Ординатура / Офтальмология / Английские материалы / The Glaucomas Volume 1 Pediatric Glaucomas_Sampaolesi, Zarate_2009

Roberto Sampaolesi, MD

Emeritus Professor

UBA (Universidad de Buenos Aires) Faculty of Medicine

Department of Ophthalmology and

Emeritus Professor

UCES ( Universidad de Ciencias Empresariales y Sociales) Honorary Professor of the Universidad del Salvador Member of the Roman Academy of Medicine

Parana 1239 1 A 1018 Buenos Aires Argentina

ISBN 978-3-540-69144-0 e-ISBN 978-3-540-69146-4 DOI 10.1007/978-3-540-69146-4

Library of Congress Control Number: 2008941263

© 2009 Springer-Verlag Berlin Heidelberg

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Juan Roberto Sampaolesi, MD

Professor

UCES (Universidad de Ciencias Empresariales y Sociales) Faculty of Medicine Department of Ophthalmology Parana 1239 1er Piso

1018 Buenos Aires Argentina

Jorge Zárate, MD

Professor

UBA (Universidad de Buenos Aires) Faculty of Medicine

Department of Ophthalmology Libertad 679

1770 Aldo Bonzi Buenos Aires Argentina

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This book is dedicated to my wife Erica. For the past 40 years she has attended all the anesthesias on children with congenital glaucoma, helped us during the examinations, and above all has supported the parents of the children with congenital glaucoma, whose experience is also difficult and emotional, requiring special care, as she has done with great tenderness and compassion. She also recorded each case in an index, which, in writing this book, was invaluable to consult the anatomical and functional results 12–40 years after the operations.

I also dedicate this book to my mother Angelita Bouzon Sampaolesi and to my father Dr. Juan Sampaolesi, from whom I learned the values of life and the ethics of our profession, which inspired me to write this book, and to my beloved children Anneliese, Juan Roberto, Mario, and Mariana. And also to my dear grandchildren, Lucas, Franco, Marina, Maximo, Camila and Santo, whose presence has brought joy to the last years of my life.

Within the practice of glaucoma, indeed within the full spectrum of ophthalmic practice, there is no aspect more challenging than caring for the child with glaucoma. The childhood forms of glaucoma present unique challenges at every level of management. The examination to establish the diagnosis and monitor the progression of the disorder requires special skills. Medications are rarely effective or indicated, and surgical outcomes are often disappointing. Even when surgery is successful in lowering intraocular pressure, vision may be lost unless meticulous attention is paid to amblyopia therapy. And, on top of all these medical and surgical challenges, there is an added emotional burden. The stakes are especially high in these young patients, since failure could mean a life-time of visual impairment. The physician must also deal with anxious young parents, whose dreams of a healthy child have just been dashed, and who now look to their doctor to give their child a normal life.

For 57 years, Prof. Roberto Sampaolesi has faced these challenges. He has operated on over 800 children with glaucoma and has followed some for over 40 years. With exceptional skill and compassion, he has watched his fortunate, young patients achieve social integration and lead healthy, normal lives. Now we are the fortunate ones in having Dr. Sampaolesi share with us, in this volume, his vast knowledge and life-time of experience with the pediatric glaucomas.

As we know, children are not small adults. With regard to their eyes, they are unique both in the normal anatomical state and in many disease processes. In Chap. 12, Dr. Sampaolesi explains the appearance of the normal anterior ocular segment in newborns and children. A solid understanding of this normal anatomy is a prerequisite to recognizing disease states, and Dr. Sampaolesi’s clear text and vivid illustrations provide the reader with an excellent starting point for the study of childhood glaucomas. He describes the gonioscopic appearance of the chamber angle in premature babies and newborns, as well as the biomicroscopic appearance of the iris, noting the variations of normal that can create confusion in distinguishing pathologic conditions. Finally, he provides helpful hints at the end

of the chapter for performing these diagnostic procedures in children.

Having acquired an understanding of the normal anatomy, the physician must now become familiar with the range of pathologic findings in the child with glaucoma. In Chap. 13, Dr. Sampaolesi reviews the pathologic chamber angle in congenital glaucoma and its implications in indications for surgery. He describes the two basic types of pathologic chamber angles in children, which he clearly illustrates with schematic, gonioscopic, and histologic pictures. If the reader thinks that a person who has been practicing for 57 years might not be up on the latest technology, we have only to look further in this chapter and find a detailed treatise on the application of the slit lamp optical coherence tomograph in the diagnosis and follow-up of children with congenital glaucoma. Dr. Sampaolesi describes the use of this exciting new technology and provides many excellent illustrations.

One of the advantages of a life-time of experience in the practice of medicine is the individual stories that can be told, which provide guidance and encouragement for young physicians who are embarking on their careers. In Chap. 17, Dr. Sampaolesi presents several clinical cases from his years of practice. These cases highlight the difficulty of recognizing glaucoma in children, many of whom came to him after initial incorrect diagnoses. In addition, they illustrate, once again, his use of the latest technology, including confocal laser tomography and frequency-doubling technology, for those young people who are old enough for these studies. He also counsels ophthalmologists not to become discouraged by failure in surgeries and severe complications, noting that perseverance will often lead to a successful outcome even in situations that may seem hopeless. In support of this, he tells of his patients who have gone on to become successful athletes and dancers or simply able to lead normal lives.

Dr. Sampaolesi reminds us that the pediatric glaucomas are not limited to newborns and young children, but can appear at any age of childhood and even into young adulthood. In Chap. 20, he describes goniodysgenesis or late congenital glaucoma, as well as a form of

pigmentary glaucoma. Once again, he turns to years of experience in describing the clinical findings, which he augments with illustrations from his practice and those of colleagues, as well as additional clinical cases.

What I believe is most valuable about this book is that it is almost like having a personal conversation with one of the giants of our profession. Dr. Sampaolesi shares with us his opinions and those of his respected colleagues. He also shares his experiences, not only of his successes, but of his heartaches and frustrations with difficult cases, and ultimately of his joy in seeing

his young patients achieve success in their lives. This book then is not only a guide to the technical aspects of treating children with glaucoma, but it is also a guide as to how we, as physicians, should approach the challenges of our profession, with humility, compassion, and determination. On behalf of all the physicians and their patients and families who will profit from this guidance, I sincerely thank Dr. Sampaolesi for sharing it with us.

M. Bruce Shields

The 1994 edition of Roberto Sampaolesi’s book on glaucoma was a handbook for glaucomatologists with knowledge of Spanish. With this English edition, this long hidden treasure becomes available to all Englishspeaking ophthalmologists. The extensively updated edition is the result of a lifelong work dedicated to ophthalmology and glaucoma by an exceptionally talented and efficient person, tireless in patient care, research, and teaching, always ready to reach out for new concepts and to turn ideas into action. The book is extraordinary in many regards. It is old-fashioned while being at the height of modernity: old-fashioned, because it is a book summarizing 60 years of experience in every field of glaucoma; modern because it is at the forefront in evaluating and using new technologies and new therapeutics; old-fashioned, because it quotes publications in many languages as far back as the times of von Graefe; modern because it makes use of new research techniques and databases such as Medline and others.

That this book could reach this level of excellence has its roots in the way Roberto Sampaolesi has accumulated knowledge and skills since his youth. After finishing medical school in 1951, he acquired a background in basic sciences – physics, chemistry, anatomy, histology and especially in physiology – spending years with Bernardo Hussay, the Nobel laureate of 1947. Then he trained in ophthalmology in a way that today has become impossible, becoming a fellow for varying periods of time with some of the best-known ophthalmologists of the time: H.K. Müller and G. MeyerSchwikerath in Bonn, W. Leydhecker in Würzburg, C. Cüppers in Giessen, for surgery with Leornardi and Bietti in Rome, Paufique in Lyon, and Schepens in Boston, not to mention that this training period began under Marc Amsler in Zurich in 1955, as documented in “Remembrances of Things Past” in Survey of Ophthalmology [1]. He became the most prominent fellow that the Eye Department of Zurich had ever had and also one of its best friends. When later he became Professor of Ophthalmology and department head in Buenos Aires, he continued broadening his network of knowledge sources, localizing with a particularly sharp instinct the new ideas and techniques of younger

and older colleagues throughout the world. With this background, he documented with painstaking accuracy what he observed in more than 8000 glaucoma patients.

A few highlights in his skills may be pointed out: The first is Sampaolesis’s experience with pediat-

ric glaucomas. He is an excellent teacher in care for newborns and infants in daily practice and measuring intraocular pressure without anesthesia in infants. In 1969, he taught us that normal newborns, infants, and children have much lower intraocular pressure than what had been assumed until that time and that this had to be considered in glaucoma control in infants. In 1973, he showed that the most reliable tool to check glaucoma in newborns and infants was the measurement of the length of the globe using echometry as long as intraocular pressure had to be measured under general anesthesia with all its sources for errors. He has been teaching since 1972 that trabeculotomy combined with -ectomy was the surgical procedure of choice in refractory congenital glaucomas.

The second is gonioscopy. Highlighted by his drawings, he stresses the undeniable importance of gonioscopy for glaucoma classification. He demonstrates how to differentiate normal findings and true dysgenetic changes in the developing angle. Gonioscopic findings are complemented by histology and electron microscopy of trabeculectomy specimens in an exemplary way.

The third regards the role of intraocular pressure and its level in open-angle glaucoma. Since his beginnings in ophthalmology, Roberto Sampaolesi has puzzled over so-called normal or low-pressure glaucoma. In 1961, in his publication on 24-h pressure curves – pressures taken when most ophthalmologists are still sleeping! – he had unveiled pressure peaks in most of these normal and low tension glaucomas. His growing experience let him withstand the wave that came around every 20 years attempting to downgrade intraocular pressure to a simple risk factor among others. The evaluation of the follow-up of approximately 7000 glaucoma patients 47 years later confirmed his 1961 findings and the recent placebo-controlled prospective multicenter studies prove what may have been

considered a hypothesis; namely that for the single eye, overly high intraocular pressure is the main cause of glaucomatous damage.

Springer Verlag deserves thanks for publishing a book that will become a landmark of both past and future knowledge. The book may become an excellent companion and a source of knowledge for every ophthalmologist caring for glaucoma patients for many years.

Reference

1.Jay B, Sampaolesi R (1996) Rembrance of things past. Surv Ophthalmol 40:400–404

GLAUCOMA was Roberto Sampaolesi’s first book dealing with all aspects of the most important and difficult disease of ophthalmology. In 1974 this book in Spanish language (2nd edition 1994) with its 904 pages was the most complete description of all aspects of glaucoma.

Having dealt with the problem of congenital glaucoma for a great deal of his life, Roberto Sampaolesi was one of the very few ophthalmologists to not only describe all aspects of its diagnosis, but also show the results of the greatest number of surgically treated patients. He was the one who finally demonstrated the enormous importance of the “Curva diaria”, which had already been mentioned by Hans Goldmann and Wolfgang Leydhecker. Roberto Sampaolesi evaluated the importance of this symptom for very early diagnosis and the precise follow up of glaucomas. This and many other aspects have already been described in the two books, which so far comprise the most complete description of this disease.

In the Spanish speaking world these classics on glaucoma are recognized as standard text books on the topic. Those of us not quite fluent in Spanish must be grateful that these texts have now been translated into English. Furthermore, the present edition gives the most complete overview of this topic – evaluating the world literature published 15 years after the last book and also summarizing the author’s own wide experience.

Looking at this collection of a life devoted to studies of glaucomas by one of the great masters of ophthalmology of our time, I think that it confirms the

statement by my/our great-grandfather in ophthalmology, Theodor Axenfeldt, who summarized his wisdom in 1929 at the 13th International Congress of Ophthalmology in Amsterdam: “Science and the art of medicine can develop their highest bloom only if all people collaborate for the great tree of life. The various branches alternate in producing flowers and fruits within the family of people. The spirit shows up here and there unpredictably. Everybody of us is responsible that he includes everything for the care of his patient regardless where it originates. So the joint cooperation and effort from all of us is essential and an indispensable duty.”

Roberto Sampaolesi, his coauthors, and the publisher deserve praise for making the publication of this summa of a lifetime of work in ophthalmology and particularly in glaucomas possible.

May this study remind us that ophthalmological originality and creativity does not only arise in the English-speaking world, although we realize that English today has become the lingua franca of science everywhere.

MD, ML. FRC Ophth (hon.)

Immediate Past President International Council

of Ophthalmology (1998–2006)