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29 Mass-Forming Inflammatory Lesions of the Orbit Zeynel A 29 Mass-Forming Inflammatory Lesions of the Orbit Zeynel A. Karcioglu any inflammatory conditions, ranging from periostitis, or periorbital and orbital mass lesions have simple foreign body granuloma (Figure 29.1) been reported.11,12 Orbital tuberculosis due to atypi- Mto the most complex vasculitis of a collagen cal mycobacteria may present as a well-delineated tissue disease, may cause proptosis owing to volume mass lesion, causing gradual displacement of the eye increase in the orbit.1–3 The great majority of these and extraocular motility disturbances.13,14 The history disorders present with typical ocular/orbital signs and of an antecedent penetrating injury is a well-known symptoms, as well as systemic manifestations, that presentation of tuberculosis, due to atypical my- would differentiate them from neoplastic conditions. cobacteria. If tuberculosis is suspected clinically, tu- Others, however, particularly the ones that tend to de- berculin skin testing will be helpful in differential velop localized mass lesions rather than infiltrating diagnosis; but a biopsy, with or without positive cul- inflammation, may simulate orbital neoplasms. This tures, is necessary for the confirmation of the disease. chapter summarizes the salient clinical features of the Histopathology of tuberculosis consists of zonal gran- more common, mass-forming inflammatory lesions, ulomatous inflammation with numerous epithelioid which can be confused with orbital neoplasms; others histiocytes surrounding a necrotic (caseating) center. are listed in Tables 29.1 and 29.2. Some related clin- Tissue diagnosis is pathognomonic only with the doc- ical presentations are detailed in Chapter 27 with il- umentation of positive acid-fast organisms. However, lustrations from cases. it is well known that acid-fast positive mycobacteria often are not demonstrated in tuberculosis, even though cultures of orbital tissue may grow M. tuber- INFECTIONS culosis or atypical mycobacteria. It should be remembered that atypical mycobacter- ial infections generally are resistant to routine antitu- Tuberculosis berculous chemotherapy; clarithromycin, an oral mac- Ocular and adnexal tuberculosis is usually seen with rolide antibiotic, has been reported to be an effective typical manifestations secondary to systemic My- medication for atypical mycobacterial infections.12 cobacterium infection, and it is rather unlikely that this entity will be confused with neoplastic disor- Fungal Infections ders.4–6 However, the clinical picture of the disease is changing, with many cases developing from atypical Commonly encountered fungal infections of the orbit mycobacteria that are resistant to traditional mul- are mucormycosis and aspergillosis. Mucormycosis tidrug treatment.7,8 With the increase in the numbers rarely produces clinical manifestations to mimic orbital of immunologically suppressed individuals secondary tumors. The fulminant course of orbital disease with to viral epidemics and the wider use of immunosup- pain, massive proptosis, extensive extraocular motility pressant antimetabolites in longer surviving cancer disturbance, and hemorrhagic chemosis, coupled with and transplant patients, the incidence of tuberculosis necrotic eschars of the nasal, oropharyngeal mucosa or has been rising steadily during the past two decades. periorbital skin, is typical of this infection and does not It has been reported that individuals with HIV/AIDS leave too much room for differential diagnosis.15 Imag- have an incidence of tuberculosis 500-fold greater than ing may be helpful by demonstrating a relationship be- that of the general population.9 tween the orbital and sinus disease. T2-weighted Extrapulmonary tuberculosis, including the orbital magnetic resonance imaging (MRI) usually reveals hy- disease, is more often seen in children and nonwhite pointensity of fungal disease, and computed tomogra- patients.10 Although ocular and adnexal infections due phy (CT) shows focal calcification of the orbitosinu- to atypical mycobacteria are rare, occasional cases pre- soidal mass.16 However, certain cases involving the senting as dacryocystitis, endophthalmitis, localized eyelids and the orbit may present with gradually de- 335 336 PART SIX: TUMORLIKE CONDITIONS OF THE ORBIT are the cases that are difficult to diagnose and may be confused with neoplasms (Figure 29.2). The diagnosis is dependent on the confirmation of septate hyphae that branch at a typical 45° angle. The Gomori methenamine silver (GMS) technique highlights the walls of the hyphae. Although the diagnosis can eas- ily be established by the identification of the organ- isms in fulminating cases, the sclerosing type may not readily reveal the causative organism in small biopsy samples. The tissue sample may show only dense fi- brous tissue, without any granulomas or organisms. Although fine-needle aspiration biopsy (FNAB) has been used to diagnose fungal infections, when as- pergillosis is suspected, it is better to perform inci- sional biopsies on the lesions. The limited sample ob- tained by FNAB is more likely to be nondiagnostic in sclerosing cases. The management of orbital as- FIGURE 29.1. Foreign body granuloma of the superior orbit in a 9- year old girl. At surgery, infiltrating foreign body granuloma com- pergillosis includes surgical debridement and antifun- posed of epithelioid cells and multinucleated giant cells was iden- gal therapy. Wide surgical excision of the involved tis- tified. The nature of the foreign body (arrow) could not be sues is suggested in the sclerosing type, since the determined. infection has a tendency to recur.19,20 Parasitic Infections veloping, localized lesions, which may be confused with rapidly growing tumors, such as rhabdomyosar- Echinococcus granulosus, otherwise known as hydatic coma and Ewing’s sarcoma (see Chapter 27). Diagnosis cyst, is probably the most common parasitic disease is established by documentation of typical, large mu- of the orbit. Hydatid disease is most commonly en- cor hyphae, which show lack of septation in tissue ex- countered in the liver (60–70%) and lungs but may amination by histopathology or cytopathology. spread hematogenously into the systemic circulation Orbital mucormycosis is an emergency situation and infect multiple extrahepatic sites. Orbital hydatid because it causes rapidly progressing necrotizing in- disease is rare, comprising less than 1% of all cases in flammation secondary to the propensity of the fungus the body. Once in the orbit, most lesions lie within to involve blood vessels. Orbital exploration should the muscle cone in children and young adults.21,22 The be done immediately to establish the diagnosis by diagnosis of orbital hydatid disease is suggested by identifying the broad, nonseptated hyphae and for ex- signs and symptoms of a unilateral, orbital space- tensive surgical debridement as well as irrigation with occupying mass, such as gradual progressive proptosis antifungal agents. and diminished extraocular motility. Eosinophilia is Orbital aspergillosis usually presents with a more present in approximately 25% of cases. CT findings insidious onset, particularly when it develops in indi- usually include a hypodense, nonenhancing, often viduals who are not immunocompromised.17 In these unilocular (but occasionally multilocular) cystic le- cases, the disease may follow a protracted course with sion, well delineated by a thin capsule that may or a well-delineated mass developing within the orbit may not show enhancement in contrast studies. MRI without diagnostic features on CT or MRI.17,18 The discloses a low intensity signal on T1-weighted im- latter group produces a densely sclerosed, chronic in- ages and a high-intensity signal on T2-weighted im- flammatory reaction with granulomatous foci. These ages. Microscopic examination of the cyst fluid TABLE 29.1. Orbital Inflammations that May Cause Space-Occupying Lesions in the Orbit. Nonspecific Specific Collagen Infections inflammations inflammations Vasculitides tissue disorders Tuberculosis Orbital pseudotumor Sarcoidosis Polyarteritis Lupus nodosa erythematosus Lyme disease Foreign body granuloma Crohn’s disease Churg–Strauss Dermatomyositis syndrome Dacryoadenitis Hematic cyst/cholesteatoma Sjögren’s syndrome Kimura‘s disease Scleroderma Mucormycosis Mucocele Wegener’s granulomatosis Behçet’s disease Rheumatoid arthritis Aspergillosis Amyloidosis Echinococcosis TABLE 29.2. Vasculitis Syndromes and Collagen Tissue Disorders that May Present with Localized or Infiltrative Space-Occupying Lesions in the Orbit. Disease Systemic findings Ocular/Orbital manifestation Wegener’s granulomatosis Necrotizing vasculitis and granulomatous inflammation in upper Orbital granulomatosis and vasculitis with proptosis; EOM disturbance; and lower respiratory tract, kidneys and skin; C-ANCA (ϩ) ON vasculitis and/or compression; conjunctivitis, scleritis; Ȇserum IgA, IgE; RF (ϩ); anemia choroidal ischemia Angiolymphoid hyperplasia with Nonspecific inflammation with lymphocytes and eosinophils in Localized, poorly delineated nonspecific inflammation in the orbit eosinophilia (Kimura’s disease) the skin, upper respiratory tract, kidney; bronchial asthma; causing proptosis and EOM disturbance eosinophilia Polyarteritis nodosa Systemic small and medium vessel vasculitis that may involve Orbital vasculitis and soft tissue necrosis causing proptosis, any organ; skin lesions; arthralgias; weight loss; peripheral EOM disturbance, scleritis and choroidal ischemia neuropathy Churg–Strauss syndrome Small-vessel
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