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Edited by Michael Forsting

Imaging of the Head


and Neck II

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Colin S. Poon  Michael Abrahams  James Abrahams

Orbit 11
Anatomy The inferior orbital fissure is located at the margin
between the lateral wall and the orbital floor. It contains
The soft tissue structures of the orbit are contained within the infraorbital (branch of V2) and zygomatic nerves, the
a bony cavity. These soft tissue structures include the globe, nerve branches from the pterygopalatine ganglion, and
the extraocular muscles, the optic nerve–sheath complex, venous connection between the inferior ophthalmic vein
the lacrimal apparatus, and various vascular and nerve and the pterygoid plexus. The inferior orbital fissure con-
structures. nects with the pterygopalatine fossa and the masticator
space/infratemporal fossa, allowing the spread of deep

• Bony Anatomy facial infection and neoplasm to the orbital apex.


The globe is essentially a spherical structure, with the
The bony orbit is a conical structure with the apex pointing wall consisting of three layers: retina (innermost), choroids
posteriorly. The orbital roof is composed of the frontal (middle), and sclera (outermost). These layers cannot be
bone and is thinner anteriorly. The medial wall is com- resolved with current clinical imaging technology, unless
posed of the frontal process of the maxillary bone anteri- they are separated by pathologic processes (e.g., retinal
orly, the lamina papyracea of the ethmoid air cells at the detachment). The globe is divided into three fluid-filled
midportion, and the sphenoid bone posteriorly. The cavities: anterior chamber, posterior chamber, and vitreous
lamina papyracea is very thin, and not surprisingly it is a cavity.4,40 The anterior chamber and posterior chamber
common site of orbital blowout fracture and spontaneous constitute the anterior segment, while the vitreous cavity
dehiscence of orbital fat. The lateral orbital wall is formed constitutes the posterior segment. The anterior chamber
by the orbital surface of the zygomatic bone. The orbital extends from the cornea to the iris. The posterior chamber
floor is formed by the orbital plate of the maxilla, the extends from the posterior surface of the iris to the anterior
orbital process of the palatine bone, and the orbital surface surface of the vitreous. The vitreous cavity is posterior to
of the zygomatic bone. The orbital plate of the maxilla is the posterior chamber.
thin and a common site of inferior blowout fracture. The anterior border of the orbit is formed by the
Multiple foramina and canals go through the bony orbital septum, a fibrous structure adherent to the inner
orbits (Box 11-1). The optic canal (also called optic foramen) margin of the orbital rim with central portions that extend
is located at the orbital apex. It is bordered by two bony
spikes of the lesser wing of the sphenoid bone, commonly
referred as the optic struts. The canal contains the optic
nerve and the ophthalmic artery, both of which are con- Box 11-1. Major Foramina of the Orbit and Their Neurovascular
tained within a dural sheath. Contents
The superior orbital fissure is located at the margin
between the lateral wall and the orbital roof. The greater Optic Canal
Optic nerve
wing of the sphenoid bone forms its lateral boundary,
Ophthalmic artery
while the lesser wing forms its medial boundary. The supe-
rior orbital fissure contains the superior ophthalmic vein; Superior Orbital Fissure
the oculomotor (III), trochlear (IV), and abducens (VI) Cranial nerves: III, IV, VI, V1
Lacrimal and frontal nerves
nerves; and the ophthalmic division of trigeminal nerve
Superior and inferior ophthalmic veins
(V1). The superior orbital fissure forms the largest com-
munication between the orbit and intracranial structures Inferior Orbital Fissure
and therefore forms a conduit for infectious or neoplastic Cranial nerve: V2
Zygomatic nerve
processes between the orbital apex and the cavernous
Infraorbital vessels
sinus.

471

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472  II  Imaging of the Head and Neck

into the tarsus of the eyelids. Although there are a few ori- blood supply from both the internal and external carotid
fices for passage of vessels, nerves, and ducts, the septum arteries, orbital arteries may serve as anastomosis between
forms an effective barrier to prevent superficial processes the two arterial systems.
from extending into the orbit proper. A pathologic process The largest orbital vein visualized on CT or MRI is
such as cellulitis may be designated as preseptal versus post- the superior ophthalmic vein. It can be seen arising near
septal. A postseptal process signals the involvement of more the base of the nose, coursing anteromedially to postero-
critical structures of the orbit, the possibility of extension laterally, and draining into the cavernous sinus. It crosses
into the cavernous sinus and intracranial structures. over the optic nerve in its mid course, at approximately 20
degrees (see Fig. 11-4). The midportion of the superior

• Soft Tissue Anatomy ophthalmic vein is an intraconal structure that lies between
the superior rectus muscle and the ophthalmic artery. The
There are seven extraocular muscles: the superior, inferior, inferior ophthalmic vein is much smaller than the superior
medial and lateral rectus; the superior and inferior oblique; ophthalmic vein. It is usually not well visualized on CT or
and the levator palpebrae superioris muscles. The levator MRI studies. Both the superior ophthalmic vein and infe-
palpebrae muscle can be seen immediately above the supe- rior ophthalmic vein receive tributaries from the veins of
rior rectus muscle. With the exception of the inferior face and nose.
oblique muscle, all extraocular muscles originate from the
annulus of Zinn, a tendinous ring in the orbital apex. They
pass anteriorly and insert on the globe just behind the
corneoscleral border. The four rectus muscles and the Imaging Techniques
fibrous septa connecting between them form the muscle
cone of the orbit. The intraconal space is filled with orbital The major modalities for imaging of the orbits include CT
fat. Orbital vessels, sensory and motor nerves to the extra- and MRI. The abundance of intraorbital fat provides good
conal muscles, and the optic nerve–sheath complex also intrinsic soft tissue contrast on CT for most clinical applica-
traverse the intraconal space. tions. The advances of multidetector CT technology now
The optic nerve may appear straight or slightly tortu- make high-resolution CT imaging possible. The source
ous depending on the eye position. It consists of three images can be reformatted in different planes, providing
segments: orbital, canalicular, and intracranial. The orbital high-resolution isotropic imaging. This renders the previ-
segment is covered by the same meningeal sheaths as the ous advantage of multiplanar capability of MRI obsolete.
brain. The normal diameter of the optic nerve is up to CT is superior to MRI for delineation of osseous structures
4 mm. A layer of cerebrospinal fluid can be seen between and calcifications. It requires short imaging time and is
the meningeal sheath and the optic nerve. therefore less sensitive to motion of the globe and eyelid.
The extraconal space represents the area between the CT imaging can be completed quickly and requires less
muscle cone and the bony orbit. This space contains orbital patient cooperation, making it ideal for imaging orbital
fat and the lacrimal gland. The lacrimal gland is located trauma.
superolateral to the globe. The upper margin of the gland Compared to CT, MRI provides superior soft tissue
is convex. The lower margin is concave and lies on the contrast. It also provides better imaging details of the intra-
levator palpebrae and lateral rectus muscles. The lacrimal cranial structures. When it is important to assess intracra-
system drains through the lacrimal ductal system near the nial abnormalities, either as direct extension of orbital
medial canthus. It consists of the superior and inferior lesions or as associated lesions in certain diseases (e.g., in
puncta, their associated ducts, the lacrimal sac, lacrimal multiple sclerosis), MRI is superior to CT.
duct, and the valve of Hasner, which is a draining orifice In the past, evaluation of suspected vascular lesions of
inferolateral to the inferior nasal turbinate. the orbits required conventional angiography. The advances
The vascular anatomy of the orbits can be well dem- in CT angiography and MR angiography now allow many
onstrated on high-resolution magnetic resonance imaging vascular lesions to be evaluated noninvasively. In some
(MRI)15 and computed tomography (CT) angiography. The cases, conventional angiography can be foregone.
primary arterial supply to the orbit is the ophthalmic CT and MRI often provide complementary roles in
artery. It is superior to the optic nerve and can be seen orbital imaging. The choice of CT versus MRI for initial
crossing the optic nerve almost perpendicularly (see Fig. imaging of the orbits depends on the clinical problem. CT
11-4). The ophthalmic artery most often originates from is usually preferred for trauma, for evaluation of the bony
the internal carotid artery. The origin is usually at the orbits or calcified lesions, and when MRI is contraindi-
anteromedial aspect of the internal carotid artery as it exits cated. For other applications, MRI is generally preferred
the cavernous sinus. Variants of its origin include the cav- because of the absence of radiation risks and its high soft
ernous segment of the internal carotid artery and the tissue contrast. MRI is the initial imaging of choice for
middle meningeal artery (i.e., external carotid artery evaluation of the optic nerve, other cranial nerves, and
branch). Secondary arterial supply to the orbits comes intracranial lesions. Exceptions can be found in a small
from the external carotid artery. Because the orbits receive number of optic nerve meningiomas, which are very small

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11  Orbit  473 11
c d c d e f g
b
e
b
f
a j
h
a

i
A g B

Frontal bone Frontozygomatic suture

Lamina
papyracea

Zygomatic
bone

C Infraorbital foramen Maxillary bone

Superior orbital Anterior


fissure Optic canal clinoid process

D Inferior orbital fissure Optic strut


Figure 11-1.  A, Coronal CT scan: normal anatomy. Lateral rectus (a), superior rectus (b), medial rectus (c), superior oblique (d), levator
palpebrae superioris (e), lacrimal gland (f), inferior oblique (g). B, Medial rectus (a), superior oblique (b), ophthalmic artery (c), superior rectus/
levator palpebrae superioris complex (d), dural sheath (e), superior ophthalmic vein (f), subarachnoid space (g), optic nerve (h), inferior rectus (i),
lateral rectus (j). C, Bony orbits at mid anterior level. D, Bony orbits at orbital apex.

and mostly calcified. These lesions may be missed by MRI processes from surrounding structures (e.g., paranasal
and are better detected by CT. sinuses, trauma, tumor). Before the era of multidetector
CT, direct coronal scanning was often performed to provide

• Computed Tomography the best spatial resolution in this plane. Multidetector CT,
however, allows high-resolution reformation in any plane.
The orbits are often included in routine CT head or maxil- Axial scanning (Fig. 11-2) also has the advantage that it
lofacial CT examinations. These screening examinations can minimize the problem of streak artifacts from dental
are usually performed according to the standard head or hardware, a problem often encountered previously with
maxillofacial CT protocols. direct coronal imaging (Fig. 11-3).
When dedicated orbital CT is performed, thin sections A typical orbital CT protocol can be performed with
(usually less than 3 mm and preferably less than 1.5 mm) scanning in the axial plane. This plane is usually chosen
are acquired. Coronal images are especially important in to be parallel to the orbital long axis. In practice, imaging
that cross-sectional evaluation of all of the intraorbital is performed in the plane parallel to the infraorbital-meatal
structures is optimal (e.g., extraocular muscles, optic line. Coronal reformation should be included in the
nerve–sheath–nasal complex, vessels, and globe) (Fig. 11- routine protocol. This can be performed in the plane per-
1). This plane is also imperative for assessing spread of pendicular to the axial plane. Parasagittal reformation, in

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474  II  Imaging of the Head and Neck

Lacrimal sac Figure 11-2.  Normal orbital anatomy. Direct axial CT


and duct scanning from inferior to superior. A to D, Soft tissue
window. E, Bone window.

Inferior rectus m. Inferior


A orbital fissure

Ciliary
bodies Medial rectus m. Anterior chamber

Lens

Vitreous
cavity

Lateral
rectus m.

Optic nerve
and sheath

B Ophthalmic artery Superior orbital fissure

Lacrimal gland Superior orbital fissure

Lacrimal Superior
C vein ophthalmic vein

a plane parallel to the long axis of the optic nerve, may Enlargement of a lesion with the Valsalva maneuver is
also be added. indicative of an orbital varix. Less commonly, cavernous
Intravenous contrast is often used in the evaluation of hemangiomas may enlarge with the Valsalva maneuver.17
inflammatory, infectious, neoplastic, and vascular orbital In patients unable to cooperate, similar effects can be
diseases. For evaluation of vascular lesions, a bolus injec- obtained by positioning the patient prone during
tion may be used for better depiction of its arterial blood scanning.
supply. CT angiography can provide good depiction of the
When orbital varix is suspected, the CT study should major vascular anatomy in the orbits. In addition to the
be repeated without and with the Valsalva maneuver. ophthalmic artery and superior ophthalmic vein, their

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11  Orbit  475 11
Superior Orbital
oblique m. Trochlear m. septum

D Superior rectus m. Lacrimal gland

Nasomaxillary Lamina
suture papyracea

Zygomatic
bone

Sphenozygomatic
suture
Greater wing
of sphenoid
Sphenotemporal
suture

Superior orbital Optic canal Optic


E fissure strut
Figure 11-2, cont’d

A B
Figure 11-3.  A, Coronal CT scan in a patient whose extensive dental hardware obscures detail in the orbits. B, Axial scanning with coronal
reformation avoids this problem, showing enlargement of the extraocular muscles on the left side. In view of the patient’s known hyperthyroidism,
this finding was thought to represent Graves’ disease.

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476  II  Imaging of the Head and Neck

a
a

A B

Figure 11-4.  CT angiography of the orbits, superior to inferior


(A to C). The ophthalmic artery (a) arises from the internal carotid
a
a artery as it exits the cavernous sinus. It enters the orbit through the
optic canal and crosses the optic nerve underneath the superior
rectus muscle. The superior ophthalmic veins (b) cross the optic
C
nerve more distally and at a more obtuse angle.

branches, tributaries, and many other smaller vessels can rapidly with distance from the coils, leading to rapid signal
often be seen and traced. The study can be performed as falloff and inadequate coverage of deeper structures.
part of a CT angiographic study of the head and neck (Fig. For routine imaging, the field-of-view should include
11-4).66 Bolus injection of iodinated contrast is required. the cavernous sinus, the optic chiasm, the optic tracts and
It is important to use a field-of-view sufficiently wide to radiations, and the nuclei of the oculomotor, abducens,
include extraocular pathology that may be associated with and trochlear nerves in the midbrain and pons.
the vascular orbital lesions, such as carotid-cavernous The protocol should include T1-weighted and T2-
fistula. weighted imaging in axial and coronal planes (Fig. 11-6).
CT dacryocystograms can be performed by administra- Intravenous gadolinium contrast is routinely used. For
tion of contrast material into the nasolacrimal duct to dedicated orbital imaging, fat suppression is usually per-
evaluate for patency (Fig. 11-5). This requires cannulation formed for T2-weighted imaging and postgadolinium
of the lacrimal duct, usually by an ophthalmologist. imaging to prevent the obscuration of enhancing lesions
by the high intraorbital fat signal (Figs. 11-7 and 11-8).

• Magnetic Resonance Imaging The fat suppression for fluid-sensitive imaging (i.e., T2-
weighting) can also be performed effectively using inver-
MRI of the orbits can be performed with the head coil. For sion recovery (Fig. 11-9).30
high-spatial-resolution imaging of the anterior orbital Orbital MRI is susceptible to image artifacts because
structures, special orbital surface coils may be advanta- of several factors.25 First, chemical shift artifacts may be
geous. However, the sensitivity of surface coils decreases seen at the interface of the orbital fat and the globe. Similar

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11  Orbit  477 11

A B
Figure 11-5.  A, Coronal reformatted image from a CT dacryocystogram shows dilation of the lacrimal duct (arrow). The nasal septum and
inferior turbinate are deviated leftward and cause obstruction at the valve of Hasner. B, The obstruction is only partial, as evidenced by the
presence of contrast material in the posterior nasopharynx (arrow).

a
f
e

d
b

c
A
d e f
g

b h

a
B C
Figure 11-6.  A, Coronal T1-weighted MRI study: normal anatomy. Superior ophthalmic vein (a), lateral rectus (b), inferior rectus (c), medial
rectus (d), superior oblique (e), superior rectus-levator palpebrae superioris complex (f). B, Axial T1-weighted MRI study: normal anatomy. Optic
nerve in the optic canal (a), optic nerve sheath complex (b), medial rectus (c), anterior chamber (d), lens (e), lid (f), medial and lateral aspects of
the orbital septum (arrow; g), lateral rectus (h). C, Axial T2-weighted MRI study: posterior visual apparatus. Position of lateral geniculate body
(arrows), path of optic radiations (arrowheads).

artifacts may also be present if silicone oil is used to fill the the air cavities of paranasal sinuses makes orbital imaging
globe in treatment of retinal detachment. These chemical susceptible to image artifacts. Exogenous metallic materi-
shift artifacts can be reduced by using fat or silicone satura- als, such as cosmetics, can also lead to susceptibility arti-
tion, using a higher gradient strength, or narrowing the facts. Third, motion artifacts may be present. To minimize
bandwidth. Second, the proximity of orbital structures to motion of the globe, a patient can be asked to fixate his or

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478  II  Imaging of the Head and Neck

her vision at a certain object when the eyes are open. Tem- orbital walls is drawn. The distance from the anterior
poral averaging can also be performed. margin of the globe to this line should not exceed
21 mm.28

Approach to Differential Diagnosis • General Principle


Various characteristics of an orbital lesion can be used
A large number of disease processes can involve the orbits, to help construct a differential diagnosis. These include its
and orbital complaints such as proptosis, orbital pain, location, anatomic structure, and imaging features and the
visual loss, and ophthalmoplegia are nonspecific. Propto-
sis is the abnormal protrusion of the globe; exophthalmos
is the abnormal prominence of the globe. On imaging,
proptosis is best evaluated at a level of the lens on axial
images. A line connecting the most distal tips of the lateral

a
b a c
h c

g d

e
f

Figure 11-7.  Coronal T1-weighted MRI study with fat saturation:


normal anatomy. a, superior rectus/levator palpebrae superioris Figure 11-9.  Coronal inversion recovery, fast-spin echo MRI study:
complex; b, superior ophthalmic vein; c, optic nerve; d, lateral rectus; normal anatomy. a, optic nerve; b, subarachnoid space with
e, dural sheath; f, inferior rectus; g, medial rectus; h, superior oblique. cerebrospinal fluid; c, optic nerve sheath.

A B
Figure 11-8.  A, Precontrast axial T1-weighted image performed without fat suppression demonstrates a mass at the left orbital apex in a patient
with known cutaneous lymphoma. B, Postcontrast T1-weighted image of the same patient in which the fat saturation pulse failed to suppress the
orbital fat (this may be due to dental artifact). The lesion demonstrates marked contrast enhancement and is now indistinguishable from the high
signal of the orbital fat. This case illustrates the importance of performing nonsuppressed precontrast images and fat-suppressed postcontrast
studies.

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11  Orbit  479 11
clinical presentation of the patient.21,34,36,58 Using a com- Box 11-3. Differential Diagnosis of Globe Lesions Associated
partmental approach, a lesion is first localized to one of with Calcification21,34
the four compartments: globe, optic nerve–sheath complex,
intraconal space, or extraconal space. Differential diagnosis Congenital
of an extraconal lesion can be further refined if it can be Degenerative
determined to be associated with the lacrimal gland and Cataracts
Optic nerve drusen
apparatus. Once the primary location of a lesion is deter-
Phthisis bulbi
mined, other parameters including imaging features (e.g.,
Retinal detachment (chronic)
characteristics of margin, associated bony changes, enhance- Retrolental fibroplasia
ment patterns), pathophysiologic basis, age of presenta- Calcification of ciliary muscle insertion
tion, and chronicity can be considered to further reduce Iatrogenic (e.g., scleral banding)
the differential diagnosis. The presence of calcification may
Trauma
also be helpful in refining the differential diagnosis, espe- Foreign body
cially for globe lesions.
Obviously, some lesions may extend over more than Inflammatory
Infection (cytomegalovirus, herpes simplex, rubella, syphilis,
one compartment. Nevertheless, this compartmental
toxoplasmosis, tuberculosis)
approach helps simplify the diagnostic thought process.
The optic nerve–sheath complex, strictly speaking, is also Neoplasm
Astrocytic hamartoma (neurofibromatosis, tuberous sclerosis, von
an intraconal structure. However, because of its unique
Hippel-Lindau syndrome)
significance, it can be considered a separate compartment
Retinoblastoma (children)
to improve the specificity of the differential diagnosis. Dif- Choroidal osteoma
ferential diagnosis of orbital lesions is summarized in
Boxes 11-2 to 11-7. Metabolic
Hypercalcemia
Apart from aiding differential diagnosis, the localiza-
Sarcoidosis
tion of a lesion in the extraclonal space versus the intra-
clonal space may also have management implications. In
general, intraconal lesions may require surgical attention,
whereas extraconal lesions may be amenable to medical
management. Box 11-4. Differential Diagnosis of Optic Nerve Sheath
Lesions21,34

Trauma
Contusion
Box 11-2.  Differential Diagnosis of Globe Lesions21,34 Hematoma
Optic nerve avulsion
Congenital Infection
Persistent hyperplastic primary vitreous Toxoplasmosis
Coat’s disease Tuberculosis
Coloboma Syphilis
Globe hypoplasia/aplasia
Noninfectious Inflammatory
Degenerative Thyroid ophthalmopathy
Optic nerve drusen Optic neuritis
Phthisis bulbi Pseudotumor
Staphyloma Sarcoidosis
Trauma Vascular
Vitreous hemorrhage Central retinal vein occlusion
Choroidal hematoma Neoplasm
Choroidal effusion Optic nerve glioma
Foreign body Meningioma
Inflammatory Neurofibroma
Orbital pseudotumor (uveal/scleral thickening) Schwannoma
Sclerosing endophthalmitis (Toxocara canis) Lymphoma/leukemia
Neoplasm Metastasis
Uveal melanoma (adults) Hemangioblastoma
Retinoblastoma (children) Hemangiopericytoma
Metastasis Miscellaneous
Choroidal hemangioma Increased intracranial pressure
Medulloepithelioma Optic hydrops

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480  II  Imaging of the Head and Neck

Box 11-5.  Differential Diagnosis of Conal/Intraconal Lesions21,34 Box 11-7. Differential Diagnosis of Lacrimal Gland and
Apparatus Lesions1,21,34
Trauma
Hematoma Trauma
Foreign body Hematoma
Infection Infection
Cellulitis Dacryoadenitis
Abscess Noninfectious Inflammatory
Noninfectious Inflammatory Pseudotumor
Thyroid ophthalmopathy Postviral syndrome
Pseudotumor Sarcoidosis
Sarcoidosis Sjögren’s syndrome
Wegener’s granulomatosis Mikulicz’s syndrome
Vascular Wegener’s granulomatosis
Carotid-cavernous fistula Neoplasm
Venous varix Papilloma
Superior ophthalmic vein thrombosis Benign mixed tumor (pleomorphic adenoma)
Venous angioma Adenoid cystic carcinoma
Arteriovenous malformation Mucoepidermoid carcinoma
Cavernous hemangioma (adults) Adenocarcinoma
Capillary hemangioma (children) Malignant mixed tumor
Lymphangioma Undifferentiated carcinoma
Neoplasm Squamous cell carcinoma
Lymphoma Sebaceous carcinoma
Metastasis Primary malignancy from adjacent structures
Rhabdomyosarcoma (children) Non-Hodgkin’s lymphoma
Hemangiopericytoma Metastasis
Neurofibroma/schwannoma (cranial nerve III, IV, VI) Dermoid/epidermoid
Ectopic meningioma Congenital
Dacryocele
Dacryocystocele

Box 11-6.  Differential Diagnosis of Extraconal Lesions21,34

Trauma
• Specific Clinical Scenarios
Fracture There are a few clinical scenarios that may be helpful to
Hematoma have their own differential consideration. The first is lesions
Infection of the lacrimal gland and apparatus.1 Lacrimal lesions are
Cellulitis most often benign inflammatory processes, with tumors
Abscess being less common. Viral adenitis is the most common
Noninfectious Inflammatory acute process. More chronic inflammatory processes
Pseudotumor include sarcoidosis, Wegener’s granulomatosis, and
Postviral syndrome (lacrimal gland) Sjögren’s syndrome. Histologically, the lacrimal gland is
Sjögren’s syndrome (lacrimal gland) analogous to the minor salivary gland in other regions of
Mikulicz’s syndrome (lacrimal gland) the head and neck. They therefore share many common
Neoplasm pathologic processes. Most lacrimal gland tumors are epi-
Metastasis thelial cell tumors, with half of these being benign mixed
Primary malignancy from adjacent structures tumors and half carcinomas. Lymphoma also occurs com-
Benign mixed tumor (lacrimal gland) monly at the lacrimal gland fossa.
Adenoid cystic carcinoma (lacrimal gland)
In a young patient presenting with leukokoria, one
Non-Hodgkin’s lymphoma
will need to exclude retinoblastoma. Other differential
Rhabdomyosarcoma (children)
considerations include developmental and congenital con-
Congenital ditions such as retinopathy of prematurity, Coats’ disease,
Cephalocele
persistent hyperplastic primary vitreous, toxocariasis, retina
Dermoid/epidermoid
dysplasia, and congenital retinal fold.

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11  Orbit  481 11
Pathophysiology • Infection
Orbital infection is most often caused by direct extension
Orbital diseases can be categorized based on their patho- from adjacent structures; hematogenous infection is less
physiology: trauma, infection, noninfectious inflamma- common. It is important to localize orbital infection to the
tion, neoplasm, vascular lesions, congenital and following compartments: (1) preseptal versus postseptal
developmental abnormalities, and degenerative condi- and (2) extraconal versus intraconal. Preseptal or extra-
tions. A more popular approach for differential diagnosis conal infection can usually be treated by standard antimi-
is based on a compartmental approach (see above). crobial therapy. Postseptal or intraconal infection requires
more aggressive management because of the risk of neuro-

• Trauma vascular injury and further intracranial spread.20 Identifica-


tion of orbital abscesses is also crucial because they may
CT is the imaging method of choice for evaluation of require surgical intervention.
orbital trauma. The most common traumatic injury is Orbital infection is most commonly caused by con-
fracture of the orbital walls. Less commonly, hemorrhage tiguous spread of sinusitis or a superficial periorbital cel-
in the globe, globe rupture, perforation and penetrating lulitis of the face. In children, infection is most commonly
injury, and contusion or avulsion of the optic nerve sheath secondary to extension from ethmoid air cells (Fig. 11-12),
may occur. A common type of orbital fracture is “blowout” whereas in adults, extension from the frontal sinus is most
fracture, which results from increased intraorbital pressure common (Fig. 11-13).63 Common organisms include Strep-
transmitted to the orbital walls secondary to blunt trauma tococcus pneumoniae and beta-hemolytic streptococci. Hae-
(Fig. 11-10). Blowout fractures most often involve the mophilus influenzae, staphylococci, and anaerobes are less
inferior and medial walls because they are the thinnest. common.
Intraorbital soft tissue contents may herniate through the
fracture. Muscle entrapment is a potential complication of
orbital fractures. Because the extraocular muscles are teth-
ered to the orbital walls by tiny fibrous strands that are too
small to image on CT or MRI, muscle entrapment may
occur even without herniation of the muscle itself.35
Evaluation for foreign bodies is best performed with
thin-section CT (Fig. 11-11). Wood fragments pose a chal-
lenge to CT evaluation because they may have variable
densities owing to differences in hydration. Wood may
appear hypodense, isodense, or hyperdense. Air may be
present within a wood fragment.55 Therefore, unusual air
pockets should be evaluated carefully.

Figure 11-11.  CT scan shows intraconal metallic foreign bodies just


medial to the medial rectus (large arrow) and intraocular (small
arrow). Scleral band in place in right globe (arrowheads).

m
m

Figure 11-12.  CT scan shows an extraconal subperiosteal abscess


Figure 11-10.  CT scan shows blowout fracture of the left orbital (small arrowheads). This is a complication of ethmoid sinusitis. A
floor with herniation of extraconal fat and inferior rectus muscle thickened, displaced medial rectus (large arrowhead) and preseptal
(arrow). m, maxillary sinus. soft tissue swelling (arrows) are seen.

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482  II  Imaging of the Head and Neck

CT is the imaging modality of choice because it muscle enlargement is severe), and inflammatory changes
can demonstrate inflammatory soft tissue changes, of the periorbital fat (Fig. 11-14; see Fig. 11-3). There may
fluid collections/abscesses, and bone changes (e.g., be increase of the intraorbital fat. The lacrimal glands may
osteomyelitis). also be affected. The extraocular muscles most often
Imaging findings vary from mild mucoperiosteal thick- affected, in descending order of frequency, are the inferior
ening or elevation to frank intraorbital abscesses. rectus, medial rectus, and superior rectus–levator palpe-
brae muscle complex.49 Periorbital soft tissue swelling and
proptosis may be seen. Involvement is usually bilateral but
• Inflammation may be asymmetrical.

• Thyroid-Associated Ophthalmopathy Direct involvement of the globe and optic nerve sheath
is uncommon. However, secondary compression of the
Thyroid-associated ophthalmopathy is an autoimmune- optic nerve sheath may occur and can lead to irreversible
mediated inflammation of the extraocular muscles and visual loss. It is important to assess for this possibility on
periorbital connective tissues. It is most often associated imaging.
with Graves’ disease, although association with other
thyroid diseases such as Hashimoto’s thyroiditis, thyroid
carcinoma, and neck irradiation has also been reported. In • Orbital Pseudotumor
approximately 10% to 20% of patients, thyroid-associated Orbital pseudotumor is also known as idiopathic orbital
ophthalmopathy may present before any other clinical inflammatory disease. It is an idiopathic nongranulomatous
symptoms or signs. Clinical presentation may include inflammatory process that often involves the extraocular
eyelid retraction, proptosis, chemosis, periorbital edema, muscles and orbital fat. Less frequently, other intraorbital
and impaired ocular motility. structures including the uveal tract, sclera, optic nerve, and
The classic imaging findings are fusiform enlargement lacrimal glands may also be involved.13,16,44 The muscular
of the extraocular muscles with sparing of the tendinous involvement is usually diffuse. As opposed to thyroid-
attachments (which may be difficult to appreciate when associated ophthalmopathy, involvement is often unilat-
eral and there is usually extension to the muscular tendon
attachments (Figs. 11-15 and 11-16). Orbital pseudotumor
is usually painful, which helps distinguish it from thyroid-
associated ophthalmopathy.
Orbital pseudotumor may be difficult to differentiate
from other tumefactive inflammatory processes and neo-
plasms. However, a quick response to a trial steroid therapy
may help establish the diagnosis.45

• Sarcoidosis
Sarcoidosis is a noninfectious granulomatous disease
that may affect any part of the optic pathway, from the
globe to the optic radiations.8 The lacrimal gland, anterior
layer of the globe, and eyelids are commonly involved. The
Figure 11-13.  CT scan shows subperiosteal abscess of the superior imaging findings can simulate pseudotumor (Figs. 11-17
orbit (arrowheads) from ethmoid or frontal sinus disease. and 11-18).

A B
Figure 11-14.  A, Axial contrast-enhanced CT scan shows Graves’ ophthalmopathy characterized by enlarged superior, medial, and inferior recti
with compromise of the orbital apex. Note the sparing of the muscle tendon insertions. B, Coronal contrast-enhanced CT scan in the same patient.

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11  Orbit  483 11

A B
Figure 11-15.  A, Axial CT scan shows pseudotumor of the orbit with swollen bilateral medial rectus (arrows), which includes tendinous
insertion on the globe. Thickening and enhancement of the globe (arrowheads) are also shown. B, Coronal CT scan shows pseudotumor of the
orbit in the same patient.

Figure 11-16.  CT scan of orbital pseudotumor with bilateral medial


rectus and left lacrimal involvement.

Figure 11-18.  Axial T1-weighted MRI study with gadolinium shows


sarcoid of the chiasm (long arrow), left cerebral peduncle (large
arrowhead), lateral geniculate body (short arrow), and superior
colliculus (small arrowhead).

nodules or infiltrates in the retrobulbar space.52,62 Enhance-


ment is generally present.
On MRI, retrobulbar masses may demonstrate marked
Figure 11-17.  Axial T1-weighted MRI study with gadolinium shows hypointensity. Although this finding is not unique, it
sarcoid of the anterior left globe (arrowheads). strongly suggests Wegener’s granulomatosis.12,52

• Optic Neuritis
• Wegener’s Granulomatosis Optic neuritis represents nonspecific inflammation of the
optic nerve that can be associated with infection, granulo-
This is a form of necrotizing granulomatous vasculitis. matous diseases, pseudotumor, postradiation, or demye-
Orbital involvement is common and is seen in slightly linating diseases. A large proportion of cases are idiopathic.
more than 50% of patients.23,29,47 Any orbital structures can Association with multiple sclerosis is established in approx-
be involved. Findings may include conjunctivitis, episcleri- imately 50% of patients.3 Imaging findings are best dem-
tis, scleritis, uveitis, optic nerve vasculitis, retinal artery onstrated on MRI, which may include enhancement and
occlusion, nasolacrimal duct obstruction, and retrobulbar T2 prolongation (Fig. 11-19). These findings can be subtle.
diseases. CT examination may demonstrate nonspecific It is important to include the whole brain in image

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484  II  Imaging of the Head and Neck

The differential diagnosis includes pseudotumor and


metastasis. Lacrimal gland involvement can be seen either
in isolation or in combination with the other manifesta-
tions of lymphoma within the orbit.

• Optic Glioma
Optic gliomas most often occur in children, especially
between the ages of 2 and 6 years. They are usually benign,
but a small number of lesions may develop aggressive
behavior.68 This lesion usually involves the anterior optic
Figure 11-19.  Coronal MRI study, inversion recovery fast-spin echo, apparatus (e.g., optic nerves, chiasm, and optic tracts) and
shows left optic neuritis. Contrast the increased signal at the left optic causes enlargement and often tortuosity of these structures.
nerve (long arrow) with the low signal of the normal right optic nerve About half of all optic gliomas occur in patients with
(short arrow). neurofibromatosis type I, and 10% to 15% of neurofibro-
matosis type 1 patients develop optic gliomas (Fig. 11-
evaluation to exclude intracranial lesions, particularly the 22).14 These lesions do not calcify.10
presence of demyelinating lesions. MRI has become the modality of choice, given the
necessity of evaluating the intracranial extent of the tumor
• Neoplasms (Fig. 11-23). Optic gliomas are typically either nonenhanc-

• Lymphoma ing or weakly enhancing. The lesions are generally isoin-


tense to slightly hypointense on T1-weighted images and
Lymphoma is the most common neoplasm in the orbit, hyperintense on T2-weighted images.19
accounting for just more than half of all cases.65 B-cell CT can help in assessing bony changes and is espe-
lymphomas of the non-Hodgkin’s type are by far the most cially valuable in detecting expansion of the optic canal.
common, although T-cell lineages have also been CT thus complements MRI in evaluation of these lesions.
described.11 Usually, orbital lymphomas are primary to the
orbit, but occasionally orbital manifestation of a systemic
lymphoproliferative process is seen. The usual appearance
is a well-defined mass within the muscle cone (Figs. 11-20 • Optic Nerve Sheath Meningioma
and 11-21; see Fig. 11-8). Less frequently, extraconal masses Optic nerve sheath meningiomas (ONSMs) are meningio-
or diffuse infiltration of the orbital fat can be seen. mas that arise from the meninges surrounding the optic

A B

Figure 11-20.  A, T1-weighted axial MRI study


shows a large mass replacing the intraconal fat in
the right orbit (arrow). B, T2-weighted axial image
of the same patient. C, Gadolinium-enhanced axial
T1-weighted image of the same patient
demonstrates marked enhancement (long arrow).
Fat suppression allows the smaller lesion to be
C
visible at the apex of the left orbit (short arrow).

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11  Orbit  485 11

A B

M
M
M
M

C
Figure 11-21.  Orbital lymphoma. A, Contrast-enhanced CT scan demonstrates a homogeneously enhancing intraconal mass (black arrow) adjacent
to the left optic nerve, causing medial deviation of the nerve (white arrow). B, Axial postgadolinium fat-suppressed T1-weighted image confirms the
CT findings (long arrow shows the enhancing mass; short arrows show the optic nerve). C, Coronal postgadolinium fat-suppressed images more
clearly demonstrate the enhancing mass (long arrow) separate from the nonenhancing left optic nerve (short arrow). M, extraocular muscles.

usually diminished visual acuity from optic nerve com­


pression or proptosis. If there is no evidence of visual loss
or intracranial extension, these lesions are often treated by
close observation. In the setting of visual loss, radiation
treatment is frequently used. Surgery is usually reserved for
intracranial extension and larger tumors.
On axial imaging, the most common presentation is
the well-known tram-track appearance, caused by the
enhancing tumor wrapping around the sheath (Fig. 11-
24). Inflammation of the dura from other causes may occa-
sionally have a similar appearance. ONSM can also present
a fusiform enlargement of the sheath on one side (Fig. 11-
25). As with all meningiomas, they enhance vividly with
contrast and often demonstrate calcification. Hyperostosis
may occasionally be seen when the lesion is at the orbital
Figure 11-22.  Axial T1-weighted MRI study shows optic glioma of apex or in the optic canal. Optic nerve glioma may initially
bilateral optic nerves, with involvement of the chiasm (arrows) in a have the appearance of a meningioma on the axial images,
patient with neurofibromatosis. but on the coronal fat-suppressed enhanced MR image, the
nerve should be seen separate from the surrounding
nerve. It is not an uncommon tumor, making up between enhancing meningioma.
5% and 7% of primary orbital tumors.31 The onset occurs Although MRI is the imaging modality of choice, thin-
at a median age of 38 years and is seen four times more section CT is often helpful because it demonstrates the
frequently in females than males.31 Because meningiomas, calcifications or hyperostosis that may be present, thus
in general, occur more frequently in patients with neurofi- aiding in the differential diagnosis. A noncontrast-enhanced
bromatosis type 2, ONSM also occurs more frequently in CT scan should be performed first so the enhancing tumor
these patients. The presenting symptom with ONSM is does not hide the calcifications.

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486  II  Imaging of the Head and Neck

Figure 11-23.  A, T1-weighted


axial MRI study with gadolinium
enhancement shows optic glioma
of the optic chiasm (arrows). B, T1-
weighted coronal MRI study with
gadolinium.

A B

A B
Figure 11-24.  A, Coronal contrast-enhanced CT scan shows optic nerve sheath meningioma (arrow). B, Axial contrast-enhanced CT scan shows
same patient as in A with tram-track appearance (arrow).

axial contrast-enhanced MRI with fat suppression allows


the enhancing lesion to be seen against the fat and bone,
g which turn dark.

m • Melanoma
Primary orbital melanoma usually presents as an ocular
lesion. It originates in the uveal tract (iris, choroid, and
ciliary bodies) and may extend posteriorly to the rest of the
orbit. On CT imaging, melanomas appear as focal soft
tissue masses with mild to moderate enhancement (Fig.
Figure 11-25.  Axial contrast-enhanced CT scan shows optic nerve
11-26).42
sheath meningioma (m). g, globe; arrow, displaced optic nerve sheath
MRI studies may help differentiate melanomas from
complex emerging from mass.
other ocular lesions, evaluate its intraorbital extent, and
search for metastatic disease.51 On MRI studies, the amount
ONSMs, particularly when in the optic canal, can be of melanin contained in melanoma determines the signal
quite small and yet cause significant symptoms. As a result, characteristics. Melanin shortens T1 and T2, thereby
they can be easily missed, unless there is a high degree of causing increased signal on T1-weighted images and
suspicion and careful inspection. MRI can therefore be mildly decreased signal on T2-weighted images (Fig.
extremely useful for finding these lesions. Coronal and 11-27).

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11  Orbit  487 11

Figure 11-26.  Coronal contrast-enhanced CT scan shows melanoma A


of the left ciliary body.

C
Figure 11-28.  A, T1-weighted sagittal MRI study shows retinal
detachment with hemorrhage. B, T2-weighted axial MRI study of the
same patient as in A. C, Coronal CT scan shows retinal detachment
(different patient). (A and B, Courtesy of Guy Wilms, MD, Universitaire
Ziekenhuizer, Leuven, Belgium.)

mas and helps differentiate the tumor from retinal


detachment.6,41
While melanotic lesions have characteristic appear-
Figure 11-27.  Ocular melanoma of the inferior aspect of the globe. ances, nonpigmented melanomas cannot be reliably dif-
Top, T1-weighted coronal MRI study. Bottom, T2-weighted coronal MRI ferentiated from other masses.54
study.

MR signal is also affected by hemorrhage, which • Metastatic Disease


is not uncommon in patients with melanotic lesions. With In adults, the most common tumor to metastasize to the
hemorrhage, the differential diagnosis includes retinal orbit is carcinoma of breast. Other primary sites include
and choroidal detachment from other causes (Fig. 11-28). lung, colon, and prostate (Fig. 11-29). In children, most
The presence of gadolinium enhancement favors melano- common primary lesions include neuroblastoma,

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488  II  Imaging of the Head and Neck

leukemia, and Ewing’s sarcoma. Metastatic lesions may hereditary form of retinoblastoma and a nonfamilial
affect any of the intraorbital structures as well as the bony sporadic form. Aside from the hereditary differences, the
orbit itself (Fig. 11-30).27,60 None of the available imaging tumors are the same. Patients with nonfamilial retinoblas-
techniques offers specificity to differentiate metastases toma have unilateral solitary tumors, whereas patients with
from the many other orbital lesions. The findings may be the familial form have a much higher rate of bilateral than
subtle, with small areas of focal thickening of the globe, or unilateral disease. Patients with the familial form of reti-
large destructive lesions.21 In addition, extension of tumor noblastoma have a highly incidence of nonocular cancers
from an adjacent structure (e.g., the paranasal sinuses) may as well.
occur (Fig. 11-31). Enophthalmos may be present in The “trilateral retinoblastoma” refers to a patient who
primary disease that is often associated with extensive has bilateral retinoblastomas and a third midline tumor.
fibrous response, such as scirrhous carcinoma of the The midline tumor is histologically the same as the intra-
breast. ocular tumor and may occur in the pineal region, suprasel-
lar region, or fourth ventricle. It is important to remember

• Retinoblastoma that the midline tumor may not be seen at the same time
as the ocular tumors but may be discovered several years
Retinoblastoma is seen primarily in infants and has an later.
occurrence of 1 in every 18,000 to 30,000 live births.50 It Because most of these lesions, which arise from the
is responsible for 1% of all childhood cancer-related deaths retina, are calcified, CT is extremely important in their
in the United States.59 Early diagnosis extends the 5-year diagnosis (Fig. 11-32). The lesions will also enhance with
survival rate to more than 90%; however, if the tumor intravenous contrast. The tumor may spread in the lym-
extends beyond the globe, the mortality rate approaches phatics or along the optic nerve to gain intracranial access.
100%.33 Retinoblastoma has been strongly linked to muta- If a tumor is discovered in one globe, very close inspection
tions on the RB1 allele of chromosome 13. Whereas about of the other globe is necessary to exclude bilateral disease.
10% of cases are said to be inherited, most retinoblastoma On initial evaluation and on follow-up examinations,
cases are not inherited. Hence, there is both a familial close inspection of the pineal region, suprasellar region,
and fourth ventricle is important to seek out trilateral
disease.
Because these tumors enhance, MRI with contrast and
fat suppression is excellent for identifying the lesion;
however, CT is better at identifying the calcification.

R L

Figure 11-29.  Axial contrast-enhanced CT scan shows prostate Figure 11-30.  Axial contrast-enhanced CT scan shows bilateral
metastasis to the left orbit roof. neuroblastoma metastasis.

A B
Figure 11-31.  A, Axial contrast-enhanced CT scan shows extension of squamous cell carcinoma of the maxillary sinus to the orbit. B, Coronal
contrast-enhanced CT scan in the same patient.

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11  Orbit  489 11

Figure 11-32.  Axial contrast-enhanced CT scan shows calcified


retinoblastoma of the left eye.

Figure 11-34.  Axial contrast-enhanced CT scan shows calcified


teratoma with areas of enhancement.

similar to neoplastic processes, it is often included in the


Figure 11-33.  Rhabdomyosarcoma of the orbit in a young child. CT differential consideration of a soft tissue mass. The imaging
demonstrates enhancing soft tissue mass (R). There is aggressive bone appearance can simulate rhabdomyosarcoma.9 On CT
destruction, with the tumor extending into the ethmoid sinus (arrow). imaging, an isodense to hyperdense soft tissue mass is
seen. Enhancement of the lesion and associated lytic bone
changes are usually present.2 On MRI study, the mass is
With bilateral disease the diagnosis is easy, but with isointense to hypointense on T1-weighted images, and
a unilateral tumor it may be more difficult. Entities such isointense to hyperintense on T2-weighted images.
as Coats’ disease and Toxocaris canis infection can be con-
fused with retinoblastoma, but these typically lack contrast
enhancement. • Teratoma
The teratoma is a rare benign lesion that contains mixed

• Rhabdomyosarcoma endodermal, mesodermal, and ectodermal elements. It


usually calcifies. Because the teratoma is usually seen in
Even though the most common malignant ocular tumors neonates, knowing a patient’s age can help one decide
in children are retinoblastoma, the most common malig- whether to include this entity in a differential diagnosis
nant orbital tumors in children are rhabdomyosarcomas.9 (Fig. 11-34).24,69
They may arise primarily or secondarily in the orbits. They
are very aggressive tumors and may grow rapidly. On CT
imaging, they are seen as enhancing soft tissue masses with • Vascular Abnormalities
associated permeative or lytic bone destruction (Fig. 11-
33). On MRI, they are hypointense to isointense on T1- • Carotid Cavernous Fistula
weighted images and isointense to hyperintense on Carotid cavernous fistula is an abnormal high-flow com-
T2-weighted images. Enhancement is variable.2 munication between the arterial and venous circulations.
This results in transmission of arterial flow into the cavern-

• Langerhans Cell Histiocytosis ous sinuses, consequently leading to reversal of flow in


venous structures draining into the cavernous sinus. Two
Langerhans cell histiocytosis is not a true neoplasm but a types of carotid cavernous fistulas have been described. The
reticuloendothelial disorder of unknown origin. Like rhab- more common type is direct fistula formed by an abnormal
domyosarcoma, it occurs most often in children. Because communication between the internal carotid artery and the
its clinical presentation and imaging features are often cavernous sinus. The less common type, indirect fistula, is

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