Ocular Oncology

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Editor
Duangnate Rojanaporn
Department of Ophthalmology
Faculty of Medicine
Ramathibodi Hospital
Mahidol University
Bangkok
Thailand

ISBN 978-981-13-2335-5    ISBN 978-981-13-2336-2 (eBook)

https://doi.org/10.1007/978-981-13-2336-2

© Springer Nature Singapore Pte Ltd. 2019

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Contents

1 Choroidal Nevus. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
Carol L. Shields and Jerry A. Shields
2 Choroidal Melanoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13
Carol L. Shields and Jerry A. Shields
3 Choroidal Metastasis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27
Greg Bever, Armin Afshar, and Bertil Damato
4 Choroidal Hemangioma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35
Duangnate Rojanaporn
5 Choroidal Osteoma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 45
Tero T. Kivelä
6 Retinal Vascular Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 61
Jose J. Echegaray, Rubens Belfort Neto, and Arun D. Singh
7 Vasoproliferative Tumors of the Retina . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 67
Duangnate Rojanaporn
8 Astrocytic Hamartoma of the Retina . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 77
Andrew W. Stacey and Mandeep S. Sagoo
9 Intraocular Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 83
Hiroshi Goto
10 Retinoblastoma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 91
Carol L. Shields and Sara E. Lally
11 Tumors and Related Lesions of the Retinal Pigmented Epithelium . . . . . . . . . . 101
Carol L. Shields and Jerry A. Shields
12 Retinal Metastasis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 115
Sachin M. Salvi, Soyang E. Kim, and Arun D. Singh
13 Peripheral Exudative Haemorrhagic Chorioretinopathy. . . . . . . . . . . . . . . . . . . 119
Peter Heydon and Mandeep S. Sagoo
14 Optic Disc Melanocytoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 123
Minoru Furuta
15 Intraocular Medulloepithelioma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 131
Shweta Rathi Gupta and Swathi Kaliki
16 Paraneoplastic-Related Retinopathy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 139
Tharikarn Sujirakul and Stephen H. Tsang
17 Sclerochoroidal Calcification. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 147
Tero T. Kivelä

v
About The Editor

Duangnate Rojanaporn, MD, FICO graduated from Mahidol University, Bangkok,

Thailand, before completing an International Council of Ophthalmology (ICO) fellowship in
medical and surgical retina at New York Eye and Ear Infirmary and a fellowship in ocular
oncology at Wills Eye Hospital, USA. She is the Director of the Ocular Oncology Service, and
course instructor for the Retina Service at the Department of Ophthalmology, Ramathibodi
Hospital. Dr. Rojanaporn is a pioneer of plaque brachytherapy, and intra-arterial chemotherapy
for retinoblastoma in the South East Asia region. She coordinates the ocular oncology program
for several international conferences and has been invited to deliver presentations at numerous
local, regional, and international meetings. Dr. Rojanaporn received an ICO fellowship grant
in 2008, a Professor Yasuo Tano travel grant for the Asia-Pacific Academy of Ophthalmology
(APAO) meeting in 2013, and an Asia-Pacific Academy of Ophthalmology Achievement
Award in 2018.

vii
‘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 in Retina

ix
 Choroidal Nevus
1
Carol L. Shields and Jerry A. Shields

1.1 Introduction most commonly detected in Whites at 5.6% (Ng et al. 2009)
(Table 1.1).
Choroidal nevus is the most common benign intraocular Population-based studies often limit inclusion criteria to
tumor, found predominantly in Caucasian patients (Chien study a certain age group, generally adults without inclusion
et al. 2017; Shields and Shields 2015; Kaliki and Shields of younger age group, or study a prominent ethnic group, or
2015). This tumor is generally asymptomatic but can carry study only a limited portion of the fundus. The NHANES
risk for visual acuity loss, especially if located near the fove- study included all ethnicities but was deficient in that it stud-
ola, and risk for transformation into malignant melanoma. ied only individuals >40 years old and was limited to two
Several features allow clinical identification of choroidal 45-degree photographs of the macula and optic disc (Qiu and
nevus and differentiation from melanoma. Shields 2015). This study potentially underrepresented the
true prevalence of choroidal nevus in US adults as one might
speculate that if the entire fundus was included, the preva-
1.2 Prevalence lence of choroidal nevus could reach as high as 20–25% (Qiu
and Shields 2015).
There have been a few population-based studies reporting
on the prevalence of choroidal nevus including the Blue
Mountains Eye Study (6.5%), Beijing Eye Study (BES) 1.3 Clinical Features
(2.9%), Singapore Malay Eye Study (SiMES) (1.4%),
Multi-­
Ethnic Study of Atherosclerosis (MESA) (2.1%), Choroidal nevus is a flat or minimally elevated mass with
Central India Eye and Medical Study (CIEMS) (0.3%), brown pigmentation (melanotic; pigmented) or without
and National Health and Nutrition Examination Survey (amelanotic; nonpigmented) (Chien et al. 2017; Sumich
(NHANES) (4.7%) (Sumich et al. 1998; Jonas et al. 2008; et al. 1998; Jonas et al. 2008; Ng et al. 2009; Greenstein et al.
Ng et al. 2009; Greenstein et al. 2011; Nangia et al. 2012; 2011; Nangia et al. 2012; Qiu and Shields 2015; Shields
Qiu and Shields 2015). Most of the population-based studies et al. 2008a; Shields et al. 2010; Shields and Shields 2015;
have focused on specific ethnic groups such as Australian Kaliki and Shields 2015). This tumor is classically in the
(Sumich et al. 1998), Chinese (Jonas et al. 2008), or Asian post-equatorial fundus (91%) or pre-equatorial fundus (9%),
Indian (Ng et al. 2009) population. The NHANES study and most nevi are pigmented (77%) (Shields et al. 2008a)
reviewed all ethnic groups in the United States (US) and (Table 1.2). The distribution of choroidal nevus is equiva-
found overall prevalence of choroidal nevus in US adult lent in all quadrants (Shields et al. 2008a). Most choroidal
population≥40 years old was 4.7%, and this lesion was nevi are extrafoveolar (94%) compared to subfoveolar (6%).
Tumor size varies depending on the study, but one tertiary
C. L. Shields (*) · J. A. Shields (*)
ocular oncology clinic-based center found mean basal tumor
Ocular Oncology Service, Wills Eye Hospital, Philadelphia, PA, diameter was 5 mm and thickness was 1.5 mm, compared to
USA a population-based study where choroidal nevus was found
Department of Ophthalmology, Thomas Jefferson Unversity, to have mean basal diameter of 1.25 mm (Sumich et al. 1998;
Philadelphia, PA, USA Shields et al. 2008a).

© Springer Nature Singapore Pte Ltd. 2019 1

D. Rojanaporn (ed.), Ocular Oncology, https://doi.org/10.1007/978-981-13-2336-2_1
2 C. L. Shields and J. A. Shields

Table 1.1 Prevalence of choroidal nevus from a population-based study (National Health and Nutrition Examination Survey 2005–2008) in the
United States: grouped by age, sex, and racea
Sex Race
Number of Male Female P White Black Hispanic Other P
patients (n = 2785) (n = 2790) valueb (n = 3012) (n = 1133) (n = 1249) (n = 181) valueb Total
Age, yrs
 40–50 1483 5.0% 4.4% 0.7 5.8% 0.7% 1.3% 4.4% 0.005 4.7%
 50–60 1322 3.3% 2.9% 0.7 3.7% 0.3% 2.9% 0.0% 0.04 3.1%
 60–70 1384 6.5% 4.4% 0.2 6.3% 0.9% 4.1% 0.8% 0.002 5.4%
 70–80 915 6.8% 6.5% 0.9 7.3% 0.0% 5.6% 1.6% 0.2 6.6%
 ≥80 471 7.5% 7.5% 0.9 7.7% 0.1% 8.5% 11.6% 0.6 7.5%
 P valueb 0.07 0.1 n/a 0.08 0.6 0.07 0.08 n/a n/a
Race
 White 3012 6.2% 5.2% 0.4 n/a n/a n/a n/a n/a 5.6%
 Black 1133 1.0% 0.2% 0.07 n/a n/a n/a n/a n/a 0.6%
 Hispanic 1249 2.8% 2.5% 0.8 n/a n/a n/a n/a n/a 2.7%
 Other 181 0.5% 3.5% 0.02 n/a n/a n/a n/a n/a 2.1%
 P valueb <0.0001 0.0001 n/a n/a n/a n/a n/a n/a n/a
Total 5575 5.0% 4.4% n/a 5.6% 0.6 2.7% 2.1% n/a 4.7%
n/a = not available, Other—East Asians, Asian Indians, and non-Hispanic multiracial individuals
Information adapted from a population-based study, 9Qui M, Shields CL. Choroidal Nevus in the United States Adult Population: Racial Disparities
and Associated Factors in the National Health and Nutrition Examination Survey. Ophthalmology 2015;122:2071–83
a
Proportions are weighted estimates of US population characteristics, taking into account the sampling design of NHANES
b
P values are unadjusted and calculated using the Rao-Scott Pearson chi-square test

Table 1.2 Choroidal nevus in 3422 eyes from an ocular oncology 1.3.1 Low-Risk Choroidal Nevus
clinic-based study: tumor features based on patient age at initial
examination
Choroidal nevus can be categorized into low or high risk for
Young Mid-adults transformation into melanoma. Low-risk nevus is one that
patients (21– Older adults
(≤ 20 years 50 years (>50 years carries low likelihood for transformation into melanoma.
old) old) old) Features include thickness ≤2 mm and absence of subretinal
(n = 63) (n = 795) (n = 2564) fluid, orange pigment, and symptoms. These lesions classi-
Nevus feature [n (%)] [n (%)] [n (%)] cally appear echodense on ultrasonography and demonstrate
Anteroposterior location
overlying retinal pigment epithelium (RPE) alterations such
 Macula 22 (35) 186 (23) 504 (20)
 Macula-equator 40 (63) 548 (69) 1821 (71)
as drusen, RPE atrophy, and dependent RPE trough from
 Equator-ora serrata 1 (2) 61 (8) 239 (9) previous subretinal fluid, RPE hyperplasia, RPE detach-
Quadrantic location ment, RPE fibrous metaplasia, and RPE osseous metaplasia
 Macula 22 (35) 186 (23) 504 (20) (Shields et al. 2008a; Kaliki and Shields 2015) (Figs. 1.1,
 Inferior 7 (11) 140 (18) 460 (18) 1.2, 1.3, 1.4 and 1.5). Rarely, choroid nevus can produce
 Temporal 11 (17) 162 (20) 631 (25) chronic RPE damage that leads to development of choroidal
 Superior 8 (13) 116 (15) 410 (16)
neovascular membrane (Shields et al. 2008a) (Fig. 1.6).
 Nasal 15 (23) 191 (24) 559 (22)
Location relative to foveolar
 Subfoveolar 9 (14) 53 (7) 143 (6)
 Extrafoveolar 54 (86) 742 (93) 2421 (94)
1.3.2 High-Risk Choroidal Nevus
Basal diameter (mm) 5.0 (5.6, 4.5 (4.7, 5 (5.2,
[median (mean, range)] 0.75–24) 0.5–14) 0.4–20) High-risk nevus is one that carries high likelihood for trans-
Thickness (mm) [median 1 (1.2, 1.5 (1.5, 1.5 (1.6, formation into melanoma. Features include thickness >2 mm,
(mean, range)] 0.6–22) 0.7–3.7) 0.7–4.5) presence of subretinal fluid, orange pigment and/or symp-
Color toms, acoustic hollowness on ultrasonography, and absence of
 Melanotic 56 (89) 591 (74) 1981 (77)
chronic features such as drusen or surrounding halo (Shields
 Amelanotic 4 (6) 83 (10) 269 (10)
 Mixed 3 (5) 121 (15) 313 (12) et al. 2009) (Table 1.3) (Figs. 1.7 and 1.8). Location can be a
Information adapted from an ocular oncology clinic-based case series,
factor as nevi within 3 mm of the optic disc are more likely
10
Shields CL, et al. Clinical spectrum of choroidal nevi based on age at to evolve into melanoma. High-risk nevus classically appears
presentation in 3422 consecutive eyes. Ophthalmology 2008;115:546–52 with little to no overlying RPE alterations such as drusen.
1 Choroidal Nevus 3

a b

c
d

Fig. 1.1 Choroidal nevus with overlying drusen. (a) Drusen covering the entire aspect of the nevus. (b) Drusen covering the central apex of the
nevus. (c) Drusen extending beyond the nevus margins. (d) Larger choroidal nevus with central retinal pigment epithelial atrophy and drusen

1.3.3 Halo Nevus phocytes acting toward the skin melanoma could target sim-
ilar antigens present on choroidal nevus cells resulting in
Halo nevus is an unusual form of cutaneous nevus, dis- halo formation. An unusual variant of halo nevus is “reverse
playing a central pigmented portion with surrounding halo” where the center is amelanotic and the surround is
­depigmented halo, most often found in young patients and melanotic. Dolz-Marco et al. has documented a relationship
believed to represent an immune response. Halo choroidal of posterior scleral bowing on EDI-OCT with halo choroi-
nevus is characterized by a pigmented nevus surrounded dal nevus (Dolz-Marco et al. 2015).
by a nonpigmented nevus halo, representing 5% of all cho-
roidal nevi (Shields et al. 2010) (Fig. 1.9). Choroidal halo
nevus has been found most often to signify a “low-risk” 1.3.4 Giant Nevus
nevus (Shields et al. 2009). In one analysis of 150 patients
with halo choroidal nevus, Shields et al. found relation- Giant choroidal nevus is defined as a nevus with basal
ship to previous history of skin melanoma (p < 0.001) and diameter ≥10 mm and represents 8% of all choroidal
no association with autoimmune dysfunction or vitiligo nevi seen in an ocular oncology referral practice (Li et al.
(Shields et al. 2010). They speculated that cytotoxic lym- 2010) (Fig. 1.10). Due to its large basal dimension and
4 C. L. Shields and J. A. Shields

a b

Fig. 1.2 Chronic choroidal nevus with overlying drusen. (a) Choroidal RPE detachments. (c) Optical coherence tomography documents over-
nevus with overlying retinal pigment epithelial (RPE) atrophy and dru- lying retinal edema, outer retinal disorganization and retraction, small
sen. (b) Autofluorescence documents the dark RPE loss as well as the RPE detachment, and choroidal mass with compression of the
ring-shaped hyperautofluorescence of soft drusen, representing tiny choriocapillaris

a b

Fig. 1.3 Choroidal nevus with overlying retinal pigment epithelial (b) Autofluorescence documents the dark appearance of RPE atrophy
(RPE) alterations. (a) Choroidal nevus with surface demonstrating and hyperplasia. The bright signal at the anterior margin likely repre-
white RPE fibrous metaplasia, RPE atrophy, and dark RPE hyperplasia. sents partially damaged RPE
1 Choroidal Nevus 5

a b c

Fig. 1.4 Choroidal nevus with overlying retinal pigment epithelial shows the minimal elevation and acoustic hollowness of the RPE
(RPE) detachment. (a) Choroidal nevus with overlying yellow RPE detachment. (d) Optical coherence tomography demonstrates the
detachment. (b) Autofluorescence documents the bright signal of the abruptly elevated RPE detachment with draped overlying retina and
RPE detachment and the dark signal of the nevus. (c) Ultrasonography deep shadowing

a b

Fig. 1.5 Choroidal nevus with overlying retinal pigment epithelial phy. Note the second nevus in the temporal macular region. (b)
(RPE) osseous metaplasia. (a) Chronic choroidal nevus with overlying Ultrasonography confirms the dense, echogenic calcific plaque overly-
white osseous changes in the RPE as well as RPE hyperplasia and atro- ing the nevus
6 C. L. Shields and J. A. Shields

Fig. 1.6 Choroidal nevus with overlying choroidal neovascularization. Fluorescein angiography documents hypofluorescence of the choroidal
(a) Ill-defined choroidal nevus with overlying shallow subretinal fluid nevus and hyperfluorescence of the choroidal neovascularization with
with exudation and precipitation, extending into the fovea. (b) leakage

Table 1.3 Clinical factors at initial examination predictive of choroidal nevus transformation into melanoma
Hazard ratio (compared
to nevus without
Mnemonic Initial Variable feature) P value
To T Thickness > 2 mm (vs. ≤2 mma,b) 2 <0.001
Find F Subretinal fluid present (vs. absent a) 3 0.002
Small S Symptoms
   Decreased vision (vs. none a) 2 0.02
   Flashes/floater (vs. none a) 2 0.002
Ocular O Orange pigment present (vs. absent a) 3 <0.001
Melanoma M Margin distance to optic nerve ≤3 mm (vs. >3 mm a) 2 0.001
Using helpful UH Ultrasonographic acoustic hollowness (vs. solid a) 3 <0.001
Hints H Halo absent (vs. present a) 6 0.009
Daily D Drusen absent (vs. present) na na
Analysis of 2514 patients from an ocular oncology clinic-based population
Information adapted from a retrospective medical record review study, 10Shields CL, Furuta M, Berman EL, et al. Choroidal nevus transformation
into melanoma: analysis of 2514 consecutive cases. Arch Ophthalmol 2009;127:981–987
a
Reference variable. Na-not significant in this study but was found significant in other studies
b
When assessing tumor thickness as a continuous variable, significance was found in the multivariate model (hazard ratio, 2.75, per 1-mm increase;
P < 0.001). When assessing distance to the optic nerve as a continuous variable, significance was found in the multivariate model (hazard ratio,
0.90, per 1-mm increase; P < 0.001)

thickness, it can be mistaken for choroidal melanoma. In 1.4 Imaging

an analysis of 322 consecutive cases, Li et al. found giant
nevus transformed into melanoma in 18% at 10 years Choroidal nevus is currently evaluated with fundus pho-
(Li et al. 2010). The features significantly predictive of tography, ultrasonography, fluorescein angiography (FA),
transformation included close proximity to the foveola indocyanine green angiography (ICGA), optical coherence
(p = 0.02) and ­ultrasonographic evidence of acoustic hol- tomography (OCT), fundus autofluorescence (FAF), and
lowness (p = 0.05) (Li et al. 2010). OCT angiography (OCTA).
1 Choroidal Nevus 7

a b

Fig. 1.7 Choroidal nevus with overlying mild orange pigment. (a) Submacular choroidal nevus with overlying orange pigment. (b) Autofluorescence
documents the hyperautofluorescence of the orange pigment representing lipofuscin within macrophages

a b

Fig. 1.8 Choroidal melanocytic tumor with overlying marked orange cin) hyperautofluorescence, suggestive of activity. (c) Optical coher-
pigment. (a) Choroidal melanocytic tumor with prominent overlying ence tomography demonstrates shallow subretinal fluid with
orange pigment, strongly suggestive of small melanoma rather than photoreceptor loss and underlying choroidal mass
nevus. (b) Autofluorescence documents the orange pigment (lipofus-
8 C. L. Shields and J. A. Shields

a b

Fig. 1.9 Choroidal halo nevus. (a) The choroidal nevus is surrounded by a subtle yellow halo. (b) Autofluorescence documents the central nevus
as dark and the surrounding halo as brighter, believed to represent unmasking of scleral autofluorescence

a 1.4.1 Ultrasonography

Ultrasonography is employed for tumor-thickness mea-

surement as well as intrinsic tumor echogenicity. Many
choroidal nevi are nearly flat and are detected as only a
single echo. Ultrasonography can provide baseline tumor
thickness for future comparison. Nevus tends to demon-
strate high internal reflectivity on A scan and echodensity
on B scan (Chien et al. 2017; Shields and Shields 2015;
Kaliki and Shields 2015). In contrast, melanoma demon-
strates by low to medium internal reflectivity on A scan
and echolucency on B scan.
b

1.4.2 Fluorescein Angiography

In the past, FA was more often employed in the evalu-

ation of choroidal nevus, but currently, FA is reserved
for nevus with subretinal fluid, hemorrhage, or exudation
to identify pinpoint RPE leaks or choroidal neovascular
membrane (CNVM). In some instances, FA can be use-
ful in the differentiation of choroidal nevus from small
choroidal melanoma as nevus remains typically hypofluo-
rescent, whereas melanoma can demonstrate intrinsic vas-
Fig. 1.10 Giant choroidal nevus. (a) Large choroidal nevus measuring cularity (double ­circulation) or overlying pinpoint RPE
>10 mm diameter (giant) and with confluent overlying drusen. (b) leaks (Chien et al. 2017; Shields and Shields 2015; Kaliki
Autofluorescence documents the dark nevus and slight hyperautofluo- and Shields 2015).
rescence of the drusen
1 Choroidal Nevus 9

1.4.3 Indocyanine Green Angiography Choroidal halo nevus shows an interesting finding on
EDI-OCT in many cases, with posterior bowing of the sclera
The role of ICGA in the management of choroidal nevus is at the site of the nevus base (Dolz-Marco et al. 2015).
yet to be defined. Studies on ICGA for intraocular tumors
reveal that this technology could be useful for differentiating
choroidal hemangioma and metastasis from melanoma and 1.4.5 Fundus Autofluorescence
nevus (Shields et al. 1995a). Thin melanoma and nevus tend
to be hypocyanescence. ICGA might be useful in delineating Fundus autofluorescence (FAF) can be a reliable marker of
overlying CNVM related to nevus. nevus as nevus tends to be hypoFAF, whereas small mela-
noma tends to be hyperFAF (Shields et al. 2008b; Almeida
et al. 2013). Shields et al. evaluated 64 eyes with choroidal
1.4.4 Optical Coherence Tomography nevus and found features of overlying RPE isoFAF or hypo-
FAF in most cases, from RPE atrophy or fibrous metaplasia
Enhanced depth imaging OCT (EDI-OCT) is a highly (Shields et al. 2008b). In contrast, small choroidal melanoma
valuable tool for evaluation of choroidal nevus, particu- demonstrates overlying RPE hyperFAF, correlating one-to-­
larly the status of the overlying retina and RPE. EDI-OCT one with lipofuscin. Recently, Albertus et al. developed a
can image choroidal nevus, even those that are flat and novel quantification method for FAF of lipofuscin and pro-
undetectable by ultrasonography (Torres et al. 2011; Shah posed that this method could be useful to clinically differ-
et al. 2012). We reported characteristic features of choroi- entiate between nevus and melanoma (Albertus et al. 2013).
dal nevus on EDI-­OCT, including choriocapillaris thinning
over the nevus apex (94%) and partial (59%) or complete
(35%) choroidal shadowing deep to the nevus, depending 1.4.6 Optical Coherence Tomography
on nevus pigmentation, RPE atrophy (43%), and photo- Angiography
receptor loss (43%) (Shah et al. 2012). It was found that
relatively low-resolution ultrasonography overestimated OCTA is a fairly new noninvasive microvascular imaging
nevus thickness measurements compared to high-resolu- technique for the retina, permitting imaging of three capil-
tion EDI-OCT by approximately 126% (Shah et al. 2012). lary plexuses including the radial peripapillary, superficial
Subretinal fluid was noted overlying nevus (16%), and the capillary, and deep capillary plexuses. OCTA enables seg-
chronicity of fluid could be important in the nevus sta- mental quantification of each plexus, not possible on conven-
tus as thinned outer retina with photoreceptor retraction tional FA (Spaide et al. 2015). A recent analysis on OCTA
(“stalactite” appearance) or absence (“cleft”) typically of nevus versus small melanoma by Valverde-Megias et al.
signifies chronic retinal degeneration from long-standing showed eyes with nevus had similar central macular thick-
fluid. This feature was more often found with nevus rather ness, foveal avascular zone area, and capillary vascular
than melanoma (Shields et al. 2012; Shields et al. 2014). density in the affected eye compared to the unaffected eye
By EDI-OCT, small choroidal melanoma shows partial (Valverde-Megias et al. 2017). By comparison, eyes with
(73%) or complete (27%) choroidal shadowing deep to small melanoma showed increased central macular thick-
the melanoma, similar to nevus. However, the major dif- ness, enlarged foveal avascular zone, and reduction of capil-
ferences are in the subretinal fluid (92% with melanoma), lary vascular density in the eye with melanoma compared to
showing typical “shaggy” photoreceptors and subretinal normal opposite eye (Valverde-Megias et al. 2017).
lipofuscin deposition (95%), both suggestive of tumor
activity (Shields et al. 2012). By comparison, EDI-OCT
of small melanoma versus nevus showed melanoma with 1.5 Risk Factors
greater irregularity of inner plexiform layer (p = 0.04),
loss of the ellipsoid zone (p = 0.02), shaggy photorecep- 1.5.1 Risk for Vision Loss
tors (p < 0.001), loss of photoreceptors (p = 0.05), loss of
external limiting membrane (p = 0.008), and intraretinal Vision loss with choroidal nevus strongly depends on the
edema (P = 0.003) (Shields et al. 2012). “Shaggy” pho- proximity of the nevus to the foveola. Shields et al. analyzed
toreceptors are speculated to represent subretinal macro- 3422 eyes with choroidal nevus for visual outcomes and
phages aligning on the posterior retinal surface or swollen found eye with subfoveolar nevus was significantly more
photoreceptors. Shields et al. documented “shaggy” photo- likely to develop reduced visual acuity (26%) compared to
receptors in 49% of small melanoma and in 0% eyes with those with extrafoveolar nevus (2%) by 15 years (Shields
nevus (Shields et al. 2012). et al. 2007). Factors predictive of visual acuity loss of three or