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14.1
14.2
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Orbital problems are sometimes seen in ophthalmology practice, but most are uncommon. My goal for this chapter is to provide a good foundation in approaching the patient with a proptotic eye. It isn’t possible to diagnose every orbital problem, but you should be able to proceed in a logical fashion and to diagnose most causes of proptosis. Orbital disease is the most complex topic in this book, and it is easy to get overwhelmed when learning about it. Get an overview initially, and then focus on the details during the second or third time through the chapter. Entire books are written on orbital disease, so the topic is almost too much to cover in one chapter. Use this text as an introduction to a more detailed study of the orbit over time.
Think of all the different tissue types found in the orbit—nerves, muscles, veins, and arteries plus glandular and connective tissue, to name a few. In addition to these tissues, pigment cells and red and white blood cells are present. A primary orbital neoplasm can arise from any of these tissue types or cells. Now consider the tissues surrounding the orbit—bones of the face and skull, the brain, the sinus and nasal tissues, and the soft tissues of the face. Each of these structures may develop a problem that extends into the orbit as a secondary orbital condition. Given the almost infinite number of processes that can involve the orbit, it is important to develop an approach to investigating the patient with a proptotic eye.
Proptosis is the hallmark of orbital disease. When you see a proptotic, or displaced, eye, you should be able to develop a basic differential diagnosis from the clues in the history and physical examination. For most patients with proptosis, the diagnostic process requires imaging studies, most commonly a computed tomography (CT) scan. Because of the huge variety of possible problems, a biopsy of the pathologic lesion is often necessary for diagnosis or treatment.
From this chapter, you should:
Learn to recognize the patient with proptosis
Learn the approach to taking a basic history and performing a physical examination
Understand when imaging is necessary and what imaging studies are best
Learn the approach to developing a differential diagnosis based on the history, physical examination, and imaging studies
Become familiar with common orbital problems in adults and children
In all adults with proptosis, you should consider the diagnosis of thyroid orbitopathy first. The current nomenclature for this chronic inflammatory condition is thyroid eye disease . Thyroid eye disease is the most common cause of unilateral or bilateral proptosis in adults. Other common causes in adults include lymphoid lesions, idiopathic orbital inflammatory disease, cavernous malformations, and metastatic disease. Some orbital neoplasms that are less common, but nevertheless deserve mention, are tumors of the optic nerve and lacrimal gland. You may also see tumors arising outside the orbit that involve the orbit secondarily. Even this small list of orbital problems can be overwhelming. Learn some of the typical features of these processes seen in the history and physical examination and their imaging characteristics. In the beginning, it is difficult to get the exact diagnosis for each patient. But soon you develop a sense of the type of problem you are dealing with on a pathogenic basis. Is the problem inflammatory or neoplastic? Is it benign or malignant? Is it congenital or acquired? In a short time, you start to recognize the specific diseases that fit.
For the most part, the common causes of proptosis in children are different from those in adults. We briefly discuss dermoid cyst, capillary hemangioma, orbital cellulitis, rhabdomyosarcoma, lymphangioma, and optic nerve glioma.
The treatment of the proptosis depends on the cause. For some causes, such as orbital cellulitis, medical treatment should be initiated without biopsy. For other patients, an incisional biopsy is required to obtain the diagnosis, for example, the biopsy of a suspected lymphoid lesion to determine if the mass is benign or malignant. Often an incisional biopsy is followed by further medical (chemotherapy or radiation therapy) or surgical (further tumor excision) therapy. In some patients, excisional biopsy or complete removal of the lesion, such as a dermoid cyst, confirms the diagnosis and completes the treatment at the same time.
Review the basic approach to the patient with proptosis at this point. The presence of proptosis, or displacement, of the eye suggests an orbital problem. The history and physical examination give you a differential diagnosis, at least on a pathogenic basis. Imaging studies such as a CT scan are usually required to refine the differential diagnosis. If the diagnosis is not clear at this point, incisional or excisional biopsy is required to obtain a diagnosis. The plan for treatment is based on the diagnosis.
An exophthalmometer is used to measure the prominence of the eye; most commonly, the Hertel exophthalmometer is used ( Figure 14.1 ). It measures the anterior projection of the eye, from the lateral orbital rim to the cornea ( Table 14.1 ). When you use the Hertel exophthalmometer, be sure to lightly push the instrument against the lateral orbital rim and make its width or base setting as narrow as is comfortable for the patient. Try to use the same base setting for an individual patient each time you measure the prominence of the eye. Measurements with the Hertel exophthalmometer are not exact, but you should be able to obtain repeatable measurements within 1 to 2 mm. As you are learning to use the Hertel exophthalmometer, compare your readings with those of a more experienced examiner so that you can make sure you are measuring correctly. Because the instrument uses the lateral orbital rim as a reference point, any surgery, disease, or trauma that changes the position of the lateral rim affects the measurements. In cases where the lateral rim is not in normal position, you can use an exophthalmometer designed by Thomas Naugle ( good-lite.com ). This device uses the forehead and cheek as reference points.
Race | Hertel measurement (mm) |
---|---|
Asian | 18 |
White | 20 |
Black | 22 |
The normal prominence of the eye in the orbit depends upon the surrounding facial bones, the structure of which varies from individual to individual and among races. The equator of the globe is at the lateral orbital rim in patients with average bone structure. You can appreciate the position of the eye relative to the rim during the physical examination by placing your index finger at the lateral orbital rim and pushing against the eye. The average Hertel measurements range from 18 to 22 mm.
Because the normal position of the eye in the orbit varies from patient to patient, we are more interested in asymmetry in the prominence of the one eye compared with the other or a change in the globe position on one side that has occurred over time. Proptosis implies an anterior displacement of the globe. An orbital mass not centered within the orbit displaces the eye off the axis, as well. Globe ptosis is the term used when the eye is pushed down by a mass (also known as hypoglobus ). As you perform the orbital examination, look for signs of axial and nonaxial globe displacement of the eye to help you locate the mass that is displacing the globe.
The orbit is conceptually and anatomically divided into surgical spaces ( Box 14.1 ).
Intraconal space
Extraconal space
Extraocular muscles
Subperiosteal space
Tenon’s space
Extraorbital space
The intraconal space, sometimes called the central surgical space, contains the optic nerve and orbital fat ( Figure 14.2 ). Many tumors arise within the intraconal space or push their way into this space. The most widely discussed tumors of the orbit, optic nerve glioma and optic nerve meningioma, occur in the intraconal space.
The extraconal space, sometimes called the peripheral surgical space, contains the lacrimal gland, superior oblique muscle and trochlea, and nerves and vessels in the extraconal orbital fat. The lacrimal gland is a common source of orbital pathologic processes. An enlarged lacrimal gland is often palpable in the upper lid and is readily accessible using an anterior orbitotomy through the upper lid skin crease.
A fibrous membrane, the intermuscular septum, extends between the anterior portion of the extraocular muscles, separating the intraconal and extraconal spaces. The muscles can become involved in neoplastic or inflammatory processes. The most common condition is thyroid orbitopathy (thyroid eye disease). Painful inflammation of the muscles, or myositis, can occur. Primary neoplasms of the muscles are very rare, but metastatic lesions occur more commonly.
The subperiosteal space is a potential space between the orbital bones and the periorbita. A hematoma can collect in this space from an adjacent fracture. A collection of pus, a subperiosteal abscess, often collects medially from an adjacent ethmoid sinus infection.
Tenon’s space lies between the eye and the fibrous capsule. Tenon’s capsule, which surrounds all but the anterior portion of the eye, is the bloodless space in which enucleation and scleral buckle procedures are performed. This space is rarely involved in orbital pathologic processes, the most common lesion being extraocular extension of a choroidal melanoma.
The extraorbital space, or periocular tissue, includes all the structures surrounding the orbit: bone, brain, sinuses, nose, skin, and conjunctiva. A variety of problems originate in these tissues and involve the orbit secondarily. We discuss some of these conditions at the end of this chapter ( Table 14.2 ).
Displacement | Etiology |
---|---|
|
|
Enlarged extraocular muscles | Thyroid orbitopathy |
Intraconal mass | Cavernous malformation |
Optic nerve tumor | Optic nerve meningioma, glioma |
|
|
|
|
|
|
|
|
|
Abscess or mucocele |
|
|
|
|
|
Benign mixed tumor of lacrimal gland |
|
Scirrhous carcinoma of the breast, silent sinus syndrome, blowout fracture |
In the 1980s, Krohel, Stewart, and Chavis described the familiar mnemonic involving the P ’s of the orbital history and physical examination. Although this system is somewhat contrived, it is a useful way to learn the orbital history and conduct the physical examination, providing a checklist of what to consider. The six P ’s they described are the following:
Pain
Progression
Proptosis
Palpation
Pulsation
Periorbital changes
In a few paragraphs, we add a seventh P to the list, the past medical history.
Pain and progression are the characteristics of orbital problems that are most helpful in developing the differential diagnosis. Pain is caused by inflammation, infection, acute pressure changes, or bone or nerve involvement. Once you become familiar with the common orbital disorders, you find that the presence or absence of pain is very helpful in developing a differential diagnosis.
Progression is the other P that refines the differential diagnosis easily. As you can imagine, some processes progress quickly, whereas others take months or years to develop. Progression can be classified as follows:
Rapid progression: occurring over hours to days
Intermediate progression: occurring over weeks to months
Slow progression: occurring over months to years
It is impossible to diagnose every cause of proptosis based on pain and progression only, but you may be surprised how easy it is to develop a differential diagnosis of a general pathogenesis. Consider the types of pathologic processes that affect the body as a whole. You probably learned these in your medical school pathology course:
Inflammatory
Infectious
Hemorrhagic
Neoplastic
Metastatic
Congenital
Now consider how the symptom of pain fits into the categories. Pain suggests inflammation, infection, hemorrhage, or perhaps a tumor growing into nerves or bone. Neoplasms, in general, do not cause pain until a complication related to the neoplasm arises. You remember from your general surgery days in medical school that the large bowel tumor sits quietly in the abdomen until there is an obstruction that causes secondary inflammation, infection, or hemorrhage. This is true of orbital tumors as well. The majority of orbital neoplasms do not cause pain until late in their course.
A sudden onset with rapid progression over minutes suggests a hemorrhage ( Figure 14.3 ). Acute processes occurring over hours to days suggest inflammation or infection. Slower processes occurring over weeks to months suggest more chronic types of inflammatory processes such as thyroid disease. Chronic conditions with a vague onset and slow progression over months suggest a benign neoplasm or lymphoma. Although this is a generalized discussion of progression, these principles are very helpful in developing a differential diagnosis in cases of proptosis.
Onset and progression of symptoms and signs are related features. Onset identifies a point in time when the problem started and how it manifested itself initially. Progression describes any change in the symptoms (and the rate of change) occurring over the period of time since the onset. For example, an orbital infection may have an onset 3 days after the start of a respiratory infection. The pain and inflammation are minimal initially but progress rapidly after onset. A contrasting example is the proptosis and globe ptosis resulting from a benign mixed tumor of the lacrimal gland. The progression is so slow, over months or years, that it is difficult for the patient to identify the exact onset of any symptoms. In many chronic conditions, the patient’s perception of the onset and the progression of the disorder may not be accurate. In these cases, the patient’s perception of onset is often when the proptosis was noted, which may not be when the process actually started. In these cases, the use of the so-called family album tomogram scan is useful. The review of these old photos can help to identify the true progression of a disorder.
Lastly, we should add the past medical history to the list of the original six P ’s. Any previous diagnosis of neoplasm elsewhere in the body must be noted. Past trauma of the face may have caused some facial asymmetry that may accentuate or diminish the appearance of a proptotic eye. Any history of thyroid disease that has already been diagnosed should be noted. This is perhaps the most important information to solicit. Don’t forget to include basic information in the history such as age and sex. Most orbital processes tend to occur at certain ages. The differential diagnoses of childhood and adult orbital disorders don’t have many diseases in common. The most common disorder that has a striking sex difference is thyroid disease, which occurs about six times more often in women than in men.
A typical history might be written like this: “A 65-year-old noted proptosis of the left eye and a mass in the lid 4 months ago. Since that time the proptosis and swelling have progressed slowly. There is no pain. There is no past medical history of trauma or thyroid disease. He was treated for lymphoma in the past. He is currently taking no medications.” Are you getting the idea? Already you are thinking about the possibility of orbital lymphoma and can look for some fullness of the superior fornix, a little globe ptosis, and a palpably enlarged lacrimal gland during the physical examination.
We have just reviewed the P ’s of the orbital patient history: pain, progression, and past medical history. You have in mind a differential diagnosis based on this history. The orbital examination can refine this differential diagnosis, and from that point, you can proceed with imaging, any laboratory examinations, a possible orbitotomy, and medical treatment.
The P ’s of the orbital examination are:
Proptosis
Palpation
Pulsation
Periocular changes
The most important part of the orbital examination is the evaluation of the proptotic eye. An orbital mass or volume-producing process pushes the eye away. The larger the mass is, the more the displacement of the globe.
In most cases, when we talk about proptosis, we are really talking about an axial displacement of the eye in an anterior direction. When you see axial proptosis, think of thyroid eye disease with enlargement of the extraocular muscles. Other intraconal disorders such as optic nerve tumors or a benign cavernous malformation may occur within that muscle cone as well and cause axial anterior displacement of the eye. In some conditions, you see nonaxial displacement of the eye. If you see the eye pushed downward, think of problems arising in the area of the lacrimal gland or, less commonly, defects in the orbital roof due to trauma, encephalocele, or frontal sinus mucocele formation. When you see the eye displaced laterally, there is usually a problem in the ethmoid sinus. The most common situation that displaces the eye laterally is a subperiosteal abscess (an acute process) arising in the ethmoid sinus and extending into the subperiosteal space. Rarely, a sinus carcinoma (a slowly progressive process) or mucocele (a very slowly progressive process) of the ethmoid sinus can cause this type of lateral displacement. You rarely see the eye being displaced upward. A number of rare conditions can cause this ( Figure 14.4 ). Although lymphoid lesions occur most commonly in the superior orbit, lymphoid lesions are so common that they are the most common cause of an inferior orbital mass. Rarely, tumors arising from the maxillary sinus can erode through the orbital floor and push the eye upward. Likewise, it is rare to see the globe pushed medially. If medial globe displacement is present, the eye usually is also being pushed downward by an enlarged lacrimal gland. You can estimate the nonaxial displacement of the eye with a ruler or use an instrument designed for this purpose known as the McCoy Tri-Square (Tri-Square Facial McCoy Padgett 162-PM-3795 stevens.ca ) ( Figure 14.5 ).
There is an exception to the rule that an orbital mass pushes the eye away from the mass. Scirrhous carcinoma of the breast is an infiltrative sclerosing tumor, which may actually cause an enophthalmos of the eye. You have already asked about the past medical history of other carcinomas, so if you heard that the patient has a history of breast carcinoma and you note that the eye is sunken, think of metastatic breast cancer ( Figure 14.6 ).
We have already talked about the use of the Hertel exophthalmometer to measure the prominence of the eye. Remember that there are normal variations among individuals and races. When you use the Hertel exophthalmometer, asymmetry between the left and right sides is more important than the actual measurement. Any asymmetry measuring more than 2 mm is significant. Don’t forget that trauma or congenital variation may be a cause of the asymmetry. Similarly, a change in the displacement of the eye based on the patient’s history or old photographs is an important finding.
The next step in the orbital examination is palpation. Start with palpation of the orbital rims and then move toward the eye, palpating the superior and inferior fornices for any anterior masses. If a mass is palpable, you want to note its shape, size, and position. Often, you can tell if the mass has a smooth border separate from adjacent tissues or is infiltrating into adjacent tissues. In some patients, the mass is fixed to bone or a nearby structure such as an extraocular muscle, suggesting an infiltrative tumor. You should try to determine if there is any tenderness in the area of the lesion as well. Infectious or inflammatory disorders often cause the skin to be erythematous and warm to touch.
Pulsations of the orbit are rare but, when present, are diagnostic. A classic finding is pulsatile proptosis. This pulsation of the eye suggests either an arterial vascular malformation in the orbit or the absence of orbital bone that allows the normal pulsations of the brain to push on the eye. The most common cause of pulsatile proptosis is the absence of the sphenoid wing seen in neurofibromatosis. If you think you are seeing a case of pulsating exophthalmos, it can be useful to confirm the globe pulsations using the Hertel exophthalmometer to view the eye from the side. If you feel the radial pulse at the same time it means that the pulsations are synchronized.
Orbital arterial vascular lesions can pulsate. If the flow is high, you may be able to hear a bruit or feel a thrill. These lesions are rare, also. Vascular abnormalities that are primarily venous are more common than arterial lesions. Venous lesions do not pulsate, but they usually show enlargement with the Valsalva maneuver or with the head in a dependent position ( Figure 14.7 ). You are likely familiar with the term thrill associated with fast-moving blood. This is described for some orbital lesions, but I have yet to ever feel an orbital thrill.
The last point to note in the orbital examination is periocular changes. These include a variety of abnormalities in the skin, conjunctiva, eye, or surrounding periocular tissues. Some periocular changes that are most useful for diagnosis are the temporal flare of the lateral portion of the upper lid and lid lag seen on downgaze in patients with thyroid eye disease ( Figure 14.8 ). Other examples of periocular changes include a conjunctival “salmon patch” suggesting orbital lymphoma (see Figure 14.19 ), fullness of the temple suggesting a sphenoid wing meningioma (see Figure 14.25 ), and periocular skin malignancy suggesting intraorbital spread of cutaneous carcinoma.
At this point, stop and think about the orbital examination and the way in which you want to proceed:
You start by evaluating the change in the position of the eye in terms of axial and nonaxial displacement, suggesting where a mass might be present and pushing the eye away.
Next you palpate the orbital rims and soft tissues to see if any abnormality is present.
Then you check briefly for any pulsations.
Last, you search for other clues in the periocular area that may provide information to develop a differential diagnosis.
The plan for evaluation of the patient with proptosis is to take the history, perform the physical examination, and follow up by imaging the orbit. Usually, at this point, you can make a differential diagnosis. You can generally narrow down the choices to one of the general pathologic categories such as neoplasm or infection. Remember that thyroid orbitopathy is the most common cause of unilateral or bilateral proptosis. If a mass is diagnosed, incisional or excisional biopsy is usually needed to confirm the diagnosis. Treatment is based upon the pathology results.
Recall the three P ’s of the history:
Pain
Progression
Past medical history
Recall the four P ’s of the physical examination:
Proptosis
Palpation
Pulsation
Periocular change
Almost all patients with proptosis require orbital imaging. One exception to this may be the patient with findings typical of stable Graves’ disease in whom the diagnosis is so apparent that no imaging is needed to confirm your clinical suspicion. CT scanning is used as the primary imaging technique for evaluation of any patient with proptosis. You should order a magnetic resonance imaging (MRI) scan of the orbit in special cases, primarily those situations in which imaging of the orbital apex and chiasm is required.
You are undoubtedly familiar with the CT scan technique. CT scanning uses ionizing radiation passed through the tissue to form a computer-generated image. Like other radiographs, excellent views of the bony structure are obtained, making the CT scan the method of choice for viewing bony orbital trauma. Remember that fat is radiolucent (black) on a CT scan. The intraconal fat gives a good natural contrast with adjacent soft tissue structures (shades of gray) without any injection of intravenous contrast agents. For these reasons, the CT scan gives excellent views of the orbital bones and the majority of orbital structures. Bone landmarks are helpful in planning orbitotomy for orbital tumors as well.
CT scanning is essential for the evaluation of orbital trauma, and it is readily available. In modern CT scanners, the orbital scanning times have been reduced to less than a minute per patient. You should order and review axial and coronal projections for all patients. Sagittal views are occasionally helpful. All projections are available without repositioning the patient. Your hospital or imaging center should be providing high-resolution orbital scans with cuts of no more than 1 to 2 mm. The cavernous sinuses and paranasal sinuses should be included with orbital scans. You should order intravenous contrast agent for the evaluation of most tumors. Contrast allergies are not uncommon, so make sure you ask about iodine or fish allergies. CT scanning remains significantly less expensive than MRI.
The generation of MR images is based on entirely different principles than those used in CT scanning. No ionizing radiation is used. An image is generated based upon the vibration of protons in tissue when a patient is placed in the magnetic field and then subjected to a series of radio wave pulses. The radiologist can vary the radio wave pulses so that different tissues generate signals (this is how the standard T1- and T2-weighted scans, and the many other specialized sequences, are generated). Some general imaging characteristics are helpful in interpreting MRI scans:
You can recognize a T1-weighted scan because the vitreous is dark.
You can recognize a T2-weighted scan because the vitreous is white (bright).
Because the density of the signal depends on the density of protons in the tissue, edema (water, i.e., protons) causes a bright signal on the T2-weighted image.
High vascular flow, such as that in the carotid artery, generates no signal (dark), or a flow void, because the protons are moving too quickly to be imaged.
The protons in bone are too tightly bound to generate a signal, so cortical bone is dark on MRI scans. Marrow spaces generate a signal.
The spatial resolution for MRI is less than for CT scanning. The tissue contrast, however, is better with MRI. However, because the fat provides a contrast to most other structures, CT can be used as the main screening technique for orbital disease. MRI plays an important role in the evaluation of specific orbital diseases and is sometimes used in addition to CT scanning. Obviously, this is an oversimplification of MRI, but these principles can guide you in evaluating the scan.
The main indication for MRI is to view the orbitocranial junction. If you suspect an optical nerve tumor, you should request an MRI scan. Because bone is not visualized, the bony artifact from the dense bones of the orbital apex seen on CT scans is not present. The soft tissues of the apex are visualized in detail. Some intraorbital organic foreign bodies are seen better with MRI scans than with CT scans. Vascular tumors or other very heterogeneous tumors are often seen more clearly on an MRI scan than on a CT scan, as well. Lastly, any secondary orbital disease originating from the brain or paranasal sinus can often be visualized best by both CT and MRI together. This allows the best view of bone and soft tissue. In the case of sinus neoplasms extending into the orbit, T2-weighted MRI sequences help to distinguish a sinus opacity caused by mucus retention (bright signal) from that caused by tumor (dark signal).
Most radiology departments have routine imaging sequences that are used under an orbital protocol. In addition, the intravenous contrast material gadolinium can be injected to enhance some pathologic processes. The imaging sequence known as fat suppression is used with gadolinium. With this technique, the normally bright orbital fat appears dark. Without fat suppression, you do not see any enhanced orbital structures against the normally bright fat background.
There are many specific sequences that help with imaging certain disease processes, for example, the FLAIR sequence is especially good for identifying optic neuritis due to demyelinating disease. Developing a working relationship with a radiologist interested in orbital disease can help you with these nuances. Similarly, working with an interventional radiologist can help you understand and deal with vascular flow issues in some of your orbital patients.
In practice, you look at T1 and T2 scans with contrast injection. Use these tips to evaluate an MRI scan:
Look at the T1-weighted scan for the best anatomic detail.
T2-weighted scans show water as bright, emphasizing edema or other fluid within a mass.
As you become familiar with specific disease processes, you can learn the individual characteristics of the T1 and T2 sequences for each process or tumor (don’t worry about that now).
Look at the fat suppression sequence (the fat is dark) on gadolinium-enhanced scans. Enhancement implies a richly vascularized tumor or inflammation (such as sarcoid, often not visible without enhancement).
MRI has several practical disadvantages compared with CT scanning. MRI is still more expensive than CT. Imaging takes significantly longer. Bone is poorly viewed. As we said above, the spatial resolution of MRI is less than that of CT, so the detail is not as clear. MRI is not safe for patients who have metallic foreign bodies or aneurysm clips in place. It is difficult, or impossible, to obtain an MRI scan for any patient who requires a ventilator, pacemaker, or cardiac monitor. MRI is often used as the primary imaging technique for children, especially if repeated scanning over the years is likely. Because there is no associated ionizing radiation, there is no risk of radiation-induced tumors developing at a later time.
Special imaging studies are available or can be arranged in consultation with your radiology colleagues.
CT
Three-dimensional CT
Imaging for stereotactic navigation
Angiography (CTA)
Valsalva maneuver
MR
MR angiography (MRA)
Angiography
Echography
CT studies for stereotactic navigation are commonly obtained by our ear, nose, and throat (ENT) and neurosurgical colleagues, especially when performing endoscopic operations. You are probably familiar with these studies, but if not, you should see the technique in action. By linking the preoperative high-resolution images with cameras in the operating room that sense the position of your instruments, you can have real-time localization of your position in the patient. This technique is especially useful where the normal anatomy is quite variable (paranasal sinuses) and for reoperations where normal landmarks have been altered. It can be helpful for you when you are operating in less familiar areas. For example, I used this when I was less experienced in skull base procedures. I don't use navigation routinely, but some surgeons find it helpful for orbital procedures such as orbital decompression. Your hospital operating room probably has a navigation system. The company representative is happy to come to the operating room with you on your first few cases to help set up the equipment. It is worth becoming familiar with the tool, if nothing else as a learning tool.
CT scanning before and during the Valsalva maneuver is helpful for detecting the venous flood seen in orbital varices. Three-dimensional CT scans produce amazing pictures ( Figure 14.9 ). These images are most useful for craniofacial anomalies and extensive facial trauma. Three-dimensional scans are valuable for planning a reconstructive operation and are also useful for teaching residents and patients. Computed tomography angiography (CTA) and magnetic resonance angiography (MRA) allow for easy viewing of the blood supply of a tumor. For a number of reasons, MRA is usually the first choice for our purposes. Arteriography remains the gold standard for vascular imaging. At the same time, therapeutic selective occlusion of feeding vessels can cure or decrease vascular flow, decreasing or eliminating the problem or, in some cases, making operation safer. Similarly, direct venous puncture and occlusion can be helpful in selected cases of varix or other mixed venous malformations. If your practice includes these patients, you need a strong working relationship with a neurointerventional radiologist. Echography has been used in the imaging of ocular and orbital diseases for many years. In the hands of experienced practitioners, useful information can be obtained. In most centers, CT and MRI have replaced echography in the study of orbital disease.
The information that is available with current imaging techniques and the expertise of our radiology colleagues is incredible. This information can be extremely valuable for diagnosis and surgical planning. A good example is Figure 14.9 , which focused our attention on the blood supply of a large congenital mass in a newborn. Vascular studies and embolization made the tumor removal safe for this premature baby weighing only 3 pounds.
As we have already discussed, many patients with proptosis undergo imaging studies. Orbital imaging serves two purposes:
Diagnostic information (I want to know what the mass is)
Information used to plan orbitotomy (I want to know the best surgical approach to taking a biopsy of the mass)
Most times, a quick look at the scan provides an idea of what is causing the proptosis. You are likely to see one of two situations:
Enlarged orbital structure
Extraocular muscle(s)
Optic nerve
Lacrimal gland
Eyeball (pseudoproptosis)
Orbital mass not arising from a specific structure
Well-circumscribed mass
Infiltrative mass
In a few situations, you may be able to make the diagnosis based on the scan alone. For example, bilateral enlargement of the extraocular muscles indicates thyroid orbitopathy until proven otherwise. More likely, the imaging suggests a few possible diagnoses. For example, an enlarged optic nerve usually indicates meningioma or glioma.
If a mass appears separate from the surrounding structures, the characteristics of the mass may help you put the lesion in a particular pathogenic category, such as neoplasm or inflammation. Based on the location of the mass in the orbit, you can determine the best surgical approach for biopsy.
Remember, the questions we are trying to answer are What is it? and What is the best surgical approach for biopsy? Some specific characteristics of an orbital mass can help you with the diagnosis and surgical approach:
Location
What is the tissue of origin?
Position of the mass: Which surgical space is involved?
Imaging clues to the biologic behavior of a mass
Relationship to adjacent soft tissues
A “pusher” (benign)
An “eater” (malignant)
Relationship to adjacent bone
Fossa formation (benign)
Bone erosion (malignant)
Shape of the mass
Size of the mass
Internal characteristics of the mass
Homogeneous or heterogeneous
Contrast enhancement
You must be able to describe the location of a lesion. Can you determine the tissue of origin? Does the mass represent an enlargement of a normal orbital structure? The lacrimal gland, optic nerve, and extraocular muscles can be enlarged, each with a separate differential diagnosis. If the mass represents infiltration of a normal structure, you are likely to be doing an incisional biopsy rather than removing the structure initially.
If you cannot tell whether the mass is derived from a normal structure, you should localize the mass within a specific orbital space and describe its position relative to normal structures within the space. We discussed the surgical spaces of the orbit (see Figure 14.2 ) earlier in this chapter under Normal Anatomy and Examination of the Orbit. The surgical spaces of the orbit are:
Intraconal space
Extraocular muscles
Extraconal space
Subperiosteal space
Tenon’s space
Extraorbital space
Knowledge of the surgical space containing the orbital mass is useful for developing a differential diagnosis and choosing the surgical approach for biopsy. The spatial relationship to the optic nerve and the anterior–posterior position within the intraconal space are especially important in choosing the orbitotomy approach. When you operate in the intraconal space, choose a surgical approach so that you do not cross the optic nerve . Approach medial intraconal masses in the anterior portion of the orbit from a medial anterior orbitotomy. Approach lateral intraconal masses in the anterior orbit from a lateral orbitotomy. Masses arising deep in the intraconal space must be approached transcranially. In medial apical lesions, you may be able to reach the area endoscopically through the ethmoid sinus. You can see that the location of a mass in the orbit is critical for the diagnosis and biopsy of the mass. We discuss the surgical approaches to the orbit in the next chapter.
This is important. Does the mass push the adjacent structures aside or does it infiltrate the adjacent tissues? Infiltrative lesions are usually malignant. Well-circumscribed lesions with smooth borders are usually benign ( Figure 14.10 ). Think of these lesions as pushers or eaters. Pushers are more likely benign. Eaters are more likely malignant. Is the mass pushing against the optic nerve, or is it growing into the optic nerve ( Figure 14.11 ) ? The latter lesion is more likely malignant. The relationship to adjacent structures, or borders, gives you a snapshot-in-time estimate of the biologic behavior of the mass. This concept is simple but extremely important.
The relationship of a soft tissue mass to the adjacent bone gives similar information about the biologic behavior of the mass. Slow-growing benign masses push the bone or cause fossa formation. Aggressive malignant tumors eat the bone or cause bone erosion. When you evaluate a lacrimal gland mass, the presence of fossa formation is typical of a benign slow-growing mixed tumor of the lacrimal gland ( Figure 14.12 ).
You learn to use the clinical information you obtain during the history and physical examination to complement the imaging studies. A poorly defined mass with indistinct borders presenting without pain is likely to be a malignant neoplasm. A poorly defined mass with indistinct borders presenting with pain is likely to be idiopathic orbital inflammatory disease, rather than any true neoplasm. Hypesthesia of the temple described by the patient with bone erosion adjacent to the lacrimal gland mass (see Figure 14.12 ) is indicative of the neurotrophic spread typical of the aggressive adenoid cystic carcinoma of the lacrimal gland.
The distinctiveness of the borders helps greatly in determining if the mass can be removed. Infiltrative lesions usually cannot be safely removed (require incisional biopsy). Well-circumscribed masses can often be removed (excisional biopsy).
The shape of the lesion is less helpful than the relationship to other tissues in determining its biologic behavior but sometimes suggests the diagnosis. Cavernous malformations are usually round. Benign mixed tumors of the lacrimal gland are said to be oval (see Figure 14.12 ). Sometimes, a particular feature of the shape is diagnostic. The kink of an enlarged optic nerve (a sharp change in the direction of the nerve) strongly suggests a glioma.
The size of the lesion doesn’t tell us a great deal about the diagnosis. Big is usually worse than small, but a small malignancy is worse than a large benign mass. Combined with clinical information, you may be able to glean something useful from the size of the mass. A large mass present for a short time (such as a rhabdomyosarcoma) is probably an aggressive tumor. A mass present for many years, whether large or small (such as a dermoid cyst), is likely to be benign. Make sure you record the size of the mass. Larger orbital masses are easier to find but more difficult to remove, and you may have to alter your surgical approach if you are planning excisional biopsy.
The internal characteristics of a mass can be helpful information. The majority of tumors are homogeneous. Heterogeneous masses may show a diagnostic pattern:
A combination of solid and cystic components in a child’s tumor is typical of lymphangioma.
The layering of fat and keratin debris in a cystic mass is diagnostic for a dermoid cyst.
Small areas of calcification in a soft tissue orbital mass are typical of malignancy.
Tram tracking of an optic nerve tumor is diagnostic for an optic nerve meningioma (parallel lines of calcification in the subarachnoid space).
Hyperostosis of the sphenoid wing is diagnostic for a sphenoid wing meningioma ( Figure 14.13 ).
Intravenous contrast agents are routinely given for CT scan and MRI evaluations of orbital tumors. Although these agents give an estimate of the blood flow to a mass, their main value is for identifying lesions that may not otherwise be seen. Examples are contrast enhancement of meningioma (see Figure 14.13 ) or rhabdomyosarcoma and inflammatory lesions such as sarcoidosis.
CT scanning is used as the primary imaging technique for orbital disease.
The main indication for MRI is to view the orbitocranial junction. On an MRI scan, what do the terms bright and dark mean? Is the vitreous on a T2-weighted scan bright or dark? Is cortical bone bright or dark? What is a flow void?
Evaluate the imaging of an orbital mass to answer two questions:
What is it?
What is the best surgical approach for biopsy?
Consider the following:
The location of the mass. Can you determine the tissue from which the mass is arising? If not, in which surgical space is it located?
Is the mass a pusher or an eater? Can you recognize the difference? How does this reflect the biologic behavior? This is one of the most important pieces of information learned from a scan.
Name three orbital lesions with characteristic imaging features.
There are too many orbital disorders to discuss them all in this text, so I have chosen to describe the most common or most important problems. The common disorders are just that, and they are the ones most often seen. Thyroid eye disease is an example. As you know, it is the most common cause of unilateral or bilateral proptosis. Thyroid disease is so common that you should consider it as a possible cause of orbital disease in any patient with proptosis. It is the cause in some patients with apparent unilateral proptosis and almost always the cause in patients with bilateral proptosis. The important disorders are included for a number of reasons. Some are classic textbook examples of orbital disease, such as optic nerve tumors. Optic nerve meningioma is an example; it is a rare problem that you need to know about, if for no other reason than because it is often in a question on board examinations. Other important disorders are included because you should not miss them, usually for a specific reason. Benign mixed tumor of the lacrimal gland is an example. Excisional biopsy should be used for this rare tumor of the lacrimal gland rather than incisional biopsy to prevent a later recurrence, which might possibly degenerate into a malignant mixed tumor. For the more common lymphoid infiltrates of the lacrimal gland, an incisional biopsy of the gland should be performed. Although benign mixed tumors are uncommon, you should at least consider the diagnosis in every patient with lacrimal gland enlargement before performing an incisional biopsy. Another example is orbital rhabdomyosarcoma in children. In any child with rapidly progressing proptosis it is necessary to rule out this life-threatening disorder. Early diagnosis results in a cure in more than 90% of patients with rhabdomyosarcoma of the orbit alone.
Each disorder that we discuss is complex in itself. Take the following approach:
Learn the common presentation—the P ’s of the history and physical examination that let you make the diagnosis.
Understand what the disease process is.
Recognize the imaging characteristics that help to confirm the diagnosis.
Understand the treatment options.
Each topic follows a format that lends itself to learning these points.
This orbitopathy is characterized by an ill-defined onset of progressive orbital inflammation causing lid swelling, proptosis, lid retraction, and strabismus. Unlike other inflammatory conditions, pain is not part of the presentation. Beware of attributing any painful orbitopathy to thyroid disease. Thyroid eye disease is the most common cause of unilateral or bilateral proptosis. As we said, at least briefly, consider this diagnosis in all patients with proptosis. Without looking at other signs and symptoms, you are more often correct about this diagnosis than any other.
Thyroid eye disease affects women five to six times more often than men. The onset is most common in the early 40s and mid-60s. The onset of symptoms is usually gradual, so that patients often cannot recognize when symptoms first appeared. The disease progresses at a highly variable rate with a similar variation in severity. In extreme cases, the disease may progress very rapidly. No pain is associated, but discomfort, more like pressure or orbital fullness, is often present. The past medical history often reveals systemic thyroid disease. A family history is common. Smoking is a risk factor in the frequency and severity of thyroid eye disease. Generally, thyroid eye disease is worse in men than women.
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