Non Specific Inflammation of the Orbital Apex: About 3 Cases

Abstract

Abstract: : Purpose: To show the diagnostic difficulties for detecting orbital apex inflammation, a suggestion of useful clinical investigations is proposed when confronted with patients presenting clinical signs caused by inflammatory processes in this particular anatomical region. Methods: We analyzed retrospectively the medical records of patients who consulted between April 2003 and December 2003 for an orbital apex syndrome. Clinical signs which led to the topographical diagnosis are lined out. The complementary exams consisted of a high resolution MRI focused on the orbital apex, a complete biological status to detect inflammatory reactions, Goldmann’s visual field and orthoptic evaluation. Two biopsies of the orbital apex were realized, one by endonasal and one by neurosurgical approach, in order to eliminate a specific etiology of the infiltrating process. Steroidtherapy was introduced and the evolution of the clinical symptoms and visual acuity was analyzed. Results: In the 3 patients ( 47, 52 and 90 years) included, 5 eyes were affected. Initial clinical signs consisted of progressive complete ophthalmoplegia in 2 cases (3 eyes). A severe optical neuropathy associated with ocular pain was the most striking feature, as it was found in all patients (5 eyes). Exophthalmia, red eye or intra ocular inflammation were absent. No specific etiology was determined by the exhaustive complementary exams. MRI showed an infiltrating process of the orbital apex but biopsy results concluded non specific inflammation and fibrosis. After steroidtherapy (methylprednisolone) regression of the infiltrating process was observed but visual acuity was improved only in 1 patient and only unilaterally. Conclusions: Non specific inflammations of the orbital apex are a rare cause of optical neuropathy. In spite of there small incidence, they should be suspected in patients presenting the association of ophthalmoplegia and optical neuropathy. Radiological investigations focused on the orbital apex region are highly recommended. The prognostic of these orbital affections is rapidly decreasing and a diagnosis at an early state is essential for treatment and thus the evolution. The diagnosis is even more difficult to assess as inflammatory affections of the orbital apex do not automatically induce visible local inflammatory reactions. Due to the posterior location in the orbit, a biopsy is in most cases very difficult to realize or might be impossible.

Keywords: orbit • neuro-ophthalmology: optic nerve • imaging/image analysis: clinical