Granulomatosis with polyangiitis - orbital involvment

Case contributed by Dr Dalia Ibrahim

Presentation

Bilateral proptosis. She gradually experienced right eye visual disturbance.

Patient Data

Age: 40-year-old
Gender: Female

Both orbits show infiltrative trans-spatial soft-tissue masses involving intra- and extraconal compartments with subsequent bilateral proptosis and stretch of both optic nerves.

Paranasal sinuses show pansinusitis with destruction of medial maxillary sinus walls and left lamina papyracea, perforation of nasal septum, and chronic neo-osteogenesis along sinus walls.

Diffuse infiltration of orbital fat on both sides by soft tissue masses eliciting persistent dark signal with subsequent bilateral proptosis and stretch of both optic nerves.

"Cushingoid facies" with excessive fat secondary to long-term steroid treatment.

Case Discussion

Biopsy proven case of granulomatosis with polyangiitis that had been treated with steroid treatment.

The differential diagnoses for orbital masses included a malignant orbital tumor, immunoglobulin G4-related autoimmune disease and an inflammatory pseudotumor.

As with other granulomatous diseases, orbital disease may appear hypointense on T2 MRI.

Complications of orbital Wegener's granulomatosis include optic nerve compression with subsequent atrophy and visual loss.

The patient had "Cushingoid facies" with excessive fat secondary to long-term steroid treatment.

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Case Information

rID: 51884
Case created: 11th Mar 2017
Last edited: 13th Mar 2017
System: Head & Neck
Inclusion in quiz mode: Included

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