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 2011
Externally publishedYes

Fingerprint

Meningitis
Inflammation
Visual Acuity
Granulomatosis with Polyangiitis
Tuberculosis
Biopsy
Orbital Diseases
Patient Acuity
Dura Mater
Diplopia
Lost to Follow-Up
Neurologic Examination
Therapeutics
Nose
Cyclophosphamide
Headache

Keywords

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

ASJC Scopus subject areas

  • Ophthalmology

Cite this

Cannon, P. S., Cruz, A. A. V., Pinto, C. T., Mastropietro, D. A., Chahud, F., Bilyk, J. R., ... Prabhakaran, V. C. (2011). A multi-centre case series investigating the aetiology of hypertrophic pachymeningitis with orbital inflammation. Orbit, 30(2), 64-69. https://doi.org/10.3109/01676830.2010.539766

A multi-centre case series investigating the aetiology of hypertrophic pachymeningitis with orbital inflammation. / Cannon, Paul S.; Cruz, Antonio A V; Pinto, Carolina T.; Mastropietro, Dante A.; Chahud, Fernando; Bilyk, Jurij R.; Selva, Dinesh; Prabhakaran, Venkatesh C.

In: Orbit, Vol. 30, No. 2, 03.2011, p. 64-69.

Research output: Contribution to journalArticle

Cannon, PS, Cruz, AAV, Pinto, CT, Mastropietro, DA, Chahud, F, Bilyk, JR, Selva, D & Prabhakaran, VC 2011, 'A multi-centre case series investigating the aetiology of hypertrophic pachymeningitis with orbital inflammation', Orbit, vol. 30, no. 2, pp. 64-69. https://doi.org/10.3109/01676830.2010.539766
Cannon, Paul S. ; Cruz, Antonio A V ; Pinto, Carolina T. ; Mastropietro, Dante A. ; Chahud, Fernando ; Bilyk, Jurij R. ; Selva, Dinesh ; Prabhakaran, Venkatesh C. / A multi-centre case series investigating the aetiology of hypertrophic pachymeningitis with orbital inflammation. In: Orbit. 2011 ; Vol. 30, No. 2. pp. 64-69.
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