subsequent uncontrolled cytokine release, who presented with Purtscher retinopathy confirmed with spectral-domain optical coherence tomography.

Clinical Characteristics, Choroidal Neovascularization, and Predictors of Visual Outcomes in Acquired Vitelliform Lesions.

PURPOSE: To quantify the temporal properties of the acquired vitelliform lesion (AVL) life cycle, define the clinical characteristics of choroidal neovascularization (NV) in this setting, and determine the predictors of long-term visual outcomes. DESIGN: Retrospective cohort study. METHODS: Clinical and imaging data from 199 eyes of 124 consecutive patients with AVLs associated with age-related macular degeneration (AMD) and adult-onset foveomacular vitelliform dystrophy (AOFVD) were analyzed. Volumetric calculations of vitelliform material were determined using spectral-domain optical coherence tomography and the temporal properties of the AVL life cycle were quantified. The clinical characteristics of NV were assessed, as were the predictors of final best-corrected visual acuity (BCVA) and change in BCVA. RESULTS: Mean age was 79.2 ± 12.1 years. AVLs grew and collapsed at approximately the same rate (P = .275). Fifteen eyes (7.5%) developed NV, of which all were type 1. In 13 of these eyes, NV occurred during the collapse phase of the AVL life cycle, after the peak AVL volume was reached. The risk of NV (P = .006) and the decline in BCVA (P = .001) were both significantly greater among eyes with AMD. Foveal atrophy was the characteristic most significantly associated with final BCVA and change in BCVA from baseline (both P < .0005). The development of NV was not predictive of long-term visual outcomes (all P = .216). CONCLUSIONS: Complications associated with AVLs typically occur during the collapse phase of the AVL life cycle. Visual outcomes and risk of NV are related to the underlying disease associated with AVLs.

Visual Acuity Is Correlated with the Area of the Foveal Avascular Zone in Diabetic Retinopathy and Retinal Vein Occlusion.

PURPOSE: To determine if the area of the foveal avascular zone (FAZ) is correlated with visual acuity (VA) in diabetic retinopathy (DR) and retinal vein occlusion (RVO). DESIGN: Cross-sectional study. PARTICIPANTS: Ninety-five eyes of 66 subjects with DR (65 eyes), branch retinal vein occlusion (19 eyes), and central retinal vein occlusion (11 eyes). METHODS: Structural optical coherence tomography (OCT; Spectralis, Heidelberg Engineering) and OCT angiography (OCTA; Avanti, Optovue RTVue XR) data from a single visit were analyzed. FAZ area, point thickness of central fovea, central 1-mm subfield thickness, the occurrence of intraretinal cysts, ellipsoid zone disruption, and disorganization of retinal inner layers (DRIL) length were measured. VA was also recorded. Correlations between FAZ area and VA were explored using regression models. Main outcome measure was VA. RESULTS: Mean age was 62.9±13.2 years. There was no difference in demographic and OCT-derived anatomic measurements between branch retinal vein occlusion and central retinal vein occlusion groups (all P ≥ 0.058); therefore, data from the 2 groups were pooled together to a single RVO group for further statistical comparisons. Univariate and multiple regression analysis showed that the area of the FAZ was significantly correlated with VA in DR and RVO (all P ≤ 0.003). The relationship between FAZ area and VA varied with age (P = 0.026) such that for a constant FAZ area, an increase in patient age was associated with poorer vision (rise in logarithm of the minimum angle of resolution visual acuity). Disruption of the ellipsoid zone was significantly correlated with VA in univariate and multiple regression analysis (both P < 0.001). Occurrence of intraretinal cysts, DRIL length, and lens status were significantly correlated with VA in the univariate regression analysis (P ≤ 0.018) but not the multiple regression analysis (P ≥ 0.210). Remaining variables evaluated in this study were not predictive of VA (all P ≥ 0.225). CONCLUSIONS: The area of the FAZ is significantly correlated with VA in DR and RVO and this relationship is modulated by patient age. Further study about FAZ area and VA correlations during the natural course of retinal vascular diseases and following treatment is warranted.

A Comparison Between Optical Coherence Tomography Angiography and Fluorescein Angiography for the Imaging of Type 1 Neovascularization.

PURPOSE: To determine the sensitivity of the combination of optical coherence tomography angiography (OCTA) and structural optical coherence tomography (OCT) for detecting type 1 neovascularization (NV) and to determine significant factors that preclude visualization of type 1 NV using OCTA. METHODS: Multicenter, retrospective cohort study of 115 eyes from 100 patients with type 1 NV. A retrospective review of fluorescein (FA), OCT, and OCTA imaging was performed on a consecutive series of eyes with type 1 NV from five institutions. Unmasked graders utilized FA and structural OCT data to determine the diagnosis of type 1 NV. Masked graders evaluated FA data alone, en face OCTA data alone and combined en face OCTA and structural OCT data to determine the presence of type 1 NV. Sensitivity analyses were performed using combined FA and OCT data as the reference standard. RESULTS: A total of 105 eyes were diagnosed with type 1 NV using the reference. Of these, 90 (85.7%) could be detected using en face OCTA and structural OCT. The sensitivities of FA data alone and en face OCTA data alone for visualizing type 1 NV were the same (66.7%). Significant factors that precluded visualization of NV using en face OCTA included the height of pigment epithelial detachment, low signal strength, and treatment-naïve disease (P < 0.05, respectively). CONCLUSIONS: En face OCTA and structural OCT showed better detection of type 1 NV than either FA alone or en face OCTA alone. Combining en face OCTA and structural OCT information may therefore be a useful way to noninvasively diagnose and monitor the treatment of type 1 NV.

LONG-TERM RETROSPECTIVE ANALYSIS OF VISUAL ACUITY AND OPTICAL COHERENCE TOPOGRAPHIC CHANGES AFTER SINGLE VERSUS DOUBLE PEELING DURING VITRECTOMY FOR MACULAR EPIRETINAL MEMBRANES.

PURPOSE: To determine the long-term effect of internal limiting membrane with associated epiretinal membrane (ERM) peeling versus single peeling alone in terms of best-corrected visual acuity and anatomical outcomes on spectral-domain optical coherence tomography. METHODS: This retrospective comparative cohort study of patients who had follow-up of >1 year and underwent surgery for ERM by a single surgeon (S.C.) from January 1, 2008 to December 31, 2012 compared cases in which the internal limiting membrane was stained with brilliant blue G to facilitate double peeling (n = 42) and single peeling (n = 43) of the ERM alone for up to 3 years of follow-up. For continuous variables, an independent two-tailed t-test was performed. For binary variables, the Fisher's exact test was performed. Statistical significance was defined as P < 0.05. RESULTS: Eighty-five of 142 patients fit the inclusion criteria. At the last follow-up, the single-peeling group were more likely to have ERM remaining in the central fovea postoperatively (P = 0.0020, becoming significant by postoperative Year 1, P = 0.022) and less likely to develop inner retinal dimpling (P = 0.000, becoming significant by postoperative Month 3, P = 0.015). At 3 years, central foveal thickness had decreased in the single-peeling group by -136.9 µm and by -84.1 µm in the double-peeling group, which was not significantly different (P = 0.08). Mean best-corrected visual acuity improved in both the groups at all time points. There was no statistically significant difference between the 2 groups at 3 years (P = 0.44; single-peeling group, 0.32 ± 0.42, Snellen 20/42; double-peeling group, 0.23 ± 0.27, Snellen 20/34). CONCLUSION: Brilliant blue G-assisted internal limiting membrane peeling for ERM results in a more thorough removal of residual ERM around the paracentral fovea. However, there is no difference in long-term best-corrected visual acuity at 3 years and a greater likelihood of inner retinal dimpling.

Central Microscotoma: A Rare Presentation of Epiretinal Membranes.

BACKGROUND AND OBJECTIVE: Patients with a symptomatic epiretinal membrane (ERM) typically complain of metamorphopsia and decreased visual acuity. We describe three patients who presented with the single complaint of a central microscotoma due to ERM, an infrequent initial symptom of this entity. PATIENTS AND METHODS: This is a retrospective, interventional, non-comparative case series. Three patients with the chief complaint of a central microscotoma related to ERMs who underwent pars plana vitrectomy by a single surgeon experienced full resolution of the preoperative microscotoma. Best-corrected visual acuity, fundus photography, and optical coherence tomography were obtained at each examination. A comprehensive neuro-ophthalmologic evaluation was performed in all cases, including magnetic resonance imaging of the orbits and brain, automated visual fields, multifocal electroretinography, multifocal visually evoked potentials, and blood tests. RESULTS: Three eyes of three patients who presented with the initial sole complaint of a central microscotoma due to ERM are included in this series. A comprehensive neuro-ophthalmologic evaluation ruled out non-retinal etiologies. After months of observation, a pars plana vitrectomy with ERM and internal limiting membrane peeling was performed in all patients. Postoperatively, the patients experienced a complete resolution of their initial, isolated complaint of a central microscotoma. CONCLUSION: Central microscotoma, as a rare stand-alone presentation of ERM, is described. This symptom resolved after a successful removal of the ERM. A greater awareness among clinicians that ERMs may present in this manner may help avoid excessive and costly medical evaluations.

Documentation of Spontaneous Macular Hole Closure in Macular Telangiectasia Type 2 Using Multimodal Imaging.

Macular holes in the setting of macular telangiectasia type 2 can be difficult to manage. The rates of anatomical closure after macular hole surgery are less favorable in patients with macular telangiectasia than in those with idiopathic macular holes. These differences may be due to the influence of unique pathogenic mechanisms that modulate macular hole dynamics in patients with macular telangiectasia. In this report, the authors document the multimodal imaging findings of a patient with macular telangiectasia demonstrating spontaneous macular hole closure. These findings may improve the understanding of the natural course of this entity and may have relevance for clinical management.

Multimodal imaging documentation of rapid evolution of retinal changes in handheld laser-induced maculopathy.

PURPOSE: To use multimodal imaging to document the relatively rapid clinical evolution of handheld laser-induced maculopathy (HLIM). To demonstrate that inadvertent ocular injury can result from devices mislabeled with respect to their power specifications. METHODS: The clinical course of a 17-year-old male who sustained self-inflicted, central macular damage from a 20-25 s direct stare at a red-spectrum, handheld laser pointer ordered from an internet retailer is provided. Retrospective review of multimodal imaging that includes fundus photography, fluorescein angiography, MultiColor reflectance, eye-tracked spectral domain optical coherence tomography (SD-OCT), fundus autofluorescence, and microperimetry is used to describe the evolving clinical manifestations of HLIM in the first 3 months. RESULTS: Curvilinear bands of dense hyperreflectivity extending from the outer retina and following the Henle fibers were seen on SD-OCT immediately after injury. This characteristic appearance had largely resolved by 2 weeks. There was significant non-uniformity in the morphological characteristics of HLIM lesions between autofluorescence and reflectance images. The pattern of lesion evolution was also significantly different between imaging modalities. Analysis of the laser device showed its wavelength to be correctly listed, but the power was found to be 102.5-105 mW, as opposed to the <5 mW described on the label. CONCLUSION: While the immediate SD-OCT characteristics are highly specific for handheld laser -induced maculopathy, this finding can undergo rapid resolution in the span of several days. In the absence of this finding, other multimodal imaging clues and a careful history may aid in recognizing this diagnosis. A greater awareness regarding inaccurate labeling on some of these devices could help reduce the frequency of this preventable entity.

Type 1 neovascularization may confer resistance to geographic atrophy amongst eyes treated for neovascular age-related macular degeneration.

BACKGROUND: To report a series of age-related macular degeneration (AMD) patients in whom progression to geographic atrophy (GA) in one eye receiving frequent intravitreal injections of anti-vascular endothelial growth factor (anti-VEGF) therapy for type 1 neovascularization (NV) was slower than that of the fellow eye with non-neovascular AMD. METHODS: Retrospective, observational case series examining the clinical course and GA progression rate in four consecutive patients in which one eye harbored type 1 neovascular AMD and was receiving anti-VEGF therapy, while the fellow eye manifested signs of non-neovascular AMD only. Eligibility criteria included anti-VEGF therapy duration of over 4 years and over 50 injections. Lesion evolution was documented via multimodal imaging. GA at baseline and final visits was quantified and GA progression rate for each eye was determined. RESULTS: Four consecutive patients were followed for a mean interval of 94 months (range 62-120). One eye harbored type 1 NV while the fellow eye remained non-neovascular. The former received a mean of 65.5 ± 15.2 anti-VEGF injections. Mean rate of GA progression in non-neovascular eyes was 0.076 ± 0.024 mm2/month and in type 1 NV eyes was 0.004 ± 0.005 mm2/month. Difference in GA progression rate between type 1 and non-neovascular eyes was found to be statistically significant (P = 0.001). CONCLUSIONS: These findings support previous hypotheses that, unlike type 2 and 3 lesions, type 1 NV may represent a neovascular AMD subtype more resilient to GA formation. This may have implications for anti-VEGF regimens in the management of type 1 NV.

Multimodal imaging of combined hamartoma of the retina and retinal pigment epithelium associated with an acquired vitelliform lesion.

BACKGROUND: We present a case of a combined hamartoma of the retina and retinal pigment epithelium associated with a subfoveal acquired vitelliform lesion induced by vitreomacular traction. The purpose of this report is to present a unifying hypothesis of these concurrent findings, as aided by multimodal imaging. CASE PRESENTATION: A 25-year-old white man presented with a 6-month history of a visual disturbance in his left eye. At presentation, ophthalmic assessment showed a combined hamartoma adjacent to his optic nerve that had caused marked corrugation within the inner retinal surface. An acquired vitelliform lesion was present in the macula with an associated epiretinal membrane as demonstrated on spectral-domain optical coherence tomography. Optical coherence tomography angiography corroborated the clinical diagnosis of combined hamartoma. CONCLUSIONS: We are not aware of previous cases of a combined hamartoma associated with an acquired vitelliform lesion. As previously proposed in acquired vitelliform lesions related to epiretinal membrane and vitreoretinal traction, we believe that macular tractional forces might interfere with retinal pigment epithelium phagocytosis of shed outer segments, leading to the occurrence of this acquired vitelliform lesion.

Jak2 mutation-positive polycythemia vera presenting as central retinal artery occlusion.

PURPOSE: To report the case of a 49-year-old man who presented with profound visual loss in his right eye because of a central retinal artery occlusion, accompanied by a stroke in the ipsilateral middle cerebral artery with left upper extremity sequelae. METHODS: The patient underwent a comprehensive ophthalmologic examination, fluorescein angiography, thorough neuroophthalmology evaluation, including magnetic resonance imaging and magnetic resonance angiography, 12-lead electrocardiogram, transesophageal echocardiogram, bilateral carotid artery Doppler, Holter monitoring, comprehensive laboratory testing, bone marrow biopsy, and genetic testing. RESULTS: Despite an initially normal hematocrit, the etiology of the central retinal artery occlusion and that of the nearly concomitant stroke in the right medial cerebral artery territory was confirmed by bone marrow biopsy to be polycythemia vera (positive JAK2 mutation). Patient received treatment with aspirin, serial phlebotomies, and hydroxyurea, demonstrating a slight improvement in vision and substantial normalization of neurologic and hematological status. At the time of this report, 2 years later, patient remains stable. CONCLUSION: Central retinal artery occlusion, a disease with a frequently devastating visual outcome, is often not an "isolated" ocular occurrence. In the absence of an obvious local trigger, a thorough work-up is always warranted, especially in younger patients, as the ocular pathology oftentimes may herald severe cerebro-cardiovascular events.

Aflibercept: a review of its use in the treatment of choroidal neovascularization due to age-related macular degeneration.

Choroidal neovascularization (CNV) due to age-related macular degeneration (AMD) is an important cause of visual morbidity globally. Modern treatment strategies for neovascular AMD achieve regression of CNV by suppressing the activity of key growth factors that mediate angiogenesis. Vascular endothelial growth factor (VEGF) has been the major target of neovascular AMD therapy for almost two decades, and there have been several intravitreally-administered agents that have enabled anatomical restitution and improvement in visual function with continual dosing. Aflibercept (EYLEA(®)), initially named VEGF Trap-eye, is the most recent anti-VEGF agent to be granted US Food and Drug Administration approval for the treatment of neovascular AMD. Biologic advantages of aflibercept include its greater binding affinity for VEGF, a longer intravitreal half-life relative to other anti-VEGF agents, and the capacity to antagonize growth factors other than VEGF. This paper provides an up-to-date summary of the molecular mechanisms mediating CNV. The structural, pharmacodynamic, and pharmacokinetic advantages of aflibercept are also reviewed to rationalize the utility of this agent for treating CNV. Results of landmark clinical investigations, including VIEW 1 and 2 trials, and other important studies are then summarized and used to illustrate the efficacy of aflibercept for managing treatment-naïve CNV, recalcitrant CNV, and CNV due to polypoidal choroidal vasculopathy. Safety profile, patient tolerability, and quality of life measures related to aflibercept are also provided. The evidence provided in this paper suggests aflibercept to be a promising agent that can be used to reduce the treatment burden of neovascular AMD.

Bilateral Orbital Abscesses After Strabismus Surgery.

Infectious orbital complications after strabismus surgery are rare. Their incidence is estimated to be 1 case per 1,100 surgeries and include preseptal cellulitis, orbital cellulitis, subconjunctival and sub-Tenon's abscesses, myositis, and endophthalmitis. This report describes the case of an otherwise healthy 3-year-old boy who underwent bilateral medial rectus recession and disinsertion of the inferior obliques. A few days after surgery, the patient presented with bilateral periorbital edema and inferotemporal chemosis. A series of CT scans with contrast revealed inferotemporal orbital collections OU. The patient immediately underwent transconjunctival drainage of fibrinous and seropurulent collections in the sub-Tenon's space and experienced rapid improvement a few days later. The patient is reported to be in stable condition in a follow-up examination performed more than a year after the reported events.

Optic Nerve Head and Retinal Nerve Fiber Layer Differences Between Caribbean Black and African American Patients as Measured by Spectral Domain OCT.

PURPOSE: There are well-established differences in optic nerve morphology between patients of African and European descent. Spectral domain optical coherence tomography (OCT) scanning has demonstrated these differences with respect to optic disc area (DA), average cup-disc ratio, cup volume, and nerve fiber layer thickness. However, the term "African descent" describes a heterogenous group with considerable variability. This study evaluates differences in optic nerve and retinal nerve fiber layer (RNFL) parameters as measured by Cirrus HD-OCT between Caribbean black and African American patients. DESIGN AND METHODS: A total of 25 African American subjects and 25 Caribbean black subjects with normal ocular examinations were consecutively recruited to this study. All patients received imaging of the optic nerve and nerve fiber layer with Cirrus HD-OCT. Optic nerve and RNFL parameters were evaluated for statistically significant differences using a t test. A mixed effect model for correlated data was then created to adjust outcome variables for (1) repeated measures and (2) optic nerve size. Two one-sided t tests were then utilized to determine equivalence. RESULTS: After adjustment for DA, RNFL thickness, cup volume, DA, inferior nerve fiber layer, and vertical cup-disc ratio demonstrated statistically significant equivalence between the 2 groups (P value <0.05). The superior nerve fiber layer quadrant was significantly different between the 2 groups and may merit further investigation. CONCLUSIONS: Findings of this study suggest that optic nerve and RNFL morphology is markedly similar between Caribbean blacks and African Americans once adjusted for optic nerve size but cannot be considered equivalent in all measures, particularly in the superior nerve fiber layer.

Gestational choriocarcinoma metastasis to the extraocular muscle: a case report.

This case report describes a biopsy-proven metastasis of gestational choriocarcinoma to the medial rectus muscle. Patient evaluation and follow up included comprehensive ophthalmologic history and examination, external and fundus photography, immunohistochemistry preparations of the medial rectus muscle specimen, MRI, ultrasound of the abdomen and pelvis, comprehensive blood tests, and CT scans of the chest, abdomen, and pelvis. The tissue specimen was obtained via a medial perilimbal conjunctival peritomy. MRI revealed a mass intrinsic to the right medial rectus muscle. Immunohistochemical staining confirmed gestational choriocarcinoma metastasis in medial rectus muscle biopsy. The patient showed general and orbital improvement following 7 subsequent cycles of chemotherapy. In conclusion, gestational choriocarcinoma may metastasize to the orbit in addition to the previously reported ocular site, the choroid. A chemotherapy regimen of etoposide, methotrexate, actinomycin-D, cyclophosphamide, and vincristine can effectively treat the intraorbital component of the disease.