DOCSLIB.ORG

Angioid Streaks and Sickle Haemoglobinopathies

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Angioid Streaks and Sickle Haemoglobinopathies Brit. Y. Ophthal. (I976) 6o, 3 I Br J Ophthalmol: first published as 10.1136/bjo.60.1.31 on 1 January 1976. Downloaded from Angioid streaks and sickle haemoglobinopathies K. C. NAGPAL, G. ASDOURIAN, M. GOLDBAUM, D. APPLE, AND M. F. GOLDBERG From the Sickle Cell Eye Clinic of the University of Illinois Eye and Ear Infirmary, Chicago, Illinois Doyne (I889) described the clinical picture of Clinical data and methods angioid streaks. Groenblad (1929) reported the A total of 356 patients had been screened in the Sickle association of skin disease with angioid streaks. Cell Eye Clinic of the University of Illinois by May Strandberg (I929) carried out histopathological 1975. The haemoglobin electrophoresis of these patients examinations of skin biopsies from Groenblad's showed that i62 had sickle cell anaemia (Hb SS); ioI patients and proved the abnormality to be that of sickle cell-haemoglobin C disease (Hb SC); 49 sickle pseudoxanthoma elasticum. Subsequently, angioid cell trait (Hb AS); 34 haemoglobin S-thalassaemia (Hb streaks have been reported in association with S-thal); eight haemoglobin AC; and two haemoglobin several other diseases, such as Paget's disease CC. (Paton, I 972), acromegaly (Holloway, I 927; Howard, Of these 356 patients five had angioid streaks and were investigated in detail. Investigations included i963; Paton, i963), and Ehlers-Danlos syndrome detailed physical examination and the following labora- (Green, Friedman-Kien, and Banfield, i966). tory determinations: Hb levels, Hb electrophoresis, The association of angioid streaks with sickle serum calcium (normal 50 ± 0o5 mEq/litre and phos- cell disease was first reported by Geeraets and phate (normal 1-5-2-9 mEq/litre) levels, alkaline phos- copyright. Guerry (ig6oa) who described an incidence of phatase (normal 20-124 international units/ml), skull angioid streaks of 6 per cent in a selected popula- and pelvic x-ray films, and skin biopsy. Haematoxylin tion of sickle cell patients (five of 69 cases). Subse- and eosin stain, Verhoeff-van Gieson stain for elastic quent reports have not substantiated such a high tissue, and von Kossa stain for calcium deposition were incidence of angioid streaks concomitant with done in all cases to rule out elastic tissue abnormalities. Ocular examination included visual acuity determi- sickle cell disease (Welch and Goldberg, I966; nation in both and anterior examination Paton, I972). Geeraets and Guerry (Ig6ob) also eyes segment with the slit lamp. A detailed fundus drawing with the http://bjo.bmj.com/ reported elastic tissue degeneration in sickle cell binocular indirect ophthalmoscope, fundus photography, patients and speculated whether intraocular and and fluorescein angiography were carried out in all general elastic tissue degeneration in sickle cell cases. disease represented a coincidental or true causative relationship. Skin biopsy studies in two sickle cell Case reports patients (Paton, 1972) failed to show any abnor- mality of elastic tissue. Case I, a 42-year-old black woman with Hb SS, was first seen in November 1972. Her included on September 29, 2021 by guest. Protected Since we had an opportunity to examine a large history several episodes of sickle cell crises with episodic fever, series of sickle cell patients, the purpose of this joint pain, abdominal pain, and jaundice. She also had a communication is to report the incidence of angioid history of mild systemic hypertension. Her blood streaks in various sickle haemoglobinopathies. It is pressure on systemic examination was I95/95 mm Hg. also our intention to demonstrate that no other Visual acuity was 20/30 in both eyes. Slit-lamp disease, such as pseudoxanthoma elasticum, Paget's examination revealed positive conjunctival sickling disease, or acromegaly, was associated with angioid sign. The right fundus showed small angioid streaks streaks in our sickle cell patients. Because others localized in the peripapillary region and two black have found elastic tissue alterations in sickle cell sunbursts (chorio-retinal scars) at 12 and 2 o'clock in patients, we examined the skin of all our patients, the retinal periphery. The macula was not involved. specifically looking for elastic tissue changes The left eye also showed peripapillary angioid streaks without involvement of the macula. An area of neovas- histopathologically. cularization at 1.30 o'clock in the equatorial region We also investigated the relationship between leaked dye on fluorescein angiography; argon laser the severity of sickle cell retinopathy and the photocoagulation successfully occluded the feeder presence of angioid streaks. vessels. Follow-up in August 1974 showed no change in Address for reprints: Morton F. Goldberg, MD, University of angioid streaks or sickle retinopathy. Laboratory evalua- Illinois, Eye and Ear Infirmary, I855 W. Taylor Street, Chicago, tion showed Hb level 9-6 g/ioo ml; Hb SS by electro- Illinois 6o6I2, USA phoresis; serum calcium 4-8 mEq/litre; phosphate 32 Bri'tish Yournal of Ophthalmology Br J Ophthalmol: first published as 10.1136/bjo.60.1.31 on 1 January 1976. Downloaded from 2 6 mEq/litre; alkaline phosphatase 67 units/ml. Skull and pelvic x-ray films were normal. Skin biopsy showed no evidence of subepithelial or dermal elastic tissue alterations. Case 2, a 46-year-old black man with Hb SC, was first seen in October I97I with the diagnosis of vitreous haemorrhage in both eyes. His past history was unre- markable. Systemic examination was normal. Visual acuity was 20/50-2 in the right eye and 20/25 in the left. Fundus examination revealed angioid streaks near the disc and a large patch of neovasculari- zation in the right eye at io o'clock with a small vitreous haemorrhage in the temporal equatorial zone. The left eye showed sea-fans at 9.30, I2, I, and 3 o'clock and angioid streaks around the disc. Follow-up in September 1974 showed small angioid streaks in both eyes, which were confined to the peripa- pillary area; the maculae were not involved. Laboratory findings were as follows: Hb I0-2 g/ioo ml; Hb SC by electrophoresis; serum calcium 4-9 mEq/ litre; phosphate 2- I mEq/litre; alkaline phosphatase FIG. I Case 3, left eye. Fundus photograph shozcing 34 units/ml. Skull and pelvic x-ray films showed findings angioid streaks consistent with sickle cell anaemia. Skin biopsy was normal. Case 3, a 36-year-old black man with Hb SS, was copyright. seen in July 1974 for sickle cell retinopathy. The patient had had a few sickle cell crises, urinary tract infection, renal vein thrombosis, and upper respiratory tract infection. General physical examination was normal. Visual acuity was 20/20 in both eyes. Slit-lamp examination showed positive conjunctival sickling sign. Fundus examination revealed prominent angioid streaks in both eyes (Figs i and 2) radiating from the peripapillary region. The maculae were not involved. http://bjo.bmj.com/ The retinal periphery showed arteriolar occlusions typical of sickle cell disease. No neovascularization was seen. Laboratory findings were as follows: Hb I0-9 g/ioo ml; Hb SS by electrophoresis; serum calcium 4-9 mEq/litre; phosphate 2-4 mEq/litre; alkaline phospha- tase 40 units/ml. Skull and pelvic x-ray films were nor- mal; abdominal x-ray films revealed gallstones. Skin on September 29, 2021 by guest. Protected biopsy showed no elastic tissue degeneration. Case 4, a 47-year-old black man with known Hb FIG. 2 Case 3. Fluorescein angiogram of same eye as S-thal, was first seen in May 1970 with the complaint in Fig. I, showing hyperfluorescence of angioid streaks of poor vision in the right eye. He had had several episodes of sickle cell crises for which he had received symptomatic treatment. with retinal pigmentary changes and vascular occlu- Ocular examination showed visual acuity of percep- sions in both the central and the peripheral area. The tion of light in the right eye and 20/20 in the left. inferior retina not involving the macula was detached Slit-lamp examination was unremarkable. Vitreous with vitreous traction and breaks in this area. White haemorrhage prevented fundus examination of the fibrous tissue was seen at 8 o'clock. There was no right eye. The left eye showed prominent wide angioid improvement in vision after cataract extraction. streaks radiating from the peripapillary region; the Laboratory findings were as follows: Hb 9-6 g/ioo macula was not involved. ml; Hb S-thal by electrophoresis; serum calcium Patches of neovascularization at I and 3 to 4 o'clock 5-I mEq/litre; phosphate 2-I mEq/litre; alkaline phos- in the equatorial region of the left eye were photo- phatase 26 units/ml. Skull and pelvic x-ray films sug- coagulated in 1971. gested Paget's disease or metastatic carcinoma. Alkaline A cataract was removed from the right eye in Sep- phosphatase levels were normal repeatedly. Systemic tember I974. The retina showed extensive degeneration examination showed no evidence of malignancy. Skin Angioid streaks and sickle hacmoglobznopath/es 33 Br J Ophthalmol: first published as 10.1136/bjo.60.1.31 on 1 January 1976. Downloaded from biopsy was normal; no elastic tissue degeneration was might have been causally related to cracks in found. Bruch's membrane. Our findings lend support to the contention of Paton (I972) that it is highly Case 5, a 49-year-old black man with Hb SS, was unlikely that an elastic tissue defect exists in first seen in January I973. History showed that he had addition to Hb abnormality. We are unable to had several episodes of sickle cell crises and joint pain which had been treated symptomatically. confirm Paton's hypothesis that chronic haemolysis Visual acuity of 20/30 in both eyes improved to results in deposition of iron in Bruch's membrane, 20/20 with -s5D sph.
Load more

Recommended publications
  • Sickle Cell Retinopathy (SCR) in a Patient with Sickle Cell Trait - a Rare Case Report
    Jemds.com Case Report Sickle Cell Retinopathy (SCR) in a Patient with Sickle Cell Trait - A Rare Case Report Vandana Panjwani1, Sachin Daigavane2, Sourya Acharya3, Madhumita Prasad4 1Department of Ophthalmology, Datta Meghe Institute of Medical Sciences (Deemed to Be University), J.N. Medical College, Sawangi (M), Wardha, Maharashtra, India. 2Department of Ophthalmology, Datta Meghe Institute of Medical Sciences (Deemed to Be University), J.N. Medical College, Sawangi (M), Wardha, Maharashtra, India. 3Department of Medicine, Datta Meghe Institute of Medical Sciences (Deemed to Be University), J.N. Medical College, Sawangi (M), Wardha, Maharashtra, India. 4Department of Ophthalmology, Datta Meghe Institute of Medical Sciences (Deemed to Be University), J.N. Medical College, Sawangi (M), Wardha, Maharashtra, India. INTRODUCTION Sickle cell trait is an inherited hematologic anomaly that affects 1 million to 3 million Corresponding Author: Dr. Sourya Acharya, Americans and 8 to 10 percent of African Americans. It also affects other races like Professor, Hispanics, south Asians, Caucasians from southern Europe, and people from Middle Department of Medicine, Eastern countries. Evidence based estimations suggests that more than 100 million Datta Meghe Institute of Medical people worldwide have sickle cell trait. Unlike sickle cell disease, where two genes Sciences (Deemed to be University), that cause the production of abnormal haemoglobin, individuals with sickle cell trait J.N. Medical College, Sawangi (M), carry only one defective gene and typically live normal lives. Extreme conditions such Wardha, Maharashtra, India. as severe dehydration and high-intensity physical activity can lead to serious health E-mail: [email protected] issues, including sudden death, for individuals with sickle cell trait.
    [Show full text]
  • R Manifestations in Sickle Cell Disease ( SCD) in Children
    Research Article Ocular manifestations in sickle cell disease ( SCD) in children Chavan Ravindra 1, Tiple Nishikant 2*, Chavan Sangeeta 3 {1Associate Professor, 2Assistant Professor, Department of Pediatrics } { 3Sr. Resident, Department of Ophthalmology} Shri.Vasantrao Naik Gover nment Medical College, Yavatmal, Maharashtra, INDIA. Email: [email protected] Abstract Introduction: Sickle cell disease (SCD) is autosomal recessive inherited condition characterized by presence of anomalous haemoglobin ‘S’ in the erythrocytes. Patients with SCD inherit an abnormal haemoglobin which becomes insoluble when deoxygenated and so distorts th e red cells and cause tissue infarction 1,2,3 . The organs mainly affected are spleen, the bones, the kidney, the lung and the skin. But any organ may be involved and the eyes are not exemption. Hence the study is taken up to find ocular manifestation in SCD in children. Aims and Objectives: To find out prevalence, nature and outcome of ocular manifestation in SCD in children’s. Material and Methods: This prospective study was conducted in pediatrics department of tertiary care hospital from Oct 2000 to April 2002. The study group includes SCD patients admitted in pediatrics ward and patients attending SCD speciality clinics, who were electrophoretica lly confirmed for diagnosis of sickle cell haemoglobinopathy. A detail history, clinical examination and routine investigation were done in each case. A systematic ophthalmological examination was meticulously done in every case which includes visual acuit y, intraocular tension measurement, slit lamp examination and fundus examination . Observation and Results: A total of 204 cases of SCD, who were electrophoretically confirmed for diagnosis of sickle cell haemoglobinopathy were enrolled during study period, out of which 120(58.82%) patients were homozygous “SS” and 84(41.17%) patients were heterozygous “AS” for SCD.
    [Show full text]
  • Pseudoxanthoma Elasticum Diagnosed Based on Ocular Angioid
    Cui et al. BMC Ophthalmology (2021) 21:307 https://doi.org/10.1186/s12886-021-02069-0 CASE REPORT Open Access A case report: pseudoxanthoma elasticum diagnosed based on ocular angioid streaks and the curative effect of Conbercept treatment Chaoxiong Cui1* , Zhanyu Zhou2, Yi Zhang2 and Ding Sun2 Abstract Background: This article is a case report of pseudoxanthoma elasticum (PXE) which was diagnosed based on significant angioid streaks (AS) with choroidal neovascularization (CNV) and regain normal visual function by intravitreal injection with Conbercept. Case presentation: A 51-year-old woman was referred to the Ophthalmology Department of Qingdao Municipal Hospital (Qingdao, China) on September 14, 2020 for metamorphopsia and loss of vision in the left eye in the preceding three days. Past history: high myopia for more than 30 years, best corrected visual acuity (BCVA) of both eyes was 1.0 (5 m Standard Logarithm Visual Acuity chart in decimal notations), hypertension for six years, and cerebral infarction two years ago, no history of ocular trauma or surgeries or similar patients in family was documented. We used methods for observation, including fundus examination, optical coherence tomography (OCT), fluorescein angiography combined with indocyanine green angiography (FFA + ICGA). Due to her symptoms and manifestations, along with the appearance of her neck skin, which resembled ‘chicken skin’, we speculated that she should be further examined at the Department of Dermatology by tissue paraffin section and molecular pathology analyses, and the diagnosis of PXE was then confirmed. After intravitreal injection with Conbercept (10 mg/ml, 0.2 ml, Chengdu Kanghong Biotechnologies Co., Ltd.; Chengdu, Sichuan, China) she regained her BCVA.
    [Show full text]
  • Ocular Complications in Sickle Cell Disease: a Neglected Issue
    Open Journal of Ophthalmology, 2020, 10, 200-210 https://www.scirp.org/journal/ojoph ISSN Online: 2165-7416 ISSN Print: 2165-7408 Ocular Complications in Sickle Cell Disease: A Neglected Issue Hassan Al-Jafar1, Nadia Abul2*, Yousef Al-Herz2, Niranjan Kumar2 1Hematology Department, Amiri Hospital, Amiri, Kuwait 2Al Bahar Eye Center, Ibn Sina Hospital, Ministry of Health, Kuwait city, Kuwait How to cite this paper: Al-Jafar, H., Abul, Abstract N., Al-Herz, Y. and Kumar, N. (2020) Ocular Complications in Sickle Cell Dis- Sickle cell disease is a common genetic blood disorder. It causes severe sys- ease: A Neglected Issue. Open Journal of temic complications including ocular involvement. The degree of ocular Ophthalmology, 10, 200-210. complications is not necessarily based on the severity of the systemic disease. https://doi.org/10.4236/ojoph.2020.103022 Both the anterior and posterior segments in the eye can be compromised due Received: May 7, 2020 to pathological processes of sickle cell disease. However, ocular manifesta- Accepted: July 14, 2020 tions in the retina are considered the most important in terms of frequency Published: July 17, 2020 and visual impairment. Eye complications could be one of the silent systemic Copyright © 2020 by author(s) and sickle cell disease complications. Hence, periodic ophthalmic examination Scientific Research Publishing Inc. should be added to the prophylactic and treatment protocols. This review ar- This work is licensed under the Creative ticle is to emphasize the ocular manifestations in sickle cell disease as it is a Commons Attribution International silent complication which became neglected issue. Once the ocular complica- License (CC BY 4.0).
    [Show full text]
  • An Unusual Case of Proliferative Sickle Cell
    AN UNUSUAL CASE OF PROLIFERATIVE SICKLE CELL RETINOPATHY Case Report By : *C Tembo, D Kasongole 1Department of Surgery, School of Medicine, University of Zambia, Lusaka-Zambia 2University Teaching Hospitals - Eye Hospital, Lusaka-Zambia *E-mail Addresses: Chimozi Tembo: [email protected] Citation Style For This Article: Tembo C, Kasongole D. An Unusual Case of Proliferative Sickle Cell Retinopathy. Health Press Zambia Bull. 2019; 3(12); pp 6-8. ABSTRACT Teaching Hospital, Lusaka-Zambia involv- was advised that he needed surgery but Sickle cell haemoglobinopathies are a ing 94 patients, looking at the ocular man- was lost to follow-up. group of inherited disorders character- ifestations of sickle cell disease, found The patient had no history of hyperten- ized by quantitative or qualitative malfor- that ocular abnormalities were high with sion, diabetes mellitus, sickle cell disease, mations of haemoglobin (Hb). Diagnosis 69% of patients showing signs of ocular TB or retroviral disease. Family history of SCD is mainly by haemoglobin elec- manifestations. However, most were not was non-revealing. There was no history trophoresis. Ocular manifestations are causing visual impairment, with only 1% of alcohol intake or smoking. wide, encompassing anterior segment, of the patients being blind as a result of On examination, the general condition non-proliferative and proliferative reti- SCD [3]. was good. There was no pallor, jaundice or nopathy. Proliferative sickle cell retinop- Though PSCR can occur in patients with cyanosis. Visual acuity was hand motion athy (PSCR) represents a very serious sickle cell trait, it is very rare and in most (HM) and 6/18 not improving with pin- complication and may result in blindness cases there are other co-existing systemic hole in the right and left eye, respectively.
    [Show full text]
  • Classification of Angioid Streaks* by R
    Br J Ophthalmol: first published as 10.1136/bjo.39.5.298 on 1 May 1955. Downloaded from Brit. J. Ophthal. (1955) 39, 298. CLASSIFICATION OF ANGIOID STREAKS* BY R. J. McWILLIAM Victoria Infirmary, Glasgow ANGIOID streaks of the retina were first described by Doyne (1889). They have since been the subject of much controversy. More than 200 cases have been reported in the literature, but histological studies have been few and the changes described have varied. The condition is frequently associated with pseudoxanthoma elasticum, with cardiovascular disease, and with Paget's disease of bone. A similar appearance may also be found after detachments of the choroid, folds of the retina, and other conditions. The fundus picture consists of reddish or dark brown streaks radiating outwards from the disc, and often seeming to originate from a similar streak running partly or completely round the disc. The constancy of this picture led Collins (1923) to postulate a definite anatomical basis for their distribution and he attributed them to deposits of altered blood pigment lying in the perivascular spaces of the posterior ciliary arteries. However the streaks occur below the retinal vessels and above those of the choroid, so that they must be located either in the deep layers of the retina or in the inner layers of the choroid. Furthermore, the streaks have been seen to be interrupted copyright. by areas of choroidal atrophy, an observation which makes it unlikely that they are related to the choroidal vessels (Spicer, 1914; Wildi, 1926). That the cause of angioid streaks might be breaks in the membrane of Bruch was first suggested by Kofler (1917).
    [Show full text]
  • Angioid Streaks Associated with Abetalipoproteinemia
    Ophthalmic Genetics ISSN: 1381-6810 (Print) 1744-5094 (Online) Journal homepage: http://www.tandfonline.com/loi/iopg20 Angioid streaks associated with abetalipoproteinemia Michael B. Gorin, T. Otis Paul & Daniel J. Rader To cite this article: Michael B. Gorin, T. Otis Paul & Daniel J. Rader (1994) Angioid streaks associated with abetalipoproteinemia, Ophthalmic Genetics, 15:3-4, 151-159, DOI: 10.3109/13816819409057843 To link to this article: http://dx.doi.org/10.3109/13816819409057843 Published online: 08 Jul 2009. Submit your article to this journal Article views: 7 View related articles Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=iopg20 Download by: [UCLA Library] Date: 02 May 2017, At: 14:12 0Ophthalmic Genetics 0167-6784/94/ Angioid streaks associated with US$ 3.50 abetalipoproteinemia Ophthalmic Genetics - 1994, Vol. 15, No. 3/4pp. 151-159 Michael B. Gorin’ 0Eolus Press T. Otis Paul2 Buren (The Netherlands) 1994 Daniel J. Rader3* Accepted 10 September 1994 Departments of Ophthalmology and Human Genetics, University of Pittsburgh School of Medicine and Graduate School of Public Health, Pittsburgh, PA 1521 3 Smith-Kettlewell Eye Research Institute, San Francisco, CA 941 15 Molecular Disease Branch; National Heart, Lung, and Blood Institute; National Institutes of Health, Bethesda, MD 20892 USA Abstract Angioid streaks were observed in two patients with abetalipo- Acknowledgements: The authors wish proteinemia. The progression of the angioid streaks was minimal over the to thank Dr. R. E. Anderson for years that these patients received vitamin A and E supplementation, though performing serum lipid analyses of in one patient the development of subretinal neovascular membranes within these patients and for his helpful the angioid streaks was the cause of rapid central visual loss.
    [Show full text]
  • Ophthalmic Manifestation of Sickle Cell Patients in Eastern India Internal Medicine Section Internalmedicine
    DOI: 10.7860/JCDR/2018/36868.11810 Original Article Ophthalmic Manifestation of Sickle Cell Patients in Eastern India Internal Medicine Section InternalMedicine SWATI SAMANT1, SRIKANT KUMAR DHAR2, MAHESH CHANDRA SAHU3 ABSTRACT Results: Male:Female ratio was 3:1. About 2/3rd of the patients Introduction: Sickle Cell Disease (SCD) is the most common were below 40 years of age. Examination of posterior segment and a serious form of an inherited blood disorder that leads revealed 5 (10%) of the patients presented with proliferative to increased risk of early mortality and morbidity. Some of the retinopathy, 15 (30%) with non proliferative retinopathy, 13(26%) ophthalmological complications of SCD include retinal changes, with optic disc changes, 7 (14%) with retinal macular changes and vitreous haemorrhage, and abnormalities of the conjunctiva. 2 (4%) had retinal detachment findings are significantly different Irrecoverable Vision loss may be a manifestation if not diagnosed at p=0.001 in ANOVA Test. Anterior segment of eye evaluation early and treated appropriately. demonstrated significant (p=0.0001) changes 18 (36%) patients suffered conjunctival vascular changes, Cataract in 8(16%) Aim: To determine different ophthalmic manifestations in SCD patients, and hyphema in only 2 (4%) patients. Both anterior patients and correlate in relation to HbS window. and posterior segment manifestations significantly (p=0.0027) Materials and Methods: A total of 49 cases of sickle cell increased with progressive increase in HbS window. disease (HbSS) that presented to IMS & SUM Hospital were Conclusion: Sickle cell patients need periodic ophthalmic evaluated for ophthalmic manifestations in Ophthalmology OPD examinations to identify treatable lesions amenable to with comprehensive eye examination, slit lamp examination, intervention and to prevent blindness.
    [Show full text]
  • CNV in CRSC Retina 2015.Pdf
    CHOROIDAL NEOVASCULARIZATION IN CAUCASIAN PATIENTS WITH LONGSTANDING CENTRAL SEROUS CHORIORETINOPATHY ENRICO PEIRETTI, MD,* DANIELA C. FERRARA, MD, PHD,† GIULIA CAMINITI, MD,* MARCO MURA, MD,‡ JOHN HUGHES, MD, PHD‡ Purpose: To report the frequency of choroidal neovascularization (CNV) in Caucasian patients with chronic central serous chorioretinopathy (CSC). Methods: Retrospective consecutive series of 272 eyes (136 patients) who were diagnosed as having chronic CSC based on clinical and multimodal fundus imaging findings and documented disease activity for at least 6 months. The CNVs were mainly determined by indocyanine-green angiography. Results: Patients were evaluated and followed for a maximum of 6 years, with an average follow-up of 14 ± 12 months. Distinct CNV was identified in 41 eyes (34 patients). Based on fluorescein angiography, 37 eyes showed occult with no classic CNV, 3 eyes showed predominantly classic and 1 eye had a disciform CNV. Furthermore, indocyanine- green angiography revealed polypoidal choroidal vasculopathy lesions, in 27 of the 37 eyes, classified as occult CNV on fluorescein angiography. In total, 17.6% of our patients with chronic CSC were found to have CNV that upon indocyanine-green angiography were recognized as being polypoidal choroidal vasculopathy. Conclusion: In our series of Caucasian patients, we found a significant correlation between chronic CSC and CNV, in which the majority of patients with CNV were found to have polypoidal choroidal vasculopathy. Our findings suggest that indocyanine-green angiography is an indispensable tool in the investigation of chronic CSC. RETINA 35:1360–1367, 2015 entral serous chorioretinopathy (CSC) is a common exogenous hypercortisolism has been observed.3,4 The Cdisease characterized by idiopathic neurosensory clinical presentation may vary widely, although visual retinal detachment, often associated with one or more symptoms are generally mild, unspecific, and transi- serous pigment epithelial detachment in the macular or tory.
    [Show full text]
  • Ocular Manifestations of Sickle Cell Hemoglobinopathy
    WIMJOURNAL, Volume No. 6, Issue No. 1, 2019 pISSN 2349-2910, eISSN 2395-0684 Mukaram Khan ORIGINAL RESEARCH ARTICLE Ocular Manifestations of Sickle Cell Hemoglobinopathy: A Study in Tertiary Eye Care Centre of North Maharashtra (India) Mukaram Khan1, Deepali Gawai2, Smita Taur3 and Bhagwat V. R4 Associate Professor1, Associate Professor2, Senior Resident3, Dept.of Opthalmology Professor & Head, Department of Biochemistry4, Shri Bhausaheb Hire Government Medical College, Dhule (Maharashtra) INDIA. Abstract: Background: Sickle cell disease (SCD) is the most common genetic disease worldwide. The increase in life expectancy of SCD patients in recent years has led to the emergence of more complications of the disease, including ocular changes, which were uncommon in the past. SCD can affect virtually every vascular bed in the eye and can cause blindness in the advanced stages. Purpose: The present study was carried out to assess the incidence and prevalence of all ocular manifestations in SCD and to correlate with age and demographical parameters. Materials & Methods: The present prospective study was conducted at tertiary care center from June 2018 to June2019 and included 146 SCD patients including Sickle cell carriers/traits (SCT). A detailed comprehensive eye examination was performed to know the status of any ocular findings. Results: Most common peripheral retinal change seen was venous dilatation and tortuosity in 55.23% SCD and 29.5% of the SCT patients. The conjunctival signs were observed in 77.55% SCD and 27.2% of the SCT patients. Other complications such as iris atrophy, temporal disc pallor, chronic maculopathy, neovascularisation, retinal detachment were rare and none of the patients had anterior chamber signs.
    [Show full text]
  • UCLA Previously Published Works
    UCLA UCLA Previously Published Works Title The interplay of genetics and surgery in ophthalmic care. Permalink https://escholarship.org/uc/item/7nj489hp Journal Seminars in ophthalmology, 10(4) ISSN 0882-0538 Author Gorin, MB Publication Date 1995-12-01 DOI 10.3109/08820539509063801 Peer reviewed eScholarship.org Powered by the California Digital Library University of California Seminars in Ophthalmology ISSN: 0882-0538 (Print) 1744-5205 (Online) Journal homepage: http://www.tandfonline.com/loi/isio20 The Interplay of Genetics and Surgery in Ophthalmic Care Michael B. Gorin To cite this article: Michael B. Gorin (1995) The Interplay of Genetics and Surgery in Ophthalmic Care, Seminars in Ophthalmology, 10:4, 303-317, DOI: 10.3109/08820539509063801 To link to this article: http://dx.doi.org/10.3109/08820539509063801 Published online: 02 Jul 2009. Submit your article to this journal Article views: 9 View related articles Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=isio20 Download by: [UCLA Library] Date: 02 May 2017, At: 14:05 The Interplay of Genetics and Surgery in Ophthalmic Care Michael 6. Gorin LTHOUGH ONLY a minor component of dures. Genetic conditions that affect the eye A the surgical volume of ophthalmic care, directly may also affect the surgicaI outcomes of genetic disorders are among the most challeng- routine procedures. The coexistence of Fuch’s ing cases for the ophthalmic surgeon. Ophthal- endothelial dystrophy in patients undergoing mic surgery may be indicated to address specific routine cataract extraction can contribute to aspects of heritable disorders that involve the postoperative corneal edema.
    [Show full text]
  • Angioid Streaks and Optic Disc Drusen in Cherubism
    A RQUIVOS B RASILEIROS DE LETTERS Angioid streaks and optic disc drusen in cherubism: a case report Estrias angióides e drusas de disco óptico no Querubismo: um relato de caso Luiz Guilherme Marchesi Mello1,2 , Fábio Petersen Saraiva2 , Mario Luiz Ribeiro Monteiro1 1. Division of Ophthalmology, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil. 2. Specialized Medicine Department, Universidade Federal do Espírito Santo, Vitória, ES, Brazil. ABSTRACT | A 65-year-old female patient was referred to our drusas em ambas as áreas dos discos ópticos e membrana hospital for evaluation for cataract surgery. Her past medical neovascular sub-retiniana na mácula esquerda. A análise history included corrective jaw surgeries for facial deformities genética revelou mutação no gene SH3BP2 compatível com o that developed during infancy and persisted through early diagnóstico de Querubismo. A avaliação clínica e laboratorial adulthood. A complete ophthalmological examination revealed descartou outros distúrbios sistêmicos. O Querubismo é uma bilateral angioid streaks, drusen in both optic disc areas, and a doença óssea rara caracterizada pelo desenvolvimento de subretinal neovascular membrane in the left macula. Genetic lesões fibro-ósseas indolores na mandíbula e maxila durante analysis revealed a mutation in the SH3BP2 gene compatible a primeira infância. Os achados oftalmológicos nesta doença with the diagnosis of cherubism. Clinical and laboratory eva- estão principalmente relacionados ao envolvimento ósseo luation revealed no additional systemic disorders. Cherubism orbitário. Este artigo descreve pela primeira vez a ocorrência is a rare disease characterized by the development of painless de estrias angióides e drusas de disco óptico no Querubismo. fibro-osseous lesions in the jaws and the maxilla in early childhood.
    [Show full text]