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  • Guideline Summary
  • NGC:009239
  • 1999 (revised 2012)

ACR Appropriateness Criteria® orbits, vision and visual loss.

Wippold FJ II, Cornelius RS, Berger KL, Broderick DF, Davis PC, Douglas AC, Germano IM, Hadley JA, McDermott MW, Mechtler LL, Smirniotopoulos JG, Waxman AD, Expert Panel on Neurologic Imaging. ACR Appropriateness Criteria® orbits, vision and visual loss. [online publication]. Reston (VA): American College of Radiology (ACR); 2012. 12 p. [31 references]

View the original guideline documentation External Web Site Policy

This is the current release of the guideline.

This guideline updates a previous version: Wippold FJ II, Cornelius RS, Broderick DF, Brown DC, Brunberg JA, Davis PC, De La Paz RL, Garvin CF, Germano I, McConnell CT Jr, McDermott MW, Mukherji SK, Seidenwurm DJ, Sloan MA, Smirniotopoulos JG, Expert Panel on Neurologic Imaging. ACR Appropriateness Criteria® orbits, vision and visual loss. [online publication]. Reston (VA): American College of Radiology (ACR); 2009. 9 p.

Age Group

UMLS Concepts (what is this?)

SNOMEDCT_US
Angiography (77343006), Choroidal detachment (27871007), Computerized axial tomography (77477000), Congenital exophthalmos (93081001), Contrast media (385420005), Contrast media (407935004), CT angiography (418272005), Differential diagnosis (47965005), Dim vision (246635007), Disorder of optic nerve (77157004), Exophthalmos (18265008), External ophthalmoplegia (19373007), Imaging (363679005), Injury of head (82271004), Internuclear ophthalmoplegia (49823009), Iridocyclitis (77971008), Magnetic resonance imaging (113091000), Magnetic resonance imaging (312250003), Magnetic resonance imaging (MRI) of vessels (241663008), Magnetic resonance imaging unit (90003000), Neoplasm of optic nerve (395505000), Ophthalmoplegia (16110005), Radiographic imaging procedure (363680008), Retinal detachment (42059000), Scleritis (78370002), Sudden visual loss (15203004), Traumatic optic nerve injury (230515007), Traumatic optic nerve injury (48233004), Unexplained visual loss (421293001), Uveitis (128473001)

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 Procedure Rating Comments RRL*
MRI head and orbit without and with contrast 8 CT may be considered the preferred imaging modality when rhinologic or paranasal sinus disease is the suspected etiology for the symptoms and signs. See statement regarding contrast in text under "Anticipated Exceptions." O
MRI head and orbit without contrast 7 CT may be considered the preferred imaging modality when rhinologic or paranasal sinus disease is the suspected etiology for the symptoms and signs. O
CT head with contrast 6 Thin slices dedicated to the orbits are useful for orbit disease and may be substituted for the complete head examination in selected patients. radioactive radioactive radioactive
CT head without and with contrast 6 CT may be considered the preferred imaging modality when rhinologic or paranasal sinus disease is the suspected etiology for the symptoms and signs. Thin slices dedicated to the orbits are useful for orbit disease and may be substituted for the complete head examination in selected patients. radioactive radioactive radioactive radioactive
CT head without contrast 5 Thin slices dedicated to the orbits are useful for orbit disease and may be substituted for the complete head examination in selected patients. radioactive radioactive radioactive
MRA head and neck without contrast 4   O
MRA head and neck without and with contrast 4 See statement regarding contrast in text under "Anticipated Exceptions." O
CTA head and neck with contrast 2 If vascular disease is suspected. radioactive radioactive radioactive radioactive
X-ray orbit 1   radioactive
Rating Scale: 1,2,3 Usually not appropriate; 4,5,6 May be appropriate; 7,8,9 Usually appropriate *Relative Radiation Level

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 Procedure Rating Comments RRL*
MRI head and orbit without and with contrast 8 See statement regarding contrast in text under "Anticipated Exceptions." O
MRI head and orbit without contrast 7   O
CT head with contrast 6 Thin slices dedicated to the orbits are useful for orbit disease and may be substituted for the complete head examination in selected patients. radioactive radioactive radioactive
CT head without and with contrast 6 Thin slices dedicated to the orbits are useful for orbit disease and may be substituted for the complete head examination in selected patients. radioactive radioactive radioactive radioactive
CT head without contrast 5 Thin slices dedicated to the orbits are useful for orbit disease and may be substituted for the complete head examination in selected patients. radioactive radioactive radioactive
MRA head and neck without contrast 4   O
MRA head and neck without and with contrast 4 See statement regarding contrast in text under "Anticipated Exceptions." O
CTA head and neck with contrast 2 If vascular disease is suspected. radioactive radioactive radioactive radioactive
X-ray orbit 1   radioactive
Rating Scale: 1,2,3 Usually not appropriate; 4,5,6 May be appropriate; 7,8,9 Usually appropriate *Relative Radiation Level

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 Procedure Rating Comments RRL*
MRI head and orbit without and with contrast 8 CT may be considered the preferred imaging modality when rhinologic or paranasal sinus disease is the suspected etiology for the symptoms and signs. See statement regarding contrast in text under "Anticipated Exceptions." O
MRI head and orbit without contrast 7 CT may be considered the preferred imaging modality when rhinologic or paranasal sinus disease is the suspected etiology for the symptoms and signs. O
CT head with contrast 6 CT may be considered the preferred imaging modality when rhinologic or paranasal sinus disease is the suspected etiology for the symptoms and signs. Thin slices dedicated to the orbits are useful for orbit disease and may be substituted for the complete head examination in selected patients. radioactive radioactive radioactive
CT head without contrast 5 Thin slices dedicated to the orbits are useful for orbit disease and may be substituted for the complete head examination in selected patients. radioactive radioactive radioactive
CT head without and with contrast 5 Thin slices dedicated to the orbits are useful for orbit disease and may be substituted for the complete head examination in selected patients. radioactive radioactive radioactive
CTA head and neck with contrast 5 If vascular disease is suspected. radioactive radioactive radioactive
MRA head and neck without contrast 4   O
MRA head and neck without and with contrast 4 See statement regarding contrast in text under "Anticipated Exceptions." O
X-ray orbit 1   radioactive
Rating Scale: 1,2,3 Usually not appropriate; 4,5,6 May be appropriate; 7,8,9 Usually appropriate *Relative Radiation Level

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 Procedure Rating Comments RRL*
MRI head and orbit without and with contrast 8 CT may be considered the preferred imaging modality when rhinologic or paranasal sinus disease is the suspected etiology for the symptoms and signs. See statement regarding contrast in text under "Anticipated Exceptions." O
MRI head and orbit without contrast 7 CT may be considered the preferred imaging modality when rhinologic or paranasal sinus disease is the suspected etiology for the symptoms and signs. O
CT head with contrast 6 CT may be considered the preferred imaging modality when rhinologic or paranasal sinus disease is the suspected etiology for the symptoms and signs. Thin slices dedicated to the orbits are useful for orbit disease and may be substituted for the complete head examination in selected patients. radioactive radioactive radioactive
CT head without and with contrast  6 Thin slices dedicated to the orbits are useful for orbit disease and may be substituted for the complete head examination in selected patients. radioactive radioactive radioactive
CT head without contrast 5 Thin slices dedicated to the orbits are useful for orbit disease and may be substituted for the complete head examination in selected patients. radioactive radioactive radioactive
MRA head and neck without contrast 4   O
MRA head and neck without and with contrast 4 See statement regarding contrast in text under "Anticipated Exceptions." O
CTA head and neck with contrast 4 If vascular disease is suspected. radioactive radioactive radioactive
X-ray orbit 1   radioactive
Rating Scale: 1,2,3 Usually not appropriate; 4,5,6 May be appropriate; 7,8,9 Usually appropriate *Relative Radiation Level

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 Procedure Rating Comments RRL*
MRI head and orbit without and with contrast 8 See statement regarding contrast in text under "Anticipated Exceptions." O
MRI head and orbit without contrast 7   O
CT head with contrast 5 Thin slices dedicated to the orbits are useful for orbit disease and may be substituted for the complete head examination in selected patients. radioactive radioactive radioactive
CT head without and with contrast  5 Thin slices dedicated to the orbits are useful for orbit disease and may be substituted for the complete head examination in selected patients. radioactive radioactive radioactive
CTA head and neck with contrast 4 If vascular disease is suspected. radioactive radioactive radioactive
MRA head and neck without contrast 4   O
MRA head and neck without and with contrast 4 See statement regarding contrast in text under "Anticipated Exceptions." O
CT head without contrast 4 Thin slices dedicated to the orbits are useful for orbit disease and may be substituted for the complete head examination in selected patients. radioactive radioactive radioactive
X-ray orbit 1   radioactive
Rating Scale: 1,2,3 Usually not appropriate; 4,5,6 May be appropriate; 7,8,9 Usually appropriate *Relative Radiation Level

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

Variant 6: Adult patient with ophthalmoplegia.

Radiologic Procedure Rating Comments RRL*
MRI head and orbit without and with contrast 9 See statement regarding contrast in text under "Anticipated Exceptions." O
MRI head and orbit without contrast 6   O
MRA head and neck without contrast 6   O
MRA head and neck without and with contrast 6 See statement regarding contrast in text under "Anticipated Exceptions." O
CT head with contrast 6 Thin slices dedicated to the orbits are useful for orbit disease and may be substituted for the complete head examination in selected patients. radioactive radioactive radioactive
CT head without and with contrast 6 Thin slices dedicated to the orbits are useful for orbit disease and may be substituted for the complete head examination in selected patients. radioactive radioactive radioactive
CTA head and neck with contrast 6 If vascular disease is suspected. radioactive radioactive radioactive
CT head without contrast 5 Thin slices dedicated to the orbits are useful for orbit disease and may be substituted for the complete head examination in selected patients. radioactive radioactive radioactive
X-ray orbit 1   radioactive
Rating Scale: 1,2,3 Usually not appropriate; 4,5,6 May be appropriate; 7,8,9 Usually appropriate *Relative Radiation Level

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

Variant 7: Head injury with visual loss.

Radiologic Procedure Rating Comments RRL*
CT head without contrast 7 Thin slices dedicated to the orbits are useful for orbit disease and may be substituted for the complete head examination in selected patients. radioactive radioactive radioactive
MRI head and orbit without contrast 7 If MRI is safe. O
MRI head and orbit without and with contrast 5 If MRI safe. See statement regarding contrast in text under "Anticipated Exceptions." O
CT head with contrast 5 Thin slices dedicated to the orbits are useful for orbit disease and may be substituted for the complete head examination in selected patients. radioactive radioactive radioactive
CT head without and with contrast 5 Thin slices dedicated to the orbits are useful for orbit disease and may be substituted for the complete head examination in selected patients. radioactive radioactive radioactive
CTA head and neck with contrast 4 If vascular disease is suspected. radioactive radioactive radioactive
MRA head and neck without contrast 3   O
MRA head and neck without and with contrast. 3   O
X-ray orbit 2   radioactive
Rating Scale: 1,2,3 Usually not appropriate; 4,5,6 May be appropriate; 7,8,9 Usually appropriate *Relative Radiation Level

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

Summary of Literature Review

Introduction/Background

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, whereas exophthalmos is an abnormal prominence of the globe. Clinically, it may be impossible to differentiate these two entities without the aid of imaging. Exophthalmos may be caused by primary ocular or bulbar disorders such as macrophthalmia or colobomatous cysts, retrobulbar disorders such as intraorbital masses and inflammation, and extraorbital disorders such as masses or inflammation in the osseous orbital wall, face, paranasal sinuses, nasal cavities, or frontal cranial fossae.

Visual loss may be caused by damage at any location along the visual pathway extending from the globe to the occipital lobes. Therefore, the choice of appropriate imaging modality and focus depends on the specific clinical condition, and may include portions 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. Imaging of brain lesions that could result in visual loss such as stroke and cerebrovascular disease, demyelination, or tumors are covered in other ACR Appropriateness Criteria® topics.

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

Imaging Modalities

Imaging analysis of orbital diseases is facilitated by a compartmental approach that establishes differential diagnoses on the basis of the location of the process within the orbit. Computed tomography (CT) and magnetic resonance imaging (MRI) are complementary diagnostic procedures and may be used together in some circumstances. For example, CT is usually used for suspected thyroid ophthalmopathy whereas MRI is preferred for suspected masses. CT is useful in evaluating bony structures, and MRI excels in evaluating soft tissues. Dedicated thin-section multiplanar orbital imaging is recommended 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 its absence of radiation and the utility of fat-suppressed contrast-enhanced images, MRI has emerged as the procedure of choice for orbital disorders, with the exception of trauma and assessment for foreign bodies. Moreover, specialized surface coils have expanded the utility of MRI. In addition to diagnostic uses for imaging, both CT and MRI are becoming indispensable tools for surgical navigation of the tissues surrounding the orbit such as the paranasal sinuses. MRI is also an investigative research tool for exploring white matter tracts and functional aspects of the visual system. Ultrasound and fluorescein angiography are also important modalities; however, these special procedures are usually performed by the ophthalmologist and are not covered in this article.

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. CT and MRI studies will demonstrate the enlarged globe with thin walls and no other lesions. 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 from accidental perforation of the eyeball. Hemorrhagic choroidal detachments often occur after a contusion, a penetrating injury, or as a complication of intraocular surgery. MRI may differentiate choroidal effusion from choroidal hemorrhage. 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 in 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 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.

Disorders of the Optic Nerve Sheath Complex

Primary disorders of the optic nerve sheath complex typically cause visual disturbances and occasionally proptosis. The primary neoplasms of the optic nerve include optic nerve tumors (gliomas, astrocytomas, and hamartomas) and meningiomas. A para-optic component of optic nerve tumors, consisting of proteinaceous subarachnoid seeding, may be seen in patients with neurofibromatosis type I and contributes to optic nerve elongation and kinking. 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. MRI is best for this differentiation. CT scans best demonstrate calcifications in lesions such as meningiomas. 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.

The papilledema associated with pseudotumor cerebri or intracranial mass may enlarge the optic nerve as detected on CT or MRI. While dilatation of the perioptic subarachnoid space is best appreciated on fat-suppressed T2-weighted images, reversal of the nerve head, manifested by bulging of the posterior portion of the globe, may be more readily detected on CT than on MRI because of the chemical shift artifact inherent to the MR studies. MRI may also monitor optic nerve damage in other disorders such as glaucoma.

Optic neuritis is best seen on MRI as focal or diffuse enlargement of the optic nerve, abnormal 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 may be seen with multiple sclerosis (MS). MRI has become an essential study for evaluating patients with suspected MS and supplements other clinical studies. Even when MRI scans of the orbit are normal, imaging of the brain may reveal foci of demyelination.

Radiation-induced optic neuropathy (RON) is best evaluated with gadolinium-enhanced fat-suppressed MRI, which may show patchy, linear, or confluent enhancement along the portions of the optic nerve, chiasm, or optic tract.

Other Orbital Neoplasms

Primary neoplasms may arise from any constituent orbital tissue. The most common tumors are benign cavernous hemangiomas and have a predilection for the intraconal space. They present as focal round or oval masses with distinctive hyperintense appearance on MR images. Vascular calcifications can be detected on CT. Complex vascular lesions such as lymphangiomas and capillary hemangiomas; schwannomas arising from branches of cranial nerves III, IV, V, and VI; primary benign and malignant lacrimal gland tumors; metastases; and lymphomatous involvement of the soft tissues of the orbit (without osseous disease) may also present as isolated masses with or without involvement of adjacent orbital structures. MRI is the preferred imaging modality for evaluation.

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. Imaging (MRI or 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. 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 it is most commonly used in conjunction with therapeutic interventional procedures.

Traumatic optic neuropathy (TON) and post-traumatic visual loss may be evaluated by investigation of the soft tissue and osseous structures surrounding 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.

Inflammatory Orbital Syndrome (IOS)

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.

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 MRI scans. In the chronic form of the disease, there is increased fibrosis in the lesions, resulting in decreased signal on T2-weighted images. 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.

A small subset of patients with isolated ocular manifestations of IOS have posterior scleritis. Posterior scleritis shows inflammatory signs in the coat of the eye (sclera) with thickening of the posterior sclera that may be identified as areas of enhancement on CT or MRI. Sarcoidosis (neurosarcoidosis) and Wegener's granulomatosis both simulate IOS, lymphoproliferative disorders, or metastatic neoplasms.

Endocrine Disorders

Thyroid ophthalmopathy (Graves' disease) may be detected in hyperthyroid, hypothyroid, or euthyroid patients. In all age groups, approximately 15% of unilateral orbital proptoses and the majority of bilateral proptoses are secondary to thyroid ophthalmopathy.

CT is usually the first modality used for evaluation. 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 the involvement of just one or two isolated muscles. 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 most affected, with relative sparing of tendinous insertions.

The inherent soft-tissue contrast of MRI 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).

Summary

  • Imaging analysis of the orbit is facilitated by a compartmental approach.
  • CT and MRI are complementary diagnostic procedures for suspected orbital pathology.
  • CT is useful in evaluating bony structures.
  • MRI is useful in evaluating soft-tissue structures, the globe, optic nerves, and intraconal and extraconal spaces.
  • CTA, MRA, and conventional angiography may be useful in vascular conditions.
  • Conventional angiography may be useful in delivering therapeutic intervention.

Anticipated Exceptions

Nephrogenic systemic fibrosis (NSF) is a disorder with a scleroderma-like presentation and a spectrum of manifestations that can range from limited clinical sequelae to fatality. It appears to be related to both underlying severe renal dysfunction and the administration of gadolinium-based contrast agents. It has occurred primarily in patients on dialysis, rarely in patients with very limited glomerular filtration rate (GFR) (i.e., <30 mL/min/1.73 m2), and almost never in other patients. There is growing literature regarding NSF. Although some controversy and lack of clarity remain, there is a consensus that it is advisable to avoid all gadolinium-based contrast agents in dialysis-dependent patients unless the possible benefits clearly outweigh the risk, and to limit the type and amount in patients with estimated GFR rates <30 mL/min/1.73 m2. For more information, please see the American College of Radiology (ACR) Manual on Contrast Media (see the "Availability of Companion Documents" field).

Abbreviations

  • CT, computed tomography
  • CTA, computed tomography angiography
  • MRA, magnetic resonance angiography
  • MRI, magnetic resonance imaging

Relative Radiation Level Designations

Relative Radiation Level* Adult Effective Dose Estimate Range Pediatric Effective Dose Estimate Range
O 0 mSv 0 mSv
radioactive <0.1 mSv <0.03 mSv
radioactive radioactive 0.1-1 mSv 0.03-0.3 mSv
radioactive radioactive radioactive 1-10 mSv 0.3-3 mSv
radioactive radioactive radioactive radioactive 10-30 mSv 3-10 mSv
radioactive radioactive radioactive radioactive radioactive 30-100 mSv 10-30 mSv
*RRL assignments for some of the examinations cannot be made, because the actual patient doses in these procedures vary as a function of a number of factors (e.g., region of the body exposed to ionizing radiation, the imaging guidance that is used). The RRLs for these examinations are designated as “Varies.”

Clinical Algorithm(s)

Algorithms were not developed from criteria guidelines.

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 (adults and children) with disorders of the orbit and optic nerve

Interventions and Practices Considered

  1. Magnetic resonance imaging (MRI) head and orbit
    • Without and with contrast
    • Without contrast
  2. Computed tomography (CT) head
    • With contrast
    • Without contrast
    • Without and with contrast
  3. Magnetic resonance angiography (MRA) head and neck
    • Without contrast
    • Without and with contrast
  4. CT angiography (CTA) head and neck with contrast
  5. X-ray orbit

Major Outcomes Considered

Utility of radiologic examinations in differential diagnosis

Methods Used to Collect/Select the Evidence

Searches of Electronic Databases

Description of Methods Used to Collect/Select the Evidence

Literature Search Procedure

The Medline literature search is based on keywords provided by the topic author. The two general classes of keywords are those related to the condition (e.g., ankle pain, fever) and those that describe the diagnostic or therapeutic intervention of interest (e.g., mammography, MRI).

The search terms and parameters are manipulated to produce the most relevant, current evidence to address the American College of Radiology Appropriateness Criteria (ACR AC) topic being reviewed or developed. Combining the clinical conditions and diagnostic modalities or therapeutic procedures narrows the search to be relevant to the topic. Exploding the term "diagnostic imaging" captures relevant results for diagnostic topics.

The following criteria/limits are used in the searches.

  1. Articles that have abstracts available and are concerned with humans.
  2. Restrict the search to the year prior to the last topic update or in some cases the author of the topic may specify which year range to use in the search. For new topics, the year range is restricted to the last 5 years unless the topic author provides other instructions.
  3. May restrict the search to Adults only or Pediatrics only.
  4. Articles consisting of only summaries or case reports are often excluded from final results.

The search strategy may be revised to improve the output as needed.

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 Given)

Rating Scheme for the Strength of the Evidence

Strength of Evidence Key

Category 1 - The conclusions of the study are valid and strongly supported by study design, analysis, and results.

Category 2 - The conclusions of the study are likely valid, but study design does not permit certainty.

Category 3 - The conclusions of the study may be valid, but the evidence supporting the conclusions is inconclusive or equivocal.

Category 4 - The conclusions of the study may not be valid because the evidence may not be reliable given the study design or analysis.

Methods Used to Analyze the Evidence

Systematic Review with Evidence Tables

Description of the Methods Used to Analyze the Evidence

The topic author drafts or revises the narrative text summarizing the evidence found in the literature. American College of Radiology (ACR) staff draft an evidence table based on the analysis of the selected literature. These tables rate the strength of the evidence for all articles included in the narrative text.

The expert panel reviews the narrative text, evidence table, and the supporting literature for each of the topic-variant combinations and assigns an appropriateness rating for each procedure listed in the table. Each individual panel member forms his/her own opinion based on his/her interpretation of the available evidence.

More information about the evidence table development process can be found in the ACR Appropriateness Criteria® Evidence Table Development document (see the "Availability of Companion Documents" field).

Methods Used to Formulate the Recommendations

Expert Consensus (Delphi)

Description of Methods Used to Formulate the Recommendations

Modified Delphi Technique

The appropriateness ratings for each of the procedures included in the Appropriateness Criteria topics are determined using a modified Delphi methodology. A series of surveys are conducted to elicit each panelist's expert interpretation of the evidence, based on the available data, regarding the appropriateness of an imaging or therapeutic procedure for a specific clinical scenario. American College of Radiology (ACR) staff distributes surveys to the panelists along with the evidence table and narrative. Each panelist interprets the available evidence and rates each procedure. The surveys are completed by panelists without consulting other panelists. The ratings are a scale between 1 and 9, which is further divided into three categories: 1, 2, or 3 is defined as "usually not appropriate"; 4, 5, or 6 is defined as "may be appropriate"; and 7, 8, or 9 is defined as "usually appropriate." Each panel member assigns one rating for each procedure per survey round. The surveys are collected and the results are tabulated, de-identified and redistributed after each round. A maximum of three rounds are conducted. The modified Delphi technique enables each panelist to express individual interpretations of the evidence and his or her expert opinion without excessive bias from fellow panelists in a simple, standardized and economical process.

Consensus among the panel members must be achieved to determine the final rating for each procedure. Consensus is defined as eighty percent (80%) agreement within a rating category. The final rating is determined by the median of all the ratings once consensus has been reached. Up to three rating rounds are conducted to achieve consensus.

If consensus is not reached, the panel is convened by conference call. The strengths and weaknesses of each imaging procedure that has not reached consensus are discussed and a final rating is proposed. If the panelists on the call agree, the rating is accepted as the panel's consensus. The document is circulated to all the panelists to make the final determination. If consensus cannot be reached on the call or when the document is circulated, "No consensus" appears in the rating column and the 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.

Type of Evidence Supporting the Recommendations

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

Potential Benefits

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

Potential Harms

Gadolinium-based Contrast Agents

Nephrogenic systemic fibrosis (NSF) is a disorder with a scleroderma-like presentation and a spectrum of manifestations that can range from limited clinical sequelae to fatality. It appears to be related to both underlying severe renal dysfunction and the administration of gadolinium-based contrast agents. It has occurred primarily in patients on dialysis, rarely in patients with very limited glomerular filtration rate (GFR) (i.e., <30 mL/min/1.73 m2), and almost never in other patients. Although some controversy and lack of clarity remain, there is a consensus that it is advisable to avoid all gadolinium-based contrast agents in dialysis-dependent patients unless the possible benefits clearly outweigh the risk, and to limit the type and amount in patients with estimated GFR rates <30 mL/min/1.73 m2. For more information, please see the American College of Radiology (ACR) Manual on Contrast Media (see the "Availability of Companion Documents" field).

Relative Radiation Level (RRL)

Potential adverse health effects associated with radiation exposure are an important factor to consider when selecting the appropriate imaging procedure. Because there is a wide range of radiation exposures associated with different diagnostic procedures, a relative radiation level indication has been included for each imaging examination. The RRLs are based on effective dose, which is a radiation dose quantity that is used to estimate population total radiation risk associated with an imaging procedure. Patients in the pediatric age group are at inherently higher risk from exposure, both because of organ sensitivity and longer life expectancy (relevant to the long latency that appears to accompany radiation exposure). For these reasons, the RRL dose estimate ranges for pediatric examinations are lower as compared to those specified for adults. Additional information regarding radiation dose assessment for imaging examinations can be found in the ACR Appropriateness Criteria® Radiation Dose Assessment Introduction document (see the "Availability of Companion Documents" field).

Qualifying Statements

The 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 examinations 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.

Description of Implementation Strategy

An implementation strategy was not provided.

IOM Care Need

Getting Better

Living with Illness

IOM Domain

Effectiveness

Bibliographic Source(s)

Wippold FJ II, Cornelius RS, Berger KL, Broderick DF, Davis PC, Douglas AC, Germano IM, Hadley JA, McDermott MW, Mechtler LL, Smirniotopoulos JG, Waxman AD, Expert Panel on Neurologic Imaging. ACR Appropriateness Criteria® orbits, vision and visual loss. [online publication]. Reston (VA): American College of Radiology (ACR); 2012. 12 p. [31 references]

Adaptation

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

Date Released

1999 (revised 2012)

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: Franz J. Wippold II, MD (Principal Author and Panel Chair); Rebecca S. Cornelius, MD (Panel Vice-chair); Kevin L. Berger, MD; Daniel F. Broderick, MD; Patricia C. Davis, MD; Annette C. Douglas, MD; Isabelle M. Germano, MD; James A. Hadley, MD; Michael W. McDermott, MD; Laszlo L. Mechtler, MD; James G. Smirniotopoulos, MD; Alan D. Waxman, MD

Financial Disclosures/Conflicts of Interest

Not stated

Guideline Status

This is the current release of the guideline.

This guideline updates a previous version: Wippold FJ II, Cornelius RS, Broderick DF, Brown DC, Brunberg JA, Davis PC, De La Paz RL, Garvin CF, Germano I, McConnell CT Jr, McDermott MW, Mukherji SK, Seidenwurm DJ, Sloan MA, Smirniotopoulos JG, Expert Panel on Neurologic Imaging. ACR Appropriateness Criteria® orbits, vision and visual loss. [online publication]. Reston (VA): American College of Radiology (ACR); 2009. 9 p.

Guideline Availability

Electronic copies: Available from the American College of Radiology (ACR) Web site External Web Site Policy.

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 are available:

  • ACR Appropriateness Criteria®. Overview. 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 External Web Site Policy.
  • ACR Appropriateness Criteria®. Literature search process. Reston (VA): American College of Radiology; 1 p. Electronic copies: Available in PDF from the ACR Web site External Web Site Policy.
  • ACR Appropriateness Criteria®. Evidence table development – diagnostic studies. Reston (VA): American College of Radiology; 2013 Nov. 3 p. Electronic copies: Available in PDF from the ACR Web site External Web Site Policy.
  • ACR Appropriateness Criteria®. Radiation dose assessment introduction. Reston (VA): American College of Radiology; 2 p. Electronic copies: Available in PDF from the ACR Web site External Web Site Policy.
  • ACR Appropriateness Criteria®. Manual on contrast media. Reston (VA): American College of Radiology; 90 p. Electronic copies: Available in PDF from the ACR Web site External Web Site Policy.
  • ACR Appropriateness Criteria®. Procedure information. Reston (VA): American College of Radiology; 1 p. Electronic copies: Available in PDF from the ACR Web site External Web Site Policy.
  • ACR Appropriateness Criteria® orbits, vision and visual loss. Evidence table. Reston (VA): American College of Radiology; 2012. 12 p. Electronic copies: Available from the ACR Web site External Web Site Policy.

Patient Resources

None available

NGC Status

This NGC 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. This summary was updated by ECRI Institute on May 26, 2010. This summary was updated by ECRI Institute on January 13, 2011 following the U.S. Food and Drug Administration (FDA) advisory on gadolinium-based contrast agents. This NGC summary was updated by ECRI Institute on October 2, 2012.

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 External Web Site Policy.

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