Keratoconus Diagnosis and Treatment Sujata Das Editor 123 Keratoconus Sujata Das Editor Keratoconus Diagnosis and Treatment Editor Sujata Das L V Prasad Eye Institute Bhubaneswar, Odisha, India ISBN 978-981-19-4261-7 ISBN 978-981-19-4262-4 (eBook) https://doi.org/10.1007/978-981-19-4262-4 © The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature Singapore Pte Ltd. 2022 This work is subject to copyright. All rights are solely and exclusively licensed by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. 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The registered company address is: 152 Beach Road, #21-01/04 Gateway East, Singapore 189721, Singapore Foreword The problem of keratoconus is gaining increasing attention all over the world as the newer diagnostic approaches made it possible to recognize this entity earlier and better. This is now considered the most common ectatic disorder of the cornea. Multiple factors contribute to the causation of keratoconus— genetic, mechanical, and other factors. The management of this problem has several options ranging from simple optical correction in the early stages to a variety of surgical procedures in the later stages, usually with high degree of success. Dr Sujata Das is a very experienced and accomplished clinician with spe- cial interest in keratoconus. Other contributing authors are all very well- known corneal specialists with excellent record of publishing. This book has benefitted immensely from this pool of talent and hence provided a compre- hensive coverage of this topic. I congratulate the authors on this effort and recommend this volume to all those who wish to get more information on keratoconus from a single source. L V Prasad Eye Institute, Gullapalli N. Rao L V Prasad Marg, Banjara Hills Hyderabad, Telangana, India v Foreword It is a great honor that I have been given the privilege to write the foreword for the book Keratoconus edited by Sujata Das. Dr Das is actively involved in cornea and anterior segment disorders, including keratoconus, corneal infections, and eye banking. She is one of the leaders in the field, who has received numerous awards such as the Developing Country Eye Researcher Award from the Association for Research in Vision and Ophthalmology Foundation and the Achievement Award from the American Academy of Ophthalmology. Keratoconus is a classic disease that has been known for a long time, and it is relatively common among corneal diseases. However, the cause of keratoco- nus has not yet been determined, and the diagnosis and treatment of keratoco- nus have changed with time. In the past, keratoconus was a non-inflammatory corneal thinning disorder and the only way to control its progression was to let it grow naturally, but now keratoconus is considered to be a chronic inflamma- tory disease whose progression can be suppressed. The paradigm shift in kera- toconus care requires eye care specialists to be up to date. The book comprises 23 chapters, with the topics ranging from public health and epidemiology to the newer paradigms in diagnosis and manage- ment. So, undoubtedly this textbook fulfils the needs of practitioners. My hearty congratulations to the authors for taking this great initiative. Department of Ophthalmology Naoyuki Maeda Osaka University Graduate School of Medicine, Suita City, Osaka, Japan vii Preface Keratoconus is an eye problem that affects young individuals. It is associated with significantly impaired vision-related quality of life (VRQoL). Many times, patients with keratoconus have associated allergy, which additionally impacts their daily activities. While early cases can be managed by glasses, there are various options for moderate to advanced disease. Whenever I examine these patients, especially children, I feel the challenges are many despite newer diagnostic techniques and treatment modalities. Coming across the increasing number of patients with keratoconus in my clinical practice drove me to editing a comprehensive book on keratoconus to help cornea specialists and general ophthalmologists to better manage kera- toconus cases. The authors are experts in their field and from various parts of the world. Technology is emerging fast for the diagnosis of keratoconus. Similarly, the armamentarium of its visual rehabilitation procedures has expanded. Collagen crosslinking is a boon for these patients. Research is underway to understand the pathogenesis of the disease and its progression. I hope this book will be a useful guide and a ready reference for ophthalmologists in managing cases of keratoconus. Bhubaneswar, Odisha, India Sujata Das ix Contents 1 Epidemiology of Keratoconus . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1 Smruti Rekha Priyadarshini and Sujata Das 2 Etiology and Risk Factors of Keratoconus . . . . . . . . . . . . . . . . . . 11 Mark Daniell and Srujana Sahebjada 3 Biomechanics of Keratoconus . . . . . . . . . . . . . . . . . . . . . . . . . . . . 23 Kanwal Singh Matharu, Jiaonan Ma, Yan Wang, and Vishal Jhanji 4 Pathophysiology and Histopathology of Keratoconus . . . . . . . . . 31 Somasheila I. Murthy, Dilip K. Mishra, and Varsha M. Rathi 5 Clinical Diagnosis of Keratoconus . . . . . . . . . . . . . . . . . . . . . . . . . 45 Zeba A. Syed, Beeran B. Meghpara, and Christopher J. Rapuano 6 Classifications and Patterns of Keratoconus . . . . . . . . . . . . . . . . 59 M. Vanathi and Navneet Sidhu 7 Differential Diagnosis of Keratoconus . . . . . . . . . . . . . . . . . . . . . 69 Elias Flockerzi, Loay Daas, Haris Sideroudi, and Berthold Seitz 8 Keratoconus in Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 89 Vineet Joshi and Simmy Chaudhary 9 Allergic Eye Disease and Keratoconus . . . . . . . . . . . . . . . . . . . . . 105 Prafulla Kumar Maharana, Sohini Mandal, and Namrata Sharma 10 Topography and Tomography of Keratoconus . . . . . . . . . . . . . . . 117 Shizuka Koh 11 Newer Diagnostic Technology for Diagnosis of Keratoconus . . . 129 Rohit Shetty, Sneha Gupta, Reshma Ranade, and Pooja Khamar 12 Acute Corneal Hydrops: Etiology, Risk Factors, and Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 151 Tanvi Mudgil, Ritu Nagpal, Sahil Goel, and Sayan Basu xi xii Contents 13 Contact Lenses for Keratoconus . . . . . . . . . . . . . . . . . . . . . . . . . . 171 Varsha M. Rathi, Somasheila I. Murthy, Vishwa Sanghavi, Subhajit Chatterjee, and Rubykala Praskasam 14 Corneal Cross-Linking in Keratoconus . . . . . . . . . . . . . . . . . . . . 183 Farhad Hafezi and Mark Hillen 15 Penetrating Keratoplasty in Keratoconus . . . . . . . . . . . . . . . . . . 193 Ankit Anil Harwani and Prema Padmanabhan 16 Lamellar Keratoplasty in Keratoconus . . . . . . . . . . . . . . . . . . . . . 205 Jagadesh C. Reddy, Zarin Modiwala, and Maggie Mathew 17 Intracorneal Ring Segments in Keratoconus . . . . . . . . . . . . . . . . 221 Andreas Katsimpris and George Kymionis 18 Intraocular Lens (IOL) Implantation in Kertaoconus . . . . . . . . 231 Seyed Javad Hashemian 19 Stromal Augmentation Techniques for Keratoconus . . . . . . . . . . 251 Sunita Chaurasia 20 Cataract Surgery in Keratoconus . . . . . . . . . . . . . . . . . . . . . . . . . 257 Wassef Chanbour and Elias Jarade 21 Refractive Surgery in Management of Keratoconus . . . . . . . . . . 267 Jorge L. Alió, Ali Nowrouzi, and Jorge L. Alió del Barrio 22 Artificial Intelligence in the Diagnosis and Management of Keratoconus. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 275 Nicole Hallett, Chris Hodge, Jing Jing You, Yu Guang Wang, and Gerard Sutton 23 Changing Paradigm in the Diagnosis and Management of Keratoconus. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 291 Rashmi Sharad Deshmukh and Pravin K. Vaddavalli About the Editor Sujata Das, MS, FRCS, AMPH, D.Sc.(h.c.) is a faculty member at the L V Prasad Eye Institute (LVPEI), Bhubaneswar, India. She received her postgraduate training in ophthalmology from MKCG Medical College and Hospital, Odisha, India, and FRCS (Glasgow) in ophthalmology. She completed her subspecialty training in cornea and anterior segment from LVPEI, Hyderabad, India. Further, she completed her ICO fellowship in cornea at the University of Erlangen- Nuremberg, Germany, and clinical fellowship in cornea at the Royal Victorian Eye and Ear Hospital and CERA, Melbourne University, Australia. She has pursued an advanced management program for health care from the Indian School of Business, Hyderabad, India, and received a DSc. (honoris causa) from the Ravenshaw University, Odisha, India. She is a recipient of several national and international awards. Dr Das is presently involved in research encompassing corneal infections, eye banking, keratoconus, and genetic analysis of Fuchs’ endo- thelial corneal dystrophy. She has authored over 140 peer-reviewed papers and book chapters to her credit. She has published a book entitled Infections of the Cornea and Conjunctiva. xiii