Saudi Journal of (2012) 26, 449–451

Case Report

Unilateral , scleritis and exudative due to recurrent orbital pseudotumor

⇑ Imtiaz A. Chaudhry, MD, PhD, FACS a, ; Saif Al-Obaisi, MD b; Osama Al-Sheikh, MD c; Alberto Galvez, MD d

Abstract

Idiopathic orbital , also known as orbital pseudotumor, describes a spectrum of idiopathic, non-neoplastic, non-infec- tious, space-occupying orbital lesions without identifiable local or systemic cause. The condition occurs mainly in young adults who may present with acute pain, proptosis, chemosis and limitations of extraocular movements. Decreased vision due to infiltration and as a result of scleritis is less common sequel of orbital pseudotumor. Herein, we present a case of unilateral orbital pseudotumor in a young male who presented with chief complaints of painful decreased vision which was attrib- uted to optic neuritis and macular edema as a result of scleritis. Imaging studies were helpful in the establishment of the correct diagnosis and patient’s complaints improved with administration of systemic .

Keywords: Orbital pseudotumor, Scleritis, Optic neuritis, Macular edema, OCT, Treatment

Ó 2012 Saudi Ophthalmological Society, King Saud University. All rights reserved. http://dx.doi.org/10.1016/j.sjopt.2012.09.003

Introduction and pain with eye movements.3–5 Decreased vision due to optic nerve infiltration, and macular edema as a result After Graves’ and lymphoproliferative disorders, of scleritis is less common sequel of orbital pseudotumor.6,7 orbital pseudotumor, is the 3rd most common ophthalmo- Herein, we present a case of unilateral orbital pseudotumor logic disease of the which accounts for approximately in a young male who presented with chief complaints of pain- 8–11% of all the orbital processes. For example, among the ful decreased vision which was attributed to optic neuritis 1264 patients referred to Wills Eye Hospital for a suspected and macular edema as a result of scleritis. Imaging studies orbital mass, inflammatory lesion accounted for 11% of the were helpful in the establishment of correct diagnosis and pa- histopathologically proven cases.1The current concept of tient’s complaints improved with administration of systemic orbital pseudotumor defines it as an idiopathic inflammatory corticosteroids. process made up of pleomorphic inflammatory cellular re- sponse and a fibrovascular tissue reaction. Histopathological Case report analysis shows a spectrum of granulomatous inflammation, admixed with non-granulomatous inflammation and fibro- A 20-year-old male presented with a two week history of 2,3 sis. The ocular manifestations of orbital pseudotumor decreased vision and severe right-sided orbital pain with may include periorbital edema, erythema, proptosis, , eye movements. His past history was significant for having

Received 4 September 2012; received in revised form 25 September 2012; accepted 25 September 2012; available online 17 October 2012. a Houston Oculoplastics Associates, Memorial Herman Medical Plaza, Texas Medical Center, 6400 Fannin St., Suite 2220, Houston, TX 77030, USA b King Khaled Eye Specialist Hospital, Department of Ophthalmology, King Saud University, Riyadh, Saudi Arabia c Oculoplastic and Orbit Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia d Neuro-Ophthalmology, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia

⇑ Corresponding author. Tel.: +1 832 868 3938; fax: +1 713 790 1130. e-mail addresses: [email protected], [email protected] (I.A. Chaudhry).

Peer review under responsibility Access this article online: of Saudi Ophthalmological Society, www.saudiophthaljournal.com King Saud University Production and hosting by Elsevier www.sciencedirect.com 450 I.A. Chaudhry et al. minimal proptosis on the same side for which he had been response to a trial of systemic corticosteroids with full recov- diagnosed to have an orbital pseudotumor 4 months earlier ery of his vision.8 but no treatment was offered. On examination his VA was Evaluation and management of patients with orbital 20/25 in OD and 20/20 in OS, his intraocular pressure was pseudotumor are challenging and the importance of its inclu- 17 mmHg in OD and 16.5 mmHg in OS. There was no relative sion in the differential diagnosis of orbital disorders is afferent pupillary defect. There was 4–5 mm of right-sided necessary.1–5,7,9 At initial presentation, orbital infections, proptosis (Fig. 1A and B). On slit-lamp examination, there Grave’s ophthalmopathy and orbital pseudotumor can occa- was evidence of dilated scleral vessels on the right side, ante- sionally demonstrate overlapping clinical features. Orbital rior chamber was deep, there was no inflammation and his pseudotumor is often sub-classified on the basis of the ana- was clear. Fundus examination revealed evidence of exu- tomic target areas within the orbit.3 A 10-year retrospective dative retinal detachment at the macular area on the right review of 65 patients with orbital pseudotumors treated at side. Slit-lamp and fundus examinations on the left side were the Massachusetts Eye and Ear Infirmary revealed isolated normal. Right-sided ultrasonography (U/S, B-scan) revealed (n = 21), isolated myositis (n = 19), concurrent evidence of posterior scleritis along with retinal detachment dacryoadenitis and myositis (n = 5), orbital apex syndrome and T-sign. Spectral Domain Optical Coherence Tomography (n = 6), and idiopathic inflammation involving the preseptal (OCT) of the macula of the OD revealed evidence of macular region, supra-orbital region, , Tenon capsule, orbital fat detachment (Fig. 1C and D). Computed tomography (CT) or optic nerve (n = 14).5 scan of the brain and orbits was compatible with right orbital On imaging studies, orbital pseudotumor may present pseudotumor as is evident by the enlarged recti muscles and with diffuse orbital mass, uveoscleral thickening, contrast proptosis. The optic nerve appeared to be diffusely thick- enhancement of Tenon’s potential space, proptosis, thick- ened along its orbital course (Fig. 2A–D). The patient was ened and optic nerve enlargement.2–5 started on systemic corticosteroids that resulted in dramatic According to the radiologic and surgical findings, focal mass improvement of his symptoms. On the third day his vision within the orbit may be the most frequent finding (43%), fol- had improved to 20/20 along with exudative retinal detach- lowed by lacrimal gland enlargement (32%), diffuse orbital ment as well as right-sided proptosis. inflammation (10%) and myositis (8%); less common findings may be peri-neuritis (2%), scleritis (2%), acute inflammation Discussion (2%) and swelling (1%).3,9 In addition to scleritis, orbital pseudotumor has been associated with systemic disorders, The case described indicates that patients with orbital including , Crohn’s disease and systemic pseudotumor may present with a spectrum of complaints erythematosis.3–5,9 An unusual case of orbital pseudo- which may include decreased vision, proptosis and painful tumor presenting with bilateral exudative retinal detachment eye movements. Physical examination and imaging studies in a 9-year-old girl has been described. Prompt diagnosis and were helpful in narrowing the differential diagnosis and prop- treatment resulted in good clinical response er treatment (Figs. 1 and 2). In contrast to our case, isolated and significant visual recovery.6 unilateral decreased vision without any other findings except Pathogenesis of orbital pseudotumor remains elusive, but the presence of optic neuritis in a case of orbital pseudotu- infection, autoimmune disorder, and aberrant wound mor has been reported by Patankar et al.8 Their patient healings have been put forward as possibilities.3 Pathological had presented with an isolated optic nerve inflammatory findings may be non-specific and may only reveal benign pseudotumor who had complaints of gradual progressive lymphoid hyperplasia and inflammatory cell infiltration with unilateral loss of vision. That patient showed a dramatic necrotizing .2–5 The inflammatory cells comprise

Figure 1. External photograph of a 20-year-old male with right eye proptosis and dilated scleral vessels (A and B). U/S (B-scan) revealed evidence of diffuse sclera thickening along with macular detachment (C) which was confirmed by spectral domain OCT of the macula of the right eye (D). Optic neuritis in orbital pseudotumor 451

Figure 2. Computed tomography scans (axial, A; sagittal, B and coronal views, C and D) of the same patient demonstrating evidence of right eye thickened extraocular muscles as well as optic nerve along its orbital track. non-specific polymorphic, lymphocytic infiltrates with macro- ( and Chlorambucil), immunosuppressants phages, polymorphonuclear leukocytes and eosinophils.2–5 (, Cyclosporine, and ), intravenous Biopsies obtained from the orbital tissue may show comple- immunoglobulins, TNF-alpha inhibitor, monoclonal antibody ment deposits and increased expression of HLA class-I anti- (Infleximab and Adalimumab) and Mycophenolate Moftil.3 gens in the inter-muscular tissue and the biopsies from pseudotumor cases and Graves’ ophthalmopathy cases References contain increased numbers of inter-muscular HLA class-II- expressing cells.3,10 Proliferating cell nuclear antigen activity 1. Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with in conjunction with the ratio of B-/T-cells may be a helpful orbital tumors and simulating lesions: the 2002 Montgomery Lecture, part 1. Ophthalmology 2004;111:997–1008. immunohistologic adjunct for differentiating purely inflam- 2. Harris GJ. Idiopathic orbital inflammation: a pathogenetic construct 3,7 matory lesions of the orbit from lymphoid tumors. and treatment strategy: the 2005 ASOPRS Foundation Lecture. Like our patient, systemic corticosteroid therapy is the cor- Ophthal Plast Reconstr Surg 2006;22:79–86. nerstone of managing orbital pseudotumors. Improvement 3. Chaudhry IA, Shamsi FA, Arat YO, Riley FC. Orbital pseudotumor: distinct diagnostic features and management. Middle East Afr J with corticosteroid therapy is of diagnostic significance specif- Ophthalmol 2008;15:17–27. ically a corticosteroid responsive orbital process is more likely 4. Mombaerts I, Goldschmeding R, Schlingemann RO, Koornneef L. to correspond to pseudotumor.2–5 Our experience has shown What is orbital pseudotumor? Surv Ophthalmol 1996;41:66–78. that over 75% of patients show dramatic improvement within 5. Yuen SJ, Rubin PA. Idiopathic orbital inflammation: distribution, clinical 24–48 h of the initiation of systemic corticosteroids. Other features, and treatment outcome. Arch Ophthalmol 2003;121:491–9. 6. Yuen KS, Lai CH, Chan WM, Lam DS. Bilateral exudative retinal associated abnormal laboratory tests may also respond to sys- detachments as the presenting features of idiopathic orbital temic corticosteroid treatment. Normally, starting dosages of inflammation. Clin Experiment Ophthalmol 2005;33:671–4. Prednisone 1.0–2.0 mg/kg/day are adequate. When improve- 7. Yan J, Wu Z, Li Y. The differentiation of idiopathic inflammatory ment is noted, dosages should be continued with a slow taper- pseudotumor from lymphoid tumors of orbit: analysis of 319 cases. 3 Orbit 2004;23:245–54. ing guided by clinical judgment. Intra-orbital injection of 8. Patankar T, Prasad S, Krishnan A, Laxminarayan R. Isolated optic corticosteroid has been found to be a useful and an effective nerve pseudotumour. Australas Radiol 2000;44:101–3. treatment of orbital pseudotumor and may be considered as 9. Gunalp I, Gunduz K, Yazar Z. Idiopathic orbital inflammatory disease. a first-line treatment in selected patients.11 Recurrences are Acta Ophthalmol Scand 1996;74:191–3. common in orbital pseudotumor especially with bilateral 10. Bijlsma WR, Elbert NJ, Kalmann R. The role of biopsy in diagnosing patients suspected of idiopathic orbital inflammation. Curr Eye Res disease process. Low-dose radiation may be considered for 2012;37:251–3. elderly patients or for those unresponsive to systemic 11. Leibovitch I, Prabhakaran VC, Davis G, Selva D. Intraorbital injection corticosteroids or in whom steroids are contraindicated.2–5 of triamcinolone acetonide in patients with idiopathic orbital Other therapies with promises include cytotoxic agents, inflammation. Arch Ophthalmol 2007;125:1647–51.