Uveitis and Scleritis as a Risk Factor for Mortality.

PURPOSE: Uveitis and scleritis may be caused by local or systemic infection, or associated with noninfectious systemic inflammatory autoimmune disease. This study explored the all-cause mortality following an individual's first presentation with uveitis/scleritis. METHODS: A cross-sectional study was conducted on all uveitis/scleritis patients diagnosed by uveitis specialists and treated in a single tertiary referral center in New Zealand between 2006 and 2020(15y). Masquerade syndromes including intraocular lymphoma were excluded. Outcome measures: demographics, etiology of uveitis/scleritis, anatomical location and all-cause mortality. RESULTS: 2723 subjects were identified. Median age of onset of uveitis/scleritis was 44.9 years (Range:1.5-99.5 years). 49.6% were female. Median follow-up from diagnosis of uveitis/scleritis was 8.0 years (IQR 4.1-11.6 years) with a total follow-up of 24 443.3 subject-years. The most frequent diagnosis was idiopathic disease (30.9%), HLA-B27-positive uveitis (20.0%), and sarcoidosis (4.7%). Infectious etiologies (24.1%) were most commonly from herpes zoster virus (9.3%) and toxoplasmosis (4.3%). The age-adjusted mortality rate was higher in subjects with idiopathic disease, sarcoidosis, Fuchs' uveitis syndrome, granulomatosis with polyangiitis/ANCA-associated vasculitis, toxoplasmosis, and herpes zoster virus, when compared to HLA-B27-positive uveitis. Hazard of mortality peaked in the first seven years following diagnosis, then subsequently declined. Patients with uveitis/scleritis had a significantly higher rate of mortality compared to the general New Zealand population (IRR 1.656 p = 0.017). CONCLUSION: Infectious etiologies of uveitis/scleritis in this cohort were high when compared to other developed nations, attributable to data from a tertiary referral center treating inpatients. Potential shared inflammatory mechanisms in the eye and other organs can lead to concurrent non-ocular disease requiring systemic treatment, impacting an individual's longevity.

Cysts and tumours of the iris: Diagnostic tools and key management considerations-A review.

The iris is a unique structure, with exquisite variations in colour and form. Pathological changes, specifically including iris cysts and tumours are relatively uncommon, difficult to diagnose, and yet potentially blinding or life-threatening. Based on a comprehensive literature review, with highly illustrated key case examples, this report aims to guide the clinician in filtering the differential diagnoses of iris cysts and tumours. Evaluation is in the context of key diagnostic clinical tools and management considerations. Diagnostic imaging techniques include serial anterior segment photography, ultrasound, anterior segment optical coherence tomography, and iris fluorescein angiography, however, the roles of computerised topography and magnetic resonance imaging are also considered in this review. Management includes categorisation in terms of solid iris tumours (melanocytic vs. non-melanocytic), or iris cysts (primary vs. secondary) that may be usefully differentiated by clinical assessment, avoiding more invasive interventions. Cystic lesions are generally benign, although implantation cysts in particular cause significant complications and surgical challenges. Most solid tumours are melanocytic and also typically benign. However, in larger lesions, rapid growth, symptoms and complications more likely indicate malignancy, requiring further investigation.

An optimized 3T MRI scan protocol to assess iris melanoma with subsequent histopathological verification - A prospective study.

INTRODUCTION: Magnetic resonance imaging (MRI) has demonstrated high levels of tissue contrast, accuracy and reproducibility in evaluating posterior uveal melanoma. Owing to smaller size, the role of MRI in detecting and characterising iris melanoma has not yet been explored. AIMS: To develop a protocol to image iris melanoma and describe the MRI characteristics of histopathological-confirmed iris melanoma. MATERIALS AND METHODS: An optimised MRI protocol, using a 3T MRI scanner and a 32-channel head coil, was developed to image iris tumours. A prospective, single-centre, 12-month study was conducted on all patients with lesions suspicious for iris melanoma. All patients were offered an MRI scan in addition to the standardised clinical procedures. Image quality comparison was made with existing clinical investigations. Iris melanoma characteristics on MRI are described. RESULTS: A successful optimised MRI scan protocol was developed that was able to detect and characterise iris melanoma. One normal participant and five patients with subsequent histopathological-confirmed iris melanoma (n = 6) were recruited. Four patients completed the full MRI sequence. All iris melanoma were detected on at least one T1- or T2-weighted images. When compared to the vitreous, all iris melanomas demonstrated hyper-intensity on T1-weighted images and hypo-intensity on T2-weighted images. On T1-mapping, T1-values of iris melanoma demonstrated an inverse relationship with the degree of tumour pigmentation. CONCLUSIONS: This study highlights an optimised, easily reproducible MRI scan protocol to image iris melanoma. Numerous MR imaging characteristics of iris melanoma are reported for the first time and a potential non-invasive tumour biomarker is described.

Tumor Profiles of Late Presentation Uveal Ring Melanoma With Novel Characteristics - A Case Series.

Purpose: The purpose of this study was to investigate the clinical features, tumor characteristics, including histopathology and cytogenetic analysis, and management of patients with uveal ring melanoma in New Zealand. Methods: A retrospective review was conducted on all uveal melanoma cases treated in a single national oncology center in New Zealand. The study period was from January 1, 2013 to December 31, 2022 (10 years). Written consent was obtained from all patients included in this case series. Results: Uveal ring melanoma of ciliary body origin (n = 4) comprised 0.7% of all uveal melanomas (n = 571). Ethnicity distribution was three patients of New Zealand European ancestry and one patient of Chinese/Pasifika ancestry. Three patients (75%) were symptomatic at presentation (spontaneous hyphema, glaucoma, and cataract), whereas one was asymptomatic but subsequently developed painful refractory glaucoma. All eyes underwent enucleation. Three eyes had primary iris biopsies with subsequent enucleation for refractory glaucoma and pain and one eye underwent primary enucleation. All cases demonstrated malignant tumor characteristics including diffuse 270 to 360-degree ciliary body ring growth pattern, epithelioid-cell type and presence of either BAP-1 expression loss or gain of MYC gene. Two cases (50%) developed distant organ metastasis - liver, parotid gland, and breast. Of those, one patient was deceased at the time of follow-up whereas one had completed treatment for metastases. Conclusions: Uveal ring melanoma is a rare tumor that is highly invasive and malignant. When detected, prompt definitive treatment should be advocated, and patient counselling should be given regarding the high risk of developing a painful, blind eye with increased risk of metastasis in the absence of treatment.

The Burden of Histologically Confirmed Uveal Melanoma in Aotearoa-New Zealand: A 21-year Review of the National Cancer Registry.

PURPOSE: The burden of uveal melanoma (UM) in Aotearoa-New Zealand (NZ), a country with the highest global burden of cutaneous melanoma, is unknown. This first, large-scale study of UM in NZ investigates survival and risks of mortality in histologically confirmed UM. METHODS: Deidentified epidemiological data on histologically confirmed UM between January 1, 2000, and December 31, 2020, were extracted from the NZ Cancer Registry. The main outcome measures were patient demographics, tumor characteristics, all-cause versus disease-specific survival, and risks of mortality. RESULTS: Histologically confirmed UM constituted 1.5% (n=703) of all-body site melanomas in NZ (n=47,997). UM predominantly affected Europeans (95%), followed by NZ indigenous Maori (4%), Asians (<1%), and Pacific Peoples (<1%), with no eye or sex predilection. Three hundred eighteen (45%) were deceased at follow-up. Of the deceased, 50% died from UM. The 1-, 5-, and 10-year survival from all-cause mortality was 94%, 68%, and 51%, and disease-specific survival was 97%, 79%, and 71%, respectively. Increasing age at UM diagnosis (>60 y), UM arising from nonspecified sites, and mixed cell UM were associated with an increased risk of disease-specific mortality. No difference in disease-specific mortality was found between sex and ethnicity on multivariate and competing risks analysis. CONCLUSIONS: Despite the government-funded public eye care and increasing research and awareness on UM globally, the burden of UM in the 21st century in NZ remains comparable to global studies. We continue to observe an earlier presentation of UM in non-European cohorts, particularly in our Maori population, and further studies on UM in NZ are warranted.

Conjunctival Melanoma in Aotearoa-New Zealand: A 21-Year Analysis of Incidence and Survival.

PURPOSE: Conjunctival melanoma (CM) is a rare and aggressive malignancy. Global studies demonstrate increased burden of disease in countries with high rates of cutaneous melanoma. There are currently no reports on CM incidence, trends, or survival within Aotearoa-New Zealand (NZ), a country with the highest global rates of cutaneous melanoma, which this study aims to address. DESIGN: This was a retrospective review using the national cancer registry. METHODS: Data on histologically confirmed CM diagnosed between January 1, 2000, and December 31, 2020, were obtained from the NZ Cancer Registry. Cases were identified using the International Classification of Disease, 10th edition (ICD-10) codes. Primary outcome measures were age-standardized incidence, trends, and survival. RESULTS: A total of 68 CM cases were identified. There was a preponderance for females (n=40, 58.8%) and CM predominantly affected European patients (n=63, 92.6%). Median follow-up was 5.0 years [interquartile range (IQR)=2.4-9.9 y] and the median age at diagnosis was 68.5 years (IQR=57.0-79.0 y), with non-Europeans presenting at a significantly younger age [-17.3 y (95% CI: -31.3 to -3.2), P =0.019] than Europeans. The annual age-adjusted incidence(±SD) was 0.6±0.2 cases per million population per year with a stable incidence trend over 21 years. All-cause mortality was found in 28 cases (41.2%) and the median time to death was 3.76 years (IQR=2.1-5.7 y). Five-year all-cause survival and disease-specific survival was 69% and 90%, respectively. CONCLUSIONS: This is the first report on CM incidence, trends, and mortality in NZ. The CM burden is in line with European and North American data, despite NZ having the highest rate of cutaneous melanoma. The incidence remained stable over 2 decades.

Endocapsular artificial iris implantation for iris defects: Reducing symptoms, restoring visual function and improving cosmesis.

BACKGROUND: To investigate repair of iris defects by endocapsular implantation of an artificial iris, in relation to visual outcomes, safety profile and patient satisfaction. METHODS: Retrospective, consecutive case series from Greenlane tertiary teaching hospital and Eye Institute, Auckland, New Zealand. Medical records of patients implanted with an endocapsular artificial iris were reviewed and followed for minimum 3 months. Patient characteristics, surgical management, clinical outcomes and subjective responses were recorded. RESULTS: Nineteen artificial irises were implanted in 18 patients. Etiologies were iris melanotic lesion excision (73.7%), trauma (10.5%), congenital aniridia (10.5%) and Urrets-Zavalia syndrome (5.3%). During postoperative follow-up [14.1 ± 12.4 months (range: 3 to 59 months)], best corrected visual acuity (BCVA) and intraocular pressure (IOP) did not change significantly [BCVA, 0.23 logarithm of the minimum angle of resolution (logMAR) (20/32 Snellen) preoperatively vs. 0.18 logMAR postoperatively (20/25 Snellen) (Z = -0.222, p = 0.824); IOP, 15 mmHg preoperatively vs. 17 mmHg postoperatively (Z = 1.377, p = 0.1447)]. Mild or self-limiting complications included: elevated IOP (42.1%), cystoid macular oedema (15.8%); persisting postoperative uveitis (15.8%) and minor vaulting of the prosthesis (15.7%). Moderate or severe complications included significant vaulting of prosthesis requiring surgical revision (5.3%) and a single eye (5.3%) with trabeculectomy and corneal graft failure. 94.4% of patients were very satisfied with the cosmesis and would be highly likely to have the procedure again. CONCLUSIONS: This study confirms that endocapsular insertion of an artificial iris is typically associated with good functional and cosmetic results and a relatively low risk of significant complications.