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Part VIII. Orbital Imaging

463

TABLE 2. Positive Predictive Values, Negative Predictive Values, Sensitivity, and Specificity of Imaging Features in Evaluating Malignant versus Benign Processes (Excluding Inflammatory Lesions) in Patients with Orbital Tumors*

 

 

Benign vs. malignant (excluding inflammatory)

 

 

 

 

Positive predictive value

Negative predictive value

Sensitivity§

Specificity#

Feature

n

%

n

%

 

n

%

n

%

 

 

 

 

 

 

 

 

 

 

 

 

Number of patients

 

 

 

 

 

 

35

 

 

65

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Orbital fat

0

NA

65

65

 

0

0

65

100

Perineural involvement

4

100

65

68

 

4

11

65

100

Fat stranding

0

NA

65

65

 

0

0

65

100

Sinus opacity frontal

3

75

64

67

 

3

9

64

98

Panorbital

0

0

63

64

 

0

0

63

97

Moulding

10

83

63

72

 

10

29

63

97

Erosion

11

73

61

72

 

11

31

61

94

Lacrimal fossa

10

63

59

70

 

10

29

59

91

Nerve distribution

0

0

58

62

 

0

0

58

89

CT–hypodense

0

0

58

62

 

0

0

58

89

T2–isointense

10

56

57

70

 

10

29

57

88

CT–hyperdense

0

0

55

61

 

0

0

55

85

Diffuse

15

60

55

73

 

15

43

55

85

Sphenoid wing

2

15

54

62

 

2

6

54

83

Primary bone anterior orbit

1

7

52

60

 

1

3

52

80

Preseptal

19

59

52

76

 

19

54

52

80

Hyperostosis

0

0

51

59

 

0

0

51

78

Regular-oval

0

0

46

57

 

0

0

46

71

Irregular

18

47

45

73

 

18

51

45

69

T2–hyperintense

4

14

40

56

 

4

11

40

62

Circumscribed

19

28

17

52

 

19

54

17

26

 

 

 

 

 

 

 

 

 

 

 

 

Abbreviation: NA, not applicable.

*Only features with significantly different occurrence between malignant and benign groups were included in the calculation.

Rate of detecting disease among patients with positive test results equals number of patients with disease (malignant) and positive test results per number of patients with positive test results.

Rate of detecting nondisease among patients without positive test results equals number of patients without disease (malignant) and negative test results per number of patients with negative results.

§Rate of positive test results among patients with disease equals number of patients with disease (malignant) and positive test results per number of patients with disease (malignant).

#Rate of negative test results among patients without disease equals number of patients without disease (malignant) and negative test results per number of patients without disease (malignant).

Source: Reprinted with permission from Ophthalmology, 112(12):2196–2207, Simon GJ, et al. Rethinking orbital imaging establishing guidelines for interpreting orbital imaging studies and evaluating their predictive value in patients with orbital tumors, Copyright (2005), with permission from American Academy of Ophthalmology.

bilateral proptosis in adults. Imaging is useful to confirm the diagnosis and to monitor disease activity in some cases. MRI with fat suppression or STIR sequence can demonstrate extraocular muscle inflammation that confirms that the disease is in an active inflammatory stage that should respond to immunosuppression. The spent disease will not respond to immunomodulation and as such this distinction is clinically useful. Features of thyroid eye disease include enlarged extraocular muscles with sparing of the myotendinous insertions (Fig. 370), lacrimal gland enlargement,

proptosis, and apical crowding. There may be dirty fat and increased orbital fat volume. There may be an association with sinus disease. In dysthyroid optic neuropathy, either the orbital apex is crowded to the extent that the optic nerve becomes compressed and an optic neuropathy ensues or the optic nerve may also lose its sigmoid shape and appear straight and the assumption is made that it is stretched by progressive proptosis with presumed microvascular sequelae (Figs. 371–373). If this were the suspected diagnosis, then CT would be a more appropriate investigation because bony

464

 

 

 

 

 

Part VIII. Orbital Imaging

 

TABLE 3. Radiological and ultrasound features of common orbital disease

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Shape/

 

 

 

 

 

Tumor

Figures

Age

Sex

Location

outline

MRI

T1

T2

 

 

 

 

 

 

 

 

 

 

 

 

Intraconal

 

 

 

 

 

 

 

 

 

 

Metastases, brain

 

Older except

M=F

Intraconal > Muscle

Ill-defined

Variable

Variable

Variable

 

metastases in 2/3

 

neuroblastoma

 

>Extra conal

mass

 

 

 

 

 

Cavernous

 

Middle Age

F>M

Intraconal. Unilateral

Oval to

Well-defined

Isointense to

Hyperintense

 

hemangioma /

 

 

 

 

round

mass, round

muscle

to muscle

 

Encapsulated

 

 

 

 

encapsulated

or oval

 

 

 

 

Optic nerve

 

 

 

 

 

 

 

 

 

 

Optic nerve glioma

 

90% < 20

F>M

Optic nerve. May be

Fusiform to

Lesion may be

Isointense to

Mildly to

 

(NF1)

 

 

 

bilateral

globular

kinked

brain

strongly

 

 

 

 

 

 

 

 

 

hyperintense

 

Optic nerve glioma

 

90% < 20

F>M

Optic nerve.

Fusiform to

Fusiform

Isointense to

Mildly to

 

(non NF1)

 

 

 

Unilateral

globular

lesion

brain

strongly

 

 

 

 

 

 

 

 

 

hyperintense

 

Malignant optic

 

Adult

MF

 

Contoured

Enhancement

 

 

 

 

nerve glioma

 

 

 

 

 

in homogene-

 

 

 

 

 

 

 

 

 

 

ity and cystic

 

 

 

 

 

 

 

 

 

 

areas

 

 

 

 

Optic nerve

 

Adult

F>M

Primary tumors

Tubular

Enhances with

Isointense

Isointense

 

meningioma - may

 

 

 

arise from optic

lesion with

gadolinium.

to nerve or

to nerve or

 

be assoc with NF2

 

 

 

nerve meninges.

tramtrack

Doughnut

hypointense

hyperintense

 

 

 

 

 

Secondary tumors

signs

sign on

 

 

 

 

 

 

 

 

arise from e.g.,

 

coronal MRI

 

 

 

 

 

 

 

 

sphenoidal ridge

 

 

 

 

 

 

 

 

 

 

(Miller 2004)

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Part VIII. Orbital Imaging

 

 

 

 

 

 

465

 

 

 

 

 

 

 

 

 

 

Contrast

 

 

Echography:

 

 

 

 

CT

enhancement

Bone involvement

Associated findings

Consistency

Spike height

Regularity

Vascularity

Attenuation

 

 

 

 

 

 

 

 

 

Bone destruction

Variable

Destruction

Breast, lung, stom-

Very hard

Medium/high

Irregular

1+ to 4+

Low

 

enhancement

 

ach, thyroid, renal,

 

 

V-pattern

 

 

 

 

 

melanoma

 

 

 

 

 

Homogenous

Heterogeneous

Remodeling

No change with

Firm but

Medium/high

Regular

No

Medium

Hyperdense

enhancement

 

valsalva maneuver

decreases in

 

 

 

 

legion. Ovoid

 

 

 

size on

 

 

 

 

legions.

 

 

 

compression

 

 

 

 

Irregular kinking

Variable

Optic canal may be

Firm

Low/medium

Regular

1 to 2+

Low

of nerve

enhancement

enlarged

 

 

to 1+

 

 

 

 

 

 

 

irregular

 

 

Fusiform

Variable

Optic canal may be

Firm

Low/medium

Regular

1 to 2+

Low

enlargement of

enhancement

enlarged

 

 

to 1+

 

 

nerve

 

 

 

 

irregular

 

 

 

 

 

Firm

Low/medium

Regular

1 to 3+

Low

 

 

 

 

 

to 2+

 

 

 

 

 

 

 

irregular

 

 

Calcification in

Uniform

Canal may enlarge.

Firm

Medium/high

1 to 2+

No

Low

20-50%. Tram

enhancement

May be hyperostosis

 

 

irregular

 

 

track sign after contrast

(continued)

466

 

 

 

 

 

Part VIII. Orbital Imaging

TABLE 3. (continued)

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Shape/

 

 

 

Tumor

Figures

Age

Sex

Location

outline

MRI

T1

T2

 

 

 

 

 

 

 

 

 

Extraconal

 

 

 

 

 

 

 

 

Lymphoma

 

50-70

F>M

Any, extraconal, lac-

Range from

Moderate

Isointense to

Hyperintense

 

 

 

 

rimal. 75%

well defined

contrast

muscle

to fat on T2,

 

 

 

 

unilateral

to ill defined

enhancement

 

brighter than

 

 

 

 

 

infiltration

 

 

T1 (not

 

 

 

 

 

 

 

 

universal)

Mucocele

Adult

Arises from parana-

Contoured

 

Depends on

Depends on

 

 

sal sinus

round to

 

protein con-

protein

 

 

 

oval

 

tent. Early:

content. Early:

 

 

 

 

 

low signal.

high signal.

 

 

 

 

 

Late:high

Late: low

 

 

 

 

 

signal

signal

Varix

Any

M+F May be assoc with

Ill defined

May collapse

Hypointense

Hypointense

 

 

intracranial venous

loculated

 

 

 

 

 

abnormalities

mass

 

 

 

Lymphangioma

<10

Diffuse

Ill-defined

Hypo fat,

Hyper muscle

 

 

 

 

or round and

hyper muscle

and fat

 

 

 

 

encapsulated

 

 

 

 

 

 

with hemor-

 

 

 

 

 

 

rhage

 

 

 

Capillary

Infancy

Superonasal. Usually

Diffuse to

Hypointense

Hyperintense

 

hemangioma

 

extraconal

contoured

to fat,

to fat and

 

 

 

 

 

hyperintense

muscle

 

 

 

 

 

to muscle

 

 

Dermoid

<25

Superotemporal

Oval to

Hypointense

Isointense /

 

 

 

 

round

to fat

hypointense

 

 

 

 

encapsulated

 

to fat

 

Lacrimal gland

 

Lacrimal gland fossa.

Contoured

 

 

 

tumors

 

Orbital lobe of gland

 

 

 

 

 

 

 

 

 

 

 

Part VIII. Orbital Imaging

 

 

 

 

 

 

467

 

 

 

 

 

 

 

 

 

 

Contrast

 

 

Echography:

 

 

 

 

CT

enhancement

Bone involvement

Associated findings

Consistency

Spike height

Regularity

Vascularity

Attenuation

 

 

 

 

 

 

 

 

 

Dense mass

Variable

Erosion and sclerosis

1% of NHL pts get

Hard/firm

Low/medium

Regular

1 to 2+

Low

 

 

uncommon

orbital disease

 

 

to 1+

 

 

 

 

 

 

 

 

irregular

 

 

Good visualization Margins enhance

Remodeling and

Sinus disease

Hard

Low/medium

Regular

No

Low

of bone anatomy

thinning

 

 

 

to 2+

 

 

 

 

 

 

 

irregular

 

 

Phleboliths

Patchy contrast

May rarely cause

Rapid acquisition

Soft

Baseline/low

Regular

No

Low

 

enhancement

bone changes (islam

CT scan with

 

 

 

 

 

 

 

et al.)

valsalva

 

 

 

 

 

Irregular mass

Diffuse

No

May enlarge with

Soft but

Low/medium

3+

No

Low to

 

enhancement

 

valsalva on imaging

becomes

 

irregular

 

medium

 

 

 

but not clinically

firm with

 

but regular

 

 

 

 

 

 

hemorrhage

 

in hemorr-

 

 

 

 

 

 

 

 

hage into

 

 

 

 

 

 

 

 

cyst

 

 

Irregular mass

Contrast

No

May have skin

Moderately

Medium/high

2 to 3+

3 to 4+

Low to

 

enhancement

 

signs

firm

 

irregular

 

medium

 

Capsule enhances May mold bone

Frim

Low/medium

Regular

No

Low to

 

on MRI

 

 

to 3+ irre-

 

high

 

 

 

 

gular

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

(continued)

468

 

 

 

 

 

 

Part VIII. Orbital Imaging

TABLE 3. (continued)

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Shape/

 

 

 

Tumor

Figures

Age

Sex

Location

outline

MRI

T1

T2

 

 

 

 

 

 

 

 

 

Benign mixed

 

30-40

F=M

Benign mixed tumor

Well defined

 

 

Isointense

tumor

 

 

 

 

almond

 

 

to brain

Adenocystic

20-40

F=M Adenocystic

May be more

 

 

 

nodular

Rhabdomyo

90% < 16

Superonasal.

Diffuse to

 

Isointense or

Hyperintense

-sarcoma

 

Unilateral

contoured

 

hypointense

to fat and

 

 

 

 

 

to brain.

muscle.

 

 

 

 

 

Hypointense

Hyperintense

 

 

 

 

 

to fat, hyper-

to brain.

 

 

 

 

 

intense to

 

 

 

 

 

 

muscle

 

Leukemia

Adult CLL.

UL or BL, may

Diffuse to

 

Isointense to

Brighter

 

Children AML

involve temporal

contoured

 

muscle

on T2

 

 

fossa

 

 

 

 

Schwannoma

adult

Extraconal

Oval to

Well defined

Hypointense

Hyperintense

 

 

 

round

fusiform

to fat,

to fat

 

 

 

encapsulated

 

isointense to

 

 

 

 

 

 

muscle and

 

 

 

 

 

 

brain

 

Neuro-fibroma

adult

Localised: any,

Oval to

Well defined

Hypointense

Hyperintense

solitary

 

superior orbit

round

on MRI.

to fat,

to fat

 

 

 

well-outlined

Fusiform

isointense to

 

 

 

 

 

 

muscle and

 

 

 

 

 

 

brain

 

Neuro-fibroma

<10

Eyelid and contig-

Ill defined

Infiltrative

Hypointense

Hyperintense

Plexiform

 

ous orbit

mass

 

to fat

to fat

Hemangio-

Any, 5th decade

M=F Superior

Oval to

 

Isointense to

Isointense to

pericytoma

 

 

round

 

brain

brain

 

 

 

well-outlined

 

 

 

Fibrous

Adult

M=F Anywhere, superior,

Oval,

T1 intermedi-

T2 high signal

Histiocytoma

 

nasal extraconal

well-outlined

ate signal

 

 

 

 

 

 

 

Part VIII. Orbital Imaging

 

 

 

 

 

 

469

 

 

 

 

 

 

 

 

 

 

Contrast

 

 

Echography:

 

 

 

 

CT

enhancement

Bone involvement

Associated findings

Consistency

Spike height

Regularity

Vascularity

Attenuation

 

 

 

 

 

 

 

 

 

 

Enhances on MRI

Bone molding

 

Firm

Medium/high

Regular

0 to 1+

Medium

 

and CT

 

 

 

 

 

 

 

May calcify

 

Bone erosion

 

Firm

Low/medium

2 to 4+

0 to 1+

Medium

 

 

 

 

 

 

irregular

 

 

Well defined

Enhancement on

Bone destruction

 

Firm

Low/medium

Regular

1 to 3+

Medium

isodense to muscle

MRI

 

 

 

 

to 1+

 

 

 

 

 

 

 

 

irregular

 

 

Irregular mass

 

Subperiosteal with

 

Soft

Low/medium

Regular

No

Low

 

 

boney destruction =

 

 

 

to 1+

 

 

 

 

granulytic sarcoma

 

 

 

irregular

 

 

 

 

(AML)

 

 

 

 

 

 

Homogenous

Heterogeneous

Bone remodeling

 

Firm

Low/medium

1 to 4+

0 to 1+

Medium

isodense to brain

Enhances on CT

 

 

 

 

irregular

 

 

 

an d MRI

 

 

 

 

 

 

 

Homogenous

Heterogeneous

Bone remodeling

 

Soft to firm

Low/medium

Regular

0 to 1+

Low to

isodense to brain.

Enhances on CT

 

 

 

 

 

 

medium

May calcify

an d MRI

 

 

 

 

 

 

 

 

Variable

Bone thinning may

NF1

Soft

Medium/high

3 to 4+

1 to 2+

Low

 

 

occur

 

 

 

irregular

 

 

Homogenous

 

Possible bone erosion

 

Firm

Low/medium

Regular

0 to 1+

Low to

lesion

 

 

 

 

 

 

 

medium

 

Moderate

Possible bone

 

Firm

Low/medium

Regular

2 to 3+

Medium

 

enhancement

remodeling

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

470

FIG. 370. Coronal computed tomography scan showing enlarged extraocular muscles (type 2 Graves’ disease)

decompression is a likely clinical outcome and CT is essential for surgical planning. Scans in patients with previous orbital decompression will show a mixture of findings depending on the nature of the

Part VIII. Orbital Imaging

FIG. 372. Coronal computed tomography scan showing enlarged muscles filling the orbital apex in a patient with dysthyroid optic neuropathy (arrows)

prior surgery. Prolapse of orbital contents into the paranasal sinuses is the desired result.

Idiopathic orbital inflammation (IOI) is a common disease of the orbit that has previously been

FIG. 371. Axial computed tomography scan showing

FIG. 373. Coronal computed tomography scan showing

enlarged muscles filling the orbital apex in a patient with

predominant enlargement of orbital fat volume (type 1

dysthyroid optic neuropathy (arrows)

Graves’ disease) (arrows)

Part VIII. Orbital Imaging

described as orbital pseudotumor. It is usually but not always unilateral and may affect the lacrimal gland, the extraocular muscles, or the intraor extraconal space. The diagnosis is one of exclusion, with lymphoma being an important differential, although lymphoma is not normally painful. In contradistinction to thyroid eye disease, IOI is said to affect the extraocular muscle tendons. Posterior scleritis can be detected by thickening of the sclera, but ultrasound is the preferred investigation to allow measurements of the scleral thickness to be made.

Sarcoidosis

Sarcoidosis is rare in the orbit but may affect the lacrimal gland, the muscles, or the optic nerve. Radiologic features are nonspecific but imaging will assist with surgical planning for biopsy.

Infection

Orbital cellulitis may be preor postseptal and as such can threaten to communicate infection with the cavernous sinus via the superior ophthalmic vein (SOV), which can be a devastating consequence. The most common scenario, however, is a subperiosteal abscess associated with contiguous sinus disease. Imaging is aimed at establishing that there is not some other pathology, such as rhabdomyosarcoma, and for possible surgical intervention as via an orbitotomy. The disease often presents in the younger age group and, as a result, sedation may be required to obtain images. Coordination between the pediatrician, radiologist, and anesthesiologist is crucial.

Mucormycosis is a disease of the immunocompromised and more frequently affects diabetics. The failure to manage this condition appropriately can be fatal. Imaging is aimed at assessing the orbit, paranasal sinuses, and cavernous sinuses, and disease extent.

471

ated (Fig. 374). The purpose of the investigation is to establish the extent of the bony defect and the degree of soft tissue entrapment. The pediatric population can mislead the clinician with the white-eyed blowout, where a trapdoor greenstick-type fracture can entrap the inferior rectus and lead to rapid muscle necrosis; urgent imaging and intervention is required. A feature that should alert the referring clinician and radiologist is if the child is systemically unwell, with constitutional symptoms including fever and severe nausea associated with muscle necrosis.

Both bone and soft tissue windows should be obtained with either direct or reformatted coronal views. The corresponding sinuses are usually opaque due to blood that may manifest as a fluid level seen on axial scans. Air inside the orbit is a telltale sign of communication between the orbits and the sinuses and surgical emphysema may also be seen. The CT equivalent of the teardrop sign seen on plain films should alert the clinician to a small fracture.

Orbital foreign bodies should be sought and excluded as far as is possible (Fig. 375). A more devastating consequence of trauma is optic nerve canal damage and or traumatic optic neuropathy. Traumatic optic neuropathy may be associated with fracture of the optic canal that can be seen on CT or may be a localized pressure effect with vascular consequences that are best seen on high-resolution MRI.

Trauma

The CT scan is the investigation of choice in most cases of trauma, particularly if there is even a vague suspicion of an orbital or intraocular foreign body.81 Fracture of the orbital rim is less common but the floor and medial wall are susceptible to blowout fracture with the zygomatic complex often associ-

FIG. 374. Computed tomography scan of orbital fracture (arrows)

472

Part VIII. Orbital Imaging

FIG. 375. Computed tomography scan of orbital foreign body (arrow)

Caroticocavernous Fistulas

Caroticocavernous fistulas may be evaluated with an MRI, although selective carotid angiograms remain the gold standard but are not without risk. CT, MRI, and orbital ultrasound will show an enlarged SOV and proptosis. Orbital color Doppler can demonstrate aterialization of the SOV. The fistulae are categorized by the anatomy of the feeder vessels and the flow rate. A history of trauma (not necessarily), a bruit and blood in Schlemm’s canal, and an elevated intraocular pressure with conjunctival arterialization should prompt the diagnosis, which can be confirmed and characterized by imaging studies.

Pediatric Disease

The most common reason for obtaining a CT scan in children is in the context of orbital infection that is addressed above. It is worth noting that children can present with orbital cellulitis and a white eye and that other conditions can mimic orbital cellulitis.

The most common benign tumor in children is the capillary hemangioma. The most common orbital malignant tumor in children is a rhabdomyosarcoma, which has a variety of presentations that can mislead the clinician. A painless proptosis or a cellulitic picture can be the first sign of disease.

Summary

When presented with orbital disease, the clinician can aid diagnosis and plan appropriate surgical intervention with the judicious use of orbital imag- ing—CT, MRI, and ultrasonography. In general terms, CT is best for bony disease, for example, fractures, and for planning decompression. MRI is more useful for soft tissue interpretation. Ultrasound is an extremely useful adjunct. The location of the tumor and the imaging and associated features are helpful in reaching a differential diagnosis and in determining the likelihood of benign versus malignant disease.82

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