935

Pictorial Essay

Sonography of the Eye in Children: Imaging Findings

G. EnrIquez1 , J. J. Gil-Gibernau2, V. Garriga1 , I. Ribes3, J.

Real-time sonognaphy is a well-accepted technique for evalu- lens. In many cases of congenital cataracts, the lens is
ating abnormalities of the eye. Most previous reports on sonog- spherically shaped, a condition called sphenophakia (Fig. 2).
naphy of the eye have been in adults [1]. In this pictorial essay, Primary vitreous is the embryonic vasculature of the eye and
we illustrate normal and abnormal findings of ocular sonogra-
supplies nutrients to the developing lens and retina during early
phy in children.
gestation. The hyaboid artery is the main arterial supply in this
network. Primary persistent hyperplastic vitreous (PPHV) is
characterized by incomplete absorption of the embryonic hya-
Technique
bid artery and hypemplasia of embryonic connective tissue [2].
Ocular sonognaphy was performed in 152 children ranging Leukoconia (an abnormal pupillary light reflection) and
from 1 month to 1 5 years old. Abnormal sonogmaphic find- micmophthalmia are the most common clinical findings. The
ings were found in 122. Examinations were performed with a diagnosis of this condition usually is made fundoscopically, but
7.5-MHz linear-array transducer on a model 1 28 Acuson unit when the patient has a lens opacity that precludes ophthalmo-
(Acuson, Mountain View, CA). Longitudinal and transverse scopic visualization of the retina, sonogmaphic examination is
views of each globe were obtained through the closed lid needed. Depending on the extent of the abnormality, a wide
with conventional coupling gel. A stand-off is a preferable variety of sonognaphic manifestations can be seen (Fig. 3).
method to improve resolution in the near field. Gain settings Most cases are unilateral. Bilateral cases can be associated
were adjusted to assess the anterior and posterior parts of with tnisomy 13, 15, 18, and 21 syndromes and Nommie’s dis-
the globe (Fig. 1). For cases in which sedation was required, ease,an X-Iinked recessive syndrome associated with bilateral
we performed ocular sonography with the patient under the Ieukoconia and microphthalmia (from PPHV), deafness, mental
general anesthesia that is normally used in young children retardation, and absence of the retinal ganglion cells.
and infants for ophthalmologic examination. In some cases, Coats’ disease (retinal telangiectasia) is a congenital retinal
we sedated our patients with 50-100 mg/kg of chlonal vascular anomaly characterized by telangiectatic retinal yes-
hydrate administered orally 30 mm before examination. sels and associated accumulation of lipoproteinaceous exudate
in the submetinal space. There is a 4 to 1 male predominance.
Sonognaphy and fundoscopy show typical findings (Fig. 4). The
Congenital Anomalies
lesion is usually unilateral, and the differential diagnosis
Congenital cataracts may vary from a few dustlike spots to includes netinoblastoma, PPHV, and netmolental fibmoplasia.
a completely dense white opacity of the lens. On sono- Coloboma is a congenital abnormality caused by disruption
grams, this latter form appears as a thickened wall of the of normal closure of the fetal optic cleft [3]. Cobobomas may

Received April 6, 1995; accepted after revision May 31 , 1995.

1 Radiology Department, Hospital Materno-Infantil VaIl d’Hebron, ps. VaIl d’Hebron, 119-129, 08035 Barcelona, Spain. Address correspondence to G. Enriquez.
2Ophthalmology Department, Hospital Materno-lnfantil VaIl d’Hebron, ps. Vail d’Hebron, 119-129. 08035 Barcelona, Spain.
3Radiology Department, Hospital Arnau de Vilanova, Ctra. de Huesca, s/n, Lleida, Spain.
AJR 1995;165:935-939 0361-803X/95/1654-935 © American Roentgen Ray Society
936 ENRIQUEZ ET AL. AJR:165, October 1995

Fig. 1 -Normal eye. Fig. 2.-Congenital cataract. Transverse sono-

A, Transverse sonogram of anterior part of globe. Lens is oval with anterior and posterior gram of right eye shows markedly echogenic
echogenic surfaces (arrows). Iris is seen as echogenic line on either side of lens (arrowheads). Vit- boundaries of lens characteristic of cataract.
reous chamber is anechoic. Compare with normal lens in Fig. iA. Lens is
B, Transverse sonogram after gain settings were adjusted to assess posterior structures. spherically shaped (spherophakia). This sono-
Echogenic area located behind globe corresponds to retrobulbar fat. Optic nerve is seen within fat graphic finding allows differentiation between
as hypoechoic structure extending posteriorly (arrows). Lateral muscles are also seen as hypo- congenital and acquired types of cataracts
echoic masses (arrowhead). because in latter, lens remains oval. Because cat-
erects preclude ophthaimoscopic examination of
globe, sonography is useful for excluding other
intraocular anomalies.

Fig. 3-3-year-old boy with bilateral primary

persistent hyperplastic vitreous.
A, Transverse sonogram of right globe shows
triangular retroiental echogenic area (arrow)
that represents hyperplastic primary vitreous.
Hypoechoic canal (white arrowheads) that cx-
tends posteriorly from center of ocular lens to
optic disk represents primitive hyaloid artery.
This artery is main arterial supply to developing
lens and retina during early gestation.
B, Transverse sonogram of left side shows
decreased size of globe. Vitreous chamber is full of
hyperechoic material representing embryonic yes-
culature and concurrent hyperpiasla of connective
tissue. Posterior margin of lens is thickened due to
associated cataract (arrow). In this eye, abnormal-
ity is much more severe than in other.

Fig. 4.-Coats’ disease in 4-year-old girl with

leukocoria.
A, Transverse sonogram of right eye shows ret-
mel detachment as two echogenic lines (arrows)
with characteristic v-shape due to fixation of ret-
ma at optic disk. Poor echoes (asterisk) in subreti-
nal space represent lipoproteinaceous exudate.
B, Ophthalmoscopic view clearly shows teiang-
iectatic retinal vessels with some aneurysms
(arrows). Huge proteinaceous exudate is also seen
in subretinal space, represented in yellow.
AJR:165, October 1995 SONOGRAPHY OF THE EYE IN CHILDREN 937

affect any part of the eye and may be complete on incomplete. Chorioretinitis in AIDS
On sonography, the optic nerve coboboma appears as a pos-
Chomioretinitis may complicate prolonged steroid treatment
tenor hemniation of the vitreous chamber extending posteriorly and may also be seen in immunocompromised patients. AIDS
as fan as the optic nerve itself. In 60-90% of cases the lesion must be recognized as a possible cause of chomioretinitis in
is bilateral. Localized retinal detachment may be seen in
children. Figure 8 emphasizes the sonogmaphic findings in one
some cases (Fig. 5).
of our patients with AIDS and chonioretinitis associated with
Many chromosomal disorders and syndromes have been
cytomegalovimus infection.
reported to be associated with coboboma (Fig. 6).

Intraocular Tumors
Intraocular Infectious Diseases
Retinoblastoma is the most common primary malignant
Intraocular inflammatory lesions may affect the retina tumor in children and most often occurs before the age of 5
(retinitis) on the chomoids (choroiditis), but usually the inflam- years. Retinoblastomas may be unilateral (70%) or bilateral
matomy process affects both ocular structures and is known (30%). Forty-five percent of retinoblastomas are caused by a
as chomiometinitis. Chonioretinitis may be infectious, trau- dominant autosome. All bilateral cases are hereditary. Retino-
matic, autoimmune, or toxic. In most cases, diagnosis is blastoma appears as a solid echogenic mass on sonographic
based on clinical findings; sonographic studies are normal. studies. Highly echogenic foci, representing calcifications, are
found in more than 95% of cases (Fig. 9). Either exophytic
growth into the vitreous on submetinal growth will result in sec-
Ocular toxocariasis
ondary retinal detachment.
Toxocara canis is a helminthic nematode that produces the Meticulous sonogmaphic examination of both the ipsilateral
lesions of toxocaniasis in children. Diagnosis is based on the and contmalateral globes should be performed to rule out mul-
presence of the specific enzyme-linked immunosorbent assay ticentnic or bilateral retinoblastoma. In our experience, sonog-
testfor Toxocara. Two main forms oftoxocamiasis have been nec- raphy is better than CT or MR imaging for bilateral implants,
ognized. The acute form, or diffuse endophthalmitis, typically but fundoscopy is superior to sonography for studying bilateral
affects children between the ages of 2 and 9 years. This form is lesions. CT and MR imaging are recommended for evaluating
nearly always unilateral and is characterized by exudation that metrobulbar and intracranial tumor extension.
affects the vitreous. The chronic form is characterized by a Retinal-pigment epithelium hamartoma is a congenital,
chorionetinal granuboma. This form is usually seen in older chil- usually unilateral, lesion that affects the retina and pigment
dren on adolescents. Sonogmaphically, it appears as a subretinal epithelium. The diagnosis is usually made fundoscopically,
echogenic mass. Vitreous bands on membranes between the but sonographic examination is recommended for evaluating
mass and the posterior pole are also seen [4] (Fig. 7). the size of the lesion and for the follow-up. Findings on sono-

‘ r

Fig. 5.-Coloboma of left eye and retinal detachment. Fig. 6-10-year-old girl with juvenile nephro-
A, Transverse sonogram of left globe shows coloboma defect as excavation in area of optic disk nophthisis and bilateral coloboma. Nephronophthl-
(black arrow). Retinal detachment is clearly visible as echogenic line on right side (white arrow). sis (renal meduliary cystic disease) was diagnosed
Retina was fixed during gentle eye movements from left to right. by renal biopsy at age 8 years. Ocular anomalies
B, Fundus photograph of same patient shows retinal detachment (arrow) and coloboma defect such as retinitis pigmentosa, coloboma, end pri-
(arrowhead). mary persistent hyperpiastic vitreous can be asso-
ciated with this renal abnormality. Transverse
sonogram of left eye shows cystic structure
behind globe (black arrow) that contains some
echogenic material and corresponds to coloboma-
tous cyst. Narrow communication between cyst
and vitreous can be seen (white arrow). Similar fee-
tures were found on right side.
938 ENRIQUEZ ET AL. AJR:165, October 1995

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Fig. 7-15-year-old boy with ocular toxocariesis. Fig. 8-10-year-old girl with AIDS and cytomeg- Fig. 9-2-year-old boy with unilateral retino-
Transverse sonogram of right globe shows alovirus infection. Transverse sonogram of right blastoma. Transverse sonogram of left eye
echogenic mess (black arrow) suggestive of toxo- eye shows two echogenic masses (arrowheads) shows large echogenic mass filling large por-
cerel grenulome, end enzyme-linked immunosor- suggestive of granulomas. Tented traction retinal tion of vitreous chamber. Hyperechogenic foci
bent assay test was positive for Toxocara canis. detachment is also seen (arrows) with cheracteris- with acoustic shadowing inside mass corre-
Echogenic lineal structure is seen attached to mass tic V-shape configuration. spond to punctate calcifications (arrows).
(white arrows) that corresponds to vitreous mem-
brene. Vitreous membranes are common in patients
with ocular toxocariasis. imaging helps exclude ret-
inoblestoma. Ninety percent of retinobiestomas
have calcificatlons in sonogrephic studies, which
are not identified in toxocaral grenulomas.

Fig. 1 0.-Retinal-pigment epithelium hamar- Fig. 1 1 -Bilateral optic disk drusen. Transverse sonogram of eyes shows echogenic foci at optic
tome. Sonogram of left globe in 5-year-old boy nerve heads (arrows). Lesion remained attached to optic disk during gentle movements of eye from
shows noncelcified echogenic lesion (arrow) left to right. Because optic disk drusen may be seen incidentally, knowledge of its sonographic
located near pepille. Ophthalmoscopically, appear- appearance is important.
ance of retinal hamertoma is characteristic, but
sonographic studies are useful for evaluate size of
lesion end follow-up.

graphic studies can be characteristic (Fig. 10). Treatment is lated reasons. On fundoscopy, they may mimic papilledema.
not required. The sonographic appearance is characteristic (Fig. 11).
The two previous tumors should not be confused with
optic disk drusen. This is a benign condition that consists of
deposits of hyaline calcific material within the head of the Ocular Trauma
optic nerve. It is frequently bilateral and is a dominant trait. Ocular trauma is a common problem in children. Sonogra-
Despite the fact that drusen will cause visual problems, most phy is indicated whenever opacification of the media pre-
will be found incidentally on sonographic studies for unne- vents adequate clinical examination.
AJR:165, October 1995 SONOGRAPHY OF THE EYE IN CHILDREN 939

Fig. 1 2.-VItreous hemorrhage. Transverse

sonogram through globe. Patient had motor vehi-
dc accident 3 days before examination. Vitreous
contains some echogenic material that repre-
sents diffuse hemornhage(asterlsk). Sonographic
appearance of vitreous hemorrhage varies from
homogeneous to heterogeneous depending on
time of lesion.

Fig. 13.-Foreign bodies. Ocular sonogram

after motor vehicle accident shows echogenic
linear structure in posterior part of vitreous,
which proved to be sliver of glass (black arrow).
Anteriorsonolucent areas correspond to soft-tis-
sue wound (asterisk) and to lens (white arrow).
12 13

Vitreous hemorrhage is the most common sonographic REFERENCES

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sonognaphy, they are seen as echogenic lesions (Fig. 13). lation.AJR 1994:162:437-440
3. Munk PL. Coloboma: evaluation with real-time sonography. AJR 1990:154:
Extensive reverberation artifact is visualized in cases of
391-392
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