Article
Case report: Wegener's granulomatosis
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Published: | September 22, 2004 |
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Outline
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Objective
We report about a patient with bilateral motility restriction and progressive visual impairment as first symptom of a Wegener's disease, which manifested after surgery of the paranasal sinuses.
Case report
A 30-year-old female patient presented with acute diplopia at the second postoperative day after removal of endonasal adenoids. Ophthalmological findings were a restriction of elevation and adduction as well as a slight protrusion of the right eye. Visual acuity and colour vision were normal on both sides. Computed and nuclear spin tomography were normal. During the next two weeks a complete oculomotor paresis on the right eye with ptosis, abduction position and loss of motility in all directions developed. Additionally an exophthalmos and a retractional nystagmus existed. Repeated nuclear spin tomography did not show any pathological findings. One month later the patient developed an acute visual impairment to 0,05 right and 0,3 left with a bilateral restriction of motility, corresponding to an oculomotor paresis. In spite of heparinisation, large-dose prednisolone and broad range antibiotic therapy the vision deteriorated to no light perception on the right eye and light perception on the left eye. After that a second operation of the paranasal sinuses was carried out in spite of repeatedly normal radiological investigations. During surgery a big nasoseptal defect appeared. Histology showed a vasculitis with vascular occlusion, necrotic tissue and granulomatous reaction at the marginal area corresponding to a Wegener's disease. The antibodies against neutrophil granulocytes were with a titre of 1:80 slightly positive. Under therapy with cyclophosphamide vision improved to 0,3 eccentric on the left eye.
Conclusions
The Wegener's granulomatosis is a rare but important neuroophthalmological emergency, which requires immediately immunosuppressive therapy. The almost loss of vision together with the restriction of motility was the first sign of Wegener`s disease. Laboratory findings and radiological investigations are not always guiding. In this case the key to diagnosis was the finding of a big nasoseptal defect.