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DEPARTMENT OF OPHTHALMOLOGY AND VISION SCIENCES Departmental Report 2015 CONTENTS INTRODUCTION Chief’s Message 5 Mission Statement 7 Who We Are INTRODUCTION RESEARCH EDUCATION CLINICAL HOW TO HELP The Foundations of Innovative Care 17 Training the Next Generation of Leaders 39 Clinical Care Changes Lives 53 Your Support Changes Lives 67 Research Programs 19 Educational Programs 41 Clinical Services 55 Equipment Needs 69 Impact Factor 33 Fellowship Program 43 Your Support Drives Innovation 71 Residency Program 47 What Comes Next 73 11 4 A MESSAGE FROM OUR CHIEF When a fundamental commitment to give the best and most compassionate care to children is combined with an ambitious, talented research and teaching agenda, set in a global environment as dynamic and diverse as ours, the impact of that commitment multiplies, benefitting countless children and their families—not only in Toronto, but across Canada and around the globe. Vision is our window to understand and appreciate the world. From the feeling we get when we see a loved one’s smile, to the way it helps us perform complex tasks, vision is one of the essential senses that drives human life. We seem to assume that children are innocent, that they are too young to have eye problems that adults commonly face, other than perhaps a few who need to wear glasses. Unfortunately, eye diseases do not discriminate between the young and the old, and when children develop an eye problem, it affects not only their sight, it also deprives them of many opportunities in the most crucial time of their lives to grow, to learn, and to play. The doctors and the eye care team in SickKids Ophthalmology believe that every child has the right to the best healthcare. With this commitment in mind, we lead a clinical program that is one of the best and largest in the world, serving over 20,000 children every year. We are the only centre in Canada that offers comprehensive services dedicated to children, providing the most complex eye care that covers all subspecialties and advanced surgical interventions. Many of our INTRODUCTION In the following pages, you will learn more about our achievements and successes. Through carefully selected images, we want to illustrate how when a fundamental commitment to give the best and most compassionate care to children is combined with an ambitious, talented research and teaching agenda, set in a global environment as dynamic and diverse as ours, the impact of that commitment multiplies, benefitting countless children and their families—not only in Toronto, but across Canada and around the globe. We also want to convey the depth and sophistication of our programs, and how satisfying it is to be part of a department that is as vibrant as SickKids Ophthalmology. You will also learn about the challenges we face. We rely on the support of donors, partners, and interested parties like you to continue our mission to promote children’s eye health and treat eye diseases by integrating excellent clinical care, rigorous research, and world-class education. Children deserve the best care because they are our future. They are like canaries in the coal mine to remind us to be present, to pay attention to what is important in life and do the right thing. I hope that this report will reaffirm your commitment to our cause and the importance of our mission. The contribution you make to our mission truly changes lives, no matter what form it takes. Sincerely, Dr. Agnes Wong talented and experienced physicians have formal training in multiple disciplines, and they are supported by an equally dedicated and compassionate team of allied health professionals. We also have an internationally renowned research program, with an excellent track record of productivity. We strive to bring children the newest, safest, and state-ofthe-art clinical care through research and innovation. Many of our physicians and scientists are engaged in basic and clinical research, and are expert leaders in their fields. We are affiliated with the University of Toronto and offer one of the best and largest paediatric ophthalmology training programs in the world. We train all levels of students and future generations of doctors who go on to work in many communities within Canada and across the globe. Our physicians and scientists have received multiple teaching awards and are sought-after lecturers at major universities and institutions around the world. We have also launched a Global Care and International Ophthalmology initiative that is driving the development of collaborative healthcare solutions worldwide. 6 Dr. Agnes Wong, Ophthalmologist-in-Chief MISSION STATEMENT Our goals are: to promote children’s eye health and treat eye diseases through excellent clinical care; to create new scientific and clinical knowledge through rigorous research; and to train future generations of healthcare professionals through our world-class educational programs. We are committed to providing the best and most innovative care to the children we treat, by integrating clinical care with research and education. We are also committed to fostering a multidisciplinary, collaborative, and respectful team environment, so that each team member enjoys opportunities for educational advancement, work satisfaction, and personal growth. INTRODUCTION 8 “It is a privilege to be a member of this talented and supportive group of practitioners and scientists, whose dedication to improving eye care for children will continue to make an impact in Canada and abroad.” —DR. STEPHEN KR AF T INTRODUCTION 10 Left to right: Drs Buncic, Wong, Vincent, Mireskandari, Héon, Kraft, and Ali WHO WE ARE OPHTHALMOLOGIST-IN-CHIEF Agnes Wong, MD, PhD Neuro-Ophthalmology, Strabismus STAFF OPHTHALMOLOGISTS Asim Ali, MD Anterior Segment, Glaucoma, Uveitis, Strabismus Andrew Budning, MD Retinoblastoma Ray Buncic, MD Neuro-Ophthalmology, Craniofacial Anomalies Dan DeAngelis, MD Oculoplastics, Orbit Brenda Gallie, MD Retinoblastoma Elise Héon, MD Ocular Genetics, Retinoblastoma Peter Kertes, MD Retina Stephen Kraft, MD Strabismus Wai-Ching Lam, MD Retina Kamiar Mireskandari, MBChB, PhD Anterior Segment, Glaucoma, Uveitis, Strabismus, Retinopathy of Prematurity Rajeev Muni, MD Retina Nasrin Najm-Tehrani, MBBCh, MSc Anterior Segment, Glaucoma, Uveitis, Strabismus, Retinopathy of Prematurity Robert Pashby, MD Oculoplastics David Smith, MD Strabismus Ajoy Vincent, MBBS, MS Ocular Genetics, Visual Electrophysiology SCIENTIST Carol Westall, OD, PhD INTRODUCTION Visual Electrophysiology 12 Dr. Nasrin Najm-Tehrani using a slit lamp to examine a patient RESEARCH THE FOUNDATION OF INNOVATIVE CARE Drs. Elise Héon and Ajoy Vincent working in the Ocular Genetics Laboratory Surgical Innovation Led by Drs. Ali, Mireskandari, and Najm-Tehrani Ocular Genetics Led by Drs. Héon and Vincent NeuroVision Led by Drs. Wong, Westall, and Kraft Our high-impact research programs allow for rapid translation of innovative methods to the bedside, and provide unparalleled research training opportunities for residents, fellows, and graduate students from local, national and international institutions. RESEARCH Telemedicine and Retinopathy of Prematurity Led by Drs. Najm-Tehrani and Mireskandari Retinoblastoma Led by Dr. Gallie SICKKIDS OPHTHALMOLOGY IS INTERNATIONALLY RECOGNIZED FOR CUTTING-EDGE RESEARCH. We collaborate with other divisions and departments at SickKids on numerous research projects, using a multidisciplinary approach that provides new perspectives on old problems. We also have an excellent track record of research productivity and funding, including having two endowed research chairs. We offer six major research programs, led by physicianscientists, scientists, and clinicians—all global leaders in their areas of expertise. Child Eye Care Advocacy Led by Dr. Wong RESEARCH 18 RETINOBLASTOMA FIGHTING RETINOBLASTOMA AROUND THE GLOBE The One Retinoblastoma World (1RBW) program is a collaborative research initiative designed to bring together caring persons from around the world to develop strategies to fight retinoblastoma. This emerging consortium held its second meeting in Toronto in October 2014. 1RBW is a forum to encourage innovation and sharing of ideas. Under the global health science leadership of Drs. Brenda Gallie and Helen Dimaras, 1RBW now offers four models for innovation: High Income 627 survivors (7.8%) 13 deaths (0.2%) Global Retinoblastoma Disease Burden Low Income 1,760 survivors (22%) 5,600 deaths (70%) Retinoblastoma is a rare but critical form of cancer that affects the eyes of 8,000 children around the world each year. 2009–2014 National Retinoblastoma Strategies and Guidelines: We started in Canada with the first National Retinoblastoma Strategy and Guidelines for care, which has now been adopted in Kenya. With Daisy’s Eye Cancer Fund International, we have supported the seven-year Kenyan National Retinoblastoma Strategy, to improve care for affected children and families across Kenya. 2014 Global mapping of retinoblastoma treatment centres: Based on our mapping resources and expertise, we launched a survey collecting information on the availability of retinoblastoma care around the world. We are developing a web-based platform that will visually illustrate this complex data, connect global caregivers to each other, and ultimately strengthen the ability to care for the global retinoblastoma population by increasing research capacity and improving collaboration. This will accelerate the translation of results from labs to clinics. 2015 Open data on retinoblastoma: To actualize the national guidelines we developed for retinoblastoma care, we are housing our research and clinical data on a Telus cloud server, so that it is accessible to each practitioner globally in the circle of care of each affected child. We are studying how this communication tool impacts quality of care, outcomes for patients, and quality of communications between professionals and families. 2020 Real-time global analysis of outcomes: Analyzing treatment outcomes is key to improving clinical care for retinoblastoma. We are developing algorithms to analyze data for similarly staged and treated patients while maintaining patient privacy, to generate an evidence-base for management of future cases. This revolutionary, first-of-its-kind concept in healthcare will use emerging technology to tap into the “brain” of our global network of experts, to optimize personalized medicine for each child. Due to the rarity and broad global distribution of retinoblastoma, randomized clinical trials have not been successful. Global sharing will allow doctors and scientists to research extremely detailed, real-life data, to advance treatment in ways previously not possible. Unfortunately, 70% of children with retinoblastoma worldwide die, particularly those born in lower income countries, where access to specialized care is limited. Although 98% of children in developed countries survive, the impacts of treatment are significant: removal of an eye, chemotherapy and many rounds of laser treatments are often required, as well as long-term repeated monitoring for cancer progression, recurrence and new cancers. Expert care for retinoblastoma is available in a limited number of centres around the world. The 8% of children born in high-income countries have potential to access expert care. By collaborating through our One Retinoblastoma World program, we’re opening a clear path to optimized care for children worldwide, with the goal of making retinoblastoma a “Zero Death Disease.” SickKids is a major international leader in retinoblastoma research, focusing on three key areas: 1 Translation of molecular knowledge to save vision in the next generation of children. RESEARCH 2 Collaborative effort to optimize outcomes for all children with retinoblastoma. 3 A global cloud-based database system collecting point-of-care data for caregivers that supports clinical choices. 20 “Vision impacts all aspects of a child’s life. I see the profound impact of the work we do on our patients every day, and I am proud to be part of a team that is deeply committed to serving their needs.” —DR. ASIM ALI RESEARCH 22 Electrophysiological tests help to decipher and characterize retinal and visual pathway disorders. SickKids Ophthalmology offers a wide array of electrophysiological tests to aid diagnosis and management of patients. Jeff Locke (orthoptist) and Elizabeth Gomes (ophthalmic assistant) performing a visual evoked potential test on Dr. Ajoy Vincent. TELEMEDICINE AND RETINOPATHY OF PREMATURITY ONTARIO ROP TELEMEDICINE PROGRAM Timmins Timmins and District Hospital NICU Retinopathy of Prematurity (ROP) is a disorder that primarily affects premature babies. The smaller a child is, the more likely they are to develop ROP, typically in both eyes. ROP is one of the most common causes of vision loss for children, and can lead to lifelong vision impairment or blindness. Screening for ROP has traditionally been performed using a technique called binocular indirect ophthalmoscopy. However, this is a difficult skill to learn, and there is a shortage of ophthalmologists who are adequately prepared to perform these examinations on at-risk, premature infants. As neonatal care advances and more premature infants survive, the demand for ROP screening is increasing. While these children may receive effective overall care from remote facilities, a shortage of ophthalmologists with ROP expertise poses difficulties for children in need of screening. Under the leadership of Drs. Najm-Tehrani and Mireskandari, our Ontario ROP Telemedicine program is working to improve these conditions. This program has ensured that infants from NICUs in Sudbury and Barrie no longer need to be transferred to SickKids for eye examinations. Additionally, infants at risk of ROP can be transferred a shorter distance to Sudbury, from the NICU at Timmins. Our model has also been used as a template to start a second telemedicine network between the McMaster Children’s Hospital in Hamilton and the Grand River Hospital NICU in Kitchener. Expansion of the program to provide coverage for other remote NICUs is being planned, and the location of the next screening site will be confirmed by the fall of 2015. Sudbury Health Sciences North NICU The program was a recipient of the Ontario Ministry of Health and Long-Term Care’s achievement award for improving access to care at the Celebration of Innovation in 2010. Through our research and clinical practice, SickKids Ophthalmology has driven the alternative use of a specialized camera to capture photographs of the interior surface of the eye, which can then be sent to expert ophthalmologists for diagnosis. While ophthalmic imaging is a skill that requires specialized training, SickKids Ophthalmology has been successful in using live, two-way audiovisual connections to train nurses to capture these images, even if they have no experience in ophthalmology or ophthalmic photography. This research has been a cornerstone for the development of the Ontario ROP Telemedicine Program, which helps train neonatal intensive-care unit (NICU) nurses already working in remote hospitals to learn this new skill, helping optimize the use of nursing resources across Canada. Barrie Royal Victoria Hospital NICU These research-based programs have not only created significant reductions in health care costs, they have improved access to care and largely eliminated the need for transfer of these fragile infants to specialized hospitals in the province for their critical eye examinations, thus avoiding morbidity associated with travelling long distances. Toronto SickKids Kitchener Grand River Hospital NICU RESEARCH 24 Hamilton McMaster Children’s Hospital RESEARCH 26 Cone photoreceptor dysfunction is the underlying cause of several retinal disorders. Dr. Westall’s Retinal Imaging Laboratory focuses on imaging single cone cells in vivo and studying the retinal micro-vasculature. Drs. Westall and Vincent discussing findings with lab members. NEUROVISION NEUROVISION DRIVES INNOVATION NeuroVision is an innovative, multidisciplinary research program that brings together clinicians and scientists to assess different aspects of visual function. NeuroVision’s multidisciplinary approach creates opportunities for exciting collaborations among a wide range of SickKids departments, as well as with local, national and international experts. Here are just some of the innovations that have been driven by the NeuroVision program’s research: The goal of the program is to understand how vision develops in normal infants and children, in comparison to children with different eye diseases. NeuroVision investigates how vision is received from the retina and optic nerve, through to the visual cortex and higher levels of brain processing. Our research also examines the basis of eye movement control and the associated development of binocular vision and depth perception. SickKids Ophthalmology is one of the few paediatric centres around the world with all of the expertise and technology needed to study visual processing in children under one roof. Clinical questions guide our research, while new knowledge revolutionizes the assessment and treatment of visual and neurological disorders in children, which can then be applied directly at the bedside. Ultimately, NeuroVision seeks to optimize visual outcomes by better understanding visual functions and dysfunctions. Papilledema due to large arachnoid cyst A large arachnoid cyst displacing the right temporal lobe Normal left temporal lobe Pons Cerebellum RESEARCH Apparatus in a soundproof chamber for testing audiovisual integration in patients with lazy eye. • New and important knowledge about the underlying mechanisms, diagnosis and management of disorders such as “lazy eye” and eye misalignment. • New ways to test for the earliest signs of diabetes in adolescents’ eyes, which will guide diagnosis and treatment before vision is affected. • How poor vision affects the development and integration of other motor and sensory systems, including eye and limb movements, as well as hearing. • Knowledge of the effects of seizure medications on retina that led to a change in position from the FDA regarding protocols when using vigabatrin. • Improving surgical planning for children with eye muscle problems. • How neurological syndromes affect the development of vision in children. • Determining optimal goals for aligning eyes, in relation to several different eye muscle conditions, in order to give children the best chance for long-term successful results. 28 “We create new knowledge by implementing a rigorous strategy of integrating clinical care with research and education.” —DR. ELISE HÉON RESEARCH 30 The genetic basis of eye disorders is intriguing with over 300 disease-causing loci. Dr. Héon’s Ocular Genetic Laboratory focuses on characterization of known and unknown genetic eye diseases. OCULAR GENETICS The field of Ocular Genetics has grown significantly in the last decade, with over 300 genes now associated with diseases of the eye. We are one of the largest users of genetic testing in Canada. This has allowed us to decipher the basis of several eye disorders through our research. Dr. Héon leads numerous genetic studies of eye disorders that are present at birth and deteriorate over time, known as retinal dystrophies. Our patients have benefitted from access to the earliest clinical trials of new therapeutic interventions, such as gene replacement therapy. The application of innovative therapies to treat inherited forms of blindness is rapidly evolving, and we are prepared to be active players in this exciting new era. SURGICAL INNOVATION Surgical Innovation is a research program that focuses on techniques to improve the management of various kinds of vision loss, including trauma, severe retinal damage, and deformities of the eye existing at birth. This means introducing new techniques and instrumentation, improving ways of planning surgery using imaging, and reviews of surgical outcomes. The goal of the program is to improve care for these patients, many of whom have very complex problems that demand unconventional solutions. Innovative techniques developed and studied in our department include conventional and selective corneal transplantation, ocular surface reconstruction and corneal neurotization surgery. CHILD EYE CARE ADVOCACY Children are not small adults—their eyes are different and require proper development to be healthy. Our Child Eye Care Advocacy research program develops unique and effective eye care strategies that are critical for promoting excellent vision. Research on strategies that improve overall health is of growing interest for decision makers when allocating resources. Our long-term goal is to devise the best and most cost-effective eye care strategies for children of all ages in Ontario. RESEARCH 32 Seeing the world from a shifted perspective: Jaime Sklar, MSc adapts to laterally-shifting prism goggles in Dr. Wong’s Eye Movement & Vision Neuroscience Laboratory. FACULT Y PUBLICATIONS BY IMPACT FACTOR (IF) 2014 IF PUBLICATION 8.3 Neurology 6.5 Canadian Medical Association Journal 6.4 Modern Pathology 6.3 Annals of Surgery 5.6 Journal of Medical Genetics AA RB DD BG EH PK WL KM NT AV 2 4.5 JAMA Ophthalmology 4.2 European Journal of Human Genetics 3.7 Investigative Ophthalmology and Visual Science CW AW 1 1 2 1 IF PUBLICATION AA RB DD BG EH PK 1.9 Eye 1.3 Canadian Journal of Ophthalmology 2 1.3 Ophthalmic Surgery, Lasers and Imaging Retina 1 1.2 Ophthalmic Genetics 1.1 Documenta Ophthalmologica 1.1 Journal of the American Association for Paediatric Ophthalmology and Strabismus N/A Practical Radiation Oncolocy N/A Neuroimage: Clinical WL KM NT AV CW AW 1 2 1 1 1 1 1 1 3.5 Journal of Plastic and Reconstructive Surgery 3.5 PLoS One 3.2 Retina 2.8 British Journal of Ophthalmology 2.8 Journal of Biomedical Optics 2.6 Virchows Archiv 1 2.5 Vision Research 1 2.0 American Journal of Medical Genetics Part A 2.0 Brachytherapy 1 2.0 Journal of Ovarian Research 1 1.9 Ophthalmologica 1.9 Canadian Urological Association Journal 1 1.9 Clinical & Experimental Ophthalmology 1 1 4 1 1 1 1 1 2 1 RESEARCH BG: BRENDA GALLIE EH: ELISE HÉON PK: PETER KERTES AA: ASIM ALI RB: RAY BUNCIC DD: DAN DEANGELIS 1 1 1 WL: WAI-CHING LAM KM: KAMIAR MIRESKANDARI NT: NASRIN NAJM-TEHRANI AV: AJOY VINCENT CW: CAROL WESTALL AW: AGNES WONG 1 1 “In the evolution of our department over the last 40 years, we have developed an enviable academic culture, in which service to our patients is rendered with outstanding quality, our teaching is the best among the academic hospitals, and our research is active, growing and world-class.” 1 —DR. R AY BUNCIC 34 EDUCATION TRAINING THE NEXT GENERATION OF LEADERS Dr. David Smith during a teaching session for trainees SUMMARY OF MAJOR EDUCATIONAL PROGRAMS Number of students per year ~190 15–20 Medical Students Dr. Najm-Tehrani Residents Dr. Mireskandari 6 Fellows Dr. Ali 6 Graduate Students Drs. Gallie, Héon, Westall, Wong 2–3 International Medical Graduates Drs. Ali, Wong 1–2 Nursing Students Beverly Griffiths, RN EDUCATION WHILE OUR RESEARCH PROVIDES THE FOUNDATION FOR THE INNOVATIVE CARE WE DELIVER TO PATIENTS, IT IS ALSO INTEGRAL TO OUR EDUCATIONAL EFFORTS. SickKids Ophthalmology is internationally recognized for its high-quality educational programs. These programs provide cutting-edge clinical training in general paediatric ophthalmology and various subspecialties to different levels of medical trainees, as well as to non-ophthalmologists. Students and trainees within the department are also actively engaged in our research programs, so that their learning is informed by the latest innovative thinking. By providing opportunities for students to participate in research development, we ensure that they become global thought leaders in their field. Our teaching practices are also integrated into our daily patient clinics and surgical sessions, to offer students the hands-on experience they need to become leaders within their fields. Our mandate also extends to the education of parents and families dealing with eye disorders, both in the clinical setting and through our support groups. 40 Dr. Ray Buncic educating a patient and family member THESE PROGRAMS INCLUDE Weekly one-hour formal department rounds on a range of subject matter, from research findings by department members and external colleagues, to instructive case presentations to research proposals for prospective or retrospective analyses. Regular sub-specialty teaching rounds and journal clubs, held by members of the department, covering all the major subspecialty areas. These rounds vary in format, including case-based teaching, reviews of recent literature, and research talks on design, statistics, and study proposals. Education of families on an ongoing basis in the clinic, through patient examinations and proposed treatments, as well as formal encounters such as ocular genetics counselling. The department is also involved in many support groups, such as the Canadian Retinoblastoma Society, Paediatric Glaucoma and Cataract Family Association, and Brandan’s Eye Research Foundation, either as board members or as participants in educational initiatives. Frequent formal research rounds, arranged by our director of research, Dr. Carol Westall. Fellows and vision science students (including undergraduate and graduate students) present their research proposals and periodic progress reports in this forum. These sessions also include teaching of research methods. EDUCATIONAL PROGRAMS Teaching clinics at the Orthoptic Clinic, which has been a valuable resource for training of residents, fellows, undergraduate medical students, and elective students from other disciplines for many years. Orthoptists—allied health-professionals specializing in ocular motility and visual development—are integrated into the teaching clinics, where they instruct trainees in examination methods and non-surgical treatment of various eye disorders. Our educational programs include several initiatives that integrate research and clinical practices, with emphasis on translational research that drives new treatments for patients. EDUCATION 42 40 YEARS, 123 ALUMNI, 25 NATIONS The SickKids Ophthalmology fellowship program is one of the world’s largest fellowship programs focused on paediatric ophthalmology and strabismus. 18 27 1 1 6 1 27 1 5 1 2 2 1 4 1 1 1 1 3 1 1 3 1 3 2 FELLOWSHIP PROGRAMS Our Paediatric Ophthalmology and Strabismus Fellowship program has trained well over 100 fellows since its inception more than 40 years ago, and is accredited by the Association of University Professors of Ophthalmology. countries, with over 20 nations represented by our alumni community. Many become department heads and are involved in fellowship education themselves, making the impact of our training both multigenerational and multinational in scope. We train four fellows every year, making it one of the world’s largest fellowship programs focused on paediatric ophthalmology and strabismus. Our goal is to provide comprehensive training in paediatric ophthalmology, as well as adult and childhood strabismus. Most of our graduates move on to academic positions in their home A key strength of our program lies in our faculty’s depth and breadth of experience, with all major subspecialties represented. All staff physicians take an active role in teaching fellows and are engaged in continuously improving the program. As a major referral centre, the range of diseases and disorders that are seen by our fellows is unparalleled. EDUCATION 8 Canada Iran USA Saudi Arabia Mexico Kuwait Colombia Oman Guyana Pakistan Chile India United Kingdom Sri Lanka Ireland Malaysia Netherlands Singapore Germany The Philippines Nigeria South Korea Egypt Australia Israel New Zealand 44 LEGEND =5 =1 RETINOBLASTOMA CLINICAL FELLOWSHIP Preceptors: Drs. Gallie and Héon NEURO-OPHTHALMOLOGY FELLOWSHIP Preceptors: Drs. Buncic and Wong The Retinoblastoma Clinical Fellowship is a one-year program that recruits fully trained ophthalmologists who will focus their life’s work on retinoblastoma—a form of eye cancer. The Neuro-Ophthalmology Fellowship program is one of a few in the world that offers training dedicated exclusively to children. We provide extensive exposure to the complete spectrum of paediatric neuro-ophthalmologic conditions. Organized around the principle of graduated responsibility, fellows develop knowledge, competence, and confidence in diagnosis, as well as skills for medical and surgical management of common and rare neuro-ophthlamologic conditions under the close supervision of staff physicians. Through multidisciplinary consultations and co-management of patients, we offer numerous opportunities for educational interactions with related specialties, including neurology, neurosurgery, neuro-oncology, neruoradiology, neuropathology and craniofacial anomalies. Fellows may enter the program based on funding they have received, or the department will seek out funding for them on a case-bycase basis when we determine that the training will play a key role in equipping the fellow to enhance global retinoblastoma care. We also welcome ophthalmologists, who are already leaders in retinoblastoma in their country, to join us for three-to-six month observerships. Past fellows continue to consult with us on difficult cases, using innovative digital tools for sharing care decisions, and actively participate in One Retinoblastoma World initiatives. EDUCATION PAEDIATRIC CORNEA AND EXTERNAL DISEASES FELLOWSHIP Preceptors: Drs. Ali and Mireskandari We are the only department in the world to have two staff physicians specially trained in both paediatric ophthalmology, as well as in cornea and external diseases, which includes developmental, infective and inflammatory diseases that lead to scarring and need for corneal transplantation. Our unique expertise and the wide variety of cases allow us to offer the world’s only dedicated training fellowship in paediatric cornea and external diseases. The aim of the fellowship is to train ophthalmologists who will become national leaders for children’s corneal disease in their home country. Our first fellow, Dr. Uri Elbaz, came from Israel in 2012 and is expected to become a national expert in this field upon his return. Another unique feature of the fellowship is a strong component of research training, with ample protected time for fellows to complete rigorous research under the guidance of experienced clinician-scientists and clinicians. Upon completion of their training, fellows are expected to become a leader in this field. Dr. Steve Kraft, Dr. Ray Buncic, and Heather MacDonald (ocular genetic counsellor) during Friday morning Ophthalmology rounds 46 OCULAR GENETICS FELLOWSHIP Preceptors: Drs. Héon and Vincent The Ocular Genetics Fellowship program involves a combination of both clinical activity and laboratory exposure in the field, tailored to the educational needs of the fellow, through a one-to-three year program. Trainees are expected to return to an academic position, apply their newly gained knowledge and contribute to advancing the field forward. Through the program, we help trainees acquire the knowledge and skills needed to diagnose a wide range of genetically determined eye disorders. These include inherited retinal dystrophies, genetically determined complex syndromes with ocular manifestations, and metabolic disorders. Knowledge underlying genetics of anterior segment disorders and cancer is also gained. Our fellows gain a deep understanding of the mechanism underlying the diseases, and learn about evolving treatment approaches in the field including gene therapy. The trainee gains good exposure to interpretation of visual electrophysiology and genetic results. They learn to analyze exome/genome data and to provide ethical genetic counseling. Teaching session for residents and fellows RESIDENCY PROGRAM SickKids Ophthalmology is responsible for the clinical and surgical training of all residents in paediatric ophthalmology at the University of Toronto (U of T). We are unique in North America as the only residency program to provide a total of eight dedicated months of training in paediatrics for residents. There are four residents at SickKids Ophthalmology during each rotation. Our commitment to training involves individualized education in clinics. We also have a comprehensive syllabus of teaching, covering all vital topics in paediatrics as part of the Toronto Ophthalmology Residency Introductory Course, and the U of T teaching series for residents. Our physicians have received numerous teaching awards from residents at U of T. In the last three years, we are proud to have inspired a third of the graduating residents to develop a specialized interest in paediatric ophthalmology. There is a great shortage of paediatric ophthalmologists, not only in the Greater Toronto Area, but also across Canada. We strive to continue to guide residents into this field by equipping them with knowledge and skills to treat children throughout their career. OUR CONFERENCES Jack Crawford Day, named after our former department chief, is an annual full-day paediatric ophthalmology conference that provides opportunities for practitioners to receive continuing medical education credits through our partnership with the University of Toronto, as well as keynote lectures by department staff and visiting faculty on the latest research in vision sciences. The Lloyd Morgan Lectureship Series was initiated in 2003 to honour the first Chief of Paediatric Ophthalmology at SickKids. We invite experts from ophthalmology or allied health fields on a periodic basis for a formal program involving a site visit and a number of lectures. The subject matter generally focuses on evolving research with clinical applications. EDUCATION Friday morning Ophthalmology rounds provide a forum for guest speakers, medical and clinic staff, as well as trainees to exchange knowledge and ideas. 48 CLINICAL CLINICAL CARE CHANGES LIVES Dr. Dan DeAngelis performing and teaching surgeries to residents and fellows CHANGING LIVES FOR THE BETTER IS OUR MISSION AT SICKKIDS. Cataract Visual Electrophysiology Cornea and External Diseases Uveitis Glaucoma CLINICAL SERVICES Strabismus NeuroOphthalmology Retinopathy of Prematurity Ocular Genetics Retinoblastoma Oculoplastics Retina CLINICAL While our educational programs help train the next generation of leaders, and our research ensures that we are developing more effective treatments, the clinical care we provide allows us to directly improve health outcomes for children. Children are not just small adults—in fact, the field of paediatric ophthalmology is a subspecialty because it deals with unique children’s eye diseases. The visual system continues to develop after birth and well into adulthood as the brain matures, so early diagnosis and treatment of eye diseases is critical for best visual outcomes. The management of paediatric eye diseases requires intricate knowledge of embryology, eye and brain development, and genetics. Special skills and equipment are also required for examining and treating children and small infants. Many children with eye problems have other related health problems or developmental issues. Paediatric ophthalmologists contribute to multidisciplinary teams by examining children to confirm or exclude signs of a multi-system disorder affecting the eye. They also work closely with allied health-professionals and schools to help care for children with visual impairments. 54 Traumatic cataract CATARACT SERVICE Drs. Mireskandari, Ali, Najm-Tehrani A cataract is a cloudy lens in the eye that disrupts the amount and quality of light entering the eye. Cataract surgery requires specialized care and consideration for children. A baby’s visual system is not fully developed, which means that cataract surgery must be accompanied by extensive rehabilitation to ensure they can develop vision later in life. Our Cataract Service provides the majority of paediatric cataract surgery in Ontario. Through a multidisciplinary approach to performing surgery, providing specialized contact lenses, glasses or lens implants, and the dedication of parents to their children’s visual rehabilitation, outcomes for surgery are continuing to improve. Optic nerve cupping Optic nerve drusen permanent impairment of vision. Treatment of cornea scarring demands specialized expertise and can require corneal transplantation. not fully developed, a child’s eye can grow out of proportion because of the increase in pressure. Treatments including medication and surgery are used to reduce this pressure. problem. Our monitoring of the visual status of a child is also critical for other services when making management decisions. Our Glaucoma Service provides the specialized care required for children with glaucoma. The majority of paediatric glaucoma surgery in Ontario is performed at SickKids. Prevention of glaucoma from injury or disease is vitally important to our team, and improved management of conditions like cataracts and uveitis should reduce the incidence of glaucoma and the burden of the disease on patients. OCULAR GENETICS SERVICE Drs. Héon, Vincent, Gallie Our Cornea and External Diseases Service consists of experts in areas such as corneal transplantation and implantation of artificial corneas. We provide innovative treatments including reconstruction of eye abnormalities, regenerating nerves connected to the cornea, and improving the shape of the cornea. We provide autologous serum tears for our patients struggling with loss of tear production, and specialized contact lenses for patients with irregular corneas, which can act as an alternative to surgery. Both members of the team have a strong research interest in the use of imaging to improve outcomes for our patients. GLAUCOMA SERVICE Drs. Ali, Mireskandari, Najm-Tehrani CORNEA AND EXTERNAL DISEASES SERVICE Drs. Ali, Mireskandari The cornea is the clear portion of the eye in front of the iris that acts as its window to the world. Problems with cornea clarity will impair sight directly, and it can easily be affected by infections or injury. A number of conditions can cloud the cornea from birth and cause CLINICAL Glaucoma is a condition that damages the optic nerve. Some children are born with it, while others develop it during childhood. Irregularities in the eye’s drainage system cause a build up of pressure within the eye that can lead to loss of vision. Glaucoma requires specialized care and consideration in children. Not only is a baby’s visual system NEURO-OPHTHALMOLOGY SERVICE Drs. Wong, Buncic Our Neuro-Ophthalmology Service provides highly specialized care to patients experiencing impaired vision as a result of diseases of the brain. These include conditions such as inflammation of the optic nerve, optic disc swelling, brain tumors and strokes. The service works very closely with other services at SickKids, including the Visual Electrophysiology Unit, neurology, neurosurgery, neuro-oncology, neuroradiology, and ocular genetics. We are often the first doctors to discover that there is a problem in the brain when a child presents with a vision 56 Stargardt Disease (juvenile macular degeneration) Ocular Genetics is fast becoming an increasingly essential part of ophthalmology, as most eye disorders are determined by variations in our genes. The Ocular Genetics program at SickKids is unique in the country and one of very few in the world. Identifying the genetic basis of an eye condition allow us to offer better counseling to our patients and helps us assess the eligibility of treatment. We provide comprehensive care and counseling for all our patients, and provide genetic testing for most of them, leveraging our state of the art facilities. Some of our patients were the first to access a genereplacement therapy trial for a blinding disorder in children. This is a stepping-stone to improve the outcome of many other conditions. Drs. Héon and Vincent care for a large referral population of inherited non-cancerous disorders, while Drs. Héon and Gallie care for our retinoblastoma patients—the most common form of eye cancer, which is genetically determined. TREATING THE WORLD’S FIRST TRIPLETS WITH RETINOBLASTOMA CASE STUDY Hemangioma OCULOPLASTICS SERVICE Drs. DeAngelis, Pashby The Ophthalmic Plastic and Reconstructive Surgery Service—commonly referred to as “oculoplastics”—provides treatments for patients that are born with or develop disorders related to the tear ducts, eyelids and eye sockets. The unit consists of both clinical and surgical divisions that deal with a wide range of disorders. Our oculoplastic patients come from across Canada with complex cases, often requiring collaboration with other surgical experts within SickKids, including specialists in neurosurgery, craniofacial surgery, head and neck surgery, and interventional radiologists that use minimally invasive image-guided procedures to diagnose and treat diseases. RETINA SERVICE Drs. Lam, Kertes, Muni Our Retina Service provides clinical consultation, care for patients requiring specialized diagnosis, and leads surgical treatments for retinal disorders. Although the majority of our patients come from Ontario, complex cases from across Canada and the world are often referred to our service. CLINICAL Retinoblastoma The Retina Service treats a wide range of retinal disorders that threaten loss of vision, including eye traumas, retinal detachment, and Coats disease. We use cutting-edge technologies to treat these complex cases, including specialized cameras that improve diagnosis, and instruments that make retinal surgery minimally invasive. RETINOBLASTOMA SERVICE Drs. Gallie, Héon, Budning Retinoblastoma is a form of cancer that rapidly develops from a child’s retina cells, and is the most common form of malignant tumor found in children’s eyes. Unfortunately, 70% of cases worldwide lead to death, with a disproportionate number of deaths occurring in developing countries where access to early detection is unavailable. In 2013, Edmonton’s Low family was happily surprised to discover they were about to welcome triplets into their life. Thomas, Mason and Luke Low were all born healthy at 34 weeks. Unfortunately, the Low family had another surprise coming—and this one was terrifying. When the boys were just a few months old, their father Richard noticed that Mason had an abnormally shaped pupil. A retinal specialist in Edmonton who examined the boys reached out to SickKids’ Dr. Gallie, who suggested images be captured of Thomas and Luke’s eyes as well. These photos, along with further examinations, illustrated that all six of the triplet’s eyes were affected by retinoblastoma, an aggressive form of cancer. Our Retinoblastoma Service manages the care of 80% of the newly diagnosed Canadian children each year, with 22 new cases predicted annually. We work in collaboration with referring doctors, so that afflicted children can travel to SickKids to receive active care. This unit is made up of a wide range of healthcare professionals, including ophthalmologists, oncologists, social workers, nurses, imaging specialists and genetic counselors. Treatment often involves use of highly specialized equipment and innovative therapies. 58 However, each child had one eye with good prospects for visual development and their tumours were small. Through support from the Alberta government, and the goodwill of numerous Toronto citizens that have donated financially and provided local accommodations, the Low’s are receiving ongoing treatment and support from SickKids’ retinoblastoma team. Today, the prognosis for all three boys is positive, and the SickKids team continues to work with the Low family to save as much of the triplet’s vision as possible, while stopping the spread of their cancer. Our team is committed to continuing to monitor their treatment to ensure the best possible visual outcomes for the Low triplets. Neovascular tufts in retinopathy of prematurity Strabismus surgery We strive to deliver care for children and families struggling with retinoblastoma as close to home as possible, while ensuring they receive optimized treatment. In cases where the disease can be stabilized, ophthalmologists within the child’s community can perform follow-up examinations. Due to the screening training for ophthalmologists across northern Ontario, in some cases patients need to be transported to SickKids for examination. Our success in the timely detection and treatment of ROP has resulted in a remarkable decrease in the number of cases in Ontario that require surgery. This allows our retinal surgery specialists to focus on providing care to out-of-province and international patients. in Alberta—where there is a young population with a high birth-rate—Dr. Gallie now works on staff at the Alberta Children’s Hospital. Every other month, she consults with children at the Alberta clinic and assesses whether they need to receive care from the SickKids team in Toronto. This saves Albertan families time and money, while reducing the emotional burden of leaving their community to receive specialized care. RETINOPATHY OF PREMATURITY SERVICE Drs. Mireskandari, Najm-Tehrani Retinopathy of Prematurity (ROP) is a potentially blinding disease that affects premature newborns. It is associated with abnormal growth in the retina’s blood vessels that leads to retinal scarring or detachment. Infants who are born at least ten weeks early, or that weigh less than 1250 grams, are at risk of developing ROP. The ROP Service unit is committed to ensuring screening for at-risk infants. We have a mandate to provide ROP screening for the premature infants in our own neonatal intensive-care unit, as well as all other NICUs in the Greater Toronto Area. While we provide CLINICAL STRABISMUS SERVICE Drs. Kraft, Smith, Wong, Mireskandari, Najm-Tehrani, Ali Strabismus is a condition where the eyes are not properly aligned, causing adverse effects on vision. Our Strabismus Service also treats a wide-range of related issues, including problems with depth perception, double vision, eye muscle problems and “lazy eye.” The Strabismus Service handles approximately 70% of the complex childhood strabismus cases in the Greater Toronto Area. We also house the only eye movement laboratory and diagnostic facility in Ontario. SickKids offers a comprehensive diagnosis and treatment base for strabismus problems in children, driven by a multidisciplinary approach that sees us collaborate with including Ocular Genetics and Neurology. The Strabismus Service is supported by the orthoptic staff, who are allied health personnel who perform the eye muscle v Iris adhesions balance tests to detect and measure eye misalignment. Orthoptists test the child’s vision in order to detect amblyopia (“lazy eye”), and they also evaluate binocular vision, including tests of stereovision and tests of the ability of the eyes to work together. They are certified by written and oral examinations of the Canadian Orthoptic Council. They are integrated into the clinical, teaching, and research activities of the Department of Ophthalmology and Vision Sciences. Until recently, the department housed one of only 4 accredited orthoptic training programs in Canada, and we hope to reopen the school to prospective students within the next couple of years. UVEITIS SERVICE Drs. Najm-Tehrani, Ali, Mireskandari middle layer of the eye—that can lead to permanent loss of vision. Screening for is a complex clinical problem that requires a multidisciplinary approach. progress in improving care for these patients by creating a network of ophthalmologists with specialized knowledge, collaborating with ophthalmology nurses, and coordinating care with rheumatology physicians and nurses. We are developing new pathways for the safe management of patients with the potential to develop uveitis, and optimizing interventions 60 Choroideremia to lower the likelihood of irreversible vision loss. This has also enabled us to offer better outcomes for patients with uveitis undergoing cataract surgery. VISUAL ELECTROPHYSIOLOGY SERVICE Drs. Vincent, Westall Our Visual Electrophysiology Unit (VEU) performs a wide array of tests to assess the health of different components of the visual system. These tests allow us to examine the integrity the retina and the optic pathway, as well as the macula, a highly specialized region in the retina that we are now able to study in great detail. Additionally, we offer various methods of assessing colour vision, clearness of vision, and 3D depth perception. Depending on the demands of the situation, the unit offers specialized tests under sedation and in operating room conditions, making us the only site in Ontario that provides this service. As such, we receive many out-of-province referrals. The unit aims to provide excellent visual function evaluation to all patients and lead paediatric visual assessments internationally. The VEU has a history of improving testing techniques and applies newly devised techniques to enhance patient care. Since electrophysiological tests are essential in the monitoring of novel gene replacement therapies currently being tested, the importance of the VEU will continue to increase. THE WORLD’S FIRST NERVE GRAFT TRANSPLANTATION TO RESTORE EYE SENSATION CASE STUDY Abby Messner lost feeling in her left eye after a non-malignant tumour in her brain was removed at age 11. As a result of this condition—called corneal anaesthesia—her eye would not blink to protect against dust and debris. Despite her best efforts to care for her eye, this led to a scar forming on her cornea that doctors worried would lead to permanent vision loss. Her condition also prevented her from enjoying her favourite activities, such as playing competitive sports like hockey and swimming, or just entering the dry air of a mall. Even a windy day posed a danger to Abby’s eye. This led SickKids’ ophthalmologist, Dr. Ali, to propose an innovative new surgery for Abby—a nerve graft that would restore feeling to her left eye. Working with plastic and reconstructive surgeons at the hospital, Dr. Ali developed a treatment that took a nerve from Abby’s calf, transplanted it into her functioning right eye, and connected it to her left eye. Within three months, sensation was restored. This new treatment addressed the root problem of the condition rather than its complications, in a less invasive fashion than other surgeries used to treat corneal anaesthesia. SickKids Ophthalmology has since used this surgery in seven other cases and continues to study the effects of this innovative treatment. CLINICAL Dr. Asim Ali with Drs. Gregory Borschel and Ronald Zuker from Plastic and Reconstructive Surgery at SickKids examining the first patient who has undergone corneal neurotization surgery. 62 HOW TO HELP YOUR SUPPORT CHANGES LIVES YOUR SUPPORT CHANGES LIVES. The wide range of diseases and disorders that can impact a child’s vision pose considerable challenges to healthcare providers, but even bigger challenges to the child and their family. We are still working to understand the full scope of the visual system and the ailments that affect it. When you support SickKids Ophthalmology, you are helping change lives. We work to provide the most comprehensive clinical care to our patients here in Toronto, while developing innovative new treatments that save eyes and lives around the world. In this section you will learn about the challenges we still face and how your support can help us in our mission. HOW TO HELP Dr. Arun Reginald reviewing and examining a patient with a resident 68 EQUIPMENT NEEDS The speed of innovation in ophthalmology is tremendous and new technologies constantly emerge to improve the diagnosis and management of eye diseases. We strive to constantly improve the instruments and diagnostic equipment we use for the care of our patients. Here are some examples of equipment we need to help us improve outcomes for the children we treat. The total cost for all of this equipment would be more than $1,000,000. Every dollar you contribute to SickKids Ophthalmology helps. 1. Next generation optical coherence tomographer (OCT) with autofluorescence (Heidelberg Spectralis): The OCT is part of the standard of care for imaging of the retina, and has widespread applications in all areas of ophthalmology. The improvements in OCT technology allow us to make diagnoses of retinal disorders earlier than ever before, and are much more child-friendly, with lower light levels and eye-trackers. 2. Handheld optical coherence tomographer (Bioptigen Envisu): This allows the use of the OCT in infants under general anaesthesia. 3. Collagen cross-linking light source: Cross-linking is a technique which is used to stop the progression of keratoconus, a condition of the cornea in which there is thinning and irregularity resulting in decreased vision, requiring a corneal transplant. This would allow us to provide this treatment for young children affected by the disease, as well as those who are developmentally delayed. 4. Flare meter (Kowa): This allows precise measurement of inflammation inside the eye, in children with uveitis and after eye surgery. This is much more accurate than manual methods of estimation and would allow us to accurately evaluate the effect of our therapies. 5. Widefield retinal imaging (Optos): This allows a complete photographic scan of the retina with minimal light, which is normally very uncomfortable, especially for younger children. This will speed up and allow for more complete examinations, while also reducing the discomfort experienced by patients. 6. Adaptive optic imaging system: This revolutionary technology permits images of the individual photoreceptor cells, which sense light in the eye. This allows us to capture images of disorders in these cells at the highest possible resolution. 7. Anomaloscope: The most accurate way to assess colour vision, a standard in all research protocols. 8. Dark adaptometer: Allows assessment of visual function in dim lighting conditions. HOW TO HELP 70 YOUR SUPPORT DRIVES INNOVATION Our world-leading research is the foundation that allows us to deliver innovative care to our patients. Below are some examples of our ongoing research projects and our needs that your support will help push forward. NEW TECHNIQUE TO IMAGE CORNEAL CELLS IN LIVE PATIENTS Corneal endothelial cells are vital to maintaining the clarity of the cornea and preserving normal vision. They cannot regenerate and can become permanently damaged or destroyed by disease or surgery. Endothelial cell loss can eventually lead to corneal swelling, pain and loss of vision. This has its greatest impact in childhood when the visual system is immature. applied to patients. Since its inception in 1994, the Ocular Genetics Program at SickKids Ophthalmology has served over 20,000 patients afflicted with all kinds of inherited eye diseases from diverse genetic backgrounds. It is uniquely positioned to lead in this exciting new era of gene replacement therapy. Your contribution will accelerate new discovery so that blind children can see again. RECRUITING CLINICIAN-SCIENTIST Clinician-scientists function as bridges between the clinical and the scientific worlds by interacting with patients, observing the clinical features of their diseases, and then developing basic science experiments to test new ideas that stem from their observations. As clinical and scientific knowledge continues to increase exponentially, recruitment of the best and brightest clinician-scientists is critical to our long-term success. However, clinician-scientists require extensive training, infrastructural support, and “protected time” for them to perform research. Funding to support their salary and research—in the form of an endowed Chair or Professorship—is crucial for us to train, recruit and retain talented clinician-scientists who recognize the relevance of scientific discovery and how it changes patients’ lives. Your contribution can help us create this important endowment. We are now able to look at these cells with a specular microscope in young children, using techniques developed in our department, either awake or under general anaesthetic. This enables us to establish normal ranges in children under the age of five and to assess the damage that occurs in a variety of eye disorders and following surgery. Your support will help us find new ways to prevent corneal blindness and reduce the need for corneal transplants in children. UNDERSTANDING NOVEL RETINAL GENES TO PREPARE FOR THE FIRST GENE THERAPY More than 60 percent of blindness among infants is caused by inherited eye diseases. With advances in large scale genetic testing, novel candidate genes are being identified at a very rapid pace. Before these novel candidate genes can be manipulated for therapeutic purpose, we need to first verify their significance in causing diseases through meticulous investigation. Once verified, the correct copies of the genes can be experimentally injected into eyes with the goal to effect a cure. These treated eyes will need to be assessed for functional and structural improvement following treatment, and when results are encouraging, new therapy could then be HOW TO HELP 72 Dr. Dan DeAngelis performing surgery WHAT COMES NEXT In the next five years, SickKids Ophthalmology will continue to build on our existing strength to achieve a new level of excellence, by developing themes that unify our clinical, research, and educational activities. This will include use of an evidencebased approach to evaluate and determine the health services that we provide, making a real impact on national and global health, and enhancing knowledge translation from research into practical application. The support of philanthropic leaders like you is more important than ever, so that we can continue to set our sights high and keep working to advance children’s eye health in profound new ways. For further information please contact Adam Starkman at The SickKids Foundation | 416-813-2942 | [email protected] Or, visit www.sickkidsfoundation.com and specify your donation to “SickKids Ophthalmology”. HOW TO HELP Dr. David Smith with a patient 74 Jennifer Colaco, RN, and Margaret Horie, RN, with a patient and mother SICKKIDSOPHTHALMOLOGY.COM