Erika Damato

Detection and Time to Treatment of Uveal Melanoma in the United Kingdom: An Evaluation of 2384 Patients

PURPOSE To determine the mode of detection of uveal melanoma and time to treatment in the United Kingdom. DESIGN Prospective cohort study. PARTICIPANTS A total of 2384 patients diagnosed with uveal melanoma at the Liverpool Ocular Oncology Center between 1996 and early 2011. METHODS A questionnaire was completed with every new patient, and the results were correlated with clinical features and treatment. MAIN OUTCOME MEASURES Tumor detection, practitioner initiating referral, referral pathway, time to treatment, baseline clinical features, and primary ocular treatment. RESULTS The referral process was initiated by an optometrist, family doctor, or ophthalmologist in 68.0%, 18.2%, and 13.8% of patients, respectively. On referral, 30.2% of patients were asymptomatic. Twenty-three percent of patients reported that their tumor was initially missed; these tended to have a more advanced tumor when they reached our center. The time from referral to treatment had a median of 49 days, exceeding 6 months in 19.8% of patients. This delay was longer in patients who reported that their tumor was missed (median, 92 vs. 40 days; Mann-Whitney, P<0.001). Ophthalmologists delayed the referral process by more than 6 months in 10.9% of patients. Primary enucleation was performed in 33.3% of patients and was more likely in those who reported that their tumor was missed (44.8% vs. 29.8%; chi-square, P<0.001). CONCLUSIONS Many patients with uveal melanoma experience long delays in treatment because their tumor was missed or misdiagnosed. Such patients tend to have a more advanced tumor by the time they reach an oncology center and are more likely to require enucleation.

Birmingham Behçet’s service: classification of disease and application of the 2014 International Criteria for Behçet’s Disease (ICBD) to a UK cohort

BackgroundThis study reports on the analysis of the application and diagnostic predictability of the revised 2014 ICBD criteria in an unselected cohort of UK patients, and the ensuing organ associations and patterns of disease.MethodsA retrospective cohort study was conducted using a database of electronic medical records. Three categories were recognised: clinically defined BD, incomplete BD and rejected diagnoses of BD. We applied the ISG 1990 and ICBD 2014 classification criteria to these subgroups to validate diagnostic accuracy against the multidisciplinary assessment.ResultsBetween 2012 and 2015, 281 patients underwent initial assessment at an urban tertiary care centre: 190 patients with a confirmed diagnosis of BD, 7 with an incomplete diagnosis, and 84 with a rejected diagnosis. ICBD 2014 demonstrated an estimated sensitivity of 97.89% (95% CI: 94.70 to 99.42) and positive likelihood ratio of 1.21 (1.10 to 1.28). The strongest independent predictors were: Central nervous lesions (OR = 10.57, 95% CI: 1.34 to 83.30); Genital ulceration (OR = 9.05, 95% CI: 3.35 to 24.47); Erythema nodosum (OR = 6.59, 95% CI: 2.35 to 18.51); Retinal vasculitis (OR = 6.25, 95% CI: 1.47 to 26.60); Anterior uveitis (OR = 6.16, 95% CI: 2.37 to 16.02); Posterior uveitis (OR = 4.82, 95% CI: 1.25 to 18.59).ConclusionsThe ICBD 2014 criteria were more sensitive at picking up cases than ISG 1990 using the multidisciplinary assessment as the gold standard. ICBD may over-diagnose BD in a UK population. Patients who have an incomplete form of BD represent a distinct group that should not be given an early diagnostic label. Behçet’s disease is a complex disease that is best diagnosed by multidisciplinary clinical assessment. Patients in the UK differ in their clinical presentation and genetic susceptibility from the original descriptions. This study also highlights an incomplete group of Behçet’s patients that are less well defined by their clinical presentation.

Choroidal schwannoma: a case series of five patients

Aim To report a case series of five patients diagnosed with choroidal schwannoma at the Liverpool Ocular Oncology Centre. Methods Patients with choroidal schwannoma were identified by searching the computerised database of the Liverpool Ocular Oncology Centre. Results The patients (3 males, 2 females) ranged in age from 15 years to 45 years. Three tumours were treated by enucleation, trans-scleral local resection, and combined bevacizumab and photodynamic therapy, respectively. Two were observed after confirmation of the diagnosis by biopsy. Conclusions Choroidal schwannoma has a variety of clinical manifestations. Associated features include hard exudates, retinal feeder vessels and serous retinal detachment. Biopsy with immunohistochemistry is required for diagnosis. Tumours not amenable to resection may respond to photodynamic therapy.

An update on the use of biologic therapies in the management of uveitis in Behçet’s disease: a comprehensive review

AbstractᅟBehçet’s disease (BD) is a systemic vasculitis characterised by a relapsing remitting course, affecting multiple organ systems. In the eye, it is a cause of potentially blinding inflammation in the form of uveitis. Management of uveitis in BD often requires the use of systemic immunosuppression, in order to reduce disease activity and prevent accumulation of irreversible damage. Whilst corticosteroids remain the mainstay of treatment, long-term use is limited by the development of adrenocorticotrophic side effects. There has therefore been significant interest in the use of corticosteroid-sparing immunosuppressive agents, and more recently, biologic therapies. Recent publications have demonstrated biologic therapy to have beneficial effects both on overall disease control, and quality of life for patients with BD. Widespread use of such agents is however limited, partly by the lack of high quality research evidence, and partly by the prohibitive cost of biologic treatments. In this review, we discuss the most recent research investigating the use of biologic therapy in uveitis due to BD, with consideration of health economics and quality of life outcomes.

Systemic Lupus Erythematosus and the Eye

Abstract Ocular involvement is common in systemic lupus erythematosus (SLE), ranging from relatively mild dry eye syndrome to severe sight-threatening disease. The recognition of ocular manifestations is important in the treatment of ocular disease and may indicate underlying active systemic inflammation. Ophthalmic sequelae may also arise from the systemic treatments required for the treatment of SLE.

Primary optic nerve sheath meningioma, Medical Radiology Series: Radiation Oncology Series Editors: Brady, Heilmann, Molls, Nieder Book Editors: B. Jeremic, S. Pitz

It may seem ambitious to dedicate a textbook to the topic of optic nerve meningioma. Optic nerve sheath meningiomas are rare but diverse. The treatment is challenging and demands excellence in imaging, surgery, and radiotherapy, all of which have advanced in recent years. For these reasons, there is plenty of scope for an in-depth and up-todate review on this subject. This slim, hardback 135-page A5 book addresses this specialized subject. The book is divided into ten relatively equal-sized chapters, each written by a different group of authors, allowing the reader to benefit from the opinion of an international panel of experts in this field. The chapters are well organized and beautifully presented. Each begins with a clear summary and is well supported by extensive references. The book is filled with photographs and radiographic images, which is especially relevant for those chapters addressing imaging of the nerve and explaining radiotherapy and planning. All the clinical photographs are in color. The radiographic images are high definition and of excellent quality. As previously mentioned, the subject is a difficult one. Opinions regarding management have been varied in the past. Clinical presentation is not specific. Histological classification of the tumor is challenging due to the lack of specimens. Imaging studies are difficult to interpret because imaging modalities have improved significantly in the past two decades so that older studies are less informative. Historically, radiotherapy did not provide dramatic benefits and was commonly associated with significant side-effects, while surgical treatment was regarded as a destructive intervention, resulting in visual loss and not reliably preventing recurrence. Before reviewing this book we had certain key questions, which we hoped would be answered by the authors. The different chapters adequately addressed all of these. Each chapter is accessible in isolation, which means that readers can go directly to that area of the book relevant to their query. A consequence of this, however, is that there is a degree of repetition when reading the book from cover to cover.