The nonspecific orbital inflammatory syndromes

SURVEY OF OPHTHALMOLOGY

VOLUME 29 * NUMBER 2. SEPTEMBER-OCTOBER

1984

REVIEW The Nonspecific JOHN S. KENNERDELL,

Orbital Inflammatory M.D., ’ AND STEVEN C. DRESNER,

Syndromes M.D.*

‘Eye and Ear Hospital, University of Pittsburgh, Pittsburgh, Pennsylvania, and “Department of Ophthalmology, Royal Victoria Hospital, itlontreal. Quebec, Canada

Abstract. The nonspecific orbital inflammatory syndromes are a peculiar group of inflammations that may occur in acute or subacute forms and can become chronic. They may be diffuse or primarily localized to a specific tissue of the orbit. The nonspecilic inflammations that are targeted toward specific tissues are myositis, dacryoadrnitis, perineuritis, and periscleritis. Each of these syndromes has definite signs, symptoms, ultrasonic and radiologic findings. Therefore, this group of nonspecific inflammations should be more clearly classified for purposes of better understanding and better management. All of these inflammatory syndromes in the acute form respond well to high doses of oral corticosteroids tapered gradually over a period of months, but may be reoccurrent or become chronic. The subacute form responds less well. Occasionally,

patients require radiation to stop the inflammation in the subacute or chronic state, but these patients are often left with a functional deficit. The cause, although presumed to be an immune disorder involving the orbital tissues, remains unknown. (Surv Ophthalmol 29:93-103, 1984)

Key words. dacryoadenitis nonspecific orbital inflammation periscleritis l pseudotumor

l

inflammation l lesion orbital inflammation l

T

he nonspecific orbital inflammations are a group of idiopathic conditions characterized by the clinical manifestations of an acute or subacute noninfective orbital inflammation which may be diffuse or localized primarily to a specific target tissue. Pain, proptosis, and a generally prompt response to systemic corticosteroids are common clinical courses. In this review, descriptions of both diffuse and localized nonspecific orbital inflammation are presented. The clinical evaluation and treatments of nonspecific orbital inflammation are discussed.

l l

myositis perineuritis

l

*

wide spectrum of nonneoplastic inflammatory lesions.” Other reviews of orbital pseudotumor have included patients who most likely had reactive lymphoid hyperplasia or atypical lymphoid hyperplasia.j”~‘7.*0 All of these entities are “pseudotumors” in that there is no frank neoplasia. In reactive lymphoid hypqrplasia and atypical lymphoid hyperplasia there: is an admixture of predominantly mature lymphocytes with plasma cells, histiocytes, lymphoplasmacytoid bodies, follicles and capillary endothelial proliferation. In atypical lymphoid hyperplasia there is also increased mitotic activity, possibly indicating a continuum of the process toward a malignant lymphoma.20 In the nonspecific orbital inflammatory syndromes, there is a nonspecific polymorphous infiltrate of inflammatory cells, notably neutrophils, lymphocytes, plasma cells, and macrophages, with various amounts of fibrosis depending on the chronicity.g Because both clinically and pathologically the nonspecific orbital inflam-

I. Terminology A. “PSEUDOTUMORS”

The nonspecific orbital inflammatory syndromes have traditionally been termed “pseudotumors,” based on the mass-like effect simulating a primary orbital neoplasm. The description oforbital pseudotumor by Birch-Hirschfeld in 1905 encompassed a 93

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matory syndromes differ from reactive lymphoid hyperplasia and atypical lymphoid hyperplasia, we prefer not to use the more generic term, “pseudotumor.” I n our definition of the nonspecific orbital inflammation, we choose to exclude orbital lymphoid lesions as well as foreign body reactions, infections and specific systemic vasculitities that affect the orbit (e.g., polyarteritis nodosa and Wegener’s granulomatosis). B. CLASSIFICATION The nonspecific inflammatory syndromes range from a diffuse process affecting the anterior, posterior, or entire orbit to a localized process which seemingly targets to a specific orbital tissue. In the past the pathologic focus could not be determined without surgical intervention.” Ultrasonography and computerized tomography, however, have made precise tissue localization possible.‘“~32~“g With the improved resolution of third and fourth generation CT scanners, diffuse orbital inflammation can be differentiated from localized “target organ” involvement. These distinctions have given us the basis for a more precise classification or orbital inflammation, as follows. I . Diffuse nonspecific orbital inflammation II. Localized nonspecific orbital inflammation A. Myositis B. Dacryoadenitis C. Periscleritis D. Perineuritis The nonspecific orbital inflammatory syndromes, therefore, may be identified as diffuse, or as myositis, dacryoadenitis, periscleritis, or perineuritis. Each of these may in turn be acute, subacute or recurrent. C. ETIOLOGY A common property of the nonspecific orbital inflammatory syndromes is that they are idiopathic yet probably related to an orbital immune reaction. Previous reports have noted the occurrence of nonspecific orbital inflammation in patients with polyarteritis nodosa, Wegener’s granulomatosis, and Crohns disease.‘,“*~% Recent reports by Ludwig and Tomsak,2R and Weinstein et a14” suggest that orbital myositis is seen more frequently in patients with autoimmune disorders. It has also been suggested that nonspecific orbital inflammation can be secondary to or concurrent with chronic sinusitis. Only three of 140 patients reviewed by Blodi and Gas? had evidence of sinusitis. Eshagian and Anderson’* have reported two patients with simultaneous nonspecific inflammation involving the orbit and the adjacent sinus.” However, the relationship at this time is still specu-

KENNERDELL

AND DRESNER

Fig. I. Left proptosis, ptosis and upper lid erythema associated with blindness and immobility of the left eye caused by diffuse nonspecific oribital inflammation.

lative and it does not contribute more to our understanding of the etiology and pathophysiology of this condition. A relationship between preceding upper respiratory infection and nonspecific orbital inflammation in children has been documented by Mottow and Jakobiec2” Purcell and Taulbee”’ have also reported two adults with orbital myositis after upper respiratory infection. Streptococcal infection of the extraocular muscles or tissue injury secondary to an autoimmune phenomenon have been postulated;‘4,“H however, the presumed immunologic mechanisms relating to nonspecific orbital inflammation in upper respiratory infections remains speculative.

II. Diffuse Nonspecific Orbital Inflammation A. ACUTE FORM Acute diffuse nonspecific orbital inflammation presents with a sudden onset of severe orbital pain with accompanying lid edema and various degrees of erythema, conjunctival chemosis, proptosis and extraocular muscle dysfunction. It occurs in children and adults and there is no sex preference. There is often pain on eye movement and pain on retrodisplacement of the globe. Diffuse nonspecific orbital inflammation anteriorly in the orbit may be associated with uveitis, papillitis, and exudative retinal detachment. Posterior orbital inflammation may be associated with early optic neuropathy.“’ In our experience, however, in acute diffuse nonspecific orbital inflammation the visual acuity, color vision and visual fields are usually initially within normal limits. B-scan ultrasonography often shows inflammatory mottling with moderate sound absorption in is the involved fat pad. 8 Low acoustic reflectivity noted on quantitative A-scan.’ Other ultrasonographic findings include a lucency noted in Tenon’s space secondary to edema fluid and a widened appearance of the optic nerve when fluid enters the nerve sheath, producing the so-called “T sign.“‘5.2”

NONSPECIFIC

Extraocular

ORBITAL

muscle

INFLAMMATORY

enlargement

95

SYNDROMES

may or may not be

present. Computed tomography shows an irregularly circumscribed enhancing mass which may obliterate normal orbital structures. Uveal scleral thickening and enhancement may also be present.‘” If the process is adjacent to an extraocular muscle or apical in location. one or more of the extraocular muscles on computerized tomography.“” may be enlarged Bony involvement or destruction is exceedingly rare,H.2%i.’ From a clinical viewpoint, orbital cellulitis is USUally considered in the differential diagnosis of acute nonspecific orbital inflammation. However, in orbital cellulitis, there is usually a concurrent sinusitis which can be ruled out by normal sinus x-ray films, or the absence of sinus involvement on computrrized tomography. Also the absence oflymphocytosis and lack of fever in these patients mitigate against orbital cellulitis. Other considerations include a ruptured dermoid cyst or the abrupt formation of chocolate cysts with an orbital lymphangioma. A ruptured dermoid, however. is more likely to be superotemporally in the orbit and x-rays or computerized tomography may disclose a bony defect in the orbit adjacent to the dermoid sequestration.“’ The history as well as old photographs may also be helpful. Patients with lymphangiomas often have a history of intermittent ecchymoses of the conjuctiva or lids. Pain is often severe but inflammation is not usually prominent. The ultrasonogram and CT scan usually will show a partially cystic mass. In children. rhabdomyosarcomas, metastatic neuroblastoma, or leukemic infiltration of the orbit may simulate nonspecific orbital inflammation. Hypocycloidal polyotomography may show bony involvement in the above conditions. Abnormal blood studies and other systemic signs can alert the clinician toward a diagnosis of one of these serious problems. Case Presentation: A 41-year-old woman was referred with a three-week history of painful left proptosis and total vision loss (Fig. 1). She had a past history of presumed left optic neuritis treated with oral prednisone. Examination revealed a normally functioning right eye and a proptosed, immobile left eye with an amaurotic pupil and associated erythema of the eyelids. She had a 3 mm left proptosis and pain in retrodisplacement of the left globe. Ultrasonography (Fig. 2) and computerized tomography (Fig. 3) showed a diffuse posterior left orbital mass. Fine needle aspiration biopsy rev,ealed nonspecific inflammatory cells including plasmaand occasional neucytes, lymphocytes, histiocytes trophils (Fig. 4). Oral prednisone, 80 mg per day..

Fi,?. 2. B-scan ultrasonogram posterior left orbital ity (arrows).

showing a diffuse irregular mass with x’cry low internal reflectiv-

Fig. 3. DiKusr superior post&or flammatory mass larrows).

orbital

nonspecific

in-

tapered over a period of one month caused a resolution of the mass with elimination of the proptosis and full recovery of extraocular motility. The total vision loss was permanent and the left optic disc became atrophic. She has not had a recurrence in four years. B. CHRONIC

FORM

The chronic form of nonspecific orbital inflammation may be a sequelae of acute recurrent orbital inflammation or may present insidiously as a subacute process. It is one of the most difficult orbital problems to diagnose and treat, and it is often complicated by permanent extraocular muscle dysfunction and visual loss. This form is characterized by the formation of dense collagenous connective tissue, frequently containing hyaline.’ The process slowly mats together all of the orbital structures, decreasing their function as it progresses; in the end stage the orbit is fixed and firm and the eye is often blind and immobile. The chronic form of nonspecific orbital inflam-

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KENNERDELL

AND DRESNER

Fig. 4. Cell block

made from fine needle aspiration biopsy shol Ming diKuse nonspecific inflamma .tory cells including plasmacytes, lyn nphocytes, histiocytes neutrophils and eosinophils.

mation differs from the acute form by often having a less dramatic response to corticosteroids. Radiation therapy may be palliative for some steroid resistant cases;“*23~25~34 however, when the sclerosis of the orbit is severe, neither steroids or radiation will be curative. In a discussion of the differential diagnosis of nonspecific orbital inflammation one unusual type of orbital inflammation, with vasculitis as a permanent feature, deserves mention. It is a rare and poorly understood condition which often presents in a fashion similar to that of subacute or chronic nonspecific orbital inflammation.lA Blepharoptosis, edema of the upper eyelid with various amounts of chemosis; proptosis, conjunctival injection, and discomfort are presentations. The cellular response is a diffuse polymorphic infiltration with eosinophils as the most prominent component. Small arteries and arterioles are cuffed by the inflammatory infiltrates. This picture has been likened to an acute hypersensitivity type of vasculitis, with fibrinoid necrosis of the vessel walls as seen with an Arthus reaction.‘* In our experience this type of orbital inflammation responds poorly to steroids. Radiotherapy has been reported by Henderson” to have limited success. We have recently treated two such patients with systemic Cytoxan with encouraging results, which will be the subject of another report. The systemic vasculitis of Wegener’s granulomatosis may affect the orbit and mimic the nonspecific orbital inflammatory syndromes. Usually, however, the condition is bilateral and concomitant sinus disof pulease is present. 36.37Other signs or symptoms monary or renal disease will usually help establish the diagnosis. Orbital lymphoid lesions including reactive lymphoid hyperplasia, atypical lymphoid hyperplasia and lymphomas may at times be considered in

the differential diagnosis of chronic or subacute nonspecific orbital inflammation. A palpable mass, displacement of the globe and various degrees of blepharoptosis are usually presenting signs. These conditions differ from nonspecific orbital inflammation in that pain, erythema, and extraocular muscle dysfunction are not commonly associated. These tumors have a predilection for the superior orbit.26.27 On computerized tomography, they appear to mold themselves in a putty-like fashion along fascial planes, the globe or the orbital bones. As with nonspecific orbital inflammation, no bony erosion or destruction is seen.45 These lesions may have a limited response to steroids, which can tend to cloud the clinical diagnosis. Biopsy is usually necessary to establish the diagnosis. Another area of concern to the clinician is the appearance of a nonspecific orbital inflammatorylike syndrome in a patient with a history of a carcinoma. Rarely, metastatic orbital carcinoma will present with pain as its predominant symptom. Breast, lung, and kidney carcinomas have a propensity for early metastasis and ocular symptoms before

Fig. 5. Left lid erythema and ptosis and hypotropia ciated with chronic superior left orbital sclerosing specific inflammation.

assonon-

NONSPECIFIC

ORBITAL

INFLAMMATORY

97

SYNDROMES

Fig. 6. Dense nonspecific inflammatory mass filling left superior orbit and molding the posterior superior left globe.

the primary lesion is diagnosed.‘” They also may have an initial response to steroids. We think that orbital lesions in patients with a history of carcinoma or with variable responses to treatment should undergo biopsy. The tine needle aspiration biopsy technique may be used to diagnose either the acute or chronic form ofnonspecific orbital inflammation.” Ifthese biopsy results are equivocal or insufficient for diagnosis, biopsy by open techniques can be done. We do incisional biopsies for lid and anterior orbital lesions and fine needle aspiration biopsy for deep orbital lesions. Case Presentation: A 36-year-old female complained of a ten-month history of left orbital pain, ptosis and diplopia. Two months prior to consultation, a left frontal sinus osteoma thought to be the cause of her orbital disorder had been removed. Postoperatively she complained of increased pain, swelling and diplopia. A course of antibiotics for suspected osteomyelitis did not improve the condition. On examination, the patient’s acuity was 201 25 + 2 in the right eye and 20/40 - 2 in the left eye. Her color vision and visual fields were normal. No afferent pupillary defects were noted. Extraocular motility examination revealed decreased upgaze, abduction and adduction of the left eye. Hertel measurements were 18 mm in the right eye and 21 mm in the left eye with a base of 105 mm. There was increased resistance to retrodisplacement of the left globe. The Ieft upper lid was edematous and erythematous (Fig. 5). Examination of the anterior segments and posterior poles was normal. Computerized tomography showed an enhancing soft tissue mass in the superior orbit (Fig. 6). A fine

needle aspiration biopsy revealed groups of well differentiated epithelial-like cells. It was thought that this might represent a meningioma. An open biopsy was therefore performed for a definitive diagnosis. Th e pathologic diagnosis was a sclerosing nonspecific inflammation (Fig. 7). Oral corticosteroids produced resolution of the pain, although the ptosis and extraocular motility dysfunction did not improve. The steroids were tapered over four months because chemical diabetes was discovered on higher doses. Four months after tapering of her steroids, the patient complained of increasing pain in the left orbit. On examination her acuity was 20130 in the right eye and 20/200 in the left eye. Her left color vision was diminished. A pericentral scotoma was evident on left visual field testing. A + 2 left afferent pupillary defect was noted. Her extraocular motility revealed increased restriction of movement on the left. Funduscopy showed mild pallor and congestion of the left optic disc. Computerized tomography showed a dense mass, now involving the entire left orbit. She was treated with 3000 rads of supervoltage radiation to the left orbit, which produced resolution of the pain and improvement of her acuity to 20/70. The ptosis and extraocular motility have remained unchanged. The dense inflammatory mass was unchanged on computerized tomography.

III. Localized Nonspecific Orbital Inflammation A. MYOSITIS

Orbital myositis is a subtype of nonspecific orbital inflammation in which one or more of the extraocular muscles are primarily infiltrated by an inflammatory process. It may be acute, subacute or

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DRESNER

Fig. 7. Biopsy of sclerosing nonspecific left superior orbital inflammation.

recurrent. In the acute form, the patient may present with pain, diplopia, proptosis, blepharoptosis, conjunctival chemosis and injection over the involved extraocular muscle(s). In acute myositis the extraocular muscle limitation is often in the field of action of the affected muscle(s).” In subacute or recurrent myositis the limitation may be indistinguishable from that seen in dysthyroid orbitopathy; restriction of gaze to the opposite field of action of the involved muscle secondary to chronic fibrosis. Earlier reports have characterized orbital myositis as a unilateral process involving a single extraocular muscle.“S” Recent studies, however, have shown that myositis often affects more than one extraocular muscle and is frequently bilateral.“,‘” Also, one orbit may be involved primarily and later a recurrence may arise on the contralateral side. The enlargement of the extraocular muscle may be easily displayed on ultrasonography or more definitively shown on computerized tomography. Trokel and Jakobiec ‘” have stated that the typical CT scan finding in orbital myositis is enlargement of the extraocular muscle, which extends anteriorly to involve the inserting tendon. This sign may or may not be present. Its presence may be a good radiologic indicator of orbital myositis; however, the absence of this sign does not rule out the diagnosis. The differential diagnosis of orbital myositis is basically the differential diagnosis of enlarged extraocular muscles which includes dysthyroid myopathy, the enlargement of the extraocular muscles as seen with carotid cavernous fistulas, cavernous sinus dural arteriovenous malformations, and metastatic or infiltrative neoplasia to the extraocular muscles. Dysthyroid myopathy is the most common cause of enlarged extraocular muscles. Involvement is almost always bilateral and asymmetric.*’ The

enlargement is usually fusiform and tapers at its insertion to the globe. Confusion of this entity with orbital myositis is only problematic in patients with euthyroid Graves’ disease. Pain, however, is only rarely a feature reported in dysthyroid myopathy.” Carotid cavernous fistulas and cavernous sinus dural arteriovenous malformations cause enlargement of the extraocular muscles, proptosis, and congestion, and can produce orbital discomfort. However, the constellation of an enlarged superior ophthalmic vein seen ultrasonographically or on computerized tomography along with fullness or dilatation of the ipsilateral cavernous sinus on computerized tomography are highly suggestive of these conditions and should not lead to confusion with orbital myositis. ’ I’ A neoplasm may directly invade or metastasize to the orbit and cause extraocular muscle enlargement.” Often, however, the enlarged extraocular muscle is adjacent to an infiltrative orbital mass? Devine and Anderson”’ have described

Fig. 8. Right medial striction of adduction

orbital myositis with of the right eye.

marked

re-

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ORBITAL

INFLAMMATORY

99

SYNDROMES

a case of a metastatic small cell tumor which mimicked orbital myositis. In this case and others, focal contrast enhancement, nodularity and irregular enlargement of the extraocular muscle may be more characteristic of a neoplasm and help distinguish it from orbital myositis.“’ Atypical cases of myositis or cases unresponsive to steroids should raise the clinician’s suspicion of neoplasia and fine needle aspiration biopsy or open biopsy should be considered. Case Presentation: An l&year-old woman complained of a 12-day history of a right inflamed lid swelling and orbital pain. The pain increased on extraocular movement. There was no past history of upper respiratory infection or concurrent medical illnesses. Her past ocular history included a “vertical muscle imbalance” since childhood. which was corrected with spectacles and prisms. Ocular examination disclosed a vision of 20/20 bilaterally. No a&rent pupillary defects were noted. Her extraocular motility revealed a marked restriction of adduction and slight restriction of abduction of the right eye (Fig. 8). Hertel measurements were 20 mm in the right eye, and 17 mm in the left eye, with a base of 96 mm. Mild right lid edema was present and the conjunctiva was injected over the right medial rectus insertion. Anterior chambers and fundi were normal. A B-scan indicated enlargement of the right medial rectus. A CT scan confirmed the presence of an enlarged right medial rectus with tendinous involvement of the inflammatory process (Fig. 9). The patient was started on 80 mg of prednisone four times per day, which produced resolution of her pain within 24 hours (Fig. IO). She was tapered of1 steroids over a one-month period and has remained asymptomatic and without extraocular motility dysfunction fr,r two years. B. DACRYOADENITIS Acute dacryoadenitis of nonspecific inflammatory origin usually presents with tenderness in the upper outer quadrant of the orbit in the region of the lacrimal gland. There may be mild erythema of the skin or mild conjunctival chemosis and an S-shaped appearance of the upper lid. The visual function and extraocular motility are usually normal. Ultrasonography reveals a mass in the region of the lacrimal fossa with homogenous internal acoustic characteristics. Computerized tomography characteristically shows an enhancing mass in the same region often with edema extending into tenons space seen on coronal views.“” These patients are usually afebrile and no lymphocytosis is found on laboratory evaluation. Viral dacryoadenitis may present in a similar fashion. It may indeed be secondary to mumps,

k’iLf. 9. B-scan showing myositis (arrows).

enlarged

right medial

Fig. 10. CT scan showing right medial involving muscle and tendon (arrows).

rectus with

rectus

myositis

mononucleosis, or herpes zoster. It differs from nonspecific orbital inflammation in that it is associated adenopathy and peripheral blood Iymphocytosis. Bacterial dacryoadenitis is not a common clinical problem.” Fever and leukocytosis are again features, often with associated purulence. Ruptured dermoids can also present with pain and erythema in the lacrimal region. Plain x-ray films or computerized tomography will disclose the characteristic adjacent bony defects associated with these lesions to help distinguish them from nonspecific orbital inflammation.” Chronic dacryoadenitis can follow acute dacryoadenitis or present as an indolent slightly tender lacrimal gland mass. Differentiating this condition from malignant tumors arising from the lacrimal gland is exceedingly important, as pain is often a concomitant symptom in patients with lacrimal carcinomas. With the latter lesions, bony erosion or destruction are often noted; however, neoplastic le-

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sions may enlarge rapidly so that radiographs in the early stages are norma1.44 Thus, chronic dacryoadenitis, in the absence of a clear cut history of a past acute dacryoadenitis, is a diagnosis of exclusion. Fine needle aspiration biopsy or incisional biopsies are required to rule out neoplasia. Case Presentation: A 41-year-old male complained of a four week history of swelling of his left upper lid associated with mild pain. He had no complaints of decreased vision or diplopia. His past medical history was noncontributory. On examination, his acuity was 20125 + 2 in the right eye and 20/20 in the left. The patient’s color vision was normal, and no pupillary abnormalities were noted. His extraocular motility revealed minimal limitation of right superior and lateral gaze. Hertel measurements were 18 mm in both eyes with a base of 102 mm. There was three mm of right ptosis and + 1 lid edema, but no masses were palpated (Fig. 11). The slip-lamp examination and funduscopy were normal. The patient was followed and three weeks later he developed a mild right proptosis. A CT scan showed an enhancing enlarged lacrimal gland with dacryoadenitis (Fig. 12). A fine needle aspiration biopsy harvested a cellular pattern of nonspecific orbital inflammation. Sedimentation rate was 22 mm per hour. Antinuclear antibody titers (ANA) and immune surveys were negative. He was treated with 80 mg of prednisone per day tapered over a one month period with prompt resolution of the lesion. No recurrence has occurred to date. C. PERISCLERITIS A peculiar type of nonspecific orbital inflammation which tends to occur around the posterior portions of the globe has been termed periscleritis.3” It is also known as sclerotendonitis, although we have

KENNERDELL

AND DRESNER

Fig. Il. Mild lateral superior orbital swelling and ptosis associated with acute dacyroadenitis.

not been impressed that all cases have tendinous involvement. This entity is a nonspecific orbital inflammation unassociated with systemic vasculitis as seen in polyarteritis nodosa, Wegener’s granulomatosis, or rheumatoid arthritis.lg It is marked by pain and often with blurred vision and can have associated iritis and choroiditis. There is often conjunctival edema and swelling of the lids. The extraocular motility is usually unaffected. Ultrasonography and computerized tomography show thickening of the scleral uveal rim. This is often associated with edema fluid extending into Tenon’s space yielding a lucent area or so-called “ring sign.“3g The differential diagnosis includes herpes zoster, episcleritis, and posterior scleritis. Adjacent lid lesions should be looked for to rule out herpes zoster. Episcleritis is a superficial inflammation which is anterior on the globe and unassociated with severe pain. Other signs and symptoms of the autoimmune diseases associated with posterior scleritis may be present to differentiate these entities from nonspecific orbital inflammation. Patients with posterior scleritis typically develop retinal detachments and

Fig. I,?. Axial (left) and Coronal (right) CT scans showing enlarged right lacrimal gland with I dacryoadenitis.

NONSPECIFIC

ORBITAL

INFLAMMATORY

SYNDROMES

fundus masses that simulate intraocular tumors. In contrast, patients with periscleritis who have less direct involvement of the sclera are likely to exhibit lesser disturbances of the posterior pole, such as mild neural retinal edema and retinal striae, although periscleritis and posterior scleritis may very well overlap. i’i A 39-year-old female comCase Presentation: plained of a four-day history of severe left orbital pain, nausea. and vomiting. She had no complaints of diplopia or decreased visual acuity. Her past medical history was noncontributory. On examination, her visual acuity was 20120 in both eyes. Her visual fields and color vision were normal. There was no proptosis; however, the left globe

was

tender

to retropulsion.

Her

Fig. 13. Mild left chemosis with periscleritis.

and lid swelling

associated

extraocular

showed minimal limitation of abduction of the left eye. There was chemosis and injection over the lateral aspect of the left globe (Fig. 13). The anterior chambers were normal, but funduscopy showed slight engorgement of the retinal veins on the left. A B-scan ultrasonogram showed fluid in Tenon’s space and periscleral inflammation. The CT scan revealed no periscleral enhancement laterally on the posterior lateral left globe (Fig. 14). She was admitted and treated with 80 mg prednisone per day and her symptoms resolved within 24 hours. Two recurrences were reported over the next year which also responded to systemic prednisone.

motility

Fig. 14. Axial CT scan showing periscleritic inflammation in thp posterior lateral aspect of the kft globe.

D. PERINEURITIS

Perineuritis, a particularly unusual type of nonspecific orbital inflammation, has been reported.” This entity may simulate optic neuritis, presenting with orbital pain, pain on extraocular motility, decreased visual acuity and disc edema. However, in distinction from optic neuritis, pain is present on retrodisplacement of the globe and mild proptosis is usually present. Ultrasonography and computerized tomography show an enlargement and enhancement of the optic nerve sheath with a diffuse, fuzzy, inflammatory cuff. A 55-year-old female comCase Presentation: plained of a one-year history of intermittent left orbital pain, decreased left visual acuity, and decreased color vision. She had been diagnosed as having optic neuritis and had improved with ACTH, but the condition had returned when the ACTH was stopped. Her past medical history was noncontributory. On examination, the patient’s acuity was 20120 in the right eye and 20/50 in the left. Her color vision was 7 of 7 in the right eye, and 0 of 7 in the left eye with the Ishihara plates. Her right visual field was normal. A cecocentral scotoma and a +2 afferent

Fig. 15. Goldmann

visual fields showing a left cecocentral scotoma associated with left orbital perineuritis.

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Fip. 16. Left: Axial CT scan showing ragged swelling of the left optic nerve in the posterior left orbit. Right: Coronal CT scan showing ragged enlargement of the left optic nerve in the posterior orbit associated with optic perineuritis.

defect were present on the left (Fig. 15). Her extraocular motility was full. Hertel measurements were 17 mm on the right, and 21 mm on the left with a base of 104 mm. There was pain on retrodisplacement of the left globe. The anterior segments were normal. Funduscopy revealed left temporal pallor. The right disc was normal. Computerized tomography showed a ragged enlargement of the optic nerve in the posterior orbit. She was started on 80 mg of prednisone, which produced prompt recovery of her visual fields and acuity. A repeat scan three months later showed resolution of the posterior orbital optic nerve swelling (Fig. 16). The laboratory evaluation including ANA, sedimentation rate, and immune survey were noncontributory.

IV. Laboratory

Evaluation

The laboratory evaluation of patients with nonspecific orbital inflammation is usually not revealing. Eosinophilia and elevated sedimentation rates have been reported in children with these syndromes;33 however, the clinical diagnosis of a nonspecific orbital inflammatory syndrome still rests mainly on the history, examination, and findings on ultrasound and computerized tomography. A normal complete blood count may help to exclude a bacterial infection. Immunologic studies such as ANA, rheumatoid factor and lupus erythematosus cell prep may be helpful in identifying patients with associated systemic diseases.

IV. Treatment The mainstay of treatment in all the orbital inflammatory syndromes is oral roids. The initial dosage recommended to 1.5 mg per kilogram per day of oral

nonspecific corticosteis from 1.0 prednisone.

Tapering is done on an individual basis. Recurrent orbital inflammation may require a more gradual tapering or a low maintenance dose to suppress the inflammation. Chronic nonspecific orbital inflammation may become resistant to steroids. Patients who are resistant to steroids or who have contraindications to steroid therapy may respond to supervoltage radiation. One thousand to two thousand rads in ten fractions are now recommended over a lo-15 day course delivered by a lateral port.‘“.“’ Recent reports on the treatment of nonspecific orbital inflammation with chlorambucil may stimulate further interest in treating recalcitrant cases with this or other immunosuppressive agents.g We feel that, except for diagnostic purposes, that there is no role for surgery in the nonspecific orbital inflammatory syndromes.

V. Summary and Conclusions The nonspecific orbital inflammatory syndromes are a heterogeneous group of conditions affecting various orbital tissues. The inflammation may be diffuse or localized to a specific target organ. Common to all of these conditions in the acute form are pain, inflammatory signs, a nonspecific infiltrate of inflammatory cells, and a dramatic response to oral corticosteroids. Chronic and recurrent orbital inflammation may require maintenance steroid therapy or radiotherapy. The etiology of the nonspecific inflammatory syndromes remains unknown. The evidence that these conditions are secondary to orbital immune dysfunction is still speculative at this time. We prefer to discard the term “pseudotumor” in discussing orbital inflammation because of its generic connotation and past confusion in the literature. Instead we

NONSPECIFIC

ORBITAL

INFLAMMATORY

describe the nonspecific orbital inflammatory syndromes as either diffuse nonspecific orbital inflammation (acute or chronic) or as localized to a specific target organ such as myositis, dacryoadenitis, periscleritis, and perineuritis.

References ‘f~~l,jS. Srgall HI). rt al: C:omputrd tomography 01 carotid-cavernous listula. dvz J nburorndwl 4:131-136. 1983 AshtonN. .\forganG: IXsrrctc carcinomatous metastasis in the cxrraocular musclrs. Ur ./ Ophthalmol Pi: 112. 197-l Birch-Hirschfcld A: Zur IXagnostik und I’atholoyic drr Orhitalturnown. Ber Utsch Ophthal Ce.r 2: 127-l 3.5. 1905 Blodi I*‘(:: Orbital inllammation. Orbrl 1. l-19. 1982 Blodi E‘C:.Gass,JI),\l: Inllamm;~tot-v pscudotumor of thr orhit. Hr ,/ Ophthn/mo/ JL’.7WU, 1968 Bullen CL. Younqe BR: C:hronic orbital m)ositis. .1rch Ophthnl-

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103

SYNDROMES

mol H7:603-619. 1979 28. I.udwi~ I. ‘l‘omsak RI,: Acute rccurrcnt orbital mysitis. ,/ C,‘[in RPuro-ophthnlmol 5.1L47. 1983 FA: Idiopathic inflammatorv orbital 29. Xlottow I.S. ,Jakohirc psucdotumor in childhood. I. Clinical Charartcrist’ics. Arch Ophtholmol 1Hi:I+l(&l417. 1978 30. .\lottow-Lippa I..Jakohicc F.4, Smith Xl: Idiopathic inllammatory orbital psrudotumor in childhood. II. Results ofdiagnostic tests and hiopsirs. Ophthalmolo,~r 8&565S57~. 1981 31. Purcrll J,J. ‘l‘aulhcc W.4: Orhitat myositis alirr upper rcspiratory tract mfcction. .irch Ophthnlmol S&:437-438, I981 .32. Rolrtman ,J, Nugcnt R: ‘fhc classification and manaxcment of awtr orhit pscudotumors. Ophthalmolu,~~ N: 10~(~1018. 1982 33 Rush ,J-1. Kcnncrdclt ,JS. 1)onin ,JF: Acute prrisclrritis - .-\ \arianr of idiopathic orhital inllammatitrn. Orbit /:221-230. I!182 ?I+.Scrqott KC:. Glascr ,JS. C:haryulu K: Radiutherap)- for idiolxthir inllammatory ~~rhitat pscudotumor. :lrr.h Ophthalmol !]!I. W&856. I98 I .35. Slavin ML, Glasrr JS: Idiopathic orbital myositis. A rrport 01 six casts. .-1rch Ophthalmol If/U: I26lLl465, 1982 36. Spalton I),J. Graham ELl. Page NGR. Sanders 5111: Ocular changrs in limited Iixms of LVrqener’s granulomatosis. Hr J Ophthalmol

163, IO. IXvinc RI). Anderson

maqnrradin~ as orbital mvositis. Ophthalmic .Suq 15:.k83-487, I982 Il. I)onaldson SS. ~Icl)ouqill IR. Eqhcrt PR. ct al: ‘l‘rcatmcnt of orhiral psurdotumor (i&pachic orbital inflammarion) hy radariun therapy. Int
RI.: S md II c r II cdrclnoma .I

I!)74

II. I .j, Iti. 17.

fij:5>3S563.

1981

:37. Str.latsma BR: Ocular manifestations ofLt’eqcnrr’s g-ranulomatows. .-ltn ,J Uphthalmol N:789-799, 1957 38. Svanr S: Prrawtc \pontancwus strrptococcal myositis. .-\ report on ‘2fatal casts with rrviw ofhtt.rature. .-I& ChirScnnd 137: l5,5I971

39. ‘l‘rokrl SI.. Hilal SK: Suhmillimrtcr rrsolution CYI‘scannin? of orbital diwases. Ophthnlmologr H7:41ZP417. 1980 40. ‘I‘rokel SI,. Hilal SK: R tcqnition ‘. and dillercntial diagnosis 01 cnlarqd rxrraocular muscles in computrd tomography. .4m J Ophthnlmol Hi:,N:i~j 12. 1979 11. ‘frokrt .SI..,Jakohirc F.4: C’lurrtlatiun ofCT scanning and pathoIoqic l?aturr.s 01 ~~phthalmir Graves disease. ~~fd~~hnlmolo,~~ fYH:jY-5tA. I98 I 12. \!‘alton E\V: PscudoLumour of the orhit and potyarteritis nodwa. ,/ C’lin Path 12:419-126, 1959 k.3. \Vcinstrin GS. 1)rcsner SC:. Slamo\its’II., Krnnrrdcll~JS: Acute and suhacutv orbital myositis. .4m,/ Ophthalmul9~~2(l9~21 i. 1983 I&. \VriThr,JL. hlc\vart \\‘B. Krohrl GB: Clinical prrsrnration and manqrmcn~ of Iacrimal gland tumrmrs. Hr,/ Ophtha/mu/6’.7~60~L 606. 19711 45 Yc~~,JH.,Jak
18. I!). ,Jakohirc F.4. ,Joncs IS: Orbital inllammations. in: Duane ‘1‘1) Irdi: Clinr~al Ophthalmolr~~ps 1’012. Hayrrstown. Harprr and Row, 1975, Chaptrr 3.5 20. ,!akohiec F.4. hlcI.ran I. 1sant RI,: C:linicopathologic ctraractrrIstics of orhital Iymphoid hvprrplasia. Ophthalmolq~v Ro‘:948& 966,

Outline

I. ‘I‘erminolog~

~1. “Pseudotumors”

1979

21. Jrllinrk I:H: The 01 hital psrudotumour syndrome and its differcntiation from cndocrinr cxophthalmos. Brnin 92;35-38. 1969 “2. Krnncrdclt ,JS, Drkkcr .I,,Johnson BI., Duhois P,J: Fine needle aspiration biopsy. Its usr in orhital tumors. Arch Ophthalmol 97:1315-1317. 1979 “3. Kcnnerdrll,JS,,J~)h~~so~~ BL. 1)cutsch 11: Radiation trcarmcnt of orbital I)mphoid hypcrplasia. Ophthalmolr~,~~ &S:94%947. 1979 2-L Krrmy AH. Halfnrr ~JN: Llltrasonic evidence oE inflammator\ thickrning and fluid collection with thr rrtrohulhar fascia: ‘l‘hc I’ sign. .4nn Ophthalmol 93:1.557-1563, 1977 25. Kim REV,Roth RE: Radiorhrrap) oforhital psrudorum~lr. Radi26. Knowles I)hl, Jakohirr FA: Ocular adnrxal Iymphoid neoplasms: CZlinical. histopathologic. electron microscopic and immunologic characteristics. Hum f’athol 13:1#&162, 1982 27. Knowlrs L)LI, ~Jakohicc FA. Halpcr ,JP: Immunolqic charactrrizntion ofocular adncxal lymphoid neopl.lqms. .4mJ Ophthal-

II.

III.

IV. i’. VI.

B. Classification C. Etiology Difluse nonspecific orbital inflammation :I. :icute form B. Chronic form Localized nonspecific orbital inflammation :\. Myositis B. Dacryoadenitis Ct. Periscleritis D. Perineuritis Laboratory evaluation Treatment Summar?

Rrprint rrquests should he addressed to John .‘,:.Li. ~ ?,yr and Ear Hospital. 230 Lothrop Strcrt. 15213.

S. Kcnnerdell. Pittsburgh. P.4