{"title":"Cogan- Reese Syndrome: A Variant of Iridocorneal Endothelial Syndrome","authors":"G. Praveena, M. Medikonda, Nandish Mashru","doi":"10.7869/DJO.366","DOIUrl":"https://doi.org/10.7869/DJO.366","url":null,"abstract":"Iridocorneal Endothelial Syndrome (ICE) is a unique ophthalmic disorder characterized by the presence of an abnormal corneal endothelium which leads to varying degrees of corneal edema, progressive iris atrophy and secondary angle closure glaucoma without pupillary block. Cogan Reese syndrome is one of the three clinical variants of Iridocorneal Endothelial Syndrome (ICE) characterized by nodular, pigmented lesions of the iris which are hallmark, and may be seen with the entire spectrum of corneal and other iris defects. Secondary glaucoma is the main sight threatening complication. Management depends on severity of ocular findings and is targeted to control secondary glaucoma medically and surgically. We hereby report two middle aged male patients who were diagnosed to have the Cogan-Reese variant of ICE Syndrome based on clinical findings.","PeriodicalId":23047,"journal":{"name":"The Official Scientific Journal of Delhi Ophthalmological Society","volume":"195 1","pages":"72-74"},"PeriodicalIF":0.0,"publicationDate":"2018-05-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73525148","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Study of A Family with Clinical Features and Inheritance Pattern of Nance Horan Syndrome","authors":"S. Patil, K. Jain, J. M. Manoher, Vimla Beniwal","doi":"10.7869/DJO.365","DOIUrl":"https://doi.org/10.7869/DJO.365","url":null,"abstract":"Congenital cataract has various modes of inheritance. In Nance Horan syndrome, X linked recessive pattern of inheritance of congenital cataract is seen. Protein truncation mutation in an NHS gene located on Xp21.2-p22.3. could be associated with this syndrome. We report a case of Nance Horan syndrome family with bilateral congenital cataract with microcornea, strabismus, nystagmus, mental retardation, dysmorphic facies and dental anomalies. Treatment consists of surgery for cataract extraction, although results are poor.","PeriodicalId":23047,"journal":{"name":"The Official Scientific Journal of Delhi Ophthalmological Society","volume":"17 1","pages":"69-71"},"PeriodicalIF":0.0,"publicationDate":"2018-05-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"84493869","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A Retinal Surprise","authors":"A. Chauhan","doi":"10.7869/DJO.362","DOIUrl":"https://doi.org/10.7869/DJO.362","url":null,"abstract":"We report a case of a 48 year old asymptomatic male who came to us for routine ocular examination but was diagnosed with retinal vasoproliferative tumour(VPT) and angioid streak.Retinal vasoproliferative tumours(VPTs) are a rare entity and after an extensive search on the internet, this is probably and to the best of our knowledge,the first reported case of VPT in association with angioid streak.","PeriodicalId":23047,"journal":{"name":"The Official Scientific Journal of Delhi Ophthalmological Society","volume":"40 12 1","pages":"61-63"},"PeriodicalIF":0.0,"publicationDate":"2018-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"72653959","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Management Of Stargardt’s Disease Through Ayurvedic Science","authors":"S. Shaw, M. N. Apoorva, K. Sujathamma","doi":"10.7869/djo.360","DOIUrl":"https://doi.org/10.7869/djo.360","url":null,"abstract":"Stargardt’s disease is a hereditary disorder of the retina. There is a gradual loss of vision during childhood or adolescence and in a few cases, vision loss may be noticed in adulthood. Involvement of photoreceptor cells presents slow loss of central vision, distorted vision and reduced color vision. Mutations in ABCA4 gene following accumulation of lipofuscin within the retinal pigment epithelium is a causative factor of the disease. Beaten bronze reflex or Snail slime reflex from the macula is the typical clinical presentation. The signs & symptoms like Avila darshana (Blurred vision) and Vyavidha darshana (distorted vision) which are mentioned in Ayurvedic classics in the context of the disease Timira, can be correlated with the clinical manifestation of Stargardt’s disease. Currently, there is no effective treatment in contemporary science for Stargardt’s disease except for gene therapy and stem cell therapy. Hence, the present clinical study was undertaken in order to understand and manage the Stargardt’s disease, with Ayurvedic treatment modalities like Virechana (Therapeutic purgative), Tarpana (Ocular therapy used to treat posterior segment disorders) and Nasya (Errhine therapy). A 25 years old male patient diagnosed with Stargardt’s disease was advised to undergo Virechana with Trivrit lehyam (A linctus made of Operculina turpethum), Nasya with Ksheerabala taila (A medicated oil prepared from Sida cordifolia and sesame oil processed with medicinal herbs) and Tarpana with Mahatriphaladi ghrita (Medicated ghee prepared out of Emblica officinalis, Terminalia bellirica, Terminalia chebula and other medicated herbs) for a course of 26 days. There was marked improvement in vision after the treatment and the results proved to be significant on the basis of clinical assessment. Abstract (fundamental units of body as per Ayurveda) vitiation which in turn produced lakshanas (signs and symptoms) like Avila darshana (blurred vision) and Vyavidha darshana (distorted vision) causing the disease. The Virechana (therapeutic purgative) and Nasya (Errhine therapy- where medicated oil is instilled in nostrils) adopted here mainly alleviate vata and pitta (Fundamental units of body as per Ayurveda) along with expulsion of morbid doshas (can be correlated with exudates) and Tarpana (ocular therapy used to treat posterior segment disorders where medicated ghee is kept over the eye for specific duration) strengthens ocular tissues and improves vision.","PeriodicalId":23047,"journal":{"name":"The Official Scientific Journal of Delhi Ophthalmological Society","volume":"26 1","pages":"56-58"},"PeriodicalIF":0.0,"publicationDate":"2018-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89682456","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Optic Disc Pit with An Atypical Presentation","authors":"N. Arora, Aniket Patel","doi":"10.7869/DJO.361","DOIUrl":"https://doi.org/10.7869/DJO.361","url":null,"abstract":"Optic Disc Pit (ODP) results from an imperfect closure of the superior edge of the embryonic fissure, usually associated with macular serous detachment. In our case report, we are presenting optic disc pit with a multilayered separation involving the nerve fibre layer and the outer plexiform layer along with neurosensory detachment.","PeriodicalId":23047,"journal":{"name":"The Official Scientific Journal of Delhi Ophthalmological Society","volume":"7 1","pages":"59-60"},"PeriodicalIF":0.0,"publicationDate":"2018-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78776713","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Congenital Glaucoma with Neurofibromatosis Type-I A Rare Occurrence","authors":"Vankireddi Mahathi, S. Karanam, M. Medikonda","doi":"10.7869/djo.363","DOIUrl":"https://doi.org/10.7869/djo.363","url":null,"abstract":"Neurofibromatosis 1 (NF1) is a rare genodermatoses in which there is involvement of the skin, eye, peripheral nervous system and skeletal system. Congenital glaucoma occurs in 1 in 300 NF1 patients. A 6 year old boy presented to us with defective vision in the left eye since 4 years. On examination the child had, café-au-lait spots, reduced visual acuity, Lisch nodules, increased corneal diameter, with glaucomatous cupping of optic nerve. Based on these findings, a diagnosis of congenital glaucoma with NF1 was made. The child was started on anti glaucoma medication for which he was non compliant and hence trabeculectomy was performed and the pressures are being maintained till date. Hence, if the diagnosis of NF1 is made, eye examinations are recommended in children for early detection of increased intraocular pressure or other ophthalmological manifestations and to reduce visual morbidity.","PeriodicalId":23047,"journal":{"name":"The Official Scientific Journal of Delhi Ophthalmological Society","volume":"48 1","pages":"64-66"},"PeriodicalIF":0.0,"publicationDate":"2018-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82619719","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Bilateral Shallow Anterior Chamber And Transient Myopia As A Presenting Feature Of Vogt Koyanagi Harada Syndrome","authors":"Rahul Sharma, Abhishek Dagar, Vivek Kumar","doi":"10.7869/DJO.357","DOIUrl":"https://doi.org/10.7869/DJO.357","url":null,"abstract":"The Vogt-Koyanagi-Harada (VKH) syndrome is a rare systemic disorder of uveitis, dysacousia, vitiligo, premature greying of the hair, eyebrow and eyelashes, and meningoencephalitis. We report a case of a 36 year old type 2 diabetic patient who presented with sudden, painless, progressive blurring of vision in both eyes with shallow anterior chamber and transient myopia. He had history of headache, mild fever, malaise with stiffness in neck and back one week before blurring of vision. Exudative retinal detachment on fundus examination along with optical coherence tomography (OCT) features and fundus fluorescein angiogram (FFA) pattern confirmed our diagnosis as incomplete VKH. The patient responded well with steroid (systemic and topical) and cycloplegic drug. This case highlights the importance of history, careful ocular evaluation, judicious use of OCT and FFA in a patient presenting with bilateral shallow anterior chamber and transient myopia to narrow down the differential diagnosis as incomplete VKH. both eye myopic refraction. Anterior segment of both shallow anterior inflammatory uveitis Posterior segment had mild vitritis in both eyes. In both the eyes, applanation tonometry was 18 millimeters of mercury. Schwalbe’s line was visible on gonioscopy in both eyes and on indentation the angles opened up to scleral spur. Fundus showed multilobular areas of subretinal fluid pockets (exudative retinal detachment) at the posterior pole and mid periphery with creamy yellow choroidal lesions, clinically suggestive of exudative pathology. There were no signs of diabetic retinopathy. On laboratory investigations, complete blood count was within normal limits, erythrocyte sedimentation rate (ESR) was 20mm/hour, Mantoux skin test was negative (3x3 mm), human immune deficiency virus (HIV) and Treponemal pallidum haemagglutination assay (TPHA) were non reactive. Serum angiotensin converting enzyme (ACE) was 24 IU (normal). Chest X ray, HRCT Chest, USG of whole abdomen was normal. Optical coherence tomography at macula of both eyes showed multiple areas of neurosensory detachment with largest volume and subfield thickness at macula & fluorescein angiogram (FFA) of early and mid-phase showed multiple hyperfluorescent Abstract","PeriodicalId":23047,"journal":{"name":"The Official Scientific Journal of Delhi Ophthalmological Society","volume":"221 1","pages":"46-50"},"PeriodicalIF":0.0,"publicationDate":"2018-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73152522","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Inclusion Cell Disease - A Rare Cause of Megalocornea with Corneal Edema","authors":"Namrata, R. Ranjan, G. Arya","doi":"10.7869/DJO.354","DOIUrl":"https://doi.org/10.7869/DJO.354","url":null,"abstract":"Introduction There are multiple causes of megalocornea and corneal edema in infantile age group. The common causes include birth trauma, Peter’s anomaly, limbal dermoid, sclerocornea, congenital hereditary endothelial dystrophy (CHED), mucopolysaccharidosis and infective/inflammatory process. It is important to consider congenital error of metabolism including mucopolysaccharidosis as an etiology for corneal edema/corneal clouding and megalocornea because these cases are rare and can be easily missed out if not evaluated properly. Case report A 11 month old male child presented with complaints of reduced weight gain, failure to thrive, recurrent respiratory infections and diarrhea. On clinical examination the patient showed coarse facies, flat face, prominent bulging eyes, large tongue, enlarged gums (Figure 1). Perinatal history was normal term vaginal delivery, and he was the first child of non-consanguineous marriage. Ophthalmological examination revealed megalocornea (horizontal corneal diameter of 16 mm – Figure 1) with corneal edema/corneal clouding. Anterior chamber was deep. No cherry red spots were seen on fundoscopic examination. Intra ocular pressure was within normal limits. On Gonioscopy, angles were open. On indirect ophthalmoscopy, the optic disc was of normal size, shape & colour. The optic cup was within normal limit. The neuroretinal rim was healthy. Retinal artery:vein ratio was maintained. The macula was healthy and the foveal reflex was present. MRI Brain was done which revealed T2 hyperintense signal involving cerebral white matter on either side uniformly involving both subcortical and periventricular white matter without abnormal T1 hypointense signal suggestive of Hypomyelination MRI is (Figure 2). T2 hypointense signal was seen in bilateral thalami (Figure 2). Therefore possibility of lysosomal storage disorder was considered. CT skull revealed premature fusion of cranial sutures suggestive of craniosynostosis (Figure 2). No anterior vertebral body beaking was noted on lateral X-Ray of dorsolumbar spine. X-Ray pelvis with femur revealed osteopenia with short thick femur (Figure 3). Ultrasonography of abdomen showed mild hepatosplenomegaly with liver Delhi J Ophthalmol 2018;28;40-1; Doi http://dx.doi.org/10.7869/djo.354 Abstract","PeriodicalId":23047,"journal":{"name":"The Official Scientific Journal of Delhi Ophthalmological Society","volume":"24 1","pages":"40-41"},"PeriodicalIF":0.0,"publicationDate":"2018-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89094899","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Corneal Ulcer Following Prolonged Topical Chloroquine Phosphate","authors":"Samarth Mishra, Sudeep Das","doi":"10.7869/djo.355","DOIUrl":"https://doi.org/10.7869/djo.355","url":null,"abstract":"Chloroquine and hydroxychloroquine are well known weapons in a rheumatologist’s armamentarium. Chloroquine, (N’-(7-chloroquinolin-4-yl)-N,N-diethyl-pentane-1,4-diamine) is an antimalarial drug which has been in use since the early 1950s, in the treatment of Discoid and Systemic Lupus Erythematoses, Rheumatoid Arthritis and dermal light sensitivity eruptions. Hobbs in 1959 recognised a retinopathy which was produced by the long-term administration of this drug. Since the proposal of its anti-inflammatory effect, chloroquine phosphate has been used for the treatment of dry eyes. Among the many side-effects following systemic use of chloroquine; pruritus, urticaria, alopecia, lymphedema of forearm, headache, nausea etc are noteworthy. Ophthalmological complications like bull’s eye maculopathy, keratopathy, corneal deposits have also been observed, although infrequently. Majority of the complications arise from the long-term use of the drug which leads to its accumulation in specific sites. Here, we present a rare case report of sterile corneal ulcer following prolonged use of preservative free topical chloroquine phosphate (0.03%w/v) in the form of unims. after","PeriodicalId":23047,"journal":{"name":"The Official Scientific Journal of Delhi Ophthalmological Society","volume":"1 1","pages":"42-43"},"PeriodicalIF":0.0,"publicationDate":"2018-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78575851","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
P. Agrawal, Varsha Yadav, V. Kathiriya, M. Kanhere
{"title":"External Ophthalmomyiasis By Sheep Nasal Bot Fly A-Rare Case Report From Tropical Region of India","authors":"P. Agrawal, Varsha Yadav, V. Kathiriya, M. Kanhere","doi":"10.7869/DJO.358","DOIUrl":"https://doi.org/10.7869/DJO.358","url":null,"abstract":"Myiasis is infection of tissues or organs of animals or man by larvae of a fly. Ophthalmomyiasis is classified as ophthalmomyiasis externa if the larvae are present on the conjunctiva, ophthalmomyiasis interna when there is intraocular penetration of larvae and orbital/ocular ophthalmomyiasis if larvae penetrate the orbit. This condition is rare in India, with no case reports from Mumbai. We report a rare case of Ophthalmomyiasis externa in a 25 year old male who presented with sudden onset foreign body sensation in his right eye, followed by intense watering, irritation and redness since 2 hours. Visual acuity was 6/6 and N6 in both eyes. The right eye showed superficial congestion for chemosis; and no evidence of a foreign body. On detailed examination, three motile larvae were found and removed from the conjunctiva, which were later identified as larvae of Oestrus ovis (Sheep nasal botfly). The patient was started on antihistaminics and antibiotics and showed improvement within 24 hours. Our case highlights two things – firstly, the importance of early detection of this condition by creating awareness amongst ophthalmologists as being one of the causes of conjunctivitis during spring/summer seasons especially in developing countries like India; and secondly, plain irrigation of the conjunctiva is unsuccessful in washing out of the larvae.","PeriodicalId":23047,"journal":{"name":"The Official Scientific Journal of Delhi Ophthalmological Society","volume":"108 1","pages":"51-52"},"PeriodicalIF":0.0,"publicationDate":"2018-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74375338","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}