sonography of eye in children

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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