8
Ultrasonography in
Orbital Differential Diagnosis
Amin M. Nasr and Grace Abou Chacra
U
ltrasonography is a noninvasive imaging tech-
nique that is essential in the evaluation of or-
bital diseases. Ophthalmic ultrasonography
employs high-frequency sound waves that provide the
high resolution required for ocular diagnosis. A lower
frequency of 8 MHz is used to obtain the penetration
needed to reach the orbital apex.1 The orbit is evalu-
ated with A- and B-mode scanning (Figure 8.1). Lesions
located in the posterior two thirds of the orbit are de-
tected through a transocular examination; lesions in
the anterior third of the orbit are detected through a
paraocular examination.2,3
The acoustic inner texture of an orbital lesion can
be correlated with its histologic features. The contents
of a normal orbit and lacrimal fossa show high re-
flectivity and marked sound attenuation owing to the
presence of large collagenous connective tissue sep-
tae, fat globules, blood vessels, and nerves.4,5 Most or-
bital pathologies display less coarse and heterogeneous
structures than the normal orbit, hence, usually have
lower reflectivity. This is in contrast to intraocular
pathology, which usually shows higher reflectivity
than the normal standard baseline displayed by the
clear vitreous body.
ASSESSMENT OF ORBITAL LESIONS
Primary Orbital Lesions
CYSTIC LESIONS
EPITHELIAL AND DERMOID CYSTS
The most common developmental cysts occurring in
the orbital and periorbital region and having a predilec-
tion for the superotemporal quadrant, are the epithelial
and dermoid cysts. Dermoid cysts contain one or more
epidermal appendages such as hair follicles, sweat
glands, and sebaceous glands; epithelial cysts contain
only stratified squamous cell epithelium. Echographi-
cally, epithelial and dermoid cysts appear on B scans as
smoothly rounded, echolucent lesions with good sound
transmission (Figure 8.2A,B). However, some dermoid
cysts show low internal amplitude echoes depending
on the number of hair shafts and other appendages
within the lesion, which invariably cause partial sound
attenuation. On A scan, epithelial cysts are low reflec-
tive. Dermoid cysts are medium to low reflective with
occasional high reflective spikes that indicate the pres-
ence of certain coarse structures such as fine hairs or
cartilaginous remnants.4,5
CONGENITAL CYSTIC EYE
Congenital cystic lesions contain protrusions through
defects present in the walls of microphthalmic eyes.
A congenital cystic eye results from failure in the in-
vagination of the primary optic vesicle and lack of dif-
ferentiation into its adult components. Echographi-
cally, the cystic portion shows typical roundish,
echolucent B-scan characteristics with very low in-
ternal reflectivity on A scan. The microphthalmic
globe, on the other hand, is highly dense on B scan
with marked shadowing. A-scan echography shows a
high reflective structure (typical of condensed tissue
structures) with marked sound attenuation almost
consistent with calcific structures.
HEMATOCELE
A hematocele is a cystic lesion (Figure 8.2C,D) that
results from spontaneous accumulation of blood. In-
variably it occurs without preexisting orbital vascular
disease.
VASCULAR LESIONS
INFANTILE HEMANGIOMA
A benign vascular tumor (Figure 8.3), infantile hem-
angioma usually appears during the first few months
of life. The majority of these lesions enlarge in size
within the first 2 years and in approximately 70% of
the cases undergo spontaneous involution by the age
of 7 years. The tumor is generally unilateral (although
bilaterality has been reported),6 and it occurs most
commonly in the superior nasal quadrant in otherwise
healthy children.
CAVERNOUS HEMANGIOMA
The most common primary benign orbital tumor of
adults (Figure 8.4A–C), cavernous hemangioma usu-
73
74 PART TWO: DIAGNOSIS OF ORBITAL TUMORS

FIGURE 8.1. Transocular and paraocular display of normal orbit.
A-scan transocular examination of a normal orbit (A, bottom) re-
veals the following echospikes from left to right: the initial spike
(I), which has no clinical significance and represents echoes gener-
ated at the tip of the probe (dead zone of the ultrasound probe); the
baseline, which is a horizontal line and represents the vitreous cav-
ity (V); the ocular wall spikes (W), which are high reflective echo-
spikes; and the orbital spikes (O), which are multiple high reflec-
tive echo spikes (⬃100%) with marked attenuation because of the
coarse, dense structures within the orbit. (B) B-scan transocular ex-
amination of a normal orbit reveals the following areas from left to
right: an echogenic area (initial line, I), which has no clinical sig-
nificance and represents echoes at the tip of the probe (dead zone
of the ultrasound probe); a clear or echolucent area representing the
vitreous cavity (V); and an echogenic area that represents the pos-
terior ocular wall and the orbital tissues (O) behind it. This normal
retrobulbar echo pattern is derived from the orbital fat globules,
which are triangular; the pattern is indented by a V-shaped, echolu-
cent area that represents the optic nerve (ON). The display in
paraocular examination (A, top) is similar to that of transocular ex-
amination, except for the presence of the cystic structure of the
globe. However, the echographic criteria are comparable.

ally appears in the third to fifth decades of life. The sulting in slowly progressive proptosis of the globe.
tumor is characteristically unilateral and solitary, al- On gross examination, cavernous hemangiomas are
though multifocal lesions have been reported.7,8 It is well-encapsulated, round to ovoid masses with violet
most commonly found within the muscle cone, re- hue reflecting the stagnation of poorly oxygenated

FIGURE 8.2. Cystic lesions (A,B)

dermoid cyst and (C,D) hemato-
cele. (A) Clinical photograph of a
child with right anterior, medial
orbital mass. (B) Transocular A
scan shows an anterior low
reflective lesion (L) shown within
smooth high reflective echo spikes
that delineate the anterior (a) and
the posterior (p) wall of the cystic
structure. V, vitreous cavity; W,
ocular wall spike; O, orbital tissue
spikes. (C) Axial CT scan shows
well-delineated round mass (M)
in the right orbit. (D) B scan (top)
displays a round echolucent orbital
lesion (L). A scan (bottom) shows
low reflectivity of the lesion (L)
with high posterior surface spike
(P) from the cyst wall. Occasion-
ally, there are moderately high
reflective, dispersed spikes repre-
senting lines of clotted blood. I,
initial spike; V, vitreous cavity;
S, sclera or ocular wall; O, orbital
tissue.
 CHAPTER 8: ULTRASONOGRAPHY IN ORBITAL DIFFERENTIAL DIAGNOSIS
blood within the tumor. Microscopically, the tumor
consists of large, dilated venous spaces lined by thin,
flattened endothelial cells, along with pericytes and
smooth muscle cells that are separated by irregular fi-
brous connective tissue septae.
LYMPHANGIOMA
Lymphangioma is a benign vascular tumor diagnosed
in early childhood (Figure 8.4D–F). Unlike infantile
hemangioma, lymphangioma enlarges progressively
during the growing years. The lymphangioma is non-
FIGURE 8.4. Vascular lesions: (A–C) cavernous hemangioma
and (D–F) lymphangioma. (A) Axial CT scan shows well-
delineated intraconal mass (M) in the left orbit. (B) B scan dis-
plays a well-defined round to ovoid echogenic lesion (L). (C) A-
scan reveals multiple, regular, highly reflective echo spikes with
the descending edges reaching a medium reflectivity indicating
the presence of blood within the venous spaces and the abun-
dant connective tissue septae.9 Sound attenuation is moderate
(angle ␬ ⫽ 45°). Color Doppler imaging shows little or no evi-
dence of blood flow attributable to the stagnant blood within the
75
FIGURE 8.3. Infantile hemangioma. (A)
Clinical photograph of a child with right
lower lid hemangioma. (B) B-scan
echogram (left) displays an irregular
echogenic lesion (L) with variable degrees
of sound attenuation. A-scan (right) shows
areas of moderate to high reflectivity
representative of the cavernous spaces.
The capillary portions of the lesion are
typically low reflective with intense,
diffuse vascularity (dynamic echography).
Color Doppler imaging provides an
overview of the significant blood flow
representative of an active arterial blood
supply.5 Histopathology shows (C)
proliferation of endothelial cells with
numerous small capillaries and (D) areas
of cavernous spaces. The intermixing of
capillaries and lobulated structures
provides the heterogenic components of
these lesions.
capsulated, often diffuse, with the capability of infil-
trating normal tissues. The tumor occurs most com-
monly in the extraconal space. Spontaneous bleeding
within the lesion is a frequent complication. Micro-
scopically, lymphangiomas are formed by endothe-
lium-lined, lymph-filled vascular channels separated
by loose connective tissue septae that are high reflec-
tive. The latter contain fine blood vessels that are re-
sponsible for the spontaneous bleeding. Unlike cav-
ernous hemangiomas, pericytes and smooth muscle
cells are not present.
vascular spaces.10 (D) Axial CT scan shows an irregular mass (M)
in the right orbit. (E) B scan displays an irregular, large lesion (L)
with multiple, dilated lymph-filled spaces (arrows).2,11 (F) A scan
reveals a regular, heterogeneous pattern with highly reflective
echo spikes separated by low reflective, dilated lymphatic spaces.
In contrast to cavernous hemangiomas, the presence of clear
fluid instead of blood in the wider intracavernous spaces of
lymphangiomas provides ample time for the ultrasound beam to
reach lower reflective levels (arrows).5 Sound attenuation is mod-
erately low (angle ␬ ⬍30°).
76 PART TWO: DIAGNOSIS OF ORBITAL TUMORS
HEMANGIOPERICYTOMA
Hemangiopericytoma is a vascular tumor originating
from the pericytes of blood vessels; consequently, it
may develop wherever capillaries are present (Figure
8.5).12 Orbital hemangiopericytomas are rare, slow-
growing, unilateral tumors with a predilection to the
superior orbit. They occur at any age, although the
majority appear in adulthood. Microscopically, the tu-
mor consists of spindle-shaped cells packed around
thin-walled blood vessels that are lined by endothe-
lial cells. The tumor is classified as sinusoidal, solid,
or mixed depending on the degree of vascularity be-
tween the tumor cells.13 Cystic changes within the
tumor may develop secondary to zones of necrosis.
ORBITAL VARICES
Primary orbital varices (Figure 8.6A,B) are congenital
venous malformations that usually become sympto-
matic in the second to fourth decade of life. The pa-
tient presents with a history of intermittent, posi-
tional proptosis. Microscopically, an orbital varix
FIGURE 8.5. Hemangiopericytoma. (A) B-scan echogram displays
a well-defined round to oval lesion (L).13,14 The internal structure
of the tumor can be irregular, showing solid and cystic components
depending on its histopathology. (B) On A scan, the internal re-
flectivity of the tumor ranges from low (as illustrated) to medium
with the latter representing tumor of a mixed nature.14 I, initial
spike; V, vitreous cavity; a, anterior and P, posterior surface spikes
of the lesion (L); S, sclera. Color Doppler imaging reveals high-
velocity blood flow.
represents an enlarged, dilated vein. The lesion is char-
acterized by a stagnant blood flow that may result in
thrombus formation.
FAST-DRAINING CAROTID CAVERNOUS FISTULA
In fast-draining carotid cavernous fistula, there is a di-
rect communication between the internal carotid ar-
tery and the cavernous sinus. This results in dilation
of the superior ophthalmic vein (Figure 8.6C,D) with
arterialization of blood flow. The patient usually has
a history of head trauma and presents with pulsating
exophthalmos, a bruit over the globe, dilated and tor-
tuous episcleral vessels, and restriction of motility.
PERIPHERAL NERVE LESIONS
NEUROFIBROMA
A benign peripheral nerve tumor, neurofibroma is
characterized histopathologically by the proliferation
of Schwann cells, peripheral nerve axons, endoneural
fibroblasts, and perineural cells. Echographically,
neurofibromas are diffuse, irregular lesions with dense
internal vascularity that closely resemble infantile
hemangiomas. However, the clinical appearance is
quite different and is more periorbital with less bluish
discoloration.
SCHWANNOMA (NEURILEMOMA)
Schwannoma is a benign peripheral nerve tumor char-
acterized by pure proliferation of Schwann cells. It
usually becomes apparent in young to middle-aged
adults and is found in 1.5% of patients with neurofi-
bromatosis type 1. The patient generally presents with
painless progressive proptosis with downward dis-
placement of the globe, since schwannomas arise
more commonly from the supraorbital and supra-
trochlear nerves. Histologically, schwannomas show
a mixture of two patterns.19 The Antoni type A (dense
and cellular pattern) and the Antoni type B (loose, ede-
matous pattern forming cystic spaces). Echographi-
cally, on B scan, schwannoma appears as a well-
defined, roundish, internally echolucent lesion.20 On
A scan, the internal structure is quite regular with
moderate to low internal reflectivity with the latter
representing the Antoni type B areas.
PSEUDOTUMOR
An idiopathic orbital inflammatory disorder, pseudo-
tumor occurs mostly in the third to fifth decades of
life. The inflammation can be diffuse or localized re-
sulting in periscleritis, sclerotenonitis, tendonitis,
myositis, dacryoadenitis, or perioptic neuritis. The pa-
tient presents with a sudden onset of unilateral eye
pain, redness, chemosis, proptosis, and diplopia.21
Some cases may present with severe chronic inflam-
mation that eventually leads to progressive fibrosis of
orbital tissues and results in a frozen globe. Micro-
 CHAPTER 8: ULTRASONOGRAPHY IN ORBITAL DIFFERENTIAL DIAGNOSIS 77

FIGURE 8.6. Vascular lesions: (A,B) orbital

varices and (C,D) fast-draining carotid cav-
ernous fistula. (A). Paraocular A scan shows
low internal reflectivity with expansion of
the lesion during Valsalva maneuver15 (ar-
row). (B) On B scan, orbital varices are lin-
ear, channeled lesions with echolucent in-
ternal structure.5 Sound attenuation is
minimal. Color Doppler imaging shows
nonpulsatile blood flow with apparent
change in dimensions during respiration.16
(C) B scan shows a large, dilated superior
ophthalmic vein (B1, arrow) which is typi-
cally meandering on sagittal topography.5,17
With dynamic echography, the size of the
venous structure changes with the arterial
pulsations from a near collapsed size (B3) to
a widely dilated channel (B1). (D) A scan
shows blurred, low reflective spikes from
fast flowing blood (arrow). Sound attenua-
tion is minimal, and color doppler imaging
shows a pulsatile, arterial-type blood flow
pattern in the superior ophthalmic vein.18

scopically, there is infiltration of the involved orbital volvement is invariably unilateral and follows the
tissues by inflammatory cells consisting of lympho- same characteristic pattern but is localized to the area
cytes, plasma cells, and eosinophils. Echographic find- of the lacrimal gland. (Other lacrimal gland conditions
ings of pseudotumor depend on the involved tissue. are discussed under lacrimal gland lesions.)

SCLERITIS RHABDOMYOSARCOMA
In scleritis the B scan shows an echolucent area be-
The most common primary malignant orbital tumor
tween the anterior and the posterior scleral wall. In
in children is rhabdomyosarcoma. It arises from un-
most cases it is associated with a linear echolucent
differentiated mesenchymal cells that have the abil-
area in the retroscleral space representing edema in
ity to differentiate into striated muscle cells. The pa-
Tenon’s space (T-sign). On A scan, the high scleral
tient presents with rapid, progressive proptosis. The
spikes are wider than normal and invariably show ab-
tumor can involve any part of the orbit with a predilec-
normal thickening. In episcleritis, the similar A- and
tion to the superior portion. The echographic findings
B-scan characteristics can be seen but in the immedi-
of rhabdomyosarcoma (Figure 8.8A,B) are quite simi-
ate retroscleral space with a distinct echolucent rim
lar to those of orbital inflammatory disease (pseudo-
between the sclera and the rest of the orbital tissues;
tumor).22 However, the age group, the clinical pre-
a low reflective A-scan pattern indicates the edema-
sentation, and the ultrasonography and CT findings
tous space that usually exists in this condition.
are usually diagnostic of this condition.
MYOSITIS
Secondary Orbital Lesions
In myositis there is usually a diffuse thickening of the
involved muscle including the inserting tendon to the
LYMPHOPROLIFERATIVE DISEASE
globe with echolucency on B scan and low reflectiv-
ity on A scan (Figure 8.7). Comparative assessment Lymphoid tumors of the orbit occupy a wide spectrum
with other muscles, especially the counterpart of the of diseases ranging from the benign pseudolymphomas
other orbit, is quite revealing for the condition. (pseudotumors) to the atypical lymphoid hyperplasias
to the malignant lymphomas. Echographically (Figure
ORBITAL PSEUDOTUMOR 8.8C,E), lymphoid tumors share the same characteris-
An orbital pseudotumor is invariably a diffuse condi- tics. The patient’s age is important in the interpreta-
tion with significant low reflectivity from the inter- tion of the ultrasound findings especially with pseudo-
nal structure of the involved area. Lacrimal gland in- tumor, rhabdomyosarcoma, and lymphoma, for which
78 PART TWO: DIAGNOSIS OF ORBITAL TUMORS
these findings are similar. Also, unilateral low reflec-
tive infiltrates in an adult is suggestive of pseudotu-
mor while bilaterality supports a diagnosis of
lymphoma.
METASTATIC TUMORS
The orbit, devoid of lymphatic channels, is reached
by metastatic tumors via the hematogenous route.
FIGURE 8.8. (A,B) Rhabdomyoarcoma. (C–E) Lymphoproliferative
disease. (A) Clinical photograph of a child shows left eye proptosis.
(B) A-scan echogram shows diffuse, low-reflective lesion (L). Occa-
sionally, there is active vascularity displayed on dynamic echogra-
phy. On B-scan the lesion is echolucent with minimal sound at-
FIGURE 8.7. Myositis. (A) Clinical photograph of a
young woman shows right eye injection. (B) B-scan
echogram shows enlarged right medial rectus
muscle (upper and middle) with thickened inser-
tion (lower) (arrows). A-scan echograms show (C)
low reflectivity of the enlarged muscle belly (M)
and (D) low reflective thickened insertion (arrow).
The most common source of metastatic tumor to
the orbit in adults is the breast followed by lung,
prostate, skin melanoma, and gastrointestinal tract
in decreasing order of frequency. In children, neu-
roblastoma is the most common source of meta-
static tumor to the orbit occurring in about 40% of
cases. The disease may be bilateral and presents
with a sudden onset of proptosis accompanied by
lid ecchymosis (Figure 8.9).
tenuation. V, vitreous cavity; S, sclera; a, anterior and P, posterior
surface spikes of the lesion. (C) Clinical photograph of an adult man
shows right eye proptosis and downward displacement of the globe.
(D) B-scan echogram shows large, diffuse echolucent orbital lesion
(L), which had low internal reflectivity on A scan (E).
 CHAPTER 8: ULTRASONOGRAPHY IN ORBITAL DIFFERENTIAL DIAGNOSIS
MUCOCELE
Mucocele is a cystic lesion filled with mucoid se-
cretions and epithelial debris that arises from the
paranasal sinuses. Orbital invasion occurs more
commonly from either frontal or ethmoidal muco-
celes. Mucoceles usually develop in adults and
produce progressive proptosis, diplopia, or ptosis.
Echographically, mucoceles present a distinct char-
acteristic pattern almost pathognomonic of the con-
dition (Figure 8.10).
FIGURE 8.10. Mucocele. (1) A-scan echogram shows a highly re-
flective anterior surface (anterior wall of the lesion, a) followed
by an echolucent low-reflective internal structure representing
the mucocele, M; B, orbital bone.24 Sound beam directed through
intraorbital portion of the mucocele. (2) The beam is moved
slightly and hits the edge of bone defect (red) and posterior wall
of the sinus (blue). When scanning the lesion from the intraor-
bital to the intrasinus site, the posterior wall of the mucocele
shifts from the intraorbital normal area into a deep intrasinus
part (shifting posterior high reflective sinusoidal pattern). (3)
Sound beam is directed entirely through bone defect. P, poste-
rior bony wall of sinus.
79
FIGURE 8.9. Metastatic carcinoma.
(A) B-scan echogram displays a poorly
defined diffuse lesion (L) with varying
echogenicity. (B) On A scan, the
internal structure is quite irregular
with the internal reflectivity ranging
from low23 to moderately high. The
characteristic “V” pattern (arrow)
results from a central zone of dense
cellular infiltrates that become more
lobulated toward the periphery
(hence, the higher ascending limbs of
the lesions).5,22
ASSESSMENT OF THICKENING
OF EXTRAOCULAR MUSCLES
Thyroid Eye Disease
The most common cause of extraocular muscle thick-
ening, thyroid eye disease, usually entails multiple,
bilateral, and asymmetrical muscle involvement (see
Chapter 28). Clinically, the inferior rectus muscle is
most often involved, followed by the medial, the su-
perior, and the lateral rectus muscles. However, echo-
graphically, the superior rectus/levator complex is
most commonly enlarged followed by the medial, the
inferior, and lateral rectus muscles.22 Microscopically,
there is perivascular infiltration by lymphocytes and
plasma cells with increased deposition of hydrophilic
mucopolysaccharides in the muscle belly, sparing the
tendon. Topographically, there is enlargement of the
belly of the involved extraocular muscle with the
acquisition of internal tissue echoes reflecting the his-
tological changes.9,25 On A scan, the internal struc-
ture is slightly irregular with medium internal reflec-
tivity (Figure 8.11).
Orbital Inflammatory Disease
(Pseudotumor) and Lymphoma
Refer to the preceding section on pseudotumor and
lymphoproliferative disease.
Metastatic Tumors
Metastatic lesions to the extraocular muscles are usu-
ally unilateral with invariably a single muscle in-
volvement (see Chapter 24).26 There is a slow, pro-
gressive, painless increase in the size of the muscle
with late onset diplopia (in contrast to the rapid,
painful, acute myositis with early diplopia). Ultra-
sonography shows echolucency in the belly of the mus-
cle with low internal reflectivity similar in pattern to
80 PART TWO: DIAGNOSIS OF ORBITAL TUMORS
FIGURE 8.11. (A) Dynamic A-scan examination of normal rectus
muscle (M). 1, Probe is directed anteriorly toward the muscle in-
sertion, which produces a small defect adjacent to the scleral spike.
As the probe is angled more posteriorly (2–4), the sound beam shifts
toward the muscle belly, which produces a wider defect that moves
from left to right. (B) A-scan echogram of an enlarged rectus mus-
cle (M) in thyroid eye disease showing slightly irregular internal
structure with medium reflectivity.
the orbital inflammatory pseudotumor category but,
in contrast, the tendon of the muscle is not involved.
Slow-Draining Carotid Cavernous Fistula
In slow-draining carotid cavernous fistula, there is an
indirect communication between the cavernous sinus
and the internal or external carotid arteries through
their meningeal branches. The patient presents with
prominent episcleral vessels, but minimal proptosis.
The small fistula results in low-flow, low-pressure
shunting that causes increase in episcleral venous pres-
sure and enlargement of extraocular muscles on the in-
volved side. Ultrasonography reveals diffuse enlarge-
ment of the muscles in the involved orbit when
compared to the counterpart muscles of the normal or-
bit. The belly of the muscles shows a slight increase in
width with a tendency of the internal reflectivity to shift
from a medium to a lower pattern owing to the vascu-
lar congestion present in such a condition. Occasion-
ally, the superior ophthalmic vein (which is usually dif-
ficult to isolate and measure in the normal orbit) shows
widening and more echographic prominence than the
other orbit. This finding is far less significant than the
enlarged hyperdynamic superior ophthalmic vein de-
tected in the active carotid cavernous fistula condition.
OPTIC NERVE ASSESSMENT
Thyroid Eye Disease
Optic neuropathy (Figure 8.12A, B, and C) occurs in
about 5% of thyroid-associated orbitopathy patients.
It is caused by direct compression of the optic nerve
or its blood supply at the orbital apex by the enlarged
extraocular muscles. Topographically, the optic nerve
shadowing is enlarged with duplication of the nerve
sheaths.27–29
Optic Neuritis
Optic neuritis is an inflammatory or demyelinating
disorder of the optic nerve. Clinically, it is divided into
retrobulbar neuritis, papillitis, and neuroretinitis. On
ultrasonographic examination the perineural sheath
thickening shows a low reflective internal structure
(in contrast to the high reflectivity of a tumorous con-
dition such as meningioma). However, there is no
shifting of fluid visible on dynamic echography such
as that seen in cerebrospinal fluid retention (thyroid,
pseudotumor cerebri). Also, the thickening of the in-
tersheath space, although significant in comparison to
the normal state, is far less than that found in other
orbital disorders.
Optic Nerve Lesions
OPTIC NERVE GLIOMA (JUVENILE PILOCYSTIC ASTROCYTOMA)
A benign, slow-growing tumor (Figure 8.12D), optic
nerve glioma arises from astrocytes within the optic
nerve. The median age of onset is about 5 years of age,
with a slight preponderance for females. Neurofibro-
matosis type 1 occurs in about 10% of cases and is
characterized by bilateral involvement of the optic
nerve. The patient presents with progressive propto-
sis and visual loss.
OPTIC NERVE SHEATH MENINGIOMA
Optic nerve sheath meningioma is a benign tumor
(Figure 8.12E) that arises from the meningoen-
dothelial cells of the arachnoid layer. The tumor
usually affects middle-aged women. Neurofibro-
matosis type 1 is found in about 16% of cases. Pre-
sentation is with slowly progressive proptosis and
unilateral visual loss. Ultrasonography shows a dif-
 CHAPTER 8: ULTRASONOGRAPHY IN ORBITAL DIFFERENTIAL DIAGNOSIS
FIGURE 8.12. Optic nerve disorders (A–C) thyroid optic neuropa-
thy, (D) optic nerve glioma, and (E) optic nerve meningioma. (A) B-
scan echogram shows crescent sign (large arrow) due to accumula-
tion of subarachnoid fluid around the optic nerve (small arrow). (B)
A-scan echogram reveals double-peaked borders representing a peri-
neural enlargement (between arrowheads) between the optic nerve
parenchyma (arrow) and the perineural sheaths. Upon lateral gaze,
retrograde “milking” of cerebrospinal fluid in this perineural en-
largement occurs, with secondary collapse of the subarachnoid
space shown on dynamic A-scan echography as almost visual ad-
herence of the two spikes (between arrowheads). (C) The change of
thickening of the intersheath space indicates a fluid retention con-
fuse thickening of the optic nerve following its me-
andering structure.
Pseudotumor Cerebri
Benign intracranial hypertension or pseudotumor
cerebri is characterized by raised intracranial pressure
in the absence of an intracranial mass lesion or men-
ingeal cancer. It occurs more commonly in young,
overweight women. The patient presents with head-
ache, neck stiffness, nausea, vomiting, and blurring of
vision. Examination reveals bilateral papilledema.
Topographically, there is duplication of the optic
nerve sheaths with significant widening of the dou-
ble-peaked borders on A scan, reflecting the expansion
of the perioptic subarachnoid space.
LACRIMAL GLAND LESIONS
Pleomorphic Adenoma (Benign Mixed Tumor)
Pleomorphic adenoma is a benign tumor that ac-
counts for 50% of epithelial tumors of the lacrimal
glands.30 Pleomorphic adenomas usually arise from
the orbital lobe and grow posteriorly, but in rare
81
dition rather than tumefaction of the perineural sheaths. (D) Optic
nerve glioma. Echographically, there is widening of the internal lu-
men of the nerve (nerve proper) showing fusiform topographic pat-
tern (arrow) (upper). The perineural sheath normal double spikes on
A-scan are invariably touching (arrows), giving the appearance of a
thick, single spike with a double head (lower). (E) Optic nerve
meningioma. On A scan, the optic nerve proper appears thinner
than normal (owing to meningeal compression), while the inter-
sheath space is significantly wider (connected arrows) with a high-
reflective internal structure between the inner and the outer peri-
neural sheaths. Measurement of the intersheath space is possible
and indicative of the pathologic thickening.
cases they may affect the palpebral lobe.31 Presen-
tation is typically in adults as a slowly progressive,
nontender, firm mass in the superotemporal quad-
rant and proptosis. On gross examination, the tu-
mor is encapsulated with nodular irregularities on
the surface. Histopathology shows the tumor to be
composed of both epithelial and mesenchymal ele-
ments.32 The epithelial elements form ducts, acini,
and irregular tubules that are dispersed throughout
a matrix of mucoid, myxoid, and chondroid tissue.
Topographically (Figure 8.13A), pleomorphic ade-
noma appears as a well-defined round to ovoid
echogenic lesion in the lacrimal gland fossa.
Lymphoid Tumors of the Lacrimal Gland
Lymphoid tumors of the lacrimal gland range from
pseudotumors to reactive lymphoid hyperplasia to ma-
lignant lymphomas of various types. They tend to in-
volve the orbital and palpebral lobes of the lacrimal
gland, resulting in anterior and posterior extension.
Lymphoid tumors share similar acoustic characteris-
tics.35 Topographically, there is diffuse enlargement
of the lacrimal gland with quite regular internal struc-
ture and low reflectivity on A scan. Sound attenua-
tion is minimal.
82 PART TWO: DIAGNOSIS OF ORBITAL TUMORS
FIGURE 8.13. Lacrimal gland lesions. (A) Pleomorphic adenoma.
A-scan echogram displays a regular internal structure with high re-
flectivity that relates histologically to the multiple contiguous tu-
bular structures embedded in the dense connective tissue.33 L, le-
sion. Sound attenuation is moderate (angle ␬ ⫽ 45°). These acoustic
characteristics of pleomorphic adenomas are similar to those seen
in cavernous hemangiomas. Since most cavernous hemangiomas
occur within the muscle cone, the location and the clinical pre-
sentation of the tumor clearly are important diagnostic clues.5,34 (B)
Carcinoma of the Lacrimal Gland
(Adenocarcinoma and Adenoid
Cystic Carcinoma)
Primary carcinomas (Figure 8.13B) account for about
50% of epithelial tumors of the lacrimal gland; ade-
noid cystic carcinoma is the most common primary
malignant tumor. These tumors usually occur in
middle-aged adults. The patient presents with unilat-
eral progressive proptosis, pain, tenderness, ptosis, and
diplopia. On gross examination, carcinomas of the
lacrimal gland are poorly encapsulated, irregular, solid
masses. Histopathologically, they are divided into ade-
noid cystic carcinoma, pleomorphic adenocarcinoma,
and mucoepidermoid carcinoma.
Dacryoadenitis
Acute dacryoadenitis is a rare condition, seen most of-
ten in children as a complication of mumps, measles,
or influenza. The patient presents with pain, swelling,
and injection over the temporal aspect of the upper
eyelid, resulting in S-shaped deformity. Topographi-
cally (Figure 8.13C), there is enlargement of the
lacrimal gland with moderate echolucency. The A-
scan exam is quite similar to that of the normal gland,
retaining the high reflectivity with widening of the
high septated internal pattern. In contrast to the
chronic lacrimal gland inflammatory disease (lym-
Carcinoma of the lacrimal gland. In the early development of these
tumors, the echographic finding shows a V-shaped pattern on A
scan, where the anterior and posterior parts of the lacrimal gland
retain the normal high-reflective character while the central por-
tion representing the carcinoma site is less reflective because of the
condensed cellularity; hence the V-shaped pattern (arrow). With an
increase in the size of the tumor, the central portion of the V area
becomes less reflective. (C) Dacryoadenitis. B-scan echogram shows
an enlarged lacrimal gland (L) with moderate echolucency.
phoproliferative disorders), the internal reflectivity of
these conditions is low, as discussed earlier.
Acknowledgment The authors greatly appreciate
the professional assistance provided by Mr. Heitham
A. Abdul-Baki, who is a third-year medical student at
the American University of Beirut, in the preparation
of the photographs for this chapter.
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