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R ece ive d A pril 6 , 1 995 ; accep ted a fte r re v is ion M ay 31 , 1995 .1 R ad io log y Depa rtm en t, H o sp ita l M ate rno -In fan til V a Il d ’H eb ron , ps . Va Il d ’H e bro n , 1 19 -12 9 , 08 03 5 B arce lo na , S pa in . A dd res s co rre sp on den ce to G . E nriqu ez .
2O ph th alm olo gy D ep ar tm en t, H osp ita l M ate rn o- ln fa ntil V aIl d ’H eb ro n, ps . V ail d ’H eb ro n, 11 9-12 9. 08 035 B arce lo na , S pa in .
3R ad io log y D epa rtm en t, H osp ita l A rn au de V ilan ova , C tra . de H ue sca , s /n , L le ida , S pa in .
93 5
AJ R 1995;165 : 935 - 939 0361 -80 3X /95 /1 65 4-9 35 © Am e rica n R oen tgen Ra y So c ie ty
P ic to ria l E ssay
Sonog raphy o f th e E ye in C h ild ren : Im ag ing F ind ings
G . En r Iquez1 , J . J . G il-G ibe rnau2 , V . G a rr iga1 , I. R ibes3 , J .
R eal-t im e sonognaphy is a w ell-accep ted techn iq u e fo r eva lu -
a tin g abno rm a lities o f th e eye . M os t p re v ious repo rts on sonog -
naphy o f the eye have been in adu lts [1 ]. In th is p ic to ria l essay ,
w e illus tra te norm al and abno rm al find ing s o f o cu la r sonog ra -
p hy in c hild re n.
Techn ique
O cula r sonognaphy was pe rfo rm ed in 152 ch ild ren rang ing
from 1 m on th to 1 5 yea rs o ld . A bnorm a l sonogm aphic find -
ings w e re found in 122 . E xam ina tions we re pe rfo rm ed w ith a
7 .5 -M H z linea r-a rra y transduce r on a m ode l 1 28 A cuson un it
(A cuson , M oun ta in V iew , CA ). Long itud ina l and transve rse
v iew s o f each g lobe we re ob ta ined th rough the c lo sed lid
w ith conven tiona l coup ling ge l. A s tand -o ff is a p re fe rab le
m e thod to im p rove reso lu tion in the nea r fie ld . G a in se ttings
w e re ad jus ted to assess the an te rio r and pos te rio r pa rts o f
the g lobe (F ig . 1 ). F o r cases in w h ich seda tion w as requ ired ,
w e pe rfo rm ed ocu la r sonog raphy w ith the pa tien t unde r the
genera l anes thes ia tha t is no rm a lly used in young ch ild ren
and in fan ts fo r oph tha lm o log ic exam ina tion . In som e cases ,
w e seda ted ou r pa tien ts w ith 50 -100 m g /kg o f ch lona l
hydrate administered orally 3 0 mm before examination.
C ong en ita l A no ma lies
Congen ita l c a tarac ts m ay va ry from a few dus tlik e spo ts to
a com p le te ly dense wh ite opac ity o f the lens . O n sono -
g ram s, th is la tte r fo rm appea rs as a th ickened w a ll o f the
lens . In m any cases o f congen ita l c a tarac ts , the lens is
sphe rica lly shaped , a cond ition ca lled sphenophak ia (F ig . 2 ).
P rim a ry v itreous is the em b ryon ic vascu la tu re o f the eye and
supp lie s nu trien ts to the deve lop ing lens and re tina du ring ea rly
ges ta tion . The hyabo id a rte ry is the m a in a rte ria l supp ly in th is
ne tw o rk . P rim a ry pe rs is ten t hype rp la s tic v itreous (PPHV ) is
cha rac te rized by in com p le te abso rp tion o f the em b ryon ic hya -
b id a rte ry a nd h ypem p las ia o f em b ryo n ic co nne c tiv e tiss ue [2 ].
Leukocon ia (an abno rm al pup illa ry ligh t re fle c tion ) and
m icm oph tha lm ia a re the m os t comm on c lin ica l find ings . T he
d iagnos is o f th is cond ition usua lly is m ade fundoscop ica lly , bu twhen the pa tie n t h as a le n s opac ity th a t p rec lu de s oph th a lm o -
scop ic v isua liza tion o f the re tina , sonogm aph ic exam ina tion is
needed . Depend ing on the ex ten t o f the abno rm a lity , a w ide
va r ie ty o f sonognaph ic m an ife s ta tions can be seen (F ig . 3 ).
M os t cases a re un ila te ra l. B ila te ra l cases can be assoc ia ted
w ith tn isom y 1 3 , 15 , 1 8 , and 21 synd rom es and N omm ie ’s d is -
ease , an X -Iin ked recess ive synd rom e assoc ia ted w ith b ila te ra l
Ieukocon ia and m ic roph tha lm ia (from PPHV ), dea fness , m en ta l
re ta rda tion , and absence o f the re tina l gang lion ce lls .
C oa ts ’ d isease (re tina l te lang iec tas ia ) is a congen ita l re tina l
v ascu la r anom a ly cha rac te r iz ed by te lang ie c ta tic re tina l yes-
se ls and assoc ia ted accum ula tion o f lip op ro te inaceous exuda te
in the subm e tina l space . T he re is a 4 to 1 m a le predom inance .
S onognaphy and fundoscopy show typ ica l find ings (F ig . 4 ). T hele s ion is usua lly un ila te ra l, and the d iffe ren tia l d iagnos is
in c ludes ne tinob la s tom a, P PH V, and ne tm olen ta l fibm op las ia .
C o lobom a is a congen ita l abno rm a lity caused by d is rup tion
o f no rm a l c lo su re o f the fe ta l op tic c le ft [3 ]. C obobom as m ay
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ENR IQUEZ E T AL .36 A JR :16 5 , O c to be r 1 99 5
F ig . 1 -N orm al eye .
A, Transve rse so nogram of an te rio r p ar t o f g lo be . L ens is o va l w ith an terio r an d pos ter io r
e ch og en ic s ur fa ce s ( a r rows) . Ir is is se en a s ec ho ge nic lin e on e ithe r s id e o f le ns ( a r rowheads) . Vit -
re ou s c ha m be r i s a n ec h oi c.
B , T ransve rs e sonog ram a fte r g a in se tt in g s w ere ad jus ted to assess poste r io r s tru c tu re s .
Echogen ic are a loc a te d beh in d g lo be correspond s to re tro bu lb ar fa t . O p tic ne rve is s een w ith in fa t
a s h y po e ch o ic s tru ctu re e xte nd ing p os ter io r ly ( a r rows) . L atera l m u sc les a re a lso se en as h ypo -
e ch o ic m asses ( a r rowhead ) .
F ig . 2 .-C o ng en ita l ca ta rac t. T ra ns ve rse sono -
g ram o f righ t ey e sh ow s m ark ed ly echogen ic
boundaries o f len s cha ra c te ris tic o f ca ta ra c t.
Com pare w ith n orm a l len s in F ig . iA . Lens is
sp her ica lly s hap ed (sph ero ph ak ia ) . T h is sono -
g raph ic fin d in g a llow s d iffe re n tia tio n be tween
co ngen ita l an d acq u ired typ es o f c a ta ra c ts
be cau se in la tte r , len s rem a ins ova l. B ecause ca t-
e re c ts p re c lu de o ph tha im oscop ic e xam ina t ion o f
g lo be , son og raph y is us e fu l fo r e xc lud in g o the r
i ntr ao cu la r a no m ali es .
F ig . 3 -3 -yea r-o ld bo y w ith b ila te ra l p rim a ry
p ers is ten t h yp erp la stic v it reo us .
A , Tran sv ers e sonog ram o f righ t g lo be sh ow s
tria ng u la r re tro ie n ta l ech og en ic a rea ( a r row)
tha t rep res en ts hype rp las tic p rim ary v itreo us .
H ypoecho ic c ana l ( wh ite a rr ow he ad s) tha t cx -
ten ds pos ter io r ly from cen ter o f ocu la r len s to
o p t ic d is k rep res en ts p rim itive h ya lo id a rte ry .
Th is ar tery is m ain a rte ria l su pp ly to de ve lop in g
len s and re tin a du rin g e arly g es ta tion .
B , T ransverse sonog ram of le ft s id e shows
d ec re ased s ize o f g lo be . V it reo us cham ber is fu ll o f
h yp erech o ic m ater ia l re p resen t ing em bryo n ic y es -
c u la tu re and con cu rre n t hype rp ias la o f co nnect ive
tiss ue . P os te r io r m arg in o f len s is th icke ne d due to
a ss oc ia te d c ata ra ct ( a r row) . In th is e ye , a bn or ma l-
i ty is m uch m ore seve re tha n in o th e r.
F ig . 4 .-C o ats ’ d ise as e in 4 -y ea r-o ld g irl w ith
leukocor ia .
A , T ran sv erse sonogram of righ t ey e shows re t-
m e l de tachm en t a s tw o ec ho ge nic line s ( a r rows)
w ith charac te r is t ic v -sh ap e du e to f ixa tio n o f re t-
m a a t op tic d is k . P oo r echo es ( as ter isk ) i n s u b re t i-
n a l s pa ce rep re sen t lipo p ro te in ac eou s exu date .
B, O ph th a lm oscop ic v iew c le arly sh ow s te ian g -
iec tat ic re tin a l ve sse ls w ith som e aneury sm s
( a r rows) . Huge p ro te in ac eo us exud ate is a lso seen
in su bre tina l s pa ce , rep re sen ted in ye llow .
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‘ r
F ig . 5 .-C o lo bom a o f le ft ey e and re tina l de tachm en t.
A , T ran sve rs e so no gram o f le ft g lo be sho ws co lo bo ma d efe c t a s excava tio n in a re a o f op tic d is k
(b la ck a rr ow ). R etin a l d e tachm en t is c lea rly v is ib le as e ch ogen ic line o n rig h t s ide (w hite a rro w).
Re tina w as fix ed d uring g en tle ey e m ovem en ts from le ft to rig h t .
B , Fundus pho to g raph o f sam e pa tien t s hows re tin a l de tachm en t ( a r row) and co lo bom a d efe c t
( a r rowhead ) .
A JR :16 5 , O c tob e r 1 995 SO NOGRAPHY OF THE EYE I N CH ILDREN 937
a ffe c t any pa rt o f the ey e and m ay be com p le te on inc om p le te .
On sonog raphy , the op tic ne rve cobobom a appea rs as a pos-
teno r hem n ia tion o f the v itreous cham be r ex tend ing pos te rio r ly
as fan as the op tic ne rve itse lf. In 60 -90% o f cases the le s ion
is b ila te ra l. Loca liz ed re tina l de tachm en t m ay be seen in
s om e c as es (F ig . 5 ).
M any ch rom osom a l d is orde rs and syndrom es have been
repo rted to be assoc ia ted w ith cobobom a (F ig . 6 ).
In trao cu la r In fec tio us D iseases
In traocu la r in flamm a to ry le s ions m ay a ffe c t the re tina
(re tin itis ) on the chom o ids (cho ro id itis ) , bu t usua lly the in flam -
m a tom y p rocess a ffe c ts bo th ocu la r s tru c tu res and is know n
as chom iom e tin itis . C hon io re tin itis m ay be in fe c tious , trau -
m atic , a u to im mune , o r tox ic . In m ost cases , d iagnos is is
based on c lin ic a l find ings ; sonog raph ic s tud ie s a re no rm a l.
O cu la r to xo ca ria sis
T o x o ca ra c a n is is a he lm in th ic nem a tode tha t p roduces the
le s ions o f to xocan ias is in ch ild ren . D iagnos is is based on the
p resence o f the spec ific enzym e -lin ked im m unoso rben t assay
tes t fo r T o x o c a ra . Tw o m a in fo rm s o fto xocam ias is have been nec-
ogn ized . The acu te fo rm , o r d iffu se endoph tha lm itis , ty p ica lly
a ffe c ts ch ild ren be tw een the ages o f 2 and 9 yea rs . T h is fo rm is
nea rly a lw ays un ila te ra l and is cha rac te r ized by exuda tion tha t
a ffe c ts the v itreous . T he ch ron ic fo rm is cha rac te r ized by a
cho rione tina l g ranubom a . T h is fo rm is usua lly seen in o lde r ch il-
d ren on ado lescen ts . S onogm aph ica lly , it appea rs as a sub re tina l
e chogen ic m ass . V itreous bands on m em branes be tw een the
m ass and the pos te r io r po le a re a lso seen [4 ] (F ig . 7 ).
C h o rio re tin itis in A ID S
C hom io re tin itis m ay com plica te p ro longed s te ro id trea tm en t
and m ay a lso be seen in imm unocom p rom ised pa tien ts . A ID S
m ust b e re cogn ize d as a po ss ib le cau se o f ch om io re tin itis in
ch ild ren . F igu re 8 em pha s iz es the sonogm aph ic find in g s in o ne
o f ou r pa tien ts w ith A IDS and chon io re tin itis assoc ia ted w ith
c yto me ga lo vim us in fe ctio n.
In traocu la r Tum ors
R e tinob la s tom a is the m os t common p rim a ry m a lignan t
tum or in c h ild ren and m os t o ften oc cu rs b e fo re th e age o f 5
yea rs . R e tinob la s tom as m ay be un ila te ra l (70% ) o r b ila te ra l
( 3 0% ) . Fo rty -five pe rcen t o f re tinob la s tom as a re caused by a
dom inan t au tosom e . A ll b ila te ra l c ases a re he red ita ry . R e tino-
b la s tom a appea rs as a so lid echogen ic m ass on sonog raph ic
s tud ie s . H igh ly echogen ic fo c i, rep resen ting ca lc ific a tions , a re
fou nd in m o re tha n 95% of ca se s (F ig . 9 ). E ith e r e xoph y tic
g row th in to the v itreous on subm e tina l g row th w ill re su lt in sec-
o nd ary re tina l d eta ch me nt.
M e tic u lous sonogm aph ic exam ina tion o f bo th the ip s ila te ra l
and con tm a la te ra l g lobes shou ld be perfo rm ed to ru le ou t m u l-
t icen tn ic o r b ila te ra l re tin ob las tom a . In ou r expe rie nce , s onog-
ra ph y is be tte r th an CT or M R im ag ing fo r b ila te ra l im p lan ts ,
bu t fu ndo scopy is su pe rio r to so nography fo r s tud y in g b ila te ra l
le s ions . C T and M R im aging a re recomm ended fo r eva lua ting
m etrobu lba r and in tra cran ia l tum or ex tens ion .
R e tina l-p igm en t ep ithe lium ham a rtom a is a congen ita l,
u sua lly un ila te ra l, le s ion tha t a ffe c ts the re tina and p igm en t
ep ithe lium . T he d iagnos is is usua lly m ade fundoscop ica lly ,
bu t sonog raph ic exam ina tion is recomm ended fo r eva lua ting
th e s iz e o f the le s io n and fo r the fo llow -up . F in d in gs on sono -
F ig . 6 -10 -ye ar-o ld g ir l w ith ju ven ile nep hro -
n op hth is is a nd b ila te ra l c olo bo ma . N ep hro no ph th l-
s is ( re n al medu l ia r y cy stic d ise as e) w as d ia gn os ed
by ren a l b iop sy at age 8 ye ars . O cu la r a nom a lie s
such as re tin it is p igm en tos a , c o lo bom a, end pri-
ma r y p ers is te nt h yp erp ias tic v itreo us c an b e a sso -
c iated w ith th is re na l ab no rm ality . T ran sv ers e
sonog ram of le ft ey e show s cys tic s tru c tu re
b eh ind g lo be (b lac k arrow ) tha t co n ta in s som e
e ch og en ic m ate ria l an d co rre sp onds to co lo bom a-
tou s cy st. N arrow com mun ic a t ion be tween c y s t
a nd v it re o us can be se en ( wh ite a rr ow ). S im ila r fe e-
tu res w ere fo und on rig h t s ide .
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- - -‘ . , . , ; ,
. - :_ -
___ ._L ’ ,
F ig . 8 -1 0 -yea r-o ld g irl w ith A ID S and cytom eg -
a lo v irus in fec tio n . T ra ns ve rse sonog ram of rig h t
eye sh ow s tw o ech og en ic m asses ( a r rowheads)
su ggest ive o f g ranu lom as . T en ted tra c t ion re tina l
de tachm en t is a lso seen ( a r rows) w i th c h e ra c te r is -
tic V -s ha pe c on fig ur atio n.
F ig . 9 -2 -ye ar -o ld b oy w ith un ila te ra l re tin o -
b la s tom a. T ran sv erse so nogram o f le ft ey e
sh ow s large e ch og en ic m ass fi llin g la rge p or -
tion o f v itre ou s ch am be r. H ype re chogen ic foc i
w ith acou st ic sh ad ow in g in s ide m ass co rre -
s po nd to p un cta te c alc if ic atio ns ( a r rows) .
op tic d isk d rusen . T h is is a ben ign cond ition tha t cons is ts o f
depos its o f hya line ca lc ific m a te ria l w ith in the head o f the O cu la r T raum a
op tic ne rve . It is frequen tly b ila te ra l and is a dom inan t tra it.
D esp ite the fac t tha t d rusen w ill cause v isua l p rob lem s , m os t
w ill b e found inc iden ta lly on sonog raph ic s tud ie s fo r unne -
93 8 ENR IQUEZ ET AL . A JR :16 5 , O cto be r 1 995
F i g. 7 - 15 -y e a r- o ld bo y w it h o cu la r to xo ca rie sis .
Transve rse sonogram o f r igh t g lo be show s
echogen ic me s s (b la ck a rr ow ) sug ges tive o f tox o-
c ere l g ren u lom e, end enzym e- lin ked im munoso r-
ben t as say te s t w as pos itive fo r To xo ca ra ca nis .
E cho ge nic lin ea l struc tu re is seen attached to m ass
(w hite a rro ws ) tha t c o rrespond s to v it reo us m em -
brene . V itre ou s m em bran es are com mon in p at ien tsw ith ocu la r toxoca ria s is . im ag in g he lps ex c lu de re t-
in ob les tom a. N in e ty p ercen t o f re tin ob ies tom as
h ave ca lc ific a tlo ns in sonog rep h ic s tud ies , w h ich
a re no t id en tified in tox oc ara l g re nu lo ma s.
F ig . 1 0.-R e tin a l-p igm en t ep ith e lium ham ar- F ig . 1 1 - B ila te ra l o p t ic d is k d ru sen . T ra ns ve rse sonog ram of ey es shows echogen ic foc i a t o p tic
tom e . So nogram o f le ft g lo be in 5 -y ea r-o ld bo y ne rve h ead s ( a r rows) . L es ion rem a ined a tta ch ed to op tic d isk du rin g gen t le m ovem en ts o f e ye from
show s nonce lc ifie d echogen ic le s ion ( a r row) le f t to rig h t. B ecause op tic d isk d ru sen m ay be seen in c id en ta lly , kn ow led ge o f its son og raph ic
lo ca te d n ea r pep ille . O ph tha lm oscop ic a lly , a ppear - a pp earan ce is im po rta n t.
a nc e o f re t ina l ham ertom a is cha ra c te ris tic , bu t
s on og raph ic s tud ies are us efu l fo r e va lua te s iz e o f
le si on e nd fo llo w- up .
graph ic s tud ie s can be cha rac te ris tic (F ig . 10 ). T rea tm en t is
n ot re qu ire d.
T he tw o pre v iou s tum o rs shou ld no t b e con fused w ith
la ted rea sons . O n fu ndo scopy , the y m ay m im ic p ap ille dem a .
The sonog raph ic appea rance is cha rac te ris tic (F ig . 11 ).
O cu la r traum a is a comm on p ro b lem in ch ild re n . S onog ra -
p hy is in d ica te d w hene ve r o pac ifica tio n o f th e m ed ia p re -
ven ts adequa te c lin ica l e xam ina tion .
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12 13
A JR :1 65 , O ct ob er 1 99 5 SONOGRAPHY O F THE EYE IN CH ILDREN 939
F ig . 1 2.-V Itreou s h em orrhage . T ran sv erse
sonogram th ro ug h g lo be . P atien t h ad m oto r veh i-
dc a cc id en t 3 d ay s b ef or e e xa m in at io n. V it re ou scon ta ins som e echogen ic m ate ria l th at re pre -sen ts dif fuse h em o rn ha ge (a ste rls k) . S o no gr ap hic
appea rance o f v itr eo us h em o rr ha ge v ar ie s f ro mhom ogeneous to hete rogeneous depend ing on
t im e o f les io n.
F ig . 1 3 .-F o re ign bod ie s . O cu la r sonog ram
a fte r m oto r v eh ic le a cc id en t s ho ws e ch og en iclin e a r s tru c tu re in poster io r p ar t o f v itreous ,
w hic h p ro ve d to b e s liv er o f g la ss (b la ck a rro w).
An te rio r s on o lu cen t a rea s co rrespond to so ft-t is -
su e w ou nd ( as ter isk ) an d to len s (w hi te a rr ow ).
V itreous hem o rrhage is the m ost comm on sonog raph ic
find ing in seve re cases o f ocu la r traum a (F ig . 1 2). In c reas ing
the ga in se tting o ften he lps de tec t acu te hem o rrhages ,
wh ich a re o ften on ly m in im a lly echogen ic .
F ore ign bod ies m ay be found in pene tra ting in ju rie s . O n
sonognaphy , they a re seen as echogen ic le s ions (F ig . 13 ).
E x tens ive reve rbe ra tion a rtifa c t is v isua lized in cases of
m e ta llic ob je c ts . T he p resence o f a im in the eye m ay p reven t
v isua liza tion o f a poss ib le ocu la r le s ion .
REFERENCES
1 . McN icho las MM J , B rop hy D P , P ow er W J, G riff in JF . O cu la r s on og rap hy .
AJ R 1994:163:921-926
2 . K as te S C, Jen k ins JJ , M eyer D , F on ta ne s i J , P ra tt C HB . P ers is ten t hy pe r-
p las t ic p r im a ry v itre ous o f th e eye : im ag ing fin d in gs w ith pa tho lo g ic co r re -
lat ion. A JR 1994 :162 :437 -440
3 . M un k P L. C olob om a: eva lu ation w ith re al-ti me s on og ra ph y. AJ R 1990 :154 :
391-392
4 . W an W L, C ano M R . P ince KJ , G ree n RL . E ch og rap h ic ch a rac te ris tic s o f
ocu la r toxoca r ias is . O phtha lmo logy 1991:98 : 28 - 32