-

- To icvicw ilc licqucncy and naiuic ol id-

iopailic maculai iclangicciasia and io classily ilc disoi-
dcis lascd on ncw clinical and imaging olscivaiions.
- A comlincd iciiospcciivc and piospcciivc
analysis ol ncwly diagnoscd paiicnis sccn ovci a pciiod
ol 3 ycais. Paiicnis wcic idcniilicd lascd on ilc Gass-
llodi classilicaiion and wcic siudicd wiil liomicios-
copy, lluoicsccin angiogiaply, and opiical colcicncc
iomogiaply.
-- Tcn paiicnis associaicd wiil ancuiysmal icl-
angicciasia (Gass-llodi gioup1) and26paiicnis wiilpcii-
lovcal iclangicciasia (Gass-llodi gioup 2) wcic ic-
ciuiicd. Nonc wiil occlusivc iclangicciasia (Gass-llodi
gioup3) wcic idcniilicd. Ncwolscivaiions lascdonclini-
cal, lluoicsccin angiogiaplic, and opiical colcicncc io-
mogiaplic lindings wcic madc.
-- Oui sciics was similai io ilai in ilc Gass-
llodi siudy in icims ol licqucncy. Ncw olscivaiions in
gioups 1 and 2 lavc cxpandcd oui lnowlcdgc ol ilc clini-
cal spcciium ol ilcsc disoidcis. A simplilicd classilica-
iion icimcd - wiil 2 dis-
iinci iypcs (iypc I, oi ancuiysmal iclangicciasia, and iypc
II, oi pciilovcal iclangicciasia) was pioposcd io pioducc
a lciici undcisianding ol ilc cniiiics andiocnlancc icacl-
ing and icscaicl. Tlc iliid iypc, occlusivc iclangicciasia,
las lccn omiiicd liom oui classilicaiion lascd on iis iai-
iiy and picscncc ol capillaiy nonpcilusion iailci ilan
maculai iclangicciasia as ilc piimaiy alnoimaliiy.

LTINAL CAPILLARY TLLANGI-

cciasia oi dilaicd iciinal
capillaiics aic usually ilc
icsuli ol anicccdcni icii-
nal vasculai inllammaioiy
oi occlusivc discasc. In ilc macula, dia-
lciic iciinopaily, lypciicnsion, vcnous
occlusion, inllammaioiy discascs, and
llood dysciasias aic ilc usual causaiivc
laciois. Howcvci, ilcic aic oilci loims ol
iclangicciasia ilai dcvclop in ilc macula
and pciilovcolai aicas wiiloui a lnown
causc. In 1993, Gass and llodi
1
cxam-
incd 1+0 suclcascs sccnai lascomPalmci
Lyc Insiiiuic, Miami, Ila, ovci a 2S-ycai
pciiod and csiallislcd a classilicaiion ol
ilcsc cniiiics wiil sulgioups and siagcs.
Tlis dcsciipiion, icimcd
--, alilougl
somcwlai complcx, is commonly uscd io-
day ly mosi iciinal spccialisis. In icccni
ycais, ncwly iccognizcd manilcsiaiions
lavc cxpandcd and iclincd ilc clinical
spcciium ol ilcsc maculai vasculopa-
ilics. Iuiilcimoic, ilc usc ol ligl-
spccd angiogiaply and opiical colci-
cncc iomogiaply (OCT) lavc piovidcd a
lciici undcisianding ol ilc naiuic ol ilc
vasculai alnoimaliiics and ilcii sccond-
aiy cllccis in ilc macula, io somc dcgicc
paiallcling lisiopailological olsciva-
iions dcsciilcd in ilc oplilalmic liicia-
iuic.
2-9
Tlc puiposc ol ilis siudy was io
dcsciilc ncw clinical and OCT olsciva-
iions in ilcsc cniiiics and io pioposc a
modilicd classilicaiion-csscniially a ic-
vision and simplilicaiion ol ilc Gass-
llodi modcl ilai wc iclci io as idiopailic
maculai iclangicciasia (IMT)-lascd on
ilcsc ncwly iccognizcd clinical and im-
aging manilcsiaiions.

Ncwlydiagnoscdpaiicnis wiilIMTwciccxam-
incd consccuiivcly in ilc piivaic olliccs ol
Viiicous-Rciina-Macula Consulianis ol Ncw
Yoil, NcwYoil, and ai ilc LuLsilci T. Mciiz
Rciinal Rcscaicl Ccnici, Manlaiian Lyc, Lai,
and Tlioai Hospiial, NcwYoil, liomJanuaiy
1, 2002, iliougl Dcccmlci 31, 200+. Tlc ol-
licc siics includcd piimaiy ccnicis sucl as ilc
lospiial clinics, asccondaiyiclciial piivaicicii-
nal piaciiccinlioollyn, NcwYoil, andamixcd
iciinal laciliiyilai was paiiiallya iciiiaiyiclci-
ial piaciicc in Manlaiian, NcwYoil. Only pa-

-
- LuLsilci T.
Mciiz Rciinal Rcscaicl Ccnici,
Manlaiian Lyc, Lai, and Tlioai
Hospiial, Ncw Yoil, NY
(Dis Yannuzzi, laidal, Iicund,
Clcn, and Landi); Dcpaiimcni
ol Clinical Plysiopailology, Lyc
Clinic, Univcisiiy ol Toiino,
Toiino, Iialy (Di Landi); and
Dcpaiimcni ol Oplilalmology
and Visual Scicncc, Univcisiiy
ol Wisconsin, Madison
(Di llodi).
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iicnis cxamincd loi ilc liisi iimc wlo lad a diagnosis madc oi
conliimcd wcic includcd. Tlc siudy paiicnis in ilc liisi 2 ycais
wcic idcniilicd iciiospcciivcly; in ilc lasi ycai, ilcy wcic ic-
ciuiicd piospcciivcly. Acaiclul lisioiy on iclaicd sysicmic dis-
cascs, spccilically sysicmic lypciicnsion, dialcics mclliius, and
iadiaiion ilciapy io ilc lcad, was oliaincd liom cacl paiicni.
A lull clinical cxaminaiion, including indiicci oplilalmos-
copy and sliilamp liomicioscopy wiil ilc Goldmann coniaci
lcns, was pciloimcd. Digiial lundus ploiogiaply, digiial lluo-
icsccin angiogiaply (IA), and OCT wcic caiiicd oui loi all ol
ilc paiicnis.
Tlc paiicnis wcic classilicd inio 3 gioups. Typc 1 is ancu-
iysmal iclangicciasia ( ). Tlis involvcs picdomi-
nanily unilaicial iclangicciasia mosily in mcnwiil vaiially sizcd
ancuiysms. Typc 2 is pciilovcal iclangicciasia ( and
). Tlis involvcs lilaicial iclangicciasia limiicd io ilc
pciilovcolai aica wiiloui visillc ancuiysms and associaicd wiil
suliciinal ncovasculaiizaiion (SRN). Nonpiolilciaiivc pciilo-
vcal iclangicciasia iclcis io cxudaiivc iclangicciasia and lovcal
aiioply (Iiguic 2). Piolilciaiivc pciilovcal iclangicciasia ic-
lcis io piolilciaiivc clangcs wiil suliciinal ncovasculaiiza-
iion and liliosis (Iiguic 3). Typc 3 is occlusivc iclangicciasia
(). Tlis involvcs paiamaculai iclangicciasia in an aica
loidciing on pciilovcal capillaiy nonpcilusion.

In ilc 3-ycai pciiod ol ilc siudy, a ioial ol 36 paiicnis

wiil IMT wcic sccn; 10 ol ilc paiicnis lad ancuiysmal
iclangicciasia, 26 lad pciilovcal iclangicciasia, and nonc
lad occlusivc iclangicciasia. Tlc casc licqucncy iaiio ol
ilcsc gioups was 1.0:2.6:0.0 cascs, icspcciivcly.

Tlc ancuiysmal iclangicciasia cascs aic dcsciilcd in
. In ilis gioup, ilcic wcic 10 paiicnis, 7 ol wlom
wcic malc and 3 ol wlomwcic lcmalc. Ninc paiicnis lad
unilaicial discasc. Tlcic was only 1 lilaicial casc in a
woman. Tlc agc iangc ol ilc paiicnis was +2 io 71 ycais,
wiil a mcan agc ol 56 ycais. Onc paiicni lad sysicmic
lypciicnsion. Oilciwisc, ilcic was no cvidcncc ol ic-
laicd sysicmic discascs.
Tlc clinical manilcsiaiions vaiicd. A consisicni lca-
iuic ol ilc micioangiopaily, lowcvci, was muliiplc
capillaiy, vcnulai, and aiiciiolai ancuiysms. Ancuiys-


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mal and iclangicciaiic alnoimaliiics wcic cvidcni in
ilc supcilicial and dccp iciinal capillaiy ciiculaiions.
Laigci ancuiysms wcic conlincd io ilc supcilicial cii-
culaiion. In addiiion, somc paiicnis lad minimal,
paicly nonpcilusion oi capillaiy isclcmia and lipid
dcposiiion. Tlc lipid was noicd paiiiculaily in associa-
iion wiil numcious small capillaiy alnoimaliiics oi
pcisisicni, local, laigci ancuiysms ( ). Tlcic
wcic no ciysiallinc viiicoiciinal inicilacc dcposiis,
plaqucs ol pigmcni cpiilclial lypciplasia, oi aicas ol
pigmcni migiaiion inio ilc iciina. In 2 paiicnis, ilc
pailological alnoimaliiy also vaiicd in iis locaiion, as
ilcic wcic aicas ol involvcmcni noi only in ilc juxia-
lovcolai and cxiiamaculai icgions lui also in ilc
pciiplcial lundus. Somc laigci macioancuiysms wcic
associaicd wiil lcmoiilagc. Tlc ccniial maculai vas-
culai alnoimaliiics wcic associaicd wiil cdcma oi
cysiic clangc in ilc iciina, sccn clinically and angio-
giaplically and conliimcd wiil OCT imaging in all ol
ilc cascs ( ). No paiicni lad piciciinal nco-
vasculaiizaiion oi SRN dcspiic ilc picscncc ol capil-
laiy isclcmia.

Tlc paiicnis wiil pciilovcal iclangicciasia aic dcsciilcd
in . In ilis gioup, ilcic wcic 26 paiicnis, 11 ol
wlom wcic malc and 15 ol wlom wcic lcmalc. Tlc agc
iangc was 3S io S2 ycais, wiil a mcan agc ol 59 ycais.
All ol ilc paiicnis lad lilaicial discasc. In a lcw cascs,
ilcic was a dcgicc ol asymmciiy wiil icgaid io scvci-
iiy. Iivc paiicnis (19.2`) lad dialcics mclliius wiil no
clinical dialciic iciinal micioangiopaily. Tlis lic-
qucncy docs noi dillci liom ilc incidcncc (1S.5`)
icpoiicd in ilc gcncial populaiion ol ilc samc agc.
10
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sponding iciinal linding. Oilciwisc, ilcic was no oilci
iclaicd sysicmic discasc in ilis gioup.
In coniiasi io ilc ancuiysmal iclangicciasia cascs,
ilc pciilovcal iclangicciasia cascs wcic all wcll dclincd
ly ilcii clinical and imaging manilcsiaiions. Laily clini-
cal clangcs consisicd ol a mild loss ol iianspaicncy ol
ilc iciina, usually in ilc icmpoial juxialovcolai aica
lui suiiounding ilc lovca in moic advanccd cascs
(Iiguic 2A and l). Tlis loss ol iianspaicncy was iypi-
cally giay and loimcd a paiiial oi complcic loiizonial
oval suiiounding ilc lovca. In ilcsc cascs, ilc iclangi-
cciaiic vcsscls wcic alscni oi laicly cvidcni clinically.
Rciinal iliclcning was also dilliculi io judgc in somc
cycs, lui ii was icadily cvidcni wiil OCT imaging
(Iiguic 2L). Wlcn visillc, ilc iclangicciaiic vcsscls
wcic sccn in ilc innci and ouici iciinal ciiculaiions.
Paiicnis wiil moic advanccd siagcs ol ilc discasc lad a
piomincni dilaiion ol capillaiics.
In paiicnis wiil caily clinical manilcsiaiions ol pcii-
lovcal iclangicciasia, lluoicsccin angiogiaply slowcd
mild, dillusc iniiaiciinal lcalagc oi siaining. In paiicnis
wiil moic piomincni iclangicciasis, lluoicsccin lcal-
agc in ilc supcilicial ciiculaiion could lc sccn sicico-
scopically io cascadc ovci ilc dccp capillaiy lcalagc


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( ). Tlc supcilicial iciinal ciiculaiions in 6 cascs
icvcalcd scgmcnial oi sccioial dilaiion ovcilying ilc di-
laicddccpiciinal iclangicciaiic vcsscls. Inilcsc cycs, somc
capillaiy ancuiysmal clangcs occuiicd, lui ilcic was no
lipid isclcmia oi piciciinal ncovasculaiizaiion. No cys-
iic spaccs wcic cvidcni in ilc pciilovcal aica dcspiic ilc
lluoicsccin lcalagc (Iiguic 2). Ai ilc lovca iiscll, ilcic
was a ciiculai oi ovoid dcpicssion ilai piovcd wiil OCT
imaging io lc an innci lamcllai cysi (Iiguic 2). Opiical
colcicncc iomogiaplic imaging also slowcd picsciva-
iion ol ilc innci limiiing mcmlianc ilai consiiiuicd
ilc aniciioi aspcci ol ilc cysi. Moic advanccd cascs
icvcalcd a dccpci innci lamcllai cysi wiil luiilci loss
ol ilc ouici iciina and a coiiclaiing visual dcclinc
( ).
Tlcic wcic no piomincni ancuiysms, lcmoiilagc, oi
lipid accumulaiion. Viiicoiciinal inicilacc ciysiallinc dc-
posiis appcaicd ai vaiious siagcs ol ilc discasc as an in-
consisicni lui claiaciciisiic linding. Somc cycs also ic-
vcalcd suliciinal plaqucs ol pigmcniaiion and dilaicd
iigli-anglc iciinal vcsscls (Iiguic 3). In somc cascs, ilc
dilaicd vcsscls wcic vcnulai; in oilcis, ilcy wcic aiic-
iiolai in naiuic. Alcwaciually lad a iciinal-iciinal anas-
iomosis (RRA) wiilin ilc iciina oi cxicnding io com-
municaic wiil ncw vcsscls lcncail ilc iciina oi SRN
(). Tlc SRNappcaicd io oiiginaic liomilc dccp
iciinal ciiculaiion. Tlc dcvclopmcni ol SRN lcd io ncu-
ioscnsoiy dciaclmcni, suliciinal lcmoiilagc, liliosis,
and visual dcclinc in advanccd siagcs ol ilc discasc
(Iiguic 3). Cysiic clangc in ilc iciina, dciaclmcni ol
ilc macula, and suliciinal ploioicllcciancc liom lilio-
vasculai manilcsiaiions wcic cvidcni ai ilis siagc wiil
OCT imaging (Iiguic 3).

Occlusivc iclangicciasia was noi sccn in ilis sciics. Tlis
is consisicni wiil iis iaiiiy.

In 19S2, Gass and Owalawa

11
pioposcd a classilicaiion ol
idiopailic maculai iclangicciasia icimcd
--. Ii was icviscd mosi icccnily in
1993 inio 3 disiinci gioups, cacl wiil a picsumcd indc-



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pcndcni ciiology, onilc lasis ol liomicioscopical andlluo-
icsccin angiogiaplic lcaiuics.
1
Sulgioups lascd piinci-
pally on dcmogiaplic dillcicncc oi clinical scvciiiy wcic
also csiallislcd in ilis classilicaiion.
Ioi cxamplc, 39 cascs wcic classilicd as gioup 1
(cquivalcni io ancuiysmal iclangicciasia in oui sciics).
A sulgioup, gioup 1A, was lascd on ilc clinical dcicc-
iion ol ilc ancuiysmal and iclangicciaiic cxudaiivc vcs-
scls inilc juxialovcolai aica. Gioup 1l (a sulsci ol gioup
1A) was dclincd as local cxudaiivc iclangicciasia lim-
iicd io 2 clocl louis oi lcss in ilc juxialovcal aicas in S
ol ilc paiicnis. Inoui cxpciicncc, cascs wiil2 clocllouis
piogicss io moic cxicnsivc discasc wiil iimc.
In gioup 2 (cquivalcni io pciilovcal iclangicciasia in
oui sciics), a sulcaicgoiy was lascd on ilc agc ai onsci.
Ninciy-iwo paiicnis wcic classilicd as gioup 2A, and 2
young lioilcis agcd 9 and 12 ycais wcic placcd in gioup
2l. Tlcsc 2 lioilcis icpicscni iaic cascs, possilly a la-
milial disoidci noi claiaciciisiic ol gioup 2 as a wlolc.
Only 1 oilci aiiiclc
12
cxisis in ilc oplilalmic liiciaiuic
on pciilovcal iclangicciasia in young individuals. Tlai
casc siudy, lowcvci, involvcd lamilial iclangicciasia and
spasiic paiaplcgia, noi cxacily an idiopailic picscnia-
iion. Iuiilcimoic, gioup 2A was also suldividcd inio 5
siagcs ol ilc discasc as lollows: siagc 1, oi occuli vascu-
lai alnoimaliiics cvidcni only ly mild siaining wiil lluo-
icsccin; siagc 2, oi mild loss ol iianspaicncy wiiloui clini-
cally cvidcni iclangicciaiic vcsscls; siagc 3, oi piomincni
dilaicd iigli-anglc iciinal vcnulcs; siagc +, oi iciinal pig-
mcni lypciplasia cxicnding inio ilc iciina; and siagc 5,
oi SRNoiiginaiing liompiolilciaiion ol iniiaiciinal cap-
illaiics lcncail ilc ncuioscnsoiy iciina. A mcilod loi
siaging a discasc is usually lascd on ilc iimc scqucncc
ol piogicssivc clangcs ilai occui as paii ol ilc naiuial
couisc ol ilc disoidci. Mosi ol ilc lindings in gioup 2
occui in somc paiicnis lui noi oilcis ai vaiious siagcs
ol ilc discasc. Wc piclci ioclassily ilis gioupinio 2siagcs,
1 wiil nonpiolilciaiivc vasculai alnoimaliiics and 1 wiil
piolilciaiivc vasculai alnoimaliiics.
Gioup 3 (oi occlusivc iclangicciasia in oui sciics) was
iaicly sccn and was suldividcd ly Gass and llodi
1
inio 2
sulgioups. Tlicc womcn wiil iclaicd sysicmic vasculai
occlusivc oi inllammaioiy discasc consiiiuicd gioup 3A,
and + mcn wiilccniial ncivous sysicminvolvcmcni com-
poscd gioup l. In ilc Gass-llodi sciics, ilcic was a ioial
ol 1+0 cascs sccn ovci a 2S-ycai pciiod: 39 cascs in gioup
1, 9+ in gioup 2, and 7 in gioup 3. Tlcii casc licqucncy
iaiio in ilc sciics was 1.0:3.1:0.2 cascs, icspcciivcly.
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Ovciall, ilc icsulis ol oui siudy aic quiic similai io ilc
Gass-llodi sciics.
1
Iiisi, ilccasclicqucncyiaiiowas almosi
idcniical, appioximaicly 1 iypc 1 casc io 3 iypc 2 cascs in
cacl sciics. Oui siudy also conliimcd ilc iaiiiy ol iypc 3,
ilcocclusivcvaiiani; 7cascs wcicsccnlyGass
1
in2Sycais,
andnoncwcicnoicdinoui siudyin3ycais. Howcvci, somc
dissimilaiiiycxisis lciwccnilcsciics lyGass andllodi and
oui sciics, andilcicaicncwolscivaiions, loilclinical and
imaging, in oui siudy. lascd on oui lindings, wc pioposc
a simplilicd icvision ol ilc Gass-llodi classilicaiions.

Gioups 1A and 1l wcic mcigcd ly omiiiing ilc disiinc-
iions lascd on 2 clocl louis ol involvcmcni in ilc jux-
ialovcal aica. Wc lclicvc ilai a small local aica in ilc
juxialovcal icgion is a lcss scvcic vaiiani in ilc spcc-
iium ol ilc discasc (Iiguic 6A and l). Mosi cascs ol an-
cuiysmal iclangicciasia wcic in mcn and wcic unilai-
cial. Ancxccpiioninoui sciics was a womanwiillilaicial
discasc. Gass and llodi
1
did noi mcniion ilc scx ol ilcii
2 lilaicial cascs. Tlcic was no iclaicd sysicmic discasc
in ilc Gass-llodi sciics oi in oui sciics.
As in ilc aiiiclc ly Gass and llodi,
1
paiicnis wiil an-
cuiysmal iclangicciasia vaiicd considcially in ilcii clini-
cal lcaiuics. As picviously dcsciilcd,
13
ilc iniiaiciinal mi-
cioangiopaily clangcs in oui sciics wcic cvidcni in ilc
supcilicial anddccpciiculaiions wiillcalagc inioilc iciina
and cysioid maculai cdcma. Wc lound minimal isclcmia
and no cvidcncc ol ncovasculaiizaiion (nciilci picicii-
nal ncovasculaiizaiion noi SRN). Lipid dcposiiion was a
claiaciciisiic lcaiuic, lui ilcic was no pigmcni piolilcia-
iion (Iiguic 5). Tlcic wcic also no viiicoiciinal ciysial-
linc dcposiis oi signs ol disciloim scaiiing. A lcy io ilc
naiuic ol ilis vasculopaily was iis disiiiluiion inilc lun-
dus. Alilougl a vasculai lcsion in ilc paiamaculai oi jux-
ialovcal aica was csscniial in iis diagnosis, ilis gioup may
also dcvclop local vasculai clangc in ilc midpciiplcial
lundus and cvcn in ilc moic aniciioi lundus (Iiguic 5).
Tlis condiiion may lc considcicd wiilin ilc spcciiumol
Coais' discasc (loil ilis sciics and ilc Gass-llodi sciics
1
cxcludcd paiicnis wiil maculai and widcspicad oi dil-
lusc iclangicciasia). Wc considci ancuiysmal iclangicc-
iasia io lc a loim ol Coais' discasc ilai is lound in ilc
macula. Oui siudy was allc io incoipoiaic OCT lindings
in cvaluaiing ilcsc paiicnis. Tlc OCT imagcs in paiicnis
wiil ancuiysmal iclangicciasia clcaily documcnicd and
conliimcd ilc picscncc and dcgicc ol maculai cdcma and
lovcal cxudaiivc cysiic clangcs, wlicl wcic picscni in all
ol ilc cascs. Tlc cdcma was usually cvidcni clinically and
angiogiaplically. In somc cycs, lowcvci, ilc cdcma cx-
icndcd lcncail ilc iciina io pioducc slallow dciacl-
mcnis ol ilc macula ilai wcic imagcd only wiil OCT
(Iiguic 6I). Alilougl laigci ancuiysms pioduccd slad-
owing ol ilc posiciioi iciina, ilcsc siiuciuics wcic also
visillc clinically and angiogiaplically; wlcic ilc sulicii-
nal lluid was associaicd wiil iuilid cxudaiion, ilcic was
coiicsponding ploioicllcciancc and posiciioi sladow-
ing (Iiguic 6I). Tlc lnownlisiopailological lindings loi
ilis gioup aic consisicni wiil oui clinical olscivaiions ol
dilaiion ol capillaiics, ancuiysms, lcalagc, and minimal
nonpcilusion.
1+

As in ilc Gass-llodi classilicaiion,
1
ilc paiicnis wiilpcii-
lovcal iclangicciasia inoui sciics lad clinical lindings lim-
iicd io ilc pciilovcolai aica. Tlc dcmogiaplics wcic also
similai. Tlcic was no scx dillcicncc, and all ol ilc cascs
wcic lilaicial. In oui sciics, ilc mcan agc ai onsci was
59 ycais, wlicl was similai io ilc mcan agc ol 55 ycais
inilc Gass-llodi sciics. Iivc paiicnis (19.2`) inilis gioup
lad dialcics mclliius. In a mixcd iacial populaiion ol pa-
iicnis wiil an avciagc agc ol 59 ycais, ilis is noi lilcly
io lc a signilicani associaiion. Tlc Gass-llodi sciics did
noi includc paiicnis wiil dialcics mclliius ai ilc iimc
ol diagnosis. Clcw ci al
15
did lind dialcics mclliius io

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lc a iisl lacioi. In oui sciics, wc icmovcd ilc Gass-
llodi gioup 2l sulcaicgoiy ilai was lascd on agc ai on-
sci. Gioup 2l compiiscd only 2 lioilcis wlo wcic agcd
9 and 12 ycais. Tlis lamilial vaiiani is cxiicmcly iaic and,
io oui lnowlcdgc, las noi lccn dcsciilcd in any oilci
sciics.
Loss ol iciinal iianspaicncy, small iclangicciaiic vcs-
scls, lluoicsccin lcalagc, and a cysiic appcaiancc io ilc
lovca wiiloui iniiaiciinal lcalagc wcic ilc picdomi-
nani and mosi consisicni clinical lcaiuics in oui sciics
(Iiguic 2). Tlc loss ol iciinal iianspaicncy iypically ap-
pcaicd giayisl.
Cascading iciinal vasculai alnoimaliiics, dcmon-
siiaicd wiil sicicoangiogiaply lui noi picviously dc-
sciilcd, wcic cvidcni in somc ol oui cascs (Iiguic 7).
Cuiiously, ilc dccp iciinal ciiculaiion ilai was clcaily
dcmonsiiaicd wiil lluoicsccin angiogiaply was noi sccn
in oilci iciinal miciovasculopailics in ilc macula. Tlc
dilaicd iigli-anglc vcsscls dcsciilcd ly Gass and llodi
1
piovcd in oui sciics io lc loil vcnulai and aiiciiolai in
naiuic (Iiguic 9). Iuiilcimoic, wc olscivcd iniiaicii-
nal and suliciinal anasiomoscs, wlicl wcic noi picvi-
ously dcsciilcd (Iiguic 9). Tlcsc iigli-anglc vcsscls may
lcad io a nciwoil ol piolilciaiing vcsscls in ilc dccp icii-
nal ciiculaiion. Tlis nciwoil can cxicnd lcncail ilc
iciina io loim a suliciinal nciwoil, olicn wiil a clcaily
disccinillc RRA oi iciinal-suliciinal anasiomosis. Tlc
SRN was noi ncccssaiily associaicd wiil pigmcni cpi-
ilclial lypciplasiic plaqucs oi iciinal migiaiion
(Iiguic 9).
1
Unlilc cloioidal ncovasculaiizaiion, wlicl
is usually associaicd wiil pigmcni cpiilclial piolilcia-
iion, oui sciics indicaicd ilai ilc SRNscldomlad an cn-
vclopc oi plaquc ol pigmcni lypciplasia (Iiguic 3 and
Iiguic 9). Tlis was also noicd in ilc aiiiclc on cxcision
ol SRN in pciilovcal iclangicciasia ly lcigci ci al.
6
Ncw
vcsscls iising liom ilc cloioid aic noi claiaciciisiic ol
iypc II, lui 1 ol oui cascs appcais io lc an cxccpiion, as
a pigmcnicd mcmlianc communicaiing wiil ilc iciinal
vasculaiuic cvolvcd wiil a lcmoiilagc and liliosis inio
a disciloim scai ( ). Tlc iciinal pigmcni cpi-
ilclium (RPL) in ilcsc paiicnis wiil pciilovcal iclangi-
cciasia was lcalily compaicd wiil ilai in paiicnis wiil
agc-iclaicd maculai dcgcnciaiion. Wlcicas an RPL dc-
iaclmcni is common in agc-iclaicd maculai dcgcncia-


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iion, ii is iaic in pciilovcal iclangicciasia. Onc ol us
(L.A.Y.) las sccn an RPL dciaclmcni in pciilovcal icl-
angicciasia only iwicc ovci ilc pasi 35 ycais, pcilaps as
a coincidcnial linding in ilcsc paiicnis in ilc sixil dc-
cadc ol lilc.
To oui lnowlcdgc, ilc piccisc mcclanism in pciilo-
vcal iclangicciasia loi ilc piolilciaiion ol iciinal capillai-
ics liomilc dccp capillaiy ciiculaiion is unlnown. In ilc
alscncc ol capillaiy isclcmia oi inllammaiion, piogics-
sivc iciinal cndoilclial ccll dcgcnciaiion may lc ilc iiig-
gciing, vasogcnic ccllulai mcclanism. Unlilc dialciic mi-
cioangiopaily, ilcic is no piciciinal piolilciaiion. Ii is
icmpiing io spcculaic ilai piolilciaiion liom ilc middlc
iciinal ciiculaiion io cxicnd lcncail ilc iciina is similai
io cxpciimcnial animal modcls oi ilc ncovasculai loim
ol agc-iclaicd maculai dcgcnciaiion lnown as iciinal an-
giomaious piolilciaiion (RAP).
16-1S
Tlcsc vasogcnic coi-
iclaics also do noi lavc dcliniic cvidcncc ol inllamma-
iion oi isclcmia as piccuisois. Paiicnis wiil RAP aic also
lnown io dcvclop RRAs. Iuiilcimoic, ilcsc RRAs ap-
pcai iolc compcnsaioiy innaiuic-iopcilusc andiodiain
an cxpanding angiomaious pioccss. Davidoil ci al
7
noicd
a icduciion in ilc calilci ol a dilaicd pcilusing aiiciiolc
and diaining vcnulc alici suigical cxcision ol ilc SRN in
pciilovcal iclangicciasia. Wc lavc noiiccd similai vcsscl
calilci clangcs lollowing ploiodynamic ilciapy in pcii-
lovcal iclangicciasia and agc-iclaicd maculai dcgcncia-
iion. Tlc RRAwiilin ilc iciina in RAP and pciilovcal icl-
angicciasia cvcniuallycxicnds lcncaililc iciina wlcnSRN
dcvclops. Howcvci, unlilc RAP, a iciinal-cloioidal anas-
iomosis docs noi usually cvolvc sincc cloioidal ncovas-
culaiizaiion is noi iypically involvcd in ilc vasogcnic pio-
ccss ol paiicnis wiilpciilovcal iclangicciasia.
19,20
Wc lclicvc
ilai ilc suliciinal plaquc ol pigmcniaiion in pciilovcal
iclangicciasia is icaciivc lypciplasia induccd ly cxicn-
sionol ilc iciinal vasculai piolilciaiionposiciioilyiosiimu-
laic RPL cclls, again similai io RAP. Lalaui ci al
21
dc-
sciilcd ilis occuiiing in lisiopailological spccimcns ol
RAP. Wc lypoilcsizc ilai ilis mcclanism loi ilc pig-
mcniaiy icsponsc is moic lilcly ilan ilc opposiic (RPL
ccll migiaiion inio ilc iciina io siimulaic vasculai piolil-
ciaiion) pioposcd ly Gass and Owalawa.
11
Oui clinical olscivaiions ol IMT coiiclaic wcll wiil
picviously dcsciilcd lisiopailological lindings. In 19S0,
Giccn ci al
S
dcsciilcd iliclcning ol iciinal capillaiics and
naiiowing ol ilc calilci ol ilc lumcn on clcciion mi-
cioscopical analysis. Tlc iliclcning liom mailcd pio-
lilciaiion ol ilc lascmcni mcmlianc in a muliilaycicd
conliguiaiion was noicd. Dcgcnciaiion ol pciicyics, and
occasionally cndoilclial cclls, was also dcsciilcd. Sui-
piisingly, ilcsc clangcs wcic also noicd io a lcssci dc-
gicc inilc pciiplcial lundus as wcll, wlcic ilcic aic iypi-
cally no clinically dcicciallc alnoimaliiics. Giccn and
collcagucs also noiiccd iciinal capillaiy piolilciaiion inio
ilc ouici iciina as lai as ilc ploioicccpioi layci. Sincc
ilis landmail aiiiclc appcaicd, oilci clinical pailologi-
cal coiiclaiions lavc addcd io oui lnowlcdgc ol IMT
alnoimaliiics. Cloioidal ncovasculaiizaiionwiiliciinal-
cloioidal anasiomosis las lccn documcnicd lisiopailo-
logically in 1 casc.
11
Rciinal-cloioidal anasiomosis is noi
iypical in pciilovcal iclangicciasia, lui ii is commonly
lnown ilai any cvcni ilai disiuils ilc RPL may picdis-
posc io cloioidal ncovasculaiizaiion, paiiiculaily in cl-
dcily paiicnis.
Tlc OCTclangcs inpciilovcal iclangicciasia ilai wcic
dcsciilcd in oui sciics lui noi in ilc aiiiclc ly Gass and
llodi
1
aic icvcaling. Tlc liisi dcicciallc clangc is icii-
nal iliclcning, noi cvidcni clinically lui imagcdwiilOCT
(Iiguic 2). Tlis linding could consiiiuic a noninvasivc
way ol maling a diagnosis in cascs wlcic ilc iclangicc-
iaiic vcsscls aic occuli in naiuic. On OCT imaging, cys-
iic clangc ai ilc lovca is an innci lamcllai cysi loidcicd
aniciioily ly ilc innci limiiing mcmlianc (Iiguic 2). Lcc
ci al
22
also olscivcd ilis linding in a singulai casc icpoii
ol pciilovcal iclangicciasia. Wc lclicvc ilai ilc icim-
iailci ilan - is ilc mosi appiopiiaic dcsciip-
iion ol ilis alnoimaliiy sincc ii is liglly lilcly ilai ilc
innci and ouici iissucs aic dciivcd liom ilc ncuiocpi-
ilclium. Tlis clangc was picviously dcsciilcd as simu-
laiing a lamcllai iciinal lolc ly Gass and Owalawa,
11
wlo
did noi lavc ilc advaniagc ol OCT imaging. Tlc couisc
ol ilc visual siaius can lc lollowcd ly documcniing ilc
piogicssivc loss ol ilc ouici iciina wiil OCT, again non-
invasivcly. Tlc piogicssion ol ilc cysi is possilly ilc ic-
suli ol Mu llci ccll dcgcnciaiion and cysiic maculai dc-
gcnciaiion ai ilc lovca. Somc OCT imaging ol clinical
lcaiuics is noi ol piaciical valuc. Tlc ciysiallinc dcpos-
iis, loi cxamplc, wcic ioo small io inducc ploioicllcc-
iancc, and ilc pigmcni liguics simply cicaicd nonspc-
cilic suliciinal icllcciancc and posiciioi sladowing
(Iiguic 3l). Tlcsc lcsions aic ioo small io lc imagcd wiil
ilc icsoluiion ol ilc sysicm. Iuiilcimoic, OCT icgis-
iiaiion is also limiicd wiil icgaid io poini-io-poini oi
pixcl-io-pixcl coiiclaiion as wcll. Howcvci, Tialuccli ci
al
23
icpoiicd nonspccilic sladowing in a singlc casc ol
pciilovcal iclangicciasia liom RPL piolilciaiion.
Wc piclci io classily pciilovcal iclangicciasia in 2
siagcs: ilc nonpiolilciaiivc siagc wlcn ilcic aic cxuda-
iivc iclangicciasia and lovcal aiioply, and ilc piolilcia-
iivc siagc wiil ilc advcni ol SRN. In ilc nonpiolilcia-
iivc siagc, ilc mcclanismloi vision loss is ilc cxudaiivc
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clangc io a dcgicc lui piincipally ilc lovcal aiioply,
wlicl is lcsi moniioicd wiil OCT(Iiguic 2). In ilc pio-
lilciaiivc siagc, OCT is lcss lclplul, cxccpi io dcicci oc-
culi maculai dciaclmcnis (Iiguic 3). Wlcn suliciinal
ncwvcsscls lavc dcvclopcd, cysioid maculai cdcma, sul-
iciinal lcmoiilagc, and liliovasculai piolilciaiion aic
usually cvidcni clinically and angiogiaplically. Inilc pio-
lilciaiivc siagc, ilc piinciplc mcilod loi vision loss
clangcs liomlovcal aiioply io liliosis. Tlus, ilis simplc
siaging ol pciilovcal iclangicciasia las an anaiomical la-
sis and a lunciional signilicancc. Tlc oilci iypical clai-
aciciisiics ol pciilovcal iclangicciasia, sucl as pigmcni
plaqucs and ciysiallinc dcposiis, occui vaiially in ilc
couisc ol ilc discasc and may pcisisi wiiloui visual con-
scqucncc loi ycais.

Occlusivc iclangicciasia las lccn dcsciilcd picviously
ly Gass and llodi.
1
In ilcii icviscd classilicaiion, 7 ol
1+0 cascs wcic dcsciilcd in ilis gioup. Tlicc cascs
lad sysicmic vasculai occlusivc discasc and + lad
lamilial oculai cciclial occlusivc disoidcis. Iuiilci-
moic, occlusivc iclangicciasia las vciy liiilc io do wiil
iclangicciasia. Ii is an isclcmic lovcal discasc loidcicd
ly a lcw dilaicd capillaiics ilai aic mosi lilcly com-
pcnsaioiy clangcs icsponding io ilc capillaiy non-
pcilusion. Accoidingly, wc suggcsi ilai ilis iypc lc
omiiicd liom ilc maculai idiopailic iclangicciasia clas-
silicaiion. In oui opinion, ii is lcsi vicwcd as a pciilo-
vcal capillaiy nonpcilusion, as an oculai manilcsiaiion
ol sysicmic oi cciclial lamilial discasc.
In summaiy, wc pioposcd a ncwclassilicaiion ol IMT.
Tlc picvious and pioposcd classilicaiions aic in .
Typc 1 (ancuiysmal iclangicciasia) is a vaiiallc lui gcn-
cially unilaicial discasc sccn mosily in mcn and is an cxu-
daiivc iclangicciasia associaicd wiil micioancuiysms and
macioancuiysms ilai lcal inio ilc macula and somc-
iimcs lcyond. Tlcic is no pigmcni, ciysiallinc dcposii,
oi ncovasculaiizaiion in ilis iypc. Tlc lcalagc is asso-
ciaicd wiil cysiic clangc in ilc macula, clcaily docu-
mcnicd wiil OCT. Typc 2 (pciilovcal iclangicciasia) is
a lilaicial pciilovcolai iclangicciaiic discasc wiil no scx
picdisposiiion. Ii is limiicd io ilc pciilovcal aica and is
noi associaicd wiil ancuiysms oi cysioid lcalagc. In ilc
nonpiolilciaiivc siagc, ilcic is an cxudaiivc iclangiccia-
sis and lovcal aiioply. In ilc piolilciaiivc siagc, ilcic is
SRN and liliosis. Tlis simplc classilicaiion will lcad io
a lciici undcisianding ol ilc cniiiics ly oplilalmolo-
gisis and iciinal spccialisis. Ii will also assisi in icacl-
ing, in luiuic icscaicl, and lopclully in dcvcloping ncw
mcilods ol ilciapy.
Icliuaiy 1+, 2005; linal ic-
vision icccivcd May 2+, 2005; acccpicd Junc 10, 2005.
- Lawicncc A. Yannuzzi, MD, LuLs-
ilci T. Mciiz Rciinal Rcscaicl Ccnici, Manlaiian Lyc,
Lai, and Tlioai Hospiial, 210 L 6+il Si, Ncw Yoil, NY
10021 (vimnyaol.com).
-- Nonc.
Tlis siudy was suppoiicd ly ilc
Macula Ioundaiion, Inc, Ncw Yoil, NY.
Wc wisl io ilanl ilc iciinal spccial-
isis ol Viiicous-Rciina-Macula Consulianis ol NcwYoil,
NcwYoil (Yalc Iislci, MD, Jay Klancnil, MD, Joln So-
icnson, MD, and Riclaid Spaidc, MD) loi ilcii assis-
iancc in icciuiiing paiicnis loi ilis sciics.

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14. Triathi R, Ashton h. Electron microscoical study ol Coats' disease.

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Tlc ARCHIVLS will lc pullisling a ilcmc issuc on ocu-
lai gcnciics io piovidc an ovcivicw ol ilc siaius ol ilis
iapidly clanging licld. Tlc issuc will (1) includc ic-
vicws ol ilc gcnciics ol majoi cyc discascs, (2) piovidc
insigli inio ilc cuiicni appioaclcs and iools nccdcd io
cvaluaic lindings liomgcnciic siudics (cg, siaiisiical gc-
nciics and gcnciic cpidcmiology), (3) suggcsi lowilcsc
iools will lc uscd in ilc luiuic io advancc undcisiand-
ing ol ilc ciiology, pailogcncsis, and iicaimcni ol dil-
lcicni cyc condiiions, and(+) piovidc clinicians wiilsomc
guidclincs loi communicaiing gcnciic iisls io ilc avci-
agc paiicni. Tlc issuc will includc a comlinaiion ol in-
viicd papcis and papcis picscniing oiiginal icscaicl.
Lcslic Hyman, PlD, Jancy Wiggs, MD, PlD, lailaia
Klcin, MD, MPHandlailaia Ncmcsuic, PlDwill lc gucsi
cdiiois loi ilis spccial issuc. Wc inviic and cncouiagc
all invcsiigaiois io sulmii manusciipis dcsciiling oiigi-
nal oculai gcnciics icscaicl ly July 1, 2006. All manu-
sciipis sulmiiicd loi ilis issuc aic suljcci io an cxpc-
diicd pcci icvicw. Laily icccipi cnsuics ilc lcsi clancc
loi acccpiancc loi ilis spccial issuc. Acccpicd manu-
sciipis noi includcdinilis issuc will lc pullislcd inoilci
issucs ol ilc ARCHIVLS. Plcasc noic in ilc covci lciici ilai
ilc sulmission is loi ilc oculai gcnciics ilcmc issuc. Tlc
cxpccicd pullicaiion daic loi ilis issuc is Januaiy 2007.
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