Part 6  Retina and Vitreous

Section 5  Vascular Disorders

Coats’ Disease and Retinal

Telangiectasia 6.25 
Ferhina S. Ali, Diana V. Do, Julia A. Haller   IN THIS CHAPTER
Additional content
available online at
ExpertConsult.com

Definition:  A localized, congenital, retinal vascular disorder consisting OCULAR MANIFESTATIONS

of abnormal telangiectatic segments of blood vessels that result in Coats’ disease is characterized by discrete zones of alteration in the
leakage. retinal vascular structure with aneurysmal dilatation, capillary dropout,
and leakage. Vision may decrease as a result of leakage from the abnor-
mal vascular channels that are formed, with consequent edema, lipid
deposition, and exudative retinal detachment. The typical fundus picture
Key Features of Coats’ disease is one of retinal vascular abnormalities associated with
• Retinal telangiectasia. localized lipid deposition and varying degrees of subneural retinal exudate
• Retinal capillary nonperfusion. (Fig. 6.25.1). Vessels may appear sheathed and telangiectatic, and they may
• Dilated intercapillary spaces. have aneurysms that are grape like, clustered, or lightbulb shaped. Often
• Lipid exudate. the vessels are adjacent to areas that lack normal capillaries (Fig. 6.25.2).
• Subretinal fluid. The severity of vascular malformation parallels the degree of surrounding
neural retinal thickening, exudation, hemorrhage, and destruction of small
vessels. Aberrant arteriovenous communicating channels are frequently
present, and occasionally true retinal neovascularization occurs. Leakage
from the abnormal vascular bed produces a cloudy subretinal exudate
Associated Features that gravitates toward the posterior pole. As the serous component of the
• Usually unilateral. exudate is resorbed by retinal vessels, the lipid-rich yellowish component is
• Male predominance. left beneath and within the outer neural retinal layers.9 Over long periods,
• Fibrovascular macular scars. this yellow exudate may stimulate the ingrowth of blood vessels and
• Leukokoria. fibrous scar tissue (Fig. 6.25.3). The vascular abnormalities occur more
• Rare systemic associations, especially muscular dystrophies. commonly superotemporally; they also are found in the macular and para-
macular areas. On average, two quadrants of retina are found to be affected
at the initial diagnosis in older patients, but young patients may have more
INTRODUCTION serious disease and more extensive retinal involvement. In more advanced
and severe cases of Coats’ disease, exudative retinal detachment develops
Retinal telangiectasia is found in a wide range of ocular disease processes. (Fig. 6.25.4).10
Most retinal telangiectases are acquired secondary to local or systemic The clinical course is variable but generally progressive. Acute exacer-
conditions such as branch retinal vein occlusion and diabetic retinopathy. bations of the disease may be interspersed with more quiescent stages.
Primary retinal telangiectasia is found in Coats’ disease, Leber’s miliary Spontaneous remissions have been reported, with spontaneous occlusion
aneurysms, idiopathic juxtafoveal telangiectasia, and other angiomatous of the vessels and resorption of the exudate, but these are the exception.
diseases. Secondary complications include neovascularization, vitreous hemorrhage,

EPIDEMIOLOGY
Coats’ disease, described by Coats1 in 1908, affects men three times as
often as women, has no reported racial or ethnic predilection, and is
usually unilateral, although as many as 10% to 15% of cases may be bilat-
eral. The average age at diagnosis is 8–16 years, although the disease has
been described in patients as young as 4 months. Approximately two-thirds
of juvenile cases present before age 10 years. Coats’ disease can also be
diagnosed in adulthood.
Although it does not appear to be inherited, recent reports implicate
genetic mutations in the development of Coats’ disease. Cremers and asso-
ciates found that 55% of cases with retinitis pigmentosa and Coats’-like
exudative vasculopathy contained a mutation in the CRB1 gene.2,3 Several
reports implicate a deficiency of norrin, a retinal protein, in the pathogen-
esis of Coats’ disease.4,5 Analysis of archival tissue from nine enucleated
eyes from males with unilateral Coats’ disease revealed a mutation in the
NDP gene on chromosome Xp11.2 in one subject. Berger and cowork-
ers6,7 developed a mutant mouse line with the Norrie’s disease model and
demonstrated abnormalities of the retinal vessels, including telangiecta-
sia, bulb-like dilatations, and underdevelopment of the capillary bed. He
562 and colleagues reported elevated intraocular levels of vascular endothelial Fig. 6.25.1  Coats’ Disease. Note the typical vascular abnormalities with aneurysmal
growth factor (VEGF) in four eyes with Coats’ disease.8 dilatation, telangiectasia, exudation, and severe lipid deposition in the macula.

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 6.25

Coats’ Disease and Retinal Telangiectasia

Fig. 6.25.2  Vessels May Appear Sheathed, Dilated, and Telangiectatic or
Feature Grape-Like Bunches of Aneurysms. Vascular changes that are saccular Fig. 6.25.5  Fluorescein Angiography of Coats’ Disease. In this eye, extensive
and lightbulb shaped may be seen as well. vascular changes are seen to extend temporally from the macula, with zones
of telangiectasia and aneurysm formation adjacent to a large area of capillary
nonperfusion. Beading of the blood vessel walls and anomalous vascular
communicating channels are present.

cataract, rubeosis iridis, and neovascular glaucoma, with phthisis bulbi in

severe cases.11,12,13

DIAGNOSIS
In children, Coats’ disease is typically diagnosed as a result of the recogni-
tion of poor vision, strabismus, or leukokoria. In patients with leukokoria,
a white pupillary reflex on photographs may be the initially noted abnor-
mality. In these cases, the disease is usually advanced, with extensive lipid
deposition and retinal detachment (see Fig. 6.25.4). In adults, the most
common presenting complaint with Coats’ disease is poor vision.
In advanced cases of Coats’ disease, rubeosis iridis, angle-closure glau-
coma, and cataract may be present. The diagnosis is confirmed ophthalmo-
scopically when the typical vascular abnormalities are seen in association
with lipid deposition and subretinal exudate. The retinal vascular abnor-
malities occur in small clusters and include kinked, looped, tortuous, and
sheathed vessels of varied and irregular caliber.

Fig. 6.25.3  Long-Standing Submacular Exudate. This may stimulate ingrowth

ANCILLARY TESTING
of blood vessels or fibrous tissue, with retinal pigment epithelium migration and Fluorescein angiography is a useful tool for delineating the nature and
hyperplasia and the formation of fibrous scars. extent of the vascular abnormalities present in this disease. Most com-
monly, numerous areas of telangiectasia and micro- and macroaneurysm
formation are seen, with beading of blood vessel walls and anomalous
vascular communicating channels (Fig. 6.25.5). Early and persistent dye
leakage documents the source of exudation and hemorrhage.9,14,15 The
microvasculature may be diffusely absent, with areas of complete capillary
nonperfusion.
Optical coherence tomography (OCT) may also be a useful tool to
monitor macular edema associated with Coats’ disease. When present,
abnormal macular thickness on OCT can be quantitatively determined
before and after treatment with laser or pharmacological therapy to gauge
the response to a particular therapy. OCT angiography reveals an abnormal
foveal avascular zone in the superficial capillary plexus.16

DIFFERENTIAL DIAGNOSIS
The severe juvenile form of Coats’ disease, which presents with exudative
retinal detachment, must be differentiated from other diseases that cause
leukokoria in childhood, including retinoblastoma, retinopathy of prema-
turity, retinal detachment, persistent hyperplastic primary vitreous, con-
genital cataract, toxocariasis, incontinentia pigmenti, Norrie’s disease, and
familial exudative vitreoretinopathy. Gass9 has pointed out that telangiec-
tatic vessels may appear on the surface of both retinoblastomas and Coats’
disease lesions. In retinoblastoma, these dilated vessels are continuous
Fig. 6.25.4  In Children, Coats’ Disease May Present as Leukokoria With with the large vascular trunks that extend into the tumor; in Coats’ disease,
Advanced Lipid Deposition and Exudative Retinal Detachment. In this eye, the the dilated vessels do not extend into the subretinal mass.9
anterior chamber is shallowed slightly, and the retina is immediately behind the Ultrasonography is a convenient, noninvasive test that may distin- 563
lens. guish between Coats’ disease and retinoblastoma and other entities. The

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 retinal detachment in Coats’ disease typically is exudative, with an absence

6 of the calcifications seen in retinoblastoma. Computed tomography (CT)

may help characterize intraocular morphology, quantify subretinal densi-
ties, detect calcium, and identify vascularity within the subretinal space
through the use of contrast enhancement. Also, CT may help detect other
Retina and Vitreous

abnormalities within the orbit or intracranial space. Examination of sub-

retinal fluid is used rarely, but it confirms the diagnosis of Coats’ disease
on the basis of cholesterol crystals and pigment-laden macrophages in the
absence of tumor cells.11
Less severe stages of Coats’ disease, especially in adults, must be differ-
entiated from other disorders that produce vascular changes and exudation.
These include inflammatory disorders such as Eales’ disease, vasculitis,
and collagen vascular disease. Tumors accompanied by exudation may
mimic Coats’ disease, as may diabetic vasculopathy with lipid deposition,
branch retinal vein occlusion with vascular remodeling and edema, rheg-
matogenous retinal detachment, radiation retinopathy, idiopathic juxtafo-
veal telangiectasia, von Hippel disease, angiomatosis of retina, exophytic
capillary hemangioma, and sickle cell retinopathy. In these cases, a thor-
ough review of the systems and medical and family histories usually help
differentiate primary from secondary disorders.11,9,10
Fig. 6.25.6  Bilateral Idiopathic Parafoveal Telangiectasia Can Often Best
Be Demonstrated With Red-Free Photography. This eye features capillary
Idiopathic Juxtafoveal Retinal Telangiectasia abnormalities present for virtually 360° in the parafoveal area. Early transit of the eye
Idiopathic juxtafoveal retinal telangiectasia is a group of disorders initially demonstrates a plexus of capillary abnormalities ringing the fovea.
described by Gass and Oyakawa17 in 1982. The disease is characterized by
onset in adulthood and presentation with mild blurring of central acuity
caused by exudate from ectatic retinal capillaries in the juxtafoveal region
of one or both eyes. They divided these patients into four categories:
groups 1A, 1B, 2, and 3.17,9

Group 1A
Group 1A disease consists of unilateral congenital parafoveal telangiecta-
sia, which typically occurs in men and affects only one eye. Retinal vas-
cular abnormalities are present in a small area, one to two disc areas in
diameter, in the temporal half of the macula. Onset of symptoms—with
visual loss in the 20/40 (6/12) or better range—typically develops at a mean Fig. 6.25.7  Optical Coherence Tomography Findings in Idiopathic Parafoveal
age of 40 years. Photocoagulation of areas of leakage may help restore Telangiectasia.
acuity.
Yannuzzi et al. proposed an updated classification.18 Broadly classified
Group 1B into Type 1, or unilateral aneurysmal dilation predominantly in men, and
Group 1B disease consists of unilateral idiopathic parafoveal telangiectasia, Type 2 perifoveal telangiectasia, bilateral telangiectasia limited to the per-
usually found in middle-aged men who have blurring caused by a tiny ifoveal area without visible aneurysms but associated with subretinal neo-
area of capillary telangiectasia confined to one clock hour at the edge of vascularization, readily identifiable on OCT angiography.19
the foveal avascular zone. Vision is usually 20/25 (6/7) or better. Photoco-
agulation usually is not considered for these eyes because of the proximity The MacTel Project
of the leakage to the fovea and the good prognosis without treatment. The
lesion may be acquired or may simply be a very small focus of congenital An international research collaboration comprising more than 30 centers,
telangiectasia. the MacTel Project, was initiated in 2005 to better understand disease
pathogenesis, epidemiology, and potential treatments of idiopathic macular
Group 2 telangiectasia type 2. The MacTel Project found a prevalence of diabetes
Group 2 disease consists of bilateral, acquired, idiopathic parafoveal telan- mellitus of 28% and hypertension of 52% in MacTel type 2, suggesting
giectasia. This variant affects patients in the fifth and sixth decades; mild a vasculopathic etiology of the disease.20 The project has also identified
blurring of vision occurs in one or both eyes. The patients typically have a genetic susceptibility locus for the disease using gene mapping, but
small, symmetrical areas of capillary dilatation, usually the size of one disc precise genetics of the disease remains unknown.21 Given the potential for
area or less, in both eyes. The vascular changes may be temporal only or neurodegenerative etiology for type 2 macular telangiectasia, the MacTel
may include all or part of the parafoveolar nasal retina too. No lipid is Project is investigating the benefits of intraocular delivery of ciliary neuro-
deposited, and minimal serous exudation is present. A hallmark is the trophic factor as a treatment for this disease.22
characteristic gray appearance of the lesions on biomicroscopic examina-
tion with occasional glistening white dots in the superficial retina. Red-free
photography often highlights these findings best (Figs. 6.25.6 and 6.25.7). Potential Treatments for Idiopathic Juxtafoveal
These patients also commonly have right-angled retinal venules that drain Retinal Telangiectasia
the capillary abnormalities and are present in the deep or outer retinal
layers. Retinal pigment epithelial hyperplasia eventually tends to develop Several treatment modalities have been explored for group 2 parafoveal
along these venules. In these patients, slow loss of visual acuity over many telangiectasia. Laser photocoagulation to leaking parafoveal vessels has not
years is produced by atrophy of the central fovea; patients also may develop been demonstrated to stop the vascular leakage or improve visual acuity.23
subretinal neovascularization, hemorrhagic macular detachment, and reti- Although intravitreal triamcinolone has been shown to reduce retinal
nochoroidal anastomosis. leakage, visual acuity outcomes are variable, and corticosteroid-related
adverse events may be limitations with longer follow-up duration.24 Ret-
Group 3 rospective and prospective studies of intravitreal injections of anti-VEGF
Bilateral idiopathic perifoveal telangiectasia with capillary occlusion is a agents such as bevacizumab and ranibizumab have resulted in decreased
rare variant in which adults experience loss of vision because of progres- retinal leakage but not improved vision.25–30 Therefore VEGF inhibition is
sive obliteration of the capillary network, which begins with telangiecta- not routinely recommended for parafoveal telangiectasia without second-
sia. The capillaries’ aneurysmal malformations are more marked than ary choroidal neovascularization. If choroidal neovascularization develops,
564 in the other, milder forms of the disease; no leakage occurs from the intravitreal anti-VEGF therapy is the most effective therapy available to
capillary bed. regress the neovascular tissue.26

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 6.25

Coats’ Disease and Retinal Telangiectasia

Fig. 6.25.8  Initial Photocoagulation of the Eye Shown in Fig. 6.25.5. Large, Fig. 6.25.9  Technique Used for Drainage of Subretinal Fluid in Eyes With
medium-intensity spots have been placed on leaking aneurysms, sparing the Extensive Exudative Detachment. The pediatric infusion cannula is sutured into
foveal avascular zone at first. More peripherally, photocoagulation covers temporal the anterior chamber through a limbal stab incision with a single Vicryl suture. This
aneurysms and is also placed in a scatter pattern in zones of nonperfusion. is placed in a convenient quadrant so that the eye can be rotated and a posterior
draining sclerotomy fashioned. The infusion runs into the anterior chamber and
around intact lens zonules, keeping the eye formed as voluminous quantities
of thick, yellow subretinal fluid are drained; this subretinal fluid is speckled with
SYSTEMIC ASSOCIATIONS cholesterol and lipid deposits.
Isolated case reports have described a number of other diseases that
occurred simultaneously in patients with Coats’ disease. In many cases,
it is doubtful that an actual causal association exists. Gass9 described a a series of 13 children who had blind eyes and bullous exudative detach-
patient who had a facial angioma and typical retinal telangiectasia and ments followed either after no treatment or after surgery that involved
another who had bilateral retinal disease and progressive facial hemiatro- intraocular infusion, drainage of subretinal fluid, and cryotherapy on one
phy. Bilateral telangiectasia and Coats’ syndrome have been reported in or more occasions. Of the six untreated eyes, four developed painful neo-
multiple family members who have facioscapulohumeral muscular dystro- vascular glaucoma and underwent enucleation. The seven treated with
phy and deafness. No definite connection has been made between other surgery all remained cosmetically acceptable and comfortable; none devel-
systemic or ocular conditions and Coats’ disease. The adult form of the oped neovascular glaucoma.
disease has been described as frequently associated with hypercholes- In selected cases of Coats’ disease with intravitreal proliferation and
terolemia, although such an association does not occur in the juvenile traction detachment, vitreous surgery may improve the clinical course.
form.31,32 Machemer and Williams36 reported successful results with surgical removal
of vitreal and preretinal membranes and destruction of leaking vessels in
TREATMENT a small series of patients.
Repeated therapeutic laser or cryotherapy treatments are typically
The major goal of treatment in Coats’ disease is to preserve or improve required in eyes that have Coats’ disease. Exudate typically begins to resorb
visual acuity or, when this is impossible, to preserve the anatomical integ- within six weeks of treatment if the abnormal vasculature has been elim-
rity of the eye. Intervention is contemplated when exudation is central inated. Depending on the amount of lipid accumulation, in many cases,
and/or extensive and progressive. In severe, untreated cases, total retinal it takes months to more than a year for complete resolution. Recurrence
detachment, iris neovascularization with glaucoma, and phthisis bulbi can of exudate after initially successful treatment signals the development of
result. Treatment of Coats’ disease is directed toward closure of the abnor- new abnormal leaking vessels; these must be searched out meticulously.
mal, leaking retinal vessels to allow resorption of exudate. In many cases, Contact lens biomicroscopy with a three-mirror lens sometimes is a useful
the visual results are poor even with successful treatment, especially when adjunct to indirect ophthalmoscopy in these cases, as is wide field fluores-
the macula is involved initially in the exudative process.12,31,33–35 cein angiography. Recurrences may occur years after initially successful
Laser photocoagulation is the treatment of choice in mild to moderate treatment, so it is particularly important to follow juvenile patients who
cases of exudation from Coats’ disease. Fluorescein angiographic guidance may develop significant problems if not followed carefully. Egerer et al.31
allows precise, localized treatment of the leaking aneurysms and vessels. recommended that all patients who have Coats’ disease should be exam-
Lesions that leak are treated directly with relatively large (200–500 µm) ined at least twice a year to catch any early recurrent problems that may
applications of moderate-intensity light (Fig. 6.25.8).11 Scatter photo- develop in a small percentage of these patients.
coagulation to areas of extensive nonperfusion are of unproven value but Intravitreal triamcinolone has been reported to give short-term benefit
may lessen the chance of secondary neovascularization. Peripheral lesions in select cases of Coats’ disease. Othman and colleagues combined intrav-
may be treated with the indirect laser, which may need to be done under itreal triamcinolone with either laser photocoagulation or cryotherapy in 15
general anesthesia in children. eyes and found that treatment resulted in reabsorption of subretinal fluid
Where subretinal fluid is present, cryotherapy, as opposed to laser, to and macular exudates.37 Other published reports demonstrate improve-
the anomalous vessels is recommended using a single freeze or freeze– ment in visual acuity and decrease in retinal thickness after intravitreal
refreeze technique. If the retina is highly elevated, it may be necessary triamcinolone.38 More recently, pharmacological therapy with intravitreal
to drain subretinal fluid to flatten the retina and allow sufficient freeze inhibitors of VEGF have been reported to have biological activity in Coats’
to reach the retinal vessels. In these cases, the retina is flattened, the eye disease.39–43 Ramasubramanian and Shields reported a retrospective analy-
reformed, and cryotherapy or laser applied (Fig. 6.25.9) (Video 6.25.1). Sub- sis of eight patients with Coats’ disease with partial or near total exudative
See clip:
6.25.1 retinal pigmentation and fibrosis usually ensue and follow the lipid reso- retinal detachment.40 Treatment with laser photocoagulation and/or cryo-
lution. If this involves the macula, visual return is commensurately poor.11 therapy plus additional intravitreal bevacizumab (1.25 mg/0.05 ml) with a
Another approach to eyes with significant retinal detachment is to mean follow-up period of 8.5 months resulted in resolution of retinopathy
perform a scleral buckling procedure.31 Harris34 reported that a scleral and subretinal fluid. Vitreous fibrosis in four patients at month five with
buckle sometimes aids the application of postoperative photocoagulation, a traction retinal detachment in three patients was possibly related to the
because it can be oriented beneath the abnormal vessels, and anomalies at use of intravitreal bevacizumab. Caution is advised when using anti-VEGF 565
the apex of the buckle can be treated effectively. Siliodor et al.33 reported therapy for patients with Coats’ disease.

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 COMPLICATIONS OF TREATMENT Visual acuity results may be quite good in patients who have very mild

6 Complications of photocoagulation and cryotherapy for Coats’ disease

include inflammation, hemorrhage, chorioretinal anastomosis forma-
vascular anomalies that do not require treatment or are discovered and
treated before the macula is involved by the exudative process. It is dif-
ficult to estimate the frequency with which this situation develops in the
tion, and retinal, chorioretinal, and subretinal fibrosis. Macular distortion general population, because reported series discuss only more severe cases
Retina and Vitreous

secondary to epiretinal membrane formation and contraction has been referred to tertiary treatment centers, and the disease is rare enough to
reported following photocoagulation for Coats’ disease and may occur even have avoided scrutiny in population-based studies.
if the disease is untreated. Gass9 reported one adult patient who developed Eyes with severe exudation and retinal detachment rarely retain vision
total retinal detachment and proliferative vitreoretinopathy after cryother- better than 20/400 (6/120), and many see much worse than this. Neverthe-
apy for peripheral retinal telangiectasia that was discovered late in life; the less, successful treatment of leaking vascular channels may salvage some
eye initially had 20/20 (6/6) acuity. vision, and this has the advantage of stabilizing the eye anatomically. Occa-
With intraocular surgical intervention, additional risks include cataract sionally an eye may be saved structurally without light perception.
formation, choroidal hemorrhage, retinal detachment, endophthalmitis, The prognosis for retaining anatomical integrity of the globe is much
glaucoma, and phthisis. better. Most eyes with Coats’ disease, however, can be saved, be cosmeti-
Intravitreal triamcinolone has been associated with elevated intraocular cally acceptable, grow and develop otherwise normally, and in many cases
pressure and cataract progression. Anti-VEGF agents have been associ- have useful vision. Amblyopia therapy, strabismus surgery, and other types
ated with retinal fibrosis evolving into traction retinal detachment in case of ancillary rehabilitation may be useful and should not be neglected as
reports.40 Careful monitoring of patients after administration of pharmaco- part of the total treatment of these patients.
logical therapies is advised.
KEY REFERENCES
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angiectasis) caused by somatic mutation in the NDP gene: a role for norrin in retinal
The clinical course in Coats’ disease is variable, but it is usually progres- angiogenesis. Hum Mol Genet 1999;8:2031–5.
sive. Continued exudation from abnormal vascular channels produces a Charbel Issa P, Finger RP, Kruse K, et al. Monthly ranibizumab for nonproliferative macular
gradual accumulation of lipid and serous retinal detachment. The downhill telangiectasia type 2: a 12-month prospective study. Am J Ophthalmol 2011;151(5):876–86.
course is more rapid in eyes with more extensive vascular abnormalities. Charbel Issa P, Holz FG, Scholl HPN. Intravitreal bevacizumab in type 2 idiopathic macular
telangiectasia. Ophthalmology 2007;114:1736–42.
Acute exacerbations of the disease may occur with intervening periods of Coats G. Forms of retinal dysplasia with massive exudation. Royal London Ophthalmol Hosp
relative stability. The end stage of the exudative process, seen in eyes with Rep 1908;17:440–525.
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onset of symptoms, is total retinal detachment, which may be followed by 1982;100:769–80.
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disease and possible therapeutic role of bevacizumab. Graefes Arch Clin Exp Ophthal-
Shields and colleagues conducted a large retrospective review of 150 mol 2010;248:1519–21.
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because the disease is not diagnosed and treated until after significant
macular lipid deposition is present. Even with good treatment and reso-
lution of the macular deposits, significant subretinal fibrosis and macular
impairment are present.

566

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For personal use only. No other uses without permission. Copyright ©2019. Elsevier Inc. All rights reserved.
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