@article {1091, title = {Optical Coherence Tomography Angiography of RPGR-Associated Retinitis Pigmentosa Suggests Foveal Avascular Zone is a Biomarker for Vision Loss.}, journal = {Ophthalmic Surg Lasers Imaging Retina}, volume = {50}, year = {2019}, month = {2019 02 01}, pages = {e44-e48}, abstract = {

RPGR-associated retinitis pigmentosa (RP) is a progressive disease with retina degeneration. Optical coherence tomography angiography (OCTA) is an imaging technique that provides novel insights. The authors report two affected male siblings who underwent OCTA imaging. The area of the foveal avascular zone (FAZ) was measured. Although the younger sibling exhibited more advanced clinical disease, his visual acuity was superior to his older sibling. OCTA imaging revealed a better preserved FAZ in the younger sibling as the reason for this. It also highlighted attenuation of choriocapillaris / choroid layers as biomarkers for disease severity. This provides new insights into retinal degeneration in RPGR-associated RP. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:e44-e48.].

}, keywords = {Biomarkers, Capillaries, Choroid, Eye Proteins, Fluorescein Angiography, Fovea Centralis, Humans, Male, Middle Aged, Retinitis Pigmentosa, Tomography, Optical Coherence, Vision Disorders}, issn = {2325-8179}, doi = {10.3928/23258160-20190129-18}, author = {Tang, Peter H and Jauregui, Ruben and Tsang, Stephen H and Bassuk, Alexander G and Mahajan, Vinit B} } @article {645, title = {COMBINED VITRECTOMY AND INTRAVITREAL DEXAMETHASONE (OZURDEX) SUSTAINED-RELEASE IMPLANT.}, journal = {Retina}, volume = {36}, year = {2016}, month = {2016 Nov}, pages = {2087-2092}, abstract = {

PURPOSE: To evaluate the safety and efficacy of combining intravitreal dexamethasone implantation (Ozurdex) with pars plana vitrectomy (PPV).

METHODS: A retrospective review was conducted on cases where Ozurdex injection was performed in the operating room in conjunction with pars plana vitrectomy. Our primary outcome measure was the presence of surgical complications in the perioperative and 3-month postoperative window. We also measured visual acuity, intraocular pressure (IOP), and macular edema at baseline, one, and 3 months after surgery.

RESULTS: Fifteen eyes in 14 cases were reviewed. There were no complications intraoperatively or at 1-month postoperatively. Two patients (2 eyes) with prior retinal detachment developed proliferative vitreoretinopathy and redetachment at 3 months. Visual acuity improved in 7 of 15 eyes, and an average improvement of 2 lines was achieved for the entire cohort. There was no overall change in intraocular pressure although 1 patient developed an increase in intraocular pressure \>5 mmHg. Five of 9 patients with baseline macular edema experienced improvement or resolution at 3 months.

CONCLUSION: Intraoperative Ozurdex in combination with PPV may be safe and effective in treating macular edema caused by many different underlying diseases.

}, keywords = {Adult, Aged, Aged, 80 and over, Combined Modality Therapy, Dexamethasone, Drug Implants, Female, Glucocorticoids, Humans, Intraocular Pressure, Intravitreal Injections, Macular Edema, Male, Middle Aged, Retinal Vein Occlusion, Retrospective Studies, Tomography, Optical Coherence, Uveitis, Posterior, Visual Acuity, Vitrectomy, Wet Macular Degeneration}, issn = {1539-2864}, doi = {10.1097/IAE.0000000000001063}, author = {Zheng, Andrew and Chin, Eric K and Almeida, David R P and Tsang, Stephen H and Mahajan, Vinit B} } @article {701, title = {Macular Hole Closure With Internal Limiting Membrane Abrasion Technique.}, journal = {JAMA Ophthalmol}, volume = {133}, year = {2015}, month = {2015 Jun}, pages = {635-41}, abstract = {

IMPORTANCE: Internal limiting membrane (ILM) abrasion is an alternative surgical technique for successful full-thickness macular hole (MH) repair.

OBJECTIVE: To study the effects of ILM abrasion as an alternative method of MH repair.

DESIGN, SETTING, AND PARTICIPANTS: Retrospective consecutive case series from January 2006 to December 2008. Demographic data and preoperative, intraoperative, and postoperative examination records of all patients were reviewed for patients who underwent ILM abrasion with a diamond-dusted membrane scraper during vitrectomy for MH repair. A total of 100 eyes underwent ILM abrasion as an alternative to traditional ILM peeling.

MAIN OUTCOMES AND MEASURES: Rate of MH closure and visual acuity (VA) outcomes at 3 months after surgery.

RESULTS: Macular hole closure was achieved with a single surgical procedure in 94 of 100 eyes (94.0\%; 95\% CI, 87.4\%-97.8\%). Among all patients, the median preoperative VA was 20/100 (range, 20/30 to hand motions; 25th quartile, 20/60; and 75th quartile, 20/160), and the median postoperative VA at 3 months after surgery was 20/60 (range, 20/20 to hand motions; 25th quartile, 20/40; and 75th quartile, 20/100). Among all patients with stage 2 MHs, 30 of 38 patients (78.9\%) had at least 2 lines of VA gain: 15 of 23 (65.2\%) were phakic, and 15 of 15 (100\%) were pseudophakic. Four of 38 patients (10.5\%) with stage 2 MHs had at least 2 lines of VA loss, and all were phakic. Among all patients with stage 3 or 4 MHs, 42 of 62 (67.7\%) had at least 2 lines of VA gain, of which 30 of 38 (78.9\%) were phakic and 22 of 24 (91.7\%) were pseudophakic. Six of 62 patients (9.7\%) with stage 3 or 4 MHs had at least 2 lines of VA loss: 4 were phakic, and 2 were pseudophakic. In total, 35.0\% (95\% CI, 25.7\%-44.3\%) of patients achieved 20/40 vision or better, and 52.0\% (95\% CI, 42.2\%-61.8\%) of patients achieved 20/50 vision or better.

CONCLUSIONS AND RELEVANCE: Abrasion of the ILM with a diamond-dusted membrane scraper at the time of vitrectomy achieves high rates of MH closure. This technique avoids complete removal of the retinal ILM basement membrane and subjacent tissues and appears to provide MH closure rates similar to those of traditional ILM peeling.

}, keywords = {Adult, Aged, Aged, 80 and over, Basement Membrane, Endotamponade, Female, Fluorocarbons, Humans, Male, Middle Aged, Ophthalmologic Surgical Procedures, Prone Position, Retinal Perforations, Retrospective Studies, Sulfur Hexafluoride, Tomography, Optical Coherence, Visual Acuity, Vitrectomy}, issn = {2168-6173}, doi = {10.1001/jamaophthalmol.2015.204}, author = {Mahajan, Vinit B and Chin, Eric K and Tarantola, Ryan M and Almeida, David R P and Somani, Riz and Boldt, H Culver and Folk, James C and Gehrs, Karen M and Russell, Stephen R} } @article {699, title = {Structural modeling of a novel CAPN5 mutation that causes uveitis and neovascular retinal detachment.}, journal = {PLoS One}, volume = {10}, year = {2015}, month = {2015}, pages = {e0122352}, abstract = {

CAPN5 mutations have been linked to autosomal dominant neovascular inflammatory vitreoretinopathy (ADNIV), a blinding autoimmune eye disease. Here, we link a new CAPN5 mutation to ADNIV and model the three-dimensional structure of the resulting mutant protein. In our study, a kindred with inflammatory vitreoretinopathy was evaluated by clinical eye examinations, DNA sequencing, and protein structural modeling to investigate the disease-causing mutation. Two daughters of an affected mother demonstrated symptoms of stage III ADNIV, with posterior uveitis, cystoid macular edema, intraocular fibrosis, retinal neovascularization, retinal degeneration, and cataract. The women also harbored a novel guanine to thymine (c.750G\>T, p.Lys250Asn) missense mutation in exon 6 of CAPN5, a gene that encodes a calcium-activated cysteine protease, calpain-5. Modeling based on the structures of all known calpains revealed the mutation falls within a calcium-sensitive flexible gating loop that controls access to the catalytic groove. Three-dimensional modeling placed the new mutation in a region adjacent to two previously identified disease-causing mutations, all three of which likely disrupt hydrogen bonding within the gating loop, yielding a CAPN5 with altered enzymatic activity. This is the third case of a CAPN5 mutation leading to inherited uveitis and neovascular vitreoretinopathy, suggesting patients with ADNIV features should be tested for CAPN5 mutations. Structural modeling of novel variants can be used to support mechanistic consequences of the disease-causing variants.

}, keywords = {Base Sequence, Calpain, Computational Biology, DNA Primers, Female, Fluorescein Angiography, Humans, Models, Molecular, Molecular Sequence Data, Mutation, Missense, Pedigree, Phenotype, Protein Conformation, Retinal Detachment, Sequence Analysis, DNA, Tomography, Optical Coherence, Uveitis, Vitreoretinopathy, Proliferative}, issn = {1932-6203}, doi = {10.1371/journal.pone.0122352}, author = {Bassuk, Alexander G and Yeh, Steven and Wu, Shu and Martin, Daniel F and Tsang, Stephen H and Gakhar, Lokesh and Mahajan, Vinit B} } @article {725, title = {Decreased macular thickness in nonproliferative macular telangiectasia type 2 with oral carbonic anhydrase inhibitors.}, journal = {Retina}, volume = {34}, year = {2014}, month = {2014 Jul}, pages = {1400-6}, abstract = {

PURPOSE: To evaluate whether carbonic anhydrase inhibitors reduce the macular thickness and/or cystic spaces in patients with macular telangiectasia (MacTel) Type 2.

METHODS: Retrospective review of patients with nonproliferative cystoid changes associated with MacTel seen at the University of Iowa between 2009 and 2012. Carbonic anhydrase inhibitors were used in 8 patients with MacTel Type 2. Five patients with MacTel Type 2 were observed during this period. Initial and final visual acuities were documented. The presence of cystic spaces and the retinal thickness were measured with spectral-domain optical coherence tomography.

RESULTS: Patients treated with oral carbonic anhydrase inhibitors showed significant reduction in both the cystoid cavities and central macular thickness when compared with the patients who were observed (-12.2 μm; P = 0.020). The reduction in retinal thickness was more pronounced in patients receiving acetazolamide (-20.13 μm; P = 0.007) compared with methazolamide (-6.25 μm; P = 0.177). There was no significant change in visual acuity in patients receiving carbonic anhydrase inhibitors. Five patients with MacTel Type 2 did not receive treatment and demonstrated no change in visual acuity, cystoid cavities, or central macular thickness.

CONCLUSION: Oral carbonic anhydrase inhibitors, particularly acetazolamide, may decrease macular cystic cavities and reduce central macular thickness but does not appear to improve visual acuity. These findings have yet to be confirmed with a prospective treatment trial.

}, keywords = {Acetazolamide, Administration, Oral, Adult, Aged, Carbonic Anhydrase Inhibitors, Female, Fluorescein Angiography, Humans, Male, Methazolamide, Middle Aged, Organ Size, Retina, Retinal Telangiectasis, Retrospective Studies, Tomography, Optical Coherence, Visual Acuity}, issn = {1539-2864}, doi = {10.1097/IAE.0000000000000093}, author = {Chen, John J and Sohn, Elliott H and Folk, James C and Mahajan, Vinit B and Kay, Christine N and Boldt, H Culver and Russell, Stephen R} } @article {745, title = {Aflibercept therapy for exudative age-related macular degeneration resistant to bevacizumab and ranibizumab.}, journal = {Am J Ophthalmol}, volume = {156}, year = {2013}, month = {2013 Jul}, pages = {15-22.e1}, abstract = {

PURPOSE: To evaluate the outcome of intravitreal injection of aflibercept in cases with exudative age-related macular degeneration, (AMD) resistant to injections of bevacizumab or ranibizumab.

DESIGN: Retrospective observational case series.

METHODS: A retrospective chart review at a single institution was conducted to identify patients with exudative AMD and choroidal neovascularization (CNV) in 1 or both eyes resistant to treatment with ranibizumab or bevacizumab who were switched to treatment with at least 3~monthly injections of aflibercept. In total, 36 eyes from 31 patients were included. The demographic data, visual acuities, central macular thickness on optical coherence tomography (OCT), complications, and number of injections were reviewed.

RESULTS: The mean patient age was 79 years (range 60-88). There were 13 male and 18 female patients. The number of prior injections with either bevacizumab or ranibizumab ranged from 6-74. After 3~monthly injections of aflibercept, there was a reduction of either subretinal or intraretinal fluid in 18 of 36 (50.0\%) of the treated eyes; the amount of fluid remained stable in 15 eyes (41.7\%) and worsened in 3 eyes (8.3\%). A significant average decrease was observed for the central macular thickness after 3 injections of 65~μm (P~= 2.9~{\texttimes} 10(-6)), with no significant change in visual acuity.

CONCLUSIONS: Aflibercept therapy appears to be beneficial in a subset of patients with neovascular age-related macular degeneration who exhibit recurrent or resistant intraretinal or subretinal fluid following multiple injections with either bevacizumab or ranibizumab.

}, keywords = {Aged, Aged, 80 and over, Angiogenesis Inhibitors, Antibodies, Monoclonal, Humanized, Bevacizumab, Drug Resistance, Drug Substitution, Exudates and Transudates, Female, Humans, Intravitreal Injections, Male, Middle Aged, Postoperative Complications, Ranibizumab, Receptors, Vascular Endothelial Growth Factor, Recombinant Fusion Proteins, Retina, Retreatment, Retrospective Studies, Subretinal Fluid, Tomography, Optical Coherence, Treatment Outcome, Vascular Endothelial Growth Factor A, Visual Acuity, Wet Macular Degeneration}, issn = {1879-1891}, doi = {10.1016/j.ajo.2013.02.017}, author = {Bakall, Benjamin and Folk, James C and Boldt, H Culver and Sohn, Elliott H and Stone, Edwin M and Russell, Stephen R and Mahajan, Vinit B} } @article {767, title = {Intraoperative sclerotomy-related retinal breaks during 23-gauge pars plana vitrectomy.}, journal = {Retina}, volume = {33}, year = {2013}, month = {2013 Jan}, pages = {136-42}, abstract = {

PURPOSE: To study the incidence and characteristics of intraoperative sclerotomy-related retinal breaks encountered during 23-gauge pars plana vitrectomy.

METHODS: A retrospective consecutive case series was assembled from the surgical logs and charts of patients who underwent 23-gauge pars plana vitrectomy. Demographic data and preoperative, intraoperative, and postoperative records were examined.

RESULTS: A total 548 eyes met the inclusion criteria. Of them, 145 eyes underwent pars plana vitrectomy for repair of a rhegmatogenous retinal detachment (RRD) and 403 eyes for other indications. Sclerotomy-related retinal breaks were found in 8 of 548 (1.45\%) eyes. No breaks were found in the 145 RRD eyes. In non-RRD cases, 8 of 403 (1.98\%) eyes had sclerotomy-related breaks. All breaks were adjacent to the superior sclerotomies. The incidence of postoperative retinal detachment was 0\% (0 of 403) in the non-RRD group. In eyes with breaks, the primary surgical indication was vitreomacular traction in six of eight eyes and epiretinal membrane in two of eight eyes. Posterior vitreous detachment was absent in six of eight eyes, and six of eight eyes were phakic. Eyes with vitreomacular traction had a significantly higher incidence of breaks (P < 0.0001). Eyes with a surgical indication other than RRD had a higher incidence of breaks, but this was not statistically significant when compared with eyes with RRD (P = 0.087).

CONCLUSION: Pars plana vitrectomy (23-gauge) is associated with a low incidence of sclerotomy-related retinal breaks and postoperative retinal detachments. Eyes with breaks are more likely to be phakic and without a preoperative posterior vitreous detachment. The presence of vitreomacular traction may be a risk factor for the development of intraoperative sclerotomy-related breaks.

}, keywords = {Aged, Aged, 80 and over, Female, Follow-Up Studies, Humans, Incidence, Intraoperative Complications, Male, Microsurgery, Middle Aged, Postoperative Complications, Retinal Detachment, Retinal Perforations, Retrospective Studies, Sclerostomy, Tomography, Optical Coherence, Visual Acuity, Vitrectomy}, issn = {1539-2864}, doi = {10.1097/IAE.0b013e31825e1d62}, author = {Tarantola, Ryan M and Tsui, Janet Y and Graff, Jordan M and Russell, Stephen R and Boldt, H Culver and Folk, James C and Mahajan, Vinit B} } @article {775, title = {Infrared imaging and optical coherence tomography reveal early-stage astrocytic hamartomas not detectable by fundoscopy.}, journal = {Am J Ophthalmol}, volume = {153}, year = {2012}, month = {2012 May}, pages = {883-889.e2}, abstract = {

PURPOSE: To describe and correlate the features of astrocytic hamartomas using multimodal imaging.

DESIGN: Prospective, noncomparative, observational case series.

METHODS: This was a single-center study of 4 patients (8 eyes) with tuberous sclerosis complex. A complete ophthalmologic examination, fundus photography, fundus autofluorescence (FAF), infrared imaging, and spectral-domain optical coherence tomography (SD-OCT) were performed for each patient. Images from each modality were analyzed and compared.

RESULTS: In 2 patients, infrared imaging and SD-OCT detected occult retinal astrocytic hamartomas that were not observed on clinical examination or color fundus photography. FAF demonstrated the greatest contrast between lesions and surrounding retina but failed to identify 1 occult lesion that was detected with infrared imaging and SD-OCT. SD-OCT revealed lesions arising from the retinal nerve fiber layer with overlying vitreous adhesions, hyperreflective dots, and optically empty spaces at all depths of the tumor. Hamartomas were hyporeflective on infrared imaging and hypoautofluorescent on FAF. FAF of some lesions demonstrated hyperautofluorescent spots.

CONCLUSIONS: Infrared imaging and SD-OCT aid in the detection of astrocytic hamartomas that are not visible on clinical examination or color fundus photography. SD-OCT enhances visualization of structural details. FAF is a useful adjunctive test to obtain greater contrast between lesions and surrounding retina. The ability to monitor structural changes over time in astrocytic hamartomas using SD-OCT may be beneficial for monitoring the success of systemic chemotherapy in the treatment of various tuberous sclerosis tumors.

}, keywords = {Adolescent, Astrocytes, Child, Diagnostic Imaging, Female, Fluorescein Angiography, Humans, Infrared Rays, Male, Ophthalmoscopes, Prospective Studies, Retinal Neoplasms, Tomography, Optical Coherence, Tuberous Sclerosis}, issn = {1879-1891}, doi = {10.1016/j.ajo.2011.10.033}, author = {Xu, Luna and Burke, Tomas R and Greenberg, Jonathan P and Mahajan, Vinit B and Tsang, Stephen H} } @article {765, title = {Intravitreal bevacizumab for peripapillary choroidal neovascular membranes.}, journal = {Arch Ophthalmol}, volume = {130}, year = {2012}, month = {2012 Aug}, pages = {1073-5}, keywords = {Adult, Aged, Aged, 80 and over, Angiogenesis Inhibitors, Antibodies, Monoclonal, Humanized, Bevacizumab, Choroidal Neovascularization, Female, Fluorescein Angiography, Humans, Intravitreal Injections, Macular Degeneration, Male, Middle Aged, Retreatment, Subretinal Fluid, Tomography, Optical Coherence, Treatment Outcome, Vascular Endothelial Growth Factor A, Visual Acuity}, issn = {1538-3601}, doi = {10.1001/archophthalmol.2012.465}, author = {Davis, Andrew S and Folk, James C and Russell, Stephen R and Sohn, Elliott H and Sohn, Elliot H and Boldt, H Culver and Stone, Edwin M and Mahajan, Vinit B} } @article {161, title = {Intravitreal bevacizumab during pregnancy.}, journal = {Retina (Philadelphia, Pa.)}, volume = {30}, year = {2010}, month = {2010 Oct}, pages = {1405-11}, abstract = {

To report the clinical course of four women treated with intravitreal bevacizumab during pregnancy.

}, keywords = {Adult, Angiogenesis Inhibitors, Antibodies, Monoclonal, Choroidal Neovascularization, Eye Infections, Fungal, Female, Follow-Up Studies, Gestational Age, Histoplasmosis, Humans, Intravitreal Injections, Off-Label Use, Pregnancy, Pregnancy Complications, Sarcoidosis, Tomography, Optical Coherence, Uveitis, Vascular Endothelial Growth Factor A, Visual Acuity}, author = {Tarantola, Ryan M and Folk, James C and Boldt, H Culver and Mahajan, Vinit B} } @article {163, title = {Patients with an acute zonal occult outer retinopathy-like illness rapidly improve with valacyclovir treatment.}, journal = {American journal of ophthalmology}, volume = {150}, year = {2010}, month = {2010 Oct}, pages = {511-8}, abstract = {

To describe 3 cases of an acute zonal occult outer retinopathy-like illness responsive to valacyclovir hydrochloride.

}, keywords = {Acute Disease, Acyclovir, Adolescent, Adult, Antiviral Agents, Electroretinography, Eye Infections, Viral, Female, Herpesviridae Infections, Humans, Male, Retinal Diseases, Retrospective Studies, Scotoma, Tomography, Optical Coherence, Valine, Visual Field Tests, Visual Fields}, author = {Mahajan, Vinit B and Stone, Edwin M} } @article {173, title = {A new macular dystrophy with anomalous vascular development, pigment spots, cystic spaces, and neovascularization.}, journal = {Archives of ophthalmology}, volume = {127}, year = {2009}, month = {2009 Nov}, pages = {1449-57}, abstract = {

To clinically phenotype an inherited macular dystrophy with peculiar intraretinal pigment spots, cysts, and hemorrhage in a 24-year-old female proband and her family.

}, keywords = {Adolescent, Adult, Amblyopia, Child, Child, Preschool, Cysts, Electroretinography, Female, Fluorescein Angiography, Humans, Macular Degeneration, Male, Middle Aged, Pedigree, Phenotype, Retinal Hemorrhage, Retinal Neovascularization, Retinal Pigment Epithelium, Retinal Vessels, Strabismus, Tomography, Optical Coherence, Visual Acuity, Young Adult}, author = {Mahajan, Vinit B and Russell, Stephen R and Stone, Edwin M} }