A multi-centre case series investigating the aetiology of hypertrophic pachymeningitis with orbital inflammation

Paul S. Cannon, Antonio A.V. Cruz, Carolina T. Pinto, Dante A. Mastropietro, Fernando Chahud, Jurij R. Bilyk, Dinesh Selva, Venkatesh C. Prabhakaran

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
Pages (from-to)64-69
Number of pages6
JournalOrbit
Volume30
Issue number2
DOIs
StatePublished - Mar 1 2011

Keywords

  • Aetiology
  • Hypertrophic pachymeningitis
  • Orbital inflammation
  • Tuberculosis
  • Wegener's granulomatosis

ASJC Scopus subject areas

  • Ophthalmology

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