Table Of ContentRetina Atlas
Series Editors: Sandeep Saxena · Richard F. Spaide · Eric H. Souied · Timothy Y.Y. Lai
Inflammatory and
Infectious Ocular
Disorders
Hyeong Gon Yu
Editor
123
Retina Atlas
Series Editors
Sandeep Saxena, MS, FRCSEd, FRCS, FRCOphth
Department of Ophthalmology,
King George’s Medical University,
Lucknow, Uttar Pradesh, India
Richard F. Spaide, MD
Vitreous Retina Macula Consultants of New York,
New York, NY, USA
Eric H. Souied, MD
Department of Ophthalmology,
University Paris-Est Créteil,
Créteil Cedex, France
Timothy Y.Y. Lai, MD, FRCS, FRCOphth
Department of Ophthalmology and Visual Sciences,
Chinese University of Hong Kong,
Hong Kong, Hong Kong
The 9-volume atlas covers validated and comprehensive information on retinal imaging, retinal
vascular disorders, macular disorders, vitreoretinal surgical diseases, infectious and
inflammatory disorders, retinal degenerations and dystrophies, pediatric retinal diseases,
oncology, and trauma. This atlas with over 100 chapters is well supported with hundreds of
high-quality images and text notes providing in-depth details and information in a well-
organized manner.
The editors Sandeep Saxena (India), Richard F Spaide (USA), Eric Souied (France) and
Timothy Y Y Lai (Hong Kong), volume editors and contributing authors are reputed eye
physicians in their field with vast clinical experience. This series has a full dedicated volume on
imaging and includes various imaging technologies like optical coherence tomography,
fluorescein angiography, etc. It provides global perspective of vitreoretinal diseases extensively
covering medical and surgical aspects of the disease. Uncommon retinal findings in diseases
such as Dengue hemorrhagic fever, malaria etc. are also covered well.
Retina Atlas is a useful go-to series meant for ophthalmology residents, retina fellows, and
retina specialists as well as general ophthalmologists.
‘Retina Atlas’ series includes the following 9 Volumes:
1. Retinal Imaging
2. Retinal Vascular Disorders
3. Macular Disorders
4. Surgical Retina
5. Inflammatory and Infectious Ocular Disorders
6. Hereditary Chorioretinal Disorders
7. Pediatric Retinal Diseases
8. Ocular Oncology
9. Trauma and Miscellaneous Disorders in Retina
More information about this series at http://www.springer.com/series/16451
Hyeong Gon Yu
Editor
Inflammatory and Infectious
Ocular Disorders
Editor
Hyeong Gon Yu
Department of Ophthalmology
College of Medicine
Seoul National University
Seoul
Republic of Korea
ISSN 2662-5741 ISSN 2662-575X (electronic)
Retina Atlas
ISBN 978-981-13-8545-2 ISBN 978-981-13-8546-9 (eBook)
https://doi.org/10.1007/978-981-13-8546-9
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Contents
1 Acute Posterior Multifocal Placoid Pigment Epitheliopathy . . . . . . . . . . . . . . . . . 1
Dae Joong Ma
2 Serpiginous Choroiditis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9
Narsing A. Rao and Julie You Kwon
3 Punctate Inner Choroidopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19
Hyeong Gon Yu and Dae Joong Ma
4 Multifocal Choroiditis and Panuveitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29
Eun Young Choi and Min Kim
5 Multiple Evanescent White Dot Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39
Ji Eun Lee
6 Acute Zonal Occult Outer Retinopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 45
Wataru Saito and Susumu Ishida
7 Birdshot Chorioretinopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 51
Taylor W. Starnes, Pooja Bhat, and Ann-Marie Lobo
8 Sympathetic Ophthalmia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 59
Cindy Ung and Lucy H. Young
9 Vogt–Koyanagi–Harada Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 67
Peizeng Yang and Zi Ye
10 Intermediate Uveitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 77
Pınar Ç. Özdal and Ilknur Tugal-Tutkun
11 Sarcoidosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 93
Hiroshi Takase
12 Behçet Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .101
Nobuyoshi Kitaichi, Kenichi Namba, and Shigeaki Ohno
13 Systemic Lupus Erythematosus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .107
Joo Young Shin
14 Posterior Scleritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .113
Sophia Zagora, Denis Wakefield, and Peter McCluskey
15 Autoimmune Retinopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .125
Sang Jin Kim
16 Intraocular Tuberculosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .131
Aniruddha Agarwal, Tripti Choudhary, Kanika Aggarwal, and Vishali Gupta
17 Evaluation of Ocular Tuberculosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .137
S. R. Rathinam and P. Lalitha
v
vi Contents
18 Cat-Scratch Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .143
Cindy Ung and Lucy H. Young
19 Syphilis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .149
Sulaiman Alhumaid and Janet L. Davis
20 Leptospirosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .159
Sivakumar Rathinam, Rajesh Vedhanayaki, and Somanath Anjana
21 Fungal Infection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .163
Un Chul Park
22 Toxoplasmosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .173
Eun Kyoung Lee
23 Toxocariasis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .179
Tae Wan Kim
24 Ocular Cysticercosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .187
Kalpana Babu
25 Diffuse Unilateral Subacute Neuroretinitis (DUSN) . . . . . . . . . . . . . . . . . . . . . . . .193
Alice Y. Zhang, Fernando A. Arevalo, Reinaldo A. Garcia,
Carlos Alexandre de Amorim Garcia Filho,
Carlos Alexandre de Amorim Garcia, and J. Fernando Arevalo
26 Human Immunodeficiency Virus Infection and Cytomegalovirus Retinitis . . . . .205
Ian Y. Wong and Yong Tao
27 Acute Retinal Necrosis and Progressive Outer Retinal Necrosis . . . . . . . . . . . . . .215
Atsunobu Takeda, Mitsuru Arima, Keijiro Ishikawa,
Eiichi Hasegawa, Yusuke Murakami, and Koh-Hei Sonoda
About the Editor
Hyeong Gon Yu is a Professor at Seoul National University (SNU) College of Medicine and
is in charge of the retina and uveitis service at Seoul National University Hospital (SNUH),
where he has set up two special clinics: the Uveitis Clinic and the Hereditary Retinal Disease
Clinic. He has been the Chair of the Department of Preclinical Research at the SNUH
Bioresearch Institute since 2016 and the founding President of the Korean Association of
Retinal Degeneration since 2014. He is a member of the ISBD (International Society for
Behçet’s Disease) council and a board member of the Korean Retina Society and Korean
Uveitis Society. He is also a member of IUSG (International Uveitis Society Group) and
Macula Society. He was the first recipient of the Korean Retina Society Academy Award and
received the Distinguished Service Award from the APAO (Asia Pacific Academy of
Ophthalmology) in 2016 and 2017.
Furthermore, he has participated in numerous clinical trials as a principal investigator or
co-investigator. His interests include clinical and translational research on ocular inflammatory
diseases as well as retinal degeneration. He has published over 200 scientific papers in interna-
tional journals, was a chief editor for several books, including Asian Retina (Elsevier),
Ophthalmic Laser Treatment, Inherited Retinal Diseases, and Uveitis (Korean Elsevier), and
contributed chapters to various textbooks, such as Retina (Mosby) and Advances in Vision
Research—Volume I and II (Springer).
vii
Acute Posterior Multifocal Placoid 1
Pigment Epitheliopathy
Dae Joong Ma
Introduction 76.6% of eyes and 20/40 or worse in 58.3% but rarely worse
than 20/400 (Fiore et al. 2009; Smith et al. 1978). The dis-
Acute posterior multifocal placoid pigment epitheliopathy ease usually shows a bilateral involvement at presentation or
(APMPPE) is a rare inflammatory eye disease affecting the sequential involvement with an interval of days to weeks.
outer retina, retinal pigment epithelium (RPE), and chorio- Anterior segments may have no or mild non-granulomatous
capillaris in mostly otherwise healthy adults between the inflammation (1+ to 2+) (Lowes 1977; Alvi and Fishman
ages of 20 and 50 years. It shows no clear gender preference. 1995). The vitreous may also show a mild to moderate
APMPPE was first described by Gass in 1968 in a series of inflammation.
three young female patients who presented with multiple The acute presentation is multiple, round, and confluent
large plaque-like lesions at the level of the RPE associated creamy gray-white plaques, typically 1- to 2-disc areas in
with temporary bilateral visual loss (Gass 1968). APMPPE is size (Figs. 1.1a, b and 1.2a). The lesions are typically
considered one of the “placoid diseases” together with ser- bilateral and normally confined to the posterior to the
piginous choroiditis (SC), relentless placoid chorioretinitis, equator. Active lesions can develop and evolve over the
and persistent placoid maculopathy due to the “placoid” course of several weeks, and lesions of various stages may
nature of the lesions and similarities on fluorescein angiogra- appear simultaneously (Figs. 1.1c, d and 1.2b, c). After
phy (FA) and indocyanine green angiography (ICGA) find- acute stage, the lesions typically resolve centrally result-
ings (Mirza and Jampol 2012). ing in hypopigmentation. Later, lesions develop mild pig-
ment mottling and pigment condensation. Finally, coarse
pigment clumping increases over several months
Etiopathogenesis (Figs. 1.1e, f and 1.2d).
Atypical APMPPE with localized serous retinal detach-
The etiology and pathogenesis of APMPPE is uncertain. It is ments have been reported, which resembles Vogt–Koyanagi–
currently believed to be an inflammation of the outer retina Harada disease (Birnbaum et al. 2010; Garg and Jampol
and RPE (Gass 1968) or secondary to a hypersensitivity- 2004). The occurrence of choroidal neovascularization
induced obstructive vasculitis, resulting in ischemic injury of (CNV) (Fig. 1.3) (Bowie et al. 2005), optic disc edema (Abu
the overlying RPE and photoreceptor (Heiferman et al. 2017). El-Asrar and Aljazairy 2002; Frohman et al. 1987), subhya-
loid hemorrhage (De Souza et al. 1999), retinal vasculitis
(De Souza et al. 1999; Abu El-Asrar and Aljazairy 2002;
Clinical Features Thomas et al. 2012), or retinal vein occlusion has also been
reported (Abu El-Asrar and Aljazairy 2002; De Souza et al.
A history of an antecedent viral or flu-like illness or recent 1999).
vaccination was reported in over one third of patients (Gass In the acute stage of the disease, the active cream-colored
1968; Holt et al. 1976). Patients manifest with a sudden onset lesions block the choroidal fluorescence during the early
of visual blurring, scotoma, metamorphopsia, or photopsias phases of FA (Fig. 1.4b). Irregular staining was observed in
(Fiore et al. 2009). Initial visual acuity is 20/25 or worse in the lesions that had previously blocked fluorescence in the
later phase of FA (Fig. 1.4c). In the late or resolved stage of
D. J. Ma the disease, the mottled RPE demonstrate window defects
Department of Ophthalmology, Jeju National University Hospital, without leakage.
Jeju-si, Jeju-do, South Korea
© Springer Nature Singapore Pte Ltd. 2020 1
H. G. Yu (ed.), Inflammatory and Infectious Ocular Disorders, Retina Atlas, https://doi.org/10.1007/978-981-13-8546-9_1
2 D. J. Ma
a b
c d
e f
Fig. 1.1 Color fundus photography of a 29-year-old male. (a, b) Initial presentation. (c, d) Two weeks. (e, f) Sixteen months after initial
presentation
In ICGA, acute lesions show hypocyanescence in the FA. Unlike FA, deep choroidal vessels are visible within
early phase (Fig. 1.4d) and become more defined in shape the active lesion during early phase of ICGA and no hyper-
in the late phase (Fig. 1.4e). These hypocyanescent lesions cyanescence develops in the late phase. These findings sup-
are numerous than the lesions observed on funduscopy and port the hypothesis that the active APMPPE lesions result
