ASNR 2015 A Wegener's Granulomatosis Case: Initial Presentation is Orbital Involvement E Ure, Y...
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Transcript of ASNR 2015 A Wegener's Granulomatosis Case: Initial Presentation is Orbital Involvement E Ure, Y...
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ASNR 2015
A Wegener's Granulomatosis Case: Initial Presentation is Orbital
Involvement
E Ure, Y Kayadibi, D Tekcan Sanli, Z I Hasiloglu
Istanbul University Cerrahpasa Medical School, Istanbul, Turkey
Presentation number:EP-87
Submission number:1433
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Disclosure
• No Disclosures
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Background
• Wegener’s Granulomstosis (WG) is a systemic disease that characterized by necrotizing granulomatous vasculitis.
• Incidence of orbital-ocular involvement in WG is around 50-60 % and first presentation form like orbital involvement is around 8-16%.
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Purpose
• In this presentation, we purpose to report a Wegener’s Granulomatosis (WG) case initially presented with eye problems.
• Our principle aim is to demonstrate the orbital involvement of WG radiologically.
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Materials & Methods
• A 9-year-old girl admitted with complaints of eyes redness, eyelids edema, ptosis and exophtalmus to our hospital.
• Orbital Magnetic Resonance (MR) Imaging, blood and urine tests and histopathological examination were performed for diagnosis.
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Results
• Orbital MR Imaging revealed bilateral intraorbital-extraconal soft tissue lesions with indistinguishable clear boundaries from extraocular muscles and lacrimal gland.
• Orbital biopsy was performed from the lesion.
• Myointimal thickening of the vessel wall, microthrombus and perivascular lymphocytic infiltration in the vessels lumen and intense fibrosis in the lacrimal gland were seen histologically.
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Magnetic Resonance Imaging Findings
Figure 2Figure 1
Figure 1 and 2 showed bilateral intraorbital-extraconal soft tissue lesions on T1WI and T2WI MR Imaging.
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Magnetic Resonance Imaging Findings
Figure 3 Figure 4
The lesions were indistinguishable from extraocular muscles and lacrimal gland on axial MR Imaging in figure 3 and 4.
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Results
• Urinalysis showed hematuria and proteinuria, renal biopsy revealed crescentic glomerulonephritis.
• Anti-Neutrophil Cytoplasmic Antibody (c-ANCA) positivity was present in patient.
• Hereby, WG was diagnosed in this patient who presented with orbital involvement initially.
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Discussion
• Orbital pathology in WG can occur as a primary form of granulomatous vasculitis involvement and also it may be propagation of paranasal sinuses inflamation to the ocular cavity.
• Orbital manifestations can be episcleritis, scleritis, lacrimal gland obstruction, ocular pain, diplopia, periorbital cellulitis, retro-orbital mass, proptosis, optic neuropathy and blindness.
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Discussion
• Radiological modalities can help to distinguish WG from other entities.
• CT can show obliteration of tissue planes and bony erosion.
• On MR Imaging, the lesions are hypo-intense in relation to orbital fat in T1 and T2-weighted imagining and enhance with gadolinium.
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Conclusion
• In this presentation, we presented a Wegener's Granulomatosis case whose initial presentation is orbital involvement.
• In conclusion, orbital-ocular involvement should be kept in mind in WG and radiological assessment must be performed from this perspective.
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References
• 1. Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 1992:116: 488–98
• 2. Thorne JE, Jabs DA. Ocular manifestations of vasculitis. Rheum Dis Clin North Am 2001: 27: 761-79
• 3. Haynes BF, Fishman ML, Fauci AS, Wolff SM. The ocular manifestations of Wegener’s granulomatosis. Fifteen years experience and review of the literature. Am J Med 1977: 63: 131-41
• 4. Woo TL, Francis IC, Wilcsek GA, Coroneo MT, McNab AA, Sullivan TJ et al. Australasian orbital and adnexal Wegener ’s granulomatosis. Ophthalmology 2001:108: 1535–43
• 5. Provenzale JM, Mukherji S, Allen NB, Castillo M, Weber AW. Orbital involvement by Wegener’s granulomatosis: imaging findings. AJR Am J Roentgenol 1996: 166: 929–34