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Diagnostic and TherapeuticChallenges Edited by H. Richard McDonaldDrs. Giuseppe Querques, Rosangela Lattanzio, Lea Querques, Francesco Bandello, and K. Bailey Freund

This case is submitted by Drs. GiuseppeQuerques,* Rosangela Lattanzio*, Lea Querques*,

and Francesco Bandello*, *Department of Ophthalmol-ogy, University Vita Salute San Raffaele, Milan, Italy;and Department of Ophthalmology, University ParisXII, Centre Hospitalier Intercommunal de Creteil Creteil,

France; commented by Dr. K. Bailey Freund, New York,New York.

Case Report

In April 2010, a 35-year-old man was referred to our department

with the diagnosis of choroidal neovascularization in his right eye

(RE). The medical history was unremarkable except for nephrec-

tomy 20 years before because of kidney atrophy. At presentation,the best-corrected visual acuity was 20/125 in RE, and 20/20 in the

left eye (LE). Anterior and posterior segment slit-lamp biomicro-

scopy of both eyes was unremarkable. Applanation tonometry

readings were 15 mmHg in both eyes. The vitreous cavities were

clear. Fundus examination of the RE showed retinal vascular

abnormalities with a prominent lesion along the inferotemporal

arcade determining intense macular exudation with retinal hemor-

rhages and hard exudates (Figure 1). Fundus examination of the LE

was not noteworthy.

Fluorescein angiography and indocyanine green angiography

revealed diffuse retinal microvascular abnormalities (microaneur-

ysms, saccular/fusiform vascular dilations, and capillary dropout)

and a retinalchoroidal anastomosis (RCA) along the inferotempo-

ral arcade of the RE and normal findings in the LE (Figure 1).

Spectral domain optical coherence tomography confirmed the

intense exudation associated with the retinal microvascular abnor-

malities and RCA involving the RE macula. Based on these find-

ings, we decided to treat the patient with intravitreal injection of

bevacizumab followed by laser photocoagulation to the area of

retinal microaneurysms, saccular/fusiform vascular dilations, capil-

lary dropout, and RCA along the inferotemporal arcade of the RE.

Three months after treatment, the patient recovered 20/25 best-

corrected visual acuity in his RE, and fundus examination, fluores-

cein angiography, and spectral domain optical coherence tomography

of the RE showed a regression of the microvascular abnormalities

and RCA along the inferotemporal arcade, with resolution of active

exudation but persistence of hard exudates (Figure 2).

Fifteen months later, the patient presented for regular follow-up

examination. Best-corrected visual acuity was 20/25 in the RE and

20/20 in the LE. Anterior and posterior segment slit-lamp biomicro-

scopy of both eyes was unremarkable. Fundus examination andfluorescein angiography showed a regression of the microvascular

abnormalities and RCA along the inferotemporal arcade, without

active exudation but persistence of hard exudates and normal findings

in the LE (Figure 3). Interestingly, a new prominent lesion associated

with retinal hemorrhages, exudation, and hard exudates developed

along the inferior arcade of the RE (Figure 3).

This case is presented for discussion of diagnosisand management.

Dr. K. Bailey Freund (New York,

New York):

Drs. Querques, Lattanzio, Querques, and Bandellohave shared with us an interesting case of a 35-year-old man with unilateral retinal vascular changes that

include capillary telangiectasia, aneurysms, and non-perfusion. Whereas other entities can produce thisconstellation of findings, Coats disease (also referredto as Type 1 idiopathic macular telangiectasia) would

seem to be the most likely diagnosis in this case.1

Asthe vascular abnormalities appear to be confined to the

inferotemporal quadrant, I would also consider ante-cedent branch retinal vein occlusion as a less likelyalternative diagnosis. However, I cannot identify a spe-cific arteriovenous crossing point that would accountfor the distribution of the vascular abnormalities in this

eye. Similar retinal vascular changes can occur in sev-eral rare retinal disorders including the ParryRombergsyndrome (progressive hemifacial atrophy), facioscapu-lohumeral muscular dystrophy, familial exudative vitre-oretinopathy, and dyskeratosis congenita.2,3,4 Thesediagnoses appear to be ruled out by the lack of support-

ive medical history and a unilateral presentation thatwould not be expected in the latter three entities.

Although the authors describe an RCA in this case,I am not fully convinced that the imaging supports this

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interpretation. Both the initial fluorescein angiography(Figure 1B) and initial indocyanine green angiogram(Figure 1C) show the abrupt termination of an infer-otemporal branch retinal artery with distal capillary

nonperfusion, suggestive of a branch retinal arteryocclusion. This occlusion may have occurred at the

site of a previous macroaneurysm that spontaneouslythrombosed. There is blockage by surrounding retinalhemorrhage that the authors may have interpreted asevidence of an RCA. With resolution of this hemor-

rhage, I can still visualize the abrupt termination ofthe artery (Figure 1) and distal ischemia, but I do not

Fig. 1. First presentation atour department (April 2010).Fundus color photograph ofthe RE (A) shows retinal vas-

cular abnormalities with

a prominent lesion along theinferotemporal arcade (arrow)determining intense macularexudation with retinal hemor-rhages and hard exudates.Fluorescein angiography (FA)

(B) and indocyanine greenangiography (ICGA) (C) revealdiffuse retinal microvascular ab-normalities (microaneurysms andcapillary dropout) (B, B1, B2,and C) and an RCA (arrowhead)along the inferotemporal arcade

of the RE and normal findings inthe LE (D). Spectral domainoptical coherence tomography

scan confi

rms the intense exu-dation associated with the retinalmicrovascular abnormalities and

RCA involving the RE macula.

Fig. 2. Images from the3-month posttreatment (intra-vitreal injection of bevacizumabfollowed by laser photocoagula-

tion) follow-up visit. Fundusphotograph (A), fluorescein angi-ography (B), and spectral domain

optical coherence tomography (C)of the RE show a regression of themicrovascular abnormalities and

retinalchoroidal anastomosisalong the inferotemporal arcade,with resolution of active exuda-tion but persistence of hardexudates.

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discern an RCA. It would be helpful to have included

optical coherence tomography line scans at the site ofthe suspected RCA. I suggest the new lesion thatoccurred during the follow-up period is another

aneurysm with surrounding exudation, retinal hemor-rhage, and capillary nonperfusion.My management of similar cases of Coats disease is

primarily aimed at preserving macular function byattempting to minimize macular edema and its asso-ciated lipid deposition that can cause permanentscarring when it develops at the fovea. I prefer to

treat the aneurysms responsible for visually threaten-ing macular edema with focal thermal laser. For

unknown reasons, these eyes rarely develop retinalneovascularization and vitreous hemorrhage. Therefore,I typically do not perform scatter laser treatment overareas of nonperfusion unless it appears that treatingthis ischemic retina might reduce vascular leakage.

Although the literature provides conflicting reportsregarding the benefit of intravitreal antivascular endo-thelial growth factor drugs in controlling macularedema in Coat disease, my experience has been thatthese agents induce a short-lived response that is ofteninadequate to prevent disease progression.5,6 It is

important to stay ahead of the disease in these casesby treating early, before lipid accumulates in the fovea.In more severe cases, laser alone may not be sufficientto control the degree of exudation. Often, this leakage

may be because of diffuse leakage from the telangi-

ectatic capillaries. In these cases, I have had successcontrolling the vascular leakage with injections ofsub-Tenon triamcinolone acetonide (40 mg/1.0 mL).

As the recurrent exudation in the authors

case(Figure 2) appears well outside the macula, I wouldsuggest that the authors observe their patient at this

point.

Editors Note:

Drs. Querques, Lattanzio, Querques, and Bandellopresent a 35-year-old man with reduced vision and

a choroidal neovascular membrane. The fluoresceinangiography and indocyanine green angiographyrevealed microaneurysms, saccular dilations, and cap-illary dropout. They felt that a retinochoroidal anasto-

mosis was present along the inferotemporal arcade andasked for comment on the diagnosis and their sub-

sequent management.Dr. Bailey Freund gives us a differential diagnosis

for the retinal vascular changes seen in this patient.

Coats diseaseBranch retinal vein occlusion

ParryRomberg syndromeFacioscapulohumeral muscular dystrophyFamilial exudative vitreoretinopathyDyskeratosis congenita

Fig. 3. Images from the lastfollow-up visit (October 2011).Fundus photograph (A) andfluorescein angiography (FA)(B) o f t he R E s how st illa regression of the microvas-cular abnormalities and reti-nalchoroidal anastomosisalong the inferotemporal ar-

cade with resolution of activeexudation but persistence ofhard exudates (A1 and B1). Anew prominent lesion associatedwith retinal hemorrhages, exu-dation, and hard exudates

appears on fundus photograph(A2) and FA (B2) along theinferonasal arcade of the RE(arrowhead). The new lesionhas developed in an area pre-

viously occupied by retinal mi-crovascular abnormalities

(Figure 1, B2, star).

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Dr. Freund believes Coats disease to be the mostlikely diagnosis, followed by branch vein occlusion(BVO), although he is unable to find an arteriovenous

crossing that would account for the distribution ofvascular changes seen.

Dr. Freund is not convinced that there is a retino-

choroidal anastomosis, as described by the presenters.He suggests that the new lesion described during thefollow-up may be another aneurysm. He discusses hispreference in treating Coats disease with thermal laser

and mentions the rarity of choroidal neovasculariza-tion in such eyes. He notes that anti-VEGF medica-tions give a short-lived response that is too ofteninadequate to prevent disease progression. But hementions some success with the use of perioculartriamcinolone.

We thank the presenters for their interesting caseand Dr. Freund for his comment.

References

1. Yannuzzi LA, et al. Idiopathic macular telangiectasia. Arch

Ophthalmol 2006;124:450460.

2. Muchnick RS, Aston SJ, Rees TD. Ocular manifestations and

treatment of hemifacial atrophy. Am J Ophthalmol 1979;88:

889897.

3. Vance SK, Wald KJ, Sherman J, Freund KB. Subclinical facio-

scapulohumeral muscular dystrophy masquerading as bilateral

Coats disease in a woman. Arch Ophthalmol 2011;129:807809.

4. Teixeira LF, Shields CL, Marr B, Horgan N, Shields JA. Bilateral

retinal vasculopathy in a patient with dyskeratosis congenita.Arch Ophthalmol 2008;126:134135.

5. Gamulescu MA, Walter A, Sachs H, Helbig H. Bevacizumab in

the treatment of idio- pathic macular telangiectasia. Graefes

Arch Clin Exp Ophthalmol 2008;246:11891193.

6. Takayama K, Ooto S, Tamura H, et al. Intravitreal bevacizumab

for type 1 idiopathic macular telangiectasia. Eye (Lond) 2010;24:

14921497.

RETINA, The Journal of Retinal and Vitreous

Diseases, encourages readers to submitDiagnostic and

Therapeutic Challenges to retina@retinajournal.com.Cases for the Diagnostic and Therapeutic Challenges

section should include a detailed history of the patient,the diagnosis, the workup, the management, and finally,

the question or questions that the submitter wishes tohave answered by the consultants.

DIAGNOSTIC AND THERAPEUTIC CHALLENGES 243