Case History An accurate recording of the patient´s history will gather significant information that will determine further diagnostic investigation and work-up. It is important to gain information on the following symptoms: Nature of complaints Main complaint Pain (spontaneous, during eye movements, upon pressing) Diplopia Visual impairment Visual field defects Lid reddening, lid swelling, asymmetry of palpebral fissure Chemosis, conjunctival injection Periorbital swelling and redness Proptosis (Valsalva maneuver), pulsating exophthalmos Crepitation of the eye lid skin on pressure Headache (type and course) Accompanying neurologic symptoms Pain or feeling of pressure in the area of the paranasal sinuses Sensory deficits such as paresthesia in the area of the teeth (most importantly in the upper jaw) Olfactory deficits Audible bruits Beginning and course of the symptoms Progression rate Acute (e.g., inflammations) Subacute (e.g., Graves’s orbitopathy) Chronic (e.g., tumors) Increase or decrease of the complaints or triggering factors Is this a first incidence or a recurrence? Has an investigation already been carried out for similar symptoms? Possible etiology Age of the patients: children in particular are affected by inflammations, injuries and certain tumors; in middle age there is predomination of Graves’s ophthalmopathy; while in older patients tumors are more likely. 10 Preceding trauma Operations (e.g., paranasal sinuses or neurosurgical interventions) Allergies General diseases such as hyperthyroidism, other endocrinal or tumorous (e.g., malignant) diseases Drug history to exclude toxic side effects from medication Previous examinations Is it the first presentation or have previous examinations already been made on the current problems? Are the findings are available? Names of the other treating physicians should be acquired and documented. If indeterminate findings or symptoms have existed for some time, patients should be asked to bring old photographs that may give a clue how long the current finding may have already existed. Family history Before a more advanced examination is carried out, the first procedure should be the inspection of the patient, preferably in an erect position. The examination room should be sufficiently well illuminated; a penlight can be used to augment ambient lighting but the examiner has to make sure not to blur the patient as it might interfere with the ensuing visual acuity testing. When carrying out the inspection, attention should be paid to findings such as facial anomalies or asymmetries, signs of inflammation or injuries, hematomas, or other(s). Always compare one eye with the other and determine whether they are symmetrical in size and position. Given that one eye is normal, unilateral eye pathologies are much more easily recognized. When a difference is observed, it is essential to determine which side exhibits the abnormality. In cases with unilateral proptosis or eyelid swelling with occlusion of the palpebral fissure, close attention has to be paid to properly examine the eye and the extraocular motility (e.g., limited extraocular motility in orbital cellulitis). The eyeballs should be checked to see if they are in the same plane as the eyebrows and maxillae. In orbital diseases the key symptom is exophthalmos. The best way to assess proptosis is by looking from behind over the shoulder of the patient or from the front after pulling up the upper eyelids and asking the patient to look down. It is very important to exclude pseudoexophthalmos (e.g., in severe myopia or an enlarged palpebral fissure) or enophthalmos of the other eye. During the inspection, attention should also be paid to a possible malposition of the affected eye in primary position (looking straight). In this position the upper eyelid covers the upper portion of the cornea. An axial anterior displacement of the eye usually indicates an intraconal disease process, such as cavernous hemangioma. However, if additional lateral displacement is present, an extraconal disease has to be ruled out. In general, the localization of the disease process should usually be sought on the opposite side of the displacement ( ▶ Fig. 3.1): If there is an inferior and medial displacement of the eye then a pathologic process in the temporal upper orbital space, such as a disorder of the lacrimal gland, has to be suspected. If there is an inferior and temporal displacement of the eye, a process in the upper nasal orbital space, such as a mucocele, should be excluded. Fig. 3.1 Patient with a traumatic orbital hematoma causing an infero-temporal displacement of the eye globe. When carrying out the inspection, possible pulsations of the eye should be ruled out. These can best be identified by looking from the lateral side and by examining with a magnifying glass. The relative height and the position of the two lid margins as well as the surface of the eyelids are observed for pathologic changes. The facial skin in the vicinity of the orbit has to be checked. In inflammatory diseases, such as orbital cellulitis, marked swelling of the eyelids can occur (see ▶ Fig. 6.3). These changes have to be differentiated from local lid inflammation (e.g., chalazion or hordeolum). Lid mobility can be affected in Graves’s orbitopathy (e.g., upper lid retraction = Dalrymple’s sign), after oculomotor nerve damage, or after trauma (ptosis). If the patient states that there has been an acute trauma previous to the presentation, one should look for signs of injury, which sometimes can be very subtle, such as small wounds and orbital hematomas ( ▶ Fig. 3.2). Fig. 3.2 Patient with a traumatic ptosis and a small wound in the upper lid caused by a foreign body injury on the right eye. During the inspection, signs of generalized diseases with possible involvement of the orbit can be disclosed such as café au lait spots in patients with neurofibromatosis. After the inspection, palpation of the periorbital regions should be carried out. As a rule, the palpation is started on the unaffected side in order to better assess pathological findings. Space-occupying processes in the anterior orbit or in the eyelids, such as lacrimal gland processes or dermoid cysts, can often be palpated subcutaneously. Attention is paid to consistency, color, shape (well-defined or diffuse), margins, pain, and movability. Changes to the bony orbital rim caused by fractures or bone defects caused by malignant tumors are likewise palpable. Fractures of the medial orbital wall (lamina papyracea) can lead to orbital and lid emphysema, which becomes noticeable through a typical crepitation upon pressure to the lid tissue. Sometimes pulsations can be felt or seen in pathologic vascular processes. The paranasal sinuses should be examined for pain over the cheek and radiating to frontal region or teeth, increasing with straining or bending down. Regional painful lymph nodes can point to an acute inflammation, whereas painless lymph node enlargement occurs more frequently in chronic processes, for example, with tumors. Pain sensation or numbness in the cutaneous distribution (dermatomes) of V1, V2 and V3 is tested by touching the skin at the nerve exits of the sensory branches of the trigeminal nerve. Ocular diseases should always be excluded if the patient complains of pain sensation in the (peri)orbital region. These encompass intraocular inflammations, ocular surface problems (e.g., sicca syndrome), asthenopia due to uncorrected refractive errors, and elevated intraocular pressure in glaucoma. The latter can be tested by a simple method, which is done bimanually with careful pressure of both index fingers on the closed upper eye lid, comparing the two sides ( ▶ Fig. 3.3). Fig. 3.3 Bimanual palpation to estimate the intraocular pressure. Note that this examination must always be performed on both eyes. Any disease process in the orbit can lead either to direct impact on the optic nerve (e.g., compression through tumor growth; posttraumatically) or to indirect nerve damage (e.g., through inflammatory mediators or perfusion disorders), which often result in impairment of visual acuity and/or disturbances in the visual field. Space-occupying lesions in the orbit can cause direct compression of the eye globe. Under certain circumstances this can lead to a significant refractive changes. The resulting refractive errors can give hints to the localization of the orbital mass ( ▶ Fig. 3.4): An intraconal process—for example, a cavernous hemangioma—leads to a shortening of the eye globe due to axial pressure and thus to hyperopia. In contrast, an extraconally located tumor—for example, processes in the lacrimal gland or a mucocele—leads to lateral compression of the eye globe, which can result in myopia, astigmatism, or a combination of both. For this reason, orbital pathologies have to be excluded in any patient, who presents with an unexplained refractive change. Fig. 3.4 Mechanism of refractive change in an eye due to intraorbital tumor formation. Although many pathologic findings of the anterior segment of the eye—such as of the cornea or of the conjunctiva—can be distinguished with the naked eye, definitive results and classification of diseases are only possible biomicroscopically using a slit lamp. Usually an edematous conjunctiva (chemosis) and/or diffuse conjunctival injection occurs with infections, allergic reactions, or intraocular inflammations. Isolated congestion of episcleral vessels is—among others—a typical sign for carotid–cavernous sinus fistula ( ▶ Fig. 3.6). Isolated injection in the area of the insertions of the rectus muscles, in association with painfully restricted extraocular motility can indicate myositis. Hemorrhages beneath the conjunctiva can occur spontaneously, after trauma or subsequent to retrobulbar hematoma ( ▶ Fig. 3.5). Isolated bleeding into the lymph vessels of the conjunctiva can also occur, for example, with an orbital lymphangioma ( ▶ Fig. 3.7). A localized, salmon-colored patched swelling of the conjunctiva is typical for lymphoma (MALT lymphoma). This typical finding gives a hint for a conclusive diagnosis at the first examination. Lower and upper lid hematoma (monocle hematoma) is common finding after lid or orbital trauma ( ▶ Fig. 16.2). In these cases an intraorbital hematoma always has to be excluded. Fig. 3.5 Dilatation of conjunctival blood vessels and congestion of episceral veins in a patient with an internal carotid artery–cavernous sinus-fistula. Fig. 3.6 Significant conjunctival chemosis and intraconjunctival hemorrhage. Fig. 3.7 Hemorrhagic conjunctival lymph vessels. Cranial, orbital or cervical bruits are caused by transferred vibrations, which are the result of turbulences in intra- or extracranial blood vessels. 11 Most frequently they occur during the systolic phase, but can also extend into the diastole or be present continuously. Often it is difficult to assign these bruits to an origin as they may arise either in the cranium or in the neck as well as from the heart. Orbital auscultation is a screening technique indicated in patients with tinnitus, pulsatile exophthalmos, persistent conjunctival injection unresponsive to therapeutic efforts, headache, and neurologic disorders. It is performed by gently placing the stethoscope over the closed eyes and should include the zygomatic and lateral regions as well as the skull ( ▶ Fig. 3.8). In order to correlate the acoustic phenomena with cardiac activity, the radial pulse is palpated simultaneously. Bruits or noises may be crucial in guiding further diagnostic evaluation. A pulse-synchronous “machine noise” over the orbit is pathognomonic for a carotid–cavernous sinus fistula. Pulse-synchronous noises can also occur in orbital/cerebral AV malformations, in intracranial space-occupying masses with raised intracranial pressure, as well as in other diseases such as vascularized tumors and bone defects (e.g., sphenoid wing aplasia in neurofibromatosis). However, these conditions are very rare. 50 Fig. 3.8 Auscultation of the orbit. One of the key symptoms of most orbital diseases is exophthalmos. Enophthalmos is encountered less frequently (e.g., in cirrhotic breast carcinoma metastasis). As facial asymmetries, osseous malformations, anomalies of the palpebral fissure, or pseudoexophthalmos (e.g., in high myopia) can mimic exophthalmos, objective measurement of the proptosis is crucial. Exophthalmometry is an important means to objectively identify clinically suspected exophthalmos and plays an important role in follow-up examinations to assess the clinical course. There are various devices available (e.g., Hertel exophthalmometer, Luedde or Naugle exophthalmometer), the Hertel exophthalmometrer being the most widely used ( ▶ Fig. 3.9). The extent of an axial forward displacement of the eyeball can be objectively determined using this device. The distance between the corneal apex and the orbital rim is measured at the level of the zygomaticofrontal suture. Values under 15 mm and above 20 mm as well as a difference between the eyes exceeding 2 mm are significant and make further investigation mandatory. The actual exophthalmometer readings as well as the interorbital distance are important parameters to be documented for monitoring purposes. Conclusions about the actual course of the proptosis can only be drawn if the correct interorbital distance is set in repeated measurements. More recent developments in this field are digital exophthalmometry 12 and Hertel exophthalmometry without contact to the orbital rim. 13 Fig. 3.9 Hertel exophthalmometry. Normal exophthalmometry values vary significantly depending on age, sex, and ethnic origin. In a meta-analysis of different studies, Rootman stated that black people usually have higher average values than whites, whereas people of Asian origin stand between the two. 5 Analyses of exophthalmometry values suggest that they increase with age 5: thus, in children between 5 and 7 years of age, the average values range from 12.6 mm (girls) to 13.7 mm (boys), but in 8- to 10-year old children they are from 14.1 mm (girls) to 13.7 mm (boys). In the adult group the average values rise to 16 mm for men and 16.5 mm for women. Palpation of the intraocular pressure (as described in Chapter ▶ 3.1.4) is only used if no other instruments are available. By comparing both sides differences in intraocular pressure can be revealed and in some cases increased intraocular pressure can be concluded. However, various tonometers are available to determine the intraocular pressure accurately, such as the Goldmann or Schiøtz tonometers, the noncontact tonometer, the I-Care tonometer, or the Perkins hand-held applanation tonometer, the most common being the Goldmann tonometer. In contrast to Schiøtz impression tonometry, which nowadays is not routinely used, almost no intraocular fluid is displaced so that Goldmann tonometry provides more reliable values. Local anesthetic drops containing fluorescein must be applied before starting the measurement. The amount of force required to flatten a 3.06-mm diameter circle of the anesthetized cornea ( ▶ Fig. 3.10) is measured with the patient in an erect position. The intraocular pressure can be read directly on the calibrated dial of the tonometer after the mires (semicircles) in the prism of the tonometer head have been properly aligned. Fig. 3.10 Applanation tonometry with the Goldmann tonometer. Changes in intraocular pressure related to orbital disease occurs mainly in Graves’s disease, arteriovenous shunts, trauma, and orbital neoplasia. In Graves’s orbitopathy, the intraocular pressure is commonly elevated because of intraorbital inflammation and edema. In upgaze the globe is often squeezed between the rectus superior muscle and the fibrotic rectus inferior muscle. This results in a temporary elevation of the intraocular pressure, which rapidly returns to normal after looking straight ahead again. This is why the intraocular pressure should be measured and documented in upgaze and downgaze as well as in the primary position. Elevated intraocular pressure can also be correlated with thrombosis of the superior ophthalmic vein or a carotid–cavernous sinus fistula. Increased intraocular pressure secondary to orbital diseases has to be differentiated from glaucoma of ocular origin such as primary open-angle glaucoma. A careful patient history has to be taken in order to identify pre-existing glaucoma. Reduced intraocular pressure occurs after intraocular inflammations with ciliary body involvement and ocular phthisis. If ocular hypotony after trauma is noted, penetrating/perforating injury of the eye (open or closed) has to be ruled out. Motility disorders can be of mechanical origin (e.g., with entrapment of the inferior rectus muscle in orbital floor fracture), inflammatory origin (e.g., with myositis), or paretic origin (e.g., as the result of nerve lesion following trauma, compression, or inflammation). 14 Palsies can occur after injury to a single nerve (such as traumatic palsy of the trochlear nerve or abducens nerve), after primary nerve damage (e.g., with a neurinoma), or after a combination of various cranial nerve injuries (such as in superior orbital fissure or orbital apex syndrome). Thus patients may complain of a variety of visual problems, such as diplopia, blurred vision, visual confusion, oscillopsia, or tilted images accompanied by vertigo. Whereas double vision can arise very early in acute diseases, it may develop quite late in slowly expanding processes (mucoceles, etc.), as over time even significant globe dislocations can be compensated. The extent of the double vision depends on the direction of gaze, on the location of the process, and on the nature of the disturbance to the nerves or muscles. The displacement of the eye by a tumor can cause double vision already in straightforward gaze, whereas paretic double vision usually occurs when looking in the direction of the primary action of the eye muscle (noncomitant strabismus: for example, abduction in abducens nerve palsy). Motility disorders can also occur as complications after orbital surgery. If these are caused by postsurgical hemorrhage or swelling, they frequently decrease spontaneously over time. Subsequent strabismus following surgical interventions (e.g., optic nerve decompression in Graves’s orbitopathy) is not uncommon. Thus, patients should be informed preoperatively about the potential risk of restriction of ocular motility and disturbing symptoms. However, postoperative double vision may be regarded as evidence that orbital decompression has been successful. Because ocular motor disorders are not uncommon in the population and may already be present, an ocular motility examination should always be obtained prior to surgery. If an orbital disease is suspected, ophthalmoscopy should be carried out after the pupil has been pharmacologically dilated. Even nonophthalmologists can examine the posterior fundus by means of a direct ophthalmoscope. A major drawback is the poor visibility of the peripheral fundus. Ophthalmologists prefer binophthalmoscopic funduscopy by means of an indirect ophthalmoscope, a contact lens, or a handheld lens (e.g., Volk lens +78 diopters) ( ▶ Fig. 3.11). Fig. 3.11 Opthalmoscopy of the central ocular fundus using a Volk lens. Funduscopic findings can give clues for orbital diseases. 15 Inflammatory or neoplastic processes can cause optic disc edema by direct compression or venous congestion (orbital papilledema, ▶ Fig. 3.12). Long-lasting compression of the optic nerve or the optic disc can result in optic atrophy ( ▶ Fig. 3.13). Opticociliary shunt vessels can be pathognomonic for optic nerve sheath meningioma ( ▶ Fig. 3.14). Venous drainage obstruction is often associated with congestion of the retinal veins. Mass-induced globe indentation may cause chorioretinal folds that are visible in funduscopy ( ▶ Fig. 3.15). Likewise, primary intraocular tumors with subsequent infiltration of the orbit can be discovered by ophthalmoscopy (e.g.. malignant melanoma of the choroid, retinoblastoma). Fig. 3.12 Papilledema with optic disc hemorrhages and retinal vascular tortuosity. Fig. 3.13 Optic disc atrophy.
3.1.3 Inspection
3.1.4 Palpation
3.1.5 Visual Acuity Test
3.1.6 Slit-lamp Biomicroscopy of the Anterior Segment of the Eye
3.1.7 Auscultation
3.1.8 Exophthalmometry
3.1.9 Tonometry
3.1.10 Motility Testing
3.1.11 Ophthalmoscopy
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