Journal of Neuro-Ophthalmology, March 1983, Volume 3, Issue 1

Acute recurrent orbital myositis.

An acute relapsing orbital inflammatory disease predominantly affecting the extraocular muscles was seen in two patients and is reported here as acute recurrent orbital myositis. The association of this disorder with other systemic diseases such as asthma, sinusitis, upper respiratory infection, Crohn's disease, and serum sickness is discussed. The similarities to other forms of acute orbital inflammatory disease such as orbital pseudotumor are noted, and a possible underlying immunologic mechanism is suggested.

]. Clin. Neuro-ophthalmoJ. 3: 41-47, 1983. Acute Recurrent Orbital Myositis IRENE LUDWIG, MD. ROBERT L. TOMSAK, MD., PhD. Abstract An acute relapsing orbital inflammatory disease pre­dominantly affecting the extraocular muscles was seen in two patients and is reported here as acute recurrent orbital myositis. The association of this disorder with other systemic diseases such as asthma, sinusitis, up­per respiratory infection, Crohn's disease, and serum sickness is discussed. The similarities to other forms of acute orbital inflammatory disease such as orbital pseudotumor are noted, and a possible underlying im­munologic mechanism is suggested. Introduction Acute orbital inflammatory disease is often de­scribed under the heading of inflammatory orbital pseudotumor. Included in the numerous case re­ports are a lesser number of cases of acute recurrent orbital inflammatory disease with extraocular mus­cle involvement as a predominant feature.'-~()·~~. ~9­: lI. ~o We describe two patients with a recurrent form of orbital myositis that was associated with exacerbations of presumed allergic respiratory dis­orders. Case Reports Case 1 A 17-year-old white woman presented in Sep­tember 1980 with a 3-week history of right frontal headache, diplopia, nausea, and vomiting. Propto­sis, hyperemia, and lid swelling of the right eye were reportedly present. She was treated by an­other physician with prednisone, 60 mg/day, which produced a rapid improvement of the head­ache. She was referred for further evaluation of persistent diplopia and inflammatory orbital signs. Her past history was significant for a similar epi­sode involving the left orbit 3 years earlier, which resulted in persistent diplopia in downgaze. On examination on September 3, 1980, visual acuities were 20/15 in both eyes There was de­creased adduction and elevation of the right eye and a 40-prism diopter exotropia. Hertel exo­phthalmometry measurements were 15 mm on the From the DepMtment of Ophthalmology. Cleveland Clinic. Cleveland. Ohio March 1983 right and 14 mm on the left. A small, resolving subconjunctival hemorrhage was present in the right eye. The remainder of the neuro-ophthalmic exam including visual fields was completely nor­mal. Ophthalmic ultrasonography was performed and showed a markedly enlarged right medial rec­tus (Fig. I). The prednisone therapy was tapered over 1 week. Twenty-seven days later, she returned with moderate lid edema, ptosis, conjunctival chemosis, and hyperemia of the left eye. There was pain in upgaze, a 25% limitation of abduction, and a 75% limitation of adduction. There was now a border­line measurable left-sided proptosis of 18 mm versus 16 mm on the right, and ultrasound showed enlargement of the medial and lateral recti of the left eye (Fig. 2). She was admitted for a diagnostic work-up, which included neurology, rheumatology, and en- Figure 1. Orbit.,1 sonogr.lm showing enlMgemenl of right me­dial rectus muscle (case I). 41 Orbital Myositis Figure 2. Orbital sonogram showing enlargement of left medial and left lateral rectus muscles (case 1). Figure 3. High-res<,lution orbit.,1 CT scan showing enlargement of b,)th medi•• 1 redi .lnd enlargement of the left lateral redus. (I\:<)te: The right orbit IS sh<nvn on the left: rJ'e I.) docrinology consults, lumbar puncture, and thy­roid function studies. In addition, an extensive battery of immunologic tests were performed, in­cluding C1q binding, complement levels, and an­tinuclear factor, all of which were normal. Orbital sector CT scan showed thickening of both medial recti, the right inferior rectus, and the left lateral rectus (Fig. 3) along with mucosal thick­ening of the maxillary and right ethmoid sinuses (Fig. 4). The patient was discharged and improved grad­ually. Two weeks after discharge, she had resolu­tion of the swelling and redness, but now had a marked esotropia due to an inability to abduct the left eye. Exophthalmometry was now symmetrical, with readings of 17 mm. Three weeks later, the improvement was marked, and the rotations were full in all directions of gaze. In December 1980, the CT scan showed less thickening of the muscles (Fig. 5) and resolution of the sinus changes (Fig. 6). In September 1981, left-sided headache, redness, and swelling of the left eye suddenly resumed. Immediate treatment with 50 mg/day of predni­sone orally resolved the symptoms within a day. The exam was normal 3 days later, except for mild residual pain in the left orbit on up- and right gaze. Summary. A teenage woman had four separate attacks of orbital myositis over a period of 4 years. Although no immunologic abnormalities were de­tected, one attack was associated with transient thickening of maxillary and ethmoid sinus mu­cosae. Journal of Clinical Neuro-ophthalmology Ludwig, Tomsak Figure 4. High-resolution CT scan demonstrating mucosal thickening in the maxillary sinuses (arrows); (case 1). Figure 5. CT scan showing disappearance of changes described in Fig. 3 (case 1). Case 2 A 56-year-old white man developed diplopia and pain on abduction of the right eye on October 3, 1981, which progressed to constant right orbital pain and right frontal headache. He saw his oph­thalmologist 5 days later, who found the vision and fields to be normal. Mild right ptosis and limitation of abduction and adduction of the right eye were noted. A CT scan confirmed swelling of the right lateral rectus muscle, polytomograms showed bi­lateral maxillary and ethmoidal haziness, and a cerebral arteriogram was normal. March 1983 The sedimentation rate was slightly elevated at 33 mm/hour. CSC, SMA-IS, thyroid function studies, VORL, and spinal tap were all normal. Immunologic testing was not done. He was placed on 60 mg prednisone daily and began to improve rapidly. When examined by us 3 weeks after the onset of symptoms, the external signs and diplopia were much reduced. His past history was fascinating; he had six other episodes of orbital inflammation with diplopia since 1959 and had kept exact dates. All episodes occurred during the cooler seasons; none were later than April. Each episode had been preceded 5-7 43 Orbit.ll MVL1sitio Figure 6. CT scan showing resolution of sinus mucosal changes described In Fig. -+ (case I) Figure 7. Top: Decreased abduction of right eye (case 2). Bottom: Narrowing of interpalpebral fissure on adduction of right eye. days by an upper respiratory infection. He also had severe allergic asthma, again since 1959, which often required steroids and always resolved in the summer months. The asthma attacks did not co­incide directly with the attacks of orbital inflam­mation, however. The patient also had a history of 44 chronic sinusitis for which he had undergone eth­moidectomy and nasal polypectomy in 1981. On examination by us, the acuities and fields were normal. There was a 14-prism-diopter esotro­pia in primary position, which worsened in right gaze (Fig. 7). Upgaze was also mildly limited. On left gaze, the right interpalpebral fissure narrowed (Fig. 7), consistent with a diagnosis of acquired orbital retraction syndrome, which is not infre- ')1 ,),) quently seen with orbital myositis.- ,-- Other than conjunctival hyperemia over the right lateral rectus insertion, the neuro-ophthalmic exam was normal. All lab studies, including Wes­tergren sedimentation rate, IgE, eosinophil count, and serum protein electrophoresis, were normal. However, the patient was still taking oral steroids when they were performed. Summary. A man with a history of allergic asthma and chronic sinusitis suffered seven epi­sodes of steroid-responsive orbital myositis over a 22-year period, which were all preceded by symp­toms of an upper respiratory tract infection. Discussion Acute recurrent orbital myositis commonly pre­sents with pain and headache on the involved side, diplopia, hyperemia of the conjunctiva and eyelids, and mild proptosis. Unilateral and bilateral cases have been described, but bilateral cases usually have several weeks separating the onset of symp­toms in either eye. The clinical course is markedly shortened by steroids, particularly when begun early, as in our first patient whose third attack was aborted. Most patients have no residual, and the ones who do Journal of Clinical Neuro-ophthalmology have permanent effects were usually biopsied or not treated.4. 7 Most patients have normal visual acuities, but visual loss as a result of associated optic neuritis, choroidal folds, or scleritis has been reported.2. 4. 7.10.11.16 Recurrences have been nota­bly seasonal in our patients, and this association has not been previously emphasized. In reported cases and in our patients, this dis­order is often seen in association with other ill­nesses, which are seemingly unrelated, but can be linked by their immunologic etiologies: 1. One of our patients had allergic asthma (case 2). 2. Two cases of almost identical syndromes fol­lowing upper respiratory infections were recently reported. I8 One had a proven streptococcal phar­yngitis, but neither had recurrent episodes. The authors postulated a poststreptococcal vasculitic process or a viral myositis. (Myositis of the lower extremities and back is known to occur in associ­ation with influenza and coxsackie viral infec­tions. 36-39 ) Mottow and Jakobiec also described an association with upper respiratory infection in six patien~s from their pediatric orbital pseudotumor series. ' 3. A clinically identical form of acute orbital myos­itis has been seen in association with Crohn's disease, with the onset of myositis coinciding with increased activity of the disease. l7 · 23-25 Of even greater interest is the observation in two patients of resolution of orbital inflammation following bowel resection. I7. 25 4. Allergic rhinitis and nonpurulent sinusitis have been described in association with pediatric orbital pseudotumor. 7.26 Although the etiology of this form of sinusitis is not fully understood, deposits of immune globulins and complement are found in the hypertrophied sinus mucosae in these pa­tients. 27 5. One case of dramatic and severe acute bilateral orbital inflammatory disease closely followed three injections of iron-dextran complex in a 25-year-old woman, accompanied by a clinical picture of serum sickness. After resolution of the inflammation, op­tic atrophy was present; bilateral orbital biopsies had been performed, however, and steroid therapy was delayed.4 6. A case of recurrent Tolosa-Hunt syndrome was reported in a 56-year-old woman, in whom flare­ups were accompanied by an elevation of the sed­imentation and a positive antinuclear factor. She had no other evidence of collagen vascular dis­ease. 28 The disorder classified as pediatric orbital pseu­dotumor is frequently clinically indistinguishable from cases of acute orbital myositis in adults.26 More recently, ophthalmologists treating acute or­bital myositis have stressed that the nature of the process is different from the typical adult pseudo­tumor, and some have suggested eliminating the March 1983 Ludwig. Tomsak term pseudotumor altogether.2. 12 The few cases of acute pediatric pseudotumor that have been biop­sied have shown a diffuse vasculitis, with poly­morphonuclear leukocytes and eosinophils sur­rounding small arteries and arterioles. Henderson initially termed this a type I pseudotumor,:J:l and noted its similarity to the Arthus reaction, which is type III, immune-complex-mediated hypersen­sitivity. 32 He now diagnoses this as orbital vascu­litis. 33 His type II pseudotumor is the more com­monly found chronic orbital inflammation seen in the older age groups and is clinically different from our two cases. Of particular interest is the patient with serum sickness,4 as biopsies showed an orbital vasculitis. Acute orbital inflammation with a his­tologic picture similar to orbital vasculitis has been experimentally produced by injecting bovine se­rum albumen into the retrobulbar space of previ­ously sensitized rabbits. 34 Histology showed more chronic inflammatory cells when biopsy was per­formed several weeks following the retrobulbar injections.:l5 Left untreated, the orbital inflamma­tion resolved and did not develop into chronic orbital pseudotumor. The need to redefine orbital pseudotumor into more precise divisions is apparent, and the use of orbital ultrasonography and CT scanning is allow­ing more specific diagnoses. This is also causing the literature on the subject to become even more confusing, because the same patient could easily fit into any of four or five different diagnoses, de­pending upon the doctor's preference and the par­ticular diagnostic tests used. The distinction between acute orbital myositis and other acute orbital pseudotumors may also prove to be unnecessary, as CT evidence of extra­ocular muscle involvement may vary at different times during the illness. It may also be arbitrary to separate pediatric from adult pseudotumor, as this type of acute orbital inflammatory disease does occur in adults, some of whom have long histories of recurrent episodes since youth. It would seem more appropriate to consider that the same immunologic processes may be occurring in all these patients and to direct attention to that rather than attempting classification by exact lo­calization of the process within the orbit or by arbitrary age limits. In our cases, blood tests were obtained only after steroids were begun, and this may account for the normal values. However, we recently examined another patient with probable orbital myositis who had a markedly elevated level of IgG and IgM immune complexes by cryoprecipitation. This pa­tient also had a strong seasonal trend to her epi­sodes and a long history of chronic sinusitis. In summary, patients with acute orbital inflam­matory disease, with or without myositis, deserve a thorough immunologic evaluation in addition to 45 Orbital Myositis computerized tomography and ultrasound exami­nations. Circulating immune complexes can be detected by total hemolytic complement (CHso) and Clg binding determinations as well as by testing blood for the presence of cryoglobulins. If biopsy mate­rial is obtained, it also should be studied for im­mune complex deposition. Inflammatory orbital pseudotumor has been a heterogeneous diagnosis for many years. Recent technological advances, which allow more careful dissection of underlying immunologic processes, promise to simplify the diagnosis and pathogenesis of related orbital inflammatory diseases. References 1. Jellinek, E.H.: The orbital pseudotumor syndrome and its differentiation from endocrine exophthal­mos. Brain 92: 35, 1969. 2. Heersink, B., Rodrigues, M.R., and Flanagan, J.e.: Inflammatory pseudotumor of the orbit. Ann. Oph­thalmol. 9(1): 17, 1977. 3. Wolter, J.R., Hey, I.E., and Schmidt, D.M.: Chronic orbital myositis. Its diagnostic difficulties and pa­thology. Am. f. Ophthalmol. 62: 292, 1966. 4. Easton, I.A, and Smith, W.T: Non-specific granu­loma of orbit (orbital pseudotumor). f. Pathol. Bac­teriol. 82: 345, 1961. 5. Coop, M.E.: Pseudotumor of the orbit. A clinical and pathological study of 47 cases. Br. f. Ophthalmol. 45: 513, 1961. 6. Chavis, R.M., Garner, A, and Wright, J.E.: Inflam­matory orbital pseudotumor. A clinicopathologic study. Arch. Ophthalmol. 96: 1817, 1978. 7. Mottow, L.S., and Jakobiec, F.A: Idiopathic inflam­matory orbital pseudotumor in childhood. Clinical characteristics. Arch. Ophthalmol. 96: 1410, 1978. 8. Smith, J.L., and Taxdal, D.5.R.: Painful ophthalmo­plegia. The Tolosa-Hunt syndrome. Am. f. Oph­thalmol. 61: 1466, 1966. 9. 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