Delhi Journal of Delhi Journal of Ophthalmology

Editor Rohit Saxena Managing Editor Rajesh Sinha

Editorial Committee Editorial Board Parijat Chandra Jitendra Jithani Rajvardhan Azad Vimla Menon Rasik B Vajpayee Tushar Agarwal M.Vanathi Atul Kumar Pradeep Sharma Rajinder Khanna Shibal Bhartiya Prakash Chand Agarwal Ashok K Grover V P Gupta Harbans Lal Munish Dhawan Swati Phuljhele Mahipal S Sachdev S. Bharti Amit Khosla B Ghosh Harinder Sethi Reena Sharma Lalit Verma Ashok Garg Kirti Singh Raghav Gupta Varun Gogia Sharad Lakhotia P K Pandey B P Guliani Ashish Kakkar Sashwat Ray P V Chaddha Ramanjit Sihota S P Garg Rachana Meel Saptorshi Majumdar Dinesh Talwar Divender Sood Arun Baweja Digvijay Singh Shraddha Puranik K P S Malik Rishi Mohan Sanjay Mishra Tanuj Dada Namrata Sharma Tarun Sharma General Information Delhi Journal of Ophthalmology (DJO), once called Visiscan, is a quarterly journal brought out by the Delhi Ophthalmological Society. The journal aims at providing a platform to its readers for free exchange of ideas and information in accordance with the rules laid out for such publication. The DJO aims to become an easily readable referenced journal which will provide the specialists with up to date data and the residents with articles providing expert opinions supported with references.

Contribution Methodology Author/Authors must have made significant contribution in carrying out the work and it should be original. It shouldbe accompanied by a letter of transmittal.The article can be sent by email to the Editor or a hard copy posted. Articles receive will be sent to reviewers whose comment will be emailed to the author(s) within 4-6 weeks. The identity of the authors and the reviewers will not be revealed to each other by the editorial team. Detailed instructions to the contributors and for advertisement are included at the end of the journal.

Editorial Process The DJO has Dr Rohit Saxena as its Editor who is assisted by a team of renowned ophthalmologists and an illustrous editorial board. The reviewers,who are leaders in their respective fields, form the back bone of the journal by setting standards for the published work.

Disclaimer The journal does not take any responsibility for the articles published in the journal unless it is explicitly stated so. The views expressed in the articles and editorials are of the authors and do not in any way reflect the policy of the Delhi Journal of Ophthalmology. The journal does not endorse or guarantee the quality or efficacy of any product or service mentioned or advertised in the journal issues. Advertisements carried in this journal are expected to conform to internationally accepted medical, ethical and business standards.

Editorial Office Dr Rohit Saxena, Room No. 479, Dr R.P. Centre for Ophthalmic Sciences, AIIMS, New Delhi-110029 Ph +91-011-26593182, Email : [email protected]

Published by Dr Rohit Saxena, Editor DJO, on behalf of Delhi Ophthalmological Society, Delhi

Editorial Assistant Varun Kumar

Vol. 21, No. 3, January-March, 2011 DJO 1 Delhi Journal of Ophthalmology

Contents

Editorial 3. Where the mind is without fear Dr. Rohit Saxena

Major Review 4. Shivani Pahuja, Ritesh Narula 9. The Operation Theatre : Basic Architecture Sapna, Saptorshi Majumdar, Pradeep Venkatesh 15. Upshoot And Downshoot In Duane’s Retraction Syndrome Suma Ganesh 19. Marcus Gunn Jaw-Winking Phenomenon : A Review Dewang Angmo, Mandeep S. Bajaj, Neelam Pushker, Supriyo Ghose 23. Review of doses of important drugs in ophthalmology Yogesh Bhadange, Bhavin Shah , Brijesh Takkar, Rajesh Sinha

Preferred Practice Pattern 28. Systematic Approach to a Case of Disc Pallor Digvijay Singh, Rohit Saxena, Pradeep Sharma, Vimla Menon

Case Reports 33. Bilateral Mooren’s Ulcer with perforation and prolapse Uday Gajiwala, Jyotsom Ganatra, Rajesh Patel, Parin Shah, Rohan Chariwala 38. Acute Onset and Angle Closure after Topiramate Administration Nidhi Verma, Ashok Kumar

Instrument Scan 40. Understanding your Direct Ophthalmoscope Digvijay Singh, Rohit Saxena, Pradeep Sharma, Vimla Menon

Original Article 45. Study of Colour Blindness in Tibetan Population Navjot Kaur, Avinash Kumar, Gurinder Kaur, Jasjeet Kaur Dhillon, K.D.Singh 48. Manual Sutureless Small Incision Technique for Exchange of dislocated Posterior Chamber IOL Lakshminarayana Pasumala

Instructions to Authors

DJO Vol. 21, No. 3, January-March, 2011 2 Delhi Journal of Ophthalmology

Editorial

Where the mind is without fear The relevance of Rabindranath Tagore’s poem for today’s ophthalmologist.

WHERE the mind is without fear and the head is held high (in OPD’s, rounds, OT’s) Where knowledge is free (conferences, symposiums, internet) Where the world has not been broken up into fragments

By narrow domestic walls (zones, institutions vs individual, govt. v/s pvt.) Where words come out from the depth of truth (in free paper sessions, articles) Where tireless striving stretches its arms towards perfection (in our work) Where the clear stream of reason has not lost its way (Duke Elder, Kanski…)

Into the dreary desert sand of dead habit (routine of OPD-rounds-OT) Where the mind is led forward by thee (God who gave us the opportunity to give sight) Into ever-widening thought and action (think and reach beyond routine clinical work) Into that heaven of freedom, my Father, let my country (us ophthalmologist) awake.

Dear friends, The relevance of the above poem is timeless and for everyone whichever occupation we may be pursuing. It is just a reminder that we must get out of our routine and think towards becoming better and more accomplished in our work and our duty of serving the patients for which we are here. Let us resolve to give our best towards the care of our patients…. The result of course is always out of our hands. This is the penultimate issue of the Delhi Journal of Ophthalmology with yours truly as the Editor and I must accept that despite the challenges of bringing out the journal regularly with a content that is somewhat accurate, interesting and scientifically sound, my period as editor has been most enjoyable and fulfilling.

Dr. Rohit Saxena

The Delhi journal of Ophthalmology is now indexed at Index Copernicus. The editorial board is involved in the task of getting the journal indexed in other sites as well as improving the quality of articles and their presentation. This is only possible with the support of each and every DOS member.

In addition to the present heads, the DJO also publishes original research including thesis work of residents. We also welcome to article comments and advise on how to improve the DJO. Any DOS member who has not received the previous four issues please contact DOS Secretariat [email protected], [email protected] or Editor, DJO editordjo@gmail. com. Some copies have come back due to incorrect addresses, so members are requested to please provide correct addresses and contact details to DOS Secretariat.

Vol. 21, No. 3, January-March, 2011 DJO 3 Delhi Journal of Ophthalmology

Major Review Endophthalmitis

Shivani Pahuja1, Ritesh Narula2 1 Guru Nanak Eye Centre, 2 Shroff Charity Eye Hospital.

Endophthalmitis is an inflammation of the internal layers of material, infectious or as a complication of suture the eye resulting from intraocular colonization of infectious removal. These patients are also at risk of infections due to agents and manifesting with an exudation into vitreous cavity. prolonged corticosteroid use following surgery. Though the word endophthalmitis means any inflammation of the internal ocular spaces but in clinical practice it is usually Endophthalmitis as a complication of vitreoretinal surgery is taken to mean inflammation secondary to intraocular infection. relatively uncommon. Scleral buckle infection usually appears Endophthalmitis can be divided into two main types based between the second and seventh day of surgery, and, presents as on the mode of infection as: Exogenous and Endogenous severe lid oedema with conjunctival chemosis associated with (ie, metastatic). Exogenous endophthalmitis results from severe ocular pain and headache. Intraocular inflammation direct inoculation as a complication of ocular surgery, foreign may develop associated with media haziness, subretinal bodies, blunt or penetrating ocular trauma. Endogenous exudation and localised exudative . In endophthalmitis [1] results from the hematogenous spread of delayed subacute cases fistula and granuloma form, resulting organisms from a distant source of infection (eg, endocarditis). in exposure of the buckle. Diabetes and prolonged duration Endogenous endophthalmitis is quite rare. Various studies have of surgery have been identified as specific risk factors in this reported incidence varying from 2% to 15%. In endogenous setting. Incidence of endophthalmitis following pars plana endophthalmitis, blood-borne organisms permeate the blood- vitrectomy has been reported as low as 0.051%. ocular barrier either by direct invasion (septic emboli) or by changes in vascular endothelium caused by substrates released The risk factors are different for isolated and cluster during infection. Destruction of intraocular tissues may be due endophthalmitis. Patient factors play a predominant role in to direct invasion by the organism and/or from inflammatory isolated postoperative endophthalmitis. Patients own bacterial mediators of the immune response. Due to increase in the flora may gain entry at the time of surgery and thus increasing spread of AIDS, more frequent use of immunosuppressive the development of postoperative endophthalmitis. It is agents, and the use of more invasive procedures, the patients very important to rule out diseases like chronic , at risk of endogenous endophthalmitis are increasing . , canaliculitis and keratoconjuntivitis sicca, chronic before planning ocular surgery for the Most cases of exogenous endophthalmitis (49% to 76% as patient. The other important intraoperative risk factors for reported by various studies) occur after intraocular surgery. isolated postoperative endophthalmitis are inadequate When surgery is implicated in the cause, endophthalmitis or conjunctival disinfection, prolonged surgery, vitreous usually begins within 1 week after surgery. Post loss, prolene haptics of IOLs. The above factors can be taken endophthalmitis [2] is the most common form, care of by the use of pre-operative topical antibiotics for 24 with approximately 0.1-0.3% of operations having this hours, facial scrub, povidone iodine into the conjunctival sac, complication. This is largely due to large number of cataract adhesive plastic drapes to separate , surgeon gloves, surgeries being done. But the exact incidence varies in subconjunctival antibiotics at the end of the surgery. The role different set ups and may be very low at certain places. The of antibiotics in the irrigating solutions is still questionable. overall incidence of endophthalmitis has decreased over One drop of 5% povidone iodine [4] has proved to be beneficial the past several decades largely due to improved surgical in protecting against endophthalmitis. technique and patient preparation. Modern estimates indicate that the incidence of infectious endophthalmitis ranges from External factors are the major risk factors in the causation 0.13% to 0.7%. of cluster postoperative endophthalmitis. There have been various reports from all over the world describing bacterial as Endophthalmitis following penetrating keratoplasty [3] may well as fungal postoperative cluster endophthalmitis. Defects occur early or late, the incidence of which is reported upto in sterlisation of instruments, contamination of tap water, 2%. The infection could be due to contamination of donor multiple dose fluids and drugs have been held responsible

DJO Vol. 21, No. 3, January-March, 2011 4 Endophthalmitis Delhi Journal of Ophthalmology for bacterial cluster postoperative endophthalmitis. Fungal Post surgical endophthalmitis can be again divided into 2 cluster postoperative endophthalmitis has been reported categories – Early onset post operative endophthalmitis and after contaminated irrigating solutions, IOLs, viscoelastics, late onset post operative endophthalmitis depending on the improper ventilation system, poor OT hygiene and even after time after the surgery at which the patient presents. hospital construction activity. To prevent the occurrence of these cluster infections, we have to remain on guard for any Majority of patients of post cataract endophthalmitis usually breach in infection control measures and use standardized present in the first post op week. Severe acute postoperative irrigating solutions and drugs. endophthalmitis usually presents within 1-4 days after surgery. Patients usually present with decreased visual acuity and pain. Infectious postoperative endophthalmitis needs to be carefully Ciliary injection is usually present. corneal oedema ,anterior differentiated from non infectious vitreous inflammation chamber reaction and vitreous inflammation are important following surgery. This form of sterile endophthalmitis is also clinical features indicating acute endophthalmitis. Afferent known as the toxic anterior segment syndrome (TASS).There pupillary defect may be present. The organisms implicated are multiple potential causes of TASS including toxic effects are Staphylococcus aureus, Streptococcus and gram- from intraocular fluids, medications, lenses, instruments, negative organisms such as Serratia marcescens, Proteus and endotoxins, and sterilization techniques. The differentiation Pseudomonas. from infectious endophthalmitis is vital , as the treatments and positive outcomes are different. Often, the presenting signs and Chronic or late endophthalmitis usually presents after about symptoms of infectious endophthalmitis occur within the first 6 weeks of . It usually presents with minimal 48 to 72 hours after surgery. If endophthalmitis is caused by pain, , granular keratic precipitates and mild vitritis. fungal or less virulent organisms, the onset of symptoms can Staph. Epidermidis[5], candida and Propionobacterium acnes occur as late as weeks to months after surgery. TASS usually are usually responsible for chronic endophthalmitis. presents sooner than infectious endophthalmitis (12 to 24 hours The most important clinical feature of P acnes endophthalmitis after surgery) and differs from infectious endophthalmitis [6] is the presence of a white intracapsular plaque composed in presentation. Patients often have decreased visual acuity, of sequestered micro-organisms in the capsular bag. It is a low corneal edema, a nonreactive, dilated , and a moderate to grade chronic smouldering type of endophthalmitis. Because severe anterior chamber reaction with cells, flare, hypopyon, of its atypical presentation it is often difficult to diagnose and and especially fibrin. Pain is mild to moderate if present. may be confused with posterior capular opacification. Patients with TASS can have marked corneal edema and even Other important type of endophthalmitis which deserve permanent, irreversible corneal decompensation. The corneal special mention include Post traumatic endophthalmitis [7]. It edema is characteristically “limbus to limbus.” Inflammation occurs in 4-13% of all penetrating ocular injuries. Incidence secondary to TASS will respond readily to topical steroid of endophthalmitis with perforating injuries in rural settings treatment as apposed to patients of endophthalmitis who will is higher when compared with non rural settings. Delay in show worsening. the repair of a penetrating injury is correlated with increased risk of developing endophthalmitis. Incidence of Clinical Features of Endophthalmitis endophthalmitis with retained intraocular foreign bodies The typical clinical characteristics are increasing pain and is 7-31%. Common agents are Staph epidermidis, Bacillus redness associated with decreasing visual acuity or sometimes species, streptococcus species, Staph. aureus, various fungi profound loss of vision. Hypopyon is usually present but may and P. acnes[8]. be absent. lid oedema and raised intraocular pressure may be present. Occasionally the signs and symptoms may be subtle Endophthalmitis associated with filtering blebs is also and misleading in the early stages until the course becomes sometimes seen in patients. Bacteria enter the eye through more fulminant and Intraocular inflammation greater than intact or leaking conjunctival filtering blebs. Streptococcus, expected in the postsurgical period should always be viewed Pneumococcus and Haemophilus influenzae are some of the with suspicion. Blurring of vision is the most common organisms. symptom and was reported by 94% of patients in the EVS study. Pain was characteristically absent in 25% cases in the HOW TO EXAMINE A CASE OF ENDOPHTHALMITIS ? EVS study group. Other clinical signs which may be present are The examination of the suspected patient should include a lid edema, conjunctival hyperemia, chemosis, circumcorneal detailed history, visual acuity assessment, examination of congestion, ring abscess, suture abscess, wound dehiscence, ocular adnexa (any sign of lid swelling), anterior chamber fibrinous AC reaction, posterior synchiae, vitreous cells, evaluation (using a slit lamp) and examination of the vitreous. vitreous exudates, retinal exudates and periphelbitis. Associated signs like leaking filtering blebs, wound leak etc.

Vol. 21, No. 3, January-March, 2011 DJO 5 Delhi Journal of Ophthalmology Endophthalmitis should be noted if any. Fundus view may be obscured because an immediate vitreal tap and instillation of intravitreal of anterior chamber reaction or due to vitreous exudates. antibiotics. Vitreous sample is immediately subjected to gram The clarity of ocular media could be assessed by indirect stain[11], KOH and calcoflour smear. The sample is also ophthalmoscopy. innoculated on blood agar, chocolate agar, liquid agar and Sabauraud’s dextrose agar. A eubacterial PCR and fungal PCR Management should be done if the facilities are available. The prognosis in postoperative endophthalmitis depends on the virulence of the microorganisms and early intervention. A combination of Intravitreal antibiotics[12] which is For early recognition of postoperative infection, frequent commonly used include Vancomycin (1 mg/0.1ml) and postoperative follow up at 24 hours, 72 hour and 7 days Ceftazidime (2.25mg/0.1ml) because 94% of the culture is necessary. Every follow up examination comprises of positive cases are caused by gram positive organisms which recording visual acuity, slit lamp biomicroscopic examination are 100% sensitive to Vancomycin and 6% of culture positive and looking for media clarity. After the clinical diagnosis of cases are by gram negative group of organisms which are 90% endophthalmitis is made, the further management depends on sensitive to Ceftazidime. the presenting visual acuity and the microbiological spectrum.. Data from the Endophthalmitis Vitrectomy Study indicate EVS did not recommend systemic antibiotics for cases of that initial management for patients who meet EVS entry acute bacterial postoperative endophthalmitis. However, criteria should include 3 port pars plana vitrectomy if patients the drug used in EVS were the once with poorer intravitreal present with vision worse than hand motions, but that an penetration. The current generation of fluoroquinolones which initial vitreous tap/biopsy with intravitreal antibiotics should have a high intravitreal penetration and achieve required MICs generally be sufficient if presenting vision is hand motions or for most organisms should be used as an adjunct. better. Systemic antibiotics were not found of benefit in this study. The indications for pars plana vitrectomy are • Poor visual acuity at presentation Limitations of Endophthalmitis Vitrectomy Study • deterioration or no improvement despite intravitreal • It only includes cases of post operative endophthalmitis antibiotics 70% of which were due to Staph. Epidermidis. The results • delayed onset endophthalmitis cannot be extrapolated to other forms of endophthalmitis • fungal endophthalmitis. such as bleb related, traumatic and endogenous which The precautions while performing pars plana vitrectomy in are more likely to be caused by organisms of greater endophthalmitis are virulence. • use of 6mm infusion cannula • Amikacin and Ceftazidime were the only systemic • clearing AC hypopyon and exudative membrane antibiotics evaluated in the EVS. Although patients in • collection of undiluted vitreous sample from midvitreous the EVS derived no demonstrable benefit from these cavity systemic antibiotics, the study made no recommendations • aim to clear only core vitreous regarding treatment with additional antimicrobial agents • IOL explantation in cases of gross infection (eg, systemic fluoroquinolones) or systemic antimicrobial Treatment approaches for P acnes endophthalmitis [14] agents for other types of endophthalmitis (eg, chronic, include bleb-associated, traumatic, fungal, and endogenous • Intra-vitreal vancomycin 1mg / 0.1 ml which has to be forms) given into the capsular bag • Potential study subjects with significant opacification of • May have to be combined with vitrectomy, total the anterior chamber or without light perception were capsulectomy and IOL explantation. excluded from the EVS. Because these eyes with more severe infection or involving more virulent organisms Fungal Endophthalmitis were excluded from the EVS, the effect might have • Endogenous fungal endophthalmitis is frequently an shifted the EVS outcomes to more favorable results. ocular manifestation of a systemic disease. • Endogenous infections usually occur in patients risk Though EVS did provide general guidelines for treatment of factors like immunosuppression, intravenous drug abuse, endophthalmitis, clinicians should individualise treatrment for bacterial sepsis, prolonged hyperalimentation, systemic each patient based on the clinical course, signs and symptoms. antibiotics, corticosteroid therapy, recent abdominal Once a clinical diagnosis of acute bacterial endophthalmitis surgery, malignancy, alcoholism, diabetes mellitus, made, it is to be considered as a medical emergency requiring trauma, and hemodialysis.

DJO Vol. 21, No. 3, January-March, 2011 6 Endophthalmitis Delhi Journal of Ophthalmology • Candida albicans is by far the most common pathogen and provides intravitreal access to antifungal agents (eg, isolated in endogenous fungal endophthalmitis. amphotericin B). Other pathogens include Aspergillus, Coccidioides, • Vitrectomy and intravitreal amphotericin B[19] should Cryptococcus, Blastomyces, and Sporothrix species. be considered in those cases of endogenous fungal • The diagnosis of endogenous fungal endophthalmitis endophthalmitis where the disease is progressing despite should be considered in patients who present with vitritis initial therapy with an appropriate systemic antifungal accompanied by a chorioretinal focus in the clinical agent. setting of a recent or current debilitating illness. Clinical • Voriconazole may be a particularly attractive agent suspicion plays an important role in identifying patients to consider for infections with fluconazole-resistant, who may have fungal endophthalmitis voriconazole-susceptible strains. • Exogenous infections usually are secondary to trauma • Choroidal neovascularization at the site of chorioretinal or surgery. A variety of fungi, including Paecilomyces, scars, epiretinal membranes, tractional retinal detachment Acremonium, and Sporothrix species, have been are some of the complications of fungal endophthalmitis. associated with endophthalmitis following intraocular surgery or trauma References • The prognosis of fungal endophthalmitis depends on 1. Albert DM, ed; Jakobiec FA. Endogenous endophthalmitis. In: the virulence of the organism, the extent of intraocular Principles and Practice of Ophthalmology. Vol 5. W B Saunders involvement, and the timing and mode of interventions. Co; 1994:3120-3125. • Blood cultures, urine cultures, sputum cultures, and 2. Albert DM, Jakobiec FA. Postoperative endophthalmitis. In: cerebrospinal fluid (CSF) cultures should be obtained Principles and Practice of Ophthalmology. W B Saunders Co; in patients suspected of endogenous endophthalmitis. 2000:2441-2462. In addition, direct examination of fungi with Giemsa, 3. Guss RB, Koenig S, De La Pena W, et al. Endophthalmitis after Gomori-methenamine-silver (GMS), and periodic-acid penetrating keratoplasty. Am J Ophthalmol 95:651-658,1983. Schiff (PAS) stains should be obtained. 4. Speaker MJ, Menikoff JA. Prophylaxis of endophthalmitis with • Culture of the fungus confirms the diagnosis. Fungal topical providone-iodine. Ophthalmology 98:1769-1775, 1991 cultures can be positive in 44-70% of patients diagnosed 5. Jett BD, Shepard B, Gilmore MS. Detection of Staphylococcus clinically. Vitrectomy samples are more sensitive for epidermidis and Staphylococcus aureus in vitreous by fungal cultures than vitreous needle biopsies. The culture polymerase chain reaction. Invest Ophthalmol Vis Sci 36:s788, must be kept at the laboratory for at least 4-6 weeks to 1995. ensure that slow-growing or fastidious fungal organisms 6. Lai YJ, Chen KH, Lin YC, Hsu WM, Lee SM. Propionibacterium are not missed. acnes DNA from an explanted intraocular detected by • A useful, recently introduced diagnostic tool for fungal polymerase chain reaction in a case of chronic pseudophakic endophthalmitis is the polymerase chain reaction (PCR) endophthalmitis. J Cataract Refract Surg 2006 ;32(3) : 522-525 [15]. The main advantages of PCR over conventional 7. Alfaro DV, Roth D, Liggett PE. Posttraumatic endophthalmitis. fungal cultures are the higher sensitivity and the rapid Causative organisms, treatment, and prevention. . results obtained with PCR. It helps to make an early 1994;14(3):206-11. differentiation between bacterial endophthalmitis and 8. EI-Asrar AM, Al-Obeidan SA, Yeboah EA. Late onset fungal endophthalmitis posttraumatic Propionibacterium acnes endophthalmitis. Eur J • Systemic amphotericin has been the treatment of choice Ophthalmol. 2004 ; 14(5) : 442-444. because of its broad-spectrum coverage; however, the 9. Johnson MW, Doft BH, Kelsey SF, Barza M, Wilson LA, Barr penetration of the vitreous cavity is poor. Doses of 5- to CC, Wisniewski SR: The Endophthalmitis Vitrectomy Study: 10-mg intravitreal amphotericin [16] have been used. Relationship between clinical presentation and microbiologic • A new systemic treatment is voriconazole [17], when spectrum. Ophthalmology 104: 261-272, 1997 administered orally or intravenously, it has good 10. Endophthalmitis Vitrectomy Study Group: The Endophthalmitis intravitreal concentrations. Intravitreal administration of Vitrectomy Study: Microbiologic factors and visual outcome. voriconazole [18] seems safe without evidence of retinal Am J Ophthalmol 22: 830-846, 1996. toxicity with concentrations up to 25 mg/mL. 11. Hahn Tw, Osterhout GJ, O’Brien TP, et al. Utility of a rapid • The advent of pars plana vitrectomy has improved the fluorescent Gram stain for recognition of live bacteria in ocular treatment results of fungal endophthalmitis. infection. Invest Ophthalmol Vis Sci 36:s797, 1995. • The advantages of pars plana vitrectomy are that it 12. Doft BH, Bazara M. Ceftazidime or amikacin:Choice of provides material for culture, removes viable organisms intravitreal antimicrobials in the treatment of postoperative and inflammatory end products from the infected vitreous, endophthalmitis. Arch Ophthalmol 112:17-18, 1994.

Vol. 21, No. 3, January-March, 2011 DJO 7 Delhi Journal of Ophthalmology Endophthalmitis 13. A.R. Watts1, J.I. Prydal1, K. Tambe1 and D.R. Jenkins. Linezolid, a New Antibiotic for the Treatment of Post-operative Endophthalmitis. Invest Ophthalmol Vis Sci 2003;44: 1451. 14. Aldave AJ, Stein JD, Deramo VA, Shah GK, Fischer DH, Maguire JI. Treatment strategies for postoperative Propionibacterium acnes endophthalmitis. Ophthalmology 1999 ; 106(12) : 2395- 2401. 15. Anand A, Madhavan H, Neelam V, Lily T. Use of polymerase chain reaction in the diagnosis of fungal endophthalmitis. Ophthalmology. Feb 2001;108(2):326-30 16. Blumenkranz MS, Stevens DA. Therapy of endogenous fungal endophthalmitis: miconazole or amphotericin B for coccidioidal and candidal infection. Arch Ophthalmol. Jul 1980;98(7):1216- 20. 17. Breit SM, Hariprasad SM, Mieler WF et al. Management of endogenous fungal endophthalmitis with voriconazole and caspofungin. Am J Ophthalmol. Jan 2005;139(1):135-40. 18. Gao H, Pennesi ME, Shah K, Qiao X, Hariprasad SM, Mieler WF, et al. Intravitreal voriconazole: an electroretinographic and histopathologic study. Arch Ophthalmol. Nov 2004;122(11):1687-92. 19. Martinez-Vazquez C, Fernandez-Ulloa J, Bordón J, Sopena B, de la Fuente J, Ocampo A, et al. Candida albicans endophthalmitis in brown heroin addicts: response to early vitrectomy preceded and followed by antifungal therapy. Clin Infect Dis. Nov 1998;27(5):1130-3.

DJO Vol. 21, No. 3, January-March, 2011 8 Delhi Journal of Ophthalmology

Major Review The Operation Theatre : Basic Architecture Sapna, Saptorshi Majumdar, Pradeep Venkatesh Dr. Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, New Delhi

Introduction - Stores room Cleanliness of the hospital environment is the best starting - Nursing staff room point to achieve the highest patient safety mandate. There is - Anaesthetist room a need to decrease the bio-burden present in the environment - Recovery room in an operating room. A systematic method of cleaning will decrease the possibility of the transmission of pathogens. C. Aseptic Zone Florence Nightingale, “The Lady with the Lamp,” and Joseph - Scrub area Lister (1827–1912), a professor at London’s King College - Preparation room Hospital were one of the first persons to realize the importance - Operation theatre of sterilization. Joseph Lister successfully introduced carbolic - Area used for instrument packing and sterilization. acid (phenol) to sterilize surgical instruments and to clean wounds. D. Disposal Zone Area where used equipments are cleaned and bio-hazardous During the 1990s, the US Department of Labor, Occupational waste is disposed. Safety and Health Administration (OSHA) passed a regulation known as the Blood Borne Pathogen Standard. The standard Marble or polished stone flooring is the preferred type with required institutions to implement policies and procedures glazed tile walls. No false ceiling is permissible. The OT for the identification of potential exposure to blood borne needs to be well ventilated such that it prevents any deposition pathogens. The Association of peri Operative Registered of dust particles. Air circulation with a laminar air flow system Nurses (AORN) developed “Recommended Practices for through High efficiency particulate air filter (HEPA) (0.3µm) Environmental Cleaning in the Surgical Practice Setting,” serves the best purpose. As per US Public Health services which was approved by AORN’s board of directors and minimum requirements for OT air are 25 changes per hour, became effective from January 1, 2003. positive pressure compared with corridors, temperature between 18 & 24º C and humidity of 50 to 55%.[1] Asepsis in Operation Theatre Aseptic technique is a set of specific practices and procedures Cleaning and Disinfection of OT performed under carefully controlled conditions with the goal Cleaning, disinfection and sterilization are the cornerstones in of minimizing contamination by pathogens. ensuring operation theatre asepsis.

The Operation Theatre: Basic architecture Cleaning The design and location of OT complex is one of the most General Cleaning: important components of OT asepsis. OT complex is located General cleaning involves scrubbing with detergents and away from the inpatient area, often in a blind wing or on rinsing with water. This is the first step, albeit very important, the top or bottom floor. It is a scientifically planned barrier before any disinfection measure can be undertaken. Spot system, such that it keeps the flow of traffic from clean areas cleaning of walls and ceiling should be undertaken as needed to dirty ones and never vice versa. It consists of 4 zones: everyday. Open shelves need to be cleaned daily with a detergent while closed cabinets may be cleaned once weekly. A. Outer zone The floor should ideally be sprayed and wet vacuum pick up Areas for receiving patients relatives, toilets, administrative used after each surgical procedure and at the end of the days’ function. schedule. All horizontal surfaces in the OT (e.g. furniture, surgical B. Restricted Zone lights, and equipment) should be damp-dusted with - Changing room an Environmental Protection Agency (EPA) registered - Patient transfer area disinfectant like lysol brand deordorizing disinfectant cleaner

Vol. 21, No. 3, January-March, 2011 DJO 9 Delhi Journal of Ophthalmology The Operation Theatre : Basic Architecture and clorox disinfecting spray III at the end of each case and Chemical Disinfection cleaned thoroughly at the end of the day. The lights and other a)Formaldehyde fumigation portable equipment should additionally be steam cleaned Commonly used to sterilize the OT. weekly. Similarly, anesthesia equipment needs to be cleaned and processed according to AORN’s recommended practices. Requirement (For an area of 1000 cubic feet) The sink area should be cleaned several times daily and kept - 500 ml of 40% formaldehyde in one liter of water as dry as possible - Stove or hot plate for heating formalin The outside of autoclaves should be cleaned daily while the - 300 ml of 10% Ammonia inside surface is cleaned weekly. Soiled linen should never be left on the floor or transported on a trolley used for other Procedure purposes. Liquid waste material such as the contents of suction Close all doors & windows air tight and switch off fans and bottles should never be disposed off in a scrub sink or utility A.C. sink but only into a container meant for the purpose. Heat formalin solution till boiling dry Leave the OT unentered over night Cleaning before subsequent surgery Enter the OT next day morning with 300ml of ammonia For each subsequent surgical case, a safe environment needs to Keep the ammonia solution for 2-3 hrs to neutralize formalin be re- established. Preparation of the OT should involve visual vapours inspection and spot cleaning of visible contamination. Walls, Open the OT to start surgery doors, surgical lights and ceilings should be spot cleaned in Advised fumigation at weekly intervals the event of being soiled with blood, tissue or body fluids. Visibly soiled areas on the floor should be cleaned using a Mode of Action new or freshly laundered mop head and an EPA- registered Formaldehyde inactivates microorganisms by alkylating the hospital-grade germicidal agent. The OT bed should be moved aminoacid and sulfhydryl groups of proteins and ring nitrogen to check for such items as sponges and instruments that may atoms of purine bases. have fallen into open spaces. Data does not support cleaning the entire floor after each case. Disadvantages OSHA indicated that Formaldehyde should be handled in Terminal Cleaning the workplace as potential carcinogen and set an employee Because a clean surgical environment assists in the reduction exposure standard for Formaldehyde that limits an 8-hour of microorganisms, each surgical procedure room and scrub/ time- weighted average exposure concentration of 0.75ppm. utility area should be terminally cleaned at the end of the day’s schedule. An EPA-registered agent lysol brand deordorizing b) Commercially available disinfectant disinfectant cleaner and clorox disinfecting spray III and Baccilocid rasant mechanical friction needs to be used to clean all surfaces, A newer and effective compound in environmental including the surgical lights, all furniture, scrub and utility decontamination with very good cost/benefit ratio, good areas, scrub sinks etc. material compatibility, excellent cleaning properties and virtually no residues. It has the advantage of being Disinfection of the OT a Formaldehyde-free disinfectant cleaner with low use There are three levels of disinfection: High, intermediate, and concentration. low. High- level disinfection kills all organisms, except high Active ingredients: Glutaral 100 mg/g, benzyl-C12-18- levels of bacterial spores and prions, and is effected with a alkyldimethylammonium chlorides 60 mg/ g, chemical germicide cleared for marketing as a sterilant by didecyldimethylammonium chloride 60 mg/g. the Food and Drug Administration like glutraldehyde based and orthopthaldehyde based agents (available in ) . Advantages Intermediate- level disinfection kills mycobacteria, most - Provides complete asepsis within 30 to 60 minutes. viruses, and bacteria with a chemical germicide registered - Cleaning with detergent or carbolic acid not required. as a tuberculocide by the Environmental Protection Agency - Formalin fumigation not required. (EPA). Low- level disinfection kills some viruses and bacteria - Shutdown of O.T. for 24 hrs not required. with a chemical germicide registered as a hospital disinfectant by the EPA. c) Aldekol A new method of fumigation has been evolved using ‘Aldekol’, a mixture containing 6% formaldehyde, 6% glutaraldehyde

DJO Vol. 21, No. 3, January-March, 2011 10 The Operation Theatre : Basic Architecture Delhi Journal of Ophthalmology and 5% benzalkonium120 chloride instruments, catheters, needles, implants, etc 2) Items that come in contact with non intact skin or mucous Disinection by radiation membranes are considered semi critical and should receive a minimum of high-level disinfection immediately before Ultraviolet radiation use. This includes the anesthesia equipment, respiratory - Daily U.V. Irradiation for 12 -16 hrs therapy equipment etc. - To be switched off 2 hrs before 3) Items that come in contact only with intact skin are categorized as noncritical items and should receive Processing of Equipment, Instruments and Other intermediate-level disinfection, low-level disinfection, Reusable Items or cleaning. This is explained as intact skin acts as an effective barrier to most organisms. Steps involved are (1) Examples of noncritical items include the tourniquets and blood pressure cuffs, linens, etc. 1. Decontamination of Equipment, Instruments, and Other Reusable Items Methods of High Level Disinfection of equipments Immediately after use, all surgical instruments, reusable gloves, and other items that have been in contact with blood Physical methods or other body fluids should be placed in a plastic bucket • HLD by Boiling: containing a solution of 0.5% chlorine for 10 minutes.[2] After Boling destroys all vegetative forms of bacteria, viruses 10 minutes, the items should be removed from the chlorine (including HBV, HCV and HIV), yeasts and fungi, but does solution and rinsed with water or cleaned immediately not kill all endospores reliably. 20 minutes of immersion time is needed, after the water has started boiling. Once the 2. Cleaning of Equipment, Instruments, and Other instruments are dry, if any pooled water remains in the bottom Reusable Items of the container, remove the dry items and place them in The instruments and other items should be scrubbed vigorously another high-level disinfected container that is dry and can be with a brush (a tooth brush is a good option) in lukewarm tightly covered. If stored in an ordinary covered container, the water with detergent to remove all blood, tissue, and other objects can be used for up to 24 hours only. residue. Cleaning instruments with ultrasonic cleaner is used for cleaning of micro surgical instruments and instruments • HLD by Steaming (moist heat) with hinged areas and serrated edges, endoscopes or other Essentially all vegetative forms of bacteria are killed by moist lumened devices such as phaco or irrigation & aspiration hand heat at temperatures of 60– 75 0C within 10 minutes [5] pieces. Hepatitis B virus, which is one of the most difficult viruses to kill, is inactivated in 10 minutes when heated to 80 0C. In 3. Sterilization and high level disinfection of equipments contrast, although many types of spores are killed when boiled Sterilization is complete destruction of all microorganisms, at 99.5 0C for 15 to 20 minutes, Clostridium tetani spores are both the vegetative forms and their spores. It is the terminus quite heat-resistant and can even survive boiling for up to 90 of the continuum where risk of contamination is, effectively, minutes.The highest temperature that boiling water or low- reduced to the lowest practical point. Although sterilization pressure steam will reach is 100 0C (212 0F) at sea level. is the safest and the most effective method for the final Because the boiling point of water is 1.1 0C lower for each processing of instruments, often sterilization equipment 1,000 feet in altitude, it is best to boil or steam items to be is either not available or not suitable. In these cases, High high-level disinfected for a minimum of 20 minutes Level Disinfection is the only acceptable alternative.[3] The threat of infection by spores or prions does not exist in • High level disinfection only by chemical method all circumstances so sterilization of all items used on or by Although a number of disinfectants are commercially patients is unnecessary. The system used to determine whether available in most countries, four disinfectants— chlorine, items should be cleaned, disinfected, or sterilized was given in glutaraldehyde, formaldehyde and peroxide— are routinely 1968 by Earl Spaulding According to the Spaulding system, used as high-level disinfectants. These chemicals can the level of processing required is based on the nature of the achieve high-level disinfection if the items being disinfected item and the manner in which it is to be used.[4] are thoroughly cleaned before immersion.[6,7] A high- 1). Items that enter sterile tissue or the vascular system are level disinfectant should be selected for use based on the categorized as critical and should be sterile when used. characteristics of the items to be disinfected, the physical area Examples of critical items include most of the surgical and the skills of personnel available to do the procedure.[8,9]

Vol. 21, No. 3, January-March, 2011 DJO 11 Delhi Journal of Ophthalmology The Operation Theatre : Basic Architecture A brief review of each of the above agents is provided here: The use of chemical solutions as a sterilization technique for surgical equipment is frequently employed, but it should be Chlorine solutions stressed that most solutions only disinfect and do not guarantee are fast acting, very effective against HBV, HCV and HIV/ sterility. When the necessity for maintaining sterility is a AIDS, inexpensive and readily available (CDC 1987; WHO critical factor, as in the implantation of prosthetic devices, 1989).[10,11,12] indwelling catheters or vascular access ports, disinfection in chemical solutions is not recommended. [16] Formaldehyde (8%), which is inexpensive and readily available, is an Glutaraldehyde( 2%) effective high-level disinfectant (HLD) but, the vapours are It is suitable for instruments that cannot be autoclaved like very irritating and it is classified as a potential carcinogen. sharp cutting instruments, plastic & rubber items, and Do not dilute with chlorinated water as a dangerous gas (bis- endoscopes. It is effective against vegetative pathogens chloromethyl-ether) can be produced. in 15 minutes and resistant pathogenic spores in 3 hrs. It is not recommended for lumen containing instruments such Glutaraldehydes as irrigating cannulae as the residual glutaraldehyde, even are less irritating than formaldehyde, but staff and clients still after rinsing, causes corneal edema, endothelial cell damage need to be protected from the fumes when mixing and using and . The recommended time period for effective these solutions sterilization is 8- 10 hours. Articles can then be stored in a covered sterile container for up to 7 days. Hydrogen Peroxide (H2O2), which must be diluted to a 6% solution, often is Chemical Sterilization (Gas) available locally and is less expensive than other chemical Ethylene Oxide (E. T. O.) disinfectants. The 3% H2O2 solutions used as antiseptics, E. T. O. kills micro organisms by alkylating their DNA. Widely however, should not be used as a disinfectant. The major used for re -sterilizing ‘packaged heat sensitive devices’ like disadvantage of peroxide is that it is highly corrosive and sharp knives and blades. should not be used to disinfect copper, aluminum, zinc or It is non-corrosive and safe for most plastic and polyethylene brass. materials. Thus, it is the preferred method for sterilizing heat labile tubings, vitrectomy cutters, cryoprobes, light pipes, Alcohols and Iodophors laser probes, diathermy leads, cannulated instruments like Although alcohols and iodophors are inexpensive and endoscopes etc. However, it is not applicable to liquids or to readily available, they are no longer classified as high-level articles in impervious packaging material. disinfectants. Alcohols do not kill some viruses and are not sporicidal. Pseudomonas species have A typical ETO sterilization cycle includes been shown to multiply in iodophors. [13] 1. Packing of the articles to be sterilized. 2. Arranging and loading the sterilizer. Sterilization of equipment 3. Air removal with a vacuum pump. Steam Sterilization (Autoclaving) 4. Heating to the required temperature (45 0C– 55 0C). Steam sterilization (frequently referred to as autoclaving) 5. Steam humidification maintained at a relative humidity of depends on the use of steam above 100 0C. Temperatures 60 %. ranging from 121-134 0C at pressures of 15-30 psi are generally 6. Exposure to the ETO at 5 psi for 12 hours or at 10 psi for recommended. Steam readily penetrates all wrapped materials 6 hours. with the destruction of all viruses and bacteria, including the 7. Gas removal by 70 psi vacuum. most resistant spores. [14,15] It acts by denaturing the major 8. Air flush by filtered air repeated 4 times to re -establish cell constituents. Minimum holding times for the sterilization atmospheric pressure. of medical equipment are 15 minutes at 121 0C, 10 minutes at 9. Aeration to elute residual ETO - Articles should be well 1260C, and 3 minutes at 1340C. aerated prior to use to minimize the potential for tissue toxicity. Flash Sterilization Ethylene oxide gas is a potential carcinogen and mutagen and It is a method of emergency sterilization. The equipments to represents a potential occupational health hazard for personnel be decontaminated are kept at 132º C at 30 lbs of pressure for operating the sterilizers. 3 minutes. Sterilization by radiation Chemical Sterilization (Liquids)

DJO Vol. 21, No. 3, January-March, 2011 12 The Operation Theatre : Basic Architecture Delhi Journal of Ophthalmology Gamma irradiation India) impregnated with spores of Bacillus stereothermophillus This is a method for cold sterilization with high penetrating are used. For ETO sterilizer, the biological indicator is a power which is lethal to DNA. Bacillus subtilis spore.

Sterilization methods of choice for articles during ocular Principles of sterile technique in OT surgery • All items in a sterile field must be sterile. 1. Linen (Gowns, Caps, Masks, Drapes)- Autoclaving. • Sterile packages or fields are opened or created as close 2. Glassware (Syringes) - Dry heat sterilization, or use as possible to time of actual use. disposables from reputed firms. • Moist areas are not considered sterile. 3. Metal instruments- Autoclaving. • Contaminated items must be removed immediately from 4. Plastic instruments/ Components- Ethylene oxide the sterile field. sterilization, formalin chamber. • Only areas that can be seen by the clinician are considered 5. Sharp edges instruments (e.g. Vannas scissors, keratome) sterile, i.e., the back of the clinician is not sterile. ETO/ Hot air oven/ Chemical disinfection. • Gowns are considered sterile only in the front, from chest 6. Sutures (including monofilament nylon) - Can be to waist and from the hands to slightly above the elbow. autoclaved. • Tables are considered sterile only at or above the level of 7. Diathermy, Cautery electrodes- Autoclaving. the table. 8. Endoilluminators/ probes- Ethylene oxide sterilization. • Nonsterile items should not cross above a sterile field. 9. Silicone oil/ buckles/ sponges- Autoclaving. • There should be no talking, laughing, coughing, or sneezing across a sterile field. Monitoring • Personnel with colds should avoid working while ill or Surveillance of Operation theatre: Microbiological apply a double mask. monitoring • Edges of sterile areas or fields (generally the outer inch) Swabs are collected from various locations in the OT and are not considered sterile. cultured as described. The areas swabbed include • When in doubt about sterility, discard the potentially 1. Operation table at the head end contaminated item and begin again. 2. Over head lamp • A safe space or margin of safety is maintained between 3. Four Walls sterile and nonsterile objects and areas. 4. Floor below the head end of the table • When pouring fluids, only the lip and inner cap of the 5. Instrument trolley pouring container is considered sterile. The pouring 6. AC duct container should not touch the receiving container, and 7. Microscope handles. splashing should be avoided. The swabs obtained are cultured for aerobic (Chocolate agar) • Tears in barriers are considered breaks in sterility. and anaerobic (Robertson’s Cooked Meat Medium) growth. [13] Legal responsibility In each hospital, an interdisciplinary team should meet Evaluation of Quality of air in OT periodically to discuss the process of cleaning the operating Settle plate method: One plate of blood agar and Sabouraud’s rooms. Role of Microbiology Departments lies in identifying dextrose agar (SDA) is placed in the center of the OT (Close the pathogens, monitoring of antibiotic therapy and proper to operation table) and the lid is kept open for 30 min. Blood education on specimen collection and transportation. They agar is then incubated at 37° C for 48 hrs,& SDA at 27° C for should also be updated with information on common 7 days. Colony counts of bacteria and fungi are reported.[5] antibiogram patterns, and communicate the same to the Slit sampler method (from given volume): Very Effective, clinical staff. They maintain data on hospital infection and highly sensitive. Fixed volume of air is sucked and bacterial surveillance of the Hospital environment. The team must counts are made.Bacterial colony count of more than 10 consider changes needed in the cleaning protocol in response per plate and fungal colony of more than one per plate are to rising infection rates with increased multidrug-resistant considered unacceptable. bacteria or newly emerging pathogens. Importance of Staff Education cannot be over emphasized. Testing efficacy of autoclaves They need to be well trained in a scientific manner with Biological indicators (BI) containing bacterial spores are specific duties and responsibilities allotted to each. Written used for monitoring the efficacy of sterilizers. Commercially policies and protocols lay the groundwork for all personnel to available spore strips (Hi- Media, Mumbai and by Cole Palmer, have the same understanding of the outcome expected.

Vol. 21, No. 3, January-March, 2011 DJO 13 Delhi Journal of Ophthalmology The Operation Theatre : Basic Architecture References 9 Tietjen LG, W Cronin and N McIntosh. 1992. High-level 1. Sehulster LM. Chinn RYW, Arduino MJ, Carpenter J, Donlan R, disinfection, in Infection Prevention Guidelines for Family Ashford D, et al Guidelines for environmental refection control Planning Programs. Essential Medical Information Systems, in health care facilities. Recommendations from CDC and the Inc.: Durant, OK, pp 74–84. Healthcare Infection Control Practices Advisory Committee 10. Centers for Disease Control (CDC). 1987. Recommendations (HICPAC) November 2003. for prevention of HIV transmission in health care settings. 2. McIntosh N et al. 1994. Practical Methods for High-Level MMWR 36(Suppl 2): 1S–18S Disinfection of Surgical Gloves. Paper presented at American 11. Rutala WA et al. 1998. Stability and bactericidal activity of Public Health Association Annual Meeting. Session no. 2285, chlorine solutions. Infect Control Hosp Epidemiol 19(5): 323– Washington, D.C., 31 October–4 November. 327. 3. Rutala WA. Disinfection and sterilization of patient care 12. Rutala WA. 1996. APIC guidelines for selection and use of items In: Herwaldt LA, Decker MD. A Practical Handbook disinfectants. Am J Infect Control, 24(4): 313–342. for Hospital Epidemiologists. Thorofare, NJ BLACK Inc; 13. Favero MS. 1985. Sterilization, disinfection, and antisepsis 1998:271-280. in the hospital, in Manual of Clinical Microbiology, 4th ed. 4. Spaulding EH. 1939. Studies on chemical sterilization of Lennette EH et al (eds). American Society for Microbiology: surgical instruments. Surg Gyne Obstet 69: 738–744. Washington, DC, pp 129–137. 5. Salle AJ. 1973. Fundamental Principles of Bacteriology, 7th ed. 14. Perkins JJ. 1983. Principles and Methods of Sterilization in McGraw- Hill Book Company: New York. Health Sciences, 2nd ed. Charles C. Thomas Publisher Ltd.: 6. Spaulding EH Role of chemical disinfection in the prevention Springfield, IL. of nosocomial infections In: Brachman PS, Eickhoff TC, eds 15. Russell AD, WB Hugo and GA Ayliffe. 1982. Principles Proceedings for the International Conference on Nosocomial and Practice of Disinfection, Preservation and Sterilization. Infections, 1970 Chicago, IL: American Hospital Association: Blackwell Scientific Publications: Oxford, England. 1971:247-254. 16. Spaulding EH. Chemical disinfection of medical and surgical 7. Spaulding EH. Chemical disinfection and antisepsis in the instruments In: Lawrence CA, Block SS. eds. Disinfection, hospital J Heap Res 1972;9:5-31. Sterilization, and Preservation Philadelphia, PA: Lea & Febiger; 8. Rutala WA. Selection and use of disinfectants in health care. In: 1968. Mayhall CG Hospital Epidemiology and Infection Control 1st ed Baltimore, MD: Williams & Wilkins; 1996:913-936.

DJO Vol. 21, No. 3, January-March, 2011 14 Delhi Journal of Ophthalmology

Major Review Upshoot And Downshoot In Duane’s Retraction Syndrome: Mechanism And Treatment Suma Ganesh Department of and , Dr Shroffs Charity Eye Hospital, New Delhi

Definition narrowing of palpebral fissure, and significant upshoot or Duane syndrome (DS) is a rare, congenital downshoot[5].Successful surgery results in a straighter disorder most commonly characterized by the inability of the head position, a lessening of and upshoot and eye to turn out. The syndrome was first described by Jakob downshoot, and better alignment in primary position. Stilling (1887) and Siegmund Türk (1896), and subsequently named for Alexander Duane who discussed the disorder in Surgical Options more detail in 1905[1]. Various surgical techniques have been employed for the treatment of upshoot and downshoot. Eisenbaum and Parks Clinical Features [6] reported performing posterior fixation suture (Faden DS is a miswiring of the eye muscles that causes some eye operation) on vertical and horizontal muscles to treat upshoot muscles to contract when they should not and other eye and downshoot in patients with Duane retraction syndrome. muscles not to contract when they should. Stabilization of the , however, did People with DS have abduction deficiency, narrowing of produce satisfactory results with elimination of the upshoot. the palpebral fissure with retraction of globe on attempted adduction, and upshoot or downshoot, which can be the most Recession of both horizontal recti prominent feature[2]. Recession of both horizontal rectus muscles has been shown to decrease the elevation and depression of the adducted eye Upshoot and Downshoot: Mechanism and improvement in retraction of the globe[7,8]. The amount The upshoots and downshoots are seen in 25% to 39% of of recession of both horizontal rectus muscles, if balanced, patients with Duane’s retraction syndrome[3]. Upshoots and will not adversely effect alignment or motility[7,8]. However, downshoots are classified into two types: mechanical and it is necessary to consider the primary position deviation when innervational[4]. deciding on the amount of surgical intervention. The medial In the mechanical type upshoot and downshoot in DS is due rectus muscle should be recessed relatively more than the to tight lateral rectus attached to the crest of the globe, which lateral rectus muscle in cases of in primary position causes the muscle to slip off the globe when the eye is adducting. whereas in the recession of lateral rectus may be This upshoot may look cosmetically very disfiguring even in proportionately increased. The following case illustrates patients with orthtropic Duane’s syndrome. Removal of the improvement with this procedure. muscle from the crest of the globe can be achieved either by a very large recession of the lateral rectus muscle or by Y Case 1 splitting the lateral rectus muscle. (figure 1a and b). A 16-year-old female presented with squinting and an In the innervational type, there is gradually increasing upshot enophthalmic appearance of left eye. Unaided visual acuity or downshoot of the eye in the horizontal position as it moves was 6/6 in both eyes. She had normal head posture .Orthoptic into adduction. This is due to co-innervation of the vertical examination showed she had exotropia of 12 prism diopters rectus muscle with the lateral rectus muscle. The innervational with limitation of movement in adduction of left eye and type can be improved with recession of the appropriate vertical severe upshoot on adduction. rectus muscle[4]. Surgery Management Large recession of the medial (10-mm) & Lateral rectus Indications for surgery (12mm) was performed in the ipsilateral eye. Postoperatively Not all patients of Duane’s Retraction Syndrome require a left 2 prism diopters and residual exotropia of 8 surgery. Indications for surgery include a significant deviation prism diopters was recorded. The enophthalmic appearance of in primary position, abnormal head posture, retraction and the eye improved and there was no upshoot during adduction.

Vol. 21, No. 3, January-March, 2011 DJO 15 Delhi Journal of Ophthalmology Upshoot And Downshoot In Duane’s Retraction Syndrome: Mechanism And Treatment syndrome. The patients showed a marked decrease in upshoot Upgaze and downshoot, without vertical deviation, after surgery. Das et al reported marked amelioration of upshoot and downshoot with Y-splitting of the lateral rectus muscle at the insertion in a patient with type-I Duane retraction syndrome with cosmetically unacceptable upshoot in adduction. The following two cases are examples to show an improvement in Primary upshots and down shoot after Y split surgery.

Downgaze

Figure 1A : Severe upshoot and downshoot on adduction

Upgaze

Primary

Downgaze

Figure 2 : The bifurcation and recession of the lateral rectus (top) for upshoot and downshoot acts by removing the muscle from the crest of the globe, with the two recessed muscle arms spread apart. Each muscle arms are vertically transposed Figure 1B : Postop pictures showing improvement after Y split such that the two arms are spread apart a total of 20 mm and each arm recessed 5-10 mm as appropriate for each case. This Y – split of lateral rectus ameliorates both the globe retraction and the upshoot and or The splitting of the ends of the lateral rectus muscle into a downshoot. Y-configuration (figure 2a & b) is a unique idea first advocated This figure is adapted from: Jampolsky A. Duane Syndrome by Jampolsky[9]. The bifurcation of the muscle decreases in Rosenbaum A L. Santiago A P. Clinical Strabismus the upward or downward rotation of the globe because the Management: Principles and Techniques 1999. W B Saunders halves are positioned to stabilize the muscle’s position on Company: 335-336. the eye. Subsequently, Rogers and Bremer[10] performed Y-splitting of the lateral rectus muscle along with medial Case 2 rectus muscle recession in five patients with Duane retraction A 12-year-old girl presented with complaints of squinting

DJO Vol. 21, No. 3, January-March, 2011 16 Upshoot And Downshoot In Duane’s Retraction Syndrome: Mechanism And Treatment Delhi Journal of Ophthalmology since childhood. Unaided visual acuity was 6/6 OU. She did Case 3 not have abnormal head posture and there was no squint in A 25- year- old male came to SCEH with complaints of the primary position. However, Version tests revealed that abnormal head posture since childhood. Unaided visual acuity there was limitation of abduction of the left eye and there was was 6/6 in both eyes. He had face turn to the right side. He severe upshoot and down shoot on adduction. had left exotropia of 20 prism diopters when his head was straight. Version testing showed a limitation of abduction Upshoot of his left eye and retraction of the globe and narrowing of the palpebral fissure on adduction was noted. A significant upshoot and downshoot was noted in elevation and depression in the adducted position. Fusion and stereopsis was present with abnormal head posture and he had stereopsis of 400 arcs second on the Titmus test. Primary

Downshoot

Figure 4A : Improvement in head posture after surgery

Figure 3 A: Preop photos

Surgery She underwent 5 mm MR recession combined with 7 mm lateral rectus recession combined with lateral rectus Y split. After surgery her upshoot and downshoot were corrected and she looked much better cosmetically. Figure 3a & b

Figure 4B : Marked upshoot in dextroversion pre operatively

Figure 3 B: Improvement in Upshoot & Downshoot Figure 4C : Improvement in upshoot post operatively

Vol. 21, No. 3, January-March, 2011 DJO 17 Delhi Journal of Ophthalmology Upshoot And Downshoot In Duane’s Retraction Syndrome: Mechanism And Treatment Preop 6. Eisenbaum AM, Parks MM. A study of various surgical The patient underwent lateral rectus recession of 8 mm approaches for the leash effect in Duane’s syndrome. Presented combined with a Y splitting of the lateral rectus 20 mm apart. at the joint session of the American Association for Pediatric Post- operatively his head posture had completely resolved Ophthalmology and Strabismus and the American Academy of but he had some residual upshoot which was not cosmetically Ophthalmology, Chicago, IL, November 5, 1989 disturbing to patient. Figure 4 a, b & c 7. von Noorden GK. Recession of both horizontal recti muscles in Duane’s retraction syndrome with elevation and depression of References the adducted eye. Am J Ophthalmol. 1992;114:311–313. 1 Duane A. Congenital deficiency of abduction associated with 8. Sprunger DT. Recession of both horizontal rectus muscles in impairment of adduction, retraction movement, contraction of Duane’s syndrome with globe retraction in primary position. J the palpebral fissure and oblique movements of the eye. Arch Pediatr Ophthalmol Strabismus. 1997;1:31–33. Ophthalmol. 1905;34:133–159. 9. Jampolsky A. Discussion of Eisenbaum AM, Parks MM. A 2. Das JC, Chaudhuri Z, Bhomaj S, Sharma P. Lateral rectus split study of various surgical approach to the leash effect in Duane’s in the management of Duane’s retraction syndrome. Ophthalmic syndrome. Presented at the joint session of the American Surgery and Lasers 2000;31:499-501 Association for pediatric ophthalmology and strabismus and the 3. Mohan K, Saroha V, Sharma A. Factors predicting upshoots American Academy of Ophthalmology, Chicago, IL, November an downshoots in duane retraction syndrome. J Pediatric 5, 1980 Ophthalmol Strabismus 10. Rogers GL, Bremmer DL. Surgical treatment of the upshoot and 4. Kraft SP. Surgical approach for Duane syndrome. J Pediatric downshoot in Duane’s retraction syndrome. Ophthalmology. Ophthalmol strabismus1988;25 : 119- 130 1984;91:1380–1382 5. Gobin MH. Surgical management of Duane’s syndrome. Br J 11. Feretis D. Papastratigakis B, Tsanparlakis J. Planning surgery in Ophthalmol. 1974;58:301–306. Duane’s retraction syndrome. Ophthalmologica. 1981;183:148– 153.

DJO Vol. 21, No. 3, January-March, 2011 18 Delhi Journal of Ophthalmology

Major Review Marcus Gunn Jaw-Winking Phenomenon : A Review Dewang Angmo, Mandeep S. Bajaj, Neelam Pushker, Supriyo Ghose Dr. Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, New Delhi Marcus Gunn jaw-winking phenomenon is the most common In 1883 Robert Marcus Gunn described a 15yr girl with a form of congenital synkinetic neurogenic . In this peculiar type of congenital ptosis that included an associated synkinetic phenomenon, the unilaterally ptotic eyelid elevates winking motion of affected eyelid on the movement of jaw1. with jaw movements. The movement that most commonly This synkinetic jaw-winking phenomenon now bears its name. causes elevation of the ptotic eyelid is lateral mandibular movement to the contralateral side. This phenomenon is usually first noticed by the mother when she is feeding or nursing the baby. This article presents a review of Marcus Gunn jaw-winking phenomenon including clinical features, pathophysiology and treatment modalities.

Figure2: Patient with Marcus Gunn Jaw-Winking phenomenon. (A) 22 yrs female with unilateral upper eyelid ptosis as part of Marcus Gunn phenomenon. (B) Left upper eyelid raises with the jaw movement to opposite side.

The Marcus Gunn phenomenon is known variously as jaw- winking (a misnomer as eyelid rises rather than falls) and more Figure 1: Patient with Marcus Gunn Jaw-Winking phenomenon. descriptively, as pterygoid-levator synkinesis2. The Marcus (A) 5year old child with unilateral upper eyelid ptosis as part of Gunn phenomenon has been associated with congenital Marcus Gunn phenomenon. (B) Elevation of concomitant upper blepharoptosis with an incidence of 4-6% [2,3,4]. Acquired eyelid with mouth opening

Vol. 21, No. 3, January-March, 2011 DJO 19 Delhi Journal of Ophthalmology Marcus Gunn Jaw-Winking Phenomenon : A Review forms have been described after eye surgery, trauma, post 2) Functional Interference Bells palsy and pontine tumors[2]. Spontaneous remission of 1. Irritation of normally dormant connection the acquired form may be expected, whereas the congenital 2. Disinhibition of pre-existing phylogenetically more form persists (no improvement with age)[12]. Patients with primitive mechanisms (Ascher): This is thought to Marcus Gunn jaw-winking phenomenon have a variable explain why individuals who are not affected will often degree of blepharoptosis in the resting and primary position. open their mouth while attempting to widely open their Although Marcus Gunn jaw-winking syndrome is usually eyes to place eye drops unilateral[12-13] it can present bilaterally in rare cases. 3. Spread of impulses by irradiation

The characteristic feature of the phenomenon is that the 3) Atavistic Reversion raising; and not winking of the affected eyelid is synchronous 1. In fish a strong associated movement of jaw opening and with and proportionate to the opening of the mouth. The wink eye opening i.e., deep muscle contracting and superficial reflex consists of a momentary upper eyelid retraction or muscle relaxing. Thus a weak levator may only elevate elevation to an equal or higher level than the normal fellow the lid when its antagonist, the orbicularis (superficial eyelid upon stimulation of the ipsilateral pterygoid muscle muscle) is reflexly relaxed by jaw opening (external 12-13. pterygoid-deep muscle contraction ) 2. EMG study suggested dysfunction in the midbrain and This response is followed by a rapid return to a lower position. brainstem The amplitude of the wink tends to be worse in downgaze. This rapid, abnormal motion of the eyelid can be the most Measurement of Mgjwp disturbing aspect of the jaw-winking syndrome. The amount of jaw-winking is the excursion of the upper eyelid with synkinetic mouth movement. It is measured with MARCUS GUNN JAW-WINKING PHENOMENON a millimeter ruler. Jaw-winking is assessed as [2,10] : Mild < The wink phenomenon i.e., retraction of the ptotic lid occurs 2mm ; Moderate 2-5mm; Severe ≥ 6mm in conjunction with stimulation of pterygoid muscle, which is elicited by opening the mouth, thrusting the jaw to the contra Frequency lateral side, jaw protrusion, chewing, smiling or sucking. This Approximately 50% of blepharoptosis cases are congenital. wink phenomenon is often discovered early, as the infant is Incidence of Marcus Gunn jaw-winking syndrome among this bottle-feeding or breastfeeding[14]. population is approximately 4-5% [12,13].

Jaw-winking ptosis is almost always sporadic, but familial Associations cases with an irregular autosomal dominant inheritance 1. Ocular pattern have been reported[15]. 1. Strabismus (50%-60%)[9] 1. Superior Rectus Palsy-25% Pathophysiology 2. Double Elevator Palsy-25% A complete explanation has not yet been advanced to elucidate 2. (5%-25%)[9] the rationale of jaw-winking phenomenon. Various theories Incidence of anisometropia among patients with Marcus have been hypothesized [15,16]. Gunn jaw-winking syndrome is reported to be 5-25%. 3. (30-60%)[9] 1) Aberrant connection Almost always secondary to strabismus or anisometropia, This hypothesis is favored by most authors, though they differ and only rarely, is due to occlusion by a ptotic eyelid. in opinion as to the location of the aberration 1. Cortical or sub cortical connections 2. Systemic 2. Internuclear connections or faulty distribution in the Systemic anomalies in association with Marcus Gunn posterior longitudinal bundle phenomenon are rare. 3. Infranuclear connection exists between motor branches 1. Cleft lip/ Cleft palate of the trigeminal nerve (CN V3) innervating the external 2. CHARGE Syndrome reported in association with pterygoid and the fibers of superior division of the bilateral cases. (CN III) that innervates the levator 3. Renal calculi (Awan 1976) muscle of the upper lid 4. Peripherally - some CN V fibers may reach the levator via Schultz and Burian (1960) reported a case of MGP associated the auriculo- temporal nerve with several systemic malformations. These included

DJO Vol. 21, No. 3, January-March, 2011 20 Marcus Gunn Jaw-Winking Phenomenon : A Review Delhi Journal of Ophthalmology ectrodactly, bilateral pes cavus with ankle varus, spina bifida procedure). occulta, bilateral undescended testis and supernumerary 3.Consider eyelid surgery only when the parents (or the incisors. patient) and the surgeon agree about whether the most cosmetically objectionable condition is the ptosis or the jaw- Race winking or whether it is a combination of both. No known racial predilection exists. 4. Many techniques are described for the correction of jaw- winking ptosis, reflecting the ongoing controversy regarding Sex the surgical management of this condition. Early reports showed jaw-winking ptosis to be more prevalent 5. If the jaw-winking is cosmetically insignificant, it can be in females than in males; however, larger case series have ignored in the treatment of the ptosis. shown an equal prevalence among males and females4, 9. If the ptosis is mild, the patient may elect not to proceed with surgery. If correction is desired, perform a Muller muscle and Age conjunctival resection (MMCR), a Fasanella-Servat procedure Marcus Gunn jaw-winking syndrome is usually evident at or a standard external levator resection[14,18]. birth. The winking phenomenon is often first noted by the If the ptosis is moderate to severe, a levator resection may be parents when the infant is feeding. indicated. Beard advocated performing more resection than normal to avoid undercorrection[13]. Treatment In severe ptosis, a super maximum (>30 mm) levator resection 1. Medical Care or frontalis suspension is necessary [19]. If amblyopia is encountered, treat aggressively with occlusion 6. Although the amount of ptosis and synkinetic eyelid therapy and/or correction of anisometropia prior to any movement is variable, those patients with more severe ptosis consideration of ptosis surgery. tend to have the worse aberrant upper eyelid movement. 2. Surgical Care 7. The jaw-wink is considered cosmetically significant if it is As with any patient who requires eyelid surgery, first address 2 mm or more [2]. associated strabismus. 8. Any attempt to repair the ptosis without addressing the jaw- winking would result in an exaggeration of the aberrant eyelid 1. Superior rectus palsy movement to a level well above the superior corneal limbus, Superior rectus palsy can be corrected by resecting the which would be unacceptable to the patient. superior rectus muscle but only in the absence of inferior 9. Several techniques have been suggested to obliterate rectus restriction. levator function, which effectively dampens the aberrant Since the superior rectus is loosely bound to the overlying eyelid movement. levator, the upper eyelid will be pulled inferiorly during Bullock advocated complete excision of the levator resection, exacerbating any ptosis already present. This can aponeurosis and muscle all the way to the orbital apex [18]. be addressed during the subsequent ptosis repair. Dillman and Anderson argued that removal of a portion of the 2. Double elevator palsy levator muscle above the Whitnall’s ligament (i.e., myectomy) Double elevator palsy manifests as a deficit in the elevation of is adequate to obliterate its function without extensive the globe in all fields of gaze. dissection and damage to eyelid structures [8,19]. It may be the result of superior rectus and inferior oblique Bowyer and Sullivan describe the removal of a portion palsy and/or inferior rectus restriction. of levator muscle above the Whitnall ligament through a Inferior rectus restriction may be suggested by the posterior conjunctival approach[16]. following Dryden et al proposed suturing the transected levator 1. Positive forced duction in elevation aponeurosis to the arcus marginalis of the superior orbital 2. Normal force generations in up gaze indicating rim[20]. This technique not only effectively deactivates an absence of superior rectus or inferior oblique the muscle but also allows the procedure to be reversed, if palsy necessary. 3. Poor or absent Bells phenomenon on the affected 10. Beard and others have advocated bilateral excision of side the levator muscle and bilateral frontalis suspension[12]. Inferior rectus restriction is treated by recession of the inferior While this approach almost completely eliminates the wink rectus muscle. and arguably results in better symmetry, it is often difficult to A combined superior rectus and inferior oblique (double persuade the parents and the patient to perform surgery on and elevator) palsy requires a transposition procedure to displace effectively damage the normal contralateral levator muscle. the medial and lateral recti muscles superiorly (Knapp’s 11. Satisfactory and predictable results also can be obtained

Vol. 21, No. 3, January-March, 2011 DJO 21 Delhi Journal of Ophthalmology Marcus Gunn Jaw-Winking Phenomenon : A Review after only unilateral levator excision on the affected side, 10. Doucet TW; Crawford JS. The quantification, natural course combined with bilateral frontalis suspension (Callahan) [24]. and surgical results in 57eyes with Marcus Gunn (Jaw-Winking) This leaves the normal functioning levator muscle to elevate Syndrome. Am J Ophthal 1981; 92: 702-707. the nonptotic eyelid in primary position but produces a lag in 11. Mauriello JA, Wagner RS, Caputo AR et al. Treatment of downgaze for improved symmetry. congenital ptosis by maximum levator resection. Ophthalmol; 12. Kersten et al advocate unilateral levator muscle excision 1986:93; 466-9. and frontalis sling only on the affected side[21]. If the 12. Doucet TW; Crawford JS. The quantification, natural course postoperative result is judged to be unsatisfactory, the parents and surgical results in 57eyes with Marcus Gunn (Jaw-Winking) or the patient can opt for further surgery to the contralateral Syndrome. Am J Ophthal 1981; 92: 702-707. side. Any amblyopia and strabismus should first be addressed, 13. Beard C. Ptosis, 3rd ed. St. Louis, CV Mosby; 1981: Pg 76- as there may be insufficient drive to lift the disinserted eyelid. 143,150-74,184 207. 13. Islam et al described a technique of dissecting a frontalis 14. Pratt SG, Beyer CK, Johnson CC. The Marcus Gunn flap hinged superiorly through a suprabrow incision that is then phenomenon. A review of 71 cases. Ophthalmology. brought down into an eyelid crease incision[22]. The frontalis 1984;91(1):27-30 flap is used to suspend the ptotic eyelid after extirpation of the 15. Duke Elder S: Normal and abnormal development; congenital levator muscle. deformities. In: System of Ophthalmology. Vol 3, pt 2. St. 14. Lemagne and Neuhaus described techniques that involve Louis: CV Mosby; 1963:900-5. transection of the involved levator followed by transposition 16. Bowyer JD, Sullivan TJ. Management of Marcus Gunn jaw of the distal segment to the brow, which effectively suspends winking . Ophthal Plast Reconstr Surg. 2004; the eyelid to the frontalis muscle[7,23]. Their techniques 20(2):92-8. maintain normal eyelid contour, as the levator aponeurotic 17. Putterman AM. Jaw-winking blepharoptosis treated by attachments are left undisturbed. the Fasanella-Servat procedure. Am J Ophthalmol. 1973; 75(6):1016-22. References 18. Bullock JD. Marcus-Gunn jaw-winking ptosis: classification 1. Gunn RM. Congenital ptosis with peculiar associated and surgical management. J Pediatr Ophthalmol Strabismus. movements of the affected lid. Trans Ophthal Soc UK. 1883; 1980;17(6):3759 3:283-7. 19. Epstein GA, Putterman AM. Super-maximum levator resection 2. Demirci H, Frueh BR, NelsonCC: Marcus Gunn Jaw-Winking for severe unilateral congenital ptosis. Ophthalmic Surg. 1984; Synkinesis: Clinical Features and Management: Ophthalmology 15(12):971-9. ,Feb2010. 20. Dryden RM, Fleming JC, Quickert MH. Levator transposition 3. Park DH, Choi WS, Yoon SH. Treatment of jaw winking and frontalis sling procedure in severe unilateral ptosis and syndrome. Ann Plast Surg 2008; 60(4): 404-9. the paradoxically innervated levator. Arch Ophthalmol. 1982; 4. Khwarg SF, Tarbet KJ, Dortzbach RK, Lucarelli MJ. 100(3):462-4. Management of moderate to severe Marcus Gunn jaw-winking 21. Kersten RC, Bernardini FP, Khouri L, et al. Unilateral frontalis ptosis. Ophthalmology 1999; 106(6): 1191-6. sling for the surgical correction of unilateral poor-function 5. Barthowski SB, Zapata J, Wyszynska. Management of MG ptosis. Ophthal Plast Reconstr Surg. 2005; 21(6):412-6; ptosis in 19 patients. J Craniomaxillofac Surg 1999; 27(1): 25- discussion 416-7. 9. 22. Islam ZU, Rehman HU, Khan MD. Frontalis muscle flap 6. Morax S, Mimoun G. Surgical treatment of MG syndrome. advancement for jaw-winking ptosis. Ophthal Plast Reconstr Ophthalmologie 1989; 3(2): 160-3. Surg. 2002; 18(5):365-9. 7. Neuhaus RW. Eyelid suspension with transposed LPS muscle. 23. Lemagne JM. Transposition of the levator muscle and its Am J Ophthalmol 1985; 100(2): 308-11. reinnervation. Eye. 1988; 2 (Pt 2):189-92. 8. Dillman DB, Anderson RL. Levator myomectomy in synkinetic 24. Callahan A. Correction of unilateral blepharoptosis with ptosis. Arch Ophthalmol 1984; 102(3): 422-3. bilateral eyelid suspension. Am J Ophthal 1972; Vol-74; Pg 9. Pratt SG, Beyer ,CK Johnson CC. The Marcus Gunn phenomenon 321-326. : A retrospective review of 71 cases. Ophthalmology 1984;90:27- 30.

DJO Vol. 21, No. 3, January-March, 2011 22 Delhi Journal of Ophthalmology

Major Review Review of Doses of Important Drugs in Ophthalmology Yogesh Bhadange, Bhavin Shah , Brijesh Takkar, Rajesh Sinha Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India.

Introduction B. Antifungal Agents Dispensing medications in the correct concentration and dosage plays a vital role in the outcome of most ocular pathologies. This article mainly describes about the preparation and doses of commonly used ocular medication in its most simplified form.

1. Topical Drugs A. Fortified Antibiotics Cefazolin or ceftazidime (50mg/ml) • Add 9.2ml of artificial tears to a vial of cefazolin, 1gm (powder for injection). • Dissolve and take 5 ml of this solution and add it to 5 ml of artificial tears. • Refrigerate. Shake well before use.

Tobramycin or gentamycin (14 mg/ml) • Withdraw 2 ml of tobramycin or gentamycin injectable vial (40mg/ml) • Add 2 ml to a tobramycin or gentamycin ophthalmic solution (5 ml) to give 14 mg/ml solution.

Vancomycin (15mg/ml; 25mg/ml; 50mg/ml) • Add 33ml of 0.9% NaCl or artificial tears to 500 mg vial to get 15mg/ml solution. • Add 20ml of 0.9% NaCl or artificial tears to 500 mg vial to get 25mg/ml solution • Add 10ml of 0.9% NaCl or artificial tears to 500 mg vial to get 50mg/ml solution C. Antiviral Agents

Amikacin (40mg/ml) • Direct use of commercially available solution.

Trimethoprim - Sulfamethoxazole(16mg/ml - 80mg/ml) • Direct use of commercially available solution

Vol. 21, No. 3, January-March, 2011 DJO 23 Delhi Journal of Ophthalmology Review of Doses of Important Drugs in Ophthalmology D. Immunosuppresive Agents

E. Autologous Serum Solution from each combination above withdrawn in separate Allow 5 ml of patient’s blood sample to clot in a test tube syringes and mounted on a diplojet injector. When the for 30 minutes. Centrifuge the serum that separates at 1500 – common plunger is depressed, the fibrin sealer solution and 2000 rpm for 10 - 15 minutes. Dilute 1 ml of supernatant with the thrombin solution are combined in the nosecone, in equal 4 ml of normal saline to get final concentration of 20%. volumes, to form the resulting fibrin sealant that is directly applied to the designated tissues. F. Contents Of Pharmacy Prepared Artificial Tears: • Hydroxyl Propyl Methyl Cellulose0.3%; 4+5 (Thrombin component; rapid release) – used in vascular • glycerine0.4%; and neurosurgeries. • sodium chloride0.4%; • thiomersal 0.005% (preservative). 2. Subconjuntival A. Antibiotics G. Hyperosmotic Agents Dilute 5 gram / 6 gram of commercially available sodium chloride in upto 100 ml of distilled water to give 5% / 6% hypersol respectively.

H. Glue Tisseel VH Fibrin sealant (Baxter AG, Vienna, Austria) 1. Large Blue Bottle: • Clottable protein - 75 to 115mg Subconjunctival drugs injected post cataract surgery: • Fibrinogen - 70 to 110mg • Gentamycin – 0.4 ml (20 -40 mg/ml) • Plasma fibronectin - 2 to 9 mg • Dexamethasone – 0.4 ml (4 mg/ml) • Factor XIII - 10 to 50 IU • Xylocaine – 0.2 ml (20 mg/ml) • Plasminogen - 40 to 120 mg (microgram) 2. Small Blue Bottle: Aprotinin solution, bovine 3000 KIU / B. ANTI-VEGF (Bevacizumab) ml Bleb Rescue: 1.25 mg / 0.05 ml subconjuctivally close to bleb. Corneal Vascularisation: 2.5 mg / 0.1 ml subconjuctivally 3. White Bottle: Thrombin 4 (bovine), freeze dried close to the limbus in the quadrant of vascularisation. reconstituted contains 4IU/ml C. Immunosuppressive Agents: 4. Large Black Bottle: Thrombin 500 (bovine), freeze dried Mitomycin C: applied subconjunctivally using specialised reconstituted contains 500 IU/ml sponges such as Meroseal in conc. of 0.02% and 0.04%. Diluting 2mg of Mitomycin C in 10 ml / 5 ml of Distilled 5. Small Black Bottle: Calcium chloride solution, 40mmol/L water gives 0.02% and 0.04% respectively. 1+2 (Fibrin component) 5 – Fluorouracil: injected subconjunctivally in a conc. of 50 mg/ml to prevent or treat a failing bleb. Bottles 1, 2, 3 and 5 are warmed for 30 minutes in Fibrotherm device. D. Cycloplegic – Mydriatics: Atropine sulphate (0.6 mg/ml Add 1 + 2 (Fibrin Sealer Solution); Resultant is warmed and vial)-0.2 ml subconjunctival dose. stirred. Add 3+5 (Calcium - Thrombin component; slow release) – 3.Intrastromal used in Ophthalmology. A. Antifungal Agents:

DJO Vol. 21, No. 3, January-March, 2011 24 Review of Doses of Important Drugs in Ophthalmology Delhi Journal of Ophthalmology Voriconazole Acetate 50microgram/0.1ml A cortisone, synthetic analogue of cortisol acetate with no Amphotericin B glucocorticoid activity; acts by suppression of extracellular 5-7.5microgram/0.1ml proteases elaborated by activated endothelial cells and also decreases VEGF level. Dose-15mg posterior juxta scleral B. Antibiotics: depot injection every 6 months for treatment of wet ARMD. Ciprofloxacin - 0.3mg/0.1ml. 5. Intravitreal Drugs C. Antiproliferative Agents A. Antibiotics Mitomycin C – for reducing the risk of postoperative Vancomycin (1mg\0.1ml) – available commercially as 500 corneal haze after surface or stromal ablation; applied at a mg powder. concentration of 0.2mg/ml (0.02%) for 12 to 120 seconds. Mitomycin C is also used intraoperatively in excision in concentration of 0.2mg/ml (0.02%) to 0.4mg/ ml(0.04%) for 5 min over the exposed stroma and the .

4. Intracameral Agents A. Antibiotics Ceftazidime (2.25mg\0.1ml) – available commercially as Prophylactic as well as Therapeutic uses. 500mg powder injection Bolus dose or maintenance dose in the irrigating fluid. Bolus dose of commonly used antibiotics

Amikacin (400microgram\0.1ml) – commercially available as 100mg in 2ml or 50 mg/ml. Antibiotics used in irrigating fluids

Gentamycin (200microgram\0.1ml) : commercially available as 80mg in 2ml or 40mg in 1ml

B. Mydriatics Adrenaline tartarate (0.1% w/v; 1:1000) is diluted ten times ie.0 .1 ml of adrenaline diluted in 0.9ml of BSS ;used as bolus dose. Dexamethasone (400microgram\0.1ml): Each 2ml vial For maintaining 0.8cc adrenaline tartarate (0.1% contains 8mg w/v 1:1000) is diluted in 350 ml of BSS. C. Miotics: Pilocarpine 0.1 ml of the drug (25mg/ml) is diluted in 0.1ml ringer lactate; B. Antifungal used as bolus dose. Amphotericin B 0.8ml of pilocarpine (0.5% of ophthalmic preparation) is (5 microgram\0.1ml) – available commercially as 50mg added to 350 cc of BSS; used as maintenance dose. powder Posterior Subtenon Injection 20mg / 0.5ml of triamcinolone acetonide either with benzyl alcohol preservative (kenacort\ tricot) or as preservative free preparation (retilone\ aurocot) is injected using the Smith and Novak’s technique with 26 Gauge needle or the cannula technique. Posterior Juxta Scleral Depot Injection of Anacortave

Vol. 21, No. 3, January-March, 2011 DJO 25 Delhi Journal of Ophthalmology Review of Doses of Important Drugs in Ophthalmology Voriconazole – available commercially as 200mg powder or Dexamethsone intravitreal implant - Porsudex (Ozurdex) 50-100micrgram\0.1ml Dexamethasone 350 mcg or700 mcg biodegradable implant (small pellets); lasts for 37 days; can be injected as an office procedure.

Fluocinolone acetonide intravitreal implant– (Retisert) 0.59 mg non-biodegradable implant injected through a pars plana incision; designed to release fluocinolone acetonide at initial rate of 0.6 µg/day; decreasing over the first month to a steady state between 0.3-0.4 µg/day over approximately 30 Fluconazole months. Intravitreal: 25 microgram/0.1 ml The implant consists of a tablet encased in a silicone elastomer cup containing a release orifice and a polyvinyl alcohol C. Antiviral Drugs membrane positioned between the tablet and the orifice. The Acyclovir silicone elastomer cup assembly is attached to a polyvinyl Intravitreal dose: 10 to 40 micrograms/0.1ml alcohol suture tab with silicone adhesive. Each RETISERT is Gancyclovir approximately 3 mm x 2 mm x 5 mm.Each implant is stored used mainly in acyclovir resistant cases; Induction dose in a clear polycarbonate case within a foil pouch within a -2 mg/0.1ml; 0.1 ml injected 2 times per week for 3 wks; peelable overwrap. Each packaged implant is provided in a Maintenance dose- 2mg/0.1 ml once a week carton which includes the package insert. Gancicovir implant (Vitrasert) Provides local sustained conc. of the drug with decrease risk E. ANTIVEGF of systemic SE without repeated injections. Therapeutic levels Pegatinib sodium (Macugen) up to 8 months.4.5 mg drug in 2.5 mm pellet completely Chemically synthesized single strand of nucleic acid, coated by drug permeable poly vinyl alcohol and incompletely complementary to RNA (APTAMER).Molecular weight of coated with impermeable ethyl vinyl acetate. Releases drug at 50 kilo daltons. Selective vegf 165 inhibitor. rate of 1 micro gm/hr. Dose Mean intravitreal concentration achieved is 4.1 microgm/ ml. 0.3 mg/0.09ml intravitreally every 4- 6 wk.T 1/2-in vitreous Valgancyclovir is 83 to 94 hrs Prodrug; Dose - 0.2 to 0.4 mg/0.1ml twice weekly till disease regresses. Bevacizumab (Avastin) Foscarnet Nonselective recombitant humanized chimeric anti VEGF Virustatic; Used in cases of resistance to gancyclovir; 1.2 mg antibody.Produced in chinese hamster ovary. Molecular in 0.05 ml injected intravitreally. weight of 150 kilo daltons thus cannot cross retinal ILM and Induction Dose RPE into subretinal space. Two injections once per week for 3 weeks. Maintenance dose Dose one injection per week. 1.25mg/0.05ml intravitreally; T ½: 20 days. Each bevacizumab Cidofovir vial is available in 4ml and 16ml vial in conc. of 25mg/ml 20mg / 0.1ml every 6 weekly. Oral probencid 2gm before injection and 1 gm after post injection Ranibizumab(Lucentis) Fomiversen Recombinant monoclonal fragment nonselective antibody 330 μg intravitreal every 2 weekly for 2 doses for induction. .Molecular weight 48 kilo daltons. Nonselective VEGF Maintenance dose: 330 μg intravitreally / month; 4th line drug antibody. treatment of CMV Dose 0.3-0.5mg/0.05ml.each lucentis carton contain 0.2ml.T1/2 - D. Steroids 10 days. Triamcinolone acetate 1 mg / 4 mg intravitreal injection; available most commonly as siRNA (bevaciranib) a suspension of triamcinolone crystals containing preservative Small interfering RNA cause elective degeneration of mRNA benzyl alcohol 0.99% (Kenacort 40 mg/ml and Tricort 40 mg/ transcript containing a sequence homologous to the siRNA ml,) and also as preservative free preparation (Triesence, (VEGF) thereby blocking the synthesis of VEGF. Dose: Retilone, Aurocot – 40 mg/ml) 70/150/300 microgram in 0.05 ml (under trial).

DJO Vol. 21, No. 3, January-March, 2011 26 Review of Doses of Important Drugs in Ophthalmology Delhi Journal of Ophthalmology VEGF trap Indications Contains Ig domain of VEGF Receptors 1 and 2; High affinity • Uveitis binding and neutralisation of • Behcet’s disease receptors. Dose: 0.05 to 4 mg intravitreally single injection at • intervals of 6 wks. (under trial) • Orbital inflammatory disease • Non-responding posterior uveitis F. Cyclosporine • Graft rejection Impairs T - Cell mediated immunity; used as steroid sparing agent Dose 2mg intravitreal implant G. Intraocular Gases

References 4. Saravia M, Zapata G, Ferraiolo P, Racca L, Berra A .Anti- 1. G Geerling, S MacLennan, D Hartwig. Autologous serum eye VEGF monoclonal antibody-induced regression of corneal drops for ocular surface disorders. Br J Ophthalmology. 2004; neovascularization and inflammation in a rabbit model of issue 88: page 1467–1474. herpetic stromal keratitis. October 2009 ;vol 247:page 1409-16. 2. Anita Panda, Sandeep Kumar, Abhiyan Kumar, Raseena 5. Safety and efficacy of intravitreal triamcinolone for cystoid Bansal, Shibal Bharti. Fibrin glue in ophthalmology. Current macular oedema in uveitis. Young S,LarkinG., Branley ophthalmology. 2009; vol 57; issue 5: page 371-379. M,Lightman S.Clinical & Experimental Ophthalmology. 3. Duch,Susana, Buchacra, Oscar, Milla, Elena, Andreu, February 2001; vol 29; issue 1: page 2–6. David,Tellez, Jesús. Intracameral Bevacizumab (Avastin) for Neovascular Glaucoma. Journal of Glaucoma. February 2009 ;vol 18 ; issue 2 : page 140-143

Vol. 21, No. 3, January-March, 2011 DJO 27 Delhi Journal of Ophthalmology

Preferred Practice Pattern Systematic Approach to a Case of Disc Pallor

Digvijay Singh, Rohit Saxena, Pradeep Sharma, Vimla Menon Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India

Disc pallor is often the sequel of various disorders and signifies partial or total optic atrophy. It may be present with symptoms or signs ranging from subtle visual dysfunction to total blindness or may even be an incidental finding during routine ophthalmological checkup. Discovering the cause of such a case can baffle neurologists and ophthalmologists alike. We describe a systematic clinically oriented approach to a case of disc pallor to help arrive at a diagnosis.

Introduction Clinical Approach An ophthalmologist is frequently faced with pallor The clinical approach to a case of disc pallor is akin to on fundoscopy and may be perplexed regarding how to solving a jigsaw puzzle. With every piece put in its place, the approach the case and identify the etiology behind this clinical picture becomes clearer. The systematic process of arriving presentation. In this paper, we describe an approach to disc at a diagnosis should begin with clues from the demographic pallor and clues to discover its etiology. profile of the patient, followed by a directed clinical history. This should precede an ocular and systemic examination Disc pallor is the manifestation of partial or total optic atrophy guided by the history. Investigations including neuro-imaging and is a consequence of loss of nerve fibers. Optic atrophy would finally establish the etiology[2,3,4]. The protocol has classically been described into primary and secondary and clinical interpretations are discussed in the appropriate types. Primary optic atrophy is secondary to a lesion affecting headings below. the visual pathway from the optic nerve head to the lateral geniculate body. The disc in such cases is flat and pale with Demographic profile clearly demarcated margins. Disc edema precedes secondary The age, gender and race of a patient is often the first clue to optic atrophy which presents with a dirty white to grey looking the diagnosis, but has to be interpreted with caution. disc with poorly delineated margins. Age The etiology of unexplained disc pallor can be revealed by The age is probably the most important of the demographic appropriate investigations in a large majority of cases. This parameters while short listing possible etiologies of disc was demonstrated in a multicentre study where of all cases pallor. A brief list of various disorders and the presenting age of optic atrophy only 8% remained unexplained. Further is below but one has to understand that there are no watertight directed investigations including neuro-imaging led to am compartments and significant overlap of diagnosis exists etiological diagnosis in another 20% of these cases. The between each age group. Glaucoma can present with disc authors had recommended use of neuro-imaging in all cases pallor at any age and has been deliberately omitted from the of unexplained optic atrophy[1]. list and further discussion. The table depicts the causes for The need for a definitive diagnosis in any case of disc pallor disc pallor categorized by age group of presentation. stems from the fact that optic nerve diseases behave in a very varied manner while carry different treatments and outcomes. Gender Some disorders such as are self limiting but may This guides us in favoring one diagnosis over another but one be recurrent whereas others like toxic neuropathies are partially has to be aware that there is no clear segregation of optic nerve reversible. Hereditary optic atrophies may be progressive and afflictions on gender. Male: LHON, Traumatic neuropathy, with rare exception, do not show improvement. An Ischemic some tapeto-retinal degenerations, toxic neuropathy( such as arteritic AION can rapidly involve lead,arsenic heavy metal occupational exposure, methyl the fellow eye if not treated on time. Damage to nerve in toxic alcohol), Nutritional deficiency(chronic alcoholism).Female: optic neuropathy can be halted by removing the offending multiple sclerosis, meningioma, autoimmune/collagen agent. vascular diseases, Sheehan syndrome, ecclampsia,

DJO Vol. 21, No. 3, January-March, 2011 28 Systematic Approch to a Case of Disc Pallor Delhi Journal of Ophthalmology

Race in toxic/nutritional deficient neuropathy, hereditary The type of optic neuropathy and severity may demonstrate neuropathy, atypical optic neuritis and arteritic anterior a ethnic variation. For example, blacks were found to have ischemic optic neuropathy. lower incidence of ischemic optic neuropathy than whites • Ocular history such as episodes, painful eye and had lower incidence of severe visual loss secondary to movements(retrobulbar optic neuritis), and idiopathic intracranial hypertension than whites.5 Caucasians proptosis are more likely to be afflicted with multiple sclerosis and • Systemic history such as fever, jaw claudication, optic neuritis than Asians or Hispanics. Overall optic atrophy palpitations, breathlessness and symptoms suggestive is more prevalent in African-Americans (0.3%) than whites of systemic illness like diabetes, hypertension, (0.05%). hyperthyroidism, recent viral infection, tuberculosis etcetera. Clinical History • History of CNS disease in form of headache, meningitis, Chief complaints seizures, projectile vomiting, Transient ischemic Blurring or decrease of vision, pain on eyeball movement, attacks, motor weakness, paraesthesia and other sensory inability to see part of visual field symptoms etcetera. • History of trauma Clinical picture and interpretation • Occupational and social history with special emphasis • Onset Acute onset (over hours to days) is seen in optic on alcohol/drug intake, smoking, diet and unsafe sexual neuritis, ischemic optic neuropathies and traumatic optic practices. neuropathy. Sub-acute onset (over few days) is seen • Family history is important to diagnose hereditary in non demyelinating inflammatory neuropathy and neuropathy. compressive neuropathy. Insidious onset is characteristic • History of previous medications guides us regarding the of toxic and nutritional deficiency neuropathy as well as possibility of toxic neuropathy. hereditary neuropathies. • Course If the symptoms had self resolved without any Ocular Examination intervention or recurrent episodes of decrease of vision A complete and thorough ophthalmic examination is are present, a previous demyelinating pathology should mandatory. The neuro-ophthalmology specific ocular be suspected. On the other hand a residual poor vision examination includes- after an episode or progressive deterioration or protracted • Visual acuity Typically disc pallor secondary to optic course should point towards any of the other pathologies. neuritis, LHON, Nutritional deficiency optic neuropathy, • Laterality Unilateral disease is found in typical NAAION and inflammatory neuropathies presents optic neuritis, ischemic neuropathy (Non-arteritic), with a visual acuity of around 20/200. Hereditary optic traumatic neuropathy and compressive lesions. Bilateral neuropathies (AD/AR), AAION, post and presentation (though may be asymmetrical) is observed traumatic optic neuropathies on the other hand present

Vol. 21, No. 3, January-March, 2011 DJO 29 Delhi Journal of Ophthalmology Systematic Approch to a Case of Disc Pallor with a poor visual acuity and even absence of light the normal subjects. A deep excavated cup is of more perception. Toxic optic neuropathies have a variable and significance compared to a large shallow cup. The size of unpredictable visual acuity. a cup can also be measured similar to that described for • Visual fields Confrontation visual fields can give a the disc above. Both direct and indirect ophthalmoscopy quick assessment of any large or hemi field or underestimates the cupping in comparison to slit lamp altitudinal defects. A carefully done confrontation field biomicroscopy. test and a tangent screen test with a red target provide a  Neuroretinal rim : This is a congregation of nerve fibers fairly accurate result. from the retina converging upon the disc to form the • Color desaturation test The patient may be shown a optic nerve. A pale disc secondary to a neuro-ophthalmic red capped bottle and asked to compare the red color disease often has a uniform thin neuroretinal rim but there separately with both eyes. The eye with disc pallor would is no focal notching or loss in contrast to glaucoma. give a washed out red or pink appearance in contrast to  Disc Margins : These should be well defined. In disc the bright red color seen by the fellow eye. edema they are usually blurred in the INST sequence. • A relative afferent papillary defect is Often one may notice a pallid disc edema in circulatory characteristically found in cases of disc pallor though it compromise of the disc. LHON may present with is absent in bilateral symmetrically affected cases. RAPD pseudoedema. can be quantitatively assessed using neutral density filters  Disc Vascularity : Kestenbaum count is the number of for comparison of any future worsening of neuropathy. capillaries observed on the disc. The normal number is 10 • Macula/Posterior pole One needs to look for presence while optic atrophy will have a count of less than 6. of exudates in the form of a star or fan or sequel thereof.  Peripapilla : Presence of peripapillary atrophy (alpha Occasionally an optic disc pit may be associated with zone and beta zone) needs to be documented. central serous choroidoretinopathy or a choroidal  Retinal nerve fibre layer : The presence of any RNFL neovascular membrane. defects should be noted. This is best examined with a red • Disc[6] free green filter.  Size This may be measured by various techniques using The appearance of the disc can give a clue about the possible a direct ophthalmoscope (use 50 cone of Welch Allen etiology[7]. Some diagnostic categories and the corresponding ophthalmoscope), indirect ophthalmoscope or on slit disc appearance are as follows: lamp biomicroscopy (When using a 90D lens multiply Ischemic Optic Neuropathy the height of the slit measured in mm by 1.3 when it 1. Retinal arterial attenuation and sheathing is focused and just equal to the disc to get disc size in 2. Pallid disc edema mm). The importance of disc size comes when a case of 3. Superior or inferior disc pallor optic disc hypoplasia is confused with a disc pallor post 4. Disc hemorrhages neuropathy. 5. Fellow eye may show small disc with absent cup( disc at  Shape : The disc normally appears round or oval. Any risk) variation from this should alert towards a congenital Optic Neuritis anomaly or traumatic avulsion. 1. Typical : Normal disc or mild temporal pallor (Unilateral)  Color : The disc is normally salmon pink in color 2. Atypical: Pallid disc edema or diffuse or bilateral disc though the actual color varies from race to race. A disc is pallor with arterial attenuation and disc hemorrhages. described as pale if it loses the pink hue to turn towards a whitish yellow color or a lemon tint. It is described as hyperemic if it becomes reddish pink (a sign of increased vascularity). There are methods described in literature to objectively or subjectively assess and document the color of the disc. These involve recording ophthalmoscopic appearances and digital stereoscopic disc images followed by analyzing them. One should establish their own protocol and document progression of disc pallor during follow up visits. A word of precaution which merits mention is that the 90D lens may make the disc look falsely healthier(less pale) due to its the yellow tint.  Cup : Disc Ratio: This shows great variability and can range from no visible cupping to 0.6 and beyond in Figure 1: Bowtie optic atrophy

DJO Vol. 21, No. 3, January-March, 2011 30 Systematic Approch to a Case of Disc Pallor Delhi Journal of Ophthalmology Hereditary optic neuropathy (AD/Non Leber’s) 1. Disc pallor is usually subtle, mostly temporal and occasionally diffuse 2. Non-glaucomatous cupping with peripapillary atrophy and/or arterial attenuation is noted. Lebers Hereditary optic neuropathy 1. Sequential bilateral presentation 2. Temporal disc pallor with RNFL defects in papillomacular bundle 3. Non-glaucomatous cupping with arterial attenuation 4. Fellow eye may show disc at risk picture and during acute vision loss stage may demonstrate pseudoedema with disc hemorrhages and peripaillary telangectasias. Optic Chiasm/Tract lesion Figure 2: Post papilledemic optic atrophy 1. Bilateral involvement 2. Bow tie atrophy of contralateral disc (Superior and inferior sparing) Compressive neuropathy 1. Unilateral or bilateral disc edema and/or disc pallor 2. Painless progressive disc pallor on follow up 3. Optico-ciliary shunts/disc collaterals Radiation neuropathy 1. Rapidly developing disc pallor (4 wks) 2. Usually disc edema not noted at any stage 3. Associated with radiation ( like findings) Traumatic neuropathy 1. Anterior lesions show early ophthalmoscopic findings Figure 3: Primary optic atrophy such as in optic nerve avulsion and disc edema with early onset disc pallor(3-4 weeks) 2. Posterior lesions show late onset disc pallor with no other fundus change Papilledema 1. A dirty grey-white optic pallor with ill defined disc details and margins are the typical signs of a secondary optic atrophy following long standing pailledema. 2. The disc may show an excavated appearance.

Systemic Examination A detailed systemic examination forms an important part of any neuro-ophthalmology workup. The key factors looked for are: 1. General demeanor to look for nutritional deficiency or Figure 4: Hemi atrophy post AION chronic illness Infectious/Infiltrative neuropathy 2. Pulse to look for any cardiovascular signs. 1. Usually following an episode of unilateral or bilateral 3. Blood pressure, specifically for ischemic optic neuropathy disc edema with macular star 4. Conjunctival pallor for anemia which may provide 2. Disc pallor is typically subtle and diffuse evidence of nutritional deficiency Toxic/Nutritional Optic Neuropathy 5. Icterus to rule out systemic illness causing jaundice 1. Temporal Disc pallor and neuropathy such as hepatitis, syphilis, viral 2. Usually symmetric bilateral but may show significant infections,leptospira, malignancy etc. Also to suspect asymmetry in early stages. toxic neuropathy.

Vol. 21, No. 3, January-March, 2011 DJO 31 Delhi Journal of Ophthalmology Systematic Approch to a Case of Disc Pallor 6. Lymphadenopathy, seen in diseases such as tuberculosis, 7. VDRL (syphilis serology) Collagen vascular diseases, syphilis, viral infections, 8. Lumbar puncture malignancy etc. Specific tests based on suspected cause 7. Specific systems including cardiovascular, respiratory, 1. Sarcoidosis: Serum ACE, serum Calcium levels, Gallium gastrointestinal, skin and musculoskeletal to rule out scan ischemic heart disease, arrhythmias, major vessel 2. Collagen Vascular disease: Immunology workup; ANCA, obstructions, tuberculosis, sarcoidosis, malignancy, Anti-dsDNA, RF, Complement levels collagen vascular diseases, viral exanthems, syphilis etc. 3. Tuberculosis: Sputum smear AFB, Quantiferon Gold 8. Central and peripheral nervous system examination to assay, Lymph node FNAC/biopsy look for any associated neurological diseases including 4. Nutritional deficiency neuropathy: Serum B12 and Folate but not limited to meningitis, encephalitis, multiple levels sclerosis, neuromyelitis optica, Friedriech ataxia and 5. Toxic neuropathy: Heavy metal screening malignancy. 6. Post infectious optic neuritis: TORCH titers On the basis of the history, ocular and systemic examination, 7. Hereditary neuropathy : LHON mutation testing one should create a list of differential diagnosis and proceed (preferably full mitochondrial sequencing or at least the towards investigations. common primary mutations) Ocular Investigations 8. Paraneoplastic syndrome: Antibody profile such as The ocular investigations should be done at baseline and CRMP5 for small cell CA of lung appropriate follow up visits. The follow up protocol can 9. Thyroid : Serum T3, T4 and TSH levels. be tailored by each institution on the basis of patient load 10. Devic’s disease: MRI spine, Anti NMO antibody titer. and human resource availability. We recommend these 11. AAION: Temporal artery biopsy, CRP levels investigations on first visit and at 1 month follow upto Conclusion establish a baseline. Then, they may be repeated once at 3 A systematic approach to a case of disc pallor will result in months and 6 monthly thereon. The investigations that should a definite diagnosis in majority of the cases without undue be done in a case of disc pallor include loss of time or resources. Nonetheless, there are still cases 1. Color vision (Ishihara or HRR plates). Note both the which remain unexplained and for them, the ophthalmologist, number and type of unread plates. on his part can keep a close follow up initially to ensure a 2. Contrast sensitivity: Pelli Robinson or FACT non progressive condition. While visual prognosis remains 3. Visual fields: Both Goldmann/Humphrey visual fields. guarded for patients with disc pallor at present, the future 4. Color and red free fundus photograph centered on the holds promise with the advent of stem cell therapy, potential disc. for optic nerve transplantation and fast progressing technology 5. Flourescein fundus angiogram needs to be undertaken for artificial vision. only if specific indication. References 6. Optical coherence tomography of the optic disc to 1. Lee AG, Chau FY, Golnik KC, Cardon RH, Wall M. The document nerve head and retinal nerve fiber status. diagnostic yield of the evaluation for isolated unexplained optic 7. Electrophysiology: Visual evoked reponse (Preferably atrophy. Ophthalmology 2005:112:757-59. pattern, if possible or else flash), Electroretinogram 2. Behbehani R. Clinical approach to optic neuropathies. Clin and Electrooculogram to look for any associated retinal Ophthalmol. 2007:1(3):233-46. dysfunction. 3. Lee AG, Brazis PW (eds). Clinical pathways in Neuro- Systemic Investigations Ophthalmology: An Evidence based approach. Thieme Medical The systemic investigative lineup for a case of disc pallor Publishers. 2003:1-35. may be divided into first line investigations and second line 4. Brazis PW, Masdeu JC, Biller J(eds).Localization in clinical or specific testing. neurology. Lippincott Williams & Wilkins. 2007:131-69. First line tests 5. Mansoui AM, Hamed LM. Racial variation of optic nerve 1. Hemogram with peripheral smear, Complete blood count disease. Neuro-Ophthalmology.1991:11:319-23. with differential count 6. Teymoorian S. Examination of the optic nerve at the slit lamp 2. Liver function test biomicroscope with a handheld lens. http://eyewiki.aao.org. 3. ESR Accessed on 2nd January 2011. 4. Mantoux test 7. O’Neill EC, Danesh-Meyer HV, Connell PP et al. The optic 5. Chest X-ray nerve head in acquired optic neuropathies. Nat rev neurol. 6. MRI of Head and with thin cuts and fat suppression 2010:6(3):221-36.

DJO Vol. 21, No. 3, January-March, 2011 32 Delhi Journal of Ophthalmology

Case Reports Bilateral Mooren’s Ulcer with perforation and Iris prolapse Uday Gajiwala1, Jyotsom Ganatra2, Rajesh Patel1, Parin Shah1, Rohan Chariwala1 1. SEWA Rural, Jhagadia-393110, Dist.-Bharuch, Gujarat, INDIA, Phone-(02645)220021, 2. Department of Ophthalmology, Kaiser-Permanente Medical Center, San Rafael, California 94903- USA

Mooren’s Ulcer is an idiopathic, rapidly progressive, painful peripheral ulcerative keratitis with no associated scleritis. It’s a diagnosis of exclusion which means all other diagnosable systemic disorders that could be responsible for the progressive destruction of must be ruled out. The etiology of Mooren’s Ulcer remains uncertain. However, recent studies indicate that it is an autoimmune disease directed against a specific target molecule in the corneal stroma, triggered in genetically susceptible individuals by one of several possible mechanisms. A 40 years old female presented to us on 25th January 2008 from rural tribal area of Sagbara with bilateral peripheral ulcerative keratitis with perforation and iris prolapse in right eye. She is known case of diabetes mellitus for last seven years. Diagnosis of Mooren’s ulcer was made after she underwent extensive medical and laboratory testing to rule out an infectious or systemic cause of corneal melt. She benefited by right eye patch graft, left eye tissue adhesive with BCL application, Both eyes conjunctival peritomy and immunosuppressive drugs along with control of diabetes. This case report aims to highlight the diagnosis and treatment of Mooren’s ulcer.

Case Report uncontrolled diabetes mellitus on the day of presentation. A 40 year old female from rural tribal area of Sagbara with low Investigations done – Hemogram with ESR, Urine routine and socio economical status presented to us on 25th January 2008 microscopy, VDRL, RA factor, HCV, X- ray chest and Joints, with complaint of decreased vision, redness, watering, ocular SGPT, ANCA, ANA, HBsAg, Scraping of the ulcer are done, pain and in both eyes for last 2 months more so in which were normal. right eye for last one week. She was having diabetes mellitus for last seven years which was controlled by medication. There She was referred to her physician to control diabetes mellitus was no history of trauma, joint pain. as well as to rule out systemic disorders causing peripheral ulcerative keratitis. She was placed on Insulin Injections to On examination, best corrected visual acuity in right eye was control DM. 6/36 and in left eye, it was 6/24. There was inferior crescent of corneal thinning involving 6 clock hours (3-9 o’clock) with After excluding systemic diseases associated with peripheral limbal involvement but no associated scleritis. In right eye, there ulcerative keratitis, A diagnosis of Bilateral Mooren’s ulcer was was a perforation with Iris prolapse at 6-7 o’clock position. In made and systemic immunosuppressive therapy was started both eyes, there was overhanging edge infiltrated with white with oral methotrexate 10 mg once a week and oral systemic cells and spreading towards centrally and circumferentially. It steroids at a dose of 1.5 mg / kg / day. was positive for fluorescein stain indicating overlying epithelial defect. The pupil was peaked inferotemporally in right eye due Right eye Iris abscission (of prolapsed part) with patch graft to perforation and in left eye due to impending perforation. (free Hand) was done after controlling diabetes mellitus. There were no signs of secondary infection and/or Iritis on Tissue adhesive with Bandage Contact Lens Application with examination. Dilated Indirect ophthalmoscopy was normal in conjunctival peritomy was done in left eye. both eyes. (No diabetic retinopathy) Locally she was started on Prednisolone eye drops 2 hourly, Presentation Ofloxacin eye drops 4 times a day, timolol maleate eye drops She was investigated to rule out systemic disease causing twice a day, 2 % HPMC eye ointment three times a day. peripheral ulcerative keratitis. RBS was 301 mg/kg showing Tissue Adhesive and Bandage Contact Lens were removed

Vol. 21, No. 3, January-March, 2011 DJO 33 Delhi Journal of Ophthalmology Bilateral Mooren’s Ulcer with perforation and Iris prolapse

RE-After 6 Months Follow Up: Right Eye:

LE-After 6 Months Follow Up:

Left Eye: Mooren’s ulcer is idiopathic by definition, occurring in complete after 6 weeks in left eye. It was nicely epithelialized and scarred absence of any diagnosable systematic disorder that could be without any overhanging edge. Right eye patch graft is doing responsible for progressive destruction of the cornea with no well after 6 months. associated scleritis.

Best corrected visual acuity in right eye 6/18 and left eye 6/18 Its exact pathophysiology remains uncertain, although a after 6 months follow up. growing body of evidence indicates that it is an autoimmune disease directed against a specific target molecule in the corneal stroma resulting in its destruction by degradative enzymes, Discussion which are released primarily by neurotrophils attracted into the Although the diagnosis of Mooren’s ulceration may be difficult area by diverse stimuli [12,13], probably triggered in genetically when a patient first presents with PUK the clinical appearance susceptible individual by one or several mechanisms. is characteristic. However, a thorough medial history, physical examination and appropriate laboratory investigations must be Wood and Kaufman having reported 9 cases concluded that performed to rule out underlying systemic conditions causing there were two clinical types of Mooren’s ulcer[5]. The first PUK, since Mooren’s Ulcer is a diagnosis of exclusion. limited type, is usually unilateral, with mild to moderate Mooren’s ulcer was first described by Bowman in 1849[1] and symptoms, generally responds well to medical and surgical then by McKenzie in 1854 as “Chronic serpiginous ulcer of treatment. This type is believed to occur in older patients and cornea or ulcus roden”[2]. Mooren’s name, however, became has become known as typical or benign Mooren’s ulcer. In attached to this rare disorder because of his publication of cases contrast, the second type is bilateral, with relatively more pain in 1863 and 1867[3]. He was the first to clearly describe this and generally a poor response to therapy in younger patients, insidious corneal problem and define it as a clinical entity. became known as atypical or malignant, Mooren’s ulcer. The Nettleship[4] summarized the accumulated reported experience benign type is bilateral in 25% of patients and the malignant with the disorder in a classic article. type is bilateral in 75 % of Patients.

DJO Vol. 21, No. 3, January-March, 2011 34 Bilateral Mooren’s Ulcer with perforation and Iris prolapse Delhi Journal of Ophthalmology Keitzman[6] published a series of 37 cases of progressive • Polyarteritis Nodosa Mooren’s ulcer in Nigeria affecting primarily healthy men • Other collagen vascular diseases between age of 20 and 30 yrs and the clinical course was very • Inflammatory bowel disease rapid. Perforation occurred in 36% of the patients. As a result, • Giant cell Arteritis the generalized belief has developed that the progressive and • Staphylococcal Marginal Keratitis relentless atypical form of Mooren’s ulcer has A predilection • Local infections causes for young men of African origin. • Terrien’s degeneration • Pellucid degeneration Lewallen and courtright[7], in their published series of • Ocular Rosacea Mooren’s ulcer, suggest that younger patients had bilateral disease less frequently than older patients (1.5:1) regardless of Treatment race. Although they found that men were 1.6 times more likely Today, most experts agree on stepwise approach in management to have Mooren’s ulcer than were women. of Mooren’s ulcer[19]]. 1. Topical Steroids Different entities have been associated with Mooren’s 2. Conjunctival Resection ulcer, often leading to conjecture that there may be a causal 3. Systemic immuno suppressive relationship. 4. Additional Surgical Procedure 5. Rehabilitation An association with helminthiasis has been suggested in Nigeria[8], Schanzlinp[9] speculated that the antigen antibody Goals reaction to helminth toxin deposited in peripheral cornea To arrest the destructive process and to promote healing and provoked the inflammation and ulceration. Recently in 2 patients re-epithelialization of the corneal surface[15-18]. with bilateral Mooren’s ulcers chronic hepatitis with infection C was documented[10,11] and they improved after treatment Steroids of the hepatitis C with interferon α2β. The authors proposed Initially: Intensive topical steroids that molecular mimicry may be involved, with the hepatitis C Prednisolone acetate 1% eye drop virus stimulating an autoimmune response to corneal antigens One hourly through cross reacting epitopes. In association with Based on the clinical presentation[20] and the low dose anterior segment fluorescein angiographic findings, there seem to be Topical Cycloplegics three distinct varieties of Mooren’s ulceration Prophylactic Antibiotics If epithelial healing does not occur within 2-3 days, the 1. Unilateral Mooren’s ulceration (UM), characterized by an frequency of tropical steroids can be increased to every half excessively painful progressive corneal ulceration in one hour. eye in elderly patients, associated with non perfusion of the Once healing occurs, the frequency can be reduced and tapered superficial vascular plexus of the anterior segment. slowly over a period of several months. 2. Bilateral aggressive Mooren’s ulceration (BAM), which Such management, especially in unilateral, benign form has occurs in young patients, progresses circumferential and met with good results. only later, centrally in the cornea. Angiography shows Topical cyclosporine-A therapy (0.5% solution) also found vascular leakage and new vessel formation which extends useful in some studies. into the base ulcer. 3. Bilateral indolent Mooren’s ulceration (BIM), which Systemic steroids usually occurs in middle aged patients presenting with progressive peripheral corneal guttering in both eyes, with Oral pulse therapy little inflammatory response. There is no change from the 60-100 mg daily of oral Prednisolone is indicated when topical normal vascular architecture on angiography except an therapy is ineffective after 7-40 days. extension of new vessels into the ulcer. When topical steroids may be dangerous because of precariously deep ulcer or infiltrate [[14, 22]. DD Topical tetracycline may be used for anticollagenolytic • Rheumatoid arthritis properties. • Wagener’s Granulomatosis A therapeutic soft contact lens or patching of eye may be

Vol. 21, No. 3, January-March, 2011 DJO 35 Delhi Journal of Ophthalmology Bilateral Mooren’s Ulcer with perforation and Iris prolapse beneficial when ulcer is deep. When a perforation is too large for tissue adhesive to seal the leak, some type of patch graft will be necessary. This may range Conjunctival Resection from a small tapered plug of corneal tissue to a penetrating • If ulcer progresses despite steroid regimen, conjunctival keratoplasty. resection should be performed [15-18]. • Under topical or sub conjunctively anesthesia, this consists Rehabilitation of conjunctival excision to bare sclera extending at Once the active ulceration has stopped and the remaining least 2 clock hours to either side of peripheral ulcer and cornea has been completely opacified, it is possible perform PK approximately 4 mm posterior to the corneoscleral limbus on these patients, even in the face of a thinned and vascularized and parallel to the ulcer[23]. The overhanging lip of cornea. ulcerating may also be removed. Post operatively Because of the immune systems remarkable memory, surgical a firm pressure dressing should be used. attempts at rehabilitation in Mooren’s ulceration, should be done only with concurrent immunosuppressant, even when The rationale of this procedure the active disease has been arrested, or is burnt out because adjacent to the ulcer contains inflammatory attempts at penetrating keratoplasty often are associated with cells that may be producing antibodies against the cornea recurrence and graft failure. and cytokines which amplify the inflammation and recruit additional inflammatory cells. Conclusion Multiple resections may be necessary. Bilateral Mooren’s ulcer in young patients can progress rapidly in a circumferential fashion towards the center of the cornea Systemic immuno suppressive therapy and can present with perforation and Iris prolapse early in a course of disease. Though clinical appearance is characteristics, Indication Mooren’s Ulcer remains a diagnosis of exclusion and systemic Bilateral or progressive Mooren’s ulcer that fails to preceding diseases associated with PUK must be ruled out. Current therapeutic attempts will require systemic cytotoxic theory to treatment options and work up have resulted in a significant bring a half to the progressive corneal destruction improvement in the prognosis of patients with Mooren’s Ulcer. With appropriate management, the eyes can usually be salvaged Commonly used agents and visual loss can be minimized.The keys to appropriate treatment are early diagnosis, judicious use of topical as well Cyclophosphamide – 2 mg / kg / day as systemic steroids, immunosuppressives, and the use of tissue Methotrexate (7.5 – 15 mg once a week) glue and patch grafting as indicated by the clinical scenario. Azathioprine (2 mg / kg body weight / day) The degree of fall in white blood cell count is considered as References the most reliable indicator of immunosuppression produced by 1. Bowman W: Case 12, pll2 in the parts concerned in the operations cyclophosphacomide. of the eye (1849), cited by Nettleship E: Chronic Serpiginous Most authors believe that the evidence for the efficacy of Ulcer of the Cornea (Mooren’s ulcer). Trans Ophthalmol Soc UK systematic immune suppressive chemotherapy for progressive 22:103-144,1902 bilateral Mooren’s ulcer is quite strong, and further believe that 2. McKenzie H: Disease of the Eye. 1854, p 631. such treatment should be employed sooner rather than later in 3. Mooren A: Ulcus Rodens. Ophthalmiatrische Beobachtungen. the care of such patients, before the corneal destruction, has Berlin, A. Hirschwald:107-110, 1867 become too extensive to need for surgery. 4. Nettleship A, Brkic S, Vackonic S: Chronic Serpiginous Ulcer of Adverse effects of these cytotoxic and immunosuppressive the Cornea (Mooren’s ulcer). Trans Ophthalmol Soc UK 22:103 medications, such as anemia, alopecia, nausea, nephrotoxicity, - 144, 1902 are likely and it must be administer in close observation of 5. Wood T, Kaufman H: Mooren’s ulcer. Am J Ophthalmol 71:417- physician. 422, 1971 6. Keitzman B. Mooren’s ulcer in Nigeria. Am J Ophthalmol Additional surgical procedures 65:679-685, 1968 Small perforations / Impending perforation may be treated 7. Lewallen S, Courtright P: Problems with current concepts of the with application of tissue adhesive – isobutyl cyanoacrylate epidemiology of Mooren’s . Ann Ophthalmol 22:52- and placement of a soft contact lens to provide comfort and to 55, 1990 prevent dislodging of the glue. 8. Majekodunmi AA. Ecology of Mooren’s ulcer in Nigeria. Doc Ophthalmol 49:211-219, 1980

DJO Vol. 21, No. 3, January-March, 2011 36 Bilateral Mooren’s Ulcer with perforation and Iris prolapse Delhi Journal of Ophthalmology 9. Schanzlin D: Mooren’s Ulceration. In: Smolin G, Thoft R, eds. 15. Chow C, Foster CS: Mooren’s ulcer. Int Ophthalmol Clin 36:1- The cornea. Boston. Little brown 1994, pp 408-414. 13, 1996 10. Wilson S, Lee W, Murakami C et al. Mooren’s type Hepatitis 16. Dinzis P, Mondino B: Management of non-infectious corneal C virus-associated corneal ulceration. Ophthalmology 101:736- ulcers. Surv Ophthalmol 32:94-110, 1987 745, 1994 17. Mondino B: Inflammatory diseases of the peripheral cornea. 11. Moazami G, Auran J, Florakis G et al: Interferon treatment of Ophthalmology 95:463-472, 1988 Mooren’s ulcers associated with hepatitis C. Am J Ophthalmol 18. Robin G, Schanzlin D, Verity S et al. Peripheral corneal disorders. 119:365-366, 1995 Surv Ophthalmol 31:1-36, 1986 12. Brown S. Mooren’s ulcer: Histopathology and proteolytic 19. Sangwan VS, Zafirakis P, Foster CS. Mooren’s ulcer: Current enzymes of adjacent conjunctiva. Br J Ophthalmol 59:670-674, concepts in management. Indian J Ophthalmol 1997;45:7-17 1975 20. Watson PG. Eye. 1997; 11 (Pt 3):349-56. 13. Young R, Watson P: Light and electron microscopy of corneal 21. Seino JY, Anderson SF. Optom Vis Sci 1998 Nov; 75(11):783-90. melting syndrome (Mooren’s Ulcer). Br J Ophthalmol 66:341- 22. Frangieh T, Kenyon K: Mooren’s ulcer. In: Brightbill FS, ed. 356,1982 Corneal Surgery: Theory, technique, and tissue, ed 2. St Louis: 14. Ferguson E III, Carreno O: Mooren’s ulcer and delimiting Mosby 1993, pp 328-335. keratotomy. South Med J 62:1170-1175, 1969 23. Foster CS: Immunologic disorders of the conjunctiva, cornea and sclera. In: Albert DA & Jakobiec FA, eds. Principles and Practice of Ophthalmology. Philadelphia: Saunders: 1994, pp 200-203.

Vol. 21, No. 3, January-March, 2011 DJO 37 Delhi Journal of Ophthalmology

Case Reports Acute Onset Myopia and Angle Closure Glaucoma after Topiramate Administration Nidhi Verma, Ashok Kumar Glaucoma Services, Nidhi Eye Hospital, Bijnor, Uttar Pradesh, India.

Topiramate is a new anti-convulsant which is used for the prophylaxis of migraine and cluster headache. We report a 17 year old male with bilateral induced acute onset myopia and secondary angle closure glaucoma following topiramate intake. In cases of bilateral acute angle closure glaucoma and induced myopia , drug induced rotation should be excluded.

Introduction Discussion Topiramate is a sulfamate substituted monosaccharide, new Acute Myopia, a rare idiosyncratic reaction to sulphonamides, anti epileptic drug used both as mono therapy and as an adjunct was first described in 1938.[2] Banta et al [1] reported the first in the control of partial and primary generalized epilepsy. It is case of topiramate ( Topomax, Ortho-McNeil) induced angle also effective in migraine prophylaxis, trigeminal neuralgia glaucoma in a 51 year old man who recently initiated the and bipolar disorders. We report the case of a adolescent with medication for mood stabilization. Case reports on ocular side topiramate induced acute onset myopia and secondary angle – effects of this drug date back to 2001.[1,3,4] In the “certain” closure glaucoma. category of WHO classification system, adverse ocular side effects associated with topiramate include abnormal vision, Case Report acute IOP elevation, acute myopia ( upto 8.75 dioptres), A 17 year old boy presented to our out patient department diplopia, and shallow anterior chamber with angle with complaint of sudden diminition of distance vision in both closures. eyes since morning. We noted induced myopia from previous High frequency UBM, AS- OCT and B-Scan ultrasound have emmetropic state to – 3.5 Dsph in right eye and -4.0 Dsph helped establish and document the patho-physiology of the in left eye. Anterior segment examination showed white and myopia and angle closure glaucoma[5-7] – uveal effusions quiet (both) eyes, but anterior chambers were shallow and the and ciliary body oedema resulting in antero-lateral rotation of iris and lens were bowed forward.(figure 1) IOP measured the ciliary body, anterior displacement of lens iris diaphragm by goldmann applanation tonometry were 36mmHg in right which contributes to the myopic shift, anterior chamber eye and 28mm Hg in left eye. Gonioscopy revealed 360′ shallowing and secondary appositional angle closure. Though appositional angle closure in both eyes. Fundus examination the exact mechanism is unclear, the fluid movement leading revealed normal optic nerve with a cup disc ratio of 0.4 in both to effusion is thought to be related to drug induced changes in eyes and bilateral retinal striae radiating from the fovea.[fig membrane potential.[8] 2] B-Scan ultra sound revealed annular peripheral choroidal The management of topiramate related acute rise of IOP effusion in both eyes.[fig 3] Oral topiramate treatment had requires stopping the drug in concert with the prescribing been commenced for migraine prophylaxis 10 days prior to physician. Medical therapy such as aqueous suppressants the onset of his symptoms at a dose of 25 mg once daily. He should be given. Laser peripheral iridotomy are not helpful had no known previous drug allergies. as angle closure is not pupillary – block related. Topical Topiramate was stopped immediately. Topical ‘Timolol + miotis are usually contra-indicated as they could precipitate Brimonidine’ combination eye drop twice daily in both eyes relative pupil block. Ophthalmologist should be cautious was started & continued for 3 days. Four days after stopping with acetazolamide as it is also a sulpha- based drug and has topiramate, visual acuity returned to normal with no refractive been reported to cause angle – closure glaucoma in a similar error, IOP was 10 mm Hg in both eyes, anterior chamber manner to topiramate.[9] depth was normal and anterior chamber angles were open Topiramate is rapidly absorbed ( serum levels peak after oral on gonioscopy. Two weeks later, repeat B-Scan ultra sound dose in 1-4 hours), has good bio- availability , a relatively showed resolution of choroidal effusion. long half- life ( 20 – 30 hours), and is predominantly excreted

DJO Vol. 21, No. 3, January-March, 2011 38 Acute Onset Myopia and Angle Closure Glaucoma after Topiramate Administration Delhi Journal of Ophthalmology in urine. If unrecognized as a drug related event, serious References outcomes could occur ( 7 cases of permanent visual loss 1. Banta JT, Hoffman K, Budenz DL, Ceballos E, Greenfield DS. following angle closure glaucoma have been reported ).[10] Presumed Ocular examination before starting topiramate cannot identify Topiramate-induced Bilateral Acute Angle-closure Glaucoma. eyes at risk. It is suggested that if the patient needs topiramate, Am J Ophthalmol. 2001;132:112–4. doi: 10.1016/S0002- it should be started with low doses and increased slowly with 9394(01)01013-3. [PubMed] [Cross Ref] regular ocular examination. Patients, who are on topiramate 2. Berns W. Proceedings of the Meeting of the Swedish therapy should be warned and advised to report immediately Ophthalmological Society, 3rd December, 1938. Acta any symptoms of eye – pain or blurred vision especially in the Ophthalmol. 1940;18:96-98. first few weeks of treatment. 3 Sen HA, O’Halloran HS, Lee WB. Case reports and small case Ophthalmologist need to be aware of the potential ocular side series: topiramate-induced acute myopia and retinal striae. Arch effects of topiramate. Ophthalmol. 2001;119:775–777. [PubMed] Although relatively rare, early recognition is key to appropriate 4. Sankar PS, Pasquale LR, Grosskreutz CL. Uveal effusion and management and maximised visual outcomes. secondary angle-closure glaucoma associated with topiramate use. Arch Ophthalmol. 2001;119:1210–1211. [PubMed 5. Shams P. 2007 Alcon Glaucoma Case Study Competition. Pinpoint Scotland Ltd; 9 Gayfield Square, Edinburgh EH1 3NT, UK: pp. 10–13. SUPP:1107:PINSCOT. 6. Levy J, Yagev R, Petrova A, Lifshitz T. Topiramate-induced bilateral angle-closure glaucoma. Can J Ophthalmol. 2006;41:221–225. doi: 10.1139/I06-012. [PubMed] [Cross Ref] Figure 1: Representative slitlamp photograph of the both eyes 7. Chalam KV, Tillis T, Syed F, Agarwal S, Brar VS. Acute demonstrating the following: A, shallow anterior chambers ; B, bilateral simultaneous angle closure glaucoma after topiramate Forward bowing of lens-iris diaphragm administration: a case report. J Med Case Reports. 2008;2 doi: 10.1186/1752-1947-2-1. [PMC free article] [PubMed] [Cross Ref] 8. Levy J, Yagev R, Petrova A, Lifshitz T. Topiramate-induced bilateral angle-closure glaucoma. Can J Ophthalmol. 2006;41:221–225. doi: 10.1139/I06-012. [PubMed] [Cross Ref] 9. Fan JT, Johnson DH, Burk RR. Transient myopia, angle-closure glaucoma, and choroidal detachment after oral acetazolamide. Am J Ophthalmol. 1993;115:813–814. [PubMed 10. Fraunfelder FW, Fraunfelder FT, Keates EU. Topiramate- associated acute, bilateral, secondary angle-closure glaucoma. Ophthalmology. 2004;111:109–111. doi: 10.1016/j. ophtha.2003.04.004. [PubMed] [Cross Ref] Figure 2. Representative fundus photograph of the right eye demonstrating retinal striae radiating from the fovea

Figure 3. Representative B-mode ultrasound image of the both eyes demonstrating: Choroidal Effusion

Vol. 21, No. 3, January-March, 2011 DJO 39 Delhi Journal of Ophthalmology

Instrument Scan Understanding your Direct Ophthalmoscope

Digvijay Singh, Rohit Saxena, Pradeep Sharma, Vimla Menon Dr. R.P. Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India

The direct ophthalmoscope is an extremely important examination tool not only for ophthalmologists but for physicians as well. It is probably the only tool in ophthalmology that can help perform a complete ocular examination. This article highlights the development, functioning and use of direct ophthalmoscopes.

A peak in the past Scientists had tried to peer into the then unknown back of the eye in the 18th and 19th century but were unsuccessful in understanding and establishing a coaxial illumination observation system. Then in the mid 19th century, several scientists noticed that if they kept a light source (pointed at the Figure 2: Helmholtz and his ophthalmoscope subject) very near their eye, then in some cases (emmetropes) they could view the red reflex and retina. It was in 1849 that Charles Babbage made what was probably the first practical Instrument detailing ophthalmoscope.[1] (Figure 1). It was a simple piece of Currently, the ophthalmoscope comes in various sizes and mirror with a silver patch rubbed off from the centre to modifications though all follow the same optical principle. make it see-through. Shortly afterwards, in 1851, Hermann Initially we describe in detail a standard direct ophthalmoscope Von Helmholtz published a monograph describing in detail and later look at the variants available with their specific the optical working of an ophthalmoscope and a designed a advantages[2]. (Figure 3) practical ophthalmoscope very similar to the ones used today. (Figure 2) Helmholtz is recognized as the inventor of the direct ophthalmoscope. An anecdote in this regard is that when Helmholtz tried to interest the king’s physician in his newly invented ophthalmoscope, he was told that it had no value as every known disease of the eye could already be diagnosed without it. Around the same time, the ophthalmoscope gave Helmholtz an instant global fame in the field of optics.

Figure 3: Detailed instrument scheme for the direct Figure 1: Babbage and his ophthalmoscope ophthalmoscope

DJO Vol. 21, No. 3, January-March, 2011 40 Understanding your Direct Ophthalmoscope Delhi Journal of Ophthalmology The ophthalmoscope consists of a metallic optical tube, as a clinical science existed well before the development of usually made of a durable light weight metal such as chrome- the ophthalmoscope as is exemplified by the founding of plated brass for proper alignment of the contents. Inside this Moorfields eye hospital in 1804 and many others[3]. tube, glass condensing lens, objective lens, mirror/prism aperture dial assembly, red-free/polarizer assembly and lamp What are all the knobs for? are sealed. The aperture dial is mounted such that it maintains Light intensity adjustment dial: This helps provide an alignment despite a fall/accidental drop from a reasonable illumination of variable intensity for eliminating the corneal height. reflex and patient comfort.

Illumination system Apertures • Incandescent lamp: This is usually a xenon halogen bright Small spot white lamp powered by a 2.5V non-rechargeable or 3.5V This provides approximately a five degrees cone and is used [NiMH (Nickel Metal Hydride) or LiION(Lithium ion)] for a small pupil. It also helps decrease corneal reflexes and rechargable battery. increases patient comfort. • Condensing lens: There are two condensing lens, one on Large spot either side of the aperture dial which focus the light onto This provides an approximately eight to ten degrees the mirror/prism. illuminated circle (though highly dependent on the refractive • Aperture dial: This has got various apertures such as status and papillary diameter). cobalt blue filter, fixation star, small spot, large spot, Macular spot/pinhole pinhole, hemispot and alit. These have a specific function This provides a small spot to observe only the fovea/macula each. without any undue light thereby minimizing patient discomfort • Reflecting mirror/prism: This is a mirror angled at45 and enabling viewing through a 1-2mm pupil. degrees which is partially reflecting or has a central Hemi-spot peephole. It makes the light cone projected upon the Reduces corneal reflex and provides retinal depth perception. patients eye appear as if it has originated from the mirror Slit itself. Most modern ophthalmoscopes utilize a prism in Accurate assessment of retinal elevations and depressions. place of a mirror for this purpose. Assessment of anterior chamber depth. Cobalt blue spot Viewing system Examination of corneal abrasions and scarring • Condensing Lens: These are aspheric lens with ranges Fixation star (with polar coordinates) varying with every ophthalmoscope model. Eg. +1-10, Accurate eccentric fixation testing, disc assessment and retinal +15, +20,+40 and -1-10,-15,-20,-25,-35 in the Heine beta mapping. 200. • Viewing window: Recessed, antireflective coated to Red free filter avoid glare. This may be combined with all filters. Contrasts features • Polarizing/red free filter: This is mounted on a separate by removing red colour and thus betters visualization of dial and enables green, red free image viewing of the blood vessels, hemorrhages and nerve fibre layer. Some fundus or a polarized view to detect nerve fibre layer. ophthalmoscopes may have a polarized filter to better evaluate nerve fibre defects. The why, what and how of direct ophthalmoscopy? Why? Condensing/focusing lens Why direct? It is a direct ophthalmoscope as the image forms They help focus the image onto the observers retina. Need to directly on the retina and there is no intermediate image akin be selected based on the subjects refractive status and distance to that seen in an indirect ophthalmoscope. at which ophthalmoscopy is done. Why ophthalmoscope? It is indeed interesting to wonder why this instrument came to be called as an ophthalmoscope What are the image properties? since the term ophthalmology was coined a good deal after The image formed by an ophthalmoscope is virtual, erect and the invention of the ophthalmoscope by Helmholtz in 1851. magnified. The area of retina imaged varies between 6.5 to 10 It is quite clearly argued in essays written in the nineteenth degrees. (Average area subtended by disc is 7 degrees vertical century that instruments and developing technology such and 5.5 degrees horizontal; thus an average sized disc should as ophthalmoscope and laryngoscopes actually led to the just fit the 5 degree cone of ophthalmoscope) development of the respective specializations. Of course eye

Vol. 21, No. 3, January-March, 2011 DJO 41 Delhi Journal of Ophthalmology Understanding your Direct Ophthalmoscope How does the ophthalmoscope function? When the ophthalmoscopy is being done from very close to Fundamental Optics the eye, the distance is less than f causing a virtual erect image The ray diagram of the direct ophthalmoscope is shown to be seen. below. It also depicts the usage of condensing lenses for eyes with [2]. (Figure 4) Magnification To understand how the ophthalmoscope magnifies, we take the example of an emmetropic eye. First we examine a small segment of a retinal vessel from 25 cm (the comfortable near vision distance). Let us suppose it subtended an angle of q0. We now view the same vessel segment from very close to the eye. Assuming the eye as a reduced lens of power 60D, we now are seeing from within the focal length of this lens thus we see a virtual erect image. On extrapolating this image to 25cm distance, you can observe that it is much larger and subtends 0 an angle of q1 . Thus we observe an angular magnification 0 0 and no linear magnification. M ang= q1 / q = distance(d) × power(D)= 0.25 × 60.This is equal to 15. (Figure 7)

Figure 4: Ray diagram depicting optics of ophthalmoscope

Image Properties The image properties depend on the working distance used for ophthalmoscopy. When done at a distance of 25 cm for distant direct examination, we observe a real inverted unmagnified 1 image of the fundus as shown in Figure5.

A object (upright arrows) at 25 cm subtends angle q at unaided observer’s eye. B Virtual Image (Large grey Arrow) of the same object subtends angle

1 Figure 5: Image properties during distant direct ophthalmoscopy q when viewed. Figure 7: Image depicting magnification property of When the fundoscopy is done from a very near distance to the ophthalmoscope subject’s eye, the image is a virtual erect one.(Figure 6) Field of view The field of view seen in a direct ophthalmoscope varies with the distance at which the examination is carried out and the pupil diameter. For example, if we observe the fundus from a distance of 15 cm in a 2mm pupil, then we can only see an area of about 200-300µ or a short segment of a vessel. In contrast on observing from very close to the eye in a well dilated pupil (8mm), we can see more than 10degrees of field. Theoretically it may be possible to see up to the equator in Figure 6: Image properties while viewing fundus during close range a fully dilated pupil in a cooperative patient on moving the ophthalmoscopy. ophthalmoscope and patients eyes appropriately. This image changes from a real to virtual may be understood with a basic knowledge of optics. The focal length of the How to perform a complete ophthalmoscopic reduced eye model is 1.67 cm. The retina therefore lies between examination? f and 2f. When we do a distant direct ophthalmoscopy, we are Steps observing the image from distance between 2f and infinity as Before proceeding, it is important to understand the instrument also, the light source is originating from this point. This thus well. gives a real inverted image forming on the observer’s eye. The first step in the use of an ophthalmoscope is todo

DJO Vol. 21, No. 3, January-March, 2011 42 Understanding your Direct Ophthalmoscope Delhi Journal of Ophthalmology examination at 1m distance. This sheds light on any retina commences about 16 degrees temporal to it. Thus, abnormalities of the , orbit and periorbita as well as if the patient is seeing straight ahead, then we know how highlights any obvious ocular deviations. much to tit the ophthalmoscope to view these landmarks. This should be followed by a distant direct examination • The patient should be asked to look straight ahead into at 22-25cm( a comfortable near vision distance). Some the distance or preferably to a target on a far off wall. ophthalmologists prefer to do this at a closer distance of 10cm • The patient should be instructed to stay steady and as it gives better details. If the examination is done at 10cm, frequently blink during the examination. we should select a +10D condensing lens to view the best • The examiner should use his right eye to view the patient’s glow. At 25 cm, a +4D lens may be used. This examination right eye and vice versa. They should keep their opposite shows a red reflex and highlights any opacities in the media hand on the patient’s forehead to support and steady it. as black images. The patient may then be asked to look in the • The examiner should keep both their eyes open during four cardinal gazes and the movement of the opacity noted. examination and imagine as if the retina is at 6 meters to Movement against the ocular movement means the opacity is prevent . behind the nodal point of the eye (i.e. in the lens or vitreous) • Normally, the examiner should continue to wear his while a movement with would indicate corneal or anterior glasses while the patient has to remove his. The field of segment opacity. The distant direct examination is also used view decreases if the examiner wears his glasses therefore to examine the lens, iris, cornea and adnexa. Any squint in a for low myopes or hyperopes (± 3Ds) and astigmats child may be picked up due to an unequal reflex (Bruckner’s (below 2.5Dc) may remove their glasses especially in a test). The presence of an RAPD can also come forth in this small pupil. step. • A trick to decide the appropriate selection of the The third step involves moving closer to the patient and condensing lens is described as follows. Observe the correspondingly increasing the power in the condensing lens light reflex on the retinal vessels. If a white line is seen to examine in detail the magnified anterior segment structures. then either the patient is emmtropic or hyperopic. In that The fourth step entails reducing the condensing lens power case, add plus lens and the highest plus when the line such that any part of the retina comes into focus. While reflex disappears is the appropriate power. This would reducing the power, the vitreous cavity will come into focus also be the approximate refractive error of the patient if and any pathology in it may be seen. Once the retina is focused, the examiner had not accommodated. If there is no line of we may localize any blood vessel and follow it backwards light reflex on the vessels, then the patient is myopic. Add against the branching pattern to reach the optic disc. Then minus power and the smallest minus lens when the reflex move temporally from the disc to reach the macula. We can appears is the refractive error of the patient. ask the patient to look into the light and the fovea will come • The lenses of the ophthalmoscope can be used to focus into focus. The blood vessels can be traced into the periphery variously the apex and base of any intraocular mass and from the disc to reach second and third order vessels. This thus helps determine its height in dioptres. completes the posterior pole examination to examine the periphery, we ask the patient to look in the four cardinal gazes How to select an appropriate ophthalmoscope? while continuing to focus the retina. The ophthalmoscope The ophthalmoscope selection should be guided by a number illuminated cone may be moved to further our view into the of factors. Prime among these is the intended clinical role. peripheral retina. For a physician who needs to evaluate whether a fundus is normal or abnormal, a basic design should suffice while an Practical tips ophthalmologist should look for one with highest quality • To eliminate the irritating corneal reflex, one can slightly optics and maximum functionality for accurate diagnosis. The tilt the ophthalmoscope and view obliquely. One can ophthalmoscope should have apertures such as the small and also decrease the illumination intensity and use a smaller large spot, cobalt blue filter, slit and the fixation star. Presence aperture. of a green filter is mandatory for diagnostic purposes. The • Corneal reflex is also negated if the patient is approached instrument should contain adequate number of focusing lenses from 15 degrees rather than from straight ahead. with a 1 dioptre minimum count for fine focusing. The battery • Use a small aperture for a smaller pupil as only an should preferably be rechargeable Li ion or NiMH which illuminated cone equal to the size of the pupil can enter or provide extended power. Beyond this minimum specifications, exit the eye while the rest will reflect off the iris creating any of the advanced machines may be used. unnecessary glare and poor contrast. • The fovea lies 3 degrees temporal to the optical axis of How to Care for the direct ophthalmoscope? the eye, the disc lies 10 degrees nasal and the peripheral The direct ophthalmoscope is a sturdy built instrument for

Vol. 21, No. 3, January-March, 2011 DJO 43 Delhi Journal of Ophthalmology Understanding your Direct Ophthalmoscope heavy handling but requires proper care for its longevity. The possible but not mutually interfering. This gives a larger view following precautions should be observed: in smaller pupils as well as eliminates corneal reflex artifacts. • The battery should be fully discharged and recharged Apart from this the incorporation of aspheric designs in lenses once every few months to maximize battery life. has led to decreased aberrations and reflex artifacts. • After fully charging the battery, the bulb should first be Battery life has been greatly enhanced with the modern NiMH switched on at sub-maximal illumination for 1-2 cases or Li ion batteries while their size and weight has more than before employing full illumination to maximize bulb life. halved. This has enabled manufacture of Pocket sized or mini • Use only genuine bulbs and replace in accordance with ophthalmoscopes which have an added convenience factor. the owner’s manual instructions. Further advancements are underway to add better and more • The condensing lenses are stuck to the dial using glue functionality to this principal tool of every clinician. which is soluble in acetone and related solvents. Therefore never use these to clean your ophthalmoscope. Conclusion • The ophthalmoscope may be cleaned using mild alcohol The direct ophthalmoscope has an immense contribution in or detergent and a soft cloth. furthering the development of ophthalmology as a specialty • A cotton bud should be used to clean the viewing window science. Familiarity with the use of this instrument would go and aperture window in a circular sweeping manner. a long way in aiding the diagnosis of diseases by physicians • Ophthalmoscopes come with twist and fit as well as and ophthalmologists alike. Although technology has brought automatic lock heads. Ascertain the head connector in the the ophthalmoscope a long way from its humble beginnings, scope and fix head accordingly. one should realize that like every instrument, it too has its • Always store the ophthalmoscope in ints case when not limitations. These include the limited field of view, poor in use. If it will be unused for a long time, remove the image visibility through hazy media, inability to appreciate battery and store. the full picture, a high need for patient cooperation and non • Dispose the NiMH or Li ion battery appropriately. stereoscopic viewing among others. If we works bearing these in mind, we are unlikely to get mislead by false signs and Innovations in direct ophthalmoscopes would gain a lot more from this brilliant instrument. The direct ophthalmoscope has come a long way from the polished mirror made by Babbage or the more practical model References of Helmholtz. 1. Keeler CR. Babbage the Unfortunate..Br J Ophthalmol Illumination technology has shifted from the use of a gas 2004;88:730-732. flame as an external source of illumination to the first directly 2. Timberlake GT, Kennedy M.The Direct illuminated ophthalmoscope made in 1915 to the current day Ophthalmoscope:How it Works and How to Use It. 2005. instruments using halogen and xenon bulbs. University of Kansas Press. The viewing systems have improved drastically over time. 3. Silvester A. The emergence of medical specialties in the The latest innovations include the panoptic ophthalmoscope nineteenth century: a discussion of the historiography. which uses axial point source optics whereby the light is History of Medicine online 2010 Priory.com publication focused at a point on the cornea before moving onwards into 4. Instruction user manual of Heine Beta200 ophhalmoscope. the eye. This enables a wider field of view (up to 5 times 5. Ghosh S, Collier A, Varikarra M, Palmer S. Fundoscopy wider) in smaller pupils. Another leap forward is the use of made easy. 2010. Churchill livingstome Elsevier. 1-21. advanced coaxial optics where the illumination and viewing 6. Fisher WA. Ophthalmoscopy, Retinoscopy and Refracion. is done along the same path of light keeping them as close as 1937:1-34.

DJO Vol. 21, No. 3, January-March, 2011 44 Delhi Journal of Ophthalmology

Origial Article Study of Colour Blindness in Tibetan Population Navjot Kaur, Avinash Kumar, Gurinder Kaur, Jasjeet Kaur Dhillon, K.D.Singh Government Medical College & Hospital, Chandigarh, India Of the 1210 male and 800 female Tibetan population in Northern India between the age group of 11-60 years, 51 males and no female were found to be having colour blindness, showing an incidence of 4.21 % and 0% respectively. The study was done with the help of Ishihara charts and Pickford Nicolson’s anomaloscope. The type of colour blindness in males in its descending order of occurrence was simple deuteranomaly 27.45% protanopia 17.64%, deuteranopia 17.64%, extreme deuteranomaly 15.68%, protanomaly 13.72%, extreme protanomaly 5.88% and tritanopia 1.96%. None of the female was colour blind. The incidence and type of colour blidness found in males is within the range of other Indian samples. Nevertheless the incidence is much lower than the values reported in European populations but is much higher than the values reported in African populations.

Introduction the plates (5). Ishihara plates consist of a series of cards on Sir John Dalton was the first scientist to give a clear description which a coloured background is printed in spots of different of his own colour blindness in 1794. This publication sizes. A letter, figure or a number is printed against this stimulated much subsequent research into the pathophysiology background in spots of the same size. To a normal subject and genetics of the condition (1). In 1801-1802, Thomas Young the figure or letter at once becomes clear, but the colour blind postulated the existence of three “principal” colours (red, subject fails to distinguish it from the background. It is easy, green and violet) from which all colours and white light can be quick to perform and type of colour defect can be ascertained obtained, (2). Von Helmholtz (1866) suggested that there are with a fair degree of accuracy. The detection of Tritan defect three types of cones containing three photochemical substances is not possible using this test. First 25 plates were used in the corresponding to the three fundamental colour sensations (3). present study as all the individuals were educated. The plates In 1881, Lord Rayleigh introduced anomaloscope for scientific from 26 to 38 are meant for illiterate persons, so these were analysis of colour defects (4). Since then many scientists have not used.The Pickford Nicolson Anomaloscope (6) is a simple worked on the incidence of colour blindness in different parts colorimeter based on the use of integrating boxes. The outside of India and the world. But very few studies are available on the dimensions of the apparatus are about 10”X 10 ½ “X5”. use of anomaloscope in India. The present study was conducted The instrument has color chances optical glass filters. It is to find out the incidence of congenital colour blindness in the manually operated. With the anomaloscope three tests were Tibetan population and to find out the type and degree of colour performed in which a red / green, a green / blue and a yellow blindness with the help of anomaloscope. A comparison witl1 / blue Rayleigh equation were used respectively. The Rayleigh other studies is made. Persons with defective colour vision are equation used is: Red (642nm) + Green (555nm) = Yellow at a disadvantage especially for employment purposes eg: as (585nm).The wavelengths of the primary filters (Red and pilots, drivers, in defence services and in technical fields like Green) used are 642nm and 555nm, to produce a yellow with a engineering and medical profession. wavelength of 585 nm. Both eyes were tested separately using different equations. It is the only instrument by means of which Method colour blindness can be correctly classified 2010 Tibetees of either sex between the age group of 11-60 years from Patiala, Ambala, Zirakpur, Mohali, Bhuppur camp Results and Puruwala camp were investigated for colour vision by the All healthy subjects with no history of chronic disease or long means of Ishihara charts and the anomaloscope. Not much work term medication were selected for the present study. Out of has been undertaken in this group of population in this region 2010 individuals (1210 males and 800 females) 51 males and of the country therefore, the Tibetan population was selected in no female was found to be having colour blindness (Table 1) the present study. In addition to the tests of colour vision, all the The age range of 51 colour blind males is 11-60. (Table II) The subjects were tested for acuity of vision by using Snellen’s test type of colour blindness in males by using Ishihara charts only chart for distant vision and Jaeger’s chart for near vision. is shown in Table III. The 15th edition of Ishihara plates (1960) was used and tests The results of the Ishihara chart tests show that 51 individuals were conducted in accordance with instructions accompanying were protan/deutan type and only one individual was of tritan

Vol. 21, No. 3, January-March, 2011 DJO 45 Delhi Journal of Ophthalmology Study of Colour Blindness in Tibetan Population type. Out of 51 protan/deutan type of individuals, 18 were of Elder. Physiology of the eye and vision. System of ophthalmology protan type, 32 were of detuan type. Another observation was 1968; 4:620. that out of 50 protan/deutan type of individuals there were only 4. Rayleigh L. Colour equations. Nature 1881; 25: 64-66. 2 whose readings were resembling the test figures totally. All 5. Ishihara S. Test for colour blindness. 15th complete edition with other protan/deutan type did not show a typical resemblance 38 plates 1960; H.K. Lewis Co. Ltd., London. in total, but a sum total of their readings show that they were 6. Pickford RW, Lackowski R. Anomaloscope. Brit J Physiol Optics of protan/deutan type. This shows that there are individual 1961; 17: 131-134, differences in the degree of colour blindness. The reading 7. William F Ganong . Vision. Review of medical physiology 2003; of one tritan type of individual resembled the test figures 21: 168. completely. The results also show that the deutan type of colour 8. Dutta PC, Kumar GO. The incidence of inherited defects of blindness is the most frequent type. The acuity of vision with colour vision in MP. Acta Genet Med Gen 1966 ; 15(4) 364-369. or without glasses in all these affected individuals was found 9. Bansal IJS. The frequency of colour blindness among the Punjabis to be normal. The type of degree of colour blindness in males of India. J Genet Hum 1967; 16 (1-2) : 1-5. as tested by the anomaloscope are shown in Table IV. As most 10. Parikh NP, Baxi AJ and Jhala HI. Incidence of colour blindness of these affected individuals are of protan/deutan type, the among three groups from Gujarat. The Indian Journal of Medical results of the red/green equation are plotted graphically (Fig 1) Sciences 1968 ; 334-7. . No monochromat was found in the present study. In all the 11. Bhasin MK. The frequency of colour blindness in Newars of affected individuals both eyes were defective, on testing each valley. Acta Genet Basel 1967; 17: 454-458. eye separately. An interesting observation in the study was that 12. Clements F. Racial differences in colour blindness. Amer J Phys most of the affected individuals did not know that they had any Anthrop 1961 ; 4: 189-204. defect regarding their colour vision. 13. Vries-de Mol EC de, Went LN. Frequency of different type s of colour vision defects in Netherlands. Human Heredity 1978 ;28: Discussion 301-317. The incidence of colour blindness is much more in males as 14. Post RH. Natural selection and colour blindness. Eug Quart 1962 compared to females. The results of the present study with ; 9: 131-146. anomaloscope show that the frequency of trichromatism 15. Rebato E and Calderon R. Incidence of red-green colour blindness was much more as compared to dichromatism and among in the Basque population. Anthropol Anz 1990 ; 48 (2) : 148-5 . trichomats, the frequency of deuteranomaly is more than that 16. Sato H. Frequency of colour defects among Japnese. Acta Soc of protanomaly. This can be explained by the heredity of colour Opthal Jap 1935 ; 38: 2227-2239. blindness (7). As colour blindness is a hereditory defect, the 17. Chan F, Mao J. Colour blindness among Chinese. Brit J Opthal incidence in different age groups is statistically insignificant. In 1950 ; 34 : 744-748. the present study the incidence of colour blindness in males was 18. Zein ZA. Gene frequency and types of colour blindness In 4.21 %. A comparison with the other studies show that there is Ethiopians. Ethiop Med J 1990 ; 28 (2) : 73-75. less variation of the incidence of colour blindness in different 19. Applemans M. Colour defects among the natives of Congo.Bull parts and communities of India as shown in Table V than in Soc Belge Opthal 1953 ; 103: 226-229. other parts of the world as shown in Table VI. This comparison 20. Simon T. Colour defects among natives of Uganda. E Afr Med J gives support to the selection relaxation hypothesis of Post (14) 1951 ; 28 :75-78. and Pickford (22). 21. Oppolzer A and Winkler EM. The incidence of colour blindness As shown in Table VII no female was found to be colour blind. in East African Negores. Anthropol Anz 1980 ; 38 (2) : 117-20. It is not possible to conclude regarding the incidence of colour 22. Pickford RW. Natural selection and colour blindness. Eugen Rev blindness in females from the present data. To find the exact 1963 ; 55 : 97-101. incidence of colour blindness in females, a further study with a 23. Tiwari SC. The incidence of colour blindness among the Tibetans. large sample is required. J Genet Hum 1969; 17 (1-2) : 95-99. 24. Mahajan OP, Gogna RS. Study of colour blindness in school References children. Indian J Physiol Pharmacol 1977; 21 (1) : 59-62. 1. Emery AE, John Dalton (1766-1844). J Med Genet 1988; 25 (6) 25. Manibabu, Mayanglambam. Colour blindness amongst the :422-426. Maring Nagas of Manipur. Journal of Human Ecology Delhi; 2. Young: Theory of colour vision. Phil Trans 1801 ; 91: 23-A. 1998 ; 9 (2) : 199-200. A course of lectures on natural physiology and the mechanical 26. Naresh S. Study of colour blindness in Jat Sikhs Indian J. Physiol arts, London 18-07. Quoted by Duke Elder. The Physiology of Pharmacol Apr 1995 ; 38 (2) : 127-130. eye and vision 1968; iv: 643. 27. Dhillon JK: Study of colour blindness in Mohammadans MD 3. Von Helmholtz: Ann Phys (LPZ) 1852; 87: 45. Quoted from Duke Thesis of Punajbi University 1979.

DJO Vol. 21, No. 3, January-March, 2011 46 Study of Colour Blindness in Tibetan Population Delhi Journal of Ophthalmology Table I Table V Showing the incidence of colour blindness In 2010 Tibetees Comparison of incidence of in males in different Part of India

Table II Showing Age Range of 51 colour blind males.

Table VI Comparison of incidence of color blindness in males in Table III different Part of India Showing type of colour blindness in 1210 males by Using Ishihara Chart Test

Table IV Showing type of colour blindness in 1210 males by Using Ishihara Chart Test Table VII Comparison of incidence of color blindness in females in different Part of India

Vol. 21, No. 3, January-March, 2011 DJO 47 Delhi Journal of Ophthalmology

Origial Article Manual Sutureless Small Incision Technique for Exchange of dislocated Posterior Chamber IOL Lakshminarayana Pasumala ESIC Hospital , Noida (Delhi/NCR)

Dislocation of Posterior Chamber Intra Ocular Lenses(PC IOL) into posterior segment with complete rupture of posterior capsule is a well known complication of Phacoemulsification with Foldable IOL implantation which can be managed by various techniques such as repositioning of the IOL in the ciliary sulcus by suturing to sclera or iris fixation and IOL exchange with either a iris claw lens or Anterior Chamber(AC) IOL.Suturing techniques result in complications like vitreous hemorrhage and IOL re-dislocation and exchange with iris claw lens and AC IOL requires large limbal corneal incisions which need suturing and result in significant and complications like iris prolapse, and iris atrophy.To obviate these problems we describe a series of 7 cases in which we have used the incision design of Manual Small Incision Cataract Surgery for exchange of dislocated PC IOL’s with AC IOL’s through a Manual Sclero Corneal Sutureless Small Incision (Manual SI) route in cases with absent capsular support.Though this route is being used in such cases there is no study till today describing it’s effectiveness and pitfalls if any in a series of cases.

Introduction implantation with complication of dislocated PC IOL into The advent of Phacoemulsification has rendered the incision vitreous with complete capsular rupture, 2 operated in our for cataract surgery smaller, compact and self sealing, center and 5 being referral patients. In all the 7 patients the facilitating use of foldable intraocular lens (IOL).Insertion of IOL was completely dislocated into vitreous. Complete Ocular foldable IOLs through these compact incisions carries with it examination and detailed Indirect Ophthalmoscopy(I/O) high risk of PC IOL dislocation in case of tears in lens capsule. examination was normal in all patients. Two patients had mild Dislocated IOL needs to be removed and requires either vitreous haze but no vitreous hemorrhage. repositioning or exchange. Various techniques described for In all the patients a peribulbar block was administered after repositioning or exchange of dislocated IOL in the absence of dilating the pupil. Superior rectus bridle suture applied, capsular support include repositioning of the PC IOL in the conjuctival peritomy done and scleral bed blanched with ciliary sulcus by suturing to the sclera [1] and iris fixation. cautery. A 6mm sclerocorneal tunnel carved as in manual SICS IOL can be exchanged with either iris claw lens or Anterior procedure described by Sanduk Ruit et al with a crescent knife. chamber lens(AC IOL) via a large limbal incision. [2] AC IOL At this time no entry is made into the anterior chamber. After implantation is technically easier but it requires an additional this a standard 3 port pars plana vitrectomy (PPV) procedure large limbal incision and carry with it intraoperative problems done and IOL is gently prolapsed into the AC and placed above like iris prolapse, iris atrophy, hyphema. To obviate the the iris (Fig 1, Fig 2). Pupil constricted with 0.5% pilocarpine problems of large uncontrolled limbal incisions in these cases (Fig 3) and AC washed with saline. Visco Elastic Device is the sclerocorneal sutureless small incision design of manual injected below and above the IOL through a clear corneal side SICS surgery is being used as an effective alternative with port incision. Superior vitrectomy ports closed with 6-0 vicryl excellent results but a systematic case series describing it’s suture. Valvular entry is made into the AC through the already use is lacking.Hence we describe in a series of 7 patient’s, the created tunnel with a keratome knife. Leading haptic of the technique for exchange of dislocated PC IOL with AC IOL PC-IOL is held with a vitreous forceps and IOL explanted through a Manual Sutureless Small Incision(manual SI) route through the tunnel incision (Fig 4). Infusion of saline through which avoids large uncontrolled limbal incisons and results in the vitrectomy cannula is kept on during the entire procedure quick and effective visual rehabilitation to the patient. to maintain good AC pressure. Kelman style, PMMA AC Materials and Methods IOL is implanted in the AC after closing the infusion port of We have operated on 7 patients using this technique between vitrectomy cannula (Fig 5). Peripheral iridectomy done and Sep 2009 and July 2010 in our hospital, all surgeries sclerocorneal lip is hydrated with saline and AC made water being performed by a single surgeon. All the patients have tight. Conjuctival edges are cauterised and subconjuctival previously undergone phacoemulsification with foldable IOL injection of a antibiotic-steroid mixture given.

DJO Vol. 21, No. 3, January-March, 2011 48 Manual Sutureless Small Incision Technique for Exchange of dislocated Posterior Chamber IOL Delhi Journal of Ophthalmology Results incision route in a series of 7 patient’s which results in less In all the 7 cases foldable acrylic IOL’s were explanted by post operative astigmatism, quick visual rehabilitation and PPV and Kelman style AC IOL’s of optic diameter 6 mm with additional benefit of decreased iris related complications. implanted in the AC. None had any contraindication for AC The technique is easy to apply as it is an extension of incision IOL implantation. Pre-op visual acuity (VA) with aphakic design of manual SICS surgery and requires less technical correction ranged from 20/80 to 20/40. All the patients expertise, saves time and can be practiced on a wide scale.To had excellent post operative visual recovery and at 8 week the best of our knowledge this is the first study describing the examination minimal refractive error noted in all (range -0.25 use of incison design of manual SICS surgery for exchange DS to- 1.00 DS spherical and astigmatism range from -0.25 of posteriorly dislocated IOL’s in a case series of 7 patients. DC to -0.75 DC) (table 1).Post operative VA ranged from 20/30 to 20/20.The wound was self sealing in all the patients and required no suture. Post-operatively none developed any retinal tears, choroidal effusions, supra choroidal hemorrhage or vitreous hemorrhage. Corneal clarity were normal pre and post operative in all the patients.

Discussion Dislocation of PC IOL into vitreous with complete capsular rupture is an often encountered problem in modern cataract surgery and can be managed with either repositioning or exchange of the IOL. Exchange with a AC IOL is a simple Figure 1: PC IOL is being brought into AC with vitreous forceps technique and the procedure requires less time unlike suture after PPV. fixation which is a technically demanding procedure [3] and it cannot be universally applied to all designs of IOLs. [2] IOL exchange with iris claw lens and AC IOLs require large limbal incisions of 7-7.5 mm with associated risk of choroidal effusions and supra choroidal hemorrhage [4,5] and also the incision requires suturing. In contrast our technique involves exchange of IOL through a self sealing sutureless sclerocorneal incision route which is routinely used for manual SICS and we did not encounter any of the problems seen with large limbal incisions. Harry S Geggel [6] has described a simplified technique for acrylic foldable IOL explantation through a scleral frown incision of 3.5-4 mm and exchanged it for another foldable IOL. In our cases we have explanted the IOLs through a self Figure 2: PC IOL prolapsed into AC and resting above iris.VED is sealing 6 mm incision and implanted a AC IOL. As manual injected above and below the IOL. SICS’s are widely performed in developing countries we have applied its incision technique in our cases with good result. On Literature search we did not find any systematic study reported for exchange of dislocated PC IOL with AC IOL through a manual SICS route so far and with this technique we encountered minimal astigmatism. Sanduk Ruit et al [7] compared Phaco with manual SICS and reported minimal astigmatism difference between Phaco and manual SICS group (0.18 D). Our technique involves a valvular self sealing incision and has decreased incidence of iris prolapse, iris atrophy. Using this technique we have explanted 4 single piece foldable acrylic IOL’s and 3 multipiece IOL’s. In conclusion we have described a useful technique for exchange of dislocated Figure 3: Iris constricted with 0.5% pilocarpine nitrate to prevent PC IOL with a AC IOL through a manual sutureless small the IOL from falling back into the vitreous.

Vol. 21, No. 3, January-March, 2011 DJO 49 Delhi Journal of Ophthalmology Manual Sutureless Small Incision Technique for Exchange of dislocated Posterior Chamber IOL References 1. Pang MP, Ilar M. A Modified technique for the management of completely dislocated posterior chamber intraocular lenses in the absence of capsular support. Retina 2003; 23: 861-63. 2. Flynn HW Jr. Pars plana vitrectomy in the management of subluxed and posteriorly dislocated intraocular lenses. Graefes Arch Clin Exp Ophthalmol 1987; 225: 169-72. 3. Wagoner MD, Cox TA, Ariyasu RG, Jacobs DS, Karp CL. Intraocular lens implantation in the absence of capsular support: a report by the American Academy of Ophthalmology. Ophthalmol 2003; 110: 840-59. 4. Sabti K, Lindley SK, Mansour M, Discepola M. Uveal effusion after cataract surgery: an echographic study. Ophthalmol 2001; Figure 4: PC IOL is being explanted through the small incision 108:100-3. route with vitreous forceps. Note no iris prolapse. 5. Beatty S, Lotery A, Kent D, O’Driscoll A, Kilmartin DJ, Wallace D, et al. Acute intraoperative suprachoroidal haemorrhage in ocular surgery. Eye (Lond) 1998; 12:815- 20. 6. Geggel HS. Simplified technique for acrylic intraocular lens explantation. Ophthalmic Surg Lasers. 2000; 31: 506-7. 7. Ruit S, Tabin G, Chang D, Bajracharya L, Kline DC, Richheimer W, et al. A prospective randomized clinical trial of phacoemulsification vs manual sutureless small- incision extracapsular cataract surgery in Nepal. Am. J. Ophthalmol 2007; 143: 32-38.

Figure 5: AC IOL in place .Wound self sealed requiring no suture.

DJO Vol. 21, No. 3, January-March, 2011 50 Delhi Journal of Ophthalmology Instruction to Authors

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DJO Vol. 21, No. 3, January-March, 2011 52