Table Of ContentKahlounetal.JournalofOpthalmicInflammationandInfection2013,3:16
http://www.joii-journal.com/content/3/1/16
ORIGINAL RESEARCH Open Access
Branch retinal artery occlusion associated with
posterior uveitis
Rim Kahloun1,2, Samah Mbarek1,2, Imen Khairallah-Ksiaa1,2, Bechir Jelliti1,2, Salim Ben Yahia1,2
and Moncef Khairallah1,2*
Abstract
Background: The purpose of this study is to report the clinical features and visual outcome ofbranchretinalartery
occlusion (BRAO)associated withposterior uveitis. This is a retrospective study including the18 eyes of 18 patients.
Allpatients underwent a complete ophthalmic evaluation. Fundus photography, fluorescein angiography,and visual
fieldtestingwere performed in allcases.
Results: Diseases associated with BRAO included active ocular toxoplasmosis in 7patients, rickettsiosis in 4, Behçet’s
uveitis in 2, West Nile virus infectionin 1, idiopathic retinal vasculitisin 1, Crohn’sdisease in1, ocular tuberculosis in
1, and idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome in1 patient. The mean initial visual
acuity was 20/50.BRAO involved thefirst order retinal artery in33.3%of the eyes, thesecond order retinal artery in
33.3%, an arteriole in27.8%, and a cilioretinal artery in 5.5%.The macula was involved in44.4%of the eyesand an
acute focus of retinitis or retinochoroiditis was associated to BRAO in 55.5%. Repermeabilization of the occluded
artery occurred in allpatients with permanent scotomas inthe corresponding visual field. The mean visual acuity at
last visit was 20/32.
Conclusions: BRAO, withsubsequent visual impairment, may occur inthe eyeswith posterior uveitis. Physicians
should be aware of such vision-threatening complication of infectious and inflammatory eye diseases.
Keywords: Branch retinalartery occlusion,Posterioruveitis, Fluorescein angiography, Visual impairment
Background Results
Infectious or inflammatory eye diseases may result in Ten patients (55.5%) were men, and eight patients
numerous vascular complications. These mainly include (44.5%) were women. The age of our patients ranged
retinal hemorrhages, retinal vascular hyperpermeability, from 18to56years(mean 37.8;median37.5).
retinal vascular occlusion, macroaneurysms, retinal or Diseases associated with BRAO were active ocular
choroidal neovascularization, and retinochoroidal anas- toxoplasmosis in 7 patients (7.5% of all toxoplasmosis
tomosis [1,2]. Although branch retinal vein occlusion cases recorded in our department) (Figure 1), rickettsio-
(BRVO) is considered to be a common complication of sis (Mediterranean spotted fever) in 4 patients (4.2% of
posterior uveitis associated with retinal vasculitis, data all Mediterranean spotted fever cases recorded in our
on the inflammatory branch retinal artery occlusion department) (Figure 2), Behçet’s uveitis in 2 patients
(BRAO) are relatively scarce. In this study we describe (1.3% of allBehçet’suveitis cases recordedin ourdepart-
the clinical features and visual outcome of BRAO asso- ment), West Nile virus infection in 1 patient (2.4% of all
ciated withposterioruveitis. cases of West Nile virus infection with posterior uveitis
recorded in our department), idiopathic retinal vasculitis
in1patient,Crohn’sdiseasein1patient,ocular tubercu-
losis in 1 patient (4% of all ocular tuberculosis cases
recorded in our department) and idiopathic retinal vas-
*Correspondence:[email protected] culitis,aneurysms,and neuroretinitis (IRVAN) syndrome
1DepartmentofOphthalmology,FattoumaBourguibaUniversityHospital,
in1patient(Table 1).
Monastir5019,Tunisia
2FacultyofMedicine,UniversityofMonastir,Monastir5019,Tunisia
©2013Kahlounetal.;licenseeSpringer.ThisisanOpenAccessarticledistributedunderthetermsoftheCreativeCommons
AttributionLicense(http://creativecommons.org/licenses/by/2.0),whichpermitsunrestricteduse,distribution,andreproduction
inanymedium,providedtheoriginalworkisproperlycited.
Kahlounetal.JournalofOpthalmicInflammationandInfection2013,3:16 Page2of5
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Figure1Branchretinalarteryocclusionassociatedwithoculartoxoplasmosis.(a)Colorfundusphotographofthelefteyeofapatientwith
oculartoxoplasmosisshowsanactivefocusofretinochorioretinitis(whitearrow)adjacenttooldpigmentedscarsinfero-temporallyandanarea
ofretinalwhiteningalongtheinferiortemporalarcade(blackarrows).(b)Earlyphasefluoresceinangiogramshowsdelayedfillingofinferior
temporalbranchretinalartery,capillarynonperfusioncorrespondingtotheareaofretinalwhitening,andhypofluorescenceofthefocusof
retinochoroiditis.(c)Colorfundusphotograph3monthslatershowsresolutionoftheretinalwhitening.Notethepresenceofapersistent
scotomaonautomatedperimetry(d).
Initial visual acuity (VA) ranged from 20/400 to 20/20 was not associated to any prior laser photocoagulation
(mean, 20/50; median, 20/40). Fundus examination (Figure3).Opticalcoherencetomography(OCT)showed
showed thefocalarea ofretinalwhitening corresponding serousretinaldetachmentin4ofthe8eyes(50%).
to the occluded artery in all cases. An acute focus of ret- Patients with ocular toxoplasmosis were treated with a
initis or retinochoroiditis was associated to BRAO in 10 combination of pyrimethamine (100 mg the first day
eyes (55.5%), including 7 eyes with ocular toxoplasmosis, then 50 mg daily), azithromycin (500 mg the first day
2 eyes with rickettsiosis, and 1 eye with Behçet’s uveitis. then 250 mg daily), and prednisone for 4 to 6 weeks.
The occluded vessel passed through the area of acute Patients with rickettsiosis were treated with a 2-week
retinitis orretinochoroiditis inallthese cases. course of oral doxycycline. Patients with Behçet’s uveitis
Fluorescein angiography (FA) revealed delayed filling were treated with systemic corticosteroids associated
of the occluded branch retinal artery and capillary non- with immunosuppressive therapy (azathioprine for the
perfusion corresponding to the area of retinal whitening first patient and a combination of azathioprine and
seenclinicallyinallcases. cyclosporine for the second patient). Patient with ocular
BRAO involved a first order retinal artery in 6 eyes tuberculosis was treated with antituberculous therapy
(33.3%), a second order retinal artery in 6 eyes (33.3%), (four drugs for 2 months followed by two drugs for 7
an arteriole in 5 eyes (27.8%) and a cilioretinal artery in months) associated with oral corticosteroids. Patients
1eye(5.5%).Themaculawasinvolvedin8eyes(44.4%). with idiopathic retinal vasculitis, Crohn’s disease, and
A cilioretinal artery occlusion associated with BRVO IRVANsyndrome were treated withoralcorticosteroids.
was recorded in one patient with Behçet’s uveitis. A During follow-up, all foci of retinitis or retinochoroidi-
BRAO associated with BRVO was recorded in one eye tis became inactive. Repermeabilization of the occluded
with tuberculous uveitis. In the eye with IRVAN syn- artery occurred in all patients with residual scotoma
drome, BRAO occurred at the level of an aneurysm and in the corresponding visual field (Figure 1). Retinal
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vessels throughout the body, including the retina [11].
Cat scratch disease, an infectious disease caused by the
gram-negative bacteria Bartonella henselae that has a
similar tropism in the retinal vessels has been previously
associated with BRAO [12-15]. No case of BRAO due to
cat scratch disease has been recorded in our series of
examinations.
Behçet’s disease is a leading cause of uveitis along the
Old Silk Road, including North African countries [23].
Behçet’s uveitis typically presents in the form of panuveitis
associated with retinal periphlebitis that may be compli-
catedwithBRVO[23].However,retinalarteryinvolvement
Table1Demographicandclinicalcharacteristicsofour
patients
Characteristics Values
Numberofpatients(eyes) 18(18)
Age(years)
Range 18-56
Mean 37.8
Median 37.5
Gender(n)(%)
Male 10(55.5)
Female 8(44.5)
Associatedinflammatoryeyedisease(n)(%)
Oculartoxoplasmosis 7(38.9)
Figure2Branchretinalarteryocclusionassociatedwith
Rickettsiosis 4(22.2)
rickettsiosis.(a)Colorfundusphotographoftherighteyeofa
patientwithrickettsiosisshowsanareaofretinalwhiteningsparing Behçet’sdisease 2(11.1)
thefovea(arrowheads).(b)Earlyphasefluoresceinangiogram Oculartuberculosis 1(5.5)
confirmsthediagnosisofbranchretinalarteriolarocclusionsparing
IRVANsyndrome 1(5.5)
thefovea(blackarrows).
Crohndisease 1(5.5)
Idiopathicvasculitis 1(5.5)
neovascularisation was recorded in one eye (patient with
WestNilevirusinfection 1(5.5)
West Nile virus infection). VA at last visit ranged from
20/200 to20/20(mean, 20/32;median,20/25). Catscratchdisease 0(0)
Siteofocclusion(n)(%)
Discussion Firstorderretinalartery 6(33.3)
Most publications on inflammatory BRAO consisted of
Secondorderretinalartery 6(33.3)
single-case reports, and to our knowledge, our study is
Arteriole 5(27.8)
the largest case series reported to date. Our findings,
Cilioretinalartery 1(5.5)
consistent with those from previous reports, show that
ocular toxoplasmosis is the leading cause of inflamma- Initialvisualacuity
tory BRAO [3-10], followed by an array of other inflam- Range 20/400-20/20
matory infectious or noninfectious retinochoroidal Mean 20/50
disorders [11-22]. Rickettsial infection was found to be
Median 20/40
the second most common cause of BRAO in our series.
Visualacuityatlastvisit
Mediterranean spotted fever, a rickettsial disease caused
by Rickettsia conorii, is endemic in the Mediterranean Range 20/200-20/20
region including North Africa [11]. Retinal vascular in- Mean 20/32
volvement in this disease is common due to a marked Median 20/25
tropism of the rickettsial organisms in the small blood IRVAN,idiopathicretinalvasculitis,aneurysms,andneuroretinitissyndrome.
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Figure3BranchretinalarteryocclusionassociatedwithIRVANsyndrome.(a)Colorfundusphotographofthelefteyeofapatientwith
IRVANsyndromeshowsanareaofretinalwhiteningalonguppertemporalvessel.Earlyphase(b)andmid-phase(c)fluoresceinangiogramsshow
branchretinalarteryocclusion.Notethepresenceofmultiplemacroaneurysmsonthecourseofthisartery.(d)Visualfieldtestingshowsmultiple
centralscotomasintheareacorrespondingtotheoccludedartery.
causing BRAO can also occur in the setting of Behçet’s In patients with IRVAN syndrome, BRAO have been
uveitis[16]. reported following laser photocoagulation of the aneur-
Inflammatory BRAO usually results in visual field de- ysms [19,20]. To the best of our knowledge, only one
fect without or with impairment of VA. The diagnosis of case of IRVAN syndrome complicated with a primary
BRAO primarily relies on clinical examination, but BRAO similar to our case has been previously reported
related clinical features might be overlooked or misdiag- [21]. The location of macroaneurysm at the junction of
nosed as signs of retinochoroidal inflammation. FA is the retinal artery tree might make it prone to spontan-
very useful in establishing a definitive diagnosis of in- eousthrombosis.
flammatory BRAO, particularly in subclinical cases or The cilioretinal artery occlusion in our patient with
those with achallengingdiagnosis. Behçet’s uveitis probably was functional in nature. An
Several mechanisms might be involved in the develop- increase of the intraluminal pressure in the retinal
ment of BRAO associated with posterior uveitis. BRAO capillaries due to the BRVO which is exceeding the
may occur at the site of an active focus of retinitis or pressure in the cilioretinal artery could lead to its oc-
retinochoroiditis. It could result from a direct compres- clusion [16].
sionofthearterybythefocusofretinitisorchorioretini- Resolution of the ocular inflammation and reperfusion
tis, leading to interruption of the blood flow. Arterial of the occluded artery occurred in all patients following
occlusion near active inflammatory foci may also be anti-infectious and/or systemic corticosteroid therapy.
explained by arteriolar contraction associated with Improvement of VA was recorded in all patients; how-
increased blood viscosity and inhibition of coagulation ever, the retinal function did not fully recover in cases
due to heparin release from the mast cells as a response with BRAO involving the macula with persistent field
toanacute inflammatory stimulus [5]. defects probably due to the atrophy of the retinal neuro-
Intheabsenceofthefocusofretinitisorchorioretinitis, epithelium or macular ischemia. Early and appropriate
BRAOcouldbeduetoperivasculitisresultingfromthein- treatment with anti-inflammatory and/or anti-infectious
filtration of the vessel wall by the inflammatory cells, medication may help induce prompt resolution of the
which may cause its thickening and as the consequence, ocular inflammation and reperfusion of the occluded ar-
disruptionofbloodflowandarterialthrombosis[14]. tery andimprove visualoutcome.
Kahlounetal.JournalofOpthalmicInflammationandInfection2013,3:16 Page5of5
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Conclusions 11. KhairallahM,LadjimiA,ChakrounM,MessaoudR,BenYahiaS,ZaoualiS,
In conclusion, BRAO may occur in association with an BenRomdhaneF,BouzouaiaN(2004)Posteriorsegmentmanifestationsof
Rickettsiaconoriiinfection.Ophthalmology111:529–534
array of infectious or non-infectious posterior uveitis 12. GrayAV,ReedJB,WendelRT,MorseLS(1999)Bartonellahenselaeinfection
entities, dominated by toxoplasmic retinochoroiditis. It associatedwithperipapillaryangioma,branchretinalarteryocclusion,and
usually causes visual field defect without or withVA im- severevisionloss.AmJOphthalmol127:223–224
13. CohenSM,DavisJL,GassDM(1995)Branchretinalarterialocclusionsin
pairment. A careful clinical examination, complemented
multifocalretinitiswithopticnerveedema.ArchOphthalmol
with FA, is mandatory in order not to miss a diagnosis 113:1271–1276
of inflammatory BRAO. The role of medical treatment 14. OrmerodLD,SkolnickKA,MenoskyMM,PavanPR,PonDM(1998)Retinal
andchoroidalmanifestationsofcat-scratchdisease.Ophthalmology
on the course of the occlusive event requires further 105:1024–1031
investigation. 15. SolleyWA,MartinDF,NewmanNJ,KingR,CallananDG,ZaccheiT,Wallace
RT,ParksDJ,BridgesW,SternbergPJr(1999)Catscratchdisease:posterior
segmentmanifestations.Ophthalmology106:1546–1553
Methods 16. BenYahiaS,KahlounR,JellitiB,KhairallahM(2011)Branchretinalartery
Thisisaretrospectivestudyincluding18eyesof18patients occlusionassociatedwithBehçetdisease.OculImmunolInflamm
19:293–295
with BRAO associated with posterior uveitis. All patients
17. YokoiM,KaseM(2004)Retinalvasculitisduetosecondarysyphilis.JpnJ
were examined at the Department of Ophthalmology, Ophthalmol48:65–67
FattoumaBourguibaUniversityHospital,Monastir,Tunisia 18. KhairallahM,BenYahiaS,AttiaS,JellitiB,ZaoualiS,LadjimiA(2006)Severe
ischemicmaculopathyinapatientwithWestNilevirusinfection.
between 1998 and 2011. The study was approved by the OphthalmicSurgLasersImaging37:240–242
ethicscommitteeofourinstitution. 19. ChangTS,AylwardGW,DavisJL,MielerWF,OliverGL,MaberleyAL,GassJD
The patients underwent a complete ophthalmic evalu- (1995)Idiopathicretinalvasculitis,aneurysms,andneuro-retinitis.Retinal
vasculitisstudy.Ophthalmology102:1089–1097
ation that included measurement of best-corrected VA,
20. TerradaC,DethoreyG,DucosG,LehoangP,BodaghiB,SouiedEH(2011)
slit-lamp examination, and dilated fundus examination. Spontaneousbrancharteryocclusioninidiopathicretinitis,vasculitis,
Fundusphotography,FA,andvisualfieldtestingwereper- aneurysms,andneuroretinitissyndromedespitepanretinallaser
photocoagulationofwidespreadretinanonperfusion.ActaOphthalmol
formedinallcases.OCTwasperformedineightpatients. 89:e542–e543
Meanfollow-upwas10months(range,6to24months). 21. VenkateshP,VergheseM,DavdeM,GargS(2006)Primaryvascular
occlusioninIRVAN(idiopathicretinalvasculitis,aneurysms,neuroretinitis)
syndrome.OculImmunolInflamm14:195–196
Competinginterest
22. ZamoraRL,delPrioreLV,StorchGA,GelbLD,SharpJ(1996)Multiple
Theauthorsdeclarethattheyhavenocompetinginterests. recurrentbranchretinalarteryocclusionsassociatedwithVaricellazoster
virus.Retina16:399–404
Authors’contributions 23. Tugal-TutkunI(2009)Behçet’suveitis.MiddleEastAfrJOphthalmol
RK,SM,IK,BJ,SBY,andMK,participatedinthesequencealignmentandin 16:219–224
thedesignofthestudy,performedthestatisticalanalysis,anddraftedthe
manuscript.RKandMKconceivedofthestudyandparticipatedinitsdesign doi:10.1186/1869-5760-3-16
andcoordination.Allauthorsreadandapprovedthefinalmanuscript. Citethisarticleas:Kahlounetal.:Branchretinalarteryocclusion
associatedwithposterioruveitis.JournalofOpthalmicInflammationand
Received:11September2012Accepted:12September2012 Infection20133:16.
Published:21January2013
References
1. BrézinAP(2012)Uveitis.PresseMed41:10–20
2. YamanakaE,OhguroN,KubotaA,YamamotoS,NakagawaY,TanoY(2004)
Featuresofretinalarterialmacroaneurysmsinpatientswithuveitis.
BrJOphthalmol88:884–886
3. MorganCM,GragoudasES(1987)Branchretinalarteryocclusionassociated
withrecurrenttoxoplasmicretinochoroiditis.ArchOphthalmol105:130–131
4. HaefligerE,MüllerO(1980)Brancharteryocclusionduetofocalnecrotizing
retinitisprobablycausedbytoxoplasmosis.KlinMonblAugenheilkd
176:613–618
5. BraunsteinRA,GassJDM(1980)Brancharteryobstructioncausedby
toxoplasmosis.ArchOphthalmol98:512–513 Submit your manuscript to a
6. PakalinS,ArnaudB(1990)Arterialocclusionassociatedwithtoxoplasmic
journal and benefi t from:
chorioretinitis.JFrOphtalmol13:554–556
7. WilliamsonTH,MeyerPA(1991)Branchretinalarteryocclusionin
Toxoplasmaretinochoroiditis.BrJOphthalmol75:253 7 Convenient online submission
8. GentileRC,BerinsteinDM,OppenheimR,WalshJB(1997)Retinalvascular 7 Rigorous peer review
occlusionscomplicatingacutetoxoplasmicretinochoroiditis. 7 Immediate publication on acceptance
CanJOphthalmol32:354–358 7 Open access: articles freely available online
9. KüçükerdönmezC,YilmazG,AkovaYA(2004)Branchretinalarterial 7 High visibility within the fi eld
occlusionassociatedwithtoxoplasmicchorioretinitis.OculImmunol
Inflamm12:227–231 7 Retaining the copyright to your article
10. MiserocchiE,ModoratiG,RamaP(2009)Atypicaltoxoplasmosis
masqueradinglateoccurrenceoftypicalfindings.EurJOphthalmol Submit your next manuscript at 7 springeropen.com
19:1091–1093
