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   2021| October-December  | Volume 11 | Issue 4  
    Online since December 9, 2021

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Retinal cell transplantation in retinitis pigmentosa
Tongalp H Tezel, Adam Ruff
October-December 2021, 11(4):336-347
Retinitis pigmentosa is the most common hereditary retinal disease. Dietary supplements, neuroprotective agents, cytokines, and lately, prosthetic devices, gene therapy, and optogenetics have been employed to slow down the retinal degeneration or improve light perception. Completing retinal circuitry by transplanting photoreceptors has always been an appealing idea in retinitis pigmentosa. Recent developments in stem cell technology, retinal imaging techniques, tissue engineering, and transplantation techniques have brought us closer to accomplish this goal. The eye is an ideal organ for cell transplantation due to a low number of cells required to restore vision, availability of safe surgical and imaging techniques to transplant and track the cells in vivo, and partial immune privilege provided by the subretinal space. Human embryonic stem cells, induced pluripotential stem cells, and especially retinal organoids provide an adequate number of cells at a desired developmental stage which may maximize integration of the graft to host retina. However, stem cells must be manufactured under strict good manufacturing practice protocols due to known tumorigenicity as well as possible genetic and epigenetic stabilities that may pose a danger to the recipient. Immune compatibility of stem cells still stands as a problem for their widespread use for retinitis pigmentosa. Transplantation of stem cells from different sources revealed that some of the transplanted cells may not integrate the host retina but slow down the retinal degeneration through paracrine mechanisms. Discovery of a similar paracrine mechanism has recently opened a new therapeutic path for reversing the cone dormancy and restoring the sight in retinitis pigmentosa.
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Gene therapy for retinitis pigmentosa
Niloofar Piri, Jacob D Grodsky, Henry J Kaplan
October-December 2021, 11(4):348-351
Rhodopsin-mediated autosomal dominant retinitis pigmentosa (RP) is the most common cause of RP in North America. There is no proven cure for the disease, and multiple approaches are being studied. Gene therapy is an evolving field in medicine and ophthalmology. In this review, we will go over the basic concept of gene therapy and the different types of gene therapy that are currently being studied to treat this disease.
  3,114 546 -
Optical-coherence tomography angiography and ultrawide-field angiography findings in eyes with refractory macular edema secondary to retinal vein occlusion switched to aflibercept: A subanalysis from a 48-week prospective study
Kimberly Spooner, Samantha Fraser-Bell, Thomas Hong, Andrew Chang
October-December 2021, 11(4):352-358
PURPOSE: To evaluate anatomical changes on ultra-wide-field fluorescein-angiography and optical coherence angiography (OCT-A) among a cohort with treatment-resistant macular edema secondary to retinal vein occlusions (RVO) switched to aflibercept. MATERIALS AND METHODS: Patients with persistent macular edema despite previous bevacizumab and/or ranibizumab were switched to aflibercept in a 48-week prospective trial. Ultra-wide-field fluorescein angiography (UWFFA) and OCT-A were performed at baseline, week-24 and week-48. The ischemic index was calculated from UWFFA and the areas of vascular perfusion. The foveal avascular zone (FAZ) were quantitatively evaluated on OCT-A. RESULTS: Eighteen patients (mean age, 70.3±8.6 years) were recruited. Mean central macular thickness (CMT) was significantly reduced at 48-weeks compared to baseline (-87.6±48.8 μm, P < 0.001 and -191.0±128.3μm, P < 0.001 among BRVO and CRVO eyes, respectively). The mean baseline ischemic index as measured on Optos wide-field angiography was 10.9%±8.3 and decreased to 5.7%±4.2 (P = 0.028), at week 48. The mean FAZ areas of the SCP and DCP reduced by -0.06 ± 0.12 mm 2 and -0.17± 0.45 mm 2 , respectively. FAZ area on OCT-A was stable in eyes with stable or improved vision but increased in size in eyes with baseline macular ischemia and those with lower gains in BCVA at week 48 (R 2 =0.719, P = 0.05 and R 2 =0.516, P = 0.01). CONCLUSION: There was a reduction in macular edema measured on OCT at 48-weeks in eyes switched to aflibercept with chronic macular edema due to retinal vein occlusion. There was also a reduction in retinal ischemia as measured using UWFFA.
  3,254 308 -
Retinopathy of prematurity in Saudi Arabia: Exploring maternal risk factors
Nooran Badeeb, Lina Raffa, Huda AhmedHussain, Dania Bamefleh, Enas Mgharbil, Sarah Alessa, Aliaa Alamri, Mohamed Albahiti
October-December 2021, 11(4):359-366
PURPOSE: Retinopathy of prematurity (ROP) is a preventable blinding disorder affecting preterm infants. To date, maternal risk factors have not been studied in Saudi Arabia. This study aims to identify possible maternal risk factors for any stage and type 1 ROP. MATERIALS AND METHODS: A total of 295 preterm infants screened for ROP between November 2013 and December 2018 at a Saudi Arabian tertiary-care hospital were included. We included infants with a gestational age ≤ 32 weeks and/or birth weight (BW) ≤1500 g. We analyzed 28 maternal and neonatal risk factors. RESULTS: The incidence of ROP at any stage and Type 1 were 31.9% and 7%, respectively. In the univariate analysis, the only maternal factor associated with any stage of ROP was spontaneous vaginal delivery (P = 0.049), but no maternal factor was an independent risk factor for type 1 ROP. Multivariate logistic regression analysis identified lower BW, lower gestational age and longer neonatal intensive care unit stay as independent risk factors for the development of ROP at any stage (P < 0.05). For Type 1 ROP, lower BW, and intraventricular hemorrhage were significant independent risk factors (P < 0.05). CONCLUSION: The only maternal risk factor related to ROP was spontaneous vaginal delivery, which increased the risk of any stage of ROP. The single most predictive risk factor for any stage of ROP and Type 1 ROP was low BW. These findings emphasize the role of the obstetrician in promoting health care and modifying maternal risk factors to prevent preterm births related to a low BW.
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Transient cortical blindness secondary to hepatic encephalopathy in a pediatric patient: A case report and literature review
Ahmad Sukari Ain-Nasyrah, Norizan Abdul Majid, Ismail Shatriah
October-December 2021, 11(4):413-416
Cortical blindness, also known as cerebral visual impairment, may occur in pediatric patients. Hepatic encephalopathy is a rare cause of cortical blindness in children. This report describes a girl with underlying type 1 autoimmune hepatitis, who complained of sudden-onset, painless visual loss in both eyes, which was associated with generalized headache and altered mental status. She was treated with intravenous antibiotics and syrup lactulose. The patient regained full visual recovery after 1 week. Prompt diagnosis and treatment are mandatory in such uncommon instances.
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Metabolic rescue of cone photoreceptors in retinitis pigmentosa
Henry J Kaplan, Wei Wang, Niloofar Piri, Douglas C Dean
October-December 2021, 11(4):331-335
Retinitis pigmentosa (RP) encompasses a group of inherited retinal dystrophies characterized by the primary degeneration of rod and cone photoreceptors. It is a leading cause of visual disability, with an incidence of ~1 in 7000 persons. Although most RP is nonsyndromic, 20%–30% of patients with RP also have an associated nonocular condition. The gene mutations responsible for RP occur overwhelmingly in rod photoreceptors. Visual loss frequently begins with night blindness in adolescence, followed by concentric visual field loss, reflecting the principal dysfunction of rod photoreceptors. Although the visual disability from rod dysfunction is significant, it is the subsequent loss of central vision later in life due to cone degeneration that is catastrophic. Until recently, the reason for cone dysfunction in RP was unknown. However, it is now recognized that cones degenerate, losing outer segment (OS) synthesis and inner segment (IS) disassembly because of glucose starvation following rod demise. Rod OS phagocytosis by the apical microvilli of retinal pigment epithelium is necessary to transport glucose from the choriocapillaris to the subretinal space. Although cones lose OS with the onset of rod degeneration in RP, regardless of the gene mutation in rods, cone nuclei remain viable for years (i.e. enter cone dormancy) so that therapies aimed at reversing glucose starvation can prevent and/or recover cone function and central vision.
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A case of fungal conjunctivitis with giant papillae treated surgically
Asami Shimokawa, Ayaka Kobayashi, Yusuke Saeki, Eiichi Uchio
October-December 2021, 11(4):417-420
Fungal conjunctivitis is a rare disorder, with low incidence and difficulty in diagnosis due to a lack of specific clinical findings. We report a case of fungal conjunctivitis which exhibited a specific clinical feature of giant papilla formation, and its diagnosis was a complex process. A 19-year-old woman with a history of atopic dermatitis and hard contact lens use was referred to us with a 3-month history of giant papillary conjunctivitis of the right eye in spite of treatment with antiallergic and corticosteroid eye drops, complicated by intraocular pressure elevation. The left eye showed no symptom of ocular surface disorder throughout the clinical course. The right eye did not respond to oral corticosteroid. Polymerase chain reaction of conjunctival scrapings against Chlamydia trachomatis was negative, and she was treated surgically by total papilla resection. Conjunctival giant papilla recurrence was not observed after surgery. Although the primary histopathological diagnosis was chronic inflammation due to atopic keratoconjunctivitis, repeated histopathological survey of excised conjunctival tissue including immunohistochemical staining revealed histiocytes, yeast type spores and hyphae, and phagocytosed spores and hyphae in macrophages. The causative organism was identified morphologically as a Candida species. Later, histopathological examination of a cervical swab revealed the presence of Candida sp. This rare case indicates that a fungal organism may underlie refractory conjunctivitis with specific giant papillary hypertrophy mimicking vernal keratoconjunctivitis.
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Cataract extraction slowed the visual field progression rate in patients with angle-closure glaucoma
Yu-Chun Cheng, Ming-Hui Sun, Wei-Chi Wu, Wei-Wen Su
October-December 2021, 11(4):386-388
PURPOSE: The purpose of this study is to evaluate the rate of progression of Humphrey visual field before and after the cataract surgery in patients with angle-closure glaucoma. MATERIAL AND METHODS: Patients with angle-closure glaucoma who underwent regular visual field (VF) examination and received cataract surgery during the follow-up periods were retrospectively reviewed. The visual field rate of progression, visual acuity (VA), intraocular pressure (IOP), and number of glaucoma medication before and after cataract surgery were compared. RESULTS: A total of 26 eyes were included. The mean follow-up duration before and after the cataract surgery were 5.14 ± 3.31 years and 5.97 ± 2.35 years. After cataract surgery, the IOP and VA improved significantly. The visual field rate of progression significantly slowed down after cataract surgery from − 1.39 ± 1.31 dB/year preoperatively to − 0.34 ± 0.64 dB/year postoperatively (P = 0.003). CONCLUSION: The ACG eyes had rapid VF decline before cataract extraction, which was slowed down significantly after cataract removal.
  2,777 253 -
Multimodal imaging in Susac syndrome with classic clinical triad presentation
Miriam Rahhal-Ortuno, Marina Martinez-Molina, Javier Orts-Llacer, Alex Fernandez-Santodomingo, Maria Andreu-Fenoll
October-December 2021, 11(4):398-401
A 22-year-old male was referred for headaches, hearing impairment, and right eye scotoma. Branch retinal artery occlusion was revealed during the ophthalmological examination. Susac syndrome was suspected due to the symptoms described and the absence of cardiovascular risk factors. An extensive ophthalmological examination, including multimodal imaging was carried out, which is of special interest as it is considered to be a rare syndrome.
  2,750 253 -
Endoscopy-assisted extraction of orbital and nasal foreign body
Yu-Te Huang, Wei-Hsun Kung, Cheng-Hsien Chang, Wei-Ning Ku, Peng-Tai Tien, Chun-Chi Chiang, Yi-Yu Tsai
October-December 2021, 11(4):421-424
Penetrating orbital injury with a foreign body is a complex problem that requires an interdisciplinary therapeutic approach. Conventionally, an external approach using either transconjuntival or transseptal entry is used by an ophthalmologist. However, there is a risk of damage to the optic nerve and orbital tissue using only the traditional approach. This study concerns a 36-year-old male who has an intra-orbital foreign body (OrbFB). Timely three-dimensional reconstruction computed tomography scan demonstrated an 8-cm-long intra-OrbFB adjacent to the optic nerve penetrating to the contralateral nasal cavity. Endoscopic inspection confirmed a chopstick fragment. Assisted by transnasal endoscopy, the chopstick penetrating from the orbit to the nasal cavity was smoothly removed. Nasal endoscopy is useful for an ophthalmologist in removing foreign body in the orbit.
  2,754 221 -
Management of traumatic dislocation of crystalline lens: Retropupillary iris-claw versus sutureless intrascleral-fixated intraocular lens
Karishma Goyal, Nagendra Shekhawat, Kamlesh Khilnani
October-December 2021, 11(4):389-394
PURPOSE: The purpose of this study was to compare the long-term efficacy and safety of posterior iris-claw lens and scleral-fixated posterior chamber lens for aphakia after traumatic posterior dislocation of the crystalline lens. MATERIAL AND METHODS: Out of 120 evaluated cases, 60 were randomly assigned in each group. A 23G vitrectomy was done and intraocular lens was implanted by two different techniques. Extensive preoperative and postoperative evaluation was performed including optical coherence tomography and Scheimpflug imaging. Follow-up was done on days 1, 7, and 28 at 3 months, 6 months, and 12 months. RESULTS: A significant improvement was found in uncorrected visual acuity in both the groups. Surgical time in iris fixation was significantly less (P < 0.001), whereas pupil peaking and pigment release were more. Difference in mean intraocular pressure and change in astigmatism in both the groups were insignificant. CONCLUSION: Both the techniques had similar good visual results. Although operating time was shorter for iris fixation, it had several disadvantages, including immediate postoperative inflammation and ovalling of the pupil. However, scleral fixation had a better outcome in terms of postoperative complications.
  2,689 240 -
Lipemia retinalis with different therapeutic responses: A report of two cases
Chun-Chieh Lai, Chun-Hsiang Chang
October-December 2021, 11(4):405-407
Lipemia retinalis is characterized by retinal blood vessels that are creamy white in color and is considered to be directly correlated to serum triglyceride levels. It is a rare retinal manifestation, with typical serum triglyceride levels above 2500 mg/dL required for its expression. We report two cases of lipemia retinalis with hypertriglyceridemia who presented with different retinal pictures during a 5-year follow-up. One showed a normalization of retinal vessels even though the serum triglyceride level was still far more than 2500 mg/dL (4660 mg/dL), but the other had persistent creamy white discoloration despite decreases in serum triglyceride level to below 2500 mg/dL (1031 mg/dL). This suggests that the absolute serum triglyceride level may not be the sole determinant of the typical retinal manifestation. In spite of this, early detection that leads to timely treatment will assist in prevention of severe ocular and systemic vascular complications.
  2,478 235 -
A study of effect of hemodialysis on macular thickness in patients with end-stage renal disease
Shwetha Suryakanth, H N Ravi Shankar, Mallikarjun M Heralgi, Pradeep Sagar, V Kavitha, S Mahesha, N Suresh Babu, Pradeep Tekade
October-December 2021, 11(4):367-371
PURPOSE: The purpose was to study the effect of hemodialysis (HD) on macular thickness in patients with diabetic retinopathy (DR) and end-stage renal disease. MATERIALS AND METHODS: In this prospective observational study, patients undergoing HD for diabetic nephropathy were recruited. None of the patients received treatment for DR per se during the study duration. Patients underwent ocular examination and optical coherence tomography before HD and were followed up on day 3 and day 30. At each visit, central subfield macular thickness (CSMT) and total macular volume (TMV) were measured and compared with baseline values using analysis of variance and post hoc test (Wilcoxon's matched-pairs signed-rank test). RESULTS: Thirty-one eyes of 19 patients were recruited in the study. The mean CSMT decreased from baseline value of 278.93 ± 45.01 μ to 239.81 ± 40.54 μ at the end of 30 days (P < 0.005). The mean TMV decreased from baseline value of 8.14 ± 0.68 mm3 to 7.80 ± 0.63 mm3 on day 30 (P < 0.005). CONCLUSION: There was a statistically significant reduction in CSMT and TMV after HD at 30 days as compared to baseline values. HD alone results in reduction of macular thickness over short term.
  2,445 264 -
Significant improvement of ocular clinical pictures of iris metastasis from small-cell lung cancer after systemic chemotherapy
Li-Ying Huang, Ning-Yi Hsia, Chun-Ting Lai, Peng-Tai Tien, Chun-Ju Lin, Jane-Ming Lin, Yi-Yu Tsai, Wen-Lu Chen
October-December 2021, 11(4):402-404
The uveal tract is the most common ophthalmic site of ocular metastasis from systemic tumors due to the luxurious blood flow within the choroidal tissue. However, iris metastasis from systemic cancer is uncommon, accounting for only 8% of cases of uveal metastasis. There are several methods for the treatment of iris tumor. Here, we reported a case of iris metastasis from small-cell lung cancer. The tumor shrunk after systemic treatment for lung cancer and showed no recurrence after a 4-month follow-up.
  2,452 228 -
Removal of massive subretinal organized blood clot with fragmatome
San-Ni Chen, Alice C Wu
October-December 2021, 11(4):395-397
The purpose of the study was to describe using fragmatome in removing subretinal organized blood clot in eyes of age-related macular degeneration and massive subretinal hemorrhage. This study was an interventional, consecutive case series. Patients with massive subretinal hemorrhage with total or subtotal retinal detachment from age-related macular degeneration and organized subretinal blood clot were managed by creating large retinectomy. The less organized blood was removed with vitrector. The subretinal organized blood clot was removed by fragmatome. The retina was reattached with perfluorocarbon liquid, and laser was applied at the margin of retinectomy. Silicon oil was infused at the end of surgery. All patients had subretinal blood totally or subtotally removed. The organized blood clot, which was difficult to be removed by vitrector was easily and efficiently removed by fragmatome. Visual acuity improved in all eyes, and retina was well attached under silicon oil. Fragmatome offers a more efficient way in removing organized blood clot, which will much shorten the duration of operation.
  2,385 248 -
Nodular posterior scleritis – The great masquerader
Naresh Babu, Karthik Kumar, Anubhav Upadhayay, Piyush Kohli
October-December 2021, 11(4):408-412
We aim to report the management of a patient who presented with a choroidal mass masquerading as an amelanotic choroidal melanoma. A 57-year-old male presented with defective vision in his right eye, which was associated with mild periocular pain. Fundus examination showed a large dome-shaped yellowish-orange subretinal mass in the macular region and exudative retinal detachment (RD). Magnetic resonance imaging (MRI) showed a 16 mm × 8 mm choroidal mass, which was hyperintense on T1-weighted images and hypointense on T2-weighted images. B-scan ultrasonography revealed a dome-shaped mass with homogeneous echogenicity, inferior RD, and fluid collection in the sub-Tenon space. There was no choroidal excavation. He was diagnosed as nodular posterior scleritis (NPS) with exudative RD in the right eye. The lesion regressed completely after treatment with oral steroids. Choroidal mass can pose a diagnostic dilemma to ophthalmologists. Atypical MRI features can further augment the confusion. Despite its low incidence, NPS should always be kept as a differential in the presence of an amelanotic choroidal mass.
  2,311 294 -
Knowledge of genetic eye diseases and genetic services and attitudes toward genetic testing and gene therapy
Pratyusha Ganne, Manikanta Damagatla, Navya Krishna Naidu
October-December 2021, 11(4):372-379
PURPOSE: Genetic eye diseases are among the top ten causes of ocular health burden. Asia accounts for nearly two-thirds of the global burden of genetic eye diseases. A great deal of resources is being invested in genetic research and development of genetic services including gene testing laboratories and genetic counseling in India. These efforts will be meaningful only if the public and clinicians are aware of their existence. This study aimed to understand the level of knowledge about genetic eye diseases and genetic services and attitudes toward genetic testing and gene therapy in four groups of participants (undergraduate medical students, paramedical staff, non-ophthalmologist doctors, and the general public). MATERIALS AND METHODS: This was a cross-sectional survey in India. Four hundred questionnaires were analyzed from the four groups of participants. Knowledge score was calculated for the different questions. To bring out the differences across the groups, Chi-square test was done with a post hoc Mann–Whitney U-test and Kruskal–Wallis test. P < 0.05 was taken as statistically significant. RESULTS: The level of awareness about genetic eye diseases was better among undergraduate students, doctors, and paramedical staff compared to the general public (P < 0.001). The majority across all three groups had a positive attitude toward genetic testing and gene therapy. However, most of the participants across all groups were not aware of the genetic facilities available in our country. CONCLUSION: This study shows a positive attitude toward genetic medicine. However, there is a need to improve public awareness about genetic eye diseases and facilities available for genetic testing and gene therapy.
  2,226 349 -
Pattern of ocular manifestations and the prevalence of severe acute respiratory syndrome coronavirus-2 in tears of hospitalized coronavirus disease 2019 patients
Gaurang Sehgal, Prempal Bal, Bhavkaran Bal, Riya Chopra
October-December 2021, 11(4):380-385
PURPOSE: Severe acute respiratory syndrome coronavirus2 (SARSCoV2) can affect eyes in various forms. Furthermore, ocular surface can act as potential port of entry and ocular secretions as possible route of transmission of SARSCoV2. The aims of the study are to identify ocular manifestation in confirmed coronavirus disease 2019 (COVID19) patients and to evaluate conjunctival secretions as the possible route of transmission of this disease. MATERIALS AND METHODS: A prospective, populationbased, observational study was done. Ocular symptoms and signs of every hospitalized patient with SARSCoV2 infection between September 15, 2020, and November 15, 2020, were recorded. Tear samples of the first 60 participants (30 each of patients with severe and moderate illness, can be removed) with ocular manifestations were selected for real-time polymerase chain reaction (RTPCR). Data were analyzed using the Statistical Package for the Social Science Software (SPSS) program, version 23. When comparing mean values, ttest was used otherwise Chisquare test. RESULTS: Out of 804 admitted patients during study period, 309 (38.4%) patients were identified with ocular manifestations, out of which 78% (241) had conjunctival hyperemia, 65.6% (203) had follicular reaction in palpebral conjunctiva, 58% (180) had chemosis, and 58% (99) had discharge and 18.4% (57) complained of burning in their eyes. Mean duration from systemic manifestation to onset of ocular symptoms was 4.52 ± 1.47 days while 8 patients (1%) had conjunctivitis as the presenting symptom. SARSCoV2 was detected in 18.3% patients (11 out of 60) using RTPCR. CONCLUSION: The presence of SARSCoV2 in ocular secretions of patients with ocular manifestations suggests that COVID19infected patients with ocular manifestations should wear protective glasses to prevent secretions contamination to healthy persons.
  2,066 281 -
The spectrum of neuro-ophthalmologic involvement in mitochondrial disorders is broad
Josef Finsterer
October-December 2021, 11(4):425-426
  1,834 191 -
Reply to “the spectrum of neuro-ophthalmologic involvement in mitochondrial disorders is broad”
Jane H Lock, Neha K Irani, Nancy J Newman
October-December 2021, 11(4):427-428
  1,782 187 -
Innovations in retinitis pigmentosa – Metabolic rescue of cones, gene therapy, retinal transplantation
Henry J Kaplan
October-December 2021, 11(4):329-330
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