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.

Downloaded for Budi Lakukua (googledotbudi@yahoo.co.id) at Tarumanagara University from ClinicalKey.com by Elsevier on August 12, 2019.
For personal use only. No other uses without permission. Copyright ©2019. Elsevier Inc. All rights reserved.

Fig. 6.25.2  Vessels May Appear Sheathed, Dilated, and Telangiectatic or
Feature Grape-Like Bunches of Aneurysms. Vascular changes that are saccular
and lightbulb shaped may be seen as well.
Fig. 6.25.3  Long-Standing Submacular Exudate. This may stimulate ingrowth
of blood vessels or fibrous tissue, with retinal pigment epithelium migration and
hyperplasia and the formation of fibrous scars.
Fig. 6.25.4  In Children, Coats’ Disease May Present as Leukokoria With
Advanced Lipid Deposition and Exudative Retinal Detachment. In this eye, the
anterior chamber is shallowed slightly, and the retina is immediately behind the
lens.
Downloaded for Budi Lakukua (googledotbudi@yahoo.co.id) at Tarumanagara University from ClinicalKey.com by Elsevier on August 12, 2019.
For personal use only. No other uses without permission. Copyright ©2019. Elsevier Inc. All rights reserved.
6.25
Coats’ Disease and Retinal Telangiectasia
Fig. 6.25.5  Fluorescein Angiography of Coats’ Disease. In this eye, extensive
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.
ANCILLARY TESTING
Fluorescein angiography is a useful tool for delineating the nature and
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
with the large vascular trunks that extend into the tumor; in Coats’ disease,
the dilated vessels do not extend into the subretinal mass.9
Ultrasonography is a convenient, noninvasive test that may distin- 563
guish between Coats’ disease and retinoblastoma and other entities. The
 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
age of 40 years. Photocoagulation of areas of leakage may help restore
acuity.
Group 1B
Group 1B disease consists of unilateral idiopathic parafoveal telangiectasia,
usually found in middle-aged men who have blurring caused by a tiny
area of capillary telangiectasia confined to one clock hour at the edge of
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
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
telangiectasia.
Group 2
Group 2 disease consists of bilateral, acquired, idiopathic parafoveal telan-
giectasia. This variant affects patients in the fifth and sixth decades; mild
blurring of vision occurs in one or both eyes. The patients typically have
small, symmetrical areas of capillary dilatation, usually the size of one disc
area or less, in both eyes. The vascular changes may be temporal only or
may include all or part of the parafoveolar nasal retina too. No lipid is
deposited, and minimal serous exudation is present. A hallmark is the
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).
These patients also commonly have right-angled retinal venules that drain
the capillary abnormalities and are present in the deep or outer retinal
layers. Retinal pigment epithelial hyperplasia eventually tends to develop
along these venules. In these patients, slow loss of visual acuity over many
years is produced by atrophy of the central fovea; patients also may develop
subretinal neovascularization, hemorrhagic macular detachment, and reti-
nochoroidal anastomosis.
Group 3
Bilateral idiopathic perifoveal telangiectasia with capillary occlusion is a
rare variant in which adults experience loss of vision because of progres-
sive obliteration of the capillary network, which begins with telangiecta-
sia. The capillaries’ aneurysmal malformations are more marked than
564 in the other, milder forms of the disease; no leakage occurs from the
capillary bed.
Fig. 6.25.7  Optical Coherence Tomography Findings in Idiopathic Parafoveal
Telangiectasia.
Yannuzzi et al. proposed an updated classification.18 Broadly classified
into Type 1, or unilateral aneurysmal dilation predominantly in men, and
Type 2 perifoveal telangiectasia, bilateral telangiectasia limited to the per-
ifoveal area without visible aneurysms but associated with subretinal neo-
vascularization, readily identifiable on OCT angiography.19
The MacTel Project
An international research collaboration comprising more than 30 centers,
the MacTel Project, was initiated in 2005 to better understand disease
pathogenesis, epidemiology, and potential treatments of idiopathic macular
telangiectasia type 2. The MacTel Project found a prevalence of diabetes
mellitus of 28% and hypertension of 52% in MacTel type 2, suggesting
a vasculopathic etiology of the disease.20 The project has also identified
a genetic susceptibility locus for the disease using gene mapping, but
precise genetics of the disease remains unknown.21 Given the potential for
neurodegenerative etiology for type 2 macular telangiectasia, the MacTel
Project is investigating the benefits of intraocular delivery of ciliary neuro-
trophic factor as a treatment for this disease.22
Potential Treatments for Idiopathic Juxtafoveal
Retinal Telangiectasia
Several treatment modalities have been explored for group 2 parafoveal
telangiectasia. Laser photocoagulation to leaking parafoveal vessels has not
been demonstrated to stop the vascular leakage or improve visual acuity.23
Although intravitreal triamcinolone has been shown to reduce retinal
leakage, visual acuity outcomes are variable, and corticosteroid-related
adverse events may be limitations with longer follow-up duration.24 Ret-
rospective and prospective studies of intravitreal injections of anti-VEGF
agents such as bevacizumab and ranibizumab have resulted in decreased
retinal leakage but not improved vision.25–30 Therefore VEGF inhibition is
not routinely recommended for parafoveal telangiectasia without second-
ary choroidal neovascularization. If choroidal neovascularization develops,
intravitreal anti-VEGF therapy is the most effective therapy available to
regress the neovascular tissue.26

Downloaded for Budi Lakukua (googledotbudi@yahoo.co.id) at Tarumanagara University from ClinicalKey.com by Elsevier on August 12, 2019.
For personal use only. No other uses without permission. Copyright ©2019. Elsevier Inc. All rights reserved.

Fig. 6.25.8  Initial Photocoagulation of the Eye Shown in Fig. 6.25.5. Large,
medium-intensity spots have been placed on leaking aneurysms, sparing the
foveal avascular zone at first. More peripherally, photocoagulation covers temporal
aneurysms and is also placed in a scatter pattern in zones of nonperfusion.
SYSTEMIC ASSOCIATIONS
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
patient who had a facial angioma and typical retinal telangiectasia and
another who had bilateral retinal disease and progressive facial hemiatro-
phy. Bilateral telangiectasia and Coats’ syndrome have been reported in
multiple family members who have facioscapulohumeral muscular dystro-
phy and deafness. No definite connection has been made between other
systemic or ocular conditions and Coats’ disease. The adult form of the
disease has been described as frequently associated with hypercholes-
terolemia, although such an association does not occur in the juvenile
form.31,32
TREATMENT
The major goal of treatment in Coats’ disease is to preserve or improve
visual acuity or, when this is impossible, to preserve the anatomical integ-
rity of the eye. Intervention is contemplated when exudation is central
and/or extensive and progressive. In severe, untreated cases, total retinal
detachment, iris neovascularization with glaucoma, and phthisis bulbi can
result. Treatment of Coats’ disease is directed toward closure of the abnor-
mal, leaking retinal vessels to allow resorption of exudate. In many cases,
the visual results are poor even with successful treatment, especially when
the macula is involved initially in the exudative process.12,31,33–35
Laser photocoagulation is the treatment of choice in mild to moderate
cases of exudation from Coats’ disease. Fluorescein angiographic guidance
allows precise, localized treatment of the leaking aneurysms and vessels.
Lesions that leak are treated directly with relatively large (200–500 µm)
applications of moderate-intensity light (Fig. 6.25.8).11 Scatter photo-
coagulation to areas of extensive nonperfusion are of unproven value but
may lessen the chance of secondary neovascularization. Peripheral lesions
may be treated with the indirect laser, which may need to be done under
general anesthesia in children.
Where subretinal fluid is present, cryotherapy, as opposed to laser, to
the anomalous vessels is recommended using a single freeze or freeze–
refreeze technique. If the retina is highly elevated, it may be necessary
to drain subretinal fluid to flatten the retina and allow sufficient freeze
to reach the retinal vessels. In these cases, the retina is flattened, the eye
reformed, and cryotherapy or laser applied (Fig. 6.25.9) (Video 6.25.1). Sub-
See clip:
6.25.1 retinal pigmentation and fibrosis usually ensue and follow the lipid reso-
lution. If this involves the macula, visual return is commensurately poor.11
Another approach to eyes with significant retinal detachment is to
perform a scleral buckling procedure.31 Harris34 reported that a scleral
buckle sometimes aids the application of postoperative photocoagulation,
because it can be oriented beneath the abnormal vessels, and anomalies at
the apex of the buckle can be treated effectively. Siliodor et al.33 reported
Downloaded for Budi Lakukua (googledotbudi@yahoo.co.id) at Tarumanagara University from ClinicalKey.com by Elsevier on August 12, 2019.
For personal use only. No other uses without permission. Copyright ©2019. Elsevier Inc. All rights reserved.
6.25
Coats’ Disease and Retinal Telangiectasia
Fig. 6.25.9  Technique Used for Drainage of Subretinal Fluid in Eyes With
Extensive Exudative Detachment. The pediatric infusion cannula is sutured into
the anterior chamber through a limbal stab incision with a single Vicryl suture. This
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
cholesterol and lipid deposits.
a series of 13 children who had blind eyes and bullous exudative detach-
ments followed either after no treatment or after surgery that involved
intraocular infusion, drainage of subretinal fluid, and cryotherapy on one
or more occasions. Of the six untreated eyes, four developed painful neo-
vascular glaucoma and underwent enucleation. The seven treated with
surgery all remained cosmetically acceptable and comfortable; none devel-
oped neovascular glaucoma.
In selected cases of Coats’ disease with intravitreal proliferation and
traction detachment, vitreous surgery may improve the clinical course.
Machemer and Williams36 reported successful results with surgical removal
of vitreal and preretinal membranes and destruction of leaking vessels in
a small series of patients.
Repeated therapeutic laser or cryotherapy treatments are typically
required in eyes that have Coats’ disease. Exudate typically begins to resorb
within six weeks of treatment if the abnormal vasculature has been elim-
inated. Depending on the amount of lipid accumulation, in many cases,
it takes months to more than a year for complete resolution. Recurrence
of exudate after initially successful treatment signals the development of
new abnormal leaking vessels; these must be searched out meticulously.
Contact lens biomicroscopy with a three-mirror lens sometimes is a useful
adjunct to indirect ophthalmoscopy in these cases, as is wide field fluores-
cein angiography. Recurrences may occur years after initially successful
treatment, so it is particularly important to follow juvenile patients who
may develop significant problems if not followed carefully. Egerer et al.31
recommended that all patients who have Coats’ disease should be exam-
ined at least twice a year to catch any early recurrent problems that may
develop in a small percentage of these patients.
Intravitreal triamcinolone has been reported to give short-term benefit
in select cases of Coats’ disease. Othman and colleagues combined intrav-
itreal triamcinolone with either laser photocoagulation or cryotherapy in 15
eyes and found that treatment resulted in reabsorption of subretinal fluid
and macular exudates.37 Other published reports demonstrate improve-
ment in visual acuity and decrease in retinal thickness after intravitreal
triamcinolone.38 More recently, pharmacological therapy with intravitreal
inhibitors of VEGF have been reported to have biological activity in Coats’
disease.39–43 Ramasubramanian and Shields reported a retrospective analy-
sis of eight patients with Coats’ disease with partial or near total exudative
retinal detachment.40 Treatment with laser photocoagulation and/or cryo-
therapy plus additional intravitreal bevacizumab (1.25 mg/0.05 ml) with a
mean follow-up period of 8.5 months resulted in resolution of retinopathy
and subretinal fluid. Vitreous fibrosis in four patients at month five with
a traction retinal detachment in three patients was possibly related to the
use of intravitreal bevacizumab. Caution is advised when using anti-VEGF 565
therapy for patients with Coats’ disease.
 COMPLICATIONS OF TREATMENT
6 Complications of photocoagulation and cryotherapy for Coats’ disease
include inflammation, hemorrhage, chorioretinal anastomosis forma-
tion, and retinal, chorioretinal, and subretinal fibrosis. Macular distortion
Retina and Vitreous
secondary to epiretinal membrane formation and contraction has been
reported following photocoagulation for Coats’ disease and may occur even
if the disease is untreated. Gass9 reported one adult patient who developed
total retinal detachment and proliferative vitreoretinopathy after cryother-
apy for peripheral retinal telangiectasia that was discovered late in life; the
eye initially had 20/20 (6/6) acuity.
With intraocular surgical intervention, additional risks include cataract
formation, choroidal hemorrhage, retinal detachment, endophthalmitis,
glaucoma, and phthisis.
Intravitreal triamcinolone has been associated with elevated intraocular
pressure and cataract progression. Anti-VEGF agents have been associ-
ated with retinal fibrosis evolving into traction retinal detachment in case
reports.40 Careful monitoring of patients after administration of pharmaco-
logical therapies is advised.
COURSE AND OUTCOME
The clinical course in Coats’ disease is variable, but it is usually progres-
sive. Continued exudation from abnormal vascular channels produces a
gradual accumulation of lipid and serous retinal detachment. The downhill
course is more rapid in eyes with more extensive vascular abnormalities.
Acute exacerbations of the disease may occur with intervening periods of
relative stability. The end stage of the exudative process, seen in eyes with
severe Coats’ disease and particularly in young patients who have an early
onset of symptoms, is total retinal detachment, which may be followed by
rubeosis iridis, neovascular glaucoma, and eventually phthisis bulbi.
Shields and colleagues conducted a large retrospective review of 150
patients with Coats’ disease to determine risk factors for poor visual
outcome and enucleation.44 Risk factors predictive of poor visual outcome
(20/200 or worse) were postequatorial, diffuse, or superior location of the
telangiectasis and exudation, failed resolution of subretinal fluid after treat-
ment, and presence of retinal macrocysts. Significant risk factors for enu-
cleation included elevated intraocular pressure and iris neovascularization.
The ultimate prognosis for eyes with Coats’ disease can be measured
in terms of two endpoints: visual acuity and anatomical stability. Unfortu-
nately, central visual acuity is frequently poor in eyes with Coats’ disease,
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
Downloaded for Budi Lakukua (googledotbudi@yahoo.co.id) at Tarumanagara University from ClinicalKey.com by Elsevier on August 12, 2019.
For personal use only. No other uses without permission. Copyright ©2019. Elsevier Inc. All rights reserved.
Visual acuity results may be quite good in patients who have very mild
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
general population, because reported series discuss only more severe cases
referred to tertiary treatment centers, and the disease is rare enough to
have avoided scrutiny in population-based studies.
Eyes with severe exudation and retinal detachment rarely retain vision
better than 20/400 (6/120), and many see much worse than this. Neverthe-
less, successful treatment of leaking vascular channels may salvage some
vision, and this has the advantage of stabilizing the eye anatomically. Occa-
sionally an eye may be saved structurally without light perception.
The prognosis for retaining anatomical integrity of the globe is much
better. Most eyes with Coats’ disease, however, can be saved, be cosmeti-
cally acceptable, grow and develop otherwise normally, and in many cases
have useful vision. Amblyopia therapy, strabismus surgery, and other types
of ancillary rehabilitation may be useful and should not be neglected as
part of the total treatment of these patients.
KEY REFERENCES
Black GC, Perveen R, Bonshek R, et al. Coats’ disease of the retina (unilateral retinal tel-
angiectasis) caused by somatic mutation in the NDP gene: a role for norrin in retinal
angiogenesis. Hum Mol Genet 1999;8:2031–5.
Charbel Issa P, Finger RP, Kruse K, et al. Monthly ranibizumab for nonproliferative macular
telangiectasia type 2: a 12-month prospective study. Am J Ophthalmol 2011;151(5):876–86.
Charbel Issa P, Holz FG, Scholl HPN. Intravitreal bevacizumab in type 2 idiopathic macular
telangiectasia. Ophthalmology 2007;114:1736–42.
Coats G. Forms of retinal dysplasia with massive exudation. Royal London Ophthalmol Hosp
Rep 1908;17:440–525.
Gass JDM, Oyakawa RT. Idiopathic juxtafoveal retinal telangiectasis. Arch Ophthalmol
1982;100:769–80.
He YG, Wang H, Zhao B, et al. Elevated vascular endothelial growth factor level in Coats’
disease and possible therapeutic role of bevacizumab. Graefes Arch Clin Exp Ophthal-
mol 2010;248:1519–21.
Kovach JL, Rosenfeld PJ. Bevacizumab (avastin) therapy for idiopathic macular telangiectasia
type II. Retina 2009;29(1):27–32.
Machemer R, Williams JH Sr. Pathogenesis and therapy of traction detachments in various
retinal vascular diseases. Am J Ophthalmol 1988;105:173–81.
Ridley ME, Shields JA, Brown GC, et al. Coats’ disease. Evaluation of management. Ophthal-
mology 1982;89:1381–7.
Shields JA, Shields CL, Honavar SG, et al. Classification and management of Coats disease:
the 2000 Proctor Lecture. Am J Ophthalmol 2001;131:572–83.
Access the complete reference list online at ExpertConsult.com
REFERENCES
1. Coats G. Forms of retinal dysplasia with massive exudation. Royal London Ophthalmol
Hosp Rep 1908;17:440–525.
2. Cremers FP, Maugeri A, den Hollander AI, et al. The expanding roles of ABCA4 and
CRB1 in inherited blindness. Novartis Found Symp 2004;255:68–79.
3. Zhao M, Andrieu-Soler C, Kowalczuk L, et al. A new CRB1 rat mutation links Muller
glial cells to retinal telangiectasia. J Neurosci 2015;35(15):6093–106.
4. Black GC, Perveen R, Bonshek R, et al. Coats’ disease of the retina (unilateral retinal
telangiectasis) caused by somatic mutation in the NDP gene: a role for norrin in retinal
angiogenesis. Hum Mol Genet 1999;8:2031–5.
5. Shastry BS, Trese MT. Overproduction and partial purification of the Norrie disease
gene product, norrin, from a recombinant baculovirus. Biochem Biophys Res Commun
2003;312:229–34.
6. Berger W, van de Pol D, Bachner D, et al. An animal model for Norrie disease (ND): gene
targeting of the mouse ND gene. Hum Mol Genet 1996;5:51–9.
7. Luhmann UF, Lin J, Acar N, et al. Role of the Norrie disease pseudoglioma gene in
sprouting angiogenesis during development of the retinal vasculature. Invest Ophthal-
mol Vis Sci 2005;46:3372–82.
8. He YG, Wang H, Zhao B, et al. Elevated vascular endothelial growth factor level in Coats’
disease and possible therapeutic role of bevacizumab. Graefes Arch Clin Exp Ophthalmol
2010;248:1519–21.
9. Gass JDM. Stereoscopic atlas of macular diseases. St Louis: CV Mosby; 1987. p. 384–9.
10. Reese AB. Telangiectasis of the retina and Coats’ disease. Am J Ophthalmol 1956;42:1–8.
11. Smithen LM, Brown GC, Brucker AJ, et al. Coats disease diagnosed in adulthood. Oph-
thalmology 2005;112:1072–8.
12. Gomez Morales A. Coats’ disease. Natural history and results of treatment. Am J Oph-
thalmol 1965;60:855–65.
13. Tarkkanen A, Laatikainen L. Coats’ disease: clinical angiographic, histopathological find-
ings and clinical management. Br J Ophthalmol 1983;67:766–76.
14. Yannuzzi LA, Gitter KA, Schatz H. The macula: a comprehensive text and atlas. Balti-
more: Williams & Wilkins; 1979. p. 118–26.
15. Theodossiadis GP. Some clinical, fluorescein-angiographic, and therapeutic aspects of
Coats’ disease. J Pediatr Ophthalmol Strabismus 1979;16:257–62.
16. Muakkassa NW, de Carlo TE, Choudhry N, et al. Optical coherence tomography angiogra-
phy findings in Coats disease. Ophthalmic Surg Lasers Imaging Retina 2016;47(7):632–5.
17. Gass JDM, Oyakawa RT. Idiopathic juxtafoveal retinal telangiectasis. Arch Ophthalmol
1982;100:769–80.
18. Yannuzzi LA1, Bardal AM, Freund KB, et al. Idiopathic macular telangiectasia. Arch
Ophthalmol 2006;124(4):450–60.
19. Zhang Q, Wang RK, Chen CL, et al. Swept source optical coherence tomography angiog-
raphy of neovascular macular telangiectasia type 2. Retina 2015;35(11):2285–99.
20. Clemons TE, Gillies MC, Chew EY, et al; Macular Telangiectasia Project Research Group.
Medical characteristics of patients with macular telangiectasia type 2 (MacTel Type 2)
MacTel project report no. 3. Ophthalmic Epidemiol 2013;20(2):109–13.
21. Parmalee NL, Schubert C, Figueroa M, et al. Identification of a potential susceptibility
locus for macular telangiectasia type 2. PLoS ONE 2012;7(8):e24268.
Downloaded for Budi Lakukua (googledotbudi@yahoo.co.id) at Tarumanagara University from ClinicalKey.com by Elsevier on August 12, 2019.
For personal use only. No other uses without permission. Copyright ©2019. Elsevier Inc. All rights reserved.
22. Chew EY, Clemons TE, Peto T. Ciliary neurotrophic factor for macular telangiectasia type
2: results from a phase 1 safety trial. Am J Ophthalmol 2015;159(4):659–66.e1.
23. Park DW, Schatz H, McDonald HR, et al. Grid laser photocoagulation for macular edema
in bilateral juxtafoveal macular telangiectasis. Ophthalmology 1997;104:1838–46.
6.25
24. Martinez JA. Intravitreal triamcinolone acetonide for bilateral acquired parafoveal telang-
ectasis. Arch Ophthalmol 2003;121:1658–64.
Coats’ Disease and Retinal Telangiectasia
25. Charbel Issa P, Holz FG, Scholl HPN. Intravitreal bevacizumab in type 2 idiopathic
macular telangiectasia. Ophthalmology 2007;114:1736–42.
26. Roller AB, Folk JC, Patel NM, et al. Intravitreal bevacizumab for treatment of proliferative
and nonproliferative type 2 idiopathic macular telangiectasia. Retina 2011;31(9):1848–55.
27. Kovach JL, Rosenfeld PJ. Bevacizumab (avastin) therapy for idiopathic macular telangiec-
tasia type II. Retina 2009;29(1):27–32.
28. Matt G, Sacu S, Ahlers C, et al. Thirty-month follow-up after intravitreal bevacizumab in
progressive idiopathic macular telangiectasia type 2. Eye (Lond) 2010;24(10):1535–41.
29. Charbel Issa P, Finger RP, et al. Monthly ranibizumab for nonproliferative macular tel-
angiectasia type 2: a 12-month prospective study. Am J Ophthalmol 2011;151(5):876–86.
30. Toy BC, Koo E, Cukras C, et al. Treatment of non-neovascular idiopathic macular telan-
giectasia type 2 with intravitreal ranibizumab: results of a phase 2 clinical trial. Retina
2012;32:996–1006.
31. Egerer I, Tasman W, Tomer TL. Coats disease. Arch Ophthalmol 1974;92:109–12.
32. Woods AC, Duke J. Coats’ disease. 1. Review of the literature, diagnostic criteria, clinical
findings, and plasma lipid studies. Br J Ophthalmol 1963;47:385–412.
33. Siliodor SW, Augsburger JJ, Shields JA, et al. Natural history and management of
advanced Coats’ disease. Ophthalmic Surg 1988;19:89–93.
34. Harris GS. Coatss disease, diagnosis and treatment. Can J Ophthalmol 1970;5:311–20.
35. Ridley ME, Shields JA, Brown GC, et al. Coats’ disease. Evaluation of management. Oph-
thalmology 1982;89:1381–7.
36. Machemer R, Williams JH Sr. Pathogenesis and therapy of traction detachments in
various retinal vascular diseases. Am J Ophthalmol 1988;105:173–81.
37. Othman IS, Moussa M, Bouhaimed M. Management of lipid exudates in Coats disease
by adjuvant intravitreal triamcinolone: effects and complications. Br J Ophthalmol
2010;94:606–10.
38. Ghorbanian S, Jaulim A, Chatziralli IP. Diagnosis and treatment of Coats’ disease: a
review of the literature. Ophthalmologica 2012;227(4):175–82.
39. Lin CJ, Hwang JF, Chen YT, et al. The effect of intravitreal bevacizumab in the treatment
of Coats disease in children. Retina 2010;30:617–22.
40. Ramasubramanian A, Shields CL. Bevacizumab for Coats’ disease with exudative retinal
detachment and risk of vitreoretinal traction. Br J Ophthalmol 2012;96:356–9.
41. Cackett P, Wong D, Cheung CM. Combined intravitreal bevacizumab and argon laser
treatment for Coats’ disease. Acta Ophthalmol 2010;88:48–9.
42. Wells JR, Hubbard GB III. The effects of intravitreal bevacizumab in the treatment of
Coats disease in children. Retina 2011;31:427–8.
43. Bohm MR, Uhlig CE. Use of intravitreal triamcinolone and bevacizumab in Coats’ disease
with central macular edema. Graefes Arch Clin Exp Ophthalmol 2011;249:1099–101.
44. Shields JA, Shields CL, Honavar SG, et al. Classification and management of Coats
disease: the 2000 Proctor Lecture. Am J Ophthalmol 2001;131:572–83.
566.e1