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

Disclosure

• No Disclosures

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%.

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.

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.

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.

Magnetic Resonance Imaging Findings

Figure 2Figure 1

Figure 1 and 2 showed bilateral intraorbital-extraconal soft tissue lesions on T1WI and T2WI MR Imaging.

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.

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.

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.

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.

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.

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