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## Complete Summary

#### GUIDELINE TITLE

Orbits, vision, and visual loss.

#### BIBLIOGRAPHIC SOURCE(S)

Zimmerman RD, Seidenwurm DJ, Davis PC, Brunberg JA, De La Paz RL,
Dormont PD, Hackney DB, Jordan JE, Karis JP, Mukherji SK, Turski PA,
Wippold FJ II, McDermott MW, Sloan MA, Expert Panel on Neurologic
Imaging. Orbits, vision, and visual loss. \[online publication\]. Reston
(VA): American College of Radiology (ACR); 2006. 9 p. \[31 references\]

#### GUIDELINE STATUS

This is the current release of the guideline.

This guideline updates a previous version: Hasso AN, Drayer BP, Anderson
RE, Braffman B, Davis PC, Deck MD, Johnson BA, Masaryk T, Pomeranz SJ,
Seidenwurm D, Tanenbaum L, Masdeu JC. Orbits, vision, and visual loss.
American College of Radiology. ACR Appropriateness Criteria. Radiology
2000 Jun;215(Suppl):579-87.

The appropriateness criteria are reviewed annually and updated by the
panels as needed, depending on introduction of new and highly
significant scientific evidence.

### \*\* REGULATORY ALERT \*\*

#### FDA WARNING/REGULATORY ALERT

**Note from the National Guideline Clearinghouse**: This guideline
references a drug(s) for which important revised regulatory and/or
warning information has been released.

-   [May 23, 2007, Gadolinium-based Contrast
    Agents](http://www.fda.gov/medwatch/safety/2007/safety07.htm#Gadolinium):
    The addition of a boxed warning and new warnings about the risk of
    nephrogenic systemic fibrosis (NSF) to the full prescribing
    information for all gadolinium-based contrast agents (GBCAs).

### COMPLETE SUMMARY CONTENT

 \*\* REGULATORY ALERT \*\*\
 SCOPE\
 METHODOLOGY - including Rating Scheme and Cost Analysis\
 RECOMMENDATIONS\
 EVIDENCE SUPPORTING THE RECOMMENDATIONS\
 BENEFITS/HARMS OF IMPLEMENTING THE GUIDELINE RECOMMENDATIONS\
 QUALIFYING STATEMENTS\
 IMPLEMENTATION OF THE GUIDELINE\
 INSTITUTE OF MEDICINE (IOM) NATIONAL HEALTHCARE QUALITY REPORT
CATEGORIES\
 IDENTIFYING INFORMATION AND AVAILABILITY\
 DISCLAIMER

### SCOPE

#### DISEASE/CONDITION(S)

Disorders of the orbit and optic nerve

#### GUIDELINE CATEGORY

Diagnosis\
Evaluation

#### CLINICAL SPECIALTY

Endocrinology\
Family Practice\
Internal Medicine\
Neurology\
Ophthalmology\
Pediatrics\
Radiology

#### INTENDED USERS

Health Plans\
Hospitals\
Managed Care Organizations\
Physicians\
Utilization Management

#### GUIDELINE OBJECTIVE(S)

To evaluate the appropriateness of initial radiologic examinations for
patients with disorders of the orbit and optic nerve

#### TARGET POPULATION

Patients with disorders of the orbit and optic nerve

#### INTERVENTIONS AND PRACTICES CONSIDERED

1.  Magnetic resonance imaging (MRI), brain and/or orbit

    -   Without contrast

    -   Without and with contrast

2.  Computed tomography (CT), brain and/or orbit

    -   Without contrast

    -   Without and with contrast

3.  Magnetic resonance angiography (MRA), head and neck

4.  Computed tomography angiography (CTA), head and neck

5.  X-ray, orbit

#### MAJOR OUTCOMES CONSIDERED

Utility of radiologic examinations in differential diagnosis

### METHODOLOGY

#### METHODS USED TO COLLECT/SELECT EVIDENCE

Searches of Electronic Databases

#### DESCRIPTION OF METHODS USED TO COLLECT/SELECT THE EVIDENCE

The guideline developer performed literature searches of recent
peer-reviewed medical journals, and the major applicable articles were
identified and collected.

#### NUMBER OF SOURCE DOCUMENTS

The total number of source documents identified as the result of the
literature search is not known.

#### METHODS USED TO ASSESS THE QUALITY AND STRENGTH OF THE EVIDENCE

Weighting According to a Rating Scheme (Scheme Not Given)

#### RATING SCHEME FOR THE STRENGTH OF THE EVIDENCE

Not stated

#### METHODS USED TO ANALYZE THE EVIDENCE

Systematic Review with Evidence Tables

#### DESCRIPTION OF THE METHODS USED TO ANALYZE THE EVIDENCE

One or two topic leaders within a panel assume the responsibility of
developing an evidence table for each clinical condition, based on
analysis of the current literature. These tables serve as a basis for
developing a narrative specific to each clinical condition.

#### METHODS USED TO FORMULATE THE RECOMMENDATIONS

Expert Consensus (Delphi)

#### DESCRIPTION OF METHODS USED TO FORMULATE THE RECOMMENDATIONS

Since data available from existing scientific studies are usually
insufficient for meta-analysis, broad-based consensus techniques are
needed for reaching agreement in the formulation of the appropriateness
criteria. The American College of Radiology (ACR) Appropriateness
Criteria panels use a modified Delphi technique to arrive at consensus.
Serial surveys are conducted by distributing questionnaires to
consolidate expert opinions within each panel. These questionnaires are
distributed to the participants along with the evidence table and
narrative as developed by the topic leader(s). Questionnaires are
completed by the participants in their own professional setting without
influence of the other members. Voting is conducted using a scoring
system from 1-9, indicating the least to the most appropriate imaging
examination or therapeutic procedure. The survey results are collected,
tabulated in anonymous fashion, and redistributed after each round. A
maximum of three rounds is conducted and opinions are unified to the
highest degree possible. Eighty percent agreement is considered a
consensus. This modified Delphi technique enables individual, unbiased
expression, is economical, easy to understand, and relatively simple to
conduct.

If consensus cannot be reached by the Delphi technique, the panel is
convened and group consensus techniques are utilized. The strengths and
weaknesses of each test or procedure are discussed and consensus reached
whenever possible. If \"No consensus\" appears in the rating column,
reasons for this decision are added to the comment sections.

#### RATING SCHEME FOR THE STRENGTH OF THE RECOMMENDATIONS

Not applicable

#### COST ANALYSIS

A formal cost analysis was not performed and published cost analyses
were not reviewed.

#### METHOD OF GUIDELINE VALIDATION

Internal Peer Review

#### DESCRIPTION OF METHOD OF GUIDELINE VALIDATION

Criteria developed by the Expert Panels are reviewed by the American
College of Radiology (ACR) Committee on Appropriateness Criteria.

### RECOMMENDATIONS

#### MAJOR RECOMMENDATIONS

**ACR Appropriateness Criteria®**

**Clinical Condition: Orbits, Vision and Visual Loss**

**Variant 1: Infant or child with orbital asymmetry, proptosis and
visual loss.**

  ---------------------------------------------------------------------------
  **Radiologic Exam    **Appropriateness   **Comments**
  Procedure**          Rating**            
  -------------------- ------------------- ----------------------------------
  MRI, brain and/or    8                    
  orbit, without and                       
  with contrast                            

  MRI, brain and/or    7                    
  orbit, without                           
  contrast                                 

  CT, brain and/or     6                    
  orbit, without and                       
  with contrast                            

  CT, brain and/or     5                    
  orbit, without                           
  contrast                                 

  MRA, head and neck   4                   If vascular disease suspected.

  CTA, head and neck   2                   If vascular disease suspected.

  X-ray, orbit         1                    

  ***Appropriateness                       
  Criteria Scale*\                         
  1 2 3 4 5 6 7 8 9\                       
  1 = Least                                
  appropriate 9 = Most                     
  appropriate**                            
  ---------------------------------------------------------------------------

Note: Abbreviations used in the tables are listed at the end of the
\"Major Recommendations\" field.

**Variant 2: Child with slowly progressive visual loss.**

  ---------------------------------------------------------------------------
  **Radiologic Exam    **Appropriateness   **Comments**
  Procedure**          Rating**            
  -------------------- ------------------- ----------------------------------
  MRI, brain and/or    8                    
  orbit, without and                       
  with contrast                            

  MRI, brain and/or    7                    
  orbit, without                           
  contrast                                 

  CT, brain and/or     6                    
  orbit, without and                       
  with contrast                            

  CT, brain and/or     5                    
  orbit, without                           
  contrast                                 

  MRA, head and neck   4                   If vascular disease suspected.

  CTA, head and neck   2                   If vascular disease suspected.

  X-ray, orbit         1                    

  ***Appropriateness                       
  Criteria Scale*\                         
  1 2 3 4 5 6 7 8 9\                       
  1 = Least                                
  appropriate 9 = Most                     
  appropriate**                            
  ---------------------------------------------------------------------------

Note: Abbreviations used in the tables are listed at the end of the
\"Major Recommendations\" field.

**Variant 3: Adult with sudden onset of painless or painful visual
loss.**

  ---------------------------------------------------------------------------
  **Radiologic Exam    **Appropriateness   **Comments**
  Procedure**          Rating**            
  -------------------- ------------------- ----------------------------------
  MRI, brain and/or    8                    
  orbit, without and                       
  with contrast                            

  MRI, brain and/or    7                    
  orbit, without                           
  contrast                                 

  CT, brain and/or     6                    
  orbit, without and                       
  with contrast                            

  CT, brain and/or     5                    
  orbit, without                           
  contrast                                 

  MRA, head and neck   5                   If vascular disease suspected.

  CTA, head and neck   5                   If vascular disease suspected.

  X-ray, orbit         1                    

  ***Appropriateness                       
  Criteria Scale*\                         
  1 2 3 4 5 6 7 8 9\                       
  1 = Least                                
  appropriate 9 = Most                     
  appropriate**                            
  ---------------------------------------------------------------------------

Note: Abbreviations used in the tables are listed at the end of the
\"Major Recommendations\" field.

**Variant 4: Adult patient with proptosis and/or painful visual loss.**

  ---------------------------------------------------------------------------
  **Radiologic Exam    **Appropriateness   **Comments**
  Procedure**          Rating**            
  -------------------- ------------------- ----------------------------------
  MRI, brain and/or    8                    
  orbit, without and                       
  with contrast                            

  MRI, brain and/or    7                    
  orbit, without                           
  contrast                                 

  CT, brain and/or     6                    
  orbit, without and                       
  with contrast                            

  CT, brain and/or     5                    
  orbit, without                           
  contrast                                 

  MRA, head and neck   4                   If vascular disease suspected.

  CTA, head and neck   4                   If vascular disease suspected.

  X-ray, orbit         1                    

  ***Appropriateness                       
  Criteria Scale*\                         
  1 2 3 4 5 6 7 8 9\                       
  1 = Least                                
  appropriate 9 = Most                     
  appropriate**                            
  ---------------------------------------------------------------------------

Note: Abbreviations used in the tables are listed at the end of the
\"Major Recommendations\" field.

**Variant 5: Adult patient with uveitis, scleritis, and visual loss.**

  ---------------------------------------------------------------------------
  **Radiologic Exam    **Appropriateness   **Comments**
  Procedure**          Rating**            
  -------------------- ------------------- ----------------------------------
  MRI, brain and/or    8                    
  orbit, without and                       
  with contrast                            

  MRI, brain and/or    7                    
  orbit, without                           
  contrast                                 

  CT, brain and/or     5                    
  orbit, without and                       
  with contrast                            

  CT, brain and/or     4                    
  orbit, without                           
  contrast                                 

  MRA, head and neck   4                   If vascular disease suspected.

  CTA, head and neck   4                   If vascular disease suspected.

  X-ray, orbit         1                    

  ***Appropriateness                       
  Criteria Scale*\                         
  1 2 3 4 5 6 7 8 9\                       
  1 = Least                                
  appropriate 9 = Most                     
  appropriate**                            
  ---------------------------------------------------------------------------

Note: Abbreviations used in the tables are listed at the end of the
\"Major Recommendations\" field.

**Variant 6: Adult patient with ophthalmoplegia.**

  ---------------------------------------------------------------------------
  **Radiologic Exam    **Appropriateness   **Comments**
  Procedure**          Rating**            
  -------------------- ------------------- ----------------------------------
  MRI, brain and/or    9                    
  orbit, without and                       
  with contrast                            

  MRI, brain and/or    6                    
  orbit, without                           
  contrast                                 

  CT, brain and/or     6                    
  orbit, without and                       
  with contrast                            

  MRA, head and neck   6                   If vascular abnormality suspected.

  CTA, head and neck   6                   If vascular abnormality suspected.

  CT, brain and/or     5                    
  orbit, without                           
  contrast                                 

  X-ray, orbit         1                    

  ***Appropriateness                       
  Criteria Scale*\                         
  1 2 3 4 5 6 7 8 9\                       
  1 = Least                                
  appropriate 9 = Most                     
  appropriate**                            
  ---------------------------------------------------------------------------

Note: Abbreviations used in the tables are listed at the end of the
\"Major Recommendations\" field.

**Variant 7: Head injury with visual loss.**

  ---------------------------------------------------------------------------
  **Radiologic Exam    **Appropriateness   **Comments**
  Procedure**          Rating**            
  -------------------- ------------------- ----------------------------------
  CT, brain and/or     9                    
  orbit, without                           
  contrast                                 

  MRI, brain and/or    7                   If MRI safe.
  orbit, without                           
  contrast                                 

  MRI, brain and/or    5                   If MRI safe.
  orbit, without and                       
  with contrast                            

  MRA, head and neck   4                   If vascular abnormality suspected.

  CTA, head and neck   4                   If vascular abnormality suspected.

  CT, brain and/or     3                    
  orbit, without and                       
  with contrast                            

  X-ray, orbit         2                    

  ***Appropriateness                       
  Criteria Scale*\                         
  1 2 3 4 5 6 7 8 9\                       
  1 = Least                                
  appropriate 9 = Most                     
  appropriate**                            
  ---------------------------------------------------------------------------

Note: Abbreviations used in the tables are listed at the end of the
\"Major Recommendations\" field.

**Orbital Disorders**

Primary diseases of the orbit may present with proptosis, visual
disturbances, and/or ophthalmoplegia. These signs and symptoms may occur
alone or in combination, and may be accompanied by pain and/or vascular
engorgement on the visible surface of the globe.

Proptosis is an abnormal protrusion of the globe from the orbit and may
be differentiated from exophthalmos, which is an abnormal prominence of
the globe. Clinically, it may not be possible to differentiate these two
entities, but once imaging is performed, the differentiation is clear.
Proptosis can be classified in one of three anatomic locations. Ocular
or bulbar disorders typically cause exophthalmos, unless there is
extraocular spread of the mass lesion. Many cases of proptosis are due
to primary retrobulbar disorders, including intra-orbital masses and
inflammation. Extraorbital neoplasms of the osseous orbital wall, face,
paranasal sinuses, nasal cavities, or frontal cranial fossae can also
cause proptosis. Similarly, a variety of infectious and inflammatory
lesions can cause proptosis, including cellulitis, abscess, mucocele,
inflammatory orbital syndrome (IOS) or orbital pseudotumor, sarcoidosis,
or Wegener\'s granulomatosis.

Several types of congenital or developmental lesions may cause proptosis
by enlarging intraorbital structures (macrophthalmia, colobomatous
cysts) or encroachment on the orbit due to osseous defects (naso-orbital
cephaloceles) or osseous overgrowth (developmental fibro-osseous lesions
such as fibrous dysplasia).

Visual loss can occur as a result of damage at any point along the
visual pathway extending from the globe to the occipital lobes.
Therefore, depending on the specific clinical conditions, imaging may be
performed of the orbits, anterior skull base, and/or brain. Visual loss
may be seen in infants and children with congenital absence of portions
of the eye or visual system as well as septo-optic dysplasia. Intrinsic
tumors of any portion of the visual pathway or extrinsic tumors of
adjacent structures (e.g., sella or suprasellar cistern) may produce
visual disturbances. Vascular occlusive diseases, inflammatory disease,
and demyelinating disease may produce transient or fixed visual
disturbances.

Ophthalmoplegia (abnormally limited eye movement) may be the result of
intrinsic abnormalities within the extra-ocular muscles, extrinsic
compression of these muscles by orbital masses, or abnormalities of the
cranial nerves and brain stem nuclei that innervate these muscles.

CT and MRI are complementary diagnostic procedures and may be used
together in some circumstances. Dedicated thin section multiplanar
orbital imaging is necessary for detecting orbital abnormalities. The
intrinsic contrast provided by orbital fat allows for excellent anatomic
visualization with either technique. Contrast enhancement is important
in assessing most orbital disorders. Because of the absence of radiation
and the utility of fat suppressed contrast enhanced images, MRI is the
procedure of choice for orbital disorders with the exception of trauma
and assessment for foreign bodies.

**Optic Nerve and Sheath Disorders**

Primary disorders of the optic nerve and optic nerve sheath typically
cause visual disturbances and occasionally proptosis in cases of large
neoplasms. The primary neoplasms of the optic nerve include optic nerve
tumors (gliomas, astrocytomas, and hamartomas) and meningiomas. There
may be a para-optic component of optic nerve tumors that is
characteristically seen in patients with neurofibromatosis type I. The
para-optic component is due to perineural arachnoidal gliomatosis
consisting of a proteinaceous material that seeds and spreads within the
optic nerves subarachnoid space and is a portion of the tumor. This
process results in optic nerve elongation and resultant kinking of the
nerve just posterior to the globe. There is no correlation between the
size of the lesion and the extent of visual loss.

Extension of tumors into the optic chiasm, optic tracts, and lateral
geniculate bodies of the thalami is more accurately depicted on MRI than
on CT. The size and shape of the optic canals are best assessed in the
axial projection, while the size and shape of the optic nerves are best
appreciated on coronal and oblique sagittal images. Many optic nerve
tumors exhibit fusiform homogeneous enhancement, while the unenhanced
portions of optic nerve tumors may represent the sites of arachnoidal
gliomatosis.

Meningiomas are the most common optic nerve sheath tumors, arising from
the arachnoidal coverings of the optic nerve. Visual loss and optic
atrophy are the usual presenting symptoms. Most cases occur in
middle-aged or elderly patients, more often in women than in men. The
tumor may be cuff-like, surrounding the optic nerves, or eccentrically
located on one side of the nerve. CT scans will often demonstrate
calcifications. Enhancement parallel to the length of the optic nerves
with the intact nerve seen within the mass (\"tram-tracking\") is seen
on both CT and MRI. MRI scans also readily depict the spread into
adjacent meninges. Parallel optic cysts may be identified surrounding
the optic nerve immediately distal to the meningioma. This process
causes trapping of the cerebrospinal fluid in the subarachnoid space and
can add to the mass effect and proptosis.

The papilledema associated with pseudotumor cerebri or intracranial mass
may be detected on CT or MRI as enlargement of the optic nerve. If
severe, there is a reversal of the optic nerve head, with bulging
forward into the posterior wall of the globe. While dilatation of the
perioptic subarachnoid space is best appreciated on fat suppressed T2
weighted images, reversal of the nerve head may be more readily detected
on CT than on MRI because of the chemical shift artifact inherent to the
MR studies. There is some correlation between the severity of the visual
loss and the detection of enlarged nerves with reversed nerve heads.
Patients with more severe visual loss demonstrate more frequent and more
severe reversal of the optic nerve head. Prominent perioptic
subarachnoid spaces and consequent mild diffuse enlargement of the optic
nerves, however, are most often asymptomatic imaging findings unrelated
to increased intracranial pressure.

**Other Orbital Neoplasms**

Primary neoplasms may arise from any constituent orbital tissue. The
most common tumors are benign cavernous hemangiomas. These lesions
typically present in adulthood with proptosis. They may occur anywhere
within the orbit, but they have a predilection for the intraconal space.
They are seen as focal round or oval masses, often with vascular
calcifications. Complex vascular lesions such as lymphangiomas and
capillary hemangiomas are multicompartment lesions that occur in
infancy. They tend to regress with age. Schwannomas arising from
branches of V3, V4, V5, and V6 may rarely occur in the orbit. V1 and V2
tumors may extend into the orbit from the cavernous sinus. Primary
benign and malignant lacrimal gland tumors may occur. Metastatic and
lymphomatous involvement of the soft tissues of the orbit (without
osseous disease) may present as an isolated intraconal mass (e.g.,
breast carcinoma and melanoma) or as involvement of an orbital structure
(e.g., lymphomatous infiltration of the lacrimal gland).

**Optic Nerve Neuropathies**

Optic neuritis cannot be reliably detected with CT. It is seen on MRI as
(focal or diffuse enlargement of the optic nerve) abnormal signal
intensity (hyperintensity on T2 weighted images) and/or enhancement.
These features are best appreciated on fat suppressed T2 weighted and
contrast enhanced T1 weighted images. Optic neuritis is caused by
autoimmune inflammation of the optic nerve, which affects the myelin
sheaths with relative preservation of the axons. Optic neuritis is the
initial manifestation of multiple sclerosis (MS) in about 20% of cases
and may occur at some point in the disease in approximately 50% of
cases. Even when MRI scans of the orbit are normal imaging of the brain
can reveal foci of demyelination that allow for differentiation of those
patients who will subsequently develop MS from those who will not. Thus,
MRI is a predictor of MS, as it can help identify a subgroup of patients
with optic neuritis whose risk of developing MS appears to be low, and
therefore it can help predict the development of MS after optic
neuritis.

Isolated visual disturbances may be caused by a variety of optic nerve
neuropathies, including radiation, chemotherapy, compressive phenomena,
or ischemia. Radiation-induced optic neuropathy (RON) is a rare
catastrophic complication of radiation therapy regimens used to treat a
variety of neoplasms of the skull base, sella, and parasellar regions.
There is typically a latency period of 6 to 36 months following
treatment. Clinically, the nerve head may appear normal, but
gadolinium-enhanced fat suppressed MR imaging will show patchy, linear,
or confluent enhancement along the portions of the optic nerve, chiasm,
or optic tract. This correlates precisely with the development of
delayed visual loss after radiation therapy. Treatment of RON with
corticosteroids may be helpful, although optic atrophy may develop with
permanent visual loss.

Compressive optic nerve neuropathies can be caused by a variety of
lesions in the orbital apex including inflammatory orbital syndrome
(IOS) (orbital pseudotumor), thyroid ophthalmopathy with muscular
hypertrophy and edema causing compression of the intraocular portion of
the optic nerve, or other systemic diseases with orbital manifestations
such as sarcoidosis or Wegener\'s granulomatosis

**Vascular Disorders**

Compression of the optic nerve may also occur as a result of cavernous
carotid fistulae, arteriovenous malformations, or orbital varices. Such
vascular anomalies may produce retrograde flow through the ophthalmic
vessels with subsequent dilatation of the orbital veins and passive
congestion of the orbital tissues. This leads to progressive increase in
the intraocular pressure and subsequent decrease in visual acuity, or
even blindness. Spontaneous thrombosis of the ophthalmic veins
occasionally occurs and may aggravate the mass effect within the apex of
the orbit and the compressive neuropathy.

Imaging (MRI and CT) will demonstrate the dilated ophthalmic veins,
facial veins, and other regional venous structures along with
enlargement of the cavernous sinus. Large edematous extraocular muscles
and periorbital structures may be identified. Proptosis is typically
present. The addition of MR angiography or CT angiography allows for
flow assessments along with the static morphologic changes. In some
cases, conventional angiography may be required to make the definitive
diagnosis, although this is most commonly used in conjunction with
therapeutic interventional procedures.

Traumatic optic neuropathy (TON) results in blindness following optic
nerve injury. The injury is typically from head trauma without direct
effects on the globe or retina. The clinical presentation of TON may be
delayed, as the patients may present with depressed levels of
consciousness. Post-traumatic visual loss can be evaluated by
investigation of the soft tissue and osseous structures around the optic
nerve and chiasm. Thin section CT scans with multiplanar reconstruction
are the most useful. Such images provide accurate identification of
indirect signs of injury to the optic nerve, such as dehiscence, or bony
fragments within the orbit or optic nerve canal, narrowing of the optic
canal, or significant bony separations which indicate likely optic nerve
injury. MR images have been shown to be more sensitive for detecting
optic nerve edema or avulsion. Differential response to steroid therapy
or orbital surgical decompressive procedures remains controversial and
shows no clear-cut advantages.

**Inflammatory Orbital Syndrome**

Inflammatory orbital syndrome (IOS) (orbital pseudotumor, inflammatory
fibromyotendinitis) may appear as an acute or chronic cause of
ophthalmoplegia, proptosis, and visual loss that develops as a diffuse
infiltrate or focal mass. In the diffuse form, there is inflammatory
infiltrate of the orbital fat, extraocular muscles, and adnexal
structures, particularly in the orbital apex. The more focal forms of
IOS commonly involve the tendinous portions of the extraocular muscles
(myositic form), the uveal structures (anterior form), the scleral
region (posterior form), or the lacrimal gland (lacrimal form). All
forms of the disease show extensive infiltration that is histologically
composed of polyclonal lymphocytes, plasma cells, neutrophils, and
macrophages with various amounts of fibrosis. Commonly, the retrobulbar
fat has a \"dirty\" appearance.

The acute form of IOS is typically unilateral, developing over days to
weeks, and is characterized by ophthalmoplegia pain, swelling, and
tenderness. The chronic form of the disease may occur bilaterally in
approximately 10%-15% of cases, develops insidiously over weeks to
months, and lacks inflammatory symptoms.

CT and MRI show intraconal or extraconal soft tissue lesions that are
diffuse or localized and commonly involve the orbital apices.
Occasionally, there may be a well-defined mass lesion that mimics a
neoplasm. In virtually all cases, there is prominent enhancement on
postcontrast CT or MR scans. In the chronic form of the disease there is
increased fibrosis in the lesions, resulting in decreased signal on
T2-weighted images.

Many cases of IOS resolve following steroid therapy, but the process may
progress to a lymphoproliferative disorder or lymphoma. The
differentiation of IOS from lymphoma is based on histopathologic
examination, with a predominant polyclonal lymphocytic population in the
earlier disorder and a monoclonal lymphocytic process in the latter
disorder. CT or MRI scans may be used to follow the course of the
illness until it resolves, or recurs in the chronic form of the disease.

As previously mentioned, there may be involvement of the optic nerve,
resulting in alterations of visual acuity and/or involvement of the
ipsilateral cavernous sinus. When there is secondary thrombosis of the
sphenoidal veins or cavernous sinus, a painful ophthalmoplegia results,
with the presumptive diagnosis of Tolosa-Hunt syndrome. Although usually
confined to the orbital soft tissues, IOS can produce bone destruction
or extraorbital extension.

A small subset of patients with isolated ocular manifestations of IOS
have posterior scleritis. These patients are seen for ocular pain and
proptosis within limitation of gaze, scleral effusions, disc edema, and
intraocular hemorrhages. Posterior scleritis shows inflammatory signs in
the coat of the eye sclera with thickening of the posterior coat of the
eye sclera that may be identified as areas of enhancement on CT or MRI.
The thickened sclera, enhanced by contrast, presents as a so-called
\"ring sign.\" Sarcoidosis (neurosarcoidosis) and Wegener\'s
granulomatosis both simulate IOS, lymphoproliferative disorders, or
metastatic neoplasms. There are no key clinical differentiating
features, nor is the response to corticosteroid therapy an indication of
the etiology. The definitive diagnosis is made by biopsy and is often
essential in order to direct primary treatment.

**Endocrine Disorders**

Thyroid ophthalmopathy (Graves\' disease) may be detected in patients
who are hyperoid-thyroid, hypoid-thyroid, or euthyroid. Many (25-50%) of
these cases occur in patients with Graves\' hyperthyroidism and
occasionally in patients with Hashimoto\'s thyroiditis. The
female-to-male ratio is 4 to 1, and approximately 15% of patients are
under age 15. In all age groups, approximately 15% of unilateral orbital
proptosis and the majority of bilateral proptosis are secondary to
thyroid ophthalmopathy.

The disease presents with eyelid lag, upper lid retraction, diffuse
conjunctival edema, and vascular injection at the insertion of the
rectus muscles. It is characterized as an immune disorder with both
cell-mediated and humoral components. The clinical features are
secondary to overproduction of glycosaminoglycans, which are produced by
stimulated fibroblasts. The overstimulation of fibroblasts is thought to
be centered on the T-cell lymphocytes, which recognize fibroblasts\'
cross-reactive antigens and release cytokines. The overly produced
glycosaminoglycans are deposited into muscle bellies of the extraocular
muscles in a perivascular location. This results in endomysial fibrosis
secondary to the mucopolysaccharide deposits.

On CT and MRI studies, there is enlargement of one or more of the
extraocular rectus muscles. Multiple muscle involvement is much more
common than one or two isolated muscle involvement. The disease is
bilateral in at least 85% of cases by imaging criteria. The inferior
rectus is most commonly and severely involved, followed by the medial
superior and lateral rectus muscles. The posterior and middle third of
the muscle bellies are affected, with relative sparring of tendinous
insertions. The inherent soft tissue contrast of MR scans provides
elegant morphologic information regarding the involvement of the
extraocular muscles in patients with thyroid ophthalmopathy. An
important role of imaging is demonstrating the relationship of the
extraocular muscles to the optic nerve at the orbital apex, and the
degree of stretching of the optic nerve due to proptosis, particularly
if surgery is contemplated. The ability to measure the T2 signal
intensity on MRI helps both in determining which patients may benefit
from corticosteroid therapy (those with high T2 values), and/or which
patients require combined therapies including cyclosporin (based on a
measurable response on serial MR images).

**Disorders of Size or Shape of the Globe**

A staphyloma represents a diffusely enlarged globe with thin scleral
margins resulting from degeneration of the bulbar coverings. The lesion
is thought to be secondary to the normal aging process and is only seen
in the elderly population, either unilaterally or bilaterally. Imaging
studies will demonstrate the enlarged globe with thin walls and no other
lesions. Most often, enlargement is an incidental finding on images that
were obtained for unrelated conditions. A diffusely enlarged globe is
seen in patients with severe axial myopia, which, unlike a staphyloma,
is a heritable condition treated by corrective lenses or keratotomy
Staphyloma is distinguished from coloboma, a congenital lesion where
there is a complete defect in the wall of the globe with focal
outpouching of the posterior globe at the optic nerve head. Coloboma may
be isolated or seen in association with other congenital anomalies of
the eye, anterior skull base, and/or brain.

**Retinal, Choroidal, and Subhyaloid Detachments**

Serous choroidal detachments result from inflammatory diseases (uveitis,
scleritis) or by accidental perforation of the eyeball. As the edema of
the choroid increases, fluid accumulates in the subchoroidal potential
space. Hemorrhagic choroidal detachments often occur after a contusion,
a penetrating injury, or as a complication of intraocular surgery. The
differentiation of choroidal effusion from choroidal hemorrhage may be
obtained using multiple MRI sequences. There is a crescentic ring-shaped
area of increased signal on both T1- and T2-weighted images. With
choroidal hemorrhages, the signal intensity varies according to the age
of the hemorrhage. In acute hemorrhages, CT may be more specific,
showing the increased density of subchoroidal hemorrhage.

Retinal detachments as a complication of systemic diseases such as
hypertension or diabetes are fairly common and rarely require imaging.
Retinal detachments may also occur with primary ocular neoplasms such as
retinoblastomas in children and as uveal malignant melanomas in adults
and elderly patients. Ocular sonography may be more accurate in
detecting small tumors; however, enhanced MR images are useful in
determining the true extent of lesions beyond the ocular structures and
also demonstrating associated retinal detachments. CT scanning has
specific value in assessing patients with retinoblastoma, since small
punctuate calcifications in the contralateral \"normal\" eye indicate
the presence of bilateral disease, altering management and prognosis.
Improvement in the differential diagnosis is based on postcontrast
T1-weighted images, which are most helpful in detecting uveal melanomas
and in differentiating melanomas from subretinal fluid collections.
There is enhancement in the case of neoplasms, but not from fluid
collections. The differentiation of an amelanotic melanoma from a
hemorrhagic subretinal hemorrhage is based on both the precontrast and
postcontrast T1-weighted images. Of note are metastatic lesions to the
retina or certain inflammatory conditions that cannot be consistently
differentiated from primary uveal melanomas. Doppler sonography may help
detect vascularity within an intraocular tumor and help differentiate
such entities from nonvascular choroidal, subretinal, or subhyaloid
effusions, or from hematomas.

**Abbreviations**

-   CT, computed tomography

-   CTA, computed tomography angiography

-   MRA, magnetic resonance angiography

-   MRI, magnetic resonance imaging

#### CLINICAL ALGORITHM(S)

Algorithms were not developed from criteria guidelines.

### EVIDENCE SUPPORTING THE RECOMMENDATIONS

#### TYPE OF EVIDENCE SUPPORTING THE RECOMMENDATIONS

The recommendations are based on analysis of the current literature and
expert panel consensus.

### BENEFITS/HARMS OF IMPLEMENTING THE GUIDELINE RECOMMENDATIONS

#### POTENTIAL BENEFITS

Selection of appropriate radiologic imaging procedures for evaluation of
patients with disorders of the orbit and optic nerve

#### POTENTIAL HARMS

Not stated

### QUALIFYING STATEMENTS

#### QUALIFYING STATEMENTS

An American College of Radiology (ACR) Committee on Appropriateness
Criteria and its expert panels have developed criteria for determining
appropriate imaging examinations for diagnosis and treatment of
specified medical condition(s). These criteria are intended to guide
radiologists, radiation oncologists and referring physicians in making
decisions regarding radiologic imaging and treatment. Generally, the
complexity and severity of a patient\'s clinical condition should
dictate the selection of appropriate imaging procedures or treatments.
Only those exams generally used for evaluation of the patient\'s
condition are ranked. Other imaging studies necessary to evaluate other
co-existent diseases or other medical consequences of this condition are
not considered in this document. The availability of equipment or
personnel may influence the selection of appropriate imaging procedures
or treatments. Imaging techniques classified as investigational by the
U.S. Food and Drug Administration (FDA) have not been considered in
developing these criteria; however, study of new equipment and
applications should be encouraged. The ultimate decision regarding the
appropriateness of any specific radiologic examination or treatment must
be made by the referring physician and radiologist in light of all the
circumstances presented in an individual examination.

### IMPLEMENTATION OF THE GUIDELINE

#### DESCRIPTION OF IMPLEMENTATION STRATEGY

An implementation strategy was not provided.

#### IMPLEMENTATION TOOLS

Personal Digital Assistant (PDA) Downloads

For information about
[availability](#identifying-information-and-availability), see the
\"Availability of Companion Documents\" and \"Patient Resources\" fields
below.

### INSTITUTE OF MEDICINE (IOM) NATIONAL HEALTHCARE QUALITY REPORT CATEGORIES

#### IOM CARE NEED

Getting Better\
Living with Illness

#### IOM DOMAIN

Effectiveness

### IDENTIFYING INFORMATION AND AVAILABILITY

#### BIBLIOGRAPHIC SOURCE(S)

Zimmerman RD, Seidenwurm DJ, Davis PC, Brunberg JA, De La Paz RL,
Dormont PD, Hackney DB, Jordan JE, Karis JP, Mukherji SK, Turski PA,
Wippold FJ II, McDermott MW, Sloan MA, Expert Panel on Neurologic
Imaging. Orbits, vision, and visual loss. \[online publication\]. Reston
(VA): American College of Radiology (ACR); 2006. 9 p. \[31 references\]

#### ADAPTATION

Not applicable: The guideline was not adapted from another source.

#### DATE RELEASED

1999 (revised 2006)

#### GUIDELINE DEVELOPER(S)

American College of Radiology - Medical Specialty Society

#### SOURCE(S) OF FUNDING

The American College of Radiology (ACR) provided the funding and the
resources for these ACR Appropriateness Criteria®.

#### GUIDELINE COMMITTEE

Committee on Appropriateness Criteria, Expert Panel on Neurologic
Imaging

#### COMPOSITION OF GROUP THAT AUTHORED THE GUIDELINE

*Panel Members*: Robert D Zimmerman, MD; David J. Seidenwurm, MD;
Patricia C. Davis, MD; James A. Brunberg, MD; Robert Louis De La Paz,
MD; Pr. Didier Dormont; David B. Hackney, MD; John E. Jordan, MD; John
P. Karis, MD; Suresh Kumar Mukherji, MD; Patrick A Turski, MD; Franz J
Wippold II, MD; Michael W. McDermott, MD; Michael A. Sloan, MD, MS

#### FINANCIAL DISCLOSURES/CONFLICTS OF INTEREST

Not stated

#### GUIDELINE STATUS

This is the current release of the guideline.

This guideline updates a previous version: Hasso AN, Drayer BP, Anderson
RE, Braffman B, Davis PC, Deck MD, Johnson BA, Masaryk T, Pomeranz SJ,
Seidenwurm D, Tanenbaum L, Masdeu JC. Orbits, vision, and visual loss.
American College of Radiology. ACR Appropriateness Criteria. Radiology
2000 Jun;215(Suppl):579-87.

The appropriateness criteria are reviewed annually and updated by the
panels as needed, depending on introduction of new and highly
significant scientific evidence.

#### GUIDELINE AVAILABILITY

Electronic copies: Available in Portable Document Format (PDF) from the
[American College of Radiology (ACR) Web
site](http://www.acr.org/SecondaryMainMenuCategories/quality_safety/app_criteria/pdf/ExpertPanelonNeurologicImaging/OrbitsVisionandVisualLossDoc11.aspx).

ACR Appropriateness Criteria® *Anytime*, *Anywhere*™ (PDA application).
Available from the [ACR Web
site](http://www.acr.org/SecondaryMainMenuCategories/ACRStore/FeaturedCategories/QualityandSafety/ac_pda.aspx).

Print copies: Available from the American College of Radiology, 1891
Preston White Drive, Reston, VA 20191. Telephone: (703) 648-8900.

#### AVAILABILITY OF COMPANION DOCUMENTS

The following is available:

-   ACR Appropriateness Criteria®. Background and development. Reston
    (VA): American College of Radiology; 2 p. Electronic copies:
    Available in Portable Document Format (PDF) from the [American
    College of Radiology (ACR) Web
    site](http://www.acr.org/SecondaryMainMenuCategories/quality_safety/app_criteria/pdf/background_dev.aspx).

#### PATIENT RESOURCES

None available

#### NGC STATUS

This summary was completed by ECRI on July 31, 2001. The information was
verified by the guideline developer as of August 24, 2001. This summary
was updated by ECRI on August 17, 2006. This summary was updated by ECRI
Institute on May 17, 2007 following the U.S. Food and Drug
Administration (FDA) advisory on Gadolinium-based contrast agents. This
summary was updated by ECRI Institute on June 20, 2007 following the
U.S. Food and Drug Administration (FDA) advisory on gadolinium-based
contrast agents.

#### COPYRIGHT STATEMENT

Instructions for downloading, use, and reproduction of the American
College of Radiology (ACR) Appropriateness Criteria® may be found on the
[ACR Web
site.](http://www.acr.org/SecondaryMainMenuCategories/quality_safety/app_criteria/ACRAppropriatenessCriteriaTermsandConditionsDoc1.aspx)

### DISCLAIMER

#### NGC DISCLAIMER

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Date Modified: 11/3/2008