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Contents 5 27 Editorial Photo Essay 55 Bilateral Anophthalmos in Two Brothers: A Case Report Garima Agrawal MS, D.C. Mehta MS Orthokeratology Kirti Singh Clinical Monthly Meeting Retina 9 69 Case 2- Posterior Sympathetic Ophthalmia in a Child Cornea & External Disease Clinical Talk - Future of Retinal Imaging, Lasers and Therapy Cyrus M. Shroff Benign Intracranial Hypertension Ronak P. Bhandari, Nayana A. Potdar, Chhaya A. Shinde, Pallavi G. Sarate 35 65 Ritesh Narula, Manisha Agarwal, S.P. Chaudhary, Priyata Seth Outcome of Endophthalmitis Cases in North East India Harsha Bhattacharjee, Satyen Deka, Hemlata Deka, Manav Jyoti Barman, Lokesh Jain, Kruto Kalita 21 Case 1- Management Dilemma in a case of Traumatic Glaucoma Julie Pegu, Suneeta Dubey, Manisha Agarwal OCT in Diabetic Macular Edema Jatin Ashar, Pradeep Venkatesh, Bhavin Shah 15 61 77 81 Abstract Membership Form Goldenhar Syndrome: A case report & review of literature Uday Gajiwala, Rajesh Patel, Manoj Gangwal 41 Pseudophakic Bullous Keratopathy Shreekant A. Damgude, B.P. Guliani 85 Tearsheet Target IOP Neuro-Ophthalmology 49 Shibal Bhartiya, Subha Bansal Relative Afferent Pupillary Defect Sumita Sethi, Mridula Mehta, Shibal Bhartiya, Sonia Bhargav www.dosonline.org 3 4 DOS Times - Vol. 14, No. 8, February 2009 Editorial Teachers in Ophthalmology Teaching in ophthalmology has changed in the recent times. This can be attributed to various factors. Learning earlier used to pass from the seniors to the juniors. However availability of the internet has facilitated procurement of information, but at the same time the person to person teaching is decreasing, as the students and teachers are depending more on the jargon of information available on the internet. The focussed teaching from person to person and the passing of skills is missing. There are no dedicated teaching sessions for post graduates and students who are left to themselves to learn the surgical skills. Similarly the clinical acumen in passing the diagnostic as well as the therapeutic skills from a senior to junior is missing. The investigative modalities are available which are analysing the anatomical structures at microscopic level, sometimes reading too much between the lines, sometimes making us aware of the subtle and preclinical cases. Research should not be machine based, it cannot be machine based; it has to be based on the benefit versus risks of a therapeutic trial or a particular procedure. We should not be dependent on machines and technologies for making a diagnosis or performing a surgical procedure. Similarly topics in conferences should not be machine or tool based but should be problem oriented. I would like to end with a quote that I read somewhere “The greatest danger in modern technology isn’t that machines will begin to think like people, but that people will begin to think like machines” Thanking you, Namrata Sharma Secretary, Delhi Ophthalmological Society www.dosonline.org 5 Welcome to the NEW www.dosonline.org Within one year of the successful re-launch of the DOS website, the site has again been completely revamped after taking into account feedback from the DOS members. Features of the new site include: 1. A signup feature, by which all new registered DOS members can receive the login details immediately in their email. 2. Forget password – DOS members who had forgotten their login password, can get it back in real time. 3. The DOS Times periodical has been uploaded as an online journal at www.dostimes.org. Registered members can access the various articles, as well as search for articles of their interest. 4. The various DOS Times videos have also been uploaded and can be viewed online. 5. Online Registration and online submission of abstracts and faculty topic form for the forthcoming DOS conference can be done at the DOS website. The NEW look Sign-up facility for new DOS members Online DOS TIMES at www.dostimes.org Online registration for the DOS Conference We encourage all members to use the DOS website and enjoy all the benefits. We welcome your suggestions and feedback in this regard. Please mail your opinions to [email protected]. 6 DOS Times - Vol. 14, No. 8, February 2009 Book Review The book "Clinical Ophthalmology Contemporary perspectives" edited by: Dr. A.K. Gupta and Dr. V. Krishna is a revised and updated version of previous edition. The book has chapters contributed by ophthalmologists of national and international repute. The book is divided into sections which include Ocular refraction, Ocular therapeutics, Cornea and Ocular surface disorders, Pediatric ophthalmology, Lens and Glaucoma, UVEA, Vitreous, Retina, Optic Nerve and Pathways, Ocular trauma, Orbit and Oculoplasty, Ocular diagnosis and Community Ophthalmology. Several new chapters on current topics have been included such as Intravitreal VEGF inhibitors and corticosteroids, Evaluation of a Patient with Orbital Disease, Basic Principles of Oculoplastic Surgery, Advances in Lacrimal Surgery, Fundus Fluorescein Angiography of Retinochoroidal Disorders and Determination of Intraocular Lens Implant Power. It is indeed a very useful conglomeration of both the basics of ophthalmology which help in understanding the fundamentals as well as the most recent developments in the field of ophthalmology. The book is highly informative, well organized and easy to read. This book on contemporary perspectives serves as unique teaching resource for all ophthalmologists especially for the budding ones. I think it is a wonderful book and should be a part of every resident’s library. It should be read by every senior resident and post graduate in ophthalmology. We owe a debt of thanks to Dr. A.K. Gupta & V. Krishna for an excellent contribution to the field of ophthalmology. Reviewed by: Dr. Namrata Sharma MD, DNB, MNAMS www.dosonline.org 7 Jatin Ashar MBBS, Pradeep Venkatesh MD, Bhavin Shah MBBS M acular edema is the most common cause of moderate visual loss in patients with diabetes. Macular edema is defined as a focal or diffuse area of retinal thickening which may or may not be associated with hard exudates. Depending on its location and size it can either a clinically significant or non-significant. A number of clinical methods and investigations have been used for long for the assessment of macular edema in diabetic patients. The slitlamp biomicroscopy has been the gold standard clinical method for looking for macular edema. Investigative modality like the fluorescein angiography have been used to quantify the intraretinal leakage in these patients, though FA is useful only as a guide for focal or grid treatment of thickened areas1. It is the retinal thickening and not the amount of leakage that is important in the determination of final visual outcome of patients with diabetic macular edema. OCT is a non contact, non invasive, micron resolution crosssectional study of retina which correlates very well with the retinal histology. Based on OCT DME can be classified into different pattern such as cystoid macular edema, spongy swelling of the retina, hard exudates, serous detachment, macular traction, taut posterior hyaloid membrane. Sponge like Thickening • most common change (Figure 1) • mostly in outer retinal layers while internal layers maintain their normal reflectivity • cross-sectional scans show swelling of the retina giving it a spongy appearance with increased retinal thickness, from a normal value of 174 ± 18µm in an area 500microns in diameter centered on fovea and a central foveal thickness of 152 ± 21 µm 2 • expanded areas of low reflectivity • backscattering seen from intraretinal fluid accumulation • ring of hard exudates with shadowing effect Cystoid Spaces Role of OCT in DME • second most common pattern (Figure 2) Diagnosis and Quantification of retinal thickening, extension of retinal thickness, macular volume, retinal morphology and vitreoretinal relationship. • intraretinal cystoid spaces • involves variable depth of retina • Defining the indication of surgery • Determine prognosis • Research tool • Follow up of patients and determining and quantifying the response to therapy (laser, medication, or surgery). Retina OCT in Diabetic Macular Edema Retinal Map Analysis Colour Map It is a topographic representation of the retinal thickness calculated from the linear scans. Figure 1: OCT showing sponge like diabetic macular edema Mean Thickness Map It provides an average thickness of each of 9 different sectors of macula and has a high reliability and reproducibility. Diabetic macular edema may present in different ways, either singly or in combinations of various patterns. The main pathology in DME is accumulation of fluid intraretinally. This is seen as reduced backscattering, seen most commonly in the outer retinal layers. Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi www.dosonline.org Figure 2: OCT showing cystoid diabetic macular edema 9 • has intervening septa in between • progresses gradually to involve the whole of retina Besides the above mentioned patterns other features such as hard exudates, epiretinal tractional membranes may also be found in patients with DME Serous Detachment Hard Exudates • increased macular thickness • high reflectivity spots with backscattering • hyporeflective areas corresponding to cysts in retina • • may cause subfoveal retinal detachment can be located in the neurosensory retina or in the subretinal space Tractional Retinal Detachment Epiretinal Membranes • foveo vitreal traction causing peak shaped detachment of fovea (Figure 3) • • sometimes satellite retinal detachment may also be found • laser can worsen this condition ERMs exerting an anteroposterior or tangential traction on the macula can be noted on the OCT scan. Taut Posterior Hyaloid Membrane • macular edema with foveal detachment • highly reflective signal arising form the inner retinal surface and extending towards the optic nerve or periphery • taut, thickened, shiny, glistening hyper-reflective membrane with striations on retina over the posterior pole with attachment to the disc and the top of the elevated macular surface • retinal thickness is greatly increased with intraretinal hyporeflective cyst like cavities (corresponding to fluid accumulation) Figure 3: OCT showing vitreomacular traction Summary of OCT based classification of DME8 Retinal Thickness Volume Morphology Epiretinal Traction 1. Fixation point: Normal: 150 + 20m Borderline: 170210 m Edema: > 210 m Meaningful only for diffuse edema involving at least the center and the 1st ring, and not for focal edema well-defined and continuous hyper-reflecting line over the inner retinal surface with at least one point of adhesion to the retina in at least one of the retinal map. 2. Central Zone: Normal: 170 + 20 m Borderline: 190 -230 m Edema: > 230 m Normal: 6.5 mm 3 + 1 Borderline: up to 8.0 mm 3 abnormal: > 8.0 mm3 Abnormal: > 8.0 mm 3 E1: simple thickening-Compact retinal thicknening, no clinically visible cystoid spaces E2: cystoid thickening - RT with cysts E2a: mild 2-4 central small cysts (horizontal diameter 150200m, vertical diameter 400 m) E2b: intermediate-cysts with petaloid configuration or with central cysts (horizontal diameter less than 300m, vertical diameter less than 600 m). E2c: severe-with coalescence of cysts with retinoschisis appearance E3: neuroepithelial detacment presence of subretinal liquid (non-reflecting space) above the hyperrflecting line of the pigmented epithelium isolated or associated with simple or cystoid retinal thickening. 3. Perifoveal and peripheral areas: Normal: 230 + 20m Boarderline: 250290m Edema: more than 290m 10 T0: absence of epiretinal hyper-reflectivity. T1: presence of a continuous line of flat hyper-reflectivity and adherent to the retina without significant retinal distortion. T2: presence of continuous line of hyper-reflectivity with multiple points of adhesion to the retina distortion. T3: antero-posterior traction with “gull wings” configuration. DOS Times - Vol. 14, No. 8, February 2009 Correlation of OCT with Histopathologic Changes in DME Histopathology • Intracytoplasmic swelling of Muller cells • Marked swelling of outer plexiform layer or Henle fiber layer • Liquefaction necrosis of the Muller cells (a) OCT • Sponge-like swellings • Prominent space with low reflectivity • Cystoid cavity formation (b) CSME is categorized by Kang et al6 into following categories Type 1: Thickening with homogenous optical reflectivity (55.2%) Type 2: Thickening with markedly decreased optical reflectivity in outer retinal layer (30.3%) Figure 4 (a-b): Pre and post triamcinolone injection OCT showing significant decrease in macular edema Type 3: Foveolar detachment without traction (11.7%) Type 4: Foveolar detachment with apparent vitreofoveal traction (2.8%) CSME corresponds to a RT more than 300 microns involving the central zone and / or RT more than 320 micron in the 1st ring. Edema confined to second ring is not considered clinically significant. • Tractional detachments • Superficial folds with ERM These patterns can be used in determining the treatment protocols for macular edema (Figure 4) • Not visible if completely attached or completely detached and too far • • can present with perifoveolar detachment, double convexity on cross sectional scan • can thickken as a sequale of fibrovascular proliferation Sponge like swelling • • cystoid swelling • • IVTA Serous detachment • • focal grid / laser laser & ppv tractional retinal detachment and taut posterior hyaloid • ppv Changes on OCT in DME Intraretinal changes Diffuse swelling: retinal swelling, no cystoid space, outerplexiform and outer nuclear layers thicken and show hyporeflectivity Posterior Hyaloid Membrane Preretinal Hemorrhage Mask the features of edema on OCT Summary of OCT based classification of DME 8 Ultra Resolution (UR) OCT4 Advantages over Stratus OCT • Ultrahigh resolution make the small distortion in retinal contour are more evident. • Axial resolution of 2 to 3 µm making the visualization of intra retinal layers simpler. • Better visualization of hard exudates on UR-OCT Cystoid spaces: hyporeflective spaces, in Henie’s and outer plexiform layer • Small cystic changes in the ganglion cell and nuclear cell layers is evident better than stratus OCT Foveolar detachment: foveal or extrafoveal cystic spaces may lead to detachment of foveola. • Thin epiretinal membrane not evident on Stratus OCT can be visualized using UR-OCT. Hard Exudates: hyperrective intraretinal deposits, outer plexiform layer • Small disruption in the photoreceptor inner segment outer segment region is also evident on UR OCT. Inner Retinal Border Role of OCT vs FA in DME • Foveolar edema-flattening of foveal pit • Dome shaped elevation of inner retinal boundary • Concave slopes due to rise of macular center FA assess the vascular leakage and quantifies it in macular edema, however, it is the actual thickness of the retina that correlates with the final visual outcome of the patient3. www.dosonline.org 11 Correlation of OCT and FA Morphologic 9 According to the modified Otani scheme , OCT findings of macular edema are characterized into four types: DRT 0.69 + 0.41 20/97 Type 2: thickening of the fovea with markedly decreased optical reflectivity in outer layers CME 0.79 + 0.39 20/123 SRD without PHT 0.82 + 0.34 20/132 Type 3: thickening of the fovea with subfoveal fluid accumulation and distinct outer border of detached retina PHR with TRD 1.0 + 0.24 20/200 PHT without TRD 0.77 + 0.44 20/118 Type 1: thickening of the fovea with homogenous optical reflectivity Type 3A: without foveal traction DRT: diffuse retinal thickening; CME: diabetic cystoid macular edema; SRD: serous retinal detachment; PHT: posterior hyaloidal traction; TRD: traction retinal detachment. Type 3B: with apparent vitreofoveal traction Fluorescein angiography findings can be categorized into three types: Type 1: focal leakage with well defined focal areas of leakage References Type 2: diffuse leakage which is widespread and ill defined 1. Giacomo Panozzo, Elena Gusson, Barbara Parolini and Andrea Mercanti.. Role of OCT in the diagnosis and follow up of diabetic macular edema. Seminars in Ophthalmology 2003, Vol. 18, No. 2, pp. 74–81 2. The eyes with previous macular focal or grid photocoagulation tend to show an increased proportion of OCT type 2 and OCT type 3A compared with eyes without prior photocoagulation. Bandello, D. Roman Pognuz,, A. Polito, A. Pirracchio, F. Menchini, M. Ambesi,. Diabetic macular edema: classification, medical and laser therapy. Seminars in Ophthalmology2003, Vol. 18, No. 4, pp. 251–258 3. Focal leakage on FA is closely related with type 1 or homogenous foveal thickening on OCT, while diffuse or cystoid leakage is related to either type 2 or type 3A or fluid collection predominantly in outer retinal layers or subretinal space. Ozdek SC, Erdinc MA, Gurelik G, Aydin B, Bahceci U, Hasanreisoglu B. Optical coherence tomographic assessment of diabetic macular edema: comparison with fluorescein angiographic and clinical findings.. Ophthalmologica. 2005 Mar-Apr;219(2):86-92. 4. Srinivasan VJ, Wojtkowski M, Witkin AJ, Duker JS, Ko TH, Carvalho M, Schuman JS, Kowalczyk A, Fujimoto JG. High-definition and 3-dimensional imaging of macular pathologies with high-speed ultrahigh-resolution optical coherence tomography. Ophthalmology.2006 Nov;113(11):2054.e1-14. Type 3: diffuse cystoid leakage with pooling in cystic spaces of macula Focal leakage on FA is mostly associated with non proliferative diabetic retinopathy while diffuse leakage with proliferative diabetic retinopathy. OCT vs RTA5 • OCT has excellent sensitivity and considerable specificity making it an excellent general screening tool for diabetic macular edema. 5. Goebel W, Franke R. Retinal thickness in diabetic retinopathy: comparison of optical coherence tomography, the retinal thickness analyzer, and fundus photography. Retina. 2006;26:49–57. • RTA has a higher specificity making it the tool to confirm the indication for laser therapy. 6. The correlation between fluorescein angiographic and optical coherence tomographic features in clinically significant diabetic macular edema. Kang SW, Park CY, Ham D-I. Am J Ophthalmol 137:313–322 7. Kim BY, Smith SD, Kaiser PK. Optical coherence tomographic patterns of diabetic macular edema. Am J Ophthalmol. 2006 Sep;142(3):405-12 8. Panozzo G, Parolini B, Gusson E, Mercanti A, Pinackatt S, Bertoldo G, Pignatto S. Diabetic macular edema: an OCT-based classification.. Semin Ophthalmol. 2004 Mar-Jun;19(1-2):13-20. 9. Otani T, Kishi S, Maruyama Y: Patterns of diabetic macular edema with optical coherence tomography. Am J Ophthalmol 1999;127:688693. Problems in Performing OCT in Diabetic Patients • Poor papillary dilatation • Media opacities in form of cataract, vitreous hemorrhage may affect the scan quality Mean Visual Acuity Association with Morphological Subtypes of DME7 V/A for patients without CME increased by 0.16 log MAR units/ 100 micron increase in retinal thickness. This reflects on the Snellen acuity from 20/20 to 20/30 In the presence of CME, the mean increase is of 0.40U on logMAR due to CME, corresponding to a change in Snellen acuity from 20/ 20 to 20/50. However, increased retinal thickness along with CME also reduces the acuity, but to a lesser extent than without CME, in such a way that a 100micron change in retinal thickness corresponds to change in log MAR of 0.085U, corresponding to a visual acuity change from 20/20 to 20/25. 12 Mean Visual Acuity Mean Visual Acuity (logMAR) (Snellen) First Author Jatin Ashar MBBS DOS Times - Vol. 14, No. 8, February 2009 Retina Outcome of Endophthalmitis Cases in North East India *Harsha Bhattacharjee MS, *Satyen Deka MS, DNB, *Hemlata Deka MS, *Manav Jyoti Barman DO, DNB, **Lokesh Jain MS, *Kruto Kalita MS I n the study 178 patients were evaluated and treated by retina specialists at our centre between 2003-2008. The diagnosis of endophthalmitis was based on presence of pain, inflammation including anterior chamber cellular reaction and flare and vitreous cells,corneal involvement, decrease in visual acuity and loss of media clarity with or without the presence of hypopyon. At all visits, the ocular examinations included best corrected visual acuity, slitlamp biomicroscopy (anterior segment examination and 90 D examination of posterior segment) and indirect ophthalmoscopy for posterior segment examination. Posterior segment evaluation with B scan USG was carried out where ever possible.Information collected include history of the precipitating cause,visual status at presentation,interval between development of symptoms and treatment at our centre,microbiological profile of organisms cultured , interventions undertaken and final visual outcome and ocular status. The primary treatment was always as per the recommendation of the Endophthalmitis Vitrectomy Study (EVS). Briefly, this included injection of intravitreal antibiotics to all patients where the presenting visual acuity was hand movement close to face (HMCF) or better and eyes with vision less than HMCF but with corneal involvement severe enough to preclude vitreous surgery. In vitrectomy cases the primary goal of surgery was to remove the core vitreous along with vitreous debris. Before vitrectomy was started 0.5 to 0.1 ml of undiluted vitreous sample was collected from the mid vitreous by manual aspiration with a 2 ml syringe for microbiological study. After that the infusion fluid was switched on and vitrectomy started. Intravitreal antibiotics were injected at the end of the procedure. Immediate inoculation of the undiluted vitreous samples were done onto blood agar, chocolate agar, non-nutrient agar, Sabouraud’s dextrose agar, thioglycolate and brain heart infusion broth. Sabouraud’s agar plates were incubated at 25 deg celcius to enhance growth of fungi while rest of the plates were incubated at 37 deg celcius. Blood agar plates were incubated under aerobic and anaerobic conditions, and chocolate agar was incubated with 5% carbon dioxide. Gram’s stain, Giemsa stain and KOH stain with calcoflour white were done. A culture was considered positive when there was growth of the same organism on two or more media, confluent growth at the site of inoculation on one solid medium, or growth in one medium with consistent direct microscopy findings. The final diagnosis was made as bacterial, fungal, polymicrobial or negative based on the above criteria. Polymicrobial infection was defined as growth on culture of more than one species or strains of bacteria or a positive bacterial culture *Sri Sankaradeva Nethralaya BeltolaGuwahati – 78102 (Assam) **Jain Eye Hospital 204-A GT Road,Shahdara, Delhi www.dosonline.org along with a positive fungal culture from a single or subsequent sample from the same eye. Intravitreal and topical antibiotics were given to all patients following standard protocol. For empiric gram negative organism coverage intravitreal amikacin (0.4 mg in 0.1 ml) or ceftazidime (2.25 mg in 0.1 ml) were given. For empiric gram positive organism coverage Vancomycin (1mg in 0.1 ml) was given. In the case of fungal micro organism being isolated intravitreal amphotericin B (0.005 mg in 0.1 ml) was given. Results Out of 178 endophthalmitis cases 104 (58% ) cases were post operative, 53 (30% ) post traumatic, 11 (6%) endogenous and 10(6%) post corneal ulcer perforation cases. Out of 104 postoperative endophthalmitis cases 9 cases were diagnosed as fibrinous uveitis. There were 3 post operative cases from our centre. Postoperative endophthalmitis The predisposing surgeries included phacoemulsification surgery (21 cases, 22%), ECCE (31 cases, 33%), SICS (29 cases,31%), phaco with trabeculectomy (1 case,1%), trabeculectomy with MMC (11 cases,12%) and VR procedures (2 cases, 2%) (Table 1). The duration between the precipitating surgery and presentation at the institute was less than 24 hours in 4 (4.2%), between 1 and 7 days in 33 (34.7%) and greater than 7 days in 58 (61%) of cases (Table 2). The presenting vision was light perception or worse in 45(47.3%), hand movement in 25 (26.3%) and better than hand movement in 25 (26.3%) of cases (Table 3). Vitreous biopsy culture was positive in 52 (54.7%) cases, with bacteria cultured in 50 (52.6%) and fungi in 8 (8.4%) of cases (sum is greater than the total of 54.7% because of polymicrobial cultures). The microbiological profile is presented Table 1: Types of Surgery Type of surgery No: of cases Phaco IOL 21 ECCE IOL 31 SICS IOL 29 Phaco + Trab 1 Trab + MMC 11 VR procedures 2 Total 95 15 Table 2: Duration of presentation of cases (Postoperative endophthamitis cases) Table 4: Microbiological spectrum of post operative endophthalmitis cases Type of micro organism Table 3: Presenting visual acuity and final visual acuity (Post operative endophthalmitis cases) in Table 4. Out of 104 cases 53 cases received intravitreal antibiotics only while 42 cases received both intravitreal antibiotics and vitrectomy. No: cultured Gram pos. Cocci 20 Gram neg. Cocci 1 Gram pos. Bacilli 8 Gram neg. Bacilli 15 Fungi 8 Negative 43 (45.2%) Polymicrobial 5.63% Table 5:Presenting visual acuity and final outcome Table 6: Duration of presentation and final outcome At followup 38 (40%) of 95 patients had improvement in vision while in 57 (60%) cases the vision remained the same or deteriorated. The anatomical outcome was poor (pthisis bulbi, evisceration or enucleation) in 12 (12.63%) of cases. In the study it is seen that the final visual outcome depended on the vision at presentation and the time interval between the precipitating surgery and the initiation of treatment at the institute. Post traumatic endophthalmitis The number of post traumatic endophthalmitis cases in the study is 53. The presenting vision was light perception or worse in 36 (67.9%), hand movement in 15 (28.3%) and better than hand movement in 2 (3.7%) of cases (Table 5). The duration between the precipitating trauma and presentation at the centre was less than 24 hours in 5 (9.4%), between 1 and 7 days in 26 (49%) and greater than 7 days in 22 (41.5%) of cases (Table 6). Vitreous biopsy culture was positive in 39 (73.5%) cases, with bacteria cultured in 36 (67.9%) and fungi in 3 (5.7%) of cases (sum is greater than the total of 73.5% because of polymicrobial cultures). Out of 53 cases 26 cases received intravitreal antibiotics only while 27 cases received both intravitreal antibiotics and vitrectomy. Table 7 shows the microbiological profile of organisms cultured from post traumatic endophthalmitis cases. 16 At followup 18 (34.9%) of 53 patients had improvement in vision while in 35 (65.1%) cases the vision remained same or deteriorated. The anatomical outcome was poor (phthisis bulbi, evisceration or enucleation) in 14 (26.4%) of cases. Endogenous endophthalmitis The number of endogenous endophthalmitis cases in the study is 11. The presenting vision was light perception or worse in 7 (63.6%), hand movement in 3 (27.2%) and better than hand movement in DOS Times - Vol. 14, No. 8, February 2009 Table 7: Microbiological spectrum (Post traumatic endophthalmitis cases) perception or worse in 6 (60%), hand movement in 2 (20%) and better than hand movement in 2 (20%) of cases (Table 11). The duration between the development of symptoms and presentation at the institute was between 1 and 7 days in 5 (50%) and greater than 7 days in 5 (50%) of cases (Table 12). Vitreous biopsy culture was positive in only 5 (50%) cases, with bacteria cultured in 4 (40%) and fungi in 1 (10%) of cases. Out of 10 cases 8 cases received intravitreal antibiotics only while 2 cases received both intravitreal antibiotics and vitrectomy. Table 13 shows the microbiological profile of organisms cultured from endophthalmitis cases following corneal ulcer perforation. Type of micro organism No: cultured Gram pos. Cocci 16 Gram neg. cocci 5 Gram pos. Bacilli 12 Gram neg. bacilli 3 Fungi 3 Negative 12 (30.8%) At followup 3 (30%) of 10 patients had improvement in vision while in 7 (70%) cases the vision remained same or deteriorated. The anatomical outcome was poor in 3 (30%) cases. Polymicrobial 9.5% Discussion 1 (9%) of cases (Table 8). The duration between the precipitating surgery and presentation at the institute was between 1 and 7 days in 5 (45.5%) and greater than 7 days in 6 (54.5%) of cases (Table 9). Vitreous biopsy culture was positive in only 2 (18.1%) cases, with bacteria cultured in 2 (18.1%) and fungi in 1(9%) of cases (sum is greater than the total of 18.1% because of polymicrobial cultures). Out of 11 cases 7 cases received intravitreal antibiotics only while 4 cases received both intravitreal antibiotics and vitrectomy. Table 10 shows the microbiological profile of organisms cultured from post traumatic endophthalmitis cases. At followup 5 (45.4%) of 11 patients had improvement in vision while in 6 (54.5%) cases the vision remained same or deteriorated. The anatomical outcome was poor in 3 (27.2%) cases. Endophthalmitis following corneal ulcer perforation In this study the number of cases of endophthalmitis following corneal ulcer perforation is 10. The presenting vision was light The main objective was to study the profile of endophthalmitis cases presenting at the institute and to observe any clinical presentation features associated with poor visual outcome. The microbiological profile of organisms cultured from the involved eyes was also studied. Among the clinical features at presentation, importance was laid on visual acuity at presentation and duration between onset of symptoms and presentation at the institute and to observe whether these two factors had any bearing on the final outcome. For the purpose of analysis the presenting visual acuity was divided into three groups. They constituted vision less than or equal to light perception, hand movement and better than hand movement. The duration of presentation was also divided into three categories, which were a duration of less than 24 hours, between 1 and 7 days and greater than 7 days. Among the post operative cases the number of ECCE cases were highest (31 cases) followed by SICS (29 cases). Visual acuity of less than or equal to light perception was present in 45 (47.3%) cases while vision of hand movement and greater than hand movement accounted for 25 cases (26.3%d”) each. Out of 45 cases having an Table 8: Presenting visual acuity and final visual acuity (Endogenous endophthalmitis ) Presenting visual acuity No: of cases Improved vision Same/deteriorated vision Poor anatomical outcome d” LP 7 (63.6%) 3 4 3 HM 3 (27.2%) 2 1 > HM Total cases 1 (9%) 11 1 5 (45.4%) 6 (54.5%) 3 (27.2%) Table 9: Duration of presentation and visual outcome (Endogenous endophthalmitis) Duration of presentation No: of cases Improved vision Same/deterioratedvision Poor anatomical outcome < 24 hours 1 – 7 days 5 (45.5%) 3 2 > 7 days 6 (54.5%) 2 4 Total cases www.dosonline.org 11 5 (45.4%) 6 (54.5%) 3 3 (27.2%) 17 Table 10: Microbiological spectrum (Endogenous endophthalmitis) Type of micro organism No: cultured Gram pos. Cocci 2 Gram neg. Bacteria presenting between 1 and 7 days, 19 (73%) of the cases had a poor outcome. Among the culture positive cases (39) there was a poor outcome in 27(69.2%) cases while improvement occurred in12 (30.8%) of the cases. As such poor presenting visual acuity and a culture positivity for any organism are indicative of an unfavourable outcome in post traumatic cases. This is in agreement with numerous studies conducted in different parts of the world. In the endogenous and post corneal ulcer perforation cases the study groups were too small to detect any specific trends. Gram pos. Bacilli Among the different causes of endophthalmitis the two most important are ocular surgery and trauma. The two however are different entities. In post traumatic endophthalmitis, the outcome will largely depend on the circumstances of injury vis a vis the extent of wound, type of injury, retained IOFB, nature of the IOFB- factors which have not been included in this study. However Gram neg. Bacilli Fungi 1 Negative 9 (81.8%) Table 11: Presenting visual acuity and final visual acuity ( endophthalmitis following corneal ulcer perforation ) Presenting visual acuity No: of cases Improved vision Same/deteriorated vision Poor anatomical outcome d” LP 6 (60%) 1 5 3 HM 2 (20%) 1 1 > HM 2 (20%) 1 1 10 3 (30%) 7 (70%) Total cases 3 (30%) Table 12: Duration of presentation and visual outcome (endophthalmitis following corneal ulcer perforation) Duration of presentation No: of cases Improved vision Same/deterioratedvision Poor anatomical outcome < 24 hours 18 1 – 7 days 5 2 3 > 7 days 5 1 4 3 Total cases 10 3 (30%) 7 (70%) 3 (30%) initial visual acuity d” LP there was visual improvement in only 5 (11.1%) cases while in 40 (88.8 %) cases the vision remained the same or deteriorated. There was poor anatomical outcome in 7 (15.5%) cases. Majority of the cases 58 (61 %) presented at the institute after 7 days and among them the outcome was poor in 41 (70.6 %) cases. Among culture positive cases 67% had a poor outcome while improvement was present in 33% cases. Therefore three factors indicative for an unfavourable outcome in postoperative cases in this study were a poor presenting visual acuity, delayed presentation and culture positivity for any organism. This has been established in a large number of studies. Table 13: Microbiological spectrum (endophthalmitis following corneal ulcer perforation) Post traumatic cases presenting with a vision of d” LP was 36 (67.9 %) out of which 27 (75 %) cases had a poor visual outcome. The anatomical outcome was poor in 11 (30.5 %) cases. The number of cases presenting late (> 7 days) were 22 (41.5%) out of which 15 (68.5%) cases had no improvement of vision. The anatomical outcome was poor in 8 (36.3%) cases. The outcome was also poor in cases presenting within 7 days. Out of 26 cases Type of micro organism No: cultured Gram pos. Cocci 3 Gram neg. cocci 1 Gram pos. Bacilli 1 Gram neg. bacilli 1 Fungi 1 Sterile culture 5 (50%) At followup 3 (30%) of 10 patients had improvement in vision while in 7 (70%) cases the vision remained same or deteriorated. The anatomical outcome was poor in 3 (30%) cases. DOS Times - Vol. 14, No. 8, February 2009 as regards post operative Endophthalmitis one can go a long way in saving the eye by early recognition of the problem and timely intervention. 7. Gupta A, Gupta V, Gupta A, Dogra MR, Pandav SS. Spectrum and clinical profile of post cataract surgery endophthalmitis in North India. Indian J Ophthalmol 2003;51:139-45. References 8. Javitt JC, Vitale S, Canner JK, Street DA, Krakauer H, McBean M, et al. National outcomes of cataract extraction. Endophthalmitis following in-patient surgery. Arch Ophthalmol 1991;109:1085-89. 9. TThompson WS, Rubsamen PE, Flynn HW, Schiffman J, Cousins SW. Endophthalmitis after penetrating trauma: risk factors and visual acuity outcomes. Ophthalmology 1995;102:1696-701. Jalali S, Das T, Gupta S. Presumed noninfectious endophthalmitis after cataract surgery. J Cataract Refract Surg 1996;22:1492-97. 2. Speaker MJ, Menikoff JA. Prophylaxis of endophthalmitis with topical povidone- iodine. Ophthalmology 1992;98:1769-75. 3. Endophthalmitis Vitrectomy Study Group. Results of the endophthalmitis study group. A randomized trial of immediate vitrectomy and of intraocular antibiotics for the treatment of postoperative bacterial endophthalmitis. Arch Ophthalmol 1995;113:1479-96. Wisniewski SR, Hammer ME, Grizzard WS, Kelsey SF, Everett D, Packo KH, et al. An investigation of the hospital charges reltaed to the treatment of endophthalmitis in the endophthalmitis vitrectomy study. Ophthalmology 1997;104:739-45. 5. Kunimoto DY, Das T, Sharma S, Jalai S, Majji AB, Gopinathan U, et al. Microbial spectrum and susceptibility of isolates. Part I. Postoperative endophthalmitis. Am J Ophthalmol 1999;128:240-42. 6. Anand AR, Therese LK, Madhavan HN. Spectrum of aetiological agents of postoperative endophtalmitis and antibiotic susceptibility of bacterial isolates. Indian J Ophthalmol 2000;48:123-28. 11. Thompson JT, Parver LM, Enger CL, Mieler WF, Liggett PE. Infectious endophthalmitis after penetrating injuries with retained intraocular foreign bodies. Ophthalmology 1993;100:1468-74. 12. Alfaro DV, Roth D, Liggett PE. Post-traumatic endophthalmitis: Causative organisms, treatment, and prevention. Retina 1994;14:206-11. 13. Jalali S, Das T, Majji AB. Hypodermic needles: a new source of penetrating ocular trauma in Indian children. Retina 1999;19:21317 14. Das T,Kunimoto DY,Sharma S,Jalali S, Majji AB,Gopinathan U,Rao TN:Relationship between clinical presentation and visual outcome in postoperative and post traumatic endophthalmitis in South Central India.IJO 2005;53:5-16 First Author Harsha Bhattacharjee MS Answer Quiz No. 8 Extra Word: SYNDROME 6. 2. DUANE 1. MOBIUS BROWN 7. 3. CENTURION HORNER WAARDENBURG 8. TURNER 4. www.dosonline.org REITER 4. 10. Williams DF, Mieler WF, Abrams GW, Lewis H. Results and prognostic factors in penetrating ocular injuries with retained intraocular foreign bodies. Ophthalmology 1988;95:911-16. 5. 1. 19 Ronak P. Bhandari MS, Nayana A. Potdar MS, Chhaya A. Shinde MS, Pallavi G. Sarate MS T he presentation of a patient with increased intracranial pressure and papilloedema is an emergency. Although this scenario may be the harbinger of a brain mass, other clinical entities should be considered. A significant number of patients presenting in the above fashion are diagnosed with the syndrome of pseudotumor cerebri. Although idiopathic intracranial hypertension, pseudotumor cerebri, and benign intracranial hypertension are synonymous with this diagnosis, the preferred term is idiopathic intracranial hypertension. abduction. It was bilateral sixth nerve palsy (Figure 2, 3). Anterior segment was within normal limits except pupils were bilaterally equal, 3 mm in size and sluggishly reacting to light. On fundus examination both eyes showed that the disc margins were blurred, discs were hyperemic (Figure 4). Retinal veins were dilated, tortuous. Spontaneous venous pulsations were absent. There were flame shaped retinal hemorrhages near the disc. There was bilateral severe papilloedema. Visual fields was not possible due to uncooperative patient. The diagnostic criteria include the following: symptoms and signs restricted to those of elevated intracranial pressure; normal findings on neuroimaging studies, excluding nonspecific findings of raised intracranial pressure; and increased cerebrospinal fluid pressure with a normal composition. A CT scan brain was done which was within normal limits (Figure 5). Routine blood investigations like complete blood count, blood sugars, liver, renal functions and serum electrolytes were within normal limits. X-ray chest was within normal limits. Patient Retina Benign Intracranial Hypertension Case Report A 19 year old male presented with frontal headache usually more in the morning since 1 month, which aggravated 2 days before presentation. He had deviation of eyes since 1 week which was noticed by his parents followed by diplopia and blurring of vision then. There was no history of fever/ loss of consciousness/ convulsion. There was no history of trauma or any medication. On examination the vitals parameters were stable. Vision of the patient was FC 10 ft in both eyes. On external ocular examination, both eyes showed evidence on non comitant convergent squint 20 degrees (Figure 1). Abduction was restricted in both eyes. In the right eye there was moderate restriction and in the left eye there was severe restriction of Figure 2: Right Lateral Rectus Palsy Figure 1: Pre-Op Photo on Presentation Figure 3: Left Lateral Rectus Palsy Lokmanya Tilak Memorial General Hospital, Sion Mumbai www.dosonline.org 21 Figure 4: Papilloedema Figure 5: Normal CT Scan was transferred to neuromedicine. CSF cytology and biochemical studies done were within normal limits. CSF culture was negative and meningitis was ruled out. However CSF manometry showed pressures greater than 45 cm H2O. (Normal is between 15-25 cm H2O). The patient was started on intravenous mannitol and oral glycerol as measures to reduce the intra cranial pressure. The ventricular system was normal on MRI brain with orbit. A diagnosis of pseudotumor cerebri was established and patient was taken for Right sided theco-peritoneal shunt surgery. (Figure 8) On post op day 2 the CSF pressure was recorded as 22 cm H2O and the symptoms of the patient improved dramatically (Figure 6). The vision on day 7 was both eyes 6/24. On fundus examination the disc edema was less in both eyes (Figure 7). Retinal hemorrhages were getting resolved. Disc hyperemia was reduced. Papilloedema was resolving in both eyes. brain tumor without a brain tumor being present (pseudo meaning false). The space around the brain is filled with water-like fluid. This space containing the fluid cannot expand. It is high pressure in this space that produces the symptoms of idiopathic intracranial hypertension (IIH). The vision after 6 weeks was 6/9 uncorrected in both eyes. The patient was advised regular follow up. His vision and field changes were monitored. Discussion Idiopathic Intracranial Hypertension is a condition of high pressure in the fluid around the brain. It is also known as pseudotumor cerebri because there are some of the signs and symptoms of a Incidence Studies of American-based populations have estimated that the incidence of pseudotumor cerebri ranges from 0.9-1.0 per 100,000 in the general population. This incidence rate increases to 1.6-3.5 per 100,000 in women, and 7.9-19 per 100,000 in women who are overweight.1 A strong predilection of this disease exists for women. In the literature, the female-to-male ratio ranges from 2:1 to 10:1. Although idiopathic intracranial hypertension may affect individuals of any age, most patients with this disease present in the third decade of life. Idiopathic intracranial hypertension does occur in the pediatric population; these younger patients are often not obese. Symptoms and Signs The symptoms most commonly reported by IIH patients followed by their frequency are Figure 6: Post Operative Photo 22 DOS Times - Vol. 14, No. 8, February 2009 Figure 8: Scar of Surgery Figure 7: Post-Op Fundus Headache (94%): Headache is present in nearly all patients with IIH and is the usual symptom for which patients seek medical attention. The headaches of the IIH patient are usually severe throbbing and daily. They are different from previous headaches, may awaken the patient and usually last for hours. Nausea and vomitting less common. The headache is often the worst head pain ever experienced. Although uncommon, the presence of pain behind the eyeball that is worsened with the movements of the eyes can occur. With Valsalva maneuver the intra cranial pressure increases and hence the symptoms. Transient visual obscurations: Visual obscurations are episodes of transient blurred vision that usually last less than 30 seconds and are followed by full recovery of vision. Visual obscurations occur in about 3/4 of IIH patients. The attacks may be involving one or both eyes. They are not correlated with the degree of intracranial hypertension or with the extent of optic nerve swelling. Visual obscurations do not appear to be associated with poor visual outcome. Table 1: Modified Dandy Criteria2 • Signs & symptoms of increased ICP - CSF pressure >25 cmH2O • No localizing signs with the exception of abducens nerve palsy • Normal CSF composition • Normal to small (slit) ventricles on imaging with no intracranial mass to subretinal hemorrhages are poor visual prognostic signs. In some instances, the disc swelling is asymmetric, or, rarely, the appearance of the optic nerve may be relatively normal. Pulsatile intracranial noises: Pulsatile intracranial noises or pulsesynchronous tinnitus is common in IIH. The sound is often unilateral. In patients with intracranial hypertension, compression of the jugular vein on the side of sound abolishes it. The periodic compressions were thought to convert the laminar blood flow to turbulent. If left untreated, chronic disc swelling eventually leads to clinically significant visual loss. Although all patients present with enlarged blind spots during their initial perimetry, uncontrolled papilledema results in progressive peripheral visual field constriction or nerve fiber bundle defects (eg, nasal depression, nasal steps, arcuate scotomas). The central visual field is affected in end-stage chronic papilledema. Visual loss: The most serious problem patients have is vision loss. About 5% of patients go blind in at least one eye. These are usually patients who do not return for follow-up evaluation. Sudden loss of central vision may result from an associated anterior ischemic optic neuropathy, a vascular occlusion, or an associated subretinal neovascular membrane. Diplopia: Patients who present with double vision most frequently complain of horizontal displacement of the images. Vertical diplopia is rare, but it has been reported The diplopia noted in patients with idiopathic intracranial hypertension is invariably due to unilateral or bilateral sixth nerve palsy. These cranial nerve palsies diminish with the lowering of the intracranial pressure. Occasionally, patients with diplopia present with oculomotor or trochlear nerve palsy. In rare instances, vertical diplopia is due to a skew deviation. Physical The most significant finding in patients with this disease is bilateral disc edema secondary to the increased intracranial pressure. This papilledema varies from patient to patient and is indistinguishable from optic nerve swelling caused by intracranial space-occupying lesions. In more pronounced cases of disc swelling, macular involvement with subsequent edema and diminished central vision may be present. High-grade and atrophic papilledema in addition www.dosonline.org Causes Most cases of pseudotumor cerebri occur in young women who are obese and, less frequently, in men who are otherwise healthy. If this disorder presents in an individual who is not overweight, ruling out associated risk factors is necessary. 23 Risk factors • Drusen of the optic nerve heads Exogenous substances • Malignant hypertension • The list of exogenous substances associated with idiopathic intracranial hypertension is extensive. Although an association exists between these substances and this disorder, the exact causal relationship is somewhat lacking in the literature. • Bilateral infiltrative/infectious/inflammatory optic neuropathy • Bilateral anterior ischemic optic neuropathy • Bilateral optic nerve papillitis Exogenous substances associated with idiopathic intracranial hypertension include amiodarone, antibiotics (eg, nalidixic acid, penicillin, tetracycline), carbidopa, levodopa, chlordecone, corticosteroids (eg, topical, systemic), cyclosporine, danazol, growth hormone, indomethacin, ketoprofen, lead, leuprolide acetate, levonorgestrel implants, lithium, oral contraceptives, oxytocin, perhexiline, phenytoin, and vitamin A (>100,000 U/d)/retinoic acid. • Bilateral optic nerve tumors (eg, glioma, meningioma). • Systemic diseases The main complication of this disorder is progressive optic neuropathy. Despite timely treatment, some patients develop an optic neuropathy related to the optic nerve edema. Generally, this dysfunction presents in a progressive fashion with constriction of the peripheral visual field; worsening nerve fiber bundle visual field defects; a decrease of color vision; and, in end-stage disease, a drop in the central visual function. Occasionally, a patient may develop an acute loss of vision due to ischemic optic neuropathy associated with papilledema. • Pathophysiology • Ironically, withdrawal from corticosteroids may result in idiopathic intracranial hypertension. The following diseases have been associated with idiopathic intracranial hypertension: anemia, chronic respiratory insufficiency, familial Mediterranean fever, hypertension, multiple sclerosis, polyangiitis overlap syndrome, psittacosis, renal disease, Reye syndrome, sarcoidosis, systemic lupus erythematosus, and thrombocytopenic purpura. Disorders of cerebral venous drainage • Cerebral venous compression by extravascular tumors or secondary thrombosis. • Radical neck dissection, even if completed only on the right side (predominant drainage from the head is via the right jugular vein). Spontaneous recanalization usually occurs, but, if delayed, chronic papilledema may result. Endocrine disturbances • Pregnancy is occasionally associated with idiopathic intracranial hypertension. This disorder can present at any stage of pregnancy. Differential Diagnosis Bilateral optic nerve head edema is presumably due to increased intracranial pressure secondary to an intracranial tumor until proven otherwise. Ruling out malignant hypertension prior to pursuing the diagnostic trail of pseudotumor cerebri is always worthwhile. If the patient's blood pressure is normal or minimally to moderately elevated, obtaining urgent neuroimaging studies to rule out a space-occupying lesion or a dural sinus thrombosis is essential. If the neuroimaging study does not demonstrate any pathology and if a lumbar puncture demonstrates a raised opening pressure, then one of the above-mentioned causes of increased intracranial pressure must be considered. In the absence of a specific offending agent, the diagnosis of the patient is presumably idiopathic intracranial hypertension. • 24 Complications Pseudopapilledema The pathophysiology of this disorder is unclear. A relative resistance to the absorption of cerebrospinal fluid across the arachnoid villi is widely presumed to be present. Other theories support an abnormality in the cerebral circulation with a resulting increase in the brain's water content. The subsequent increase in the intracranial pressure is transmitted to the structures within the intracranial cavity, including the optic nerves. The disease commonly occurs in women who are overweight. The role of obesity in this disorder is unclear. Obesity has been proposed to increase intra-abdominal pressure, which, in turn, raises cardiac filling pressures. This rise in pressure leads to impeded venous return from the brain (due to the valveless venous system that exists from the brain to the heart) with a subsequent elevation in intracranial venous pressure. If not treated appropriately, chronic interruption of the axoplasmic flow of the optic nerves with ensuing papilledema due to this pressure may lead to irreversible optic neuropathy.3 Mortality and Morbidity The morbidity of this disorder is mainly related to the effects of papilledema. If left untreated, long-standing disc edema results in an irreversible optic neuropathy with accompanying constriction of the visual field and loss of color vision. In end-stage papilledema, central visual acuity is also involved.4 Conclusion The diagnosis of IIH is made by identifying the typical symptoms of the disease along with documentation of a high spinal fluid pressure (done during a gaurded spinal tap). The neurologic examination is normal except for the presence of papilledema. Sometimes double vision occurs, caused by limitation of lateral eye movement. Lastly, neuroimaging procedures such as CT scans or MRI scanning are normal.The best way to prevent visual loss is to test vision regularly as well as assess visual fields. Patients should be followed frequently with tests of vision until the doctor is confident that there is no vision loss occurring. Vision testing should then be done once or twice a year or whenever new DOS Times - Vol. 14, No. 8, February 2009 symptoms occur. Unfortunately, IIH is a life-long disease and tends to occur during periods of weight gain. The symptoms though are very treatable and, if treatment is started early enough, the vision loss is reversible. Although rare a strong suspicion of IIH in chronic severe headaches and immediate investigations followed by proper surgery can salvage the vision of the patient even in established papilloedema. Besides the vetriculo peritoneal shunt another surgical option would have been optic nerve sheathotomy. References 1. Procedures for Idiopathic Intracranial Hypertension in the United States, 1988-2002". Neurosurgery 57 (1): 97-108. PubMed. Retrieved on 2006-11-08. 2. Friedman, Deborah I.; Daniel M. Jacobson (November 26, 2002). "Diagnostic criteria for idiopathic intracranial hypertension". Neurology 59 (10): 1492-1495. PubMed. Retrieved on 2006-11-08. 3. Brazis PW, Lee AG: Elevated intracranial pressure and pseudotumor cerebri. Curr Opin Ophthalmol 1998 Dec; 9(6): 27-32 4. Friedman DI, Jacobson DM: Idiopathic intracranial hypertension. J Neuroophthalmol 2004 Jun; 24(2): 138-45. Curry, William T. Jr.; William E. Butler, Fred G. Barker (July 2005). "Rapidly Rising Incidence of Cerebrospinal Fluid Shunting First Author Ronak P. Bhandari MS Congratulations Congratulations to Dr. A.K. Grover who has been conferred Padma Shri by the Government of India. Congratulations “Dr. Amit Tarafdar” for receiving International ophthalmologist Education Award from American Academy of Ophthalmology. Congratulations “Dr. Ranjeet Kishore Rana” for receiving “Best Paper Award” in cataract and refractive surgery at the SAARC conference 2008 held at Delhi. www.dosonline.org 25 Kirti Singh MD, DNB, FRCS(E) I t is the temporary reduction or modfification of myopia achieved by the programmed application of specially designed contact lenses which reshape the cornea. A reversible technique it involves use of an overnight lens-wearing protocol. Orthokeratology has been around since 1960 when George Jessen created the first orthokeratology design from PMMA material, These early lenses used were conventional flat-fitting, rigid contact lenses and induced significant with-the-rule corneal toricity due to decentration. 1, 2 With the advent of reverse-geometry lens designs made of high Dk material in late 1980 this undesirable effect has decreased drastically3,4,5,6 Reverse-geometry lenses, propagated by Wlodyga and Stoyan, are fitted with a base curve flatter than the central corneal curvature. The flat base curve applies pressure to the central treatment zone, achieving sphericalization of the prolate cornea and resulting in reduction of myopic refractive error. To aid centration a secondary curve is ground which is steeper than the central base curve. 7 Cornea Orthokeratology How does it work? Tear film meniscus behind the contact lens hydraulically redistributes epithelial cells under the lens from the centre towards periphery. The central flat base curve exerts a positive pressure on the central corneal epithelium. As a consequence of this central corneal epithelium is thinned out. The steep secondary curve , creates an annular tear pool which by virtue of negative pressure leads to mid peripheral corneal epithelial and stromal thickening (Figure 2)8, 9,10, 11 Reduction in corneal epithelial thickness by 20 microm or more has been documentedover a 3 month period of ortho K wear.12 The end result is a reduction in corneal convexity and sagittal corneal height, which effectively reduces myopic error. Age plays a direct role in this corneal shaping as this response has been noted to be rapid in children and young adults but reduced and delayed in older people. 11 (Figure 3) Reverse geometry design of Ortho-k LK lenses Central Back Optic Zone Radius (BOZR) The radius of curvature of this zone is calculated as the flat K minus the desired amount of correction minus the compression factor of 0.75D. Reverse curve 2nd Curve Figure1: 5 Curve Reverse geometry Lens The steep reverse curve is calculated to align back surface of the lens with cornea. Alignment curve or 3rd and 4 th Curve This 1.0 to 1.5 mm wide bearing zone is made flatter than the reverse curve, thereby closely aligning the peripheral cornea. This curve helps in centering the lens and enhancing the orthokeratology effect. Figure 2: Corneal reshaping Guru Nanak Eye Centre, Maulana Azad Medical College, New Delhi www.dosonline.org Figure 3: 5 Curve Reverse geometry Lens bulls eye fluorescein pattern 27 Peripheral curve Select initial lens from Trial Kit This aids lens lift, ensures adequate tear exchange and helps in removal of lens. This initial lens is based on the adjusted flat K. Flat k is adjusted with eccentricity “e “as per table provided. Material Eccentricity range 0.46 to 0.54 , flat K value is not adjusted. Since this lens is worn during sleep only high Dk material ( > 85) is compatible with allowing sufficient oxygen transmission to prevent corneal hypoxia like Boston XO Centration of the shaping lens is evaluated on the fluorescein pattern It can be used for which types of patients: • Age - juvenile to adult myopes • Range : -1.00D to -6.00D • Cylindrical error -2.00D or less with-the-rule corneal astigmatism OR < -0.75D against-the-rule astigmatism. • Flat K range - 40.00D(8.44mm)~45.50D(7.42mm) Refractive endpoint is typically reached with these lenses after 7 to 10 days of wear with use of an overnight 7-8 hour lens-wearing protocol.3,4 28 Centration is critical to achieve the ortho-k effect. Decentered lenses do not produce the desired myopia reduction, in addition they distort the cornea. A good fluorescein pattern is a bull’s eye pattern , with a central zone of light touch of 3.5 - 4.0 mm in diameter, a mid peripheral ring of fluorescein pooling, or ‘tear reservoir’, under the steeper secondary curve and a peripheral circular band of alignment tapering to the edge lift. (Figure 3) A well fitting lens should move approximately 1mm with blinking Vision Examination Remove lenses after 1 hour of wearing. Evaluate patient’s visual acuity before and after lens wearing. Fitting Process Variation of effect on the basis of Eccentricity “e “ Corneal topography The closer the “e” value is to zero, the more spherical the cornea Evaluate corneal size, Flat-K value and eccentricity. • Flat-K value: Radius of curvature for centre. Radius of curvature for periphery also needs to be assessed by adapting corneal eccentricity with flat-k value. Higher the “e” value, more rapidly does the cornea flatten towards the periphery, thus the potential to flatten central corneal curvature is greater. • Corneal Eccentricity “e “: defined as the rate at which the cornea flattens from the central area to the peripheral area. In low eccentricity, oblate corneas the effect of Ortho K is reduced. Some examples of ortho K trial fits; Corneal size: this determines which part of cornea comes in contact with the alignment curve. Normal corneal size of over 11.6 mm requires a 10.6 mm diagnostic/ inventory lens. Good Centration- fluorescein fit and topography picture (Figure 4a & 4b) Figure 4a: Good Centration Figure 4b: Fluorescein fit and topography picture • Lens movement is quick and proper as natural. (1-2mm) DOS Times - Vol. 14, No. 8, February 2009 Figure 5b: Fluorescein fit and topography picture Figure 5a: Poor Centration (Steep Fit) • Base Curve Zone Touch: Central black part (4~6mm) Some examples • Reverse Curve Zone: Green colored ring with equal width Case I: 21-year-old female, -3.50D myopia. • Alignment Zone : Black part with equal width • Keratometry: (Rh):- 7.48mm (Rv):-7.61mm (flat-K value) Poor Centration (Steep Fit) fluorescein fit and topography picture (Figure 5a & 5b) • Corneal topography: Cornea size - 12.3mm. • Eccentricity - 0.49(0.46-0.54) • Lens movement is slight (1mm“!) and rides low Thus trail lens selected would be : • Base curve zone Touch: Touch zone narrow. ( < 4mm) • • Reverse Curve Zone: Air bubble/ thick ring Eccentricity as less than 0.5 therefore No need to need to adjust flat-K value. • Alignment Zone: Very black part • Initial trial lens selected = 7.61 • Initial lens selected • 10.6mm/7.63/-3.50D Poor Centration ( Flat Fit) fluorescein fit and topography picture (Figure 6a & 6b) OR 10.6mm/7.58/-3.50D • Lens movement is excessive • Fluorescein pattern: check centering and alignment. • Base curve zone Touch zone is wide over 5mm • Tear meniscus size and shape, lens movement • Reverse Curve Zone: Green ring has irregular width • Alignment Zone: Not black, with fluorescein If lens adheres, air bubbles would be noted in the reverse curve (reservoir) zone then - change the central bearing (make the fit steeper) Eccentricity Eccentricity Trial set - Flat K Flat K Adjustment Power Adjust 0.30 - 0.39 0.50-0.75D steep 43.50(7.76)-43.75(7.72) -3.50~-3.75D 0.40 - 0.45 0.00-0.25D steep 43.00(7.85)-43.25(7.80) -3.00~-3.25D 0.46 - 0.54 Flat K 43.00D(7.85mm) -3.00D 0.55 - 0.60 0.25-0.50D flat 42.75(7.90)-42.50(7.94) -2.75~-2.50D 0.61 - 0.65 0.50-0.75D flat 42.50(7.94)-42.25(7.99) -2.50~-2.25D 0.66 - 0.70 0.75-1.00D flat 42.25(7.99)-42.00(8.04) -2.25~-2.00D www.dosonline.org 29 Figure 6b: Fluorescein fit and topography picture Figure 6a: Poor Centration (Flat Fit) Figure 7: Topography Image after One Hour (a) Initial Map (b) After 1H Wearing Evaluate ortho-k effect Lens prescription and dispensing • After 1 hour wearing, remove initial lens. • Check unaided visual acuity, Slit Lamp examination and corneal topography. Finally the lens with Corneal Diameter 10.6mm/7.58/-3.50D has been ordered and was dispensed to the patient. • Perform Over-refraction to determine lens’ target power It is observed that her visual acuity before lens wearing was 6/36) and after lens wearing is 6/6 . Topography Image after One Hour We can see that here red rounded part (corresponds to the alignment curve) is centered after 1hour of wear (Figure 7a & 7b). After 3 days, this red rounded part becomes more evident. (Figure 8) 30 Does this technology work? Unaided vision improvement and reduction of the myopic refractive error have been documented now by many authors in different ethnicities.13,14,15,16 The average percentage reductions in spherical equivalent have been noted to be in the range of 84 - 92% and errors of till -5D have been corrected.15,16 A multicentre study recently found evidence to state that overnight orthokeratology is efficacious and safe for young myopic patients.17,18 DOS Times - Vol. 14, No. 8, February 2009 Figure 8: After 3 Days This has been confirmed by a large cohort studies evaluated over a long term period of at least 12 months.14,19 2. Binder PS, May CH, Grant SC. An evaluation of orthokeratology. Ophthalmology 1980;87:729-44. 3 Nichols JJ, Marsich MM, Nguyen M, et al. Overnight orthokeratology Optom Vis Sci 2000;77: 252-9. 4. Swarbrick HA, Alharbi A. Overnight orthokeratology induces central corneal epithelial thinning. Invest Ophthalmol Vis Sci 2001;42:S597 5. Mountford J. An analysis of the changes in corneal shape and refractive error induced by accelerated orthokeratology. ICLC 1997;24:128-44. 6. Lui W-O, Edwards MH. Orthokeratology in low myopia. Part 1: efficacy and predictability. Cont Lens Anterior Eye 2000;23:77-89. 7. Wlodyga RJ, Bryla C. Corneal molding: the easy way.Contact Lens Spectrum 1989;4:58-65. 8. Swarbrick HA. Orthokeratology (corneal refractive therapy): what is it and how does it work? Eye Contact Lens. 2004 Oct; 30(4):1815; discussion 205-6. 9. Alharbi A, Swarbrick HA. The effects of overnight orthokeratology lens wear on corneal thickness. Invest Ophthalmol Vis Sci. 2003 Jun;44(6):2518-23. Problems encountered with this lens 10. Walline JJ, Holden BA, Bullimore MA et al. The current state of corneal reshaping. Eye Contact Lens. 2005 Sep;31(5):209-14. Corneal staining was the most commonly observed complication with ortho-k and more than 80 per cent of patients were advised to apply ocular lubricants.20 11. Jayakumar J, Swarbrick HA. The effect of age on short-term orthokeratology. Optom Vis Sci. 2005 Jun;82(6):505-11. Another optical problem noted is decreased corneal optical quality in terms of increased higher-order aberrations and decreased contrast sensitivity. This correlates with the magnitude of myopic correction.21, 22,23 However, these induced optical higher order aberrations along with the improvement in uncorrected visual acuity are fully reversible after discontinuation of the Ortho K lens even after 1 year of use.23 Lens decentration during overnight wear can lead to diplopia, glare and reduction in the orthokeratology effect. In a study involving 270 eyes over a 6 month period of OrthokK lens wear decentration was more in patients with greater initial astigmatism and use of smaller lenses. 24 Microbial keratitis is the most severe, potentially vision-threatening adverse response associated with orthokeratology contact lens wear. The organisms identified have been Pseudomonas aeruginosa and Acanthamoeba species. 25, 26 It has been associated with the use of tap water in care regimens for overnight orthokeratology and the patient must be forewarned against using tap water. Conclusions Ortho-K lenses are effective for myopia correction by overnight wearing about 7 hours. Refractive endpoint is typically reached after 7 to 10 days of wear with the use of an overnight lens-wearing protocol. Under close monitoring, overnight ortho-k is effective and safe for reducing low to moderate myopia and the treatment is well accepted by the children. References 1. Kerns RL. Research in orthokeratology. Part VIII: results, conclusions and discussion of techniques. J Am Optom Assoc 1978;49:308-14. www.dosonline.org 12. Soni PS, Nguyen TT, Bonanno JA. Overnight orthokeratology: visual and corneal changes. Eye Contact Lens. 2003 Jul;29(3):13745 13. Johnson KL, Carney LG, Mountford JA, Collins MJ, Cluff S, Collins PK.Visual performance after overnight orthokeratology. Cont Lens Anterior Eye. 2007 Mar;30(1):29-36. Epub 2007 Jan 9. 14. Soni PS, Nguyen TT; XO Overnight Orthokeratology Study Group. Overnight orthokeratology experience with XO material. Eye Contact Lens. 2006 Jan;32(1):39-45. 15. Cheung SW, Cho P, Chui WS, Woo GC. Refractive error and visual acuity changes in orthokeratology patients. Optom Vis Sci. 2007 May;84(5):410-6. 16. Chan B, Cho P, Cheung SW Orthokeratology practice in children in a university clinic in Hong Kong. Clin Exp Optom. 2008 Sep;91(5):453-60. Epub 2008 Mar 18. 17. Walline JJ, Rah MJ, Jones LA The Children’s Overnight Orthokeratology Investigation (COOKI) pilot study. Optom Vis Sci.2004 Jun;81(6):407-13. 18. Rah MJ, Jackson JM, Jones LA, Marsden HJ, Bailey MD, Barr JT Overnight orthokeratology: preliminary results of the Lenses and Overnight Orthokeratology (LOOK) study. Optom Vis Sci. 2002 Sep;79(9):598-605. 19. Van Meter WS, Musch DC, Jacobs DS, Kaufman SC, Reinhart WJ, Udell IJ.Safety of overnight orthokeratology for myopia: a report by the American Academy of Ophthalmology. Ophthalmology. 2008 Dec;115(12):2301-2313.e1. Epub 2008 Sep 20. 20. Chan B, Cho P, Cheung SW.Orthokeratology practice in children in a university clinic in Hong Kong. Clin Exp Optom. 2008 Sep;91(5):453-60. Epub 2008 Mar 18. 21. Hiraoka T, Okamoto C, Ishii Y, Kakita T, Okamoto F, OshikaT. Time course of changes in ocular higher-order aberrations and contrast 31 sensitivity after overnight orthokeratology. Invest Ophthalmol Vis Sci. 2008 Oct;49(10):4314-20. Epub 2008 May 23. 22. Hiraoka T, Okamoto F, Kaji Y, Oshika T. Optical quality of the cornea after overnight orthokeratology.Cornea. 2006 Dec;25(10 Suppl 1):S59-63. 23. Hiraoka T, Okamoto F, Kaji Y, Oshika T…Hiraoka T, Okamoto C, Ishii Y, Okamoto F, Oshika T. Recovery of corneal irregular astigmatism, ocular higher-order aberrations, and contrast sensitivity after discontinuation of overnight orthokeratology. Br J Ophthalmol. 2008 Nov 19. [Epub ahead of print] 24. Yang X, Zhong X, Gong X, Zeng J. Topographical evaluation of the decentration of orthokeratology lenses. Yan Ke Xue Bao. 2005 Sep;21(3):132-5, 195. 25. Watt KG, Swarbrick HA. Trends in microbial keratitis associated with orthokeratology. Eye Contact Lens. 2007 Nov;33(6 Pt 2):3737; discussion 382. 26. Shehadeh-Masha’our R, Segev F, Barequet IS, Ton Y, Garzozi HJ. Orthokeratology associated microbial keratitis. Eur J Ophthalmol. 2009 Jan-Feb;19(1):133-6. Author Kirti Singh MD, DNB, FRCS(E) 32 DOS Times - Vol. 14, No. 8, February 2009 Cornea Goldenhar Syndrome: A case report & review of literature Uday Gajiwala MS, Rajesh Patel MS, Manoj Gangwal DO, FCPS G oldenhar syndrome also called oculo auriculo vertebral dysplasia or first & second branchial arch syndrome, is mostly unilateral, occurs most commonly in men and involves anomalies of face, ears, eyes and vertebrae in most cases. When malformations primarily involves the jaw, mouth and ear of one side, the disorder is referred as Hemifacial microsomia, but if abnormalities of vertebrae and the eyes are also present, the disorder is often called Goldenhar syndrome. Case History A fifty year female patient with negative family history presented with dimness of vision in left eye and painless swelling in left eye since birth. On ophthalmic examination, Best Corrected Visual Acuity in right eye was 6/6 but in left eye it was 6/36 not improving with refraction. There was against the rule astigmatism of 3 dioptre due to inferolateral limbal dermoid of about 5X5 mm size. She had 15 degrees concomitant divergent squint with full eyeball movement. There was no other ocular abnormality. Fundus examination was normal in both eyes. Photographs On ENT examination, patient had pre auricular skin tags which were lying in line joining tragus & angle of mouth. Patient had no complaint of deafness in both the ears. X-ray PNS & Schullers views were also normal with normal external auditory meatus. CVS & CNS examination was normal. X-ray cervical spine was not showing occipitalisation of atlas or hemi vertebrae which is usually associated with Limabal Dermoid in Goldenhar syndrome. CT scan was not showing any abnormality. Patient was not ready for its excision as prognosis for visual improvement was poor due to meridional amblyopia and she was not worried about cosmetic problem in this age. Thus, the patient was having following findings: • Limbal dermoid • Abnormalities of ear (pre auricular skin tag) Discussion The incidence of this syndrome is 1:3000 to 1:26,500 with onset at birth. It occurs in family &/or as sporadic cases. Familial cases are consistent with autosomal dominant, autosomal recessive and multifactoral patterns of inheritance. Chromosomal abnormality or defective gene disorder is common in men (70%). It can be unilateral or bilateral but mostly it is unilateral. Pathogenesis Genetic defect: abnormal neural crest morphology-malformation of the derivatives of the first & second branchial arches-facial, auricular, ocular, vertebrae & other anomalies. Variable phenotypic expression is characteristic with this syndrome & the spectrum of phenotypic anomalies can range from mild to severe even within Limbal Dermoid Pre-Auricular Skin Tag Sewa Rural, Jhagadia, Bharuch, Gujarat www.dosonline.org 35 Normal X Ray Cervical spine the same affected family. It may be present in monozygotic twins with only one twin being affected. Dermoid Showing Hair Ocular Manifestations Epibulbar Tumors Review of Literature First identifies by M.Goldenhar in 1952 First called oculo auriculo vertebral dysplasia by R.J. Gorlin. Clinical Features Facial asymmetry Dermoid or lipodermoids. • Found in 35% of cases. • Yellow or pink, solid ovoid masses up to 10 mm in diameter. • Unilateral or bilateral. • May impair vision. 65-70% cases have some facial asymmetry & in 20% of cases the asymmetry is marked. Others The right side of the face is more severely affected in 60% of cases. • Strabismus 10-33% has bilateral facial involvement and/or mandibular regions on the affected side. • Microphthalmia or anophthalmia. • Blepharo-phimosis in 10% of cases. There also may be unilateral hypoplasia of the facial musculature with facial muscle weakness and depressor anguli oris hypoplasia on the affected side. • Narrowing of palpebral fissure • Retinal anomalies. Macrostomia • Upper lid colobomas. Greatly exaggerated width of the mouth due to lateral cleft like extension of the corner of the mouth. Other manifestations External Ear May range from mildly dystrophic ear to microtia to anotia. Pre-auricular skin tags, pits and/or sinuses and occur between the tragus and the angle of the mouth. Usually unilateral but can be bilateral in 33% of cases. Middle Ear Narrow or atretic Inner Ear Conductive &/or sensorineural hearing loss. 36 • Neurological • Microcephaly or hydrocephaly. • Occipital encephalocele. • Arnold chiari malformation. • Mental retardation in 5-15% of patients. • Spina bifida. Congenital Heart disease • VSD, PDA, Tetralogy of Fallot, Coarctation of Aorta. • Transposition of Great arteries. DOS Times - Vol. 14, No. 8, February 2009 Renal Management • Ectopia &/or fused kidneys. Supportive • Renal agenesis. Multidisciplinary approach • Multicystic dysplastic kidneys. • Pediatrics, plastic, orthopaedic, ophthalmic and ENT surgeons • Hydronephrosis, hydro-ureter, ureteral duplication. • Early and regular hearing tests. • Uretero-pelvic junction obstruction. • Genetic counselling. • Vesico-ureteral reflux. Prognosis Musculoskeletal • Scoliosis Normal life span and intelligence in a majority of those patients without significant complications. • Talipes equino varus (club feet) References • Bifid or digitalized thumb • Hypoplastic Thumb 1. Baraitser M. & R. M. Winter, color atlas of congenital malformation syndrome (1st edition) P: 40-41 (1996). 2. Baum J. L. & Feingold M., ocular aspects of Goldenhar syndrome. AJO, 75:250, 1973. 3. Bergsma D. (Ed) Birth defects, atlas and compendium. Baltimore: Williams & Wilkins. 4. Benjamin S. N. & Allen H. F. classification for limbal dermoids, choriostomas & branchial arch anomalies: presentation of an unusual case. Arch Ophthalmology, 87:305, 1972. 5. Geeraets W. J. ocular syndromes (3rdedi) Philadelphia: Lea & Febiger, 1976. Jones K. L., Smith's Recognizable patterns of human malformations (5th edi), P: 642-645 (1997). Vertebral 6. Journal of Genetics, Human 1:243 (l952). • Cervical fusion in 20-25% of cases. 7. Journal of Pediatrics 63:991-999 (1963). • Platybasia & occipitalisation of the atlas in 30% of cases. 8. • Klippel-Feil anomaly. Mansour A. M. et. al, ocular findings in facio auriculo vertebral sequence (Goldenhar Gorlin syndrome) AJO 100:555, 1985. • Hemi vertebrae 9. • Hypoplasia of vertebrae Gorlin RJ, Cohen MM Jr, Levin LS.Oculoauriculovertebral Spectrum.In: Syndromes of Head and Neck.3rd ed.oxford University Press: New York; 1990.p.641-9 Investigations Skeletal X-Rays Skull • Hypoplasia of the maxillary, temporal & malar bones. • Hypoplasia or absent mandibular ramus & condyles. • Reduced pneumatization of mastoid region. CT/MRI • Agenesis of or lipoma in corpus callosum • Calcification of falx cerebri • Hypoplastic septum pellucidum. • Intra-cranial dermoid cyst. 10. Das A, Mazumdar S, Chatterjee SS.Atypical case of Oculo-Facioauriculo-vertebral Dysplasia (Goldenhar Gorlin Syndrome).IJO 1999; 47:131-3 11. A. Das, B.Ray, D.Das, S.Das.A Case of Goldenhar Gorlin syndrome with unusual association of Hypoplastic thumb.IJO 2008; 56:150-2 First Author Uday Gajiwala MS www.dosonline.org 37 Shreekant A Damgude MBBS, B.P. Guliani MS P seudophakic bullous keratopathy refers to the development of irreversible corneal oedema following cataract surgery which initially develops in stroma progressing towards intercellular epithelial region with characteristic formation of bullae. Though with newer surgical techniques and IOL designs, incidence of this complication has decreased dramatically, it still represents an important cause of visual disability following routine and complicated cataract surgery. Pathophysiology Corneal transparency depends on ability of cornea to remain in dehydrated state. Corneal epithelium and endothelium are both semipermeable membranes that create a barrier to the flow of water and other electrolytes. The major factor which prevents corneal hydration is Na+K+ AT Pase pump which lies within lateral cell membrane in endothelial layer of cornea. Endothelium is incapable of mitotic activity. At birth endothelial cell count is approximately 7500 cells /mm2. In older adults it is approx. 2500-2700 cells / mm2. Normal rate of endothelial cell loss after age of 20 is approx. 0.5 % per year. Surgical trauma, inflammation and corneal dystrophies (Fuch's endothelial dystrophies, posterior polymorphous dystrophies) can accelerate this normal aging loss. The final common pathway in development of PBK is damage to corneal endothelium when cell density reaches to critically low level of about 300-500 cells / mm2. It leads to rearrangement of remaining cells to cover posterior corneal surface in the form of polymegathism (greater than normal variation of corneal endothelial cell size resulting in irregular large and small cells) and pleomorphism(variation in cell shape; increased proportion of non-hexagonal cells). Descemetl's membrane is produced in excess amount.Endothelial pump fails and stromal oedema develops. Cornea Pseudophakic Bullous Keratopathy The lack at adhesive proteins in epithelial basement membrane (fibronectin, laminin and collagen type IV) and accumulation of antiadhesive proteins, such as tenacin C and thrombospondin -1, leads to loss of contact of epithelial cells with each other and with underlying subepithelial tissue, resulting in subepithelial bullae and fibrosis3. Incidence: Current incidence is about 0.1% FDA report on intraocular lens (1978-1982)4 is shown in Table 1. Table 1: Association of PBK with of intraocular lens Incidence of PBK Type of lens 0.06% PC IOL 1.2 % AC IOL 1.5 % Iris fixated lens Long term incidence of PBK has been found to be 15%. It is also correlated with endothelial cell loss5 in cataract surgery. (Table 2) Table 2: Endothelial cell loss in cataract surgery ECCE SICS PE 4.72 % 4.21% 5.41% Clinical features Poor visual acuity: It is due to inability of stroma to maintain corneal transparency. Epithelial oedema can be responsible for great changes in visual activity due to irregularity in corneal refractive surface. Mild stromal Oedema may fluctuate in response to changing I.O.P. ( Imbibition pressure = I.O.P.- Swelling pressure). Later on epithelial oedema ensues because of anterior movement of aqueous and fluid in stroma leading to formation of blisters and bullae. Condition typically worsens during night because of lack of evaporation and lack of hypertonic environment. Histopathology of PBK Prime feature is attenuation and loss of corneal endothelial cells with associated epithelial bullae and stromal oedema1. There is thickening of the posterior collagenous layer of descemet's membrane and decrease in stromal keratocytes2 Figure 1: Bullous Keratopathy Department of Ophthalmology Safdarjung Hospital, New Delhi www.dosonline.org 41 Etiology Preoperative risk factors Specular microscopy: Though there is no direct correlation between endothelial cell density and oedema, significant correlation has been found between variation in cell shape (Plesmorphism) and size (Polymegathism) and development of postoperative corneal oedema. Pleomorphic cells react adversely to intraocular surgery. Polymegathic cells may not fit together leaving gaps and compromising the endothelial structural barrier. Figure 2: Formation of Bulla between epithelial basement membrane and Bowman’s membrane Incidence of preoperative endothelial dystrophy in PBK has been found in 67% of patients (Retrospective analysis).9 Pseudoexfoliation syndrome has been associated with an increased incidence of PBK, due to higher rate of vitreous loss but also due to clinically & histopathologically distinct keratopathy (Irregular thickening of Descemet's membrane and focal accumulation of locally produced pseudoexfoliation material. A reduced preoperative endothelial cell count of 10.5 to 11.1%. is demonstrated in patients with pseudoexfoliation syndrome undergoing cataract surgery10,11. Endothelial cell density less than 2000 cells/mm2 was found in patients with previous history of corneal diseases, angle closure glaucoma, pseudoexfoliation syndrome and a history of trauma12. Intraoperative risk factors Surgical trauma to endothelium Type of cataract surgery Lenses made of methacylate adhere instantaneously to endothelial surface when contact upon lens insertion occurs.13 Figure 3: Aphakic bullous keratopathy oedema doesn't cause severe visual loss. However epithelial oedema can cause a significant drop in vision. Pain / discomfort: Pain occurs due to exposure of corneal nerves to noxious environment.6 As the oedema progresses, bullae are formed their rupture results in pain, photophobia & epiphora. Slit lamp examination reveals folds in descemet's membrane and obvious overall thickening of central and peripheral cornea. In more advanced cases, vesicles & bullae may be seen on corneal surface. In patients with predisposing conditions (like fuch's dystrophy & Posterior Polymorphous dystrophy), cornea guttata may be seen. Guttate excrescences appear as "golden brown" confluent endothelial lesions & give posterior surface a "beaten metal appearance"7,8. 42 Reusable cannulas with viscoelastic can result in toxic residues being introduced into the eye. Hence disoposable annuals should be used. During intraocular irrigation, physiologic saline causes corneal swelling and endothelial damage. Ringers solution does so to a lesser degree. Balanced salt solution enriched with bicarbonate, dextrose and glutathione (BSS plus) has been shown to protect the endothelium better than BSS alone14,15. It has been found that HPMC is not as protective as sodium hyaluronate during phacoemulsification. Protective benefit of sodium hyaluronate is improved when used in combination with chondroitin sulphate.16 Toxic substances used to disinfect instruments may inadvertently be introduced into the eye. Water, not saline, should be used to rinse the instruments. Intracular irrigation must be just adequate enough. Excess may cause endothelial injury. For topical and intracameral anaesthesia only 0.5 ml of 1% preservative free lidocaine should be used17,18. Other preparations may cause corneal toxicity. Intraocular epinephrine19, benzal konium chloride preserved viscoelastic, vancomycin (> 1 mg / ml)20 and inadvertent exposure of endothelium to 5% povidone-iodine21 can cause endothelial toxicity. DOS Times - Vol. 14, No. 8, February 2009 Digital analysis of photographs can be used to assess parameters such as coefficient of variation (for polymegathism) and percentage of hexagonal cells present (for pleomorphism). Endothelial cells that show great variability in size and shape are considered to be under physiologic stress and are abnormal. Specular photomicrographs can be used to assess corneal diseases such as Fuch's dystrophy & posterior polymorphous dystrophy. Former shows guttate excrescences while later shows patchy areas of normal endothelium adjacent to abnormal endothelium with vesicles and plaques. Ultrasound pachymetry and optical pachymetry Both are used to measure corneal thickness. • (N) central corneal thickness is 550 um centrally increasing upto 770um peripherally. Corneal thickness above 0.6 mm (600 um) is suspect for corneal oedema. • Serial measurements are helpful to note progression; and also to assess effectiveness of therapeutic regimens. Descemet's detachment is more common with clear corneal incisions which may lead to postoperative corneal oedema. • Ultrasonic pachymetry is more reproducible and requires less skill than optical. Postoperative causes Optical pachymetry is especially useful in measuring the depth of corneal pathology, when fall thickness of corneal stroma is not involved and for therapeutic reasons to estimate the depth of this pathology. It is preoperative investigation for excimer laser phototherapeutic keratectomy. Figure 4: Late painful bullous keratopathy Routine uncomplicated phacoemulsification results in 9% endothelial cell loss at 1 year postoperatively. In one series, it has been found to be 11.9%22 Regardless of type of surgery and type of IOL implanted, continuing endothelia cell loss more than usual 1% per year occurs in patients who have undergone cataract extraction. Treatment Medical Care Type of lens implanted Topical hyperosmotic agent Anterior chamber IOLs contribute to endothelial damage by "Intermittent touch" between IOL & corneal.23 2% and 5% sodium chloride solution and ointment. IOL haptics & footplates can cause chronic irritation with low grade inflammation IOLs are also known to disrupt the normal flow of aqueous in anterior chamber which is the nutrient flow to endothelial cells.24 Iris supported lenses may cause greater endothelial loss as they can have contact with endothelium during ocular saccades25. Closed loop ACIOL have been responsible for large amount of corneal pathology. Following ICCE, incidence at PBK has been found to be 2.3% in retrospective study of 1041 patients.26 Mechanism: These agents create hypertonic tear film, drawing water out of cornea. Because evaporation of tear film is minimal at night with eyes closed, corneal oedema tends to be worse in morning. Hence 5% sodium chloride ointment should be applied at nigh to conjunctival cul-de-sac. Typical regimen includes application of Muro 128, 2-5% drop hourly in affected eye until noon. As the day progresses, evaporation of tear film begins to create relative hypertonicity of tears drawing fluid out of cornea. This accounts for typical history of improving vision towards end of the day. Imaging Studies Pressure lowering agents Specular microscopy • Lowering of intraocular pressure can decrease corneal oedema & thickness in postoperative setting, even if intraocular pressure is normal or only mildly elevated. • β-blockers & -agonists are first line agents for this purpose. • Prostaglandins analogs & miotics should be avoided because both drugs may adversely affect intraocular inflammation. • Utility of topical CA (-) is under question because they may cause endothelial toxicity in compromised corneas. This is photographic method of assessment of endothelium in vivo. Light is projected onto cornea and reflected images from optical interface (endothelium and aqueous) can be visualized. High magnifications photographs allows quantification of cell density. Corneas with cell densities less than 1000 cells / mm2 are at moderate to high risk of developing corneal oedema following intraocular surgery. www.dosonline.org 43 Topical steroids These should be used in cases of uveitis provided infectious keratitis & epithelial defects are absent. Hydrophillic contact lenses This fashioned membrane was then secured to the edge of defect by 10-0 nylon sutures which were either continuously running interrupted or a combination of both. These on extended wear basis, can be used to decrease pain associated with epithelial bullae. Attention was given to make the membrane flattened tightly anto corneal surface and approximate to the epithelial edge. These lenses as such do not reduce the amount of oedema. These can improve visual activity to the extent that they mask surface irregularity. These contact lenses can be used in association with 5% hypertonic saline (as cornea) and thus they can improve visual acuity by decreasing epithelial & stromal oedema. Pain associated with PBK can be due to rupture of bullae with exposure of corneal nerves endings or swelling of epithelium leading to stretching of nerve endings. These lenses alleviate pain as long as the lens remains in place. Acts as effective precorneal protective layer. Shields abnormal epithelium from environment and prevents bullae from bursting. The lens doesn't prevent formation of bullae but perhaps when new bullae do occur, the corneal nerves' endings are not exposed to drying & other noxious stimul, when the lens cover them. Fitting of lens is an important consideration. Lenses with excessive movement can irritate epithelium & be uncomfortable. Lenses that are too tight can act as suction cap and result in inflammation and even anterior uveitis (Tight lens syndrome). Surgical Care Conjunctival flap In 1958, Gundersen introduced the technique of using conjunctiva alone without use of tenon's capsule to cover the cornea. It is onlay flap of thin conjunctiva without use of tenon's capsule. The exclusion of tenon's capsule increases flap longevity. A Gundersen-type flap undermines the superior bulbar conjunctiva & moves in & down to cover the cornea with intact "bridges" nasally and temporally. The flap is thought to increase the local blood supply, promote healing and replace damaged or defective corneal epithelium27. Amniotic membrane transplantation In some cases BCL was applied. Results: This turned immensely successful for ocular pain relief in 43 of 48 eyes with painful bullous keratopathy. This is technically easier to perform avoid potential complications of conjunctival flap such as ptosis, it gives better cosmetic appearance. Hence it is superior to conjunctival flap. It provides additional advantage in that resultant cornea doesn't manifest surgically induced limbal stem cell deficiency, which is invariably created by conjunctival flap. As a result such corneas are amenable for corneal transplantation if necessary. Amniotic transplantation also restores corneal epithelial integrity. All except 4 eyes (92%) maintained healed and intact epithelium during the entire follow up period. Bullae resolved in 90% cases. Even with recurrent epithelial oedema, pain did not occur. Amniotic basement membrane prevents epithelial apoptosis, enhances epithelial migration, differentiation. It also reinforces adhesion of basal epithelial cells29,30,31. It also prolongs life span of corneal and conjunctival progenitor cells32. Stromal matrix inhibits TGF-β, hence causing less scars postoperatively32. Amniotic membrane transplantation proxides new and nonantigenic human basement membrane for renewed expansion of epithelial cells33. Cautenisation of Bowman's membrane It can be performed for pain relief. This procedure is thought to produce dense fibrous barrier between corneal stroma and epithelium so that fluid can not permeate into epithelial cells and produce ballous changes. Study of Amniotic membrane transplantation performed in 50 eyes28 Anterior stromal pundure in bullous kercetopathy Important steps of procedure 28 patients of bullous keratopathy (1996-99) were studied with minimum follow up of 1 month. In all these patients approximately 200 punctures were given with bent 26 G needle sparing peripheral cornea. Loose epithelium was first removed with sponge, 0.12 forceps or a blade to where epithelium became quite adherent. In general this would create large corneal epithelial defect upto 1 or 2 mm from limbus. Preserved amniotic membrane was removed from storage medium and cut to same size as debrided corneal surface, with basement membrane surface up in all cases. The side of BM could be distinguished from stromal side by touch 44 with sponge. The former was not sticky, while the later was & could be caught by the sponge. A clinicopathologic study34 • Symptromatic relief was noted in all. • 20 patients had complete relief. • 8 patients experienced mild symptroms such as tearing and occasional pain. DOS Times - Vol. 14, No. 8, February 2009 • Complete regression of bullae in 10 patients. • Partial regression of bullae in 18 patients. • Visual activity decreased in 12 patients. • Objective evidence of subepithelial fibrosis and scarring was seen in all. Anterior stromal puncture is an effective, simple and low cost alternative for treating patients with symptomatic bullous keratopathy35. Annular keratotomy It is used to treat pain associated with bullous keratopathy in eyes with poor visual potential. A partical thickness corneal incision is made with a trephine and relieves pain by severing branches of corneal ciliary nerves to decrease corneal sensation36. Diamond burr polishing of basement membrane • One patient had persistent epithelial defect that required botulinum toxin ptosis. Corneal transplantation Penetrating keratoplasty for PBK: • Review study of 81 causes40 of PK done for PBK during 1987-93. • IOL explant was done in 24 (29.6%) cases. • IOL was retained in 12 (14.9%) cases. • IOL exchange was done in 45 (55.5%) cases. • Minimum follow up period was 6 months. • Graft remained clear in 54 (66.6%) patients. • Commonest cause of graft failure was "graft rejection"(17.2%). Following epithelial debridement, 4.5 mm to 5 mm diamond burr can be used gently to polish basement membrane throughout area of epithelial debridement37. They recommended explantation of all closed loop AC IOLs and iris claw IOLS and that AC recomstraction during PK with IOL exchanged should be done using PCIOL or open loop kelman type ACIOL. Phototherapeutic keratectomy for bullous keratopathy Failure of retained lens in PK for PBK41 Nidek EC 5000 excimer laser used for performing PTK (3 types) for PBK38. Failure rate Lens 34% ACIOL Superficial PTK 8 to 25 um ablation 29% Iris supported lens Intermediate PTK 50 to 100 um ablation 6% PCIOL Deep PTK 25% of stromal thickness ablation In this study from LVPEI, they suggested, Thomas et al39 proposed that PTK is effective procedure and bullae resolve and pain is abolished in large number of patients with superficial ablation. Anterior segment reconstruction during PK →Better outcome Anterior, +Gonioplasty, +Iridoplasty, Vitrectomy But in this study, it was proposed that preterminal neural plexus of cornea is located just deep to Bowman's membrane. Hence moderately deep ablation would have superior effect on decreasing the pain; by ablation of neural plexus. Advantage of Anterior Vitrectomy • It will lead to decreased swelling of corneal stroma by decreasing the quantity of mucopoly sacharides and hence decreasing osmotic load. Advantage of gonioplasty & Irioplasty • To restore pupil to normal round configuration Increased scarring associated with deeper ablation may result in increased stability of epithelium. • Prevents development of glaucoma and allograft rejection related to formation of anterior synechiae due to centripetal tension exerted on iris. • Give additional support to AC lens. • Improves optical function of iris. • Superficial PTK showed improvement in 62% cases whereas 38% required penetrating keratoplasty. Intermediate PTK showed improvement in 40% cases whereas 60% required penetrating keratoplasty. • ↓ CME • Prevents decentration of IOL by vitreous strands Deep PTK: Mean ablation performed was of 206 um thickness. To perform IOL exchange either with PCIOL or an open loop kelman type ACIOL. • 66% (16 out at 24) showed improvement. Explantation of all iris fixated lens / closed loop IOLS. • One required PK. • To avoid delay in corneal surgery once PBK is diagnosed. • One required amniotic membrane transplantation. • Anterior segment reconstruction and monitoring of I.O.P., both pre & postoperatively. • Two patients required retreatment. • Three patients continued having discomfort www.dosonline.org CME is important cause for poor visual recovery in such patients. Despite risk of infection, secondary glaucoma and graft rejection, 45 penetrating keratoplasty still remains treatment to get maximum improvement in visual acuity. intraocular lens implantation, Jpn J Ophthalmol 37:414, 1993. 2. Champion R, Green WR : Intraocular lenses: a histopathologic study of eyes, ocular tissues and intraocular lenses obtained surgically, Ophthalmology 92:1628,1985. 3. Maseruka H, Ataullah SM, Zardi L et al : Tenascin - cytotactin (TNC) variants in pseudophakic / aphakic bullous keratopathy corneas, Eye 12:729, 1998. 4. FDA report on intraocular lens (1978-1952) stark WJ, Worthen DM, Holladay JT Ophthalmology 90:311-317, 1983. 5. George R, Rupauliha P, Sripriya AV, Rajesh PS, Vahan PV, Praveen S. Ophthalmic Epidemiol. 2005 Oct; 12(5): 293-7. 6. Ljubimov AV, Burgeson RE, Butkowski RJ, Couchman JR, WuRR, Ninomiya Y, et al. Extracellular matrix alterations in human corneas with bullous kercetopathy. Invest Opthalmol Vis Sci 1996;37:9971007. 7. Waring GO III, Rodrigues MM, Laibson PR. Corneal dystrophies. II. Endothelial dystrophies. Surv. Ophthalmol 1978;23:147-168. 8. Cibis GW, Krachmer JH, Phelps CD, et al. The clinical spectrum of posterior polymorphous dystrophy. Arch ophthalmol 1977; 95: 14291537. 9. Lugo M, Cohen EJ, Eagle RC, Parker AV Ophthalmic Surg. 1988 Jan; 19(1):16-19. In general, options for IOL exchange are • Modern flexible loop ACIOL. • PCIOL in ciliary sulcus. • Iris fixation of PCIOL. • Suturing of PCIOL in sulcus. Flexible ACIOL should be reserved for • Eyes with minimal anterior segment pathology. • Less than 900 angle synechiae. • Well controlled I.O.P. Generally width of IOL chosen should correspond to a measurement of horizontal white to white corneal diameter plus 1 mm. Sutured intraocular lenses should be reserved for • Extensive anterior segment pathology. • Lack of iris support for ACIOL. • Patients with glaucoma. No study till date has clearly pointed to advantage of one technique or style of intraocular lens replacement in terms of graft survival, vision or development of secondary complications. Hence it remains the choice of particular surgeon. Newer techniques 11. Wirbelauer C, Anders N, Pham DT et al : Corneal endothelial cell changes in pseudoexpoliation syndrome after cataract surgery, Arch Ophthalmol 116 (2) : 145, 1998. DLEK (Deep lamellar endothelial Keratoplasty) 12. Ishikawa : Risk factors for reduced corneal endothelial cell density before cataract surgery, J Cataract Refract Surg 28 : 1982, 2002. In patients with normal stroma and superficial cornea, where only endothelium is damaged; this procedure is a very good treatment modality, with following distinct advantages:24 13. Kaufman HE, Katz JC : Endothelial damage from intraocular insertion, Invest Ophthalmol 15:996, 1976. • This preserves healthy portions of patients' native cornea. • It avoids additional risks and postoperative complications. • Additional benefit is that postoperative astigmatism is very minimal. Flap endokeratoplasty Free papers accepted in XXV congress of ESCRS in Sept. 2006 compares "Flap endokeratoplasty versus penetrating keratoplasty for PBK: 5 yrs. follow up results"42. 5 yrs follow up shows all clear grafts, no significant difference between BCVA, endothelial cell count or mean postoperative refraction. Visual recovery and postoperative astigmatism were better in patients who have undergone Flap EKP. Preliminary results show that flap EKP could be a successful alterative to penetrating keratoplasty for treatment of PBK. This might be the future as far as treatment of PBK is concerned. References 1. 46 10. Wirbelauer C, Anders N, Pham DT et al: Early postoperative endothelial cell loss after corneoscleral tunnel incision and phacoenulsification in pseudoexfoliation syndrome, Ophthalmologe 94 (5) : 332,1997. Liu G et al: Histopathological study of pseudophakic bullous keratopathy developing after anterior chamber or iris-supported 14. Edelhauser HF et al : Intraocular irrigating solutions : their effect on the corneal endothelium, Arch Ophthalmol 93: 648, 1975. 15. Li J et al : Effects of BSS and BSS + irrigation solutions an rabbit corneal transendothelial electrical potential differences, Cornea 12: 199, 1993. 16. Glasser DB et al : Endothelial protection and viscoelastic retention during phaconulsification and intraocular lens implantation, Arch Ophthalmol 109: 1438, 1991. 17. Martin RG, Miller JD, Cox CC 3rd et al : Safety and efficacy of intracameral injections of unpreserved lidocaine to reduce intraocular sensation, J Cataract Retract Surg 24 : 961, 1998. 18. Elvira JC, Hueso JR, Martinez-Toldos J et al : Induced endothelial cell loss in Phacoemulsification using topical anaesthesia plus intracameral lidocaine, 1 J cataract Retract Surg 25: 640,1999. 19. Edelhauser HF et al : Corneal oedema and intraocular use of epinephrine, Am J Ophthalmol 93: 327, 1982. 20. Sandboe FD, Medin W, Bjerknes R : Toxicity of vancomycin on corneal endothelium in rabbits; Acta Ophthalmologica scand 76 : 675, 1998. 21. Alp B N, Elibol O, Sargon MF et al : The effect of povidone iodine on the corneal endothelium, Cornea 19 : 546,2000. DOS Times - Vol. 14, No. 8, February 2009 22. Zhongua Yan Ke Za Zhi 2000 Jul; (36) 4 : 282-4. He W, Lu P. 23. Sugar J, Mitchelson J, Kraff M : Endothelial trauma and cell loss from intraocular lens insertion, Arch Ophthalmol 96 : 449, 1978. 24. Paula Wynn. E medicine article, University of Iowa 2005 Nov. 25. Drews RC : Intermittent touch syndrome, Arch Ophthalmol 100 : 1440, 1982. 26. Kirsten Baggesen, E. B. Knudsen, K. Naeser. Acta Ophthalmologica scandinavia 1999. 27. Gundersen T. Surgical treatment of bullous keratopathy, Arch Ophthalmol 1960; 64 : 260 - 67. 28. Renato T.F. Pires, Scheffer C.G. Tseng. Arch Ophthalmol. 1999; 117 : 1291-97. 29. Khodadoust AA, Silverstein AM, Kenyon KR, Dowling JE. Adhesion of regenerating corneal epithelium : the role of basement membrane. Am J Ophthalmol 1968; 65 : 39-48. 30. Guo M, Grinnell F. Basement membrane and human epidermal differentiation in vitro. J Invest Dermatol 1989; 93 : 372-8. 33. Fukada K, Chikama T, Makamura M, Nishida T. Differential distribution of subchains of basement membrane components type IV collagen and laminin among the amniotic membrane, cornea and conjunctiva. Cornea 1999; 18 : 73-9. 34. Anterior stromal puncture in bullous keratopathy; a clinicopathologic study Sridhar MS Rao GN. Cornea 2001 Aug 20 (6) : 573-9. 35. Anterior stromal puncture in the treatment of bullous keratopathy: 6 months follow up. Gomes JA, Haraquchi DK, Zambrano DU, Izquierdo Junior L, Cuhna MC, de Freitas D. Cornea 2001 Aug 20(6) : 570-2. 36. Annular Keratotomy for the treatment of painful bullous keratopathy.Koeniq SB. Am J Ophthalmol. 1996 Jan; 121 (1) : 934. 37. David Verdier. E medicine article on Map dot fingerprint dystrophy, 2005 Dec. 38. Raj Maini, Laurence Sullivan, Grant R Snibson, Hugh R Taylor, Michael S Laughnan. Br J Ophthalmol 2001; 85 : 912 - 915. 39. Thoman et al. Br J Ophthalmol 1995; 79: 335-338. 31. Boudreau N Werb Z, Bissell MJ. Suppression of apoptosis by basement membrane requires three dimensional tissue organisation and withdrawal from cell cycle. Proc Natl Acad Sci USA 1996; 93 : 3500-13. 40. Agrawal Vinay, Vagh Mustali, Sangwan Virender, Rao Gullapalli N. Indian J Ophthalmol 1994; 42 (2) : 75-80. 32. Tseng SCG, Li D-Q, Ma X. Suppression of TGF-? receptor type II and myofibroblast differentiation in cultured human corneal and limbal fibroblasts by amniotic membrane matrix. J cell Physiol 1999; 179 : 325 - 35. 42. M. Alfayez. Flap EKP vs Penetrating keratoplasty for PBK : 5 yr. Follow up results. XXX congress of ESCRS. Sept. 2006. 41. Sugar. Analysis of corneal endothelial and graft survival in PBK. Trans Am Ophthalmol Soc. 87 : 762-801, 1989. First Author Shreekant A. Damgude MBBS www.dosonline.org 47 Sumita Sethi MS, Mridula Mehta MS, DNB, Shibal Bhartiya MS, Sonia Bhargav MS, DNB T he Relative Afferent Pupillary Defect (RAPD), or MarcusGunn Pupil, named after British ophthalmologist Robert Marcus Gunn, is an extremely significant and highly objective clinical finding in the examination of the visual system1,2. There are many different conditions leading to RAPD, from severe glaucoma to an optic nerve tumor. Also significantly, there are many conditions which lead to a profound loss of vision (such as a complete vitreous hemorrhage), which will not give RAPD. It is a valuable clinical sign that should be used properly and not extrapolated to the point of irrelevance. Normally the usual pupillary response to direct light is that both pupils contract equally. While moving the light quickly from one eye to the other, both pupils should hold their level of contraction. When light is shown in the good eye, both pupils constrict. Then shifting it to the other eye, an injured optic nerve will also transmit (a) light but to a lesser and slower degree. As a result, when the light is moved from the good to the bad eye the brain interprets this as a decrease of light being presented. The brain’s response is to dilate both pupils to let in more light. This dilation response is in both eyes, despite only one eye being affected. This is the essence of the RAPD3,4,5 (Figure 1a & b). Neuro-Ophthalmology Relative Afferent Pupillary Defect Neuroanatomy and physiology of the pupillary light reflex pathway Pupillary size is governed by the balance of actions of two opposing muscle groups of the iris: the dilator and sphincter pupillae. Regulation of the pupillary size is predominantly achieved by reflex mechanisms in response to the amount of ambient light. (Figure 2 ) Constriction: The majority of retinal axons provide afferent information to the lateral geniculate nucleus en route to the primary visual areas on the calcarine cortex. However, approximately 10% of the fibers bypass the lateral geniculate nucleus and are relayed to the pretectal area of the rostral midbrain. These fibers travel through the brachium to synapse at the level of the superior colliculus. Second-order neurons subsequently relay pupillary light information to Edinger-Westphal nuclei bilaterally. This dual and near simultaneous innervation provides the anatomical basis for both the direct and the consensual light reflexes. From the EdingerWestphal nucleus, tertiary parasympathetic neurons travel in the superficial dorsomedial aspect of the ipsilateral occulomotor nerve (b) Figure 1: Schematic diagram showing (a) : normal pupillary response to a bright source of light (b): pupillary response in case of relative afferent pupillary defect Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi-110029 www.dosonline.org Figure 2: The pupillary light reflex pathway. (ON-optic nerve, OT-optic tract, PT-pretectal nucleus, EW-Edinger Westphal nucleus, CG-ciliary ganglion, SCN-short ciliary nerves 49 to reach the ciliary ganglion. The ganglion then gives rise to 8 to 10 short ciliary nerves , which subdivide into 16 to 20 branches. Approximately 3% of these fibers ultimately converge upon and innervate the pupillary sphincter muscles (promoting constriction), thereby contributing to the regulation of pupil size. Dilation: Pupillary dilation is mediated through three-neuron sympathetic (adrenergic) pathways that originate in the hypothalamus and descends caudally to the first synapse, located in the cervical spinal cord (levels C8-T2). The second-order neuron then travels from the sympathetic trunk, through the brachial plexus, over the lung apex. It then ascends to the superior cervical ganglion .The third-order neuron ascends within the adventitia of the internal carotid artery, through the cavernous sinus, where it is in close relation to the sixth cranial nerve. The oculosympathetic pathway then joins the ophthalmic (V1) division of the trigeminal nerve, and innervate the iris dilator muscle. Clinical examination and quantification of Rapid afferent papillary defect The evaluation of the pupils is best performed in a dimly illuminated room. This allows for some pupillary dilation, while also allowing for the observation of the consensual pupillary response. It is reasonable to test the individual reaction of each pupil initially. In some cases one pupil may not be reactive due to a variety of conditions. In cases where neither pupil reacts to light, no further testing can be done, except for testing the pupillary response to accommodation (focusing at near). The “swinging flashlight test” is a popular and an established way for identifying an RAPD6-10. In this test, a strong, steady light is used. The light is shone into one eye, quickly switched to the other and is repeated back and forth. Since light in one pupil causes both pupils to constrict, quickly switching from one eye to the other will give a “relative” indication of the functioning of each eye and optic nerve. If both eyes are equally dysfunctional, no “relative” defect would be found. The results of the test can be graded as follows (as per the I-V system of Bell et al)9. Grade I, initial weak constriction with greater redilatation; Grade II, initial stall and greater redilatation; Grade III, immediate pupillary dilatation; Grade IV, immediate pupillary dilatation following 6 seconds of illumination; Grade V, immediate pupillary dilatation with no secondary constriction at all. Certain standard conditions have been identified for applying this grading. The patient fixes on a 6/60 Snellen letter at 6 metres in a darkened room. An indirect ophthalmoscope head set (standardised as for the luminance measurements) is used as the light source. A swinging flashlight test is than carried out with each pupil illuminated for 3 seconds. However subjective grading leads to discrepancies between clinicians, is difficult to quantify, and limits its use in diagnosis. By accurately quantifying the defect additional information can be provided, which may aid management—for example, assessing retinal ischaemia following central retinal vein occlusion.13 Thompson et al describe a method of quantification of RAPD using neutral density filters7.The swinging flashlight test is 50 performed with increasing density of filter before the healthy eye until the sign disappears or is reversed. Neutral density filters (NDF) are available in the form of a rack or bar containing 10 filters of increasing density. The density of the filters is measured in log units ranging from 0.3 to 3.0 in steps of 0.3. Servais et al quantified RAPD using NDF in patients with CRVO13 ; 90% of non-ischaemic CRVO had an RAPD of 0.3 log unit or less and none greater than 0.9. Ischaemic CRVO had RAPD of 1.2 or greater in 91% and none less than 0.6. Ramsay et al have shown that crossed polarised filters (CPF) produce exponential attenuation of light and can be used in the same way as NDF to assess RAPD in CRVO.11 However the limitation with the use of NDF and CPF is their scarce availability in the clinical setting. The Sbisa or Bagolini filter bar, is similar to NDF, and is used commonly by orthoptists to assess density of suppression in strabismus. It consists of 17 sequential red filters. While Sbisa bar filters are red and NDF neutral, it is the apparent brightness not colour that determines pupil response (Figure 4 a & b). Sbisa bars are generally much easier to obtain than NDFs in most departments, McCormick et al, have described their use in the same way as the NDF, to neutralise and therefore quantify a relative afferent pupillary defect14. Quantifying RAPD by these methods depends upon clearly identifying an end point. The end point can be taken as either the elimination of the sign (brisk constriction with the same redilatation in both eyes) or reversal (looking at the eye with the RAPD-brisk constriction by direct illumination and dilatation by indirect illumination). Step vise approach to a patient of RAPD Knowing whether the patient is aware of changes in vision. If so, are these changes acute or gradual? An acute optic neuropathy or retinal detachment would be associated with sudden vision loss, while gradual vision loss might support the existence of a compressive lesion.15-17 Eliciting significant ocular and medical history. Does the patient have a history of vascular disease, cancer, auto-immune disease, recent infections or trauma? Carefully examining the patient while paying special attention to the retina and optic nerve. Optic nerve findings can be subtle or nonexistent. For instance, a relatively normal appearing nerve might be present in a retrobulbar Optic Neuritis. The need to refer for further testing depends on the case. If the patient’s history and retinal examination do not offer an obvious explanation for the RAPD, one must assume that a condition affecting the optic nerve or optic tract is causing it. An immediate visual field and color vision test should be performed. Depending on the condition, the patient may need to be referred for emergent neuro-imaging and laboratory testing . For example, an individual who presents with an RAPD as a result of swollen optic nerve and a history of leukemia, would require emergent imaging and radiation treatment if the swollen nerve represented a leukemic infiltrative optic neuropathy. Computed tomography (CT Scan) and Magnetic resonance imaging (MRI) are valuable tools for diagnosis of optic nerve DOS Times - Vol. 14, No. 8, February 2009 Figure 3: CT scan findings in a few patients with RAPD: (a): Sclerosis of greater wing of sphenoid bone with enhancing soft tissue components in the middle cranial fossa and orbit on right side, s/o Sphenoid wing meningioma, extending to the orbit on right side (b): Oblong mass in the right orbit – retrobulbar region, s/o probable diagnosis of optic nerve glioma (c): Well defined intensely enhancing intraconal lesion in the right orbit, s/o probable diagnosis of cavernous hemangioma disorders (especially optic nerve tumours and traumatic optic neuropathy) and in orbital diseases causing compressive damage to optic nerve (Figure 3 a,b,c). • Orbital disease – Including compressive damage to the optic nerve from thyroid related orbitopathy (compression from enlarged extraocular muscles in the orbit), orbital tumors, or vascular malformations. A RAPD is an index of significant optic nerve or retinal disease, when there is a difference in the disease process between the two eyes. If both eyes have severe but equal disease, there will be no RAPD. Thus, a “bilateral” RAPD does not exist. • Radiation optic nerve damage • Miscellaneous optic neuropathies, such as Leber’s optic neuropathy (usually eventually bilateral) and other inheritable optic neuropathies. • Severe disease in one eye,causing a RAPD ,will not result in anisocoria. The pupil of the diseased eye will be equal in size to that of the other eye due to the consensual light reaction (unless the iris itself is diseased or unreactive). • Optic nerve infections or inflammations - Cryptococcus can cause a severe optic nerve infection in the immunocompromised. Sarcoidosis and Lyme disease can cause inflammation of the optic nerve. • One functioning pupil is sufficient to determine the presence of an RAPD, (due to the presence of consensual light reflex). • • The visual acuity does not necessarily correlate with an RAPD. Some conditions lead to a marked reduction of visual acuity with an RAPD, while others spare the central vision. Usually, extensive loss of peripheral vision is associated with an RAPD. Surgical damage to the optic nerve - This could include damage following retrobulbar anesthesia; damage following orbital hemorrhage related to eye, orbital, sinus, or plastic surgery; damage following neurosurgical procedures such as pituitary tumor resection; and damage related to migration of an orbital plate after surgery to correct a blow-out fracture. Characteristics of the Relative Afferent Pupillary Defect • Conditions leading to a Relative Afferent Pupillary Defect Retinal Causes of a Relative Afferent Pupillary Defect Optic nerve disorders Usually retinal disease has to be quite severe for an RAPD to be clinically evident. Symmetrical bilateral retinal disease will not show an RAPD. • Optic neuritis - Even very mild optic neuritis with a minimal loss of vision can lead to a very strong RAPD. • Ischemic optic neuropathies - These include arteritic (Giant Cell Arteritis) and non-arteritic causes. Usually associated are a loss of vision or a step deformity in the visual field. • Glaucoma – Though glaucoma normally is a bilateral disease, asymmetric optic nerve involvement can result in an RAPD. • • Traumatic optic neuropathy - This includes direct ocular trauma, orbital trauma, and remote head injuries which can damage the optic nerve as it passes through the optic canal into the cranial vault. Optic nerve tumor - This is a rare cause, and includes primary tumors of the optic nerve (glioma, meningioma) or tumors compressing the optic nerve (sphenoid wing meningioma, pituitary lesions, etc.) www.dosonline.org • Ischemic retinal disease - including ischemic central retinal vein occlusion, central retinal artery occlusion, severe ischemic branch retinal or arterial occlusions, severe ischemic diabetic or sickle-cell retinopathy. • Ischemic ocular disease (Ocular ischemic syndrome) – due to obstruction/compression of the ophthalmic or carotid artery on one side. • Retinal detachment - An RAPD may be seen if the macula is detached, or if at least two quadrants of retina are detached. • Severe macular degeneration - If unilateral and severe • Intraocular tumor - Retinal and choroidal tumors if severe (including melanoma, retinoblastoma, and metastatic lesions) 51 (a) Figure 4 (a): Neutral density filter (b): Sbisa bar. Note that Sbisa bar filters are red and NDF neutral Retinal infection – Extensive retinitis (Cytomegalovirus, herpes simplex,). Amblyopia If severe, can lead to a relative afferent pupillary defect. Usually the visual acuity would be 20/400, or worse. Cerebral Vascular Disease Usually, it is an optic nerve disorder that leads to an RAPD, rather than an optic tract or visual cortex disorder. However, there tends to be a higher percentage of crossed vs. uncrossed nerve fibers at the optic chiasm. Thus, in a patient with a homonymous hemianopia from an optic tract disorder, an RAPD maybe noted in the eye with the temporal visual field defect. The nasal retina serves the temporal visual field, and these are the fibers that would cross at the chiasm. Conditions not leading to a Relative Afferent Pupillary Defect • Bilateral nutritional or metabolic optic neuropathies . 1. Wikipedia contributors, ‘Marcus Gunn pupil’, Wikipedia, The Free Encyclopedia,17July2008,21:11UTC,<http://en.wikipedia.org/w/ index.php?title=Marcus_Gunn_pupil&oldid=226320762>. 2. Thompson HS. Pupillary signs in the diagnosis of optic nerve disease.Trans Ophthalmol Soc UK 1976;96:377-81. 3. Kardon, R. Anatomy and physiology of the autonomic nervous system. In: Walsh and Hoyt Clinical Neuro-ophthalmology, 6th ed, Miller, NR, Newman, NJ, Biousse, V, Kerrison, JB (Eds), Williams & Wilkins, Baltimore 2005. p.649. 4. Johnston, JA, Parkinson, D. Intracranial sympathetic pathways associated with the sixth cranial nerve. J Neurosurg 1974; 40:236. 5. Digre, KB. Principlesand techniques of examination of the pupils, accommodation and lacrimation. In: Walsh and Hoyt Clinical Neuroophthalmology, 6th ed, Miller, NR, Newman, NJ, Biousse, V, Kerrison, JB (Eds), Williams & Wilkins, Baltimore 2005. p.715. 6. Enyedi, L.B. et al. A Comparison of the Marcus Gunn and Alternating Light Tests for Afferent Pupillary Defects.Ophthalmology 5/1998; 105:871-873. 7. Thompson HS, Corbett JJ, Cox TA. How to measure the relative afferent pupillary defect.Diagnostic and Surgical Techniques 1981;26:39–42. 8. Browning DJ, Tiedeman JS. The test light affects quantitation of the afferent pupillary defect.Ophthalmology 1987;94:53–5. 9. Bell RA, Waggoner PM, Boyd WM, et al. Clinical grading of relative afferent pupillary defects.Arch Ophthalmol 1993;111:938–42. 10. Sun F, Tauchi P, Stark L. Binocular alternating pulse stimuli: experimental and modelling studies of the pupil reflex to light.Math Biosci 1983;67:225–45. 11. Ramsay A, Williamson TH, Parks S, et al. Crossed polarising filters to measure relative afferent pupillary defects: reproducibility, correlation with neutral density filters and use in central retinal vein occlusion.Eye 1995;9:624–8. 12. Thompson HS. Pupillary signs in the diagnosis of optic nerve disease.Trans Ophthalmol Soc UK 1976;96:377–81. • Refractive Error 13. Servais GE, Thompson HS, Hayreh SS. Relative afferent pupillary defect in central retinal vein occlusion.Ophthalmology 1986;93:301– 3. • Media Opacity including Cataract, Corneal scar, Hyphema, vitreous hemorrhage. Previous eye surgery 14. A McCormick, R Bhola, L Brown, D Squirrel, J Giles, I Pepper. Quantifying relative afferent pupillary defects using a Sbisa bar. Br J Ophthalmol. 2002 September; 86(9): 985–987. • Strabismus • Conditions with an Efferent Pupillary Defect 15. Johnson R, Bell R. Relative afferent pupillary defect in a lesion of the pretectal afferent pupillary pathway. Can J Ophthalmol. 1987;22:282-284. • • 52 Bilateral retinitis pigmentosa References (b) • • • Third Cranial Nerve Palsy • Adie’s Pupil • Horner’s Syndrome Mild retinal diseases • Mild background diabetic retinopathy • Central serous choroidopathy • Non-ischemic vein occlusions • Mild macular degeneration Conditions which are typically bilaterally symmetrical will not show an RAPD: 16. Forman S, Behrens M, Odel J, Spector R, Hilal S. Relative afferent pupillary defect with normal visual function. ArchOphthalmol. 1990; 108:1074-1075. 17. Girkin C, Perry J, Miller N. A relative afferent pupillary defect without any visual sensory defect. Arch Ophthalmol. 1998;116:15441545. First Author Sumita Sethi MS DOS Times - Vol. 14, No. 8, February 2009 Photo Essay Bilateral Anophthalmos in Two Brothers: A Case Report Garima Agrawal MS, D.C. Mehta MS A rare case of bilateral congenital anophthalmos in two brothers both having the same congenital anomaly is reported. Anophthalmos is a rare congenital anomaly and the same anomaly in two siblings is even rarer. Anophthalmos is a condition in which there is absence of one or both eyes. It is a congenital anomaly. Here we report a case of two siblings with bilateral congenital anophthalmos. Case Report Two children one 6 months old and the other 2 years old were brought to our OPD by their parents for ocular examination. On examination the babies appeared healthy and smiling (Figures 1-4) except for the eyes. There was no systemic abnormality. On ocular examination eyelids were present but curved in. Eyelashes were present. Lacrimal puncta were present. Palpebral aperture was almost absent. After the eyelids were retracted no ocular tissue was visible clinically except for the conjunctiva in both the eyes of both the children. (Figures 1-6). Both the orbits were small. Ultrasonography revealed no normal ocular tissue. The 2 children had no other siblings. They were both full term normal deliveries. The parents were both normal. Mother’s antenatal history was normal and she had not taken any drug during the 2 pregnancies. Figure 1: 2 year sibling with bilateral anophthalmos Figure 3: Left eyeball not seen on retraction of the eyelids in child in figure 1. Figure 2: Right eye ball not seen on retraction of the eyelids in child in figure 1 Figure 4: 6 month old sibling with bilateral anophthalmos M&J Institute of Ophthalmolgy B J Medical College, Ahmedabad. www.dosonline.org 55 Figure 5: Right eyeball not seen on retraction of eyelid of child in Figure 4 Discussion The first case of anophthalmos was reported by Thomas Bartholin in 1657. His case had a bilateral anophthalmos associated with abnormalities of nose, mouth, lips and extremities. Von Hippel collected 87 cases in literature out of which 64 were bilateral and 23 unilateral1. Primary clinical anophthalmos as described by Duke Elder consists of a small but well formed orbit. The lid are curved in with small orbital aperture. The orbital cavity is lined with conjunctiva. The lacrimal puncta and the eyelashes are usually present or either may be absent. Certain other associated ocular abnormalities described are ankyloblepharon, epicanthus, absent lacrimal gland, absent optic nerve, narrow optic canal, an abnormal development of optic chiasma and lateral geniculate body1. True anophthalmos is a histopathological diagnosis where there are no recognizable ocular structures present in the orbit. The term clinical anophthalmos is used when there is no evidence of a globe or tissue on clinical examination but without any histopathological confirmation (Duke Elder 1964)1. A number of cases of anophthalmos have been reported. Those in siblings are very rare. Ashley (1947) has reported a case of bilateral anophthalmos in a brother and sister2. Sensi A et al (1987) reported monolateral or bilateral anophthalmos recurring in the absence of other associated defects in 6 members of a family. The malformation appears to be inherited as a dominant(autosomal/X linked) trait with incomplete penetrance3. Ben Ezra D, Sela M, Peer J reported a case of four uneventful pregnancies and deliveries in one family. 2nd sibling suffered from bilateral anophthalmia while 4th presented with a unilateral microphthalmia. The microphthalmia was due to in utero globe perforation as evident by a central corneal scar with cataractous lens4. Other than this there have been reports of isolated bilateral 56 Figure 6: Left eyeball not seen on retraction of eyelid of child in Figure 4. anophthalmos in one child. Few associated with other abnormalities and few not. Rosenbaum(1917) reports a case of congenital bilateral anophthalmos of a male child who presented with absence of both eyes at birth. In other aspects the child was healthy and well developed. On the third day however the child died in convulsions. No autopsy was done5. Maria DL et al (1967) reported a case of bilateral congenital anophthalmos in a premature male child6. Chapu BA (1975) reported a case of congenital bilateral primary anophthalmos in a full term otherwise healthy child at birth1. Brunquell PJ et al (1984) report a 27 year old man with X linked true anophthalmos. No evidence of optic globe, nerves or chiasma was found. Rudimentary structures suggesting optic tracts were present. Lateral geniculate nuclei were present but gliotic calcrine cortex was thinner but had usual lamination. The normal patches of cytochrome oxidase activity in layers II and III of visual cortex were absent7. Albernaz VS et al reported the imaging findings in patients with clinical anophthalmos. They found that patients with bilateral anophthalmos represented a distinct group fom those with unilateral anophthalmos. Bilateral anophthalmos was associated with absence of optic chiasma, reduced size of posterior optic pathways and agenesisn or dysgenesis of corpus callosum. Patients with unilateral anophthalmos had severe cranio-facial anomalies. Imaging of the face is helpful in patients with unilateral anophthalmos8. The etiology of this disorder is complex Genetic and environmental causes have been postulated. Studies that can specifically identify the causative factors for this disorder are warranted. Conclusion A case of bilateral anophthalmos in two brothers is reported. This is a very rare case where the same anomaly of bilateral anophthalmos has been seen in two brothers. The author could not come across a similar article in literature. DOS Times - Vol. 14, No. 8, February 2009 References 1. Chapu BA. Bilateral primary anophthalmos. Indian J Ophthalmol.1975;23(4):36-37. 2. Ashley. Bilateral Anophthalmos in a brother and sister.J Hered.1947;38:174-176. 3. Sensi A, Incorvaia C, Sebastian A, Calzolari E.Clinical Anophthalmos in a family. Clinical Genetics.1987;32(3):156-159. 4. Ben Ezra D, Sela M, Peer J. Ophthalmologica 1989;198(3):140-4. 5. Rosenbaum.Congenital bilateral anophthalmos. Can Med Assoc J.1919 March;9(3):254-256. 6. MariaDL, Srivastava SK, Sankholkar PS. Bilateral congenital anophthalmos. Indian J Ophthalmol 1967;15:241-2. 7. Brunquell PJ, Papale JH, Horton JC, Williams RS, Zgrabik MJ, Albert DM, Hedley-Whyle CT. Sex linked hereditary bilateral anophthalmos. Pathologic and radiologic correlation. Arch Ophthalmol.1984;102(1):108-113. 8. Albernaz VS, Castillo M, Hudgins PA, Mukherji SK. Imaging findings in patients with clinical anophthalmos.Am J Neuroradiology.1997;18(3):555-561. First Author Garima Agrawal MS www.dosonline.org 57 Julie Pegu MS, Suneeta Dubey MS, Manisha Agarwal MS S erious elevation of intraocular pressure (IOP) after trauma is the result of functional impairment of the aqueous outflow pathways. With blunt injury, the typical sequence of events is an initial tearing of the angle structures (contusion angle injury), which is frequently associated with intraocular bleeding and inflammation and results in scarring and permanent reduction in the facility of outflow. Traumatic glaucoma is often refractory to conventional treatment and most cases may require surgical intervention. However, there is no set protocol for the surgical management as the results are unpredictable because of the complex nature of trauma. The treatment therefore, should be individualized depending on the extent of injury and the visual prognosis. This case represents the dilemma a clinician can encounter while managing a case of traumatic glaucoma. perception of light, and IOP was 50 mm Hg. There was presence of ciliary congestion, diffuse corneal epithelial edema and diffuse hyphema. The pupil was irregular, semi dilated and sluggishly reacting to light. There were multiple sphincter tears present superiorly. The rest of the anterior segment (AS) view was hazy on day one. B scan USG showed signs of vitreous hemorrhage. Routine anti glaucoma treatment was initiated with tab acetazolamide (half tab TID) and timolol maleate, 0.5%, (BID), along with topical corticosteroids and cycloplegics. On day four, the hyphema had cleared sufficiently and the AS view was clearer which showed the presence of mild inferior iridodonesis (Figure 1). The inferior iridodonesis made us to suspect the presence of some amount of Clinical Meeting: Clinical Case 1 Management Dilemma in a case of Traumatic Glaucoma Case A 14 year male presented to us on 15th may 2008 with the history of blunt trauma to the right eye with a cricket ball, following which he complained of severe pain, redness and marked diminution of vision in the right eye. There was no history of presence of any systemic disease or blood disorders. There was no family history of glaucoma. On slit lamp examination, the left (LE) was essentially within normal limits. The vision in the right eye was decreased to Figure 2: Vitreous from behind the lens blocking the lumen of the tube Figure 1: Slit lamp picture of the right eye on the fourth day Figure 3: Pulling of the vitreous tag with an end gripping forcep after anterior vitrectomy Dr. Shroff's Charity Eye Hospital, 5027 Kedarnath Road, Daryaganj, New Delhi www.dosonline.org 61 Figure 4: After vitrectomy the tube was cleared of the vitreous Few vitreous strands were seen inferiorly in anterior chamber but not blocking the tube zonular dehiscence inferiorly but there was no obvious phacodonesis or vitreous prolapse. The IOP was still high at 46 mm Hg. Consequently, anti glaucoma treatment was stepped up to maximal medical treatment (glycerol syrup and brimonidine eye drop were added). From day six today 24, the vision had improved from counting finger at one meter to 6/36, but the IOP was still very high at early forties. Gonioscopy done on the third week, revealed angle recession extending 120 to 180 degrees with a resolving blood clot present inferiorly. Rest of the angle was wide open. The left eye had wide open angles. The vitreous hemorrhage had cleared by the third week and the fundus was found to be within normal limits with a cup disc ratio of 0.4 : 1. The LE had a C:D ratio of 0.3:1. At this stage we decided to intervene surgically. We were weighing two options, either to do trabeculectomy with MMC or a tube implant. In this case, we decided to choose the later as the patient was young and the presence of inferior iridodonesis could have complicated the trabeculectomy surgery, as sudden decompression of the anterior chamber (AC) might lead to vitreous prolapse. With valved implants the results tend to be more predictable as the AC is maintained throughout, and complications like postoperative hypotony are uncommon. So, right eye Ahmed valve implantation was done on day 28. The surgery was uneventful. On first postoperative day the IOP came down to 10 mm Hg but on the fourth day, the IOP again rose to 52 mm Hg and we noticed a band of vitreous coming from behind the inferior part of the lens and blocking the tube (Figure 2). It is important to mention here that there was no vitreous in the AC pre-operatively but due to the negative pressure in the AC, vitreous got pulled from the site where mild inferior iridodonesis was noted. Anti glaucoma drugs were started but the IOP was uncontrolled and on day 10 surgical removal of the vitreous tag was done. The procedure involved cutting the vitreous tag midway with vitrectomy cutter and then pulling the tag from the tube lumen with an end gripping forcep (Figure 3). Postoperatively, the IOP was controlled and the tube was free of vitreous block (Figure 4). In the last follow up (at 6 months), the IOP was maintained at low teens, vision was 6/9, the tube was free of any blockage (Figure 5) with a well formed bleb (Figure 6). 62 Figure 5: At 6 months follow up Figure 6: Well formed bleb at 6 months follow up Discussion In severe trauma with associated findings like hyphema, angle recession and vitreous hemorrhage, the outlook for trabeculectomy and other drainage procedures is relatively unfavourable. Trabeculectomy is reported to be more frequently successful in patients over the age of 40 years1 than in younger patients. In one of the study2, the success rate of trabeculectomy was only 38% (17/45) in patients aged 10 to 29 years whereas it was 65% (43/65) in patients aged 30 to 49 years. As the young people are more susceptible to trauma, the surgical prognosis becomes poorer because of their relative youth. The young age of the patients produces a strong tendency for blebs to scar down and fail. The adjunctive use of cytostatic agents have shown favourable results when applied but its use can have the serious disadvantage of tending to produce dangerously thin blebs in patients who have a long expectancy of life3. Also, the use of mitomycin C can lead to DOS Times - Vol. 14, No. 8, February 2009 serious ocular complications like late endophthalmitis and ocular hypotony that may result in permanent visual loss4,5. There are limited studies on the long term surgical outcome in traumatic glaucoma. In one of the largest series of 87 cases of traumatic glaucoma by Mermoud et al6 with a mean follow up of 23 months (range, 3 to 72 months), the success rate in trabeculectomy group (47 cases) was 53%, in trabeculectomy combined with antimetabolite group (20 cases) it was found to be 70% and in the molteno implant group (20 cases) it was 60%. Of concern were three cases of late bleb infection in the group that received postoperative antimetabolite therapy, one of whom lost all sight. In another long term series 3, insertion of molteno implants in traumatic glaucoma produced IOP control at 10.9 years in 76% of the cases (40 cases). There were no implant related infections. However, there were eleven IOP related surgical interventions, most related to blockage or malposition of the internal end of the tube, but the IOP was controlled after clearance of the vitreous with vitrectomy. In traumatic glaucoma, the success rate of the surgical complications is expected to be higher because of the complex trauma, but if treated with vigilance and on time, most of it can be managed with a favourable prognosis. with anti metabolites or the use of an artificial draining device, with frequent follow ups3. Finally, patients having filtration surgery should be carefully instructed on the early signs and symptoms of bleb-related endophthalmitis and told to report immediately if these occur, as early treatment appears to be associated with an improved prognosis for visual recovery and bleb function. References 1. Stewart RH, Kimbrough RL, Bacch H, Allbright M. Trabeculectomy and modifications of trabeculectomy. Ophthalmic Surg 1979; 10:7680. 2. Gressel MG, Heuer DK, Parrish RK II. Trabeculectomy in young patients. Ophthalmology 1984; 91:1242- 6. 3. Fuller JR, Bevin TH, Molteno AC. Long term follow up of traumatic glaucoma treated with Molteno implants. Ophthalmology 2001; 108:1796-1800. 4. Greenfield DS, Suner IJ, Miller MP, et al. Endophthalmitis after filtration surgery with mitomycin. Arch Ophthalmol 1996; 114:9439. 5. Higginbotham EJ, Stevens RK, Musch DC, et al. Bleb related endophthalmitis after trabeculectomy with mitomycin C. Ophthalmology 1996;103:650-6. 6. Mermoud A, Salmon JF, Barron A, et al. Surgical management of post-traumatic angle recession glaucoma. Ophthalmology 1993; 100:634-42. Conclusion Traumatic glaucoma encompasses a spectrum of disease. Because of the greater chance of failure of primary trabeculectomy in traumatic glaucoma, it has been suggested that this type of glaucoma would be best managed by trabeculectomy combined First Author Julie Pegu MS Monthly Clinical Meetings Calendar 2008-2009 Dr. R.P. Centre for Ophthalmic Sciences Centre for Sight 3rd August, 2008 (Sunday) 7th December, 2008 (Sunday) Venu Eye Institute & Research Centre Mohan Eye Institute 7th September, 2008 (Sunday) 28th December, 2008 (Sunday) Army Hospital (R&R) DOS Picnic 28th September, 2008 (Sunday) Sunday, 4th January, 2009 World Sight Day Shroff Charity Eye Hospital 8th October, 2008 (Wednesday) 1st February, 2009 (Sunday) Sir Ganga Ram Hospital Guru Nanak Eye Centre 2nd November, 2008 (Sunday) 22nd February, 2009 (Sunday) Midterm Conference of DOS Bharti Eye Foundation 22nd & 23rd November, 2008 (Saturday - Sunday) 8th March, 2009 (Sunday) Annual Conference of DOS 20th-22nd March, 2009 (Friday, Saturday & Sunday) www.dosonline.org 63 Ritesh Narula MS, Manisha Agarwal MS, S.P. Chaudhary MS, Priyata Seth DO W e report a case with an atypical presentation of sympathetic ophthalmia (SO) in a child caused by multiple surgical interventions in the exciting eye. Fundus fluorecein angiography and Optical coherence tomography findings were noted. Early diagnosis and management with intravenous methyl-prednisolone and immunosuppressants helped in salvaging the only seeing eye of the child. Case report A nine- year- old boy presented with decrease of vision in the left eye for 3 days duration. He had a history of cataract surgery with intraocular lens implantation done at the age of nine months elsewhere followed by membranectomy under general anesthesia. He had a history of blunt trauma with a finger to the right eye causing retinal detachment for which he underwent pars plana vitrectomy with IOL repositioning and was subsequently diagnosed to have recurrent total retinal detachment for which no intervention was advised. On examination the best corrected visual acuity in the right eye was inaccurate projection of rays and in the left eye 6/24, N24. Applanation tonometry recorded an intraocular pressure of 6 mm of Hg in the right eye and 14 mm of Hg in the left eye. Slit lamp examination of the right eye showed organized blood clot in the anterior chamber with vascular in-growth (Figure 1) and the left eye showed minimal retrolental flare. There was no view of the fundus in the right eye and the fundus examination of the left eye showed pocket of sub-retinal fluid (SRF) at the macula. Fundus fluorescein angiography (FFA) of the left eye showed multiple sites of choroidal leakage with coalescence of dye under serous detachment of the retina. (Figure 2a-e).Optical coherence tomography of the left eye showed multiple pockets of subretinal fluid with hyperreflective echoes underneath the serous retinal detachment. (Figure 3) Clinical Meeting: Clinical Case 2 Posterior Sympathetic Ophthalmia in a Child The child was diagnosed as sympathetic ophthalmia and treated with intravenous methylprednisolone for 3 days followed by a tapering dose of oral prednisolone along with topical prednisolone acetate 1% six times a day and atropine-1% two times a day in the left eye. Enucleation of the right eye was advised which was refused by the father of the child. Follow-up at one week the BCVA in the left eye improved to 6/9, < N36 (pharmacologically dilated pupils under atropine-1%). Applanation tonometry recorded an IOP of 16mm of Hg in the left eye. Fundus examination of the left eye showed a residual pocket of shallow SRF. At one month follow-up the BCVA in the left eye was 6/6, N9P. Applanation tonometer recorded an IOP of 38mm of Hg. Fundus examination and OCT of the left eye showed no evidence of SRF at the macula. The child was started on oral and topical antiglaucoma drugs. Topical prednisolone-1% was stopped and oral prednisolone was tapered and stopped. At two months the child presented with distortion of images in the left eye. The BCVA in the left eye was 6/12P, N18P. Fundus examination and OCT of the left eye showed SRF at the macula suggestive of recurrent inflammation in the left eye. (Figure 4) Diagnosing as recurrent sympathetic ophthalmia the child was treated again with intravenous methylprednisolone followed by oral and topical prednisolone. Oral Azothioprine - 1.5 mg/kg body weight was started after a systemic evaluation and complete blood counts. Enucleation of the right eye was done after an informed consent. Histopathology of the enucleated eye showed organized haemorrhage in the anterior and posterior chamber with histiocytic & foreign body giant cell & granulomatous reaction. The child showed aprompt recovery and is maintain a best corrected visual acuity of 6/6, N6 after 6 months of follow up. Discussion Figure 1: Right eye showing organized blood clot with vascular ingrowth Retina and Oculoplasty Services Dr. Shroff’s Charity Eye Hospital, Daryaganj, New Delhi www.dosonline.org Sympathetic ophthalmia, also known as sympathetic uveitis, is a rare bilateral granulomatous panuveitis that occurs after a penetrating injury to an eye. Following injury to an eye-a result of either surgery or accident-a variable period of time passes before a sight threatening inflammation develops in both eyes. The injured eye is known as the exciting eye and the fellow eye as the sympathizing eye1. The various surgical procedures implicated for sympathetic ophthalmia are- cataract extraction (particularly when complicated), iris surgery (iridectomy), retinal detachment repair, vitrectomy, paracentesis, cyclodialysis and keratectomy2. The risk 65 Figure 2 (A-E): Fundus fluorescein angiography (FFA) of the left eye showed multiple sites of choroidal leakage with coalescence of dye under serous detachment of the retina Figure 3: Optical coherence tomography of the left eye showed multiple pockets of subretinal fluid with hyperreflective echoes underneath the serous retinal detachment of SO increases when these surgical procedures are accompanied or followed by additional operations, particularly in the posterior segment of the eye3. Advances in eye care especially in surgical techniques have dramatically decreased the incidence of SO in the past century. The incidence of post-vitrectomy SO has been estimated at 0.01%4. On review of the literature SO is said to be extremely small after 3 months though it never reaches zero2, and the typical symptoms are photophobia, loss of accommodation or watering and signs 66 Figure 4: Fundus examination and OCT of the left eye showed SRF at the macula suggestive of recurrent inflammation in the left eye DOS Times - Vol. 14, No. 8, February 2009 include bilateral low grade persistent uveitis associated with granulomatous (“mutton-fat”) or small, white keratic precipitates, diffuse thickening of the iris or iris nodules and Dalen Fuchs nodules on fundus examination though not pathognomic of SO1. However our case differed in the following ways- the child developed SO several years after the last surgical intervention in the exciting eye, he presented with a sudden drop of vision there was only mild retrolental cells and flare in the sympathizing eye with no signs of anterior segment inflammation in either eye. Lastly on fundus examination there was serous retinal detachment at the macula. All these factors made the diagnosis of SO difficult in our case but based on clinical suspicion we started the child on treatment and were able to salvage the eye. Enucleation of the exciting eye having no visual potential helps in preventing the recurrence of SO by decreasing the antigen load causing inflammation repeatedly. Histopathology of the enucleated eye also helped in confirming the diagnosis of SO in our case. The presentation in this case closely resembles an entity described in literarture as “ Posterior sympathetic ophthalmia”. It was first described by Mcpherson et al 5 as a variant of sympathetic ophthalmia with predominantly posterior segment signs and minimal AC reaction. In a long term study of 40 patients from North India by Gupta et al6; 36 of these patients presented with evidence of predominantly posterior segment involvement with 22 eyes of these having no anterior segment inflammation. Whether these cases represent an atypical form of sympathetic ophthalmia or just the early stage of classical presentation is debatable. We report this case to highlight that SO may have an atypical clinical presentation and a high clinical suspicion maybe required for a timely diagnosis and management to salvage the vision in the sympathesizing eye which at many a times is the only seeing eye of the patient as in this case. References 1. Duke-Elder S, Perkins ES. Diseases of the Uveal Tract. Vol 9. In: Duke-Elder S, ed. System of Ophthalmology. Vol 9. London, England: Henry Kimpton; 1966: 558–559. 2. Lubin JR, Albert DM, Weinstein M. Sixty-five years of sympathetic ophthalmia. A clinicopathologic review of 105 cases (1913– 1978).Ophthalmology 1980; 87:109–21. 3. Goto H, Rao NA. Sympathetic ophthalmia and Vogt-KoyanagiHarada syndrome. Int Ophthalmol Clin 1990; 30:279–85. 4. Gass JD. Sympathetic ophthalmia following vitrectomy. Am J Ophthalmol1982; 93:552–8. 5. McPherson SD Jr, Dalton HT. Posterior form sympathetic ophthalmia.Trans Am Ophthalmol Soc. 1975;73:251-63. 6. Gupta V, Gupta A, Dogra MR. Posterior sympathetic ophthalmia: a single centre long term study of 40 patients from north india. Eye. 2008 Dec;22(12):1459-64. First Author Ritesh Narula MS www.dosonline.org 67 Cyrus M. Shroff MD T he practise of vitreo-retinal medicine and surgery has changed dramatically in the past few years. Gone are the days when retinologists were the butt of jokes like "They always carry their vision charts on them - their hands!" The past year has been a satisfying one for us, a year primarily of consolidation of some of the recent advances in diagnostics, drugs (the anti-VEGF revolution!) and lasers. We will review the advances made in the retinal scenario along with exciting future possibilities under the following headings: • Retinal Imaging • Lasers • Drugs and their applications -with special reference to AMD and Diabetic Retinopathy. Expanding indications of anti-VEGF agents. • Pushing the frontiers • Gene Therapy • Nano Medicine • Artificial Retina- bionic eye Retinal Imaging Spectral and ultra-high resolution OCT are now quite firmly ensconced in the armamentarium of retinal diagnostics and these have the advantage of 3 Dimensinal imaging. Also 100% of the mapped area is measured in Spectral domain compared to only 5% in time domain, so there are less chances of missing a small lesion with the newer modalities. (Figure 1) One of the manufacturers of SD-OCT (Bioptigen) provides a novel feature of a hand-held probe opening up new capabilities to researchers and clinicians - it's easily used on experimental animals, in small children and immobile patients. Soon we may also have capability of intra-operative OCT attached to the microscope. that may occur with disease, in the RPE cell monolayer in outer retina and subneurosensory retinal space. FAF gives information over and above that obtained by fundus photography, fundus fluorescein angiography and optical coherence tomography. It would be useful in understanding pathophysiologic mechanisms, phenotype-genotype correlation, identification of predictive markers for disease progression and monitoring of new therapies. Examples of diseases where this holds promise are AMD especially the dry type, macular and diffuse retinal dystrophies, central serous retinopathy, chorio-retinal inflammatory disease and Angioid streaks. It's clinical value coupled with its simple, efficient and noninvasive nature is increasingly appreciated. Clinical Meeting: Clinical Talk Future of Retinal Imaging, Lasers and Therapy Retinal lasers The pattern scan laser (PASCAL) has been an interesting development. The PASCAL laser's capabilities can be maximally exploited in fresh cases for pan retinal photocoagulation where it is indeed very quick. (Figure 3) Pan retinal photocoagulation can also be achieved with lower delivery of energy to the retina. However when fill in treatment is required it often needs to be used as a single spot laser, as also when there are media opacities or hemorrhage. High cost and lack of indirect ophthalmoscopic delivery have also been limiting factors. Nonthermal Retina Regeneration Therapy (2RT; Elex, Adelaide, Australia), is a short-pulsed laser treatment (pioneered by John Marshall and Peter Hamilton of UK) that is intended to treat diabetic maculopathy and macular edema at an earlier stage of the disease. The therapy may also have application in the treatment of early-stage age-related macular degeneration. The laser used for 2RT is a Q-switched, green Nd:YAG laser with a wavelength of 532 nm. Its pulse duration is 3 nanoseconds with energy of 1 J per pulse which causes the RPE cells to release matrix metalloproteinase enzymes, which are believed to 'clean up' the In the future we may be using adaptive optics SLO for ultimate high resolution OCT which can resolve a cone and could be used for the early detection of retinal degeneration. A combination of Spectral OCT and SLO which can perform microperimetry of small specific areas of retina Functional results can be overlaid on fundus image FA / ICG image and thickness map hence it combines analysis of structure and function. (Figure 2) Fundus autofluorescence imaging (FAF) has been in the spotlight. There was an excellent review in "Retina" of March 2008 by Schnitz - Valckenburg, Holz, Bird & Spaide1. This is a novel imaging method that allows mapping of lipofuscin (LF) and other fluorophores Dr. Shroff's Charity Eye Hospital & Shroff Eye Centre, New Delhi www.dosonline.org Figure 1: Time Domain versus Spectral Domain OCT 69 Pro-re-nata (PRN) basis with visual acuity, OCT and FA criteria. In the 'treat and extend' strategy it is recommended to treat till the macula is dry and continue to treat with extended time span between injections, even when dry. Figure 2: Combination OCT ICG "Combination treatment" combining PDT, and anti-VEGF agents, with or without intra-vitreal steroids, also has made the treatment more finite. Reduced fluence PDT has improved the safety profile. The results of two multicenter trials, SUMMIT trial and PDEX II trial comparing combination therapy to ranibizumab monotherapy are eagerly awaited. The SUMMIT trial has 3 arms; Standard fluence PDT with intra-vitreal ranibizumab, reduced fluence PDT with intra-vitreal ranibizumab and monthly intra-vitreal ranibizumab. In the PDEX II trial another effective modality, intravitreal steroids has been added. It compares reduced fluence PDT with 0.5 mg ranibizumab and 500mg dexamethasone to monthly ranibizumab. Two agents of promise in phase III trials are VEGF trap and Epiretinal Brachytherapy with Lucentis. VEGF trap is a recombinant protein which binds and inactivates circulating VEGF. Phase II studies with VEGF trap showed mean best corrected visual acuity improvement of 5.7 letters and reduction in CMT in all groups at 12 weeks compared to baseline. Figure 3: Pascal laser bruch's membrane. The laser thus triggers what is described as photoregeneration of the retinal pigment epithelium and Bruch's membrane. With 2RT, there is potential to have a laser treatment that affects only the RPE without damaging the photoreceptor cells, meaning that we can get closer to the fovea without damaging central vision. For the first time, there is the possibility of very early intervention in patients with diabetic macular edema to preserve a greater degree of functional vision for a longer time. Figure 4: Neovista Drugs and their Applications Age related macular degeneration In AMD management we have entered a 'consolidation phase". The two major challenges are early detection and dosing regimes. Early detection of AMD progression is the single most important factor for minimising the risk of permanent vision loss. Educating at risk population and general practitioners, good referral network and use of telemedicine should help towards this goal. The challenge of reducing the number of intra-vitreal anti-VEGF agents while striving to maintain the benchmark visual gain in trials with monthly injections (MARINA & ANCHOR) is a daunting one. The most commonly used strategy is to give a 'loading dose' of three, 4-6 weekly injections followed by further treatment on a 70 Figure 5: Neurotech NT 501 DOS Times - Vol. 14, No. 8, February 2009 Phase III study (View 2) is recruiting approx. 1200 subjects in over 200 centres worldwide. Primary objective is to assess the efficacy of intra-vitreal VEGF Trap compared to Ranibizumab in preventing vision loss in subjects with all subtypes of neovascular AMD. Epiretinal Brachytherapy (NEOVISTA) Beta radiation is applied to the macula after a vitrectomy, using a strontium 90 isotope source (Figure 4). A multicenter feasibility study enrolled 34 participants with any CNV type. These patients received a single 24 Gy treatment of epiretinal brachytherapy with two injections of intra-vitreal Avastin - one dose prior to or at the time of radiation delivery and another, one month later. Analysis of 18 month follow up on 25 patients showed mean improvement in visual acuity of 10.7 letters. 44% gained 15 or more letters and 8 % gained 30 or more letters. This is better than the visual results with current standard of care. CABARNET (CNV secondary to AMD treated with Beta Radiation Epiretinal Therapy) is a large phase 3 multicentre ongoing trial comparing combination treatment (Radiation plus Ranibizumab) to Ranibizumab monotherapy. The future therefore definitely lies in a combined attack on multiple sites of the angiogenesis cycle. Bevasiranib (CAND5) is a siRNA which switches off VEGF production, but clinical effect is not seen till the preexisting VEGF is cleared. It has a potentially longer duration of effect than currently available anti-VEGF agents. It is well tolerated at multiple doses. Phase 3 trial comparing initial neutralization of VEGF - 3 doses of Ranibizumab followed by Bevasiranib every 8 or 12 weeks (maintenance) versus Ranibizumab alone is currently underway. Fenretinide is a Retinoic acid derivative It is an orally administered drug being studied for use in geographic atrophy associated with dry AMD and it acts by reducing accumulation of lipofuscin in RPE cells. The Lucentis Vs Avastin debate rages on. The retinal world eagerly awaits the results of the Comparison of AMD Treatments, Lucentis Vs Avastin Trial (CATT). This is a phase III trial currently recruiting patients (target 1200 patients) at 57 locations. Objective is to evaluate relative efficacy and safety of treatment of neovascular AMD with Lucentis and Avastin with fixed and variable dosing schedules. Preliminary results are expected in 2010 and study would be completed by 2011. However Avastin continues to be used vigorously all over the world. Available data suggests good efficacy and safety profile though it lacks the level of evidence of a randomized, controlled clinical trial. The sheer economics of it makes it available to almost any patient who requires it. Diabetic Retinopathy The publication that grabbed headlines this year was" A Randomised Trial Comparing intra-vitreal Triamcinolone Acetonide and Focal/Grid Photocoagulation for Diabetic Macular Edema - a trial conducted by the Diabetic Retinopathy Clinical Research Network" - DRCR. net. Their conclusions were that over a 2 year period, focal/grid photocoagulation is more effective and has fewer side effects than 1 mg or 4 mg doses of preservative free triamcinolone for most patients with DME who have characteristics similar to the cohort in this trial. They recommended that focal/grid photocoagulation should currently be the benchmark against which other treatments are compared in clinical trials for DME. www.dosonline.org Figure 6: Bionic Eye Important limitations of this study are that focal and diffuse edema have not been separated in terms of treatment requirement and metabolic status of the patient has also not been a criteria. The fact that the 4-mg intravitreal triamcinolone group had a greater positive treatment response on visual acuity and retinal thickening at 4 months, whereas the photocoagulation group had a greater positive response later, raises the possibility that combining focal/grid photocoagulation with intravitreal triamcinolone may produce greater benefit for DME than either focal/grid photocoagulation or intravitreal triamcinolone alone. A DRCR.net study currently is evaluating a combination of intravitreal triamcinolone and focal/grid photocoagulation, a combination of ranibizumab and photocoagulation, and ranibizumab alone in eyes with characteristics similar to those included in the current study (protocol available at www.drcr.net). Pushing the frontiers Gene Therapy: According to Paul A. Silving , MD, Phd, director of NEI, the eye is an especially good target for gene therapy. It is a "wonderful place to test therapy ideas", since the eye is a "Separate Compartment", we only need to administer a microscopic quantity of gene vector (instead of circulating it in the body) and the results are obviously measurable. Michael Redmond's pioneering work showed that the gene RPE65 was critical for metabolism of Vitamin A for proper photoreceptor functioning. A mutation in RPE 65 accounts for 10% of all Leber's Congenital Amaurosis ( LCA) patients. Recent Phase I Studies of gene therapy for young adults with a mutation in the RPE65 genes and a severe form of recessive retinitis pigmentosa have used adeno-virus mediated delivery to introduce a human RPE 65 gene construct to the RPE. Results from two trials for LCA have suggested moderate visual improvement in some adults as monitored by psychophysical tests and studies are in progress to see if greater visual improvement can be achieved safely in younger patients. A Canadian & American research group from Montreal Children's hospital hopes to begin a human gene therapy trial in Canada within 5 years for LCA, Stargardt macular dystrophy & retinitis pigmentosa. 71 Nano Medicine: This is a highly specific medical intervention at nano scale (1-100nm) for curing diseases or repairing damaged tissues. Applications in Retinal Diseases include: • Drugs & Delivery (Neurotech NT-150) • Retinal Tissue Engineering (RTE) Neurotech (Figure 5) NT- 501 intra-vitreal implant is a Ciliary Neurotrophic Factor (CNTF) secreting encapsulated cell device. CNTF is a cytokine, which is a survival factor for various neuronal cells and seems to prevent neurodegeneration. A semi-permeable membrane encapsulates genetically engineered human RPE cells that secrete CNTF. It prevents host antibodies and immune cells from entering the device but allows nutrients to diffuse in, to nourish the cells within and CNTF to diffuse out. The purpose is to support and rescue degenerating retinal tissue using neurotophic factors. This holds promise for retinitis pigmentosa and dry age related macular degeneration. Phase I trial is completed. Phase II & III patients have been recruited and results are expected by 2009/2010. RTE: Replacement of damaged retinal cells using retinal progenitor cells delivered on bioresorbable polymer scaffold and transplanted into sub-retinal space is a promising therapeutic strategy. Likewise, using tissue engineering strategies, sheets of retinal pigment epithelium (RPE) are grown on synthetic biodegradable polymers for subsequent transplantation. Optoelectric retinal prosthesis "Bionic Eye": Argus II, 60 electrode epiretinal prosthesis has been developed by researchers at. Doheny Eye Institute, Los Angeles. 16 subjects with advanced retinitis pigmentosa. have been implanted with this device at 8 centres. Orientation and mobility with device 'on' were better than with device 'off ' with door and line tests. Author Cyrus M. Shroff MD S.B. Eye Hospital Mainpuri (UP) – 205001 Applications are invited for the following posts:(a) Eye Surgeon:- MS/DNB/DOMS having experience in phaco/S.I.C.S./IOL. (b) O.T. Assistant:- Trained with experience Contact with full Bio-Data Immediately to:- Director Dr. J.K. Purang (Eye Surgeon) Mob.: 09811021192, 011-25446622, 25163444 Email: [email protected] 72 DOS Times - Vol. 14, No. 8, February 2009 J E Moore1,2, J E Graham1, E A Goodall1, D A Dartt3, A Leccisotti1,4, V E McGilligan1, T C B Moore1 British Journal of Ophthalmology 2009;93:66-72 1 3 Abstracts Concordance between Common Dry Eye Diagnostic Tests Centre for Molecular Biosciences, University of Ulster, Northern Ireland, UK, 2 Royal Group Hospitals, Belfast, Northern Ireland, UK Schepens Eye Research Institute, Boston, Massachusetts, USA, 4 Casa di Cura Rugani, Siena, Italy AIM Large variations in results of diagnostic tests for mild to moderate dry eye are widely recognised. The purpose of this study was to assess if there was concordance between common dry eye diagnostic tests. METHODS A total of 91 subjects were recruited to the study. The tear film and ocular surface were evaluated using the phenol red thread test (PRT), tear film break-up time (TBUT), biomicroscopic examination and impression cytological assessment of conjunctival goblet cells. Dry eye symptoms were assessed using McMonnies’ dry eye questionnaire (MQ) and statistical correlations between all tests were assessed. RESULTS This study cohort did not include severe aqueous deficient dry eye patients as determined by the PRT. A statistically significant difference was noted between PRT results and all other tests (p<0.001). Only Meibomian gland pathology, MQ, reduced goblet cell density and TBUT (<7s) demonstrated correlation determined by McNemar’s test. CONCLUSION A correlation was found only between tests assessing lipid/mucous deficiency (Meibomian gland evaluation, goblet cell density, TBUT and MQ). Prevalence of Dry Eye at High Altitude: A Case Controlled Comparative Study Gupta N, Prasad I, Himashree G, D'Souza P. High Alt Med Biol. 2008 Winter;9(4):327-34 Department of Ophthalmology, Lady Hardinge Medical College and Associated Smt. Sucheta Kriplani Hospital and Kalawati Saran Children Hospital, New Delhi, India. High altitude is associated with physiological as well as pathological changes in the eye related to adverse environmental conditions that result in increased tear evaporation and contribute to a higher incidence of dry eye in these regions. We aimed to study the difference in prevalence of dry eye at high altitude and at low altitude. The prevalence of dry eye among the natives and the army soldiers who were recently posted at high altitude was also studied and compared. 200 adults above 20 years of age were enrolled. 100 subjects were recruited at a high altitude region (study group), of which 50 were native Ladakhis and 50 were soldiers recently posted at Leh, Ladakh, India (height; 3300 m above sea level; temperature: 18 degrees C to 24 degrees C). 100 subjects, age and sex matched, were screened at a low altitude region, New Delhi, India (218 m above sea level; temperature: 19 degrees C to 24 degrees C) to serve as the control group. Prevalence of dry eye was assessed through standard questionnaires (McMonnies' Questionnaire (MMI), Ocular Surface Disease Index Questionnaire (OSDI), and Schirmer's basic secretion test. On the basis of the parameters studied (symptoms, MMI, OSDI and Schirmer's test), dry eye was diagnosed in 20% of subjects screened at high altitude and in 9% of subjects in the control group screened at low altitude. In the study group, the prevalence of dry eye was significantly higher amongst the native population (54%) than in the army soldiers who were recently posted at that region (26%). The difference was statistically significant (p < 0.005). In conclusion, dry eye is more common at high altitude, particularly in the native population. Awareness among people residing at high altitude and the treating medical personnel needs to be created for early detection and treatment of dry eye to prevent vision-threatening complications. www.dosonline.org 77 Plasmin enzyme-assisted vitrectomy for primary and reoperated eyes with stage 5 retinopathy of prematurity Wu WC, Drenser KA, Lai M, Capone A, Trese MT Retina. 2008 Mar;28(3 Suppl):S75-80 Antiangiogenic therapy with intravitreal bevacizumab for retinopathy of prematurity Retina. 2008 Mar;28(3 Suppl):S19-25 Quiroz-Mercado H, Martinez-Castellanos MA, HernandezRojas ML, Salazar-Teran N, Chan RV Associated Retinal Consultants, PC, Royal Oak, Michigan, USA. Retina Service, Asociacion Para Evitar La Ceguera en México PURPOSE (APEC), Mexico City, Mexico. [email protected] To review the surgical outcome of plasmin enzyme-assisted vitreoretinal surgery in managing stage 5 retinopathy of prematurity (ROP). PURPOSE METHODS To evaluate the role of antiangiogenic therapy with intravitreal bevacizumab for retinopathy of prematurity (ROP). A retrospective, consecutive interventional cases series of 80 eyes (68 patients) with stage 5 ROP, treated with autologous or maternal plasmin enzyme-assisted vitreoretinal surgery, were reviewed. All study patients underwent surgery between 1995 and 2004 with plasmin enzyme-assisted vitreoretinal surgery. Thirty-eight eyes had previous vitreous surgery without retinal breaks (Group 1). Fifteen eyes had previous vitreous surgery with retinal breaks (Group 2). Twenty eyes received previous laser, and/or cryotherapy, and/or scleral buckling, but no vitrectomy (Group 3). Seven eyes did not receive any treatment previously (Group 4). METHODS RESULTS RESULTS Postoperative anatomic outcome, functional results, and surgical complications in each group of eyes at an average follow-up of 49 months were recorded. Following surgery of all 80 eyes, anatomic success was achieved in 68.8%. Six eyes (7.5%) achieved 20/60 to 20/600 vision. Fifty-nine eyes (73.8%) achieved vision worse than 20/600 to light perception. Eleven eyes (13.8%) achieved no light perception. Visual results were uncertain in 4 eyes (5%) because of the inability to measure reliably. Thirteen patients (18 eyes; mean age +/- SD, 4 +/- 3 months; mean follow-up, 6 months) were included in the study. We found neovascular regression in 17 eyes. One patient with stage IVa ROP had spontaneous retinal reattachment after an intravitreal injection of bevacizumab. There were no serious ocular or systemic adverse events. CONCLUSION Plasmin enzyme-assisted vitrectomy in eyes with and without previous vitrectomy surgery can achieve visual improvement in stage 5 ROP. Early intervention with vitreous surgery for stage 4A ROP will achieve better anatomic and visual results and reduce the number of children with stage 5 ROP. In this noncomparative, prospective, interventional case series, bevacizumab was injected into the vitreous of patients with ROP in three different groups: group I, patients with stage IVa or IVb ROP who had no response to conventional treatment; group II, patients with threshold ROP who were difficult to treat with conventional therapy because of poor visualization of the retina; and group III, patients with high-risk prethreshold or threshold ROP. CONCLUSION The use of bevacizumab may be promising in the treatment of patients with ROP. Further studies need to be performed to determine the safety and long-term efficacy of intravitreal injection of bevacizumab, either as first-line therapy or after failure of conventional therapy. DOS Correspondent Noopur Gupta MS, DNB 78 DOS Times - Vol. 14, No. 8, February 2009 Forthcoming Events : National February 2009 25-26 CHENNAI July 2009 20-22 CHENNAI Neuro-Ophthalmology Update 2009 Sankara Nethralaya, 18, College Road, Chennai, Tamil Nadu Contact Person & Address S. Ambika, Conference Secretariat E-mail: [email protected], [email protected] March 2009 20-22 NEW DELHI Annual Conference of Delhi Ophthalmological Society Contact Person & Address Dr. Namrata Sharma Room No. 474, 4th Floor, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, Ansari Nagar, New Delhi – 110029 Ph.: 011-65705229, Fax: 26588919, E-mail: [email protected], Website: www.dosonline.org Indian Intraocular Implant & Refractive Surgery Convention Hotel Taj Coromandel, Chennai Tel : +91-44-2811 2811 Fax : +91-44-2811 5871 Email : [email protected] Website : http://www.iirsi.com October 2009 2-4 BHAVNAGAR, GUJARAT 37th Annual Gujarat Ophthalmological Conference Vision-2009 Contact Person & Address Dr. Nilesh Parekh 22, ‘VINAY’ Behind Central Salt, Opp. New Filter Tank, Bhavnagar-364002 Mobile : +09428408788, Fax : +91-0278-2566388 Email : [email protected] Website : http://www.iirsi.com Forthcoming Events : International February, 2009 6-8 ROME, ITALY May, 2009 3-7 FLORIDA 13th ESCRS Winter Refractive Surgery Meeting Rome, Italy Phone: +3535 1209 1100 / Fax: +353 1209 1112 Email: [email protected] Web Site: http://www.escrs.org March, 2009 17-22 CHICAGO Illinois Eye Review Chicago, Illinois, United States Contact Name: Cindy Phone: 312.996.6590 Fax: 312.996.7770 Email: [email protected] Web Site: http://www.IllinoisEyeReview.org April, 2009 4-8 SAN FRANCISCO, CA, USA ASCRS/ASOA Symposium and Congress Francisco, USA Phone: 701 591 2220 / Fax: 1703 591 0614 Web Site: http://www.ascrs.org 16-18 GENEVA, SWITZERLAND 4th International Congress on Glaucoma Surgery Organising Secretariat O.I.C. Srl - Organizzazione Internazionale Congressi Viale Matteotti, 7 - 50121 Firenze, Italy Phone: 39/055/50351, Fax: 39/055/5001912 E-mail: [email protected] www.dosonline.org Greater Fort Lauderdale/Broward County Convention Center 1950, Eisenhower Blvd., Fort Lauderdale, Florida - 33316 Phone: 1.240.221.2900, Email: [email protected] 16-19 INDONESIA Asia Pacific Academy of Ophthalmology (APAO) Indonesian Ophthalmologists Association (IOA) Department of Ophthalmology Faculty of Medicine, Universitas Indonesia, Jalan Salemba Raya No. 6, Jakarta - 10430, Indonesia Phone : (62-21) 3190 7282, Fax : (62-21) 392 7516 E-mail : [email protected] June, 2009 13-16 NETHERLANDS SOE 2009, 17th Congress of the European Society of Ophthalmology, Contact: Congrex Sweden AB Attn: SOE 2009, P.O. Box 5619 SE-114 86 Stockholm, Sweden Tel: +46 8 459 66 00, Fax: +46 8 661 91 25 E-mail: [email protected] September, 2009 12-16 BARCELONA, SPAIN XXVII Congress of the ESCRS Phone: +35312091100, Fax: 35312091112 Email: [email protected], Web Site: http://www.escrs.org 79 Delhi Ophthalmological Society (LIFE MEMBERSHIP FORM) Name (In Block Letters) __________________________________________________________________________ S/D/W/o _____________________________________________________________ Date of Birth _____________ Qualifications _________________________________________________________ Registration No. __________ Sub Speciality (if any) ___________________________________________________________________________ ADDRESS Clinic/Hospital/Practice ______________________________________________________________________ _______________________________________________________________ Phone __________________ Residence ________________________________________________________________________________ _______________________________________________________________ Phone __________________ Correspondence ___________________________________________________________________________ _______________________________________________________________ Phone __________________ Email ___________________________________________________________ Fax No. ________________ Proposed by Dr. ____________________________________ Membership No. _________ Signature __________________ Seconded by Dr. ____________________________________ Membership No. _________ Signature __________________ [Must submit a photocopy of the MBBS/MD/DO & State Medical Council / MCI Certificate for our records.] I agree to become a life member of the Delhi Ophthalmological Society and shall abide by the Rules and Regulations of the Society. (Please Note : Life membership fee Rs. 3100/- payable by DD for outstation members. Local Cheques acceptable, payable to Delhi Ophthalmological Society) Please find enclosed Rs.___________in words ____________________________________________________ by Cash Cheque/DD No.____________________ Dated_____________ Drawn on______________________________________ Three specimen signatures for I.D. Card. Signature of Applicant with Date FOR OFFICIAL USE ONLY Dr._______________________________________________________________has been admitted as Life Member of the Delhi Ophthalmological Society by the General Body in their meeting held on________________________________ His/her membership No. is _______________. Fee received by Cash/Cheque/DD No._______________ dated_________ drawn on __________________________________________________________________. (Secretary DOS) www.dosonline.org 81 INSTRUCTIONS 82 1. The Society reserve all rights to accepts or reject the application. 2. No reasons shall be given for any application rejected by the Society. 3. No application for membership will be accepted unless it is complete in all respects and accompanied by a Demand Draft of Rs. 3100/- in favour of “Delhi Ophthalmological Society” payable at New Delhi. 4. Every new member is entitled to receive Society’s Bulletin (DOS Times) and Annual proceedings of the Society free. 5. Every new member will initially be admitted provisionally and shall be deemed to have become a full member only after formal ratification by the General Body and issue of Ratification order by the Society. Only then he or she will be eligible to vote, or apply for any Fellowship/Award, propose or contest for any election of the Society. 6. Application for the membership along with the Bank Draft for the membership fee should be addressed to Dr. Namrata Sharma, Secretary, Delhi Ophthalmological Society, R.No. 474, 4th Floor, Dr. R.P. Centre for Ophthalmic Sciences, AIIMS, Ansari Nagar, New Delhi - 110 029. 7. Licence Size Coloured Photograph is to be pasted on the form in the space provided and two Stamp/ Licence Size Coloured photographs are required to be sent along with this form for issue of Laminated Photo Identity Card (to be issued only after the Membership ratification). 8. Applications for ‘Delhi Life Member’ should either reside or practice in Delhi. The proof of residence may be in the form Passport/ Licence/Voters Identity Card/Ration Card/Electyricity Bill/MTNL (Landline) Telephone Bill. DOS Times - Vol. 14, No. 8, February 2009 Anagram Time Columns DOS Quiz Each of the following words is a jumbled ophthalmic or related term. There is, however, an extra letter in every set of letters. These extra letters will also form a eight letter ophthalmic word when unjumbled. So get cracking. 1. UNAMED ___ ___ ___ ___ ___ ____ 2. NOWROB ___ ___ ___ ___ ___ ____ 3. SHORREN ___ ___ ___ ___ ___ ___ ____ 4. RETURNY ___ ___ ___ ___ ___ ___ ____ 5. RETERNI ___ ___ ___ ___ ___ ___ ____ 6. BIRSUMO ___ ___ ___ ___ ___ ___ ____ 7. CURENETION ___ ___ ___ ___ ___ ___ ___ ___ ___ ____ 8. BARWUDGARDEN ___ ___ ___ ___ ___ ___ ___ ___ ___ ___ ___ ____ Answers on page number 43 Missed DOS Times Copy If you have missed your copy of DOS Times. Please Contact: Secretary DOS : Dr. Namrata Sharma Room No. 474, 4th Floor, Dr. Rajendra Prasad Centre for Ophthalmic Sciences , All India Institute of Medical Sciences, Ansari Nagar, New Delhi – 110029 Ph.: 91-11-65705229, Fax: 91-11-26588919, E-mail: [email protected], Website: www.dosonline.org Saurabh Sawhney DO, DNB Ashima Aggarwal MS, DNB Insight Eye Clinic, New Delhi Sharma Eye Hospital Arya Chowk, Ambala City Requires 1. ONE Oculoplasty (Part Time) 2. ONE Squint Surgeon (Part Time) 3. ONE Ophthalmic Surgeon proficient in SICS/ Phaco surgeries (Fulltime) 4. TWO Optometrists (preferably from AIIMS) Raj Eye Hospital Naseerabad, Gorakhpur Requires ONE Fulltime Retina Surgeon with preferably fellowship experience Contact: Dr. J.S. Guja Sai Retina Foundation Ph.: 2673688, 26279867, 9350102339 www.dosonline.org 83 Determinants of Target IOP Glaucoma Suspect at Moderate Risk for Visual Loss • IOP level at which optic nerve damage occurred • • Extent and rate of progression of glaucomatous damage, if known Fellow of eye with established GON (excluding secondary unilateral glaucoma). OHT with multiple risk factors (thin CCT, high lOP, suspicious disc) • GLC gene mutations associated with severe glaucoma • Presence of other risk factors • Recurrent optic disc hemorrhages • Patient’s age • PXF syndrome • Expected life span • Younger age • Medical history Emperical Formula for Target IOP Glaucoma Suspect or Other Condition with Low Risk for Glaucomatous Visual Loss Target IOP = “Maximum IOP – Maximum IOP% - Z” More important • Z is an optic nerve damage severity factor. • OHT(Ocular hypertension) • Z Optic Nerve damage • Older age • 0→Normal disc and Normal visual field • PACS (anatomically narrow angle with no PAC signs or raised lOP) • 1→Abnormal Disc and Normal visual field • Pigment dispersion syndrome with normal lOP • 2→Visual field loss not threatening fixation • Glaucoma suspect disc, including disc asymmetry • 3→Visual field loss threatening or involving fixation • Family history of glaucoma Risk Categories Less important Glaucoma with High 5-year Risk for Progressive Visual Loss or High 5-year Risk for Visual Disability • Steroid responder, steroid user • Myopia Moderate to advanced Glaucomatous optic neuropathy (GON) with correlating visual field(VF) loss and • Zone β peripapillary atrophy • Demonstrated progression over a short time • Diabetes mellitus • Higher lOPs • Uveitis • Bilateral VF loss • Systemic hypertension • Pigmentary and (Pseudoexfoliative)PXF glaucoma • Very advanced VF loss, fixation threat, or glaucoma-related visual disability • Young age with advanced disease • Secondary glaucoma • ACG (angle closure glaucoma) Glaucoma with Moderate 5-year Risk for Visual Loss or Glaucoma Suspect with High Risk for Visual Loss • Mild GON with correlating early VF loss and higher lOP • Mild-to-moderate glaucoma with low IOP2 • PAC with high lOP and PAS • Younger age www.dosonline.org Tearsheet Target IOP American Academy of Ophthalmology Guidelines • Glaucoma patients with mild damage (optic disc cupping but no visual field loss) → Reduction of 20-30% from baseline • Glaucoma patients with advance damage → Reduction of 40% or more from baseline • Normal pressure glaucoma → Reduction of 30% from baseline • Ocular hypertension → Reduction of 20% from baseline • Open angle glaucoma with IOP in the mid to high 20s → Target IOP range 14-18 mmHg 85 • Advanced Glaucoma → Target IOP < 15 mmHg Glaucoma Suspect at Moderate Risk for Visual Loss: • OHT whose IOP > 30 mmHg with no sign of optic nerve damage → Target IOP < 20 mmHg • Monitor closely for change or treat depending on risk and patient preferences Asia Pacific Glaucoma Guidelines • Glaucoma with High Risk for Progressive Visual Loss or Visual Disability: Pressure reduction of 40% or 1 to 2 SD below the population mean (9 to 12 mmHg), if achievable safely. Treat if risk(s) increase(s) with pressure reduction of 20% or 1 SD above the population mean, whichever is lower • Glaucoma with Moderate Risk for Visual Loss or Glaucoma Suspect with High Risk for Visual Loss: Pressure reduction of >30% or population mean, whichever is lower. The fellow eye of unilateral glaucoma may require the same target as the affected eye depending on risk and state. Glaucoma Suspect with Low Risk for VisualLoss Monitor, no treatment. Shibal Bhartiya MS, Shubha Bansal DNB Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi-110029 86 DOS Times - Vol. 14, No. 8, February 2009