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|Title:||A multi-centre case series investigating the aetiology of hypertrophic pachymeningitis with orbital inflammation|
|Citation:||Orbit, 2011; 30(2):64-69|
|Publisher:||Taylor & Francis The Netherlands|
|Paul S. Cannon, Antonio A.V. Cruz, Carolina T. Pinto, Dante A. Mastropietro, Fernando Chahud, Jurij R. Bilyk, Dinesh Selva and Venkatesh C. Prabhakaran|
|Abstract:||INTRODUCTION: To describe our attempt in establishing a definitive diagnosis in patients with hypertrophic pachymeningitis in combination with orbital inflammatory disease and report on the outcome. MATERIALS AND METHODS: This was a retrospective case series of all patients presenting with hypertrophic pachymeningitis in association with orbital inflammation in 4 centres. Ophthalmic and neurological examination data, laboratory data, histology data, treatment plans and clinical outcome data were recorded. Patients underwent orbital/brain computed tomography and magnetic resonance imaging. RESULTS: Six patients were identified; the median age was 46.5 years. Headache was the commonest presenting symptom, followed by diplopia and reduced visual acuity. Three patients underwent orbital biopsy, 1 patient underwent dura mater biopsy, 1 patient underwent both and 1 patient underwent nasal biopsy. Four patients were diagnosed with Wegener granulomatosis and 2 patients with tuberculosis. Corticosteroid therapy was initiated in 4 patients, with steroid-sparing drugs added later. Two patients received anti-tuberculosis treatment and 1 patient was commenced on pulsed cyclophosphamide. On follow-up, 1 patient required an exenteration for a painful blind eye and 1 patient’s visual acuity remained at no perception to light. One patient had complete resolution of symptoms on treatment, 1 patient had persistent reduced visual acuity and 1 patient was lost to follow-up. CONCLUSION: We postulate that the combination of orbital inflammation and pachymeningitis is strongly suggestive of Wegener granulomatosis, although it may take a number of years to confirm. Tuberculosis should also be considered.|
|Keywords:||Orbital inflammation; hypertrophic pachymeningitis; aetiology; Wegener's granulomatosis; Tuberculosis|
|Rights:||Copyright © 2011 Informa Healthcare USA, Inc.|
|Appears in Collections:||Opthalmology & Visual Sciences publications|
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