A Multi-Centre Case Series Investigating the Aetiology of Hypertrophic Pachymeningitis with Orbital Inflammation

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

ACKNOWLEDGMENTS

This paper is dedicated to the memory of Dr. Venkatesh C. Prabhakaran, whose untimely death in July 2009 has robbed the specialty of oculoplastics of an eminent talent. He will be greatly missed. We are grateful to Mr. Alan Hoare, Clinical Photographer, South Australian Institute of Ophthalmology for his assistance in formatting the clinical images.

Declaration of interest: Dr. Cannon received funding from the Royal College of Ophthalmologists, the Dickinson Trust, Pfizer and the Ethicon Foundation for Ophthalmic Fellowship training in Adelaide. These sponsors had no role in the design or conduct of this study. The authors alone are responsible for the content and writing of the paper. No funding was obtained for this study.