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Textbook Retinal and Choroidal Imaging in Systemic Diseases 1St Edition Jay Chhablani Ebook All Chapter PDF
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Retinal and
Choroidal Imaging in
Systemic Diseases
Jay Chhablani
Parthopratim Dutta Majumder
J. Fernando Arevalo
Editors
123
Retinal and Choroidal Imaging
in Systemic Diseases
Jay Chhablani
Parthopratim Dutta Majumder
J. Fernando Arevalo
Editors
J. Fernando Arevalo
The Johns Hopkins University
Baltimore, MD
USA
Who needs another book? More pictures? New technology? Is there anything else
to learn?
The answers to these questions are very simple. Working backwards, one appre-
ciates the fact that learning never stops. It is our objective as physicians to continu-
ally learn so as to better ourselves and therefore improve the care we provide to our
patients. While there are many ways of learning, someone must always be a student
and someone must always be the teacher. Is there new technology? Of course there
is and it is our obligation as students of ophthalmology to learn that technology,
relying on good teachers to help us understand that which is new and different. We
are fortunate in that we are witness to incredible advances in technology in our life-
times, but we must be committed to not letting the technology get away from us.
Therefore we look to proven methods of teaching, provided by good teachers and
educators, to enlighten us and keep us up to date on the newest technologies in our
field. And who is it that said that a picture is worth a thousand words? As we know
that most of us learn from pictures and a well-illustrated document, with concise
and well-organized writing, to explain those illustrations which helps enormously in
the learning process. Pattern recognition is key to our profession and thus imaging
is key to what we do every day. And finally one must ask, do we need another book?
And the answer is unquestionably, yes. And the reason for that is simple. The best
way to keep up with new technology is through a well-illustrated text that serves as
a reference and provides a well-organized, meticulously scripted, heavily refer-
enced document. The textbook does exactly that.
We, the students, are fortunate in that doctors Jay Chhablani, Parthopratim
Majumdar, and J. Fernando Arevalo are superb teachers who have assembled a list
of experts to address a variety of systemic conditions. By using various imaging
modalities they are able to illustrate and better define these conditions. By focusing
these modern imaging technologies on selected systemic conditions, we have an
unusual format for the learning process. Whether we are ophthalmologists who spe-
cialize in retinal diseases, general ophthalmologists, uveitis specialists, ocular
oncologists, internists, or family physicians interested in understanding the lan-
guage of imaging technology in ophthalmology, these teachers instruct us as never
before. Beautifully illustrated and beautifully written, this textbook will undoubt-
edly become a mainstay of the practicing physician as well as medical students and
residents alike. This text will become a first resource for so many individuals trying
v
vi Foreword
to understand the complexity of the many systemic diseases which are demonstrated
through ophthalmic imaging.
We must compliment and acknowledge the editors of this extremely important
text for their efforts and their successful accomplishments in bringing to the reader-
ship this very valuable text.
Alexander J. Brucker
Scheie Eye Institute
University of Pennsylvania
Philadelphia, PA
USA
Preface
Various systemic diseases involve the eyes, and in few diseases, the eyes could pro-
vide the first clue for the systemic disease. Retinal findings on clinical examination
have been reported previously in systemic diseases. Advancement of posterior seg-
ment imaging has significantly improved the understanding of pathophysiology and
has become an essential part in management strategy of posterior segment diseases.
Advanced imaging techniques such as enhanced depth imaging, oximetry, adaptive
optics, and retinal blood flowmetry are now being explored in subjects with sys-
temic diseases. With improved understanding on such imaging techniques in vari-
ous systemic diseases, the knowledge about pathomechanism, early diagnosis, and
more targeted therapeutic approaches has improved.
In this book, we focus on findings with various imaging modalities in various
systemic conditions. Systemic conditions included, but are not limited to, neuro-
logical diseases such as Alzheimer’s disease, multiple sclerosis, Parkinson’s dis-
ease, schizophrenia, and migraine; systemic vasculitis such as Behçet’s disease;
systemic lupus erythematosus; ocular toxicity secondary to systemic drugs;
blood dyscrasias such as sickle cell disease and hematologic disorders; renal
diseases; trauma-related conditions such as Purtscher-like retinopathy, whiplash
retinopathy, and shaken-baby syndrome; intracranial hypertension; cancer-asso-
ciated retinopathy; gastrointestinal diseases; immunologic diseases such as auto-
immune retinopathy and sarcoidosis; systemic infectious diseases such as
tuberculosis and choroidal and retinal metastasis; oculoneurocutaneous syn-
dromes; Vogt-Koyanagi-Harada disease; pregnancy; systemic hypertension; and
Valsalva retinopathy.
This book, entitled Retina and Choroidal Imaging in Systemic Diseases, is the
first attempt to provide information on retinal and choroidal findings using advanced
imaging technologies in systemic diseases. This book is intended for ophthalmolo-
gists, retina specialists, uveitis specialists, ocular oncologists, and internal medicine
specialists.
We wish that this book improves the current understanding about the imaging
findings in systemic diseases which helps for early diagnosis and management of
these conditions.
vii
viii Preface
We would like to thank all the authors who shared their experience and valuable
time and effort for the book. We would like to thank the Springer staff, who guided
us to make this book happen. At the end, we would like to thank our patients, col-
leagues, and families who supported us to bring this book in a timely manner.
1 Neurological Diseases������������������������������������������������������������������������������������ 1
Uppal Gandhi, Preeti Patil Chhablani, Akshay G. Nair,
and Jay Chhablani
2 Retinal Vasculitis in Systemic Disease�������������������������������������������������������� 15
Irfan Khan and Ashvini Reddy
3 Multimodal Imaging in Drug-Related Retinal Toxicity �������������������������� 29
Remya Mareen Paulose, Jay Chhablani, and William F. Mieler
4 Retinal Manifestations in Hematological Disorders �������������������������������� 51
Giulio Barteselli, Maura Di Nicola, and Laura Dell’Arti
5 Retinal Manifestations of Renal Diseases�������������������������������������������������� 67
Aniruddha Agarwal and Alessandro Invernizzi
6 Imaging of Retinal and Choroidal Manifestations
of Gastrointestinal Disease�������������������������������������������������������������������������� 79
Francisco J. Rodríguez, Catalina Becerra,
and María Cristina Gabela
7 Ocular Sarcoidosis ������������������������������������������������������������������������������������ 103
David Ehmann and Sunir Garg
8 Autoimmune Retinopathy ������������������������������������������������������������������������ 113
David Ehmann and Sunir Garg
9 Systemic Infectious Diseases �������������������������������������������������������������������� 125
Aniruddha Agarwal and Vishali Gupta
10 Imaging of Retinal and Choroidal Metastases���������������������������������������� 153
Akshay Gopinathan Nair, David Fell, Sherief Raouf,
and Swathi Kaliki
11 Phakomatosis���������������������������������������������������������������������������������������������� 163
Nishant Radke, Carol L. Shields, J. Fernando Arevalo,
and Jay Chhablani
ix
x Contents
xi
xii About the Editors
paper. He has attended and presented papers in various national and international
conferences. His areas of interest include medical management of uveitis and scle-
ritis, uveitis in autoimmune disorders, and phacoemulsification in uveitic cataracts.
He is now working as consultant in the Department of Uvea and Intraocular
Inflammation, Sankara Nethralaya, Chennai. He has published many articles in
various peer-reviewed and non-peer-reviewed journals. He is the editor of INSIGHT,
the scientific journal of medical and vision research foundation. He is the founder
cum chief editor of the popular ophthalmology portal www.eophtha.com.
xiii
xiv Contributors
xvii
xviii Authors’ Biography
completion of his internship at Mercy Hospital and Medical Center in San Diego,
CA (1980), he completed his 3-year ophthalmology residency at the Bascom Palmer
Eye Institute, University of Miami, Miami, FL (1980–1983). This was followed by
a 1-year vitreoretinal fellowship at the Eye Institute, Medical College of Wisconsin,
Milwaukee, WI (1983–1984). He then returned to the Bascom Palmer Eye Institute
where he served as chief resident and clinical instructor (1984–1985). Dr. Mieler
then completed a second fellowship in ocular oncology at Wills Eye Hospital,
Thomas Jefferson University, Philadelphia, PA (1985). In 1985, he joined the full-
time faculty at the Medical College of Wisconsin, where he became professor of
ophthalmology (1992) and was awarded the Jack A. and Elaine D. Klieger chair in
ophthalmology (1998). Dr. Mieler then joined the faculty at the Baylor College of
Medicine, in Houston, TX, as professor of ophthalmology (1999–2004). He then
accepted the position of professor and chairman of the Department of Ophthalmology
at the University of Chicago (2004–2008), prior to his current position at the
University of Illinois at Chicago (2008–present).
Dr. Mieler has authored or co-authored 350 scientific papers, 85 book chapters,
and 7 textbooks, including The Retinal Atlas, 2nd edition (2017), along with present-
ing 25 named lectures. He is/has been the principal investigator or co-investigator of
more than 60 scientific grants and collaborative studies. He has served the American
Board of Ophthalmology (ABO) as a board director (1998–2005), as chairman of
the board (2005), as associate executive director (2006), and as emeritus director
(2006–present). He also served on several committees with the American Board of
Medical Specialties (ABMS). Dr. Mieler is also the past president of the Macula
Society (2003–2004), and he received the Gass Medal (2013). He has served on the
executive committees of the Retina Society and the American Society of Retina
Specialists (ASRS). In 2011, he was named recipient of the Founders Award by the
ASRS. He is a past member of the Pan-American Board of Directors (2001–2008),
and he has chaired the PAAO Foundation Grants Committee (2006–2011). Dr.
Mieler has served on the ARVO Board of Trustees (2010–2016), representing the
Retina Section, and was president of ARVO (2014–2015). He received the
Distinguished Service Award from ARVO (2016). He has served on the editorial
board of the Archives of Ophthalmology, RETINA, and Current Eye Research and
currently serves on the editorial board of the Asia-Pacific Journal of Ophthalmology
and the American Journal of Ophthalmology Case Reports. He also serves as a
scientific reviewer for 34 additional scientific journals. From the American Academy
of Ophthalmology (AAO), he has received the Honor Award (1992), the Lifelong
Education for the Ophthalmologist Award (2000), the Senior Honor Award (2001),
and the Life Achievement Honor Award (2011). He also has served as a member of
the AAO Council (2001–2008) and the EyeNet Editorial Advisory Board (2003–
2007) and is the chair of the Schepens Award (2007–2010). He also has served the
AAO as a media spokesman, as a member of the CME Committee, and as associate
secretary for the AAO Subspecialty Day programs (2011–2015). Most recently, he
was elected to serve on the AAO Board of Trustees (2017–2020).
Authors’ Biography xxvii
various scientific meets and forums. He has been awarded the best paper in neuro-
ophthalmology at the All India Ophthalmological Conference in 2011, the
Kanthimathinathan Gold Medal for the best graduating resident, and the best poster
award at the American Academy of Ophthalmology Meet in 2013 at New Orleans.
His areas of special interest are ocular surface and eyelid tumors, ocular trauma,
ophthalmic imaging, and neuroimaging.
Dr. Nair is a faculty member at Lokmanya Tilak Municipal Medical College,
Mumbai, India, and is also affiliated with Advanced Eye Hospital and Institute in
Navi Mumbai and Aditya Jyot Eye Hospital in Mumbai.
Illustrator: Temple
Language: English
by DAVID MASON
Illustrated by TEMPLE
Grady did not say he was sorry. It would have been of no use
whatever. Nor did he point out that the sun came out so infrequently
that his mistake was one which could be excused. Among the Kya
there are very few mistakes which can be excused, and stepping on
the shadow of a chief is not one of them.
Neither did Anla make any reference to the long friendship between
them, because there would have been no point in doing so. Anla's
eyes grew darker, and the wrinkles at their corners deepened, but
his words were calm, the correct words for such a time.
"Your name was Shassa," Anla said. "You have broken the ghost-
cloak of the Chief, and your name cannot be Shassa. From this
place and this time I take back your name, Shassa, and you have no
name."
Grady did not say anything, because a Kya cannot hear the words of
a man without a name; besides, there was nothing to say, though a
great deal to think about. The Berenice was due in four days. Four
days during which a man without a name would have to avoid the
custom which decreed that such a man must be killed. Killed as soon
as possible, because each day he continued to live was a day which
must be removed from the calendar, a day on which no man's birth
date might be celebrated, or any animal killed for food, or any root
taken from a garden.
Grady turned, and walked slowly, with a stiff back, down the path
away from the Chief's house. To run, or to show fear, would be fatal;
the Kya were themselves in a state of shock at the thing which had
happened, and it would be an hour or more before they began to
prepare for what they had to do. Therefore, Grady held his spine
straight, feeling a cold spot between his shoulderblades where the
first iron-headed arrow might strike in.
Ahead of him, through the village, the silent children ran on light feet,
darting into the houses and out again—the children, who were the
bearers of news. He saw three of them dash toward the agency, and
enter it; and in a moment, as he came up the path, Shallra came out
on the porch, carrying a clay pot in her hands.
"You who were Kotasa," she said, "take this, and drink it to free me
of your name."
It was the standard form of divorce among the Kya, and if the eyes of
Shallra had not been bright with tears, Grady might have slipped. He
took the clay pot, but he did not drink, because he could smell the
faint and bitter odor about it, which was not the odor of the fruit wine
that it should have held.
"Why?" he asked her, quietly.
"Because it is an easier death than the knife and the arrow," she
said, and added, "When you were Kotasa, you were—a good man
for me. Drink the wine." She said it pleadingly. He shook his head.
"I am sorry," Shallra said, and there is nothing harder for a Kya to
say. But she added something even harder for a Kya woman to say;
his name, his proper name, which she had always known but could
never use. Then she walked away, and out of Grady's life, because
he was now a man without a name.
He set the pot down carefully on the agency's steps and went inside.
As he closed the door, there was a high, whistling noise, and a sharp
thud against the door planks. He did not need to look to know that an
arrow stood in its wooden panels.
Grady closed the heavy wooden shutters carefully, not even jumping
when a second arrow whickered through the last shutter as it swung.
He lit a table lamp and took the heavy, seldom-used rifle from the
wall. He did not need to check it; he had oiled and cleaned it once a
week for two years. Instead, he laid it on the table and took a book
down from a shelf.
"General Code of the Federation Authority," Grady read the words on
the spine, and opened it. "Extent of responsibilities of individuals on
mandated planets...."
Under the circumstances, Grady discovered, he could kill any
number of Kya if he were so inclined. The Authority would require a
full report, in quadruplicate, of the circumstances—and as another
arrow struck the door, Grady wondered wryly who would make out
that report.
Also, Grady was not in the least inclined to kill any Kya. If doing so
would have saved his life, he would have shot any number of them
without any particular qualms. But there were no reasons at all to
think that killing any of them would do Grady any good. And Grady
thoroughly understood why it was that they had to kill him. He was
no more angry with the Kya than he had been with the Imperial
Guards, five years before, when they had come up Kanno Hill with
their band playing and their bayonets gleaming. He could remember
how military and colorful they had looked, in comparison to the
overalled, grimy rabble who stood beside him; and how they had
come up that hill again and again, fewer of them each time, and the
band losing a bit of verve on the last. Grady's anger then had been
at the damned fool, whoever he was, who ordered those useless
charges; and his anger now was with himself, because it had been
his own mistake.
There was a growing murmur outside the agency. The villagers were
gathering in the street, and in the yards behind the building. There
was no way out now, and nowhere to go if there had been a way out.
Grady got up, and walked to the door. He opened the sliding panel a
crack and peered out.
The rain had begun again, and through its thin gray curtain he could
see the ranks of villagers, silent, standing around the house, along
the railings, and watching. The men stood in front, each holding his
weapons, his bow bent in his hands. There was Lahrsha, who had
been brother in the Lodge to Grady, and whose blood had been
mixed with his to seal the tribal bond. There was Ahl, whose small
son Grady had nursed through a bad week. There were Grady's
friends and neighbors and brothers, each with an arrow on the nock
for Grady.
"It's a queer thing to happen," Grady said to himself, aloud. The
sound of his own voice startled him; he had become so much a Kya
that to him a man without a name should not have a voice.
The arrows struck oftener now. Grady saw a small group of men
move away, and then return, carrying a short log.
The door, Grady thought. They'll break it down, and come in, with
their grave faces and their polite ways, and they'll cut my throat. And
it won't matter if I kill one or two or ten of them; they'll do it anyway.
They won't hear an argument, because they can't hear me at all,
without a name; they won't even hear any noise I make when they
finish me off.
The log had begun to beat against the door, with a steady thunder.
Grady opened a cabinet, and took out a jar of brown liquid. Quickly
he drank it and sat down, his face graying. His head fell forward on
his arms, and the book of regulations fell to the floor, atop the unfired
rifle.
The Berenice swung outward, riding home to port with an empty
hold. The Mallor Company would not be pleased, but there were
other jobs. And the mate, sitting across the messroom table from
James Grady, put the matter in its simplest terms.
"Just one of those things," the mate said. "You can't be blamed.
They'll take another agent without any fuss, I imagine."
"No doubt of it," Grady said. "Can't say I'm glad to leave, though. It
was a good place."
"I still don't get it," the mate said. "We came in and found you in the
agency, out cold with coca. The door was down, and arrows all over
the place. Why didn't they come in and dig a knife into you?"
"Customs and taboos," Grady said. "I took a chance on it, but I was
pretty sure I was right. Common sense—by their standards. Man's
asleep—his ghost is walking around. If you kill him in his sleep, you
free his ghost, which is very bad, very strong magic. So you have to
wake him up to kill him. And they couldn't wake me up; I was full of
that coca leaf, enough for a week."
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