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Clinical and Imaging Features of Orbital Inflammatory Diseases and Sequelae of Ocular Inflammation

Tan, LT; (2013) Clinical and Imaging Features of Orbital Inflammatory Diseases and Sequelae of Ocular Inflammation. Doctoral thesis , UCL (University College London).

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Abstract

Wegener's granulomatosis (WG) is an uncommon multisystem small-vessel vasculitis characterised by granulomatous inflammation. Involvement of ocular and orbital structures in patients with both systemic and limited WG is common and may be a presenting feature in up to 16% of patients. However, the diagnosis of WG presenting with orbital features can be difficult and is often delayed due to the variability of clinical presentation and the lack of a definitive serological or histological diagnostic marker. The purpose of the study is to identify features that may help to differentiate orbital Wegener’s from other orbital inflammatory conditions, including those presenting with lacrimal gland enlargement as a predominant feature. This is a retrospective study of 256 patients who underwent orbital and adnexal biopsy at Moorfields Eye Hospital during a period of 21 years. We have found that a significantly larger proportion of patients with WG exhibited sinonasal symptoms at presentation compared to other orbital inflammatory conditions. Imaging features strongly suggestive of WG included sinonasal and orbital wall erosion as well as nasal septal destruction. These features also correlated well with WG in the subset presenting with lacrimal gland involvement. Severe orbital or ocular inflammation is a major cause of visual loss and ocular morbidity. It may, in a proportion of cases, lead to phthisis bulbi, an irreversible, end-stage condition where rehabilitation is often palliative. In the final part of the study we performed a cross sectional survey of patients with phthisis bulbi in the uveitis clinic to identify the main causes of phthisis and examined the risk factors and disease course leading to development of the phthisical eye. The main cause of phthisis in our study was inflammatory, including 2 patients with WG. Other causes included traumatic, infective and post surgical aetiologies.

Type: Thesis (Doctoral)
Title: Clinical and Imaging Features of Orbital Inflammatory Diseases and Sequelae of Ocular Inflammation
Language: English
Additional information: Permission for digitisation not received. No access allowed to print copy as contains confidential patient information.
Keywords: Granulomatosis with polyangiitis (GPA), Wegener's granulomatosis (WG), imaging features, phthisis bulbi
UCL classification: UCL
UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > Institute of Ophthalmology
URI: https://discovery.ucl.ac.uk/id/eprint/1416285
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