Ординатура / Офтальмология / Английские материалы / Orbital Tumors Diagnosis and Treatment_Karcioglu_2005

Acomplete ocular history and a physical examination are mandatory for every orbit patient. In an emergency, some parts of the eye ex-

amination can be postponed, unless the results are pertinent for the patient’s differential diagnosis.

Sensory problems, including numbness and tingling, as well as cold/heat sensation, should also be inquired about. Visual history related to clarity and color vision should be questioned. Awareness of a positive or negative scotoma should be checked.

History taking should begin with the most important issue: the chief complaint of the patient. The answer may be “pain around my eye,” “protrusion of my left eye,” “double vision,” or simply “When I went to my ophthalmologist to renew my glasses, the doctor noticed such and such and advised me to come and see you.”

The physician’s inquiry should include general questions about the onset and the duration of the chief complaint. The patient’s systemic health should be investigated, and inquiries of past and present illnesses, injuries, surgeries, and drug treatment should be made.1,2 Particular attention should be paid to history of cancer, thyroid disorders, and past episodes of infectious diseases.2,3 The allergy history of the patient should also be questioned.

If the patient is complaining of “bulging of the eye,” it may be beneficial to ask for an old photograph or a driver’s license to verify the presence or absence of orbital changes. The onset of proptosis should be questioned in detail. Did it begin with an acute episode of redness and swollen eyelids and/or conjunctiva? Did it begin as a painful episode? Did the pain ease afterward? Did the pain increase or decrease during the course of the disease? Is the pain intermittent, and does it radiate to a particular spot on the patient’s face? Does the patient feel a dull pain behind the eye when moving the eyes from right to left and up and down? Was the proptosis totally painless? Does the proptosis get worse when the patient sneezes or coughs? Was the proptosis intermittent, getting worse during certain times of the day, and did this intermittency stabilize later in the course of the disease? Does the patient feel irritated and gritty in the eyes; is the protruding eye worse in this regard? Does or did the patient hear a bruit (running-water sound) after the development of the proptosis? Does the patient have double vision?

The ocular examination should include the best corrected visual acuity and intraocular pressure applanation in the primary position and in different vertical and horizontal gazes. A neuro-ophthalmologic examination, including motor and sensory functions, pupillary examination, contrast sensitivity, color vision assessment, confrontation visual fields, and central visual acuity with an Amsler grid should also be done (see Chapter 7).

During biomicroscopy, the integrity of the corneal and conjunctival epithelium should be checked as well as the conjunctival and subconjunctival blood vessels for dilatation, fusiform distension, tortuosity, and congestion. Chemosis of varying degrees is also a common finding in orbital tumor patients (Figure 6.1).

Fully dilated indirect ophthalmoscopy should be performed on every patient because many orbital diseases cause a wide variety of funduscopic changes, which may provide clues regarding the location, size, and the nature of the orbital pathology.4 The major fundal manifestations of a space-occupying mass in the orbit include chorioretinal folds, retinal vascular changes, and optic disk edema and/or atrophy (Table 6.1).

Chorioretinal folds appear as a series of delicate striae, which are most often present in the posterior pole (Figure 6.2). Lines are usually parallel, but rarely they may radiate haphazardly to all directions.5 Although chorioretinal folds are most commonly seen with orbital tumors, they are also seen with mucoceles and other types of cysts and also in cases of orbital injury.6–9 Most chorioretinal folds are without symptoms and do not affect visual acuity.

The etiopathogenesis of chorioretinal folds is not known, but they are most likely due to the compression of the globe by a space-occupying lesion in the orbit.10 However, the compression theory fails to explain the process fully, since in some cases folds are

FIGURE 6.1. (A) Congestion of the conjunctiva with tortuous vessels in an orbital tumor. (B) Marked chronic chemosis in a proptotic orbit with long-standing optic nerve meningioma.

present without scleral indentation, and in other patients, rapidly expanding orbital tumors lead to scleral indentation without chorioretinal folds.11 There is no clear relationship between the size of the spaceoccupying lesion and the extent or direction of the chorioretinal folds. Furthermore, the position of the chorioretinal striae is not a very dependable finding on which to localize the compressing orbital lesion.5 Although choroidal folds have a typical appearance on fluorescein angiography, appearing as alternating light and dark lines, this test is not helpful in adding anything to the clinical workup of a patient in the presence of chorioretinal folds. The folds usually regress

FIGURE 6.2. Fundus photograph showing horizontally aligned choroidal striae in a patient with intraconal cavernous hemangioma.

after treatment of the associated orbital pathology.12 However, they may persist many months or even years after the exclusion of the primary orbital disease.

Congestion and increased tortuosity of retinal veins is another significant finding secondary to spaceoccupying mass lesions of the orbit, which occur more commonly with masses located in midorbit, causing stasis through the vortex veins. The diameters of engorged retinal veins are best measured and compared with the fellow eye by means of fluorescein angiography.

Disk edema, optic nerve atrophy, and, occasionally, optociliary shunt vessels13–15 are other findings that should be noted during the funduscopic examination of the orbit harboring a space-occupying lesion (Figures 6.3, 6.4, and 6.5). Optociliary vessels are venous shunts that develop to carry blood from the retinal vasculature to the juxtapapillary choroidal circulation when the retinal venous return at the optic nerve head is blocked secondary to optic nerve tumors and other pathology.16 Therefore, “retinochoroidal venous shunt” is a better name for these collateral vessels, which usually develop in meningioma, and, rarely, in optic nerve glioma, central retinal vein oc-

clusion, juxtapapillary tumors, or cysts. As opposed to choroidal folds and vascular changes, optic disk changes are better seen by means of direct ophthalmoscope or a contact lens. Optic nerve changes and other neuro-ophthalmologic features are detailed in Chapter 7.

The funduscopic examination of the orbit patient is also important to detect posttreatment changes secondary to surgical complications, radiation therapy, chemotherapy, and second primary malignancies.4,17,18

ORBITAL EXAMINATION

The external examination of the patient should assess the facial features and critically evaluate the symmetry of the ocular, eyelid, and orbital structures. Phys-

ical examination of the periorbital structures, including eyelids and conjunctiva, should include inspection of appearance and function, which are commonly altered by a space-occupying lesion in the orbit. The horizontal distance between interpalpebral fissures and the width of the palpebral fissures should be measured and recorded. Additionally, the distance between the margin of the upper eyelid and the upper eyelid crease, as well as the amount of inferior scleral exposure, should be measured (Figure 6.6). The comparison of these values to those of the fellow eye is usually helpful because most orbital tumors present with unilateral structural abnormalities.

Levator function should be determined on both sides and carefully recorded. Although there are many methods to assess the levator function, it is usually sufficient to measure the margin reflux (MRD: dis-

tance between the margin of the upper eyelid and the corneal light reflex) and to obtain the full range of vertical motion [Burke levator function (BLF)] of the upper lid. Normal values for MRD and BLF are 3–5 and 15–18 mm, respectively.19

The appearance of the eyelids in relation to the globe should be observed individually by alternate cov-

A

B

FIGURE 6.6. Normal eyelid and periorbital distances in a young adult (A) versus an elderly patient with bilateral proptosis (B). (VPF, vertical palpebral fissure; HPF, horizontal palpebral fissure; MRD, margin reflex distance; MCD, margin crease distance; IPD, interpalpebral distance.)

erage of the eyes while the physician is facing the patient; then the eyes should be viewed simultaneously from the same distance. Slowly growing masses of the orbit usually do not alter the anatomic relationship of the eyelids to the globe, and extraocular motility is affected only at extreme gazes; therefore, when observed one side at a time, these patients may look normal or close to normal. When both eyes are observed simultaneously, however, the proptosis of one eye and/or lid distortion becomes much more obvious.

The most important structural feature to rate in the examination of an orbit with a space-occupying lesion is proptosis, which is also known as exophthalmos, protrusion, or the displacement of the globe beyond the orbital rim.20 Proptosis is an old term having its roots in Galenic terminology, meaning “falling forward” or “falling out.” In its original context, however, it was used for traumatic prolapse of the uvea, particularly of the iris.21 Exophthalmos, on the other hand, is a rather new term, initially appearing in the seventeenth century, to describe the forward thrust of the eyes resulting from a systemic straining, such as hanging. After Robert Graves’s association of the protrusion of the eyes with thyroid disease, exophthalmos came to be commonly referred to as a manifestation of thyroid-associated orbitopathy or Graves disease.

The term “proptosis” is commonly used to describe a forward displacement of the eye, secondary to a spaceoccupying lesion in one orbit, in most cases a tumor or a cyst. The displacement of the eye is determined based on the distance in an anterior–posterior plane between the front surface of the cornea and the anterior

FIGURE 6.7. Evaluation with Hertel exophthalmometer.

perimpose them on the measuring scale (Figure 6.7). The Hertel exophthalmometer offers accurate and reproducible measurements and the advantage of comparing one eye with the other during the same examination. In Hertel exophthalmometry, the distance between the two lateral orbital rims is engraved on the horizontal bar, which carries the sliding mirrors. This baseline measurement should be kept at a constant in repeat examinations to ensure dependable comparisons between measurements; ideally the same person should perform the exophthalmometry at each evaluation.

The Naugle exophthalmometer (orbitometer) is another instrument used to measure globe displacement (Figure 6.8). This device is designed to sit on vertical

fixation bars that rest on superior and inferior periorbital rims, rather than the lateral canthi. Therefore, it measures not only enophthalmos and exophthalmos, but hypoand hyperophthalmos as well. The main advantage of this instrument is that it renders accurate measurements even when the lateral rim is irregular or missing.30,31 This instrument is particularly useful in evaluating patients with maxillofacial trauma.32

An exophthalmometry measurement above 21 mm or a difference of more than 2 mm between the two eyes is usually considered abnormal. Measurements less than 14 mm are considered to be an enophthalmos.33,34

The direction of the globe displacement may carry diagnostic significance; if the globe is pushed down and out, natural location for the space-occupying lesion is superiotemporal (i.e., a lacrimal gland tumor) (Table 6.2).35 The degree of asymmetry may also be important. Slowly growing, benign lesions may produce extreme asymmetrical proptosis. On the other hand, the thyroid disease usually produces less symmetrical displacement of the globes. The proptosis direction is usually downward because the majority of the primary orbital tumors develop in the upper half of the orbit. Lateral displacement, on the other hand, is usually seen as a result of secondary orbital lesions, such as a mucocele or squamous cell carcinoma, originating from the ethmoidal sinuses. Squamous cell carcinoma may displace the globe upward when it originates from the maxillary sinus. In some instances, simple transillumination with a muscle light reveals the cystic nature of an orbital lesion; this is particu-

larly true for anterior orbital masses (Figure 6.9). Displacement of the globe toward the nose is quite rare because very few space-occupying lesions develop on the lateral aspect of the orbit.

The proptotic eye should also be examined from the standpoint of ocular motility in cardinal positions and compared with the normal eye. Recordings of deviations should be made whenever applicable. Although accurate recordings of prism values may be useful for the posttreatment follow-up of the patient, detailed diplopia measurements in prism diopters are not always possible or necessary. The amount of deviation can be quickly approximated by a red glass test in the clinic.36 The author’s preference is to use a modified Maddox cross engraved on transparent plastic that has a white fixation light in the center (Figure 6.10). When the patient is seated with straight head position at 50 cm, the numbers on the cross indicate the amount of deviation in degrees. A red glass is positioned in front of the fixating eye, and the patient is asked to point at the “white light” and the “red light” on the surface of the device (Figure 6.11).

The position of the visual axis in a proptotic eye may provide useful information. In some patients with slowly growing orbital masses, the proptotic eye adapts to the fellow normal eye and may have parallel visual axis. This is commonly seen in dermoid cysts, benign mixed tumors of the lacrimal gland, and slowly growing neural tumors. In contrast, rapidly growing or posteriorly located masses, as well as metastatic tumors to the extraocular muscles, invade the nearest neuromuscular structures,

resulting in malfunction of the motility with consequent strabismus.

The rotation of the proptotic eye should be assessed. The rotation disturbance caused by proptosis can be grouped into one of two categories: (1) abnormal rotation of the eye turning toward the affected quadrant, usually seen with slowly growing masses, or (2) abnormal rotation resulting from secondary neuromuscular invasion. In the latter situation, the abnormal rotation of the eye is not limited toward the direction of the affected sector. Infiltrating orbital lesions, on the other hand, produce the most severe impairment of ocular rotation, whether they are neoplastic or inflammatory in origin. Orbital cellulitis, diffuse pseudotumor, secondary squamous carcinoma from the ethmoid sinus involving the posterior orbit,

FIGURE 6.10. Maddox cross with a fixation light in the center. The scale of the cross is adjusted so that when the patient is at 50 cm, the engraved numbers on the plastic panel indicate the amount of deviation in degrees.

and metastatic scirrhous breast carcinoma are good examples of this pathology (see Chapter 24).

Generally, extraocular motility disturbance accompanying acute inflammatory disorders develops rapidly and may be painful. The ophthalmoplegia of Graves disease, on the other hand, develops gradually without pain and may, in some instances, appear prior to exophthalmos. In patients with known hyperthyroidism, ophthalmoplegia is usually noted in upward gaze secondary to slow infiltration of the superior rectus muscle by glycoproteins and chronic inflammatory cells. As the disease progresses, the extraocular motility disturbance becomes generalized, and finally, the eye may come to a standstill in the abnormal position of upward or downward gaze owing to extensive scarring of the extraocular muscle, in the direction of the deviation. The infiltrating tumors, on the other hand, have a tendency to “freeze” the eye in the primary position of gaze because of their haphazard infiltration into the muscles and the soft tissues of the orbit.

External examination must include the changes of the soft tissues of the eyelids, conjunctiva, and periorbital skin. Edema, hyperemia, and tenderness of these tissues may be a part of the clinical picture in orbital tumors; however, anteriorly located tumors such as lymphomas may cause a certain degree of lower lid edema and conjunctival chemosis. Edema of the lids, greater in the lower than in the upper eyelid, is occasionally associated with hemangioma and neurogenic tumors, presumably secondary to long-stand- ing venous stasis. Soft tissue involvement with the resulting edema and/or retraction is more commonly associated with Graves disease and pseudotumor (Figure 6.6B). Hyperemia is also more often encountered with inflammatory lesions, particularly in acute pre-

FIGURE 6.11. Evaluation of diplopia with a red glass positioned in front of the fixating eye of the patient. The patient points to white and red lights on the Plexiglas surface of the Maddox cross.

sentations.37 However, redness of the eyelids may be seen in any rapidly growing malignant tumor that is located anteriorly, such as more malignant types of lymphoma, leukemia, rhabdomyosarcoma, and metastatic tumors.38,39 Ecchymosis should not be confused with hyperemia. The former is most often seen in metastatic neuroblastoma but may also be encountered with amyloid and leukemic infiltrates (Figure 6.12). A typical feature of ecchymosis, secondary to neuroblastoma, is its changing appearance from day to day.40 Xanthelasmas on the eyelids and periorbital skin

FIGURE 6.13. Palpation of the orbit under general anesthesia prior to surgery.

FIGURE 6.14. Examination of the orbit with Doppler instrument.

shunts, can be felt and may be useful findings in the differential diagnosis.42 Auscultation with a stethoscope or Doppler testing is a much more dependable approach to detect bruit in the orbit (Figure 6.14). The use of a color Doppler instrument with conventional B-scan ultrasonography may be useful to study vascular tumors of the orbit as well as the blood flow characteristics of other tumors (see Chapter 8).43,44

During the clinical evaluation, one should keep in mind that the orbit is a small chamber, occupied by tissues of many types, located in a very compact fashion. Because of the close relationship of different tissue types, certain disease entities of different etiology and different tissue origin may clinically present with remarkably similar signs and symptoms. For example, a vascular tumor, a cholesteatoma, and a sarcoidosis granuloma may develop very similar clinical and radiological features. Another example of this dilemma may be experienced in the early stages of a subperiosteal abscess, secondary to ethmoiditis, which may mimic a secondary squamous cell carcinoma originating from the same sinus. Furthermore, there is surprising variability in the clinical manifestations of individual disorders; good examples of this are metastatic tumor to the orbit, idiopathic orbital inflammation, and Graves disease. Similar confusion may occur in the evaluation of ultrasonography, computed tomography scans, and magnetic resonance images. Although many diseases tend to be confidently interpreted on the basis of imaging, others may be very confusing. For instance, although the enlargement of extraocular muscles is quite typical of Graves disease, it may be seen in other conditions, including metastatic carcinoma, hematoma, rhabdomyosarcoma, inflammatory orbital pseudotumor, and parasitic infections.

The ophthalmologist should have a systematic approach to the workup of orbital tumor patients and keep in mind that one aspect of evaluation will not necessarily offer the final diagnosis. Therefore, a systemic history and physical examination, ocular/or-

bital examination, laboratory, imaging, and consultation findings should be evaluated as a whole to ensure proper assessment of the patient.

References

1.Grove AS. Orbital disease: examination and diagnostic evaluation, Ophthalmology 1979;86:854.

2.Kennedy RE. An evaluation of 820 orbital cases. Trans Am Ophthalmol Soc 1984;82:134–157.

3.Krohel GB, Stewart WB, Chavis RM. Orbital Disease: A Practical Approach. New York: Grune & Stratton; 1981.

4.De La Paz MA, Boniuk M. Fundus manifestations of orbital disease and treatment of orbital disease. Surv Ophthalmol 1995;40:3–21.

5.Newell FW. Choroidal folds. Am J Ophthalmol 1973;75:930– 942.

6.Leatherbarrow B, Noble JL, Lloyd IC. Cavernous hemangioma of the orbit. Eye 1989;3:90–99.

7.Bullock JD, Egbert PR. The origin of choroidal folds: A clinical, histopathological, and experimental study. Doc Ophthalmol 1974;37:261–293.

8.Cangemi FE, Trempe CL, Walsh JB. Choroidal folds. Am J Ophthalmol 1978;86:3880–3887.

9.Newell FW. Fundus changes in persistent and recurrent choroidal folds. Fr J Ophthalmol 1984;62:32–35.

10.Friberg TR. The etiology of choroidal folds. A biomechanical explanation. Graefes Arch Clin Exp Ophthalmol 1989;227: 459–464.

11.Hampton GR. Scleral foldings: a manifestation of orbital lymphoma. Ann Ophthalmol 1982;14:561–562.

12.Kroll AJ, Norton EWD. Regression of choroidal folds. Trans Am Acad Ophthalmol Otolaryngol 1970;74:515–526.

13.Karcioglu ZA, Yulug A, Haik BG. Tumors of the optic nerve. In: Margo C, ed. Clinical Problems in Ophthalmology. Philadelphia: WB Saunders; 1994:105–114.

14.Sarkies NJ. Optic nerve sheath meningioma: diagnostic features and therapeutic alternatives. Eye 1987;1:597–602.

15.Tyson SL, Lesell S. Resolution of optociliary shunt vessels.

J Clin Neuro-Ophthalmol 1986;6:205–208.

16.Imes RK, Schatz H, Hoyt WF, et al. Evolution of optociliary veins in optic nerve sheath meningioma. Arch Ophthalmol 1985;103:59–60.

17.Chacko DC. Considerations in the diagnosis of radiation injury. JAMA 1981;245:1255–1258.

18.Alberti WE, Sagerman RH. Radiotherapy of Intraocular and Orbital Tumors. Berlin: Springer-Verlag; 1993.

19.Gladstone GJ, Black EH, Myint S, et al. Oculoplastic Surgery Atlas, Eyelid Disorders. New York: Springer-Verlag; 2002.

20.Burde RM, Savino PJ, Trobe JD. Proptosis and adnexal masses. In: Clinical Decisions in Neuro-Ophthalmology. 2nd ed. St. Louis: CV Mosby; 1992:379–416.

21.Henderson JW. Orbital Tumors. New York: Raven Press; 1994.

22.Migliori ME, Gladstone GJ. Determination of the normal range of exophthalmometric values for black and white adults. Am J Ophthalmol 1984;98:438.

23.Luedde WH. An improved transparent exophthalmometer. Am J Ophthalmol 1938;21:426.

24.Chang AA, Bank A, Francis IC, Kappagoda MB. Clinical exophthalmometry: a comparative study of the Luedde and Hertel exophthalmometers. Aust N Z J Ophthalmol 1995;23: 315–318.

25.Nasr AM, Ayyash I, Karcioglu, ZA. Unilateral enophthalmos secondary to acquired hemilipodystrophy. Am J Ophthalmol 1997;124:572–575.

26.Hertel E. Ein einfaches Exophthalmometer. Albrecht von Graefes Arch Ophthalmol 1905;60:171–174.

27.Tengroth B, Bogren H, Zackrisson U. Human exophthalmometry, Acta Ophthalmol 1964;42:864.

28.Knudtzon K. On exophthalmometry: the results of 724 measurements with Hertel’s exophthalmometer on normal adult individuals. Acta Psychiatr Neurol Scand 1949;24:523–537.

29.Yeats, RP, van Rens E, Taylor CL. Measurement of globe position in complex orbital fractures. I. A modification of Hertel’s exophthalmometer, using the external auditory canal as a reference point. Ophthalmic Plast Reconstr Surg 1992;8:114–118.

30.Cole HP, Couvillion JT, Fink AJ, et al. Exophthalmometry: a comparative study of the Naugle and Hertel instruments. Ophthalmic Plast Reconstr Surg 1997;13:189–194.

31.Naugle TC Jr, Couvillion JT. A superior and inferior orbital rim-based exophthalmometer (orbitometer). Ophthalmic Surg 1992;23:836–837.

32.Schmitz JP, Parks W, Wilson IF, Schubert W. The use of the Naugle orbitometer in maxillofacial trauma. J Craniomaxillofac Trauma 1999;5:13–18.

33.Iliff NT. The ophthalmic implications of the correction of late enophthalmos following severe midfacial trauma. Trans Am Ophthalmol Soc 1991;89:455–548.

34.Cline RA, Rootman J. Enophthalmos: a clinical review. Ophthalmology 1984;91:229.

35.Reese AB. Expanding lesions of the orbit. Bowman lecture.

Trans Ophthalmol Soc UK 1971;91:85.

36.von Noorden GK. Binocular vision and ocular motility therapy and management of strabismus. St Louis: CV Mosby; 1980:175–212.

37.Noel LP, Clarke WN, Peacocke TA. Periorbital and orbital cellulitis in childhood. Can J Ophthalmol 1981;16:178.

38.Ridgeway EW, Jaffe N, Walton DS. Leukemic ophthalmopathy in children. Cancer 1976;38:1744–1749.

39.Zimmerman LE, Font FL. Ophthalmologic manifestations of granulocytic sarcoma (myeloid sarcoma or chloroma). Am J Ophthalmol 1975;80:975–990.

40.Musarella MA, Chan HSL, DeBoer G, et al. Ocular involvement in neuroblastoma: prognostic implications. Ophthalmology 1984;91:936–940.

41.Karcioglu ZA, Sharrara N, Boles T, Nasr AM. Orbital xanthogranuloma clinical and morphologic review of eight cases.

Ophthalmolic Plast Reconst Surg 2003;19:372–381.

42.Hedges TR. Carotid cavernous fistula—a re-evaluation of orbital signs. Ophthalmic Surg 1973;4:75.

43.Erickson SJ, Hendrix LE, Massaro BM, et al. Color Doppler flow imaging of the normal and abnormal orbit. Radiology 1989; 173:511.

44.Lieb WE, Muller-Forell WS, Wichmann W. Ophthalmologic imaging methods. In: Muller-Forell WS, ed. Imaging of Orbital and Visual Pathway Pathology. Berlin: SpringerVerlag; 2002:9–10.