The ciliopathies are a group of rare diseases that often affect multiple systems within the body, and are caused by defects in the function or structure of cilia. When cilia go wrong, there are profound consequences; these are discussed in detail for the first time in Ciliopathies: a reference for clinicians. The book provides a clinical overview and reference to this newly emergent group of disorders ranging from Alstroem syndrome to putative ciliopathic disorders. Each chapter provides an in-depth discussion on a specific disorder, including the latest scientific research together with a description of its features, and practical guidelines on diagnosis. The authors also examine the evidence for dysfunction of cilia in cancer and more common disorders. Ciliopathies: a reference for clinicians will appeal to those involved in the care of patients with ciliopathies, including specialists in the fields of nephrology, diabetes, cardiology, and ophthalmology, and non-clinical researchers interested in cilia biology.

With a revolution in the surgical management of mild to moderate glaucoma, new procedures allow for the use of microinvasive techniques that avoid disruption of the conjunctiva and formation of a bleb. These approaches range from laser therapies targeting the outflow system of the eye to the implantation of miniature devices in Schlemm's canal or the suprachoroidal space. The emergence of this new branch of glaucoma surgery, often coupled with micro-incisional cataract surgery, requires the practicing surgeon to learn about anatomical nuances to ensure success. MIGS: Advances in Glaucoma Surgery will aid the novice resident as well as the seasoned ophthalmic surgeon facing these new issues in glaucoma treatment. The book addresses preoperative patient selection advice, intraoperative pearls for success, and post operative management skills, which are some of the important aspects of the process towards the adoption of MIGS. Why you will want to read MIGS: Advances in Glaucoma Surgery: * Forward thinking contributions from seasoned MIGS experts who have intimate knowledge of the devices benefits and potential pitfalls in their use * Basic knowledge is provided that will allow each surgeon to tailor their treatment plan while leveling the learning curve * Assists in indentifying patients with a milder form of the disease and higher target pressures who may benefit from the MIGS devices MIGS: Advances in Glaucoma Surgery is the first book of its kind to cover the range of devices in this arena using expert contributors and offering real world pearls for practice that can be used in the operating room. Practicing cataract and glaucoma physicians, residents, and fellows will benefit from this timely resource as the thought process for how to treat glaucoma has advanced.

to the Second Edition here have been significant changes in pediatric Chapter 56 by Maya Eibschitz-Tsimhoni, MD, is a T ophthalmology and strabismus since the first wonderful contribution to the literature, as it reviews edition. Great effort has gone into incorporat 235 important ocular disorders that have systemic ing recent advances into this second edition. Each manifestations, and it includes a detailed glossary of chapter in the book has been revised, and over half of terms. them have been completely rewritten. In addition to As with the first edition, our goal is to present a updating and revising the entire book, we have added comprehensive textbook of pediatric ophthalmology three new chapters: Chapter 7 on electrophysiology and strabismus written in a clear, reader-friendly style. and the eye, Chapter 1 7 on strabismus surgery, and Our hope is that the readerwill find the second edi Chapter 56 on congenital syndromes with ocular man tion of Pediatric Ophthalmology and Strabismus to ifestations. Chapter 17 is the definitive work on pedi be scientifically informative, clinically useful, and en atric ocular electrophysiology, bar none, and was fin joyable to read. ished just weeks before the untimely death of its author, Dr. Tony Kriss (see tribute in Chapter 17).

The macular carotenoids play key roles in eye health and retinal disease. Age-related macular degeneration (AMD), the most common cause of acquired blindness in much of the world, is associated with low levels of macular pigment. Macular pigment is also essential for enhancing visual performance by reducing glare disability and improving photostress recovery. Carotenoids and Retinal Disease presents an up-to-date, thorough volume devoted to the chemistry, pathobiology, visual science, and medical and public health significance of the macular carotenoids. With contributions from an international group of leading experts, this book covers a range of topics, from macular anatomy to clinical trials. It begins with a chapter tracing the discovery of macular pigment through the more recent functional recognition of carotenoids. The text covers AMD risk factors, epidemiology, pathogenesis, and classifications. It reviews evidence from epidemiological studies of relationships between AMD and the carotenoids lutein, zeaxanthin, and meso-zeaxanthin, as well as evidence from clinical trials on the effects of macular carotenoid supplementation in subjects with AMD and normal subjects. The book explores the use of molecular genetics in studying macular pigment and AMD pathogenesis; bioavailability of macular pigment; functions of lutein, zeaxanthin, and meso-zeaxanthin; and the identification of macular carotenoid binding proteins involved in pigment uptake and transport. It also covers xanthophyll-membrane interactions, and the macular carotenoids in human serum and their capacity to protect against AMD. Further, the implications of light distribution on the retina for AMD are discussed. Advancing our understanding of how the macular carotenoids enhance vision and prevent vision loss, this book provides a valuable reference for researchers and clinicians involved in the treatment and prevention of retinal disease.

This timely publication fills a large gap in the ophthalmic literature which has so far lacked a monograph on the clinically very important subject of macular edema. The book presents the most up-to-date scientific concepts concerning the etiology and pathogenesis of blood retinal barrier breakdown such as tight junction associated protein dysfunction, and changes in fluid transport properties of the retinal pigment epithelium. The bulk of the book is clinically oriented and addresses novel imaging and diagnostic techniques for the detection of macular edema as well as the clinical context of a panoply of ocular diseases which induce macular edema, such as diabetes, other vasculopathies, uveitis, and many others. New light is shed on the association between highly active antiretroviral therapy and the induction of macular edema in HIV-positive patients. Novel drug treatment regimens with steroids and carbonic anhydrase inhibitors as well as new ways of applying laser and surgical therapies are also discussed in detail, and practical treatment guidelines are given. This book will be helpful for vitreoretinal specialists as well as for the practising ophthalmologist confronted with patients suffering from macular edema.

"To explain all nature is too difficult a task for anyone man or even for anyone age. Tis much better to do a little with certainty, and leave the rest for others that come after you, than to explain all things ... " Sir Isaac Newton (1642-1727) This book describes and discusses some new aspects of col or vision in primates which have emerged from a series of experiments conducted over the past 8 years both on single ganglion cells in monkey retina and on the visually evoked cortical potential in man: corresponding psychophysical mechanisms of human perception will be considered as well. An attempt will be made to better understand the basic mechanisms of color vision using a more comprehensive approach which takes into account new mechanisms found in single cells and relates them to those found valid for the entire visual system. The processing of color signals was followed up from the retina to the visual cortex and to the percepq.tal centers, as far as the available techniques permitted."

Held in Amsterdam, The Netherlands, September 3-4, 1981

Silicone oil was introduced into ophthalmic surgery by Paul Cibis in the early 1960's in an attempt to treat giant retinal tears and cases where retinal detachment had failed to respond to conventional scleral buckling tech niques. His understanding of the nature of vitreous pathology and how it related to complex retinal detachment laid the foundation for modern vit reoretinal surgery. Cibis's success with these problems together with his rationale for sepa rating membranes from the retina led others to try their hand at learning his techniques. At the same time much criticism was levelled at the concept of injecting what was regarded as a foreign material into the eye, as well as to the whole idea of operating within the vitreous cavity. Some of this was personal, some due to a lack of understanding of the underlying pathology and some due to disappointment following surgical failure us ing the new methods. Early results were certainly encouraging but it was not until the advent of pars plana vitrectomy that it became possible to develop more refined methods in combination with the use silicone oil so that much better results could be obtained.

Posterior Capsular Rupture: A Practical Guide to Prevention and Management addresses a nightmare of every cataract surgeon: a posterior capsular rupture. The book details each aspect of the process, from prevention of a posterior capsular rupture to management and IOL placement. Dr. Amar Agarwal and his contributors, leading international ophthalmic surgeons, discuss the latest techniques in easy-to-understand text and format and include more than 250 high-quality photographs that enhance reader comprehension. Posterior Capsular Rupture: A Practical Guide to Prevention and Management presents a variety of complicated cases and complications such as posterior polar cataracts, IOL scaffold & glued IOL, sutured IOLs, management of dislocated IOLs, and corneal damage & posterior capsule rupture, to name a few. & Additionally, a video website accompanies Posterior Capsular Rupture: A Practical Guide to Prevention and Management and allows readers to gain a better understanding of the subject. As a bonus, readers will be able to submit questions and receive clinical answers from Dr. Agarwal through the video website. Practising ophthalmologists, residents, and post-graduate students alike will benefit from this book's clear portrayal of this difficult medical issue.

Proceedings of the 5th Biannual International Meeting on Angiogenesis: From the Molecular to Integrative Pharmacology, held July 1-7, 1999, in Crete, Greece. Angiogenesis, as a vastly complex biological process, has challenged researchers from all basic scientific disciplines, including pharmacology, biochemistry, physiology, embryology and anatomy. The significance of this phenomenon for the study of disease states has also interested clinicians from a number of specialist fields. This multidisciplinary work reflects the growth of awareness of concepts such as angiogenesis based therapy, the enormous therapeutic and commercial potential of which has attracted major research and investment in recent years. This volume, which aims to bridge the gap between basic and clinical methodology and understanding, presents the most up-to-date developments in this field.

This Volume of the Documenta Ophthalmoligica Proceeding Series presents the scientific papers read during the 13th Congress of SIDUO, the International Society for Ultrasonic Diagnosis in Ophthalmology, held in Vienna in the Summer of 1990. It was the second time that SIDUO had selected Vienna as a site for its biennial congresses in the 28 years of its existence. Previously, the 3rd SIDUO Meeting had taken place as part of the 1st World Congress for Ultrasonic Diagnosis in Medicine organized by Karl C. Ossoinig in 1969. Ossoinig, the pioneer of Standardized Echography opened the scientific sessions of SIDUO 13 with the First Jules Fran~ois Memorial Lecture on the Optic Nerve reviewing the modern examination techniques for precise measurements of the orbital optic nerve and its various sheaths and for an accurate diagnosis and differential diagnosis of diseases affecting the optic nerve, highlighting the new field of prophylactic recognition of early optic nerve compression (e. g. , in Graves' orbitopathy) and the important confirmation of CON in the presence of other conditions affecting the optic nerve functions. The first scientific session then dealt with a variety of interesting conditions of the optic nerve and of the extraocular muscles ranging from inflammatory diseases such as optic neuritis and orbital myositis to congenital anomalies, from glaucomatous changes to IIH, and from normal anatomical (dynamic) findings to malignant tumors such as lymphomas and leucemic meningeosis.

The Sclera is the definitive text on the diagnosis and treatment of scleral diseases, with in-depth chapters on non-infectious scleritis, infectious sclertitis, non-inflammatory diseases of the sclera, and systemic implications of scleral disease. Basic physiology, anatomy, and biochemistry are addressed as well. Written for the ophthalmologist, the revised and updated new edition of The Sclera contains the most current information available on newer characterizations of the composition of the sclera, immune derangements of it, and success in medical and surgical treatment of the inflammatory diseases of the sclera.

We in ophthalmology are fortunate to be able to measure quantitatively many aspects of ocular morphology and visual function. These measurements are either objective (e. g. electroretinography, tonometry, electromyography, visually evoked responses, etc.) or subjective and psychophysical (e. g. visual acuity, visual fields, color vision, etc.). One aspect of corneal physiology and pathology which has so far escaped careful and reliable measurements is corneal sensitivity. Previous attempts have been rather crude and can be compared to measuring intraocular pressure by digital compression. Professor Draeger has for the last decade and a half directed his attention to the question of esthesiometry of the cornea. He has a gift for constructing and devising new ingenious ocular instruments. His handheld tonometer is a splendid example. He has now applied the principle of this instrument to the new electronic optical esthesiometer. In many publications he has reported on his first results using this modern technique to study corneal physiology and evaluate certain pathologic conditions. In this monograph Professor Draeger has collected all of his material and reports on his long experience. The usefulness of this instrument is just beginning to be appreciated. It is obvious that this new measuring device will help us in the fitting of contact lenses, evaluating herpes simplex infections of the cornea, follow the postoperative course of patients with a penetrating keratoplasty, etc.

This volume of Documenta Ophthalmologica Proceedings Series collects the scientific papers presented at the 2nd International Symposium on Retinal Pigment Epithelium and the 4th Meeting of the European Macula Group held in Genoa, May 29-June 1, 1996. The Symposium on Retinal Pigment Epithelium was promoted by the University Eye Clinic of Genoa as the natural continuation of the first Symposium held with great success in Genoa in 1988. The previous Meetings of the European Macula Group were held in Coimbra (1988), Crete (1989) and Athens (1994). I was greatly pleased and honoured to host the fourth congress of this distinguished Society and I am grateful to Gabriel Coscas, Jose Cunha-Vaz and George Theodossiadis, found ers of the Society, for selecting Genoa on this occasion. The two meetings integrated well in an unicuum and brought together an exceptional number of outstanding retinal specialists coming from all over the world. All the aspects of the current research concerning retinal pigment epithelial and macular diseases were covered. Several interesting presentations regarded new techniques of retinal and choroidal imaging. A full session was dedicated to the latest advances in culture and transplantation of retinal pigment epithelial cells. Age-related macular degeneration was a major subject for discussion, including new approaches to treatment. This topic was high lighted by a mini-symposium on drusen, including a series of superb lectures on classification, clinicopathological studies, indocyanine green imaging, and laser treatment for prevention of choroidal neovascularization.

Roots of the theory and practice of ocular pharmacology may be traced to the ancient Mesopotamian code of Hammurabi and then to several papyri reflecting the clinical interests of the Egyptians. The evolution of its art and science was irregularly paced until the nineteenth century when Kohler, in 1884, proved the anesthetic effect of cocaine on the cornea, and when Fraser, Laquer, Schmiedeberg, Meyer, and others studied the pharmacology of the autonomic nervous system by way of observations of the pupil. Advances in the past few decades have been nothing short of explosive. How can the student, physician, or basic research scientist stay in touch with these electrifying studies? To help with the answer to this question, the authors set as their goal the development of increased understanding so that the student, research scientist, and ophthalmologist can cope with the latest discoveries. The authors want to narrow what appears to be an ever-increasing gap between basic science and ophthalmology. The basic aspects of pharmacology have been presented in light of the natural physiology. In this regard, while distinctions among endogenous mechanisms, drug effects, and the pathogenesis of disease are to be separately recognized, appreciation must be given to the concept that both the desirable and unwanted manifestations or functions caused by either disease or drugs must very often represent a quantitative change in normal metabolic pathways.

Glaucoma simplex or open angle glaucoma is a slow, progressive illness with an insidious course which can lead to blindness. According to our con temporary state of knowledge, the illness begins with a decreased outflow of aqueous humor. This leads, sometimes after a period of decreased aqueous humor production due to a feedback mechanism, to an increase in intra ocular pressure (lOP). An elevated lOP can eventually lead to optic nerve damage, which manifests itself morphologically as glaucomatous papillary excavation and functionally in the development of a visual field defect. The classic triad of elevated lOP, papillary excavation and visual field defect, on which the diagnosis of glaucoma was also based in earlier times, is then pre sent. To this can now only be added that tonography usually shows a de crease in aqueous humor outflow and that gonioscopy must reveal an open angle. On the basis of statistical findings, Goldmann has calculated that there may be an interval of as long as 18 years between the initial increase in lOP and the development of visual field defects. This point of view (which has been confirmed in practice) that the onset of the illness can be demon strated by measurement of the lOP has introduced a new element into the discussion around glaucoma, namely, the concept of ocular hypertension. This implies simply that there is an (statistically) elevated intraocular pres sure, even though the optic nerve may not (yet) be damaged.

The phenomenon known as Leao's spreading depression is investigated in this collection of ten original articles. New ideas about the fundamental mechanisms, which focus on the involvement of polyunsaturated fatty acids and free radicals, are presented. The relationship of spreading depression with clinical entities such as migraine, epilepsy, ischemic and protein-deficiency conditions of the nervous tissue are discussed as well. Studies dealing with the application of non-invasive techniques to monitor the disease are included.

Pediatric Oculoplastic Surgery is the definitive text for ocular disorders and how they apply specifically to children. Addressing everything from genetic considerations, embryology and anatomy, anesthesia, dermatologic considerations, benign and malignant tumors, fetal wound healing, establishing propoer diagnosis, timing for surgical interventions and surgical procedures. This volume is an essential addition to the library of every pediatric ophthalmologist and reconstructive surgeon. The distinguished contributors have combined their surgical knowledge and experience with over 1,100 color photographs and custom illustrations to present comprehensive and readily usable information on the identification and treatment of a wide range of ocular disorders.

Contents Introduction .......................................... . 1 The Primate Eye ...................................... . 2 Embryology of Retina and Choroid ....................... . 4 Microscopic Anatomy .................................. . 4 Retina ............................................ . 4 Choroid ........................................... . 8 Material and Methods .................................. . 10 Fine Structure of the Retina 14 RetinaI Pigment Epithelium ............................. . 16 Photoreceptor Cells ................................... . 30 Outer Plexiform Layer and Horizontal Cells .................. . 64 Bipolar, Radial Clial, and Amacrine Cells .................... . 76 Canglion Cells and InternaI Limiting Membrane ............... . 98 Spatial Density of RetinaI Cells .......................... . 112 Fine Structure of the Choroid ........................... . 116 Choroidocapillaris and Its Fiber System ..................... . 118 Arteries, Veins, and Lymphatic Spaces ...................... . 134 Choroidal Nerves .................................... . 142 Cells of Choroidal Connective Tissue ....................... . 148 References ........................................... . 153 Index ................................................ . 157 vii This volume describes the morphology of the primate re tina as seen with the electron microscope. As it is an atlas, the electron micrographs are its most In trad lietian important part. The text accompanies the figures, highlighting selected topics either to explain structures or to point out structure-function relation ships. A scholarly review of the whole spectrum of research on the re tina and choroid is not feasible in a single volume. Thus, whenever available, review artides or monographs, rather than original work, are cited for reference."

The eye has fascinated scientists from the earliest days of biological in vestigation. The diversity of its parts and the precision of their interaction make it a favorite model system for a variety of developmental studies. The eye is a particularly valuable experimental system not only because its tissues provide examples of fundamental processes, but also because it is a prominent and easily accessible structure at very early embryonic ages. In order to provide an open forum for investigators working on all aspects of ocular development, a series of symposia on ocular and visual devel opment was initiated in 1973. A major objective of the symposia has been to foster communication between the basic research worker and the clinical community. It is our feeling that much can be learned on both sides from this interaction. The idea for an informal meeting allowing maximum ex change of ideas originated with Dr. Leon Candeub, who supplied the nec essary driving force that made the series a reality. Each symposium has concentrated on a different aspect of ocular development. Speakers have been selected to approach related topics from different perspectives."

In its long series of annual reports, the Belgian Society of Ophthal- mology has a tradition of bringing into focus the recent advances in ophthalmology. But it seems surprising that one has to go back to 1940 to find a major report on the corneal diseases, when R. Rub- brecht wrote an "Aperr;u de la pathologie et de la therapeutique generale des maladies de la cornee". However, this omission is only apparent. In fact, many reports, since then, have contributed to our knowledge of the advances in corneal research. The corneal dimensions were documented in "La biometrie ocu- laire clinique (Y. Delmarcelle et aI., 1976)", the fine structure of the cornea in "L'ultrastructure de tissues oculaires (L. Missotten, 1964)", and many aspects of its metabolism in "Les verres de contact (P. Cochet et ai, 1967)" and "Les complications oculaires des erreurs congenitales du metabolism (J.P. Groux et O. Kallay, 1971)" . Bacterial keratitis was a main topic in "La therapeutique par les antibiotiques autres que la Penicillamine en Ophtalmologie (1. Michiels, 1952). Fungal corneal infections were described in detail in "Les mycoses oculaires" (J . Fran90is et M. Elewaut-Rysselaere, 1968)" and the toxic effects of drugs were dealt with in "Les effets nocifs des medications generales sur I'appareil visuel (J. Michiels et coil. 1972)".

The 14th Congress of SIDUO, held in Tokyo from October 26 to October 30, 1992, was the first congress meeting to be held in Asia in the 30-year history of SIDUO. The congress was organized by the Department of Oph- thalmology, St. Marianna University School of Medicine, with the support of the Japanese Ophthalmological Society, the Japan Society of Ultrasonics in Medicine and the Japan Society of Ophthalmologists. The organizing committee consisted of the following members. Congress President: Sadanao Tane, M. D. (Professor and Chairman, St. Marianna University School of Medicine) Vice-presidents: Atsushi Sawada, M. D. (Professor and Chairman, Miyazaki Medical College) Masayasu Ito, Ph. D. (Tokyo University of Agriculture and Technology) Secretary General: Yasuo Sugata, M. D. (Tokyo Metropolitan Komagome Hospital) Finance Committee: Koji Ohashi, M. D. (Assistant Professor, St. Marianna University School of Medicine) Akira Komatsu, M. D. (Assistant Professor, St. Marianna University School of Medicine) Toshio Kaneko, M. D. (Assistant Professor, St. Marianna University School of Medicine) Publicity and Exhibition Committee: Hideyuki Hayashi, M. D. (Assistant Professor, School of Medicine, Fukuoka University) Akihiro Kaneko, M. D. (National Cancer Center) The Honorary Presidents were Yukio Yamamoto, M. D. (Tokyo Tama Geriatrics Hospital) and Yasuo Uemura, M. D. (Professor Emeritus, Keio University) . The opening ceremony began with the Francois Memorial Lecture given by Professor Peter Till (Standardized Echography: Quantitative Analysis of XlI Tissue Backscatter - A Major Source of Information for Tissue Diagnoses).

Noninvasive Diagnostic Techniques in Ophthalmology explores the special noninvasive tools developed to function as diagnostic indicators and to further our understanding of ocular function. The volume's focus is on new development in instrumentation and techniques for studying the cornea, lens, retina, vitreous, and aqueous dynamics; whereby special attention is given to how each technique has improved our understanding of basic processes and diagnostic capability. Theoretical aspects, possible sources of error, current problems and limitations, safety evaluation, and future applications and directions are considered. Topics examined include ophthalmic image processing; magnetic resonance imaging of the eye and orbit; diagnostic ocular ultrasound; corneal topography; holographic contour analysis of the cornea; wide field and color specular microscopy; use of the Fourier transform method for statistical evaluation of corneal endothelial morphology; confocal microscopy; in vivo corneal redox fluorometry; evaluation of cataract function with the Scheimp-flug camera; fluorescence and Raman spectroscopy of the crystallin lens; in vivo uses of quasi-elastic light scattering; fundus reflectometry; and clinical visual psychophysics measurements. The book offers discussions of fractal analysis of human retinal blood vessel patterns, scanning laser tomography of the living human eye, fundus imaging and diagnostic screening for public health, and digital image processing for ophthalmology, as well as a detailed appendix comprising additional topics and sources.

The region of the skull base was long considered a surgical barrier because of its complex anatomy. With few exceptions, the region immediately beyond the dura or bony skull base constituted a "no man's land" for the surgeon working from the other direction. A major reason for this was the high morbidity associated with operative procedures in that area using traditional dissection techniques. This situation changed with the advent of the operating microscope. Used initially by ear, nose and throat specialists for resective and reconstructive surgery of the petrous bone and parana sal sinuses, the operating microscope was later introduced in other areas, and neurosurgeons began using it in the mid-1960s. With technical equality thus established, the groundwork was laid for taking a new, systematic, and interdisciplinary approach to surgical problems of the skull base. Intensive and systematic cooperation between ear, nose and throat surgeons and neurologic surgeons had its origins in the departments of the University of Mainz bindly supported by our chairmen Prof. Dr. Dr. hc Kurt Schiirmann (Department of Neurosurgery) and Prof. Dr. W. Kley (Depart ment of Ear, Nose and Throat Diseases, Head and Neck Surgery). The experience gained from this cooperation was taught in workshops held in Hannover from 1979 to 1986, acquiring a broader interdisciplinary base through the participation of specialists from the fields of anatomy, patholo gy, neuroradiology, ophthalmology, and maxillofacial surgery.

This monographic work authored by eminent neurophysiologists will be of major interest to researchers investigating the visual system or working in behavioral neuroscience and sleep research. The book deals with the neuronal circuits of the visual thalamocortical system, the brainstem and basal forebrain modulatory systems and their neurotransmitters acting upon these circuits, and the neuronal activities in the visual thalamocortical system as changed during shifts in behavioral states of vigilance from wake to sleep. Data discussed consist of recent studies on light and electron microscopy, extra- and intracellular recordings of thalamic and cortical neurons, neurotransmitter actions, and state-dependent cellular activities in the visual system.