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Orbital Wegener’s granuloma resulting from direct extension of nasal disease through a surgical rhinostomy
  1. Moorfields Eye Hospital, London EC1V 2PD
  1. Mr Anthony S L Kwan, Department of Pathology, Institute of Ophthalmology, London EC1V 9EL.

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Editor,—Wegener’s granulomatosis is a multisystem granulomatous disease of unknown aetiology but known to have numerous ocular complications including orbital inflammatory disease. We report a patient with “limited Wegener’s granulomatosis” (Wegener’s disease without renal involvement) who developed an orbital inflammatory mass by a direct extension of nasal inflammatory disease through a rhinostomy formed during lacrimal drainage surgery.


A 46 year old man, with a 7 year history of quiescent bilateral nasal sinus disease due to nasal mucosal biopsy proved Wegener’s granulomatosis, was seen in the lacrimal clinic at Moorfields Eye Hospital. He was maintained on daily oral prednisolone 10 mg and azathioprine 150 mg. He had a 7 month history of bilateral epiphora and lacrimal irrigation demonstrated obstruction of the nasolacrimal ducts. Biopsy of the lacrimal sac mucosa at the time of left dacryocystorhinostomy was clear of granulomatous disease and he received no additional postoperative immunosuppression. Postoperatively all healed well and computed tomographic scan (CT scan) showed no orbital disease. At 11 months, while still on the original dose of immunosuppression, he presented with left retrobulbar ache. Visual acuity was unimpaired (Snellen 6/6 right eye, 6/9 left eye) and there was no relative afferent pupil defect. The left globe was displaced superolaterally by 2 mm and there was 3 mm of relative proptosis with no restriction of left ocular movements (Fig 1). An ill defined inferomedial orbital mass, of soft tissue attenuation and in continuity with the nasal cavity at the site of rhinostomy, was shown by CT scan (Fig 2). The proptosis and orbital signs resolved after a 6 month course of reducing dose of oral prednisolone 60 mg and cyclophosphamide 250 mg and the lacrimal drainage fistula remains patent. A right dacryocystorhinostomy was performed 3 months later, while the patient was on an increased maintenance dose of oral prednisolone 17.5 mg and cyclophosphamide 100 mg, and his postoperative course has been uneventful to 24 months of follow up.

Figure 1

Patient at 11 months after left dacryocystorhinostomy, with the left globe displaced anteriorly and superolaterally.

Figure 2

Axial (A) and coronal (B) CT scans at 11 months after surgery, showing a left inferomedial orbital mass extending into the orbit from the nose, through the site of rhinostomy (small arrows). The globe, medial rectus, and inferior rectus (large arrow, (A)) are displaced and surrounded by the mass (B, C).


Nasolacrimal duct obstruction has been reported in 7% of patients with Wegener’s granulomatosis1 and dacryocystorhinostomy in these patients may be associated with postoperative wound necrosis and the formation of nasocutaneous fistula.2 Although dacryocystectomy has been suggested as a treatment for nasolacrimal duct obstruction in this disease,3 others have reported more encouraging results with dacryocystorhinostomy.4 5

Extension of nasal Wegener’s granulomatosis into the orbit after dacryocystorhinostomy has not previously been reported, although non-contiguous involvement, probably due to reactivated orbital disease, has been recorded at 1 month after a dacryocystorhinostomy in Wegener’s granulomatosis.3 The disease is known to involve ocular and orbital tissues and several papers report the coexistence of orbital and nasosinus disease, with erosion of intervening bone.3 6 7 In the case reported here, orbital involvement was the direct result of the disease process passing through an iatrogenic bony opening created during dacryocystorhinostomy and, in view of this, we suggest that such patients should be monitored postoperatively for signs of orbital disease and be considered for additional perioperative systemic immunosuppression.


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