DJO_Oct_Dec_2022

Volume 32 | Issue 6 | October - December 2022 https://journals.lww.com/djo Theme: i Innovations (surgical)

Delhi Journal of Ophthalmology Editorial Board Editor-in-Chief Kirti Singh Official Journal of Delhi Ophthalmological Society Volume 32 Number 6 October-December, 2022 DJO Associate Editors Annu Joon Devesh Kumawat Divya Jain Mainak Bhattacharya Arshi Singh Khushboo Chawla Priya Saraf Assistant Editors Akanksha Ankita Bhardwaj Anjali Mehta Bhupesh Charu Khurana Deepanjali Arya Himshika Aggarwal Gunjan Budhiraja Jatinder Bali Jatinder Bhalla Jigyasa Sahu Manisha Agarwal Neha Chawla Neha Rathi Nisha Choudhary Palak Gupta Pooja Bansal Priyadarshi Gupta Priyanka Golhait Prachi Dave Rahul Mayor Rajat Jain Ritu Aurora Shipra Sharda Shruti Bhattacharya Shweta Vishwanath Siddharth Baindur Suma Ganesh Sumit Grover Siddharth Madan Tanvi Gaonkar V.Krishna Vaibhav Khanna Vaibhav Nagpal Vineet Sehgal Section Editors Arun Nrayanswami Bhavna Chawla George L. Spaeth Milind Pandeya Sonal Dangda Satish Kotta M. Vanathi Rajesh Sinha Ruchi Goel Vinod Kumar International & Emeritus Editor A. K. Grover Atul Kumar Bithi Chowdhary Deepak Verma Jolly Rohtagi J.S.Titiyal N.P. Singh Mahipal S. Sachdev M.D. Singh M. Vanathi Namrata Sharma Pawan Goyal Pradeep Sharma Praveen Vashisht Rakesh Bhardwaj Ramanjeet Sihota Ritu Arora Rajender Khanna Sarita Beri Suneeta Dubey S.C. Dadeya Advisory Board Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022 i

Contents Editorial Yesterday’s Magic is Today’s Science........................................................................................................1 Dr. Kirti Singh Guest Editorial Innovations: The Eureka Euphemism........................................................................................................3 Dr. Santosh G Honavar Review Article Descemet’s Membrane Detachment - An Update.....................................................................................5 Abhijeet Beniwal, Anitha Venugopal, Murugesan Vanathi, Radhika Tandon Original Articles Comparison of Surgical Outcomes of Cataract Extraction by Manual Small Incision Cataract Surgery and Phacoemulsification in Phacomorphic Angle Closure ........................................ 13 Deepika Singh, Parveen Rewri, Sagarika Aggarwal Clinical Profile and Management of Neovascular Glaucoma in North India..........................................19 Ketaki Rajurkar, Suneeta Dubey, Monica Gandhi, Prachi Gurav, Julie Pegu A New Perspective on Eyelid Laceration Classification: Proposal Based on Study Conducted at a Tertiary Eye Care Centre in South India ........................................................................24 Christina Daisy Philips, Modini Pandharpurkar Improving Quality of Life of Low vision Patients with Living Space Modifications ..............................29 Ashwini Kumar (MBBS DOMS) Case Reports Leber Hereditary Optic Neuropathy: Still a Dilemma? ........................................................................... 31 Nishi Meghna Satish, Soveeta Rath, Kritika Chopra, Rolli Khurana, Neha Chawla, B.P. Guliani Bilateral Subconjunctival Hemorrhage in a Young Female following COVID 19 Vaccination...............................................................................................................................34 Sohini Mandal, Ananya P.R., Prafulla Kumar Maharana Bilateral Retinitis Pigmentosa with Bilateral Microphakia: An Uncommon Association......................37 Anupriya Aggarwal, Rajwinder Kaur, Balbir Khan, Priyanka Dahiya Iatrogenic Induced Complete Herniation of Vitreous - Atypical Presentation of Vitreous Touch Syndrome without Corneal Edema/ Decompensation in a Bilateral Aphakic Patient with Pseudoexfoliation Glaucoma............................................................................................ 40 Prasanna Venkatesh Ramesh, Shruthy Vaishali Ramesh, Meena Kumari Ramesh, Ramesh Rajasekaran Appearances can be Deceptive................................................................................................................43 Mukta Sharma, Rajneesh Thakur, Shalini Customised Surgical Management of Congenital Upper Lid Coloboma ............................................... 46 Nitika Beri, Gopal Krushna Das, Pramod Kumar Sahu, Isha Sharma, Rahul Bhatia, Nitish Kumar ii Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022

Photo Essay Multimodal Imaging in a Case of Stargardt’s Disease............................................................................50 Shruti Bhattacharya, Khushboo Chawla, Priyadarshi Gupta, Meenakshi Thakar Unilateral Anterior Luxation of the Crystalline Phakic Lens in a Patient with Bilateral Isolated Microspherophakia ...................................................................................................................52 Prasanna Venkatesh Ramesh, Shruthy Vaishali Ramesh, Aji Kunnath Devadas, Prajnya Ray, Meena Kumari Ramesh, Ramesh Rajasekaran Omnibus Humanus (Masters, Change makers, Out of box thoughts) Eye Donation: Crucial Bedside Manners of Eyeball Harvesting Team....................................................54 Dr. Anjali Mehta Theme Sections Newer Nuances in Orbital Surgery..........................................................................................................56 Kasturi Bhattacharjee, Vatsalya Venkatraman Emerging Innovations in Cataract Surgery.............................................................................................61 Nirupama Kasturi, Ninan Jacob, Ajax Jossy, Arup Chakrabarti Surgical Innovations in Strabismus Surgery – A Brief Review ...............................................................72 Isha Chaturvedi, Pradeep Sharma Innovations to Make Glaucoma Surgery Safer.......................................................................................78 Kirti Singh Innovations in Glaucoma Surgeries ........................................................................................................82 Vanita Pathak Ray, Rahila Ramzan Surgical Innovations in Vitreo-Retinal Surgery ..................................................................................... 88 Sumant Vinayak Sharma, Raja Narayanan Cover Image a) 3D skull reconstruction (Kasturi Bhattacharjee) b) Visitec i-ring (Arup Chakrabarti) c) Endpoint of endocyclophotocoagulation (Vanita Pathak Ray) d) WY Incision SICS Trabeculectomy (Kirti singh) e) Sir Harold Ridley (IOL Inovator) Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022 iii

DOS Executive Members 2022-2023 Dr. Subhash C. Dadeya DOS Office Bearers Executive Members DOS Representative to AIOS Ex-Officio Members Dr. Pawan Goyal Dr. Rohit Saxena Vice President Dr. Rajendra Prasad President Dr. Jitender Singh Bhalla Secretary Dr. Sandhya Makhija Joint Secretary Dr.Alkesh Chaudhary Treasurer Dr. Kirti Singh Editor Dr. Jitender Bali Library Officer Dr. J.S. Titiyal Dr. M. Vanathi Dr. Namrata Sharma Dr. O. P. Anand Dr. Gagan Bhatia Dr. Vivek Gupta Dr. Vivek Kumar Jain Dr. Prafulla Maharanaa Dr. Amar Pujari Dr. Bhupesh Singh Dr. Pankaj Varshney iv Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022

Editorial My grandmother was operated for cataract at the ripe age of 65. She was subsequently prescribed high plus glasses for her aphakia. A petite, fragile lady she used to search her for glasses to use the washroom at night and often called out to us to help her. She was very uncomfortable with the cumbersome weight of her spectacles, and often left them lying around. Gradually she resigned herself to her minimal near vision, misty distant vision and discarded her glasses, till she went to meet her maker. I never understood the enormity of her problem till I read the self-described problems faced by a bilateral aphake Dr Alan Wood “This infirmity cannot be cured, it must be endured,while describing the multitude of difficulties ensuing out of optical aberrations, magnifications and field limitations. The will to perform cataract surgery better, and do away with the disabling aphakia , led Sir Harold Ridley to implant the first intraocular lens in November 1949. This landmark innovation in the face of tremendous odds, even hostility led to abolishment of aphakia and transformed lives of millions. The need to see better has always been the guiding force of all surgical innovations in ophthalmology, as evident by cataract surgery being the earliest documented surgery. Couching evidence exists in Egyptian and Indian history from tomb of King Khasekhemwy 2700 BC to Sushrut Samhita 600 BC. Innovations in cataract surgery have traversed a quantum jump with Jacques Daviel’s extra capsular cataract surgery in 1747, to safer intra-capsular cataract extraction by Samuel Sharp in 1753, followed by even safer alpha-chymotrypsin ICCE by Joaquin Barraquer in 1957. Cryoextraction ruled in 19th century, till the 1970s which saw the re-emergence of ECCE. Phacoemulsification pioneered by Charles Kelman in 1967 after an epiphany moment after getting his tooth drilled by his dentist, remained shrouded in obscurity till the innovation of continuous circular capsulorrhexis by Gimbel and Neuhan in 1991. Ophthalmic viscosurgical devices isolated by Balazs in 1934, from rooster combs required extensive refinements prior to intraocular use in human eye by 1976, with less expensive alternative, methylcellulose used by Fechner a year later. William Blake profound statement “What is now proved was once only imagined”has proved truly prophetic for cataract surgery Cataract surgery has evolved into a procedure, with expectations of spectacle independence. The humble PMMA IOL inserted by Dr Ridley, at one point labelled as the ‘time bomb’ in eye has undergone various avatars from aspheric, multifocal, toric, extended depth focus to adjustable lens. Cataract surgery has evolved to be the most predictable, safe and effective surgery thanks to the innumerable innovators working to live up to Arthur C Clarke famous statement “ The only way of discovering the limits of the possible is to venture a little way past them into the impossible. ” This issue of Delhi Journal of ophthalmology with theme of “I innovations (surgical)” seeks to detail few of the surgical innovations in ophthalmic surgery which we now take for granted.. Orbital reconstruction with electromagnetic navigation, computer aided manufacturing (CAM) using digital imaging and communications in medicine (DICOM) files translating into three dimensional customized orbital reconstruction. Innovations in ocular biometry, IOL power calculation, intraoperative aberrometry, ultrasonic aspirator and minimally invasive strabismus surgery techniques. Glaucoma surgery modifications to reduce the inherent complications of trabeculectomy or find the safer anti-fibrotic. Vitreo-retinal surgery innovation from Kasner open sky vitrectomy, to 25 G vitrectomy, hypersonic vitrectomy and robotic surgery Accepting innovations as the ideal way to surgical growth, Idea/ motivation development exploration assessment long term follow up (IDEAL) framework has been accepted as the structured approach to surgical innovations. The components being phase 1 including (case report/series including detailed explanation) to phase 2 a (technique stability) and finally 2b (multi-centric, prospective cohort study). Ophthalmology has been privileged to be the surgical discipline with an avalanche of surgical innovations over the past few decades, all with the aim of improving visual recovery with precision and safety. No one puts it better than the master innovator Thomas Edison - There is a way to do it better. Find it. This issue brings forth few surgical innovations follow this dictum. Yesterday’s Magic is Today’s Science © 2023 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow 1

Dr. Kirti Singh MD, DNB, FRCS, FAIMER, DHA Editor, Delhi Journal of Ophthalmology Dir Prof and Director GNEC, State Nodal Officer NPCB Guru Nanak Eye Center, Maulana Azad Medical College and Associated Hospital’s Ranjit Singh Marg, New Delhi, India. E-mail: [email protected] References • Woods AC. The adjustment to aphakia. Br J Ophthal 1964, 48, 349 • Gimbel HV, T Neuhann. Cataract Refract Surg 1990; 16(1):31-7. Development, advantages, and methods of the continuous circular capsulorhexis technique • Balazs EA, Hutsch E. In: Irvine AR, O¥ Malley C, eds. Replacement of vitreous with hyaluronic acid, collagen and other polymers. Advances in vitreous surgery. Springfield, Illinois: Thomas; 1976; 601-623. • Fechner PU, Fechner MU. Methylcellulose and lens implantation. Br J Ophthalmol. 1983; 67:259-263 This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. Cite This Article as: Singh K. Yesterday’s magic is today’s science. Delhi J Ophthalmol 2022;32:1-2. Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/dljo.dljo_53_23 2 Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022

Guest Editorial “Alice had begun to think that very few things indeed were really impossible.” ― Lewis Carroll, Alice in Wonderland The Oxford dictionary defines innovation, noun, as the introduction of new things or ideas. Humankind has evolved from hunters and cavemen to architects of great civilizations thanks to their ability to innovate. While the search and research involved may be a conscious effort, the actual innovation is most often an outcome of chance, an unexpected turn of events and a product of desperate times. How then does one strive to invent and innovate? The Base Matter “Begin at the beginning,” the King said, very gravely, “and go on till you come to the end: then stop.” ― Lewis Carroll, Alice in Wonderland To innovate, one must have a clear understanding of the principles of existing tools and machinery, for instance the slit lamp, the ophthalmoscope and a retinoscope. This would entail understanding the physics of optics and refraction, something that all ophthalmologists learn in school, medical school and first year of residency training. In addition, a thorough knowledge of the anatomy and physiology of each structure of the eye is necessary to develop a new surgical technique. Only then, one can experiment on live tissue without the fear of harmful consequences. The Past Speaks “Dear, dear! How queer everything is to-day! And yesterday things went on just as usual. I wonder if I’ve been changed in the night?” ― Lewis Carroll, Alice in Wonderland The coin of the past has two sides to it, one that describes the steps or process of invention of a new technique, instrument or drug with potential advantages and benefits and the other side where problems, and faults become evident. Whichever way the coin faces, it works for us because one gets to learn why a method lasted for years and why a technique became obsolete. Unless one knows what has been tried in the past, one is likely to make the same mistakes. On the other hand, a good and standard method can be built or improved upon to make it more accessible, affordable, and amenable for use. Going back to the past is often like going down a rabbit hole, where one article leads to another, including previously published original works describing new surgical techniques, commentaries and letters to editors and subsequent change in ideology with more recent original works. But unless one takes the leap, Wonderland is likely to remain a thing of tales. A Team Works “‘Well, now that we have seen each other,’ said the Unicorn, ‘if you’ll believe in me, I’ll believe in you. Is that a bargain?’” ― Lewis Carroll, Alice in Wonderland At a time when connectivity is the most powerful tool in the world, it is ironical that most people suffer from loneliness. It is impossible to find answers to all questions and doubts by oneself and one should not shy away from asking for help. A scientific paper is never the work of a single author and every authorship form requires a clear description of the contribution of each author. Discussing with peers, juniors and mentors is a refreshing process that stimulates the brain to think of possibilities and give shape to random fleeting ideas. This includes reaching out to people outside your own field, like radiologists, physicists, geneticists, biomedical engineers, pharmaceutical companies, optometrists, and doctors from other specialties. The Ruthenium plaque that was developed in India for treatment of eye cancers was a collaborative effort of Ophthalmologists, physicists, oncologists, and radiologists. A Learners Guide to Galaxy “Curiouser and curiouser!” ― Lewis Carroll, Alice in Wonderland Today, most of us have access to unlimited journals with rich contents. The Indian Journal of Ophthalmology has a section on ‘Innovations in Ophthalmology’. Attending conferences, both physical and virtual allow interaction with senior and eminent ophthalmologists and there is always a section on recent advances in every seminar. Journal clubs, regular case presentations and discussions within an institute are helpful for the students. Keeping oneself abreast about the new trials, pilot studies, future potentials is a simple way to ensure a dynamic learning process with constant stimulus. An active mind with a bit of creativity is one of the most powerful tools which can be harnessed with the right amount of inspiration. All it takes is a little spark to light a fire. Innovations: The Eureka Euphemism © 2023 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow 3

Keep to Safe, Keep it Simple “Speak English!” said the Eaglet. “I don’t know the meaning of half those long words, and, what’s more, I don’t believe you do either!” ― Lewis Carroll, Alice in Wonderland The pandemic was an ugly teacher. But it did bring out the scientific best in many doctors by simply restricting the options available. For an idea to be accepted widely, it is imperative that the raw materials are easily available at a reasonable cost and the final benefit clearly surpasses the effort or time that was required in its procurement. The utilization of smartphone cameras for clinical photography and screening of some eye diseases, smartphone based applications for ophthalmologists, and advancement in teleconsultation are very simple yet unique inventions that are being inculcated globally, both in the developed and the developing countries, in daily practice. Understanding the need, and the problem and then working towards finding a solution is a logical path to follow. On its way, one may stumble upon a solution. For doctors, science is the friend, philosopher and guide, but keeping a pocketful of imagination is surprisingly helpful. If You Fall, Get Up “I don’t see how he can ever finish, if he doesn’t begin.” ― Lewis Carroll, Alice in Wonderland Everybody fails, but everybody does not persist. A lot of doctors are willing to try new technologies, techniques and learn new skills. But very few are willing to retry. For every article that is categorized as Clinical Trial, it may be prudent to label them as Clinical Trial and Error. That leaves the scope for improvement and further studies instead of facing rejection at the initial stages. But till that happens, it will be the survival of the fittest. The history of corneal transplant is smeared with failures before it became the most successful transplant surgeries. One must be prepared to fail but determined to attempt again. India has been recognized as the hub of frugal innovations with the coining of the term ‘Indovation’. (1) Process reconfiguration, Gandhian innovations and frugal engineering are some of the approaches leading to these low-cost, high quality innovations. (2) While there are several existing sustainable and affordable models of healthcare delivery like the Aravind Eye Care system and the L.V.Prasad system, and there are many new developments in providing low cost intraocular lenses, a substantial amount of potential remains untapped. There is a need for affordable innovations in low vision aids, compact and transportable biometers, tonometers, ultrasound and OCT machines, cameras, glaucoma drainage devices, implants, drugs (target, biosimilars, chemotherapeutic) and surgical instruments. A large part of the incentive comes from non-profit sector. A more dedicated effort from the corporate and government sectors can give the necessary boost to innovative ophthalmologists to create, patent and market products. “It takes all the running you can do, to keep in the same place. If you want to get somewhere else, you must run at least twice as fast as that!” ― Lewis Carroll, Alice in Wonderland Dr. Santosh G Honavar MD, FACS, FRCOphth Santosh G Honavar, Centre for Sight, Road No. 2, Banjara Hills, Hyderabad India. E-mail: [email protected] References 1. Honavar SG. “Indovation” in ophthalmology – The potential power of frugal innovations. Indian J Ophthalmol 2019;67:447-8 2. 2. https://theprint.in/opinion/aravind-eye-care-to-jaipur-foot-india-didwell-in-frugal-innovation-but-new-innovators-needed/782927/ This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. Cite This Article as: Honavar SG. Innovations: The eureka euphemism. Delhi J Ophthalmol 2022;32:3-4. Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/dljo.dljo_54_23 4 Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022

Abstract Review Article Introduction Descemet’s membrane (DM), the basement membrane for the corneal endothelial layer, of thickness 8-10µm, is secreted by single layer of squamous epithelial cells that compose the endothelium. This endothelial layer maintains the corneal transparency. Any insult to this layer resulting in its damage necessitates a corneal posterior lamellar transplant (endothelial transplantation – Descemet’s stripping endothelial keratoplasty (DSEK) / Descemet’s stripping automated endothelial keratoplasty (DSAEK) or Descemet’s membrane endothelial keratoplasty (DMEK). DM morbidity1 can occur due to multiple factors that include a) Hereditary conditions such as Fuch’s endothelial dystrophy (FECD); Posterior polymorphous dystrophy b) DM detachments (DMD) may be either spontaneous (dystrophy, hypotony) or iatrogenic c) Traumatic - birth trauma, injuries d) Toxic - deposits of copper as in Wilson’s disease. DMD was first identified by Weve and co-workers in 1927.2 The most common etiology of DMD is the accidental iatrogenic damage to DM during intraocular surgery. DMD occurs in almost 43% of cases after cataract surgery 3 and also during other surgical interventions such as iridectomy, keratoplasty, vitrectomy, trabeculectomy, sclerotomy etc.4 The reported incidence of DM in extra capsular cataract extraction is 2.6% and in phacoemulsification is 0.52%.5 Left untreated, DMD can lead to corneal opacification and subsequent loss of vision. Hence, it is imperative to understand the pathogenesis, clinical presentation, early recognition of DMD and its management strategies. Clinical anatomy: DM has an anterior banded zone (2-4µm) and a posterior non-banded zone (>4µm). It is composed of collagen types IV and VII along with non-collagenous components such as fibronectin, laminin, nidogen, vitronectin keratin, dermatan and chondroitin sulphate proteoglycans.6 DM does not have a capacity to regenerate after any injury. DM is attached to the posterior stroma by an amorphous zone of extracellular matrix known as interface matrix, which is about 0.5 to 1µm thick and contains randomly arranged collagen fibrils and proteoglycans. This layer is of significant interest during DMEK procedure. DMD can occur either spontaneously Descemet’s membrane is basement membrane of corneal endothelial cells, which in turn are responsible for maintaining corneal transparency. Numerous causes can lead to Descemet’s membrane detachment (DMD), with the most common etiology being post-surgical. Older age, blunt instruments and faulty technique predispose to DMD. DMD management is an emergency in order to avoid permanent damage leading to scarring of cornea. Various classifications of DMD and management protocols have been described. DMD should be suspected in cases of unexplained edema after surgery. Anterior segment optical coherence tomography (ASOCT) is the most important investigation to pick up DMD and also quantify its extent. Desmetopexy with air/gas is initial treatment of choice, could be aided by viscoelastic/suture fixation. Non responsive cases might need endothelial keratoplasty. Keywords: Descemet’s membrane detachment, anterior segment optical coherence tomography, HELP algorithm Address for correspondence: Murugesan Vanathi, MD, Cornea, Lens and Refractive Surgery Services, Dr. R.P. Centre for Ophthalmic Sciences, AIIMS, New Delhi, India. E-mail: [email protected] This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected] Cite This Article as: Beniwal A, Venugopal A, Vanathi M, Tandon R. Descemet’s membrane detachment - An update. Delhi J Ophthalmol 2022;32:5-12. Descemet’s Membrane Detachment - An Update Abhijeet Beniwal1 , Anitha Venugopal2 , Murugesan Vanathi1 , Radhika Tandon1 1 Department of Cornea, Lens and Refractive Services, Dr. R.P. Centre, AIIMS New Delhi, India, 2 Department of Consultant and Cornea Incharge, Aravind Eye Hospital, Tirunelveli, Tamil Nadu, India Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/dljo.dljo_55_23 Submitted: 01‑Jul‑2022 Accepted: 06‑Nov‑2022 Published: 31-May-2023 © 2023 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow 5

or by iatrogenic trauma due to abnormality in this interface matrix and its attachment with the posterior stroma. Etiology The etiologies for DMD can be varied, may occur due to the following causes 1) Genetic and Congenital causes: a) Fuch’s corneal endothelial dystrophy, b) TGF-beta gene mutation, c) Intrinsic abnormality of the interface matrix, 2) Acquired ocular disorders such as, Age >65 years with low endothelial count, eyes with Pseudoexfoliation syndrome, eyes with primary angle closure glaucoma are prone for DMD when intervene for cataract surgery 3) Iatrogenic factors: Use of blunt microsurgical instruments7,8 improperly structured corneal entry incisions, novice surgeons, postoperative persistent hypotony following over-filtering bleb in trabeculectomy procedures. 4) Idiopathic: DMD has been reported in healed keratitis patient where no previous intraocular surgery has been performed.9 Pathogenesis Pathogenesis of DMD is elaborated in the (flowchart 1). The mechanisms that result in occurrence of DMD differs in various intraocular procedures include: 1) Following cataract surgery: DMD occurs most commonly following cataract surgery (in both small incision cataract surgery (SICS) and phacoemulsification procedure. DMD commonly arises from traumatic tears at the incision site. The reported incidence of vision threatening DMD after extracapsular extraction and phacoemulsification was 2.6% and 0.52% respectively 4, 5. The surgical risk factors include usage of blunt instruments, inadvertent insertion of instruments in between corneal stroma and Descemet membrane, inappropriate incisions such as oblique, too anterior incisions and shelved incisions, tight side port incisions or the phacoemulsification tunnel incisions, that do not fit the cannula or the phacoemulsification probe respectively, inadvertent injection of ocular viscoelastics or the intracameral antibiotics in-between DM and stroma, handling sharp instruments in shallow anterior chamber and the inexperienced surgeon.1 Higher total ultrasound time and higher cumulative dissipated energy are also risk factors.10 Preoperative risk factors such as primary endothelial disorders can predispose to spontaneous DMD even in uneventful cataract surgery. The decision making for treatment depends on the proper preoperative slit lamp assessment and ASOCT images of the DM tear margins, area of involvement, origin of the tear margins, distance of DMD from the stroma and the associated intraoperative complications aids in management of the DMD following cataract surgery. 2) Following keratoplasty: Spontaneous DMD has been reported after PK (20 years after surgery for keratoconus)11 and DALK for corneal ectasia.12 Mechanical pulling effect of presence of retro corneal membrane along the graft-host junction, on the DM, and progressive thinning and ectasia at the graft-host interface have been hypothesised to cause spontaneous DMD. DMD following DALK can occur due to micro/macro perforations during host corneal dissection, retained DM of the donor cornea,13,14 and unrecognised type 2 bubble. 3) Following trabeculectomy: Early diagnosis and management of DMD in post glaucoma procedures are challenging compared to cataract surgery. The causes include- accidental insertion of instruments between stroma and DM, blunt keratotomies, shallow anterior chamber, hypotony, inadvertent injection of saline or viscoelastic between stroma and DM, pre-disposed DM interface abnormalities would lead to DMD. Globus DMD have been reported after trabeculectomy along with choroidal effusion, with failed two attempts of DM reposition with intracameral air.15 Finally, DMD resolved with Transconjunctival closure of scleral flap with 10/0 nylon sutures, choroidal tap and intracameral injection of 20% sulphur hexafluoride. 4) Following chemical injury: Mostly DMD occurs due to inflammation and fibrosis in the anterior chamber. The mechanisms include, Tractional DMD due to severe cellular damage in the stroma and endothelial layer, formation of inflammatory retro corneal membrane associated with organizing blood clots, causing Tractional effect on the DM, the rupturing of neovascularisation leads to haemorrhagic DMD. Spontaneous DM resolution has been reported in such cases when diagnosed early, also with 20% SF6 injection.16 5) Following Birth trauma: DM tears occurs following forceps injuries. The mechanism includes, attempted forceps delivery, compression of globe against the orbital roof- slipping of forceps blade over the inferior orbital edge and thus typically vertical and linear tears.17 The symptoms include corneal edema since after birth, may resolve spontaneously with hypertonic saline in few cases, or can be managed with intracameral air, superior iridectomy and penetration of the overlying cornea with the needle of a 10-0 nylon suture and release of viscous fluid. The signs of DMD are unilateral corneal edema since after birth, localised to the area of tear, tears are linear vertically long involves central and midperipheral cornea. The complications are astigmatism, amblyopia and high myopia.18 6) Following interstitial keratitis: Most commonly, haemorrhagic DMD occurs due to corneal neovascularisation in syphilis patients. The management involves multiple steps, first: surgical drainage of the blood through corneal incision and then second: intracameral air tamponade to facilitate reattachment of DMD.19 6 Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022

7) Following radial keratotomy (RK): RK cause endothelial damage and corneal decompensation, reports show that the rate of endothelial loss from 3.3% to 0.4% every year at the 7-year follow-up. Spontaneous DMD was reported in a 43-year-old man, 20 years after RK, with spontaneous resolution of DM occurring after eight months. The DMD occurrence has been attributed to corneal dialysis through RK incisions.20 Morphological Classification of DMD DMD has been classified depending upon its morphology of extent, height of detachment from stroma, area of cornea involved and its position with respect to the pupil. 1) Samuel Classification (1928) is based on pathological features.21 This DMD classification given in 1928 described DMD as either active (pushed back) or passive (pulled back and torn away) due to differences in elasticity between the parenchyma and the glass membrane. Samuel also stated that this classification was more relevant pathologically, and no particular importance could be ascribed to these forms of detachment from the surgical standpoint. 2) Mackool and Holtz classification (1977) is based on the height of detachment from the stroma described the DMD as planar or non-planar (Table 1).22 Both planar and non-planar detachments may be further subdivided into (a) peripheral and (b) combined peripheral/ central detachments. 3) Jain Classification (2013) is based on the extent of DMD (Table 2).23 4) Jacob classification (2015) is based on the etiology, clinical features, anterior segment optical coherence tomography (ASOCT) findings, intraoperative factors and management protocol (Table 3).24 5) HELP Algorithm (2015) is based on Height, Extent, Chord Length and Relation to the Pupil (Table 4).7 Management strategies have been recommended based on these 4 parameters 6) Samarawwickrama (2016) Classification: In 2016, Samarawwickrama et al classified DMD depending on the involvement of the visual axis as peripheral and central DMD and proposed management guidelines based on this.25 Clinical Characteristics Unexplainable/persistent corneal edema following intraocular surgeries such as cataract, keratoplasty, glaucoma surgeries and radial keratotomy leads to the detection of DMD on careful slit lamp examination. The common symptoms include delayed or poor visual improvement, foreign body sensation and watering. The clinical signs of DMD (Figure 1) include: - Localized corneal edema with a demarcation line separating oedematous from non-oedematous clear cornea would be seen in slit-lamp - Double AC formation - The DMD margins would be seen with the narrow-slit lamp beam DMD occurrence has been reported at occur at one month to 30 years post-operatively (after penetrating keratoplasty) 1,26. The progressive corneal edema, usually tends to obscure details of the detached DM. DMD, if localised, may spontaneously reattach. DMD involving < 50% of the cornea, may clear with resultant polymegathism, and subsequent scaring of the DM (Figure 2). ASOCT imaging is a useful tool to recognise the extent and the direction of DMD especially in cases with poor corneal clarity due to edema (Figure 3-5). Permanent corneal decompensation would result in poor vision, with continued discomfort from watering and foreign body sensation due to bullae formation. Timely intervention is required to mitigate corneal morbidity. Early intervention for endothelial damage with endothelial transplantation procedures (DSEK/DSAEK or DMEK) affords good visual rehabilitation in eyes with visual potential. Long-standing corneal edema with repeated bullae rupture leads to anterior corneal scaring necessitating penetrating keratoplasty. Investigation Poor visibility of the anterior chamber in eyes with suspected DMD due to corneal edema requires investigative imaging modalities to diagnose, treat and monitor the patient with DMD. 1) Anterior segment optical coherence tomography (ASOCT): ASOCT enables high resolution cross-sectional images of the anterior segment through the relatively hazy cornea and minimal experience is required for image acquisition. Large DMD in DALK surgery can be settled with microscope integrated intra-operative -OCT aided Descemetopexy with interface fluid drainage. DMD classifications based on ASOCT imaging enabled detailed outlining of management. Figure 1: Corneal edema in a case of post trabeculectomy with shallow Descemet’s Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022 7

The HELP algorithm elaborates the recommended intervention in accordance to the extent and height of the DMD.9 2) Ultrasound biomicroscopy: Ultrasound biomicroscopy (UBM) is also a useful imaging tool in accurately locating DMD and guiding the DM repair, but is less often preferred to ASOCT.27 The major limitations include the need for a skilled technician, is a contact procedure requiring patients’ cooperation and is time consuming. Comparison of the efficacy in diagnosing anterior chamber angle structures with ASOCT and UBM observed ASOCT to be superior, being a non-contact and faster procedure providing multiple higher resolution images of superior image quality. 3) Gonioscopy in DMD: Gonioscopy can be of useful tool in peripheral DMD to assess the extent, length and height of DMD 3. However, the associated corneal edema usually tends to limit its use. Management Decision making in the management of DMD depends on the careful preoperative evaluation of the age of the patient, etiology of DMD, extent, height, length and as well as location of detachment. Timely management and prompt postoperative care is needed for best visual outcome. Management of DMD is largely a case-based approach. The recommendation in accordance to the HELP algorithm 9, is as follows: a) DMD with length<1mm, height <100um in any zone: medical management b) DMD with length 1-2mm, height 100-300um in zone 2 and 3: surgical management c) DMD with length > 2mm, height > 300um in zone 3: surgical management d) If medical therapy fails at 4 weeks: surgical management Medical Management in DMD is aimed at decreasing corneal edema and inflammation. Topical therapy with hyperosmotic agents, anti-inflammatory and anti-glaucoma agents is initiated. Surgical intervention comprises of (i) Intracameral gas injection (non-expansile) (C3F8 14% or SF6 20% injection) or sterile air injection was undertaken or (ii) Descemetopexy with sutures (Figure 6) Figure 2: Old Descemet detachment with scarring. a) Conservative Medical Management: Figure 3: ASOCT of the patient of figure 1 showing shallow DMD extending over a large area. 8 Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022

The conservative therapy includes, topical steroids, hyperosmotic agents (hypertonic saline 5% drops, and 6% ointment). Topical steroids (Prednisolone acetate 1% eye drops) help in controlling the inflammation and reduces the risk of developing DM fibrosis and scar. Hypertonic saline eye drops imbibe fluid from the corneal stroma and help in dehydrating the cornea. This improves the vision transiently. Medical management is indicated in small, peripheral, planar DMD with non-scrolled edges, in young patients. DMD of height <100mm, limited to a small area will resolve with conservative medical management. Prospective study on 116 cases of DMD, reported a success rate of 96.9% in the group managed with medical therapy in comparison to surgical treatment.7 Eyes with DMD persisting for more than 4 weeks have been recommended to be considered for surgical intervention. b) Surgical: Surgical intervention modalities that have been described in the management of DMD 1 includes: 1) Descemetopexy with air/ gas (SF6/C3F8) / Suture 2) Mechanical tamponade with intracameral sodium hyaluronate 3) Manual reposition 4) Descemetotomy 5) Interface fluid Drainage 6) Endothelial keratoplasty – DSEK/ DSAEK or DMEK procedure Descemetopexy Sparks et al. in 196728 first described the reattachment of DMD with injection of air in the anterior chamber in three eyes following cataract surgery. The rapid absorption of air and need for retention of tamponade for extended period of time led to the concept of using iso-expansile gases, such as 2-14% C3F8, 14-20% SF6, which would remain in the anterior chamber providing an effective tamponade for 6-8 weeks and 2 weeks respectively. Viscoelastics29 and mattress sutures30 have been used to aid in the fixation of the detached Descemet’s membrane to the posterior cornea along with using air. Indications for preferring the use of gases over air includ.31- 33 1) Inferior non-planar DMD 2) Superior DMD with scrolled edges 3) Central planar or non-planar DMD 4) Long standing DMD 5) Taut DMDs following fibrosis and inflammation The advantages of using gases instead of air are that: - Gas reduces the need for repeated injections - Gas stays in anterior chamber for extended period of time till the endothelium becomes functional Disadvantages with the use of gas-Descemetopexy are: - Endothelial dysfunction has been reported34,35 in animal studies - Raised intraocular pressure and pupillary block glaucoma - Uveitis Surgical technique: Entry incision into the anterior chamber is made opposite or away to the site of DMD or the area where DM is intact. Syringe filled with continuous gas or the sterile air is injected into the anterior chamber slowly and steadily until the reattachment of DM is enabled and is maintained for at least 15-20 minutes. The drainage of pre-Descemetic fluid is done by intermittent ironing of the overlying with the cannula or coupled with stab incisions. Depending on the surgeon’s choice, 1/3rd of the gas or the air can be released to avoid pupillary block. The side port closure can be done with either sutured or stromal hydration. Preoperative peripheral iridotomy or intraoperative peripheral iridectomy is indicated Figure 4: ASOCT of the patient in figure 2 showing central DMD. Figure 5: ASOCT of old Descemet’s detachment in a patient post cataract surgery with pseudophakic bullous keratopathy Figure 6: DMD managed with suture fixation Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022 9

to prevent pupillary block. It is important to maintain the supine position after the procedure at least for 24 hours. Postoperative treatment with topical steroids (dexamethasone eye drops), antibiotics, and cycloplegics is advised. Retrospective study of 112 cases of DMD with air alone reported 71% success rate, with 15 cases requiring re-surgery of which 60% were successfully reattached.1 One patient with persistent edema underwent endothelial keratoplasty. No difference was observed between air versus gas descemetopexy. Appositional angle closure (18%), pupillary block (2.1%) and uveitis (2.7%) were the complications noted. Gas tamponade is advised in cases where air descemetopexy has not been successful. Suture fixation: Trans corneal suture fixation with 10-0 monofilament nylon suture may be combined with gas or air descemetopexy in the management of DMDs. This is preferred in indications such as 36,37 1) Large DMD 2) Recurrent DMD with primary pathology of the cornea 3) Intractable DMD in post cataract surgery patients 4) In cases of complicated cataract surgery that was associated with intraoperative complications such as posterior capsular rupture, dislocated IOL, aphakia, zonular dialysis etc. Advantages - Large peripheral DMD can be easily managed - No risk of pupillary block - If combined with air/gas, no necessity to do complete air filling of AC Disadvantages - Risk of suture related infection and endophthalmitis - Variable and unpredictable success - Risk of tenting of DM while suturing - Risk of induced corneal astigmatism - Management of central and large DMD without the aid of air or gas is tedious Manual repositioning Manual repositioning of DMD is not advisable in recent times due to associated risk of injury to endothelial cells. This procedure has been described for unscrolling the edges of taut DMD with the iris spatula and cyclodialysis spatula followed by injection of air/gas.28,38 Descemetotomy Lowenstein et al 39 described Descemetotomy in 1993 in post keratoplasty eyes with retained host DM. Descemetotomy can be achieved with either the surgical removal of membrane or creation of break in the host DM with the help of Nd-YAG laser. Interface fluid drainage Drainage of interface fluid between DM and stroma is done using 20-gauge micro-vitreoretinal blade, 23-gauge needle and curved needle of 10-0 monofilament suture for drainage. The success in drainage of interface fluid can be monitored and confirmed intraoperatively with intraoperative microscope integrated OCT 13. Intraoperative OCT assisted descemetopexy with stromal vent incisions (to allow for the egress of large fluid collection between the detached DM and posterior stroma) along with intracameral gas injection helps in resolution in cases of nonresolving Descemet’s membrane detachment40 (Figure 7) DSAEK DSAEK is considered in cases of DMD with damaged endothelium and enables visual improvement. Indications: - Failure of repeat procedures of descemetopexy - Progressive endothelial decompensation - Persistent corneal edema after successful reposition of DM indicating damaged corneal endothelium - Failure of post keratoplasty DMD reposition with air/gas DSEK can provide a successful outcome in patients with extensive DMD (>70% area) after phacoemulsification. Long standing corneal edema leads to anterior stromal scarring which compromises the visual acuity following endothelial keratoplasty procedures. Visual outcomes of BCVA >20/40 has been reported to be achievable in early intervention DSEK, within 6 months of cataract surgery for persistent corneal edema following cataract surgery.41 Descemet Membrane Endothelial Keratoplasty (DMEK) DMEK is favoured by most of the corneal surgeons due to its exceptional visual and refractive outcomes and rapid visual recovery. Fuchs endothelial dystrophy and pseudophakic bullous keratopathy are the main indications for DMEK. DMEK grafts are prepared just prior to the transplantation. The DM is separated from the scleral spur with a hockey stick knife Figure 7: Intraoperative OCT aided intrastromal vent incisions with intracameral air injection in management of refractory bullous DMD 10 Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022

and then with slow and steady movement, the DM is stripped from the stroma with the McPhersons forceps. The DM is trephined with 7.0 to 8.0mm punch, separated and aspirated into the modified Jones tube and inserted into the anterior chamber after descemetorhexis. After checking the correct orientation with the Moutsouris sign, the graft is unfolded inside the anterior chamber with fluid and air.42 In patients with DMD, the decision of DMEK becomes controversial in patients with reasonable visual acuity. Cases scarred and taut DM, and failed descemetotomies and recurrent DMD with progressive corneal decompensation are definite indications for DMEK. Conclusion DMD is a vision-threatening complication following cataract surgery. Though several treatment strategies are available, decision making on conservative management, medical or surgical treatment depends on the preoperative evaluation of the height, length and extent of the detachment, as well as location. Intraoperative protection of endothelium with soft shell technique and appropriate technique of corneal incisions, instrument insertion into the anterior chamber and use of sharp instruments will help to prevent the occurrence of DMD. AS-OCT imaging can confirm the presence and the extent of DMD in cases with hazy oedematous corneas and helps in determining appropriate line of therapeutic intervention. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. References 1. Odayappan A, Shivananda N, Ramakrishnan S, Krishnan T, Nachiappan S, Krishnamurthy S. A retrospective study on the incidence of postcataract surgery Descemet’s membrane detachment and outcome of air descemetopexy. British Journal of Ophthalmology. 2018 Feb 1;102(2):182-6. 2. Weve, H.: Separation of the Membrane of Descemet After Extraction of Lens, Nederl Tijdschr v Geneesk 2:398, 1927. 3. Monroe WM. Gonioscopy after cataract extraction. South Med J. 1971;64:1122-4. 4. Marcon AS, Rapuano CJ, Jones MR, Laibson PR, Cohen EJ. Descemet’s membrane detachment after cataract surgery: management and outcome. Ophthalmology. 2002 Dec 1;109(12):2325-30. 5. Khng CY, Voon LW, Yeo KT. Causes and management of Descemet’s membrane detachment associated with cataract surgery--not always a benign problem. Annals of the Academy of Medicine, Singapore. 2001 Sep 1;30(5):532-5. 6. Bourne WM, Johnson DH, Campbell RJ. The ultrastructure of Descemet’s membrane: III. Fuchs’ dystrophy. Archives of ophthalmology. 1982 Dec 1;100(12):1952-5. 7. Kumar DA, Agarwal A, Sivanganam S, Chandrasekar R. Height-, extent-, length-, and pupil-based (HELP) algorithm to manage postphacoemulsification Descemet membrane detachment. Journal of Cataract & Refractive Surgery. 2015 Sep 1;41(9):194553. 8. Singhal D, Sahay P, Goel S, Asif MI, Maharana PK, Sharma N. Descemet membrane detachment. Survey of ophthalmology. 2020 May 1;65(3):279-93. 9. Beniwal A, Bafna R, Sharma N. Descemet detachment in a phakic patient. Indian Journal of Ophthalmology. 2020 May 1;68(5):9067. 10. Sharma N, Bandivadekar P, Agarwal T, Shah R, Titiyal JS. Incision-site Descemet membrane detachment during and after phacoemulsification: risk factors and management. Eye & Contact Lens. 2015 Sep 1;41(5):273-6. 11. Gorski M, Shih C, Savoie B, Udell I. Spontaneous Descemet membrane detachment 20 years after penetrating keratoplasty for keratoconus. Cornea. 2016 May 9;35(7):1023-5. 12. Lin X, Wu Y, Fu Y, Dai Q. Spontaneous reattachment of Descemet membrane detachment after deep anterior lamellar keratoplasty: A case report. Medicine. 2018 Feb;97(8). 13. Sharma N, Aron N, Kakkar P, Titiyal JS. Continuous intraoperative OCT guided management of post-deep anterior lamellar keratoplasty Descemet’s membrane detachment. Saudi Journal of Ophthalmology. 2016 Apr 1;30(2):133-6. 14. Vengayil S, Vanathi M, Panda A, Khokhar S. Anterior segment OCTbased diagnosis and management of retained Descemet’s membrane following penetrating keratoplasty. Contact Lens and Anterior Eye. 2008 Jun 1;31(3):161-3. 15. Sharifipour F, Nassiri S, Idan A. Descemet’s membrane detachment management following trabeculectomy. Journal of Ophthalmic & Vision Research. 2016 Jul;11(3):323. 16. Zhang B, Pan F, Yao YF. Spontaneous resolution of extensive Descemet membrane detachment caused by sodium cyanide injury to the eye. Cornea. 2012 Nov 1;31(11):1344-7. 17. Lloyd RI. Birth injuries of the cornea and allied conditions. Transactions of the American Ophthalmological Society. 1937;35:212. 18. Lambert SR, Drack AV, Hutchinson AK. Longitudinal changes in the refractive errors of children with tears in Descemet’s membrane following forceps injuries. Journal of American Association for Pediatric Ophthalmology and Strabismus. 2004 Aug 1;8(4):368-70. 19. Höllhumer R, Mz AZ, Watson S. Hemorrhagic Descemet membrane detachment following syphilitic interstitial keratitis. Cornea. 2016 Sep 1;35(9):1255-6. 20. MacRae SM, Rich LF. Long-term effects of radial keratotomy on the corneal endothelium. Journal of Refractive Surgery. 1998 Jan 1;14(1):49-52. 21. Samuels B. Detachment of Descemet’s membrane. Transactions of the American Ophthalmological Society. 1928;26:427. 22. Mackool RJ, Holtz SJ. Descemet membrane detachment. Archives of Ophthalmology. 1977 Mar 1;95(3):459-63. 23. Jain R, Murthy SI, Basu S, Ali MH, Sangwan VS. Anatomic and visual outcomes of descemetopexy in post-cataract surgery Descemet’s membrane detachment. Ophthalmology. 2013 Jul 1;120(7):1366-72. 24. Jacob S, Agarwal A, Chaudhry P, Narasimhan S, Chaudhry VN. A new clinico-tomographic classification and management algorithm for Descemet’s membrane detachment. Contact Lens and Anterior Eye. 2015 Oct 1;38(5):327-33. 25. Samarawickrama C, Beltz J, Chan E. Descemet’s membrane detachments post cataract surgery: a management paradigm. International journal of ophthalmology. 2016;9(12):1839. 26. Shalchi Z, O’Brart DP, Ilari L. Bilateral Descemet membrane detachment following cataract surgery. JAMA ophthalmology. 2013 Apr 1;131(4):533-5. 27. Radhakrishnan S, Goldsmith J, Huang D, Westphal V, Dueker DK, Rollins AM, Izatt JA, Smith SD. Comparison of optical coherence tomography and ultrasound biomicroscopy for detection of narrow anterior chamber angles. Archives of ophthalmology. 2005 Aug 1;123(8):1053-9. 28. Sparks GM. Descemetopexy: Surgical reattachment of stripped Descemet’s membrane. Archives of Ophthalmology. 1967 Jul 1;78(1):31-4. 29. Donzis PB, Karcioglu ZA, Insler MS. Sodium hyaluronate (Healon ®) in the surgical repair of Descemet’s membrane detachment. Ophthalmic Surgery, Lasers and Imaging Retina. 1986 Nov 1;17(11):735-7. 30. Amaral CE, Palay DA. Technique for repair of Descemet membrane detachment. American journal of ophthalmology. 1999 Jan 1;127(1):88- 90. Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022 11

31. Ellis DR, Cohen KL. Sulfur hexafluoride gas in the repair of Descemet’s membrane detachment. Cornea. 1995 Jul 1;14(4):4367. 32. Kremer I, Stiebel H, Yassur Y, Weinberger D. Sulfur hexafluoride injection for Descemet’s membrane detachment in cataract surgery. Journal of Cataract & Refractive Surgery. 1997 Dec 1;23(10):1449- 53. 33. Sharma A, Singh SK, Bhutia PL, Pant R. Perfluoropropane (C3F8) injection for Descemet’s membrane detachment in cataract surgery. Nepalese Journal of Ophthalmology: a Biannual Peer-reviewed Academic Journal of the Nepal Ophthalmic Society: NEPJOPH. 2015 Jan 1;7(1):74-8. 34. Lee DA, Wilson MR, Yoshizumi MO, Hall M. The ocular effects of gases when injected into the anterior chamber of rabbit eyes. Archives of Ophthalmology. 1991 Apr 1;109(4):571-5. 35. Landry H, Aminian A, Hoffart L, Nada O, Bensaoula T, Proulx S, Carrier P, Germain L, Brunette I. Corneal endothelial toxicity of air and SF6. Investigative Ophthalmology & Visual Science. 2011 Apr 1;52(5):2279- 86. 36. Kumar AM, Vaithianathan V. Descemet’s membrane detachment managed with perfluro-n-octane liquid. Indian Journal of Ophthalmology. 2012 Jan;60(1):71. 37. Benatti CA, Tsao JZ, Afshari NA. Descemet membrane detachment during cataract surgery: etiology and management. Current opinion in ophthalmology. 2017 Jan 1;28(1):35-41. 38. Das AB. Reposition of Descemet’s membrane after cataract extraction. Indian Journal of Ophthalmology. 1972 Mar 1;20(1):20. 39. Loewenstein A, Geyer O, Lazar M. Descemetotomy. Journal of Cataract & Refractive Surgery. 1996 Jul 1;22(6):652. 40. Singh A, Vanathi M, Sahu S, Devi S. Intraoperative OCT assisted descemetopexy with stromal vent incisions and intracameral gas injection for case of non-resolving Descemet’s membrane detachment. Case Reports. 2017 Jan 6;2017:bcr2016217268.. 41. Kim JJ, Kim HK. Descemet membrane stripping endothelial keratoplasty for Descemet membrane detachment following phacoemulsification. Canadian Journal of Ophthalmology. 2015 Feb 1;50(1):73-6. 42. Weissbart SB, Hammersmith KM, Ayres BD, Rapuano CJ, Nagra PK, Raber IM, Azari AA. Influence of early Descemet stripping endothelial keratoplasty on visual outcomes in pseudophakic corneal edema. American Journal of Ophthalmology. 2016 Dec 1;172:58-63. 12 Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022

Abstract Original Article Introduction Phacomorphic glaucoma is a type of secondary acute angle closure characterized by rapid increase in intraocular pressure (IOP) with shallowing of anterior chamber caused by mature or hyper-mature cataract. Phacomorphic glaucoma was called so historically, but “glaucoma” implies an optic neuropathy. Since, majority of phacomorphic glaucoma patients do not have glaucomatous optic neuropathy at presentation, so the term “phacomorphic angle closure” (PMAC) was proposed, which seems to be more appropriate.1 PMAC is less common in Western countries. In India, back in 1980s phacomorphic glaucoma was the cause of cataract extraction in 3.9 % of cases, more frequently among females.2 In PMAC, acute clinical presentation is typically preceded by gradual, painless decrease in vision. The initial management of PMAC is aimed at reducing corneal edema and IOP. The definitive treatment of PMAC is cataract extraction.3 Cataract surgery in setting of PMAC might be challenging, due to shallow anterior chamber and raised IOP from swollen lens. Thus, shortening the duration of attack, effective control of IOP and uncomplicated cataract extraction are keys to improve visual outcomes in these eyes. With evolution of techniques of cataract extraction, the preference has shifted from sutured extracapsular cataract extraction (ECCE) toward manual small incision cataract surgery (MSICS), and phacoemulsification in eyes with PMAC also.2,4,5 The present study was designed to evaluate and compare outcomes of cataract extraction in eyes Purpose: To compare the surgical outcomes of cataract extraction in eyes with phacomorphic angle closure by manual small incision cataract surgery (MSICS) and phacoemulsification. Methods: A prospective, randomized, comparative study included patients of phacomorphic angle closure (PMAC) between August 2018 to December 2019, at a tertiary care teaching facility in rural western Haryana of Northern India. Patients were randomly allocated to one of the two groups-MSICS group and PHACO group, for cataract surgery. Surgical outcomes were compared in terms of intraoperative events, visual acuity, intraocular pressure (IOP) and endothelial cell loss (ECL). Association between delay in presentation, IOP at presentation and surgical outcomes were studied. Results: The difference between surgical outcomes by MSICS and phacoemulsification in eyes with PMAC was not significant. The median un-aided visual acuity at final follow-up was 0.5. A total of 18 (56%) gained un-aided visual acuity of ≥0.5, six (37%) in MSICS group and 12 (75%) in PHACO group (χ2 4.57, p 0.03). The mean IOP at final follow up was 12.5mmHg, with no significant difference between two groups (p 0.7). ECL in MSICS group was 5.64 (±1.1) % and in PHACO group 5.89 (±1) % (p 0.5). Eyes with lower pre-operative endothelial cell count suffered higher loss (Spearman r 0.364, p 0.04). Conclusions: Both MSICS and phacoemulsification are safe and effective for cataract extraction in eyes with PMAC with anterior chamber depth (ACD) of 1.15-2.09 mm. Keywords: Phacomorphic angle closure glaucoma, small incision cataract surgery, phacoemulsification, endothelial cell loss Address for correspondence: Deepika Singh, MS, Department of Ophthalmology Maharaja Agrasen Medical College, Agroha (Hisar) Haryana - 125 047, India. E-mail: [email protected] This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected] Cite This Article as: Singh D, Rewri P, Aggarwal S. Comparison of surgical outcomes of cataract extraction by manual small incision cataract surgery and phacoemulsification in phacomorphic angle closure. Delhi J Ophthalmol 2022;32:13-8. Comparison of Surgical Outcomes of Cataract Extraction by Manual Small Incision Cataract Surgery and Phacoemulsification in Phacomorphic Angle Closure Deepika Singh, Parveen Rewri, Sagarika Aggarwal Department of Ophthalmology, Maharaja Agrasen Medical College, Agroha (Hisar) Haryana, India Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/dljo.dljo_59_23 Submitted: 10‑Nov‑2022 Accepted: 12‑Jan‑2023 Published: 31-May-2023 © 2023 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow 13

with PMAC by two contemporary techniques, namely MSICS and phacoemulsification. Methods A prospective, randomized, comparative study was done in department of ophthalmology of a tertiary care teaching institute between August 2018 to December 2019. The study was approved by Institutional Ethical Committee (Approval No-MAMC/Pharma/ICE/18/260) and adhered to tenants of declaration of Helsinki. Study protocols were discussed with eligible patients and an informed consent was taken for participation. The diagnosis of PMAC was based on a set of criteria including sudden onset of pain in eye with total/mature senile (aged ≥50 years) cataract with preceding history of painless, gradual diminution of vision, visual acuity limited to perception of light (PL) and accurate projection of rays (PR), raised intraocular pressure (>22 mmHg), shallow anterior chamber (Van Herick (VH) grade ≤II), occluded anterior chamber angles on gonioscopy in absence of significant reaction in anterior chamber (cells/flare < +2). Patients aged >50 years, of either gender, fulfilling the inclusion criteria were included in study (Figure 1). Eyes with corneal opacity precluding phacoemulsification, sub-luxated lens, complicated cataract and other types of lens-related glaucoma were excluded. Preoperative evaluation included slit-lamp examination, visual acuity assessment, Goldman applanation tonometry (L-5110, Inami, Tokyo, Japan), four mirror gonioscopy (Volk Optical, Inc.), endothelial cell count (ECC) (REM 3000; Tomey Corporation, Japan), automated keratometry (Topcon, Japan), axial length measurement (APPA SCAN- 2000, Appaasamy, India) B-scan (Appasamy Marvel II AB-scan with UBM, Appaasamy, India), angle imaging and lens vault evaluation (RS-300, Nidek Co Ltd., Japan). To reduce IOP and corneal edema a standard treatment protocol was followed, which included intravenous mannitol (20% w/v) after test dose of 75 ml, oral acetazolamide, topical IOP lowering eye drop and topical prednisolone (1%) eye drop. Once IOP decreased below 22 mm Hg and cornea became clearer, patient was taken-up for surgery. Eligible patients were invited to participate in study and an informed consent was taken. Patients were randomized into one of the two groupsMSICS group, cataract extraction was done by manual small incision cataract surgery (MSICS), and PHACO group, in which patients underwent phacoemulsification. Consecutive patients were alternately allocated to each group starting from MSICS group. MSICS and phacoemulsification were performed by surgeons, proficient in respective techniques of cataract extraction. All surgeries were done under peribulbar anesthesia. Phacoemulsification (Fortas CV-30000; Nidek, Japan) was performed through a 2.8 mm supero-temporal clear cornea incision. Trypan blue dye (0.06%) was used to stain the capsule of the cataractous lens. The anterior capsule was punctured and liquified cortex was aspirated using a 30 G needle mounted on 2-cc syringe, introduced through the side port. The anterior chamber was filled with cohesivehydroxypropyl methylcellulose ophthalmic (HPMC) 2% w/v solution (Appavisc, Appasamy Ocular Devices (P) Ltd, Solan, India) and dispersive-Sodium Hyaluronate 1.4% w/v (Viscosafe 14, Intas Pharmaceutical Ltd. India) ophthalmic viscoelastic devises (OVD) using Arshinoff soft-shell technique.6 The curvilinear capsulorrhexis was preceded by decompression of the bag by aspirating the cortex using 26 G needle followed by gentle tap with the hydro-canula. The technique is slight modification of the technique originally described by Rao et al. 7 The Continuous curvilinear capsulorrhexis was done using a cystitome needle. If it ran out, modified utratas’ capsulorrhexis forceps was used to complete the capsulorrhexis. Anterior chamber was re-filled with OVDs as and when required during capsulorrhexis. This followed limited hydro-procedures using ringer lactate solution. The nucleus was phacoemulsified using stop and chop technique. Foldable hydrophilic intraocular lens (IOL) was injected into the bag under OVDs, which was aspirated using bi-manual technique. The corneal wounds were sealed by hydrating the ports. In MSICS, superior rectus bridle suture using 5-0 silk was placed. A fornix-based conjunctival flap was created; hemostasis was achieved with bipolar cautery. A self- sealing frown shaped 6 mm incision was made 1.5 mm behind the limbus to create sclero-corneal pocket with a parabolic groove convex towards the limbus. Through side port entry capsulorrhexis was done in same manner as described for phacoemulsification. A 2.8 mm keratome was advanced through the sclero-corneal tunnel to enter into the anterior chamber in order to make the internal corneal incision. The tunnel was extended using 5.5 mm keratome. Nucleus was first prolapsed into the anterior chamber using lens dialer through the side port and then delivered with an irrigating lens loop through the tunnel. Residual cortex was aspirated using a Simcoe cannula. A posterior chamber 6.0 mm optic PMMA (Poly methyl methacrylate) IOL was implanted in the bag through the tunnel and dialed in position. Side port was sealed by hydrating the cornea. Finally, conjunctival apposition was done using bipolar diathermy cautery. Figure 1: Representative picture of eyes of a patient with phacomorphic glaucoma 14 Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022

Postoperatively fixed drug combination of topical moxifloxacin and dexamethasone (0.5%) in weekly tapering doses for 6 weeks and homatropine (1%) was given for 1 week. In patients with moderate to severe corneal edema topical sodium chloride (6%) was prescribed, and those with IOP >22 mmHg was started on either timolol (0.5%) or brimodine (1%). At every postoperative visit, slit lamp examination was done, and visual acuity and IOP were recorded. The primary outcome measures included visual acuity and IOP on post-operative visits on 1,7,28,90 and 180 days. Secondary outcome measures included frequency of intraoperative complications, namely iris prolapse, Descemet’s membrane detachment (DMD), and posterior capsular rent (PCR); and postoperative corneal clarity, anterior chamber reaction. The ECC was done at last follow up visit. The data was collected using a structured proforma and entered in excel sheet (Microsoft Co.; USA). The analysis was carried out using Statistical Package for Social Sciences (SPSS, Version 20.0) software. All the continuous variables were described as median and interquartile range (IQR, 25th and 75th quartile), and categorical variables were presented as absolute numbers and percentage. The parametric continuous variables between the groups were compared using unpaired t test, and non-parametric variables using Mann-Whitney test. Paired t-test was applied for intra-group preoperative and postoperative comparisons. Chi-square test of independence was used for association between categorical variables. The relationship between delay in duration of presentation, baseline IOP, corneal edema, with visual acuity and IOP was done using Spearman correlation coefficient. The level of significance (p value) was set at < 0.05. Results Baseline characteristics During the study period of 15 months, a total of 32 patients of PMAC presented in our department. The median age at presentation was 65 (IQR 7.5; range 50-90) years, majority of the patients (59.3%) were in age group of 61- 70 years. The study cohort had gender preponderance towards females, and women to men ratio was 2.2:1. The median duration between onset of symptoms and presentation was 3 (IQR 3, range 1-28) days. Most (n=28; 87.5%) patients presented within 7 days of onset of symptoms (Figure 2). The median IOP at presentation was 39 (IQR 18, range 25-59) mmHg. The median anterior chamber depth (ACD) was 1.79 (IQR 0.45, range 1.15-2.09) mm. The baseline characteristics of two groups were comparable (Table 1). The cornea was edematous in 28 (88%) patients. The median duration for conservative management before taking up for cataract extraction was 21.5 (IQR 9.5) hours. Intraoperative complications Intraoperative complications were seen in 2 (6%) cases, which included extension of capsulorrhexis (Argentinian flag sign) in one eye and focal DMD one eye, both were in MSICS group. No eye had iris prolapse or PCR in either group. Postoperative outcomes All the patients completed 6 months (Q2 180, IQR 1, range 173-194 days) follow up. Visual acuity progressively improved (Figure 3a) whereas IOP decreased with time (Figure 3b) during follow up. The median un-aided, decimal visual acuity on first post-operative day was 0.05 (IQR 0.07, range 0.001-0.5). The median visual acuity was marginally better for PHACO group but the difference was statistically insignificant (Table 2). A total of 10 (45%) patients, five in each group, had visual acuity ≥0.1. The median un-aided visual acuity at final follow-up was 0.5 (0.3). A total of 18 (56%) gained un-aided visual acuity of ≥0.5, six (37%) in MSICS group and 12 (75%) in PHACO group (χ2 4.57, p 0.03). Thirty-one (97%) patients had unaided visual acuity ≥0.1 at 6 months (Figure 4a). One patient had unaided visual acuity 0.08 due to large astigmatism (-9.0 DC). On multiple regression analysis neither delay in presentation nor IOP at presentation was associated with visual acuity on first post-operative day (p 0.2) or at final follow up. No eye developed any glaucomatous change in optic nerve head. The median IOP on first post-operative day was 12 (3.5) mmHg. Twelve (37%) eyes had IOP more than 18 mm Hg, 3 (19%) in MSICS group and 9 (56%) in PHACO group (χ2 4.8, p 0.02). First post-operative day IOP was not related with IOP at presentation (r 0.04, p >0.05). The mean IOP at final follow up was 12.5 (4.5) mmHg, with no significant difference between two groups. (Table 2). Three (9%) eyes had IOP ≥ 19 mmHg, 2 (12.5%) in MSICS group and 1 (6%) in PHACO group (χ2 0.36, p 0.5). All of these eyes had gonio-synechiae spread over >180° of angle, and were continued on IOP lowering eye drops. Duration of delay in initial presentation was related to IOP at final follow up (p<0.05). Overall, 18 (56%) eyes had corneal edema on first postoperative day, subsided in 11(61%) eyes by first week and in all by third post-operative visit. The rate of corneal edema was significantly (χ2 4.57, p 0.03) higher among patients of PHACO group (12; 75%) than MSICS group (06; 37%). Occurrence of corneal edema was not related to delay in presentation (r 0.05, p 0.7), Figure 2: Scatter diagram showing case-wise day of presentation. Eighteen of 32 (56.25%) cases presented within 72 hours of the onset of symptoms Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022 15

ACD (r 0.05, p 0.7) or IOP at presentation (r 0.12, p 0.4). Fourteen (43%) eyes had AC reaction of grade ≥ +2, five (31%) in MSICS group and 9 (56%) in PHACO group (χ2 2.03, p 0.1). The preoperative median ECC was 2450 (IQR 405) cells/mm2, and postoperatively at 6 months 2303 (IQR 403) cells/mm2, a surgical loss of 5.78 (±1.92) percent (Figure 4b). The loss in MSICS group was 5.64 (±1.1) % and PHACO group 5.89 (±1) % (p 0.5). Eyes with lower pre-operative ECC suffered higher loss (Spearman r 0.364, p 0.04). Discussion PMAC is not uncommon in India although incidence has decreased over the time. Jain et al., in 1983 reported phacomorphic glaucoma in 3.9% cases of senile cataract.2 In our previous work on white cataracts, we found lens-related glaucoma constituted 0.69 % of all senile cataracts and 5% of all white cataracts in persons aged >50 years.3 A noticeable proportion of senile cataracts wait till maturity before taking up cataract surgery.8 But it’s surprising despite painful eye, a large proportion of PMAC patients seek medical intervention usually after a variable delay.2,4,9 In our study median duration of delay was 3 days. We did not find any significant effect of delayed presentation on surgical outcomes unlike previous studies.2,9 This could be due to shorter duration of delayed presentation (median 3 days) and major proportion of patients presenting early (56% within 72 hours). In a study by Jain et al. 45% of the patients presented between 6 to >20 days.2 In a study by Pradhan et al.71% of patients had a delay of >10 days.9 Similar to our findings, a study by Lee et al., reported favorable outcomes because maximum delay was 15 days.5 Visual outcomes were good in our study, and 97% patients had un-aided visual acuity better than or equal to 0.1 at 6 months. The IOP crossed >30 mm Hg in most cases and associated with shallow anterior chamber and some reaction in anterior chamber.2-5,9 Therefore, cataract surgery in eyes with PMAC offers unique challenge of operating in pressurized and inflamed eyes. The outcomes of cataract extraction with ECCE, MSICS and phacoemulsification have reported intraoperative difficulties and complications Figure 3: Comparative change of primary outcome measures in two groups at each follow up visit (a) Median un-aided visual acuity (b) Median intraocular pressure a b Figure 4: (a) Scatter diagram to show dispersion of un-aided visual acuity at 6 months. Fifty three percent eyes had visual acuity better than or equal to 0.5 (b) Overall postoperative loss of endothelial cell count and comparison between two surgical groups. a b 16 Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022

in these eyes.2,4,5,9,10 Closed chamber cataract extraction as in MSICS and phacoemulsification cushion against more serious complications like vitreous loss and suprachoroidal hemorrhage, described with ECCE.2,11 Both MSICS and phacoemulsification has been shown to be safe in eyes with PMAC in non-comparative studies.8,12 Rajkumari et al. found MSICS was effective in controlling IOP and achieving good visual acuity with minimal complications in eyes with PMAC.9 Similar conclusions were drawn by Lee et al. in their series of 26 eyes with PMAC, operated by phacoemulsification.5 Moraru et al. reported difficulty in phacoemulsification in eyes with PMAC, forcing conversion to ECCE in 13 of total 38 (34%) eyes.10 In our comparative study, we did not find any significant difference in surgical outcomes of MSICS and phacoemulsification in terms of intraoperative complications. Cataract extraction alone has been shown to achieve good IOP control in PMAC.4,9,13 Good IOP control was achieved after cataract extraction in both the groups in this study irrespective of IOP at presentation and delay in presentation. We recorded significantly higher IOP in PHACO group on first operative day. This could have resulted either from corneal edema or more intense anterior chamber reaction in eyes in PHACO group. Similar to the results of study by Lee et al. formation of PAS was not associated with IOP at presentation or delay in presentation.5 However, in eyes with PAS, the IOP may rise further requiring additional intervention.5 The difference in endothelial cell loss between phacoemulsification and MSICS has not been found significant in eyes with various grades of cataract.14,15 Hwang et al. in their study demonstrated higher endothelial cell loss in eyes with shallow anterior chamber depths and hard cataracts.16 The effect of cataract surgery techniques on endothelial cell loss in eyes with PMAC has not been included in non-comparative studies.4,5,9,10,12 We did not find any difference in detrimental effect of MSICS and phacoemulsification on ECC. The rate of intraoperative complications in our study was 6%. We had low rate of complications in both groups and no serious complication happened. Difficulties faced during cataract surgery in PMAC are on account of shallow anterior chamber, intumescent lens and raised IOP. We owe low complication rate in our study to three factors. First, optimal and aggressive reduction of IOP pre-operatively. We did all surgeries with pre-operative IOP ≤21 mmHg. Secondly, we tried to achieve central, curvilinear capsulorrhexis after decompressing the bag. And finally, frequent re-filling of anterior chamber with OVDs to protect cornea and posterior capsule. Use of cohesive OVDs is effective in maintaining the depth of anterior chamber and preventing running away of anterior capsule during capsulorrhexis.5 Vitreous tap and pars plana vitrectomy prior to phacoemulsification has been shown to deepen the anterior chamber to safeguard against complications in PMAC or shallower eyes with extremely shallow or flat anterior chambers.17,18 In our study, the minimal ACD was 1.15 mm and capsular bag decompression along with optimum use of OVDs prevented intraoperative complications effectively. Uneventful capsulorrhexis can be achieved by decompressing the bag.5,7 Postoperatively corneal edema and anterior chamber reaction are common after cataract extraction in eyes with PMAC.5,9,12 Lee et al. reported similar trend, and corneal edema persisted for one week after phacoemulsification.5 The difference was significant on first operative day in favor of MSICS, but it mitigated by first week. Overall, comparison between two techniques of cataract extraction revealed that both MSICS and phacoemulsification were safe and effective for eyes with PMAC. The advantage of one technique over other during postoperative course were transient, and final outcomes were similar. References 1. Tham CC, Lai JS, Poon AS, Chan JC, Lam SW, Chua JK, Lam DS. Immediate argon laser peripheral iridoplasty (ALPI) as initial treatment Table 2: Comparison of changes in visual acuity and intraocular pressure in two study group during follow up visits Visual Acuity (Decimal) IOP (mmHg) Median (IQR) Median (IQR) MSICS PHACO P MSICS PHACO P Day 1 0.03 (0.11) 0.075 (0.07) 0.9 13 (7) 18.5 (5) 0.03 Day 7 0.1 (0.085) 0.1 (0.22) 0.9 13 (4) 14.5 (3.5) 0.5 Day 28 0.22(0.12) 0.18 (0.14) 0.6 13(4) 12 (2) 0.6 Day 90 0.31(0.21) 0.22 (0.10) 0.6 12(2) 14(3) 0.01 Day 180 0.4 (0.19) 0.56 (0.23) 0.15 12(3.5) 13(4.5) 0.7 Table 1: Comparison of demographic and pre-operative ocular characteristics of patients in two study groups Characteristic MSICS PHACO P (Median/IQR) Age (in years) 66 (7.5) 64.5(9) 0.17 Delay duration (days) 3 (3.5) 4.5(4) 0.2 IOP at presentation (mmHg) 37.5(20) 39 (16.5) 0.9 Corneal edema (number; %) 15 (93) 13(81) 0.2 Endothelia cell count* (n=29) 2433 (545) 2478 (324) 0.5 Anterior Chamber Depth (mm) 1.67(0.56) 1.81(0.29) 0.2 Axial length (mm) 22.24 (1.29) 22.51(1.28) 0.6 AC Angle (degree) 6.85(2.55) 7.65(2.1) 0.6 AOD-500 (mm) 0.119 (0.043) 0.130 (0.022) 0.2 Lens Vault (µm) 1173 (177) 1186 (187) 0.8 *Endothelial count was done after initial IOP management and some clearing of cornea Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022 17

for acute phacomorphic angle-closure (phacomorphic glaucoma) before cataract extraction: a preliminary study. Eye 2005; 19:778-783. 2. Jain IS, Gupta A, Dogra MR, Gangwar DN, Dhir SP. Phacomorphic glaucoma—management and visual prognosis. Indian J Ophthalmol 1983; 31:648-653. 3. Tomey KF, al-Rajhi AA. Neodymium: YAG laser iridotomy in the initial management of phacomorphic glaucoma. Ophthalmology 1992; 99:660-665. 4. Rajkumari V, Kaminibabu KS, Bhabanisana RD, Victor R. Manual small incision cataract surgery in phacomorphic glaucoma: Surgical technique and outcome in north-eastern India. J Current Glau Prac 2013; 7:43-48. 5. Lee SJ, Lee CK, Kim WS. Long-term therapeutic efficacy of phacoemulsification with intraocular lens implantation in patients with phacomorphic glaucoma. J Cataract Refract Surg 2010; 36:783-789. 6. Arshinoff SA, Norman R. Tri-soft shell technique. J Cataract Refract Surg 2013; 39:1196-1203. 7 .Rao SK, Padmanabhan P. Capsulorhexis in eyes with phacomorphic glaucoma. J Cataract Refract Surg 1998; 24:882884. 8. Rewri P, Lohan A, Aggarwal S, Chodhary P, Singhal A. Cataract surgical reach: Falling short to catch white cataracts! Indian J Ophthalmol 2021; 69:1575-1578. 9. Pradhan D, Hennig A, Kumar J, Foster A. A prospective study of 413 cases of lens-induced glaucoma in Nepal. Indian J Ophthalmol 2001; 49:103-107. 10. Moraru A, Pînzaru G, Moţoc A, Costin D. Functional results of cataract surgery in the treatment of phacomorphic glaucoma. Rom J Ophthalmol 2017; 61:202-206. 11. Ling R, Cole M, James C, Kamalarajah S, Foot B, Shaw S. Suprachoroidal haemorrhage complicating cataract surgery in the UK: epidemiology, clinical features, management, and outcomes. Br J Ophthalmol 2004; 88:478-480. 12. Ramakrishanan R, Maheshwari D, Kader MA, Singh R, Pawar N, Bharathi MJ. Visual prognosis, intraocular pressure control and complications in phacomorphic glaucoma following manual small incision cataract surgery. Indian J Ophthalmol 2010; 58:303-306. 13. Senthil S, Chinta S, Rao HL, Choudhari NS, Pathak-Ray V, Mandal AK, Garudadri CS. Comparison of cataract surgery alone versus cataract surgery combined with trabeculectomy in the management of phacomorphic glaucoma. J Glaucoma 2016;25: e209-13 14. George R, Rupauliha P, Sripriya AV, Rajesh PS, Vahan PV, Praveen S. Comparison of endothelial cell loss and surgically induced astigmatism following conventional extracapsular cataract surgery, manual smallincision surgery and phacoemulsification. Ophthalmic Epidemiol 2005; 12:293-297. 15. Zetterström C, Laurell CG. Comparison of endothelial cell loss and phacoemulsification energy during endocapsular phacoemulsification surgery. J Cataract Refract Surg 1995; 21:5558. 16. Hwang HB, Lyu B, Yim HB, Lee NY. Endothelial Cell Loss after Phacoemulsification according to Different Anterior Chamber Depths. J Ophthalmol 2015:210716 doi: 10.1155/2015/210716. Epub 2015 17. Dada T, Kumar S, Gadia R, Aggarwal A, Gupta V, Sihota R. Sutureless single-port transconjunctival pars plana limited vitrectomy combined with phacoemulsification for management of phacomorphic glaucoma. J Cataract Refract Surg 2007; 33:951954. 18. Chang DF. Pars plana vitreous tap for phacoemulsification in the crowded eye. J Cataract Refract Surg 2001; 27:1911-1914. 18 Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022

Abstract Original Article Introduction Neovascular glaucoma (NVG) is a secondary, refractory type of glaucoma that accounts for 0.7–5.1% of glaucoma in Asian population. 1,2 NVG is caused by a variety of ocular pathologies such as proliferative diabetic retinopathy, central venous occlusion, ocular ischemic syndrome, uveitis etc., It is a particularly severe form of glaucoma, which is characterized by poor visual prognosis. It can present with very high intraocular pressure, is resistant to conventional antiglaucoma treatment and can blind an individual if not treated in time. 3 The other reason to dread NVG, it can present in the other eye also if an individual is predisposed to the same. Early recognition of the disease is important before total peripheral anterior synechiae (PAS) and intractable glaucoma sets in. Diabetic retinopathy followed by venous occlusions are noted to be the commonest causes of NVG. According to a recent study, BRVO affects 4 per 1000 persons and CRVO affects 0.8 per 1000 persons. 4 Indian population, with a high proportion of diabetics and diabetic retinopathy (the leading cause of NVG), is theoretically, also prone to neovascular glaucoma. Being a developing nation with limited access to health care, the patients tend to present late. Purpose: To report the etiology, clinical features, treatment and outcomes of neovascular glaucoma (NVG) in a tertiary care ophthalmic center. Background: NVG is a devastating ocular disease often leading to loss of vision. The current standard of care includes retinal phtocoagulation and control of increased intraocular pressure with medical and surgical therapy like trabeculectomy, Ahmed Glaucoma Valve (AGV) and Trans scleral cyclophotocoagulation (TSCPC) with studies comparing them showing varied results. We compared these treatment modalities to find out which is better for IOP control. Methods: Retrospective file review. Results: 230 eyes of 217 patients with NVG were studied. Mean age of the population was 56.99 ± 13.8 years. Venous occlusion (37.4%) was the commonest cause of NVG followed by diabetic retinopathy (35.2%). All the patients were managed medically to begin with. Ninety one patients underwent surgical management. Pan retinal photocoagulation was done for 68.75% patients. Anti‑ vascular endothelial growth factor (VEGF) was given intravitreally for 56.25% patients prior to surgery. Thirty six patients underwent trabeculectomy with Mitomycin C (MMC), eleven patients underwent AGV implantation and thirty three underwent TSCPC. Best corrected visual acuity at baseline was 2.24 ± 0.99. Final visual acuity was 2.67 ± 1.1. Best corrected visual acuity (BCVA) was either stable or improved from baseline in 46.25% patients and deteriorated in 53.5%. All three modalities were equally effective in reducing IOP from baseline (p = 0.00) and there was no statistically significant difference between them taking IOP into consideration as success criteria (p = 0.60). Hyphaema was the commonest complication (22.5%). Conclusion: Venous occlusion was the commonest cause of NVG in our setup. All three modalities of treatment, AGV, trabeculectomy with MMC and TSCPC are effective in controlling the disease process. The visual prognosis however remains poor despite surgical treatment due to late presentation and fast progression. Keywords: Neovascular glaucoma, etiology, treatment Address for correspondence: Ketaki Rajurkar, MS, FICO Assistant Professor, Guru Nanak Eye Centre, Maulana Azad Medical College, Maharaja Ranjeet Singh Marg, New Delhi. E-mail: [email protected] This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected] Cite This Article as: Rajurkar K, Dubey S, Gandhi M, Gurav P, Pegu J. Clinical profile and management of neovascular glaucoma in North India. Delhi J Ophthalmol 2022;32:19-23. Clinical Profile and Management of Neovascular Glaucoma in North India Ketaki Rajurkar1 , Suneeta Dubey2 , Monica Gandhi2 , Prachi Gurav2 , Julie Pegu2 1 Department of Ophthalmology , Guru Nanak Eye Centre, Maulana Azad Medical College and Associated Hospitals, 2 Department of Ophthalmology, Shroff Charity Eye Hospital, Kedarnath Lane, Daryaganj New Delhi, India Submitted: 14‑Dec‑2022 Accepted: 17‑Feb‑2023 Published: 31-May-2023 Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/dljo.dljo_56_23 © 2023 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow 19

Often diagnosed at a late stage when visual acuity is already severely diminished, treatment is based on managing the underlying ischemic drive that is causing neovascularization and on intraocular pressure (IOP) control through antiglaucoma medications or surgery. Patients with extensive PAS usually remain uncontrolled on medical therapy and often need surgical management. Current surgical options for IOP control in NVG comprise augmented trabeculectomy, glaucoma drainage devices (GDD) and Trans scleral cyclophotocoagulation (TSCPC) while prognostic factors include young age, previous vitrectomy and postsurgery complications. 5-7 Thus, results of NVG post surgery are average to poor in most studies. The reported studies from India give etiologic considerations. Hence, a study giving clinical picture as well as studying the outcome of NVG is warranted. To address this paucity of data in Indian scenario, this study was conducted. Materials and Methods After institutional ethical clearance, a retrospective file review of our patients from January 2006 to August 2016 was done. All the patients presenting to our hospital with IOP>21 mmHg on applanation tonometry associated with neovascularization of the iris and/or angle of the anterior chamber detected by slit lamp bio-microscopy and gonioscopy were enrolled. All patients with NVG were investigated to determine the cause. Other data noted from the records included age, gender, affected eye, visual acuity (VA), IOP, degree of PAS, number of glaucoma medications used at presentation and at last visit, types of intervention, use of anti- vascular endothelial growth factor (Anti‑VEGF) agent (Bevacizumab[Avastin] 1.25 mg [0.05ml]), and post‑operative complications. Etiology of NVG was reported for all patients managed in the hospital. Patients with a follow up of less than 1 month were excluded, as were those without IOP and VA data at the final visit. For surgically managed patients, IOP and medications were noted at 1, 2, 3, 6, 12 month and 6 monthly after that. For the first month, any complication was noted on day 1, 7, 1 month and 2 month. Long term complications post surgery were also noted. Surgical management done was- trabeculectomy with Mitomycin C (MMC), Ahmed Glaucoma Valve (AGV) implantation (New World Medical, Inc., Rancho Cucamonga, CA, USA) and Trans scleral Cyclophotocoagulation (TSCPC). Cataract surgery was combined wherever necessary. Trabeculectomy with MMC (0.02%) was done with fornix based flap and a rectangular scleral flap. 8 AGV implantation was done in cases with extensive PAS, pseudophakic patient and visual prognosis was expected to be good. It was done by standard technique described in literature. 9 For TSCPC, eye was treated with the G probe with about 20 spots avoiding the 3 and 9 o’clock positions with parameters adjusted to avoid a ‘pop’. Success was defined as an IOP between 6 and 21 mmHg without topical or systemic glaucoma medications with retention of presenting VA; this visual criteria was applied to patients with a VA of light perception or better. Snellen’s VA was converted to the logarithm of the minimal angle of resolution (LogMAR) for analysis. An improvement or decrease in visual acuity was defined as a change of two or more lines on the LogMAR scale. For those with low vision, a difference of one low vision category post-surgery was considered as change. Patients with incomplete data (lack of IOP and VA) and follow up less than two month were excluded from the analysis of outcomes. Surgical failure was defined as insufficient IOP reduction (IOP >21 mmHg, <20% IOP reduction, use of a systemic carbonic anhydrase inhibitor or further glaucoma surgeries), devastating complications(loss of light perception, phthisis bulbi, and endophthalmitis), or significant hypotony (hypotony with IOP <5 mmHg continuing >6 months and until the last follow-up visit or hypotony that required intervention). True success was defined as IOP less than or equal to 21 mm hg without any antiglaucoma medications. Qualified success was defined as IOP less than or equal to 21 mm hg with antiglaucoma medications. Statistical Analysis Categorical variables were presented in number and percentage (%) and continuous variables were presented as mean ± SD and median. Normality of data was tested by Kolmogorov-Smirnov test. If the normality was rejected then non parametric test was used. Statistical tests were applied as followsQuantitative variables were compared using ANOVA/Kruskal Wallis Test (when the data sets were not normally distributed) between the three groups and Paired T test/Wilcoxon ranked sum test across follow up. Qualitative variables were correlated using Chi-Square test/Fisher’s exact test. Kaplan Meier Survival analysis curve was used to find out the success rate and Cox Proportional regression analysis to assess the factors significantly affecting the success rate. A P value of < 0.05 was considered statistically significant. The data was entered in Microsoft Excel spreadsheet and analysis was done using Statistical Package for Social Sciences (SPSS) version 21.0. Table 1: Age Distribution Frequency Percentage 1) ≤ 20 5 2.17% 2) 21‑30 6 2.61% 3) 31‑40 15 6.52% 4) 41‑50 37 16.09% 5) 51‑60 67 29.13% 6) 61‑70 76 33.04% 7) 71‑80 21 9.13% 8)>80 3 1.30% Total 230 100.00% 20 Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022

Results A total of 230 eyes (217 patients) were diagnosed as NVG in the time period from January 2006 and August 2016. 144 were male and 73 female. Age range was 7 to 89 years. Mean age of the population was 56.99 ± 13.8 years (Median 60 years). Age distribution is shown in [Table 1]. 117 were right eyes and 113 left eyes. All the patients were given medical treatment. Pan retinal photocoagulation (PRP), Anti- vascular endothelial growth factor(Anti-VEGF) treatment was offered to all patients wherever applicable. Diagnosis was made for patients wherever possible. However, some patients only had one visit, so, diagnosis was not possible for such patients (38 patients belonged to this category). The most common diagnosis was venous occlusion‑ 86 patients(37.4%), followed by diabetic retinopathy‑ 81 patients (35.2%). The other diagnoses are mentioned in [Table 2]. Best corrected visual acuity (BCVA) at baseline was 2.24 ± 0.99 (Median 2.3). Final visual acuity was 2.67 ± 1.1 (Median 2.9). BCVA showed no change from baseline in 21 patients (26.25%), deteriorated in 43 (53.75%) and improved in 16 (20%). 80 patients underwent surgery. 36 patients underwent trabeculectomy with MMC, 11 patients underwent AGV implantation and 33 underwent TSCPC. Anti‑ VEGF agent was given in 45 patients (56.25%). PRP was done for 55 patients (68.75%). Difference in change in BCVA was statistically significant between groups. TSCPC group showed maximum deterioration in vision. Change in BCVA based on type of surgery is given in [Table 3]. Commonest complication was hyphaema seen in 18 patients (22.50%). The other complications are listed in [Table 4]. Four eyes status post TSCPC underwent phthisis. Recurrence of NVG was seen in 17 patients (21.25%). Success rates of surgeries are given in [Table 5]. Long term success rates are given in [Table 6]. Change in the number of medications from baseline was statistically significant at all the time points till 2 years after which it was not significant. Summary of baseline characters, BCVA and IOP are given in [Table 7]. On plotting Kaplan‑Meier Survival curve, long term success rate at 1 year was 63.79%, 2 year was 60.25% and 3 year was 60.25% (Figure 1). On calculating cox proportional hazard ratio for factors such as age, cause of NVG (diabetic retinopathy and venous occlusion), complications, presenting BCVA, type of surgery, anti VEGF, degrees of PAS or PRP, none of the factors affected the success rate. Table 3: Change in Best corrected visual acuity after surgical management of NVG. Surgical management of NVG Total P Trabeculectomy + mmc Tube implantation TSCPC BCVA Change 1) No change 6 (16.67%) 0 (0.00%) 15 (45.45%) 21 (26.25%) 0.002 2Deteriorated 19 (52.78%) 7 (63.64%) 17 (51.52%) 43 (53.75%) 3) Improved 11 (30.56%) 4 (36.36%) 1 (3.03%) 16 (20.00%) Total 36 11 33 80 Figure 1: Kaplan-Meier Survival curve.Long term success rate at 1 year: 63.79%, 2 year: 60.25% 3 year: 60.25 %. Table 2: Cause of Neovascular Glaucoma Number of Patients Percentage Diabetic Retinopathy 81 35.2 Venous Occlusion 86 37.4 Uveitis 9 3.9 Trauma 2 0.9 Retinal Detachment 7 3.0 Eales 4 1.7 Coats 1 0.4 Ocular Ischaemic Syndrome 2 0.9 Total 230 100.0 Not Known 38 16.5 Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022 21

Discussion The commonest cause of NVG in this study was venous occlusion 37.4% followed by diabetic retinopathy 35.2%. In one ninety two patients etiology could be determined. In thirty eight patients due to single visit, no fundus view or patient unwilling for further investigations, cause could not be determined. Our results match with study by Liu L10 where CRVO (39.2%) was reported as the commonest cause in 120 NVG eyes. Gadia et al. 11 studied NVG in North Indian eyes and noted that venous occlusion and diabetic retinopathy were the commonest causes (22% Ramakrishnan et al.12 studied the cause of NVG in 42 South Indian eyes and found that PDR (45.23%) followed by CRVO (33.33%) were the commonest causes. Recent studies quote the prevalence of diabetic retinopathy in North India as 11.6% and in South India as 17.6%. 13 Our data seems to reflect difference in diabetic retinopathy prevalence between north and south India. Most of the other studies report a higher incidence of diabetes related NVG than venous occlusion related NVG. Diabetic retinopathy (39.7%) followed by CRVO (21.3%) were the commonest causes in a study from China. 14 Similar reports are available from other studies. 15‑17 Visual acuity results as with other studies are sobering. Improvement was seen in 20% eyes, 26.25% showed no change and 53.75% deteriorated. This is seen in most other NVG studies. Liao et al. 14 reported that baseline acuity was poor and could only be preserved or improved in 59% of cases while 41% worsened, with 16 eyes deteriorating to no light perception. Sahyoun et al.18 reported stable visual acuity in case of intravitreal Bevacizumab use with AGV and statistically significant deterioration in case of non‑use of Bevacizumab with AGV. In a study by Sun et al.19 on 44 eyes post glaucoma surgery or vitrectomy, 72.9% of the eyes showed improved visual acuity, 20.5% remained unchanged and 6.8% became worse. The commonest surgery done in our study was trabeculectomy + MMC followed by TSCPC. The type of surgery was determined by the phakic status, degrees of PAS, vision and IOP of the patient. Despite the type of surgery, we found good results with all the techniques. Trabeculectomy group success rate was 75%, TSCPC group 81.82% and AGV group 51.52%. There was no statistically significant difference between the groups. But such a comparison between the groups is not possible since the sample size, criteria for selecting type of surgery for these patients is different and the baseline visual acuity, IOP and prognosis of these patients is different. Shen et al.16 compared results of trabeculectomy and MMC with AGV implantation and found no difference between the two techniques. Long term success rate at 1 year was 63.79%, 2 year was 60.25% and 3 year was 60.25%. These results are similar to many other studies. Liao et al.14 reported that success was achieved in 84.8% at 1 year, 47.5% at 3 years and 21.9% at 5 years. Surgical success rate was 86.9 ± 4.3% (± standard error), 74.0 ± 6.1%, and 51.3 ± 8.6% at 1, 3, and 5 years in a study with trabeculectomy and MMC 8. Shen et al. 16 noted that success was 70% and 65% at 1 year and 60% and 55% at 2 years after Ahmed Glaucoma Valve and trabeculectomy, respectively. Thus, the success rate in NVG is less towards 5 years requiring additional medications or repeat surgery. Cox proportional hazard ratio calculated for age, diabetic retinopathy/CRVO as causative for NVG, presenting BCVA, surgical management of NVG, complications, hyphaema, anti‑VEGF use, PRP and degrees of PAS, showed no influence on the success rate. Liao et al.14 also found similar results. Cox proportional hazards model showed that factors like systemic disease, lens status, complications, anti- VEGF agents, PRP and prior‑intraocular surgery do not influence the treatment outcome of NVG. Higashide T 8 has noted lower preoperative IOP (≤ 30 mmHg), hypotony and vitrectomy after trabeculectomy as causative factors for surgical failure. Anti‑VEGF therapy inhibits the NVI and the fibroblasts proliferation, allowing better IOP control 20, 22 and a lower rate of intraoperative bleeding and hyphema. 22, 27 However, it does not affect the BCVA 18 and effect on the success rate of surgery is also controversial. 18, 24 In this study, all three types of glaucoma surgery were found to be effective with no difference statistically in terms of IOP control. Though there was difference in terms of BCVA change, this can be attributed to the selection criteria for each Table 4: Complications post surgery in NVG Complications Frequency Percentage Hyphaema 18 22.50% Hypopyon 3 3.75% Shallow anterior chamber 2 2.50% Vitreous haemorrhage 1 1.25% Table 5: Success and failure rate of surgical treatments of NVG. Surgical management of NVG Total P Trabeculectomy + mmc AGV TSCPC 0.060 Success Failure 9 (25.00%) 2 (18.18%) 16 (48.48%) 27 (33.75%) Success 27 (75.00%) 9 (81.82%) 17 (51.52%) 53 (66.25%) Total 36 11 33 80 22 Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022

of the surgeries. Best visual prognosis patients were allotted to trabeculectomy or AGV group and worse visual prognosis patients underwent TSCPC. This study is the largest study on NVG in north India till date. The limitations of the study include the retrospective design, exclusion of a large number of cases due to poor follow up and lack of data. Conclusion Venous occlusion followed by diabetic retinopathy were found to be the commonest causes of NVG in this study. Cases present late, follow up is poor and management is determined by visual acuity, degree of PAS and phakic status of the patient. Despite adequate management, the prognosis is poor in many cases, thus indicating need for prevention of NVG, early diagnosis and aggressive management. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. References 1. Wong TY, Chong EW, Wong WL, Rosman M, Aung T, Loo JL, et al. Prevalence and causes of low vision and blindness in an urban malay population: the Singapore Malay Eye Study. Arch Ophthalmol. 2008; 126 (8):1091–9. 2. Narayanaswamy A, Baskaran M, Zheng Y, Lavanya R, Wu R, Table 6: Longterm success rate of NVG. Total P 2 MTH Failure 29 (36.25%) 0.0001 Success 51 (63.75%) 6 MTH Failure 8 (24.24%) 0.123 Success 25 (75.76%) 1 YR Failure 4 (15.38%) 0.486 Success 22 (84.62%) 2 YR Failure 1 (6.25%) 0.504 Success 15 (93.75%) 4 YR Failure 3 (37.50%) 0.091 Success 5 (62.50%) Table 7: Parameters of NVG patients (Baseline and Final) Mean ± SD Median Min-Max Age 55.2 ± 14.24 58.5 7‑80 BCVA logmar presenting 2.24 ± 0.99 2.3 0.3‑4 Presenting IOP 41.81 ± 13.03 42.5 14‑70 final f/u days after NVG sx 406.89 ± 543.76 183 30‑3285 Final IOP 20.04 ± 11.51 17 4‑54 Final IOP Reduction 46.46 ± 39.11 56.76 ‑185.71‑92.86 BCVA logmar final 2.67 ± 1.1 2.9 0.3‑4 Degree of PAS 311.79 ± 79.62 360 0‑360 Wong WL, et al. The prevalence and types of glaucoma in an urban Indian population: the Singapore Indian Eye Study. Invest Ophthalmol Vis Sci. 2013; 54 (7):4621–7. 3. Sohan Singh Hayreh. Neovascular glaucoma. Prog Retin Eye Res. 2007 September; 26 (5): 470–485. 4. Sophie Rogers, Rachel L. McIntosh, Ning Cheung et al; The Prevalence of Retinal Vein Occlusion: Pooled Data from Population Studies from the United States, Europe, Asia, and Australia. Ophthalmology. 2010 Feb; 117 (2): 313–9.e1 5. Sivak‑Callcott JA, O’Day DM, Gass JD, Tsai JC. Evidence‑based recommendations for the diagnosis and treatment of neovascular glaucoma. Ophthalmology. 2001; 108 (10):1767–76. quiz1777, 1800. 6. Takihara Y, Inatani M, Fukushima M, Iwao K, Iwao M, Tanihara H. Trabeculectomy with mitomycin C for neovascular glaucoma: prognostic factors for surgical failure. Am J Ophthalmol. 2009; 147 (5):912–8. 918 e911. 7. Nakatake S, Yoshida S, Nakao S, Arita R, Yasuda M, Kita T, et al. Hyphema is a risk factor for failure of trabeculectomy in neovascular glaucoma: a retrospective analysis. BMC Ophthalmol. 2014; 14:55. 8. Higashide T, Ohkubo S, Sugiyama K Long‑Term Outcomes and Prognostic Factors of Trabeculectomy following Intraocular Bevacizumab Injection for Neovascular Glaucoma. PLoS one. (2015) 10 (8): e0135766 9. Suneeta Dubey, Vijeta Sharma, Anugya Agrawal et al; Safety and efficacy of Ahmed glaucoma valve implantation in refractory glaucomas in Northern Indian eyes. Saudi Journal of Ophthalmology (2015) 29, 103–108. 10. Liu L. Etiology analysis of neovascular glaucoma in 120 cases. Int J Ophthalmol. 2011; 11 (3):521–3. 11. Ritu Gadia et al; Current profile of secondary glaucomas. Indian J Ophthalmol 2008; 56:285‑9. 12. Hari Ramakrishnan et al. Aetiology of neovascular glaucoma among patients attending a tertiary eye care centre in South India. International Journal of Scientific & Engineering Research, 2015 (8); 1784‑86. 13. Kaveeshwar SA, Cornwall J. The current state of diabetes mellitus in India. AMJ 2014; (7) 45‑48. http//dx.doi.org/10.4066/AMJ.2014.1979 14. Na Liao et al. Neovascular glaucoma: a retrospective review from a tertiary center in China. BMC Ophthalmology (2016) 16:14. 15. Al‑Shamsi HN, Dueker DK, Nowilaty SR, Al‑Shahwan SA. Neovascular glaucoma at king khaled eye specialist hospital-etiologic considerations. Middle East Afr J Ophthalmol. 2009; 16 (1):15–9. 16. Shen CC, Salim S, Du H, Netland PA. Trabeculectomy versus Ahmed Glaucoma Valve implantation in neovascular glaucoma. Clin Ophthalmol. 2011; 5:281–6. 17. Woodcock MG, Richards JC, Murray AD. The last 11 years of Molteno implantation at the University of Cape Town. Refining our indications and surgical technique. Eye (Lond). 2008; 22 (1):18–25. 18. Marwan Sahyoun et al; Long‑Term Results of Ahmed Glaucoma Valve in Association With Intravitreal Bevacizumab in Neovascular Glaucoma; J Glaucoma 2015; 24: 383–388. 19. Sun et al. Anti-VEGF treatment is the key strategy for neovascular glaucoma management in the short term. BMC Ophthalmology 2016 16:150 20. Horsley MB, Kahook MY. Anti‑VEGF therapy for glaucoma. Curr Opin Ophthalmol. 2010; 21:112–117. 21. Ghanem AA, El‑Kannishy AM, El‑Wehidy AS, et al. Intravitreal bevacizumab (avastin) as an adjuvant treatment in cases of neovascular glaucoma. Middle East Afr J Ophthalmol. 2009; 16:75–79. 22. Sevim MS, Buttanri IB, Kugu S, et al. Effect of intravitreal bevacizumab injection before Ahmed glaucoma valve implantation in neovascular glaucoma. J Int Ophtalmol. 2013; 229:94–100. 23. Ma KT, Yang JY, Kim JH, et al. Surgical results of Ahmed valve implantation with intraoperative bevacizumab injection in patients with neovascular glaucoma. J Glaucoma. 2012;21:331–336 24. Minwen Zhou, Xun Xu, Xiulan Zhang et al; Clinical Outcomes of Ahmed Glaucoma Valve Implantation With or Without Intravitreal Bevacizumab Pretreatment for Neovascular Glaucoma: A Systematic Review and Meta‑Analysis. J Glaucoma 2015; 00:000–000. Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022 23

Abstract Original Article Introduction The eyelids serve multiple key purposes other than just maintaining the structural integrity of the globe. They also control the brightness of lights by altering the palpebral fissure height, act as the windshield wipers of the eye by spreading tear film over the ocular surface, facilitate outflow via the lacrimal drainage system and add to the aesthetic beauty of face. Various studies have shown the increasing prevalence of ocular injuries of which the rate of hospitalised eye injuries was found to be 13.2/100,000.1 This amounts to an additional cost of healthcare and ineffective treatment impacts the selfesteem of patients due to impaired cosmesis complicated by a traumatic eyelid laceration. A simple yet graded classification of eyelid laceration would help in stratifying these injuries, enable health care professionals unfamiliar with core ophthalmology document the injury precisely and reduce bias; decide timely treatment options , aid in prognostication and subsequent patient education and study outcomes . This article presents such a classification system for eyelid lacerations without the involvement of globe. Materials and methods This study was carried out at our institute from July 2018 to July 2020. Our hospital being a tertiary eye care centre has a huge inflow of patients at the emergency department of which lacerations of the eyelid constitute approximately twenty percent. International studies have estimated the Context and aim: Eyelid lacerations can result in morbid changes, both functionally and aesthetically. For meticulous repair and best outcome, a systematic and stratified approach is warranted. Hence we aimed at formulating and employing a simple classification for eyelid lacerations presenting at our emergency department which can also aid for research purposes. Methods: A single centre prospective study was conducted at our tertiary eye care centre from July 2018 to July 2020. We designed a classification system based on existing classifications and clinicoanatomical presentations. Sarojini devi eye Hospital Eyelid Laceration ( SHEL) classification was then prospectively applied to the patients presenting to our hospital. Results: An uncomplicated and comprehensive classification was designed including two major types of laceration with relevant subtypes. Eyelid injuries in a total of 300 patients were evaluated in this study. Interobserver variation and bias was noticed to be reduced and a more universal approach was achieved in planning repairs timely and effectively. This classification was implemented in the lid repair protocol and used in other in-house studies based on eyelid lacerations. Conclusion: The classification appeared to be a dependable system to address and document various eyelid lacerations and would guide oculoplastic surgeons to provide optimal outcomes. Keywords: Eyelid Laceration, Eyelid Repair, Eyelid Laceration Classification, Injury Classification, Periocular Trauma Address for correspondence: Modini Pandharpurkar, Professor and Head of the Department, Department of Ophthalmology, Sarojini Devi Eye Hospital, Osmania Medical College, Hyderabad, Telangana, India. E-mail: [email protected] This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected] Cite This Article as: Philips CD, Pandharpurkar M. A new perspective on eyelid laceration classification: Proposal based on study conducted at a tertiary eye care centre in South India. Delhi J Ophthalmol 2022;32:24-8. A New Perspective on Eyelid Laceration Classification: Proposal Based on Study Conducted at a Tertiary Eye Care Centre in South India Christina Daisy Philips, Modini Pandharpurkar Department of Ophthalmology, Sarojini Devi Eye Hospital, Osmania Medical College, Hyderabad, Telangana, India Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/dljo.dljo_57_23 Submitted: 16‑Dec‑2022 Accepted: 23‑Jan‑2023 Published: 31-May-2023 24 © 2023 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow

lifetime prevalence of ocular injuries to be 14.4% –19.8%, whereas the incidence rate of hospitalised eye injuries was found to be 13.2/100,000.1,2 We were able to design a system of classification after reviewing previous articles and discussing with our peers for assessing and documenting eyelid lacerations on a first contact basis. We applied this classification prospectively to 300 patients presenting to our hospital. Details about the classification The classification is broadly classified into two types namely Type S for single zone and Type M when more than one zone was involved. Type S injuries can be further categorised based on the zone involved. To address the anatomical component with regards to the periorbital region, we adopted the nomenclature which was originally designed by Spinelli3 and later adapted and modified by Mohapatra DP et al in their System for peri-ocular trauma classification (SPOT) for periocular injuries.4 In our classification there are four zones namely Upper lid(UL), Lower lid (LL), Medial canthus (MC) and Lateral canthus (LC). Mohapatra et al defined upper lid as the region extending from a point 1 mm lateral to the lacrimal punctum to 3mm medial to lateral ocular commissure on the palpebral margin and the corresponding area on the lower lid was designated as the lower lid zone. An imaginary perpendicular line drawn from the palpebral margin to orbital rim at each of these points helped in demarcating the zones more clearly. The remaining areas were marked as medial canthus at the medial end and lateral canthus at the lateral end. To describe the anteroposterior tissue involvement, the depth of injury is assessed based on the lamellae involved. L1, L2,L3 were given for anterior, middle and posterior lamella respectively. Anterior lamella includes skin and orbicularis muscle. Middle lamella includes tarsal plate and orbital septum and posterior lamella include retractors and palpebral conjunctiva [Figure 1]. A superficial tear indicates the involvement of L1, partial thickness tear upto L2 and a full thickness would involve till L3(all three lamellae breached). A full thickness laceration at the medial or lateral canthi can also include a periosteal breach in addition to the above. To indicate tissue loss, a suffix ‘a’ meaning there is no tissue loss per se and ‘b’ indicating significant tissue loss is added. A note on the type of tissue lost is made (ie, skin, muscle, fat). Lacrimal system involvement in medial canthal injuries is demonstrated with a ‘c’ as suffix of which c1 denotes upper punctum and c2 denotes the lower punctum. To show the side of involvement ‘a’ (RE) or (LE) was included in the nomenclature for right or left, respectively. To focus the classification purely on the eyelids, globe injuries and skeletal injuries were excluded. The final scheme of classification was named as the Sarojini devi eye Hospital Eyelid Laceration ( SHEL) classification [Figure 2] [Table 1]. This classification was then prospectively applied to patients with acute eyelid injuries attending the emergency services at our hospital. A few case scenarios with clinical pictures have been included below. Case 1 A 42year old male sustained a full thickness laceration to his left eye following road traffic accident. There was involvement of his left eye lower lid and medial can thus with no tissue loss but was accompanied by a lower canalicular tear. Here two zones have been affected. The laceration was sutured and a Mini Monaka stent was implanted for the canalicular tear. Hence based on the SHEL classification classification,was classified as (LE) Type M LL L3a + MC L3ac2 [Figure 3 ] Case 2 A 35 year old male presented with left eye upper lid partial thickness laceration extending upto the middle lamella with no tissue loss, after being hit with a stone. In this case, only one zone has been involved hence was classified as (LE) Type S UL L2a [Figure 4] Case 3 A 47 year old male after a road traffic accident presented with a superficial laceration involving the right eye lateral canthus Figure 1: Schematic diagram of zones with anatomical landmarks Figure 2: Categorization in the classification based on zones involved Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022 25

and lower lid. The laceration was sutured instead of trying glue as it involved two zones. (RE) Type M LL L1 + LC L1 [ Figure 5] Case 4 A 14 year old boy, following dog bite, presented with an extensive laceration. There were full thickness lacerations involving the right eye upper and lower lid along with medial canthal injury involving both the upper and lower canaliculi. There was significant tissue loss which included skin and muscle tissue. According to the classification, it was classified as (RE) Type M UL Lb + MC L3b (c1+c2) + LL L3b [Figure 6] Discussion Owing to the increasing industrialisation and urbanisation in the cities, the incidence of trauma pertaining to eye are on the rise. A large group of patients presenting with eyelid lacerations are young (less than 45 years of age). A meticulous approach is mandated to reduce visual, functional and aesthetic morbidity.8 Majority of such injuries are usually dealt with in the general casualty theatres where a primary repair would be done not necessarily respecting the intricate anatomy eyelids have unlike the other areas of the body. This tendency often leads to unsatisfactory results. We have also come across the difficulty faced by health care workers from different departments to assess, document and verbally communicate about the nature and severity of the injury. A comprehensive and stratified classification hence will aid in an universal approach to such cases among the healthcare system hierarchy.5 There are various well established classification systems for eyeball and facial skeletal injuries.6 After reviewing indexed journals and database , we found the International Classification of diseases (ICD -10) classifying eyelid injuries as superficial and deep (S 00.1, S 00.2, S 01.1)7 and the System for peri-ocular trauma classification for periocular injuries (SPOT ) classification by Mohapatra et al. 4 For a more easy and simpler classification which can be used by medical professionals who may not be thoroughly proficient with the surgical anatomy of the eyelids and its implications, we designed the SHEL classification. The clinicoanatomical foundation for this classification has been the anatomical zones of the periocular region defined by Spinelli and Jelks3 and the SPOT classification.4 As eyeball and skeletal injuries have been Figure 3: (LE) Type M LL L3a + MC L3ac2 Figure 4: (LE) Type S UL L2a Figure 5: (RE) Type M LL L1 + LC L1 Figure 6: (RE) Type M UL L3b + MC L3b(c1+c2) + LL L3b 26 Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022

omitted, this classification is exclusively made for eyelids but also addresses canalicular injuries. We then applied this system of classification to all patients presenting with eyelid trauma to assess its clinical applications and various adjustments were made during this time. The SHEL classification enabled us to triage the patients with eyelid lacerations once admitted. As several patients with laceration may be admitted simultaneously, cases which needed immediate repair to avoid compromising on best possible outcome were posted first. This eventually helped our theatre schedules to be more organised. It also enabled us to plan judiciously based on the available resources and skillset to approach different varieties of laceration. Cases were graded and classified and posted to ophthalmic surgeons based on the surgical experience expected for repairing. This ensured maximum utilisation of available trained surgical hands to provide the best outcome to every patient,both functionally and aesthetically.8 Application of this classification at the time of admission process aided in easier inter department communication and the documentation process was less complicated. If a patient had to be referred to other departments for non-ocular procedures, it became easier for the clinicians to comprehend the nature of injury with a more surgical perspective. Table 1: Showing the antero-posterior anatomical structural basis of the Sarojini devi eye Hospital Eyelid Laceration (SHEL) Classification ZONE ANATOMICAL REGION NATURE OF INJURY UL UPPER EYE LID L1=ANTERIOR LAMELLA L2=MIDDLE LAMELLA (a=without tissue loss,b=with tissue loss) L3=POSTERIOR LAMELLA (a=without tissue loss,b=with tissue loss) LL LOWER EYE LID L1=ANTERIOR LAMELLA L2=MIDDLE LAMELLA (a=without tissue loss,b=with tissue loss) L3=POSTERIOR LAMELLA (a=without tissue loss,b=with tissue loss) MC MEDIAL CANTHUS L1=ANTERIOR LAMELLA L2=MIDDLE LAMELLA (a=without tissue loss,b=with tissue loss) L3=POSTERIOR LAMELLA (a=without tissue loss,b=with tissue loss) If canalicular injury present , add suffix c1 - Upper canaliculus,c2 - Lower canaliculus LC LATERAL CANTHUS L1=ANTERIOR LAMELLA L2=MIDDLE LAMELLA (a=without tissue loss,b=with tissue loss) L3=POSTERIOR LAMELLA (a=without tissue loss,b=with tissue loss) Another advantage of applying this classification was that it helped in prognostication at the time of admission and enabled the attending surgeons to educate the patients about the depth of injury and the various options available for repair based on the scope of the department. This prepared the patients to have a more suitable expectation about the outcome and they showed more trust in the procedure and adherence to follow up and revision surgeries,if needed. Since cases have been properly graded and documented, this record can be used later for other studies on periocular trauma. This classification is, however, not without limitations. It doesn’t include burn or acid injuries of the eyelids and the aetiological component of the injury is not addressed. Conclusion The Sarojini devi eye Hospital Eyelid Laceration (SHEL) classification is a simple and comprehensive system for easy grasping and inclusion in treatment protocols. It has multiple uses which also expands to academic prospects. This classification can make communication among health care workers and even laymen much easier. It can aid surgeons to assess the injury at clinicoanatomical level, plan surgical intervention and hence provide optimal outcomes. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. References 1. Woo JH, Sundar G. Eye injuries in Singapore--don’t risk it. Do more. A prospective study. Annals of the Academy of Medicine, Singapore. 2006 Oct 1;35(10):706-18. 2. Kretlow JD, McKnight AJ, Izaddoost SA. Facial soft tissue trauma. InSeminars in plastic surgery 2010 Nov (Vol. 24, No. 04, pp. 348-356). © Thieme Medical Publishers. 3. Spinelli HM, Jelks GW. Periocular reconstruction: a systematic approach. Plastic and reconstructive surgery. 1993 May 1;91(6):1017-24. 4. Mohapatra DP, Thiruvoth FM, Chittoria RK, Kumar SD, Kumar SH, Kumar S, Babu P, Kumar E. Proposal of a new classification scheme for periocular injuries. Indian J Plast Surg. 2017 JanApr;50(1):21-28. doi: 10.4103/ijps.IJPS_207_16. PMID: 28615806; PMCID: PMC5469230. 5. AudigéL, Bhandari M, Hanson B, Kellam J. A concept for the validation of fracture classifications. Journal of orthopaedic trauma. 2005 Jul 1;19(6):404-9. 6. Manolidis S, Weeks BH, Kirby M, Scarlett M, Hollier L. Classification, and surgical management of orbital fractures: experience with 111 Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022 27

orbital reconstructions. Journal of Craniofacial Surgery. 2002 Nov 1;13(6):726-37. 7. World Health Organization. International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10)-WHO Version for. 2016. 8. Subramanian N. Reconstructions of eyelid defects. Indian journal of plastic surgery: official publication of the Association of Plastic Surgeons of India. 2011 Jan;44(1):5. 9. A manual of Systematic Eyelid surgery-J.R.O.Collin,Third edition;11-13,115-145,147-165 28 Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022

Abstract Original Article Introduction Low vision is defined as visual impairment despite treatment, surgery, or standard refractive correction, but with the potential to use the residual vision. The World Health Organization describes a person with low vision (LV) as one who has an impairment of visual function, even after treatment and/or standard refractive correction, and has a visual acuity (VA) of <6/18 to perception of light (PL), or a visual field of <10° from the point of fixation, but who uses–or is potentially able to use–vision for the planning and/or execution of a task for which vision is essential. 1 Low vision (LV) is characterized by irreversible visual loss, decreased visual field, glare, and contrast, and decreased ability to perform daily activities such as reading or writing, and some people who suffer from this condition may be socially withdrawn. The prevalence of LV in a population‑based cross‑sectional study in India was reported to be 1.05% in the year 2000, with a burden of 10.6 million people requiring LV services. The magnitude of LV is estimated to be 54.5 million in India.2 Purpose The aim of the study was to explore clinical profile of patients requiring Low Vision Aids (LVA) and assess the effectiveness of common everyday household artefacts to improve the quality of life (QOL) of patients with low vision. As opposed to optical devices such as tele-lens and other more expensive low vision aids, an attempt was made to explore the availability and use of household artefacts, readily available or modifiable in a patients’ household, to improve the quality of life among patients with low vision. Methodology A cross‑sectional analysis of LV patients attending a Low Vision Clinic from November 2021 to February 2022 was done. Around 138 patients were enrolled after informed consent. Distance vision, near vision and best corrected visual acuity (BCVA) were assessed. Colour vision, contrast sensitivity and visual fields were done, wherever possible. The burden of low vision is rapidly increasing globally, and more specifically, in the developing world. The study aims to assess various commonly used low visual aids and the Final outcome in patients. 36 patients with low vision were examined, investigated and treated by the author in the period between Dec 2018 to April 2019, using a variety of new instruments and rehabilitation techniques. Following this, there was improvement in visual acuity in 32 patients as well as improvement in quality of living where patients were able to undertake several household and profession related tasks and activities. Through this, the author concludes that for low vision patients, following proper diagnosis, and treatment, proper rehabilitation tools and techniques are needed to bring improvement the quality of life. Keywords : Visual Rehabilitation, House Hold Artefacts And Living Space Modification Address for correspondence: Ashwini Kumar, MBBS, DOMS Consultant Ophthalmologist and Low Vision Aid Specialist, Mahavir Netralaya, Patna. E-mail: [email protected] This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected] Cite This Article as: Kumar A. Improving quality of life of low vision patients with living space modifications. Delhi J Ophthalmol 2022;32:29-30. Improving Quality of Life of Low vision Patients with Living Space Modifications Ashwini Kumar (MBBS DOMS) Department of Ophthalmology, Mahavir Netralaya Patna, India Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/dljo.dljo_58_23 Submitted: 10-Feb-2023 Accepted: 21-Feb-2023 Published: 31-May-2023 © 2023 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow 29

A semi-structured interview was undertaken to understand their abilities and dis-abilities around their day-to-day activities and tasks (modified Activity of daily living tool). Condition of their residence/living space, availability of household objects and their ability to modify them for their use, were studied. Household modifications were categorised as per need for use in improving QOL. These were as follows: • Kitchen and cooking: Change of wall colour along with light source and direction. Special or modified utensils for safety and user friendliness, techniques for pouring hot and cold beverages differently. • Outdoor and Indoor field of vision: Mobility training; walking cane; foldable walking cane with alarm. • Common household activities and finer work: needle threader, torch light; coloured chocolate beads, mobiles with usage of voice commands. • Writing and reading: Signature guide • Money related: Cardboard Notex for counting money. Patients were followed up physically or telephonically to assess their improvement and overall satisfaction at a five‑point scale, scale 5 denoting maximal satisfaction. Results Of the 140 patients, 79% were male. Age distribution was 23% patients in first two decades, 55% in 3rd -5th decades, 20% in 6-7th decade, with only 2% being in 8th decade or older. Best corrected visual acuity is depicted in Table 1. Activities of daily living causing difficulty were identified as viewing/using mobile phones by 45%, viewing TV by 21%, undertaking outdoor tasks by 20%, undertaking household work by 12%, undertaking finer work like writing and reading by 10%, undertaking kitchen work 14% and occupation related 25%. Multiple responses were permitted. Follow up after counselling was possible for all 138 patients. Their responses related to living space modifications and household artefacts were as follows(multiple responses):81% benefitted from the counselling; 8% reported improvement through modifications in household lighting, 8% through changes in kitchen ambience and utensil modifications. Among objects 10% benefitted by using torch light, 25% from money counting Notex, 9% by walking stick or cane, and 36% by a mix of all. Patient satisfaction was high with 65% rating 5/5, 32% rating 4/5 and 3% rating 3/5. Conclusion Patients with low vision suffer from a complex set of problems ranging from loss of confidence, hesitancy, fear and overall visual and physical inabilities and challenges to live their day to day lives. 3,4 While they can be from any age range, most difficulties are faced during the productive years of their lives. Therapy using a mix of counselling, modification of household ambience and use of household artefacts have a combined result of improving overall patient satisfaction and quality of life. References 1. Congdon NG, Friedman DS, Lietman T. Important causes of visual impairment in the world today. JAMA 2003;290 (15):2057-60 2. World Health Organization. Global Data on Visual Impairments. Geneva: WHO; 2010. Availableat: http://www.who.int/blindness/ GLOBALDATAFINALforweb.pdf. Accessed 11.12.14. 3. Jose J, Thomas J, Bhakat P, Krithica S. Awareness, knowledge, and barriers to low vision services among eye care practitioners. Oman J Ophthalmol. 2016; 9 (1):37-43. 4. Margrain TH. Helping blind and partially sighted people to read: the effectiveness of low vision aids. Br J Ophthalmol. 2000;84 (8):919-21 Table 1: Patients followed up Total patients 140 Male 110 (79%) Female 30 (21%) Age Young (First 2 decades) 23% Mid‑aged (3rd to 5th decades) 55% Senior citizens (6th and 7th decade) 20% Elderly (8th decade or older) 2% 30 Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022

Abstract Case Report Introduction Leber Hereditary Optic Neuropathy (LHON) is an inherited mitochondrial neuropathy which affects the retinal ganglion cells and causes axonal degeneration of the optic nerve.1 The prevelance of LHON is 1 in 8,500 to 1 in 50,000 people.2 The patients typically present with painless bilateral, sequential loss of central vision. Approximately 50% of men and 10% of women who carry one of the primary LHON mutations present with visual loss.3 The three primary mutations observed in 90% of the cases are G11778A in ND4(most common), G3460A in ND1, and T14484T in ND6.4 Case report Case 1 A 14-year-old boy complained of painless vision diminution in right eye (RE) since 1.5 years for which he was advised spectacle correction. Six months later, he noticed a decrease in vision in his left eye (LE). There was no significant ocular or systemic history. His 16-year-old maternal had a similar history of progressive, painless vision loss in BE. Best-corrected visual acuity (BCVA) was 6/60 (Snellen’s) in both eyes. Ocular examination revealed a Relative Afferent Pupillary Defect (RAPD) Grade 1 in his left eye. The only other remarkable ocular finding was temporal pallor of the left optic disc and defective colour vision in BE. The contrast sensitivity of both eyes was log 1.05. Visual field analysis revealed a central scotoma in the macular region BE. On suspicion of bilateral optic neuropathy an MRI and a VEP was done. The MRI brain and orbit were normal, but the VEP showed a decreased P100 amplitude in BE. On reviewing the clinical presentation and the presence of relevant family history, a provisional diagnosis of LHON was made. PCR and genetic sequencing confirmed a mutation of G11778A. Case 2 An 11-year-old boy presented with bilateral blurring of vision with difficulty in identifying red colour since 2 months. There was no history of headache, nausea, previous spectacle use, ocular trauma or previous chronic illness. On further probing into the history, the patient’s mother had bilateral disc pallor There was similar history in his sisters, maternal grandmother, great-grandmother and maternal aunt’s sons. Leber hereditary optic neuropathy (LHON), a mitochondrial disease, is characterized by bilateral subacute loss of central vision owing to degeneration of the retinal ganglion cells and optic nerve. We report 2 cases of bilateral diminution of vision with optic disc pallor. A genetic study was planned, which revealed G11778A and T14484T mutation respectively, thereby confirming LHON. The reports highlight the basis for differential diagnosis of visual loss in childhood. It presents the findings of recently published studies and the insight they provide on the complex pathophysiology of LHON. It discusses management modalities and emphasizes upon the importance of genetic counselling. Keywords: Lhon, Mitochondrial Disease, Genetic Mutations, Genetic Counselling, Childhood Vision Loss Address for correspondence: Nishi Meghna Satish, MBBS Department of Ophthalmology, VMMC and Safdarjung Hospital, New Delhi, India. E-mail: [email protected] This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected] Cite This Article as: Satish NM, Rath S, Chopra K, Khurana R, Chawla N, Guliani BP. Leber hereditary optic neuropathy: Still a dilemma? Delhi J Ophthalmol 2022;32:31-3. Leber Hereditary Optic Neuropathy: Still a Dilemma? Nishi Meghna Satish1 , Soveeta Rath2 , Kritika Chopra1 , Rolli Khurana3 , Neha Chawla1 , B.P. Guliani1 1 Department of Ophthalmology VMMC and Safdarjung Hospital, 2 Department of Pediatric Ophthalmology and Strabismus and Neuro-Ophthalmology, Dr. Shroff’s Charity Eye Hospital, Daryaganj, New Delhi, 3 Department of Ophthalmology, Military Hospital, Ahmedabad, Gujarat, India Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/dljo.dljo_74_23 Submitted: 13‑Nov‑2022 Accepted: 19‑Jan‑2023 Published: 31-May-2023 © 2023 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow 31

On evaluation, his BCVA was 6/120 RE and 1/60 LE. Positive ocular findings included RAPD Grade 2 RE, temporal disc pallor (Figure 1) and defective colour vision in BE. MRI was unremarkable. Based on this clinical presentation, patient was advised mitochondrial NGS gene panel. Genetic analysis showed a homoplasmic pathogenic missense variation in the MT-ND6 gene (chrM:14484T>C). He was further advised low vision services. Discussion We report here two cases of LHON with different genetic mutations. Both cases were first clinically suspected as bilateral optic neuropathy but the presence of positive family history and absence of MRI findings prompted genetic testing in them. These cases provide an insight into the importance of detailed history taking as well as specificity of genetic testing wherever the clinician is suspicious of an underlying disease. The molecular genetics for LHON was established in 1988. Mitochondrial mutations account for 90 percent of the cases, namely m3460G>A encoding ND1, m11778G>A for ND4, and m 14484T>C for ND6, respectively. The m14484T>C mutation is associated with better visual recovery.5,8 Fifty percent of males, and 10% of females with a pathogenic mtDNA mutation develop optic neuropathy. The peak onset of LHON is 15 to 35 years, with 10 percent of carriers may be affected before age twelve.6,11 Fifty percent of male carriers and only ten percent of female carriers are affected with visual loss.7,9 It is mainly a clinical diagnosis featuring the signs of a central or ceco-central scotoma, impaired color vision, and optic nerve head pallor. All of these features were present in our patients. LHON may be asymptomatic in carriers, and symptoms can be termed as subacute onset if the duration of onset of symptoms is less than six months, dynamic between 6-12 months, and chronic if the presentation is > 1 year.8,13 It can be slowly progressive, childhood LHON (onset of symptoms less than 12 years of age) or late-onset (onset of symptoms after 45 years of age). A subacute presentation and maternal family history compelled us to do genetic testing and pointed us towards the correct diagnosis. The treatment for LHON mainly consists of disease-modifying agents, gene therapy, and symptomatic treatment for visual loss.9,14 Idebenone was the first approved treatment of LHON in the European Union.10,15 It acts by stimulating ATP production by bypassing the complex I subunit of the respiratory chain. Gene therapies mainly consist of allotopic rescue and recombinant mitochondrial therapy. Other modalities include antioxidants, antiapoptotic drugs like cyclosporine A, mitobiogenesis using oestrogen or metformin neuroprotective agents, and stem cell therapy.9,14 Due consideration should be given to improving the patient’s overall quality of life, which starts with timely diagnosing the disease—explaining its inheritance patterns and poor visual prognosis to the patient and family. Provision of Low vision aids and modifications in the lifestyle to avoid mitochondrial toxins such as tobacco, alcohol, and exposure to environmental pollutants, are other supplementary modalities. Conclusion Leber Hereditary Optic Neuropathy is a rare eye disease, and an inherited mitochondrial DNA mutation causes it. It characteristically affects young males. The diagnosis and management of this disease should be an integrated approach by the ophthalmology and neurology departments - mainly directed at stopping the disease progression and provide symptomatic relief to the patients. Visual prognosis tends to be poor; however, several newer treatment modalities are being considered and tried. The latest treatment available is the injection of intravitreal gene therapy which is still under trial. Financial support and sponsorship Nil. Figure 1: Optic disc images of right and left eye showing temporal pallor in both eyes 32 Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022

Conflicts of interest There are no conflicts of interest. References 1. Sadun AA, La Morgia C, Carelli V. Leber’s Hereditary Optic Neuropathy. Curr Treat Options Neurol. 2011 Feb;13(1):109-17. doi: 10.1007/s11940-010-0100-y. PMID: 21063922. 2. P. Y. W. Man, P. G. Griffiths, D. T. Brown, N. Howell, D. M. Turnbull, and P. F. Chinnery. The epidemiology of Leber hereditary optic neuropathy in the North East of England. American Journal of Human Genetics. 2003 JAN; 72(2): 333–9. 3. Valentino ML, Barboni P, Ghelli A, Bucchi L, Rengo C, Achilli A, Torroni A, Lugaresi A, Lodi R, Barbiroli B, Dotti M, Federico A, Baruzzi A, Carelli V. The ND1 gene of complex I is a mutational hot spot for Leber’s hereditary optic neuropathy. Ann Neurol. 2004 Nov;56(5):631- 41. doi: 10.1002/ana.20236. PMID: 15505787. 4. Manickam AH, Michael MJ, Ramasamy S. Mitochondrial genetics and therapeutic overview of Leber’s hereditary optic neuropathy. Indian J Ophthalmol. 2017 Nov;65(11):1087-1092. doi: 10.4103/ijo. IJO_358_17. PMID: 29133631; PMCID: PMC5700573. 5. Jurkute N, Harvey J, Yu-Wai-Man P. Treatment strategies for Leber hereditary optic neuropathy. Curr Opin Neurol. 2019 Feb;32(1):99-104. doi: 10.1097/WCO.0000000000000646. PMID: 30516647. 6. Newman NJ, Carelli V, Taiel M, Yu-Wai-Man P. Visual Outcomes in Leber Hereditary Optic Neuropathy Patients With the m.11778G>A (MTND4) Mitochondrial DNA Mutation. J Neuroophthalmol. 2020 Dec;40(4):547-557. doi: 10.1097/WNO.0000000000001045. PMID: 32969847. 7. Klopstock T, Yu-Wai-Man P, Dimitriadis K, Rouleau J, Heck S, Bailie M, Atawan A, Chattopadhyay S, Schubert M, Garip A, Kernt M, Petraki D, Rummey C, Leinonen M, Metz G, Griffiths PG, Meier T, Chinnery PF. A randomized placebo-controlled trial of idebenone in Leber’s hereditary optic neuropathy. Brain. 2011 Sep;134(Pt 9):2677-86. doi: 10.1093/brain/awr170. Epub 2011 Jul 25. PMID: 21788663; PMCID: PMC3170530. 8. Carelli V, La Morgia C, Ross-Cisneros FN, Sadun AA. Optic neuropathies: the tip of the neurodegeneration iceberg. Hum Mol Genet. 2017 Oct 1;26(R2):R139-R150. doi: 10.1093/hmg/ddx273. PMID: 28977448; PMCID: PMC5886475. 9. Amore G, Romagnoli M, Carbonelli M, Barboni P, Carelli V, La Morgia C. Therapeutic Options in Hereditary Optic Neuropathies. Drugs. 2021 Jan;81(1):57-86. doi: 10.1007/s40265-020-01428-3. PMID: 33159657; PMCID: PMC7843467. 10. Giorgio V, Petronilli V, Ghelli A, Carelli V, Rugolo M, Lenaz G, Bernardi P. The effects of idebenone on mitochondrial bioenergetics. Biochim Biophys Acta. 2012 Feb;1817(2):363-9. doi: 10.1016/j. bbabio.2011.10.012. Epub 2011 Nov 4. PMID: 22086148; PMCID: PMC3265671. Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022 33

Abstract Case Report Introduction Although the SARS-CoV-2 vaccines are reported to have acceptable safety profiles in phase 3 randomized controlled trials (RCTs), concerns regarding potential rare side effects including risk of thromboembolism remain a reason for vaccine hesitancy. Thromboembolic complications associated with thrombocytopenia have been frequently reported following immunization with Covishield, some of which have proved to be fatal. However, in view of benefits clearly outweighing the risks, administration of this vaccine has been continued in full flow.1,2 Subconjunctival hemorrhage, also referred to as hyposphagma, refers to accumulation of blood in the subconjunctival space, that might occur secondary to Valsalva maneuver (coughing, sneezing, straining, vomiting), ocular surgery or trauma, viral conjunctivitis, anticoagulation, systemic vascular disease (hypertension, diabetes, arteriosclerosis), or those with systemic conditions causing decreased coagulability. It is typically asymptomatic and resolves spontaneously.3 Here we report a case of bilateral subconjunctival hemorrhage following Covishield vaccination. Case presentation A 21-year-old female came to the outpatient department with sudden onset redness in both eyes a day after receiving the first dose of vector vaccine against Covid-19 (OxfordAstraZeneca/ Vaxzevria/ Covishield). Unaided visual acuity was 20/20 OU with intraocular pressure of 12mm Hg OD and 14mm Hg OS. Anterior segment examination revealed bilateral diffuse bright red patches of subconjunctival hemorrhage in the interpalpebral region (Figure 1a and 1b). Dilated fundus examination revealed a normal posterior segment. She gave history of one episode of nausea, vomiting and giddiness during the first hour of vaccination which was managed subsequently by the Adverse Effects Following Immunization (AEFI) screening team, following which she had a persistent headache. The next morning, she was alarmed to see the bright red appearance of the whites of both eyes. The patient had no other bleeding manifestations elsewhere. Blood pressure measurement revealed 116/70 mmHg. Complete blood count revealed normal platelet counts. Fibrinogen level, liver function tests (LFTs), lactate dehydrogenase (LDH), haptoglobin, and reticulocyte counts were also within normal range. Prothrombin time (PT) and International normalized A 21-year-old female presented to our centre with bilateral subconjunctival hemorrhage, a day after being administered Covishield vaccine. There were no other ocular or systemic complaints or history of adverse effects following immunization. Complete blood counts were within normal limits with slightly deranged blood clotting parameters. Subsequently, the patient was treated conservatively and was kept on close follow up which revealed complete resolution of the hemorrhage in a week’s duration. Keywords: Subconjunctival Haemorrhage, Covid-19, Covaxin, Covishield, Vaccination Address for correspondence: Prafulla Kumar Maharana, Associate Professor of Ophthalmology, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India. E-mail: [email protected] This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected] Cite This Article as: Mandal S, Ananya PR, Maharana PK. Bilateral subconjunctival hemorrhage in a young female following COVID 19 vaccination. Delhi J Ophthalmol 2022;32:34-6. Bilateral Subconjunctival Hemorrhage in a Young Female following COVID 19 Vaccination Sohini Mandal1 , Ananya P.R.1 , Prafulla Kumar Maharana1 1 Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/dljo.dljo_72_23 Submitted: 23‑Nov‑2022 Accepted: 14‑Jan‑2023 Published: 31-May-2023 34 © 2023 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow

ratio (INR) were mildly deranged (PT-14.4s, INR- 36.9s). Reverse Transcriptase Polymerase Chain Reaction (RTPCR) testing for COVID-19 showed negative result. There was no history of ocular trauma, precluding the most common cause of subconjunctival hemorrhage, especially in the young.3 Blood pressure was within acceptable range thus ruling out hypertension as the cause for the same and is in fact, more commonly seen in older individuals.3 The patient did not have any symptom (mucoid discharge, watering and photophobia) or sign (lid edema, conjunctival chemosis) of acute hemorrhagic conjunctivitis.4 There was no evidence of preceding Valsalva maneuver (cough, vomiting, sneezing, weight lifting) which can lead to sudden severe venous congestion and subconjunctival hemorrhage.5 The patient was kept on close follow up in view of mild derangement of PT and INR, which normalized on repeat testing. The patient was noted to have almost complete resolution of symptom and sign a week after (Figure 2a and 2b). Discussion Four subgroups of vaccines against the coronavirus have been developed, i.e., mRNA vaccines (BNT162b2, PfizerBioNTech; mRNA-1273, Moderna), protein subunit vaccines (NVX-CoV2373, Novavax), vector vaccines (Ad26.COV2, Janssen Johnson & Johnson, ChAdOx1 nCoV-19/ AZD1222, Oxford-AstraZeneca, aka Vaxzevria or Covishield), and whole virus vaccines (PiCoVacc, Sinovac; BBIBP-CorV, Sinopharm). A distinctive syndrome of vaccine-induced immune thrombotic thrombocytopenia (VITT) associated with pathogenic antiplatelet factor 4 antibodies (anti-PF4 Abs) has been reported after two adenoviral vector vaccines against SARS-CoV-2, ChAdOx1 nCoV-19 and Ad26.COV2.6,7 However, this thrombotic complication linked to SARS-CoV-2 vaccines is extremely rare with an estimated incidence of 0.73 per 100,000 doses of the ChAdOx1 vaccine.8 Ocular hemorrhagic features such as subconjunctival/ subretinal/ intraretinal may be one of the early or often the Figure 1: (a) Right eye slit lamp image (diffuse illumination) shows nasal and temporal subconjunctival hemorrhage at presentation shows nasal (b) Left eye slit lamp image (diffuse illumination) shows nasal and temporal subconjunctival hemorrhage at presentation a b Figure 2: (a) Right eye slit lamp image (diffuse illumination) shows almost complete resolution of the subconjunctival hemorrhage at 1 week of follow up, with small patches of remnant hemorrhage. (b) Left eye slit lamp image (diffuse illumination) shows almost complete resolution of the subconjunctival hemorrhage at 1 week of follow up, with small patches of remnant hemorrhage. a b Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022 35

presenting manifestation of systemic thromboembolic events therefore, recent history of COVID-19 infection and/or vaccination must be elucidated. Systemic examination and blood investigations such as complete blood count, liver/ kidney function tests, blood coagulation profile are necessary to order in such cases and immediate physician consult must be taken if these levels are significantly deranged unlike that in our case. Patients should be kept under close monitoring in view of possible systemic life-threatening hemorrhagic features. In our case, the only preceding history was vaccination, so we thought it could be the underlying cause. However, we could not establish any direct causal relationship. A literature review suggested several such reports so we thought to present this case to stimulate further research. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. References 1. Merchant HA. CoViD vaccines and thrombotic events: EMA issued warning to patients and healthcare professionals. J Pharm Policy Pract. 2021 Mar 24;14(1):32. 2. PINHO AC. COVID-19 Vaccine AstraZeneca: benefits still outweigh the risks despite possible link to rare blood clots with low platelets [Internet]. European Medicines Agency. 2021 [cited 2021 Oct 24]. Available from: https://www.ema.europa.eu/en/news/covid-19-vaccineastrazeneca-benefits-still-outweigh-risks-despite-possible-link-rareblood-clots. 3. Doshi R, Noohani T. Subconjunctival Hemorrhage. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 [cited 2021 Oct 24]. Available from: http://www.ncbi.nlm.nih.gov/books/ NBK551666/. 4. King AB, Walsh FB. Trauma to the head with particular reference to the ocular signs; injuries involving the hemispheres and brain stem; miscellaneous conditions; diagnostic principles; treatment. Am J Ophthalmol. 1949 Mar;32(3):379–98. 5. Chiu CH, Chuang YY, Su LH. Subconjunctival haemorrhage and respiratory distress. Lancet Lond Engl. 2001 Sep 1;358(9283):724. 6. See I, Su JR, Lale A, Woo EJ, Guh AY, Shimabukuro TT, Streiff MB, Rao AK, Wheeler AP, Beavers SF, Durbin AP, Edwards K, Miller E, Harrington TA, Mba-Jonas A, Nair N, Nguyen DT, Talaat KR, Urrutia VC, Walker SC, Creech CB, Clark TA, DeStefano F, Broder KR. US case reports of cerebral venous sinus thrombosis with thrombocytopenia after Ad26.COV2.S vaccination.JAMA. 2021;325(24):2448–2456. 7. Greinacher A, Thiele T, Warkentin TE, Weisser K, Kyrle PA, Eichinger S. Thrombotic thrombocytopenia after ChAdOx1 nCov-19 vaccination. N Engl J Med. 2021;384(22):2092–2101. 8. Chan BTB, Bobos P, Odutayo A, Pai M. Meta-Analysis of Risk of Vaccine-Induced Immune Thrombotic Thrombocytopenia Following ChAdOx1-S Recombinant Vaccine. 36 Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022

Abstract Case Report Introduction Retinitis pigmentosa (RP) refers to a group of inherited retinal dystrophies that are defined by the primary degeneration of rod and cone photoreceptors. RP is the most common cause of vision impairment, with a prevalence rate of 1:4000 worldwide.1 It may be X-linked, autosomal dominant, or autosomal recessive.2 Night blindness is a common early sign of RP, followed by concentric visual field loss, which reflects the primary malfunction of rod photoreceptors, and central vision loss later in life caused by cone dysfunction.1 Although often bilateral and symmetrical, presentations can also be unilateral.2 Microphakia or spherophakia is a single congenital anomaly of lens which is rare but usually present with ectopia lentis. Small lens diameter is the characteristic feature of microspherophakia.3 Microphakia, a congenitally abnormally small lens, which is the outcome of abnormal embryonic differentiation, commonly coexists with spherophakia (a spherically shaped lens).5 We present a case of bilateral microphakia and retinitis pigmentosa in this case report. Case Report A 33-year-old male presented to ophthalmology department with chief complaint of significant decrease of vision in both the eyes for 4-5 years which was gradual, progressive and painless. The past history of decrease in vision during night since childhood was present with no previous eye consultation. His medical history was unremarkable. His family history was negative for any ocular disease. On examination, his uncorrected visual acuity was hand movements in both the eyes with no improvement with pinhole. Intraocular pressure was assessed using Goldmann Applanation Tonometry and was within normal limits at 10 mm Hg in the right eye and 14 mm Hg in the left eye. Anterior segment examination revealed bilateral phacodonesis, a well-formed anterior chamber, a mature cataract in the right eye, and an immature thick cataract in the left eye. Bilateral microphakia was seen with 360 degree intact zonular support [Figure 1]. Right eye A-scan revealed anterior chamber depth of 2.25 mm and the lens thickness was 4.70 mm. Left eye lens measured 4.32 mm in thickness and the anterior chamber depth was 2.69 mm. Corneolenticular contact was not observed. A A series of progressive inherited retinal dystrophies known as retinitis pigmentosa include degeneration of rods followed by the loss of cone photoreceptors. This case report of 33-year-old male describes an uncommon Bilateral Retinitis Pigmentosa With Bilateral Microphakia: An Uncommon Association association of bilateral retinitis pigmentosa with bilateral microphakia. Keywords: Microphakia, Retinitis Pigmentosa Address for correspondence: Rajwinder Kaur, MBBS, MS, Professor Department of Ophthalmology Adesh Institute of Medical Sciences and Research, Barnala Road, Bathinda, Punjab, India. E-mail: [email protected] This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected] Cite This Article as: Aggarwal A, Kaur R, Khan B, Dahiya P. Bilateral retinitis pigmentosa with bilateral microphakia: An uncommon association. Delhi J Ophthalmol 2022;32:37-9. Bilateral Retinitis Pigmentosa with Bilateral Microphakia: An Uncommon Association Anupriya Aggarwal1 , Rajwinder Kaur1 , Balbir Khan2 , Priyanka Dahiya1 1 Department of Ophthalmology, Adesh Institute of Medical Sciences and Research, Barnala Road, Bathinda, Punjab, 2 Department of Ophthalmology, Gian Sagar Medical College and Hospital, Banur, India Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/dljo.dljo_75_23 Submitted: 08‑Feb‑2022 Accepted: 21‑Jan‑2023 Published: 31-May-2023 © 2023 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow 37

dilated fundus examination of the left eye showed waxy disc pallor, arteriolar attenuation, and bone spicule pigmentation in the peripheral retina [Figure 2]. Right eye fundus examination was not possible due to mature cataract. An ultrasound of the right eye showed echogenic deposits in the lens suggestive of cataract-related changes along with few curved echogenic membranes in the vitreous but no evidence of vitreous or retinal detachment was found. Right eye cataract surgery with anterior vitrectomy was performed and +18.00 diopter scleral fixed intraocular lens was implanted. There were no difficulties during surgery and postoperative period was uneventful. Following cataract surgery, a right eye fundus examination also revealed waxy disc pallor, arteriolar attenuation, and bone spicule pigmentation in the peripheral retina. Discussion Retinitis pigmentosa (RP), which affects more than 1.5 million people globally, is a leading cause of vision impairment and blindness. Photoreceptors and the retinal pigment epithelium both gradually degenerate in RP. Even though few unilateral RP cases have been reported, bilateral RP cases are more common.2,6 The early signs of RP include difficulty adapting to the dark and night blindness (nyctalopia). Loss of the mid-peripheral visual field is another symptom that might occasionally manifest, however it is rarely noted as an early sign. The central retina remains relatively preserved until the final stages of the disease. The defining characteristics of RP are three clinical features: waxy pallor of the optic nerve, attenuation of retinal capillaries, and pigmentation of bone spicules. Fundus examination may be normal in the early stages of RP, as bone spicule‒shaped pigment deposits are either absent or sparse, vascular attenuation is minimal, and the optic disc is normal. RPE cells, which separate from Bruch membrane after photoreceptor degeneration, migrate to intraretinal perivascular locations, where they produce melanin pigments, are responsible for the pigmentation of the bone spicules. These bone spicules often arise in the mid-periphery, where the concentration of rod cells is highest. According to ISCEV standards, full-field electrophysiological testing aids in the diagnosis and is crucial for quantifying the severity of the disease and tracking its progression. A comprehensive family history is essential in each patient suspected of having RP, and a pedigree chart is recommended.1 Figure 1: Showing Bilateral Microphakia Figure 2: Left eye showing features of Retinitis Pigmentosa 38 Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022

Small and spherical lenses are the typical characteristics of microspherophakia. In normal individuals, the equatorial diameter of the lens increases from 6.5 mm at birth to around 9.0 mm by the age of 15, and it remains same throughout life. The sagittal diameter increases to approximately 4.75 to 5.0 mm by the age of 80 to 90 years. On full mydriasis, the lens equator was visible and lens tremors were noted on changes in posture of patient. The most common complication, glaucoma, affects up to 51% of eyes and is assumed to be caused by pupillary block produced by spherical lenses, which is linked to improper lens zonule growth. In our case, neither shallow AC nor secondary glaucoma was present. Systemic diseases such WeillMarchesani syndrome,7 Marfan syndrome, hyperlysinemia, Alport syndrome, Lowe’s syndrome, Klinefelter syndrome, Peter’s anomaly, mandibulofacial dysostosis, cri-du-chat syndrome, and chondrodysplasia punctate are associated with microspherophakia.4 No clinical characteristics of these illnesses were found in our patient. In the literature, isolated microspherophakia and retinitis pigmentosa have also been linked.8 As far as we know, there haven’t been any reports of bilateral RP being associated with microphakia. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. References 1. Verbakel SK, van Huet RA, Boon CJ, den Hollander AI, Collin RW, Klaver CC, Hoyng CB, Roepman R, Klevering BJ. Non-syndromic retinitis pigmentosa. Progress in retinal and eye research. 2018 Sep 1;66:157-86. 2. Gupta S,Kamble VK.Unilateral Retinitis Pigmentosa.J Ophthalmol & Vis Sci. 2019; 4(1):1029. 3. Nirankari MS,Maudgal MC.Microphakia.Br J Ophthalmol.1959 May;43(5)314-6. 4. Liu Q, Wang X, Zhang S. Visual quality observation of clear lens extraction by ultrasonic phacoemulsification and intraocular lens implantation in a child with microspherophakia: A case report. Medicine. 2020 Aug 21;99(34). 5. Esson DW. Clinical atlas of canine and feline ophthalmic disease. John Wiley & Sons; 2015 Oct 12. p. 207-9. 6. Weller JM, Michelson G, Juenemann AG. Unilateral retinitis pigmentosa: 30 years follow-up. Case Reports. 2014 Feb 10;2014:bcr2013202236. 7. Jethani J, Mishra A, Shetty S, Vijayalakshmi P. Weill-Marchesani syndrome associated with retinitis pigmentosa. Indian journal of ophthalmology. 2007 Mar 1;55(2):142-3. 8. Gupta G, Gupta P, Thakur A, Murugan B, Ram J. Isolated microspherophakia with retinitis pigmentosa. Indian journal of ophthalmology. 2019 Jul 1;67(7):1160-. Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022 39

Abstract Case Report Introduction A 70-year-old female presented with blurring of vision. She gave history of ocular surgery without lens implantation 20 years back in the left eye (OS) and five years back in the right eye (OD). On examination, visual acuity was hand movements in both eyes (OU). The slit lamp examination revealed pseudoexfoliation (PXF) with aphakia in OU with complete herniation of vitreous knuckle, bulging through the pupillary aperture with corneal endothelial touch without corneal edema/ decompensation in OD and aphakia in OS without vitreous disturbance. In OS, the patient was diagnosed with PXF, aphakia, and vitreous touch syndrome (Figure 1 B and D). Vitreous Touch syndrome is a late complication of intra capsular cataract extraction (ICCE) where the vitreous bulges through the pupillary aperture and attaches to the corneal endothelium. No age, gender and racial predisposition have been documented for this syndrome.1 This patient had previously undergone ICCE with a presumably intact vitreous face. Eventually, the herniation of the vitreous into the anterior chamber resulted in corneal endothelial touch (Figure 2). Surprisingly there was no corneal decompensation for this patient (Figure 3). In OD, the patient presented with aphakia and PXF (Figure 1 A and C). The deposition of fibrillary white flaky PXF material on the cornea was noted (Figure 4 A and B) with peri-pupillary transillumination defects (Figure 1A). Patient gave history of cataract surgery done 5 years back with no placement of intraocular lens (IOL). The possible A 70-year-old female presented with blurring of vision and gush of warm fluid in the left eye (OS). She gave history of ocular surgery without lens implantation 20 years back in OS and five years back in the right eye (OD). On examination, visual acuity was hand movements in OS. The slit lamp examination revealed pseudoexfoliation with aphakia in both eyes with complete herniation of intact vitreous knuckle, bulging through the pupillary aperture and touching the corneal endothelium without corneal decompensation in OS and remnant posterior capsular rent in OD without vitreous disturbance. Keywords: Vitreous Herniation, Vitreous Touch Syndrome, Aphakia Address for correspondence: Prasanna Venkatesh Ramesh, MS, DNB, MNAMS, FGSR, FAICO (Glaucoma), FICO (Glaucoma) Department of Glaucoma and Research, Mahathma Eye Hospital Private Limited, No. 6, Seshapuram, Tennur, Trichy, Tamil Nadu, India. E-mail: [email protected] This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected] Cite This Article as: Ramesh PV, Ramesh SV, Ramesh MK, Rajasekaran R. Iatrogenic induced complete herniation of vitreous - Atypical presentation of vitreous touch syndrome without corneal edema/ decompensation in a bilateral aphakic patient with pseudoexfoliation glaucoma. Delhi J Ophthalmol 2022;32:40-2. Iatrogenic Induced Complete Herniation of Vitreous - Atypical Presentation of Vitreous Touch Syndrome without Corneal Edema/ Decompensation in a Bilateral Aphakic Patient with Pseudoexfoliation Glaucoma Prasanna Venkatesh Ramesh1 , Shruthy Vaishali Ramesh2 , Meena Kumari Ramesh2 , Ramesh Rajasekaran3 1 Department of Glaucoma and Research, Mahathma Eye Hospital Private Limited, Trichy, 2 Department of Cataract and Refractive Surgery, Mahathma Eye Hospital Private Limited, Trichy, 3 Department of Paediatric Ophthalmology and Strabismus, Mahathma Eye Hospital Private Limited, Trichy, India Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/dljo.dljo_76_23 Submitted: 09‑Dec‑2022 Accepted: 23‑Jan‑2023 Published: 31-May-2023 40 © 2023 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow

reason for no IOL placement could have been PXF-related issues, eventually leading to posterior capsule dehiscence, complicating the surgery and very poor compliance after surgery. In OU, patient had advanced secondary glaucoma (Figure 5) with poor visual prognosis, so treatment planned was conservative. Figure 1: Slit lamp photography. A. Anterior segment photography of right eye showing pupillary ruff atrophy (red arrows) and remnant posterior capsule with opacification. B. Anterior Segment photography of left eye showing vitreous in the anterior chamber with vireo-corneal contact (red arrows). C. High magnification of slit lamp image showing pseudoexfoliation (PXF) material deposit on the corneal endothelium (red arrow). D. Anterior segment photography of left eye showing pupillary ruff atrophy (red arrows) and peripheral iridotomy Figure 2: A and B. Vitreo-corneal touch seen on Scheimpflug imaging system in normal and inverse colour, respectively. C. Anterior segment OCT of left eye revealing vitreous touch syndrome Figure 4: A and B. PXF material deposit seen on Scheimpflug imaging system in normal and inverse colour, respectively of the right eye. C. Anterior segment OCT of same eye showing normal open angles Figure 3: A and B. Normal specular microscopy picture of right and left eye, respectively Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022 41

Figure 5: A. Fundus photograph of the left eye showing glaucomatous optic atrophy. B. OCT of the retinal never fibre layer revealing the same finding. C. Normal OCT macula of the same eye. D. B-scan of the right eye done in view of hazy media revealing few nuclear fragments in the vitreous cavity Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient’s guardian as given consent for the child’s images and other clinical information to be reported in the journal. The patient’s guardian understands that the child’s name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. References 1. 1.Snip, RC; Kenyon, KR; Green, WR (Feb 1975). “Retrocorneal fibrous membrane in the vitreous touch syndrome”. American Journal of Ophthalmology. 79 (2): 233–44. 2. Couch SM, Bakri SJ. Use of triamcinolone during vitrectomy surgery to visualize membranes and vitreous. Clin Ophthalmol. 2008 Dec. 2(4):891-6. 3. Schmidt JC, Chofflet J, Hörle S, Mennel S, Meyer CH. Three simple approaches to visualize the transparent vitreous cortex during vitreoretinal surgery. Dev Ophthalmol. 2008. 42:35-42. Pars plana vitrectomy may be planned in OS to prevent corneal decompensation.2,3 42 Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022

Abstract Case Report Introduction Skin and soft tissue diseases form a large and heterogeneous group of mesenchymal extraskeletal and dermatologic lesions in humans. Diseases of the skin and soft tissue can develop virtually anywhere in the body including extremities, trunk, retroperitoneum and the head and neck region. Signs and symptoms vary according to the type and location of the lesion. If they are close to the surface of the body, they may appear as a lump. Common benign soft tissue tumors include: 1. Lipomas -benign tumors composed of fatty tissue 2. Angiolipoma 3. Benign fibrous histiocytoma 4. Neurofibroma 5. Schwannomas 6. Neurilemmoma 7. Hemangiomas 8. Myxoma In addition to the myriad of cysts (epidermoid, trichilemmal, mucous, ganglion, embryologic, steatocystomas), subcutaneous masses may also arise from any cell type (mesenchymal, neural, adipose, adnexal), presenting as benign or their malignant counterparts.1 Case Report A 46 years old male presented in our OPD with history of painless, slowly progressive mass over left brow region for the last one year. It was oval soft to firm well circumscribed mass, about 2-3cms in size. Overlying skin appeared normal. Patient had already undergone FNAC in other department but it was not diagnostic because of insufficient tissue. He was advised MRI but he refused because of financial constraints. Ocular examination was normal. We made a diagnosis of benign soft tissue tumor and planned excision biopsy of that lesion. During surgery we were surprised to see a deep red colored highly vascular lesion (Figure 1 and 2). Specimen was then sent for HPE, which stated the presence of large dilated vessels with cyst like lumen lined by single layer of endothelial cells, suggestive of Cavernous Hemangioma (Figure 3 and 4) Discussion Cavernous hemangioma is an abnormal cluster of tightly packed, thin-walled capillaries which resemble a raspberry. It has blood-filled spaces, separated by connective tissue. Hemangiomas can range in size from a fraction of an inch to larger ones. Cavernous hemangiomas can affect about 1 in Soft tissue tumors (STT) are a heterogeneous group of benign and malignant lesions. Some are assumed reactive, and others are clearly neoplastic. They can occur in skin, subcutaneous tissue, muscles, ligaments, nerves and blood vessels. These tumors vary widely in how they look and behave. More than 99% of STT are benign in nature. Here we report a case which we presumed to be lipoma but it turned out to be cavernous hemangioma. Keywords: Soft Tissue Tumors; Cavernous Hemangioma; Benign; Orbital Tumors Address for correspondence: Mukta Sharma, (MS Ophthalmology) Department of Ophthalmology Dr. RKGMC, Hamirpur, HP, India. E-mail: [email protected] This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected] Cite This Article as: Sharma M, Thakur R, Shalini. Appearances can be deceptive. Delhi J Ophthalmol 2022;32:43-5. Appearances can be Deceptive Mukta Sharma1 , Rajneesh Thakur2 , Shalini1 1 Department of Ophthalmology, Department of Ophthalmology , Dr. RKGMC, Hamirpur, 2 Department of Pathology, Department of Ophthalmology , Dr. RKGMC, Hamirpur, HP, India Access this article online Quick Response Code: Website: https://journals.lww.com/djo DOI: 10.4103/dljo.dljo_77_23 Submitted: 04‑Dec‑2022 Accepted: 07‑Feb‑2023 Published: 31-May-2023 © 2023 Delhi Journal of Ophthalmology | Published by Wolters Kluwer - Medknow 43

200 people. They are usually discovered between the ages 20 and 30 years. Although they are quite common, only 1 in 10 people will ever develop symptoms.2 This benign lesion develops due to endothelial dysmorphogenesis from a lesion which is present at birth. Cavernous hemangioma is a misnomer as it does not display endothelial hyperplasia. The abnormal tissue causes a slowing of blood flow through the cavities, or “caverns”. The blood vessels do not form the necessary junctions with surrounding cells, and the structural support from the smooth muscle is hindered, causing leakage into the surrounding tissue. It is the leakage of blood, referred to as hemorrhage, that causes a variety of symptoms known to be associated with the condition. Symptomatic visual impairment occurs as a result of involvement of the optic nerve, extraocular muscles or surrounding vasculature.3 Cavernous Hemangioma can be present in : Central nervous system Spinal cord Head and neck Chest Abdominal Musculoskeletal/soft tissue Cavernous hemangiomas are the most common vascular lesions of the orbit in adults, being commonly seen in middle Figure 1: Intraoperative picture of the lesion Figure 2: Deep red vascular lesion sent for HPE Figure 3: HPE section seen under 10X magnification Figure 4: Histopathology picture of the lesion showing dilated spaces lesion showing dilated spaces with single lined endotheliumstratified squamous epithelium alongwith lacrimal gland and hair follicles 44 Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022

age, with a female predilection. Over 80% of orbital cavernous hemangiomas are located within the intraconal compartment, most commonly in the lateral aspect.They usually present as progressive painless proptosis.4 Cavernous venous malformation is diagnosed radiologically after a thorough clinical assessment. The differential diagnosis for a well circumscribed, round to oval, solid orbital mass include: peripheral nerve sheath tumors (schwannoma and neurofibroma), hemangiopericytoma, fibrous histiocytoma, solitary fibrous tumor and melanoma.5,6 Management of cavernous venous malformation is dependent on the presence or absence of symptoms. For small, asymptomatic lesions, annual follow up including pupil exams, visual acuity, color vision, exophthalmometry, dilated fundoscopy, visual fields and CT or MRI studies is appropriate. For larger lesions causing diplopia or visual disturbances, surgical excision is the treatment of choice. There is no current role for radiotherapy. Treatment of cavernous venous malformation is via surgical excision. There are currently no medical therapies for the treatment of cavernous hemangioma.The choice of approach to surgical excision of a cavernous venous malformation depends on the location and size of the tumor. Cavernous venous malformations involving the anterior two-thirds of the orbit can be resected via an anterior eyelid, transconjunctival or transcaruncular approach. More posteriorly located tumors may require a lateral orbitotomy. A transcranial approach may be required for lesions involving the orbital apex. A cryoprobe is often used to aid in removal with minimal blood loss. Even large tumors are usually removed easily and completely.7,8 Conclusion The management of skin and soft tissue diseases in middleincome countries is faced with many challenges ranging from late presentation, limited financial resources, difficulty in establishing the correct diagnosis due to paucity of experts in these regions who can satisfactorily handle skin and soft tissue diseases and nonexistence of ancillary diagnostic techniques such as immunohistochemistry services and fluorescence microscopy which aid in making correct pathological diagnosis. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. References 1. Picci, P. Epidemiology of Soft Tissue Lesions.Diagnosis of Musculoskeletal Tumors and Tumor-like Conditions. Springer, Cham. Htt.2020. 2. hields JA and Shields CL. Eyelid, Conjunctival and Orbital Tumors. 2nd ed. Philadelphia: Lippincott, Williams & Wilkins; 2008: 522. 3. Oncology. 2012;10(1):p. 188. doi: 10.1186/1477-7819-10-188. 4. Orbit, Eyelids and Lacrimal System, Section 7. Basic and Clinical Science Course, AAO, 2020-2021, p. 75-77. 5. Rootman J, Heran M K, Graeb D A. Vascular malformations of the orbit: classification and the role of imaging in diagnosis and treatment strategies. Ophthal Plast Reconstr Surg. 2014;30(02):91–104. 6. Carter CS, Patel RM. Cutaneous soft tissue tumors: diagnostically disorienting epithelioid tumors that are not epithelial, and other perplexing mesenchymal lesions. Mod Pathol. 2020 Jan;33(Suppl 1):66-82. doi: 10.1038/s41379-019-0387-5. Epub 2019 Nov 4. PMID: 31685962. 7. Dutton JJ, Bryne SF, Proia AD. Diagnostic Atlas of Orbital Disease. 1st ed. Philadelphia: W.B. Saunders Company; 2000: 66 8. Gelli MC, Pasquinelli G, Martinelli G, Gardini G. Cutaneous meningioma: histochemical, immunohistochemical and ultrastructural investigation. Histopathology. 1993;23(6):576–578. Delhi Journal of Ophthalmology ¦ Volume 32 ¦ Issue 6 ¦ October-December 2022 45