Astrocytic outer retinal layer thinning is not a feature in AQP4-IgG seropositive neuromyelitis optica spectrum disorders.

BACKGROUND: Patients with anti-aquaporin-4 antibody seropositive (AQP4-IgG+) neuromyelitis optica spectrum disorders (NMOSDs) frequently suffer from optic neuritis (ON) leading to severe retinal neuroaxonal damage. Further, the relationship of this retinal damage to a primary astrocytopathy in NMOSD is uncertain. Primary astrocytopathy has been suggested to cause ON-independent retinal damage and contribute to changes particularly in the outer plexiform layer (OPL) and outer nuclear layer (ONL), as reported in some earlier studies. However, these were limited in their sample size and contradictory as to the localisation. This study assesses outer retinal layer changes using optical coherence tomography (OCT) in a multicentre cross-sectional cohort. METHOD: 197 patients who were AQP4-IgG+ and 32 myelin-oligodendrocyte-glycoprotein antibody seropositive (MOG-IgG+) patients were enrolled in this study along with 75 healthy controls. Participants underwent neurological examination and OCT with central postprocessing conducted at a single site. RESULTS: No significant thinning of OPL (25.02±2.03 µm) or ONL (61.63±7.04 µm) were observed in patients who were AQP4-IgG+ compared with patients who were MOG-IgG+ with comparable neuroaxonal damage (OPL: 25.10±2.00 µm; ONL: 64.71±7.87 µm) or healthy controls (OPL: 24.58±1.64 µm; ONL: 63.59±5.78 µm). Eyes of patients who were AQP4-IgG+ (19.84±5.09 µm, p=0.027) and MOG-IgG+ (19.82±4.78 µm, p=0.004) with a history of ON showed parafoveal OPL thinning compared with healthy controls (20.99±5.14 µm); this was not observed elsewhere. CONCLUSION: The results suggest that outer retinal layer loss is not a consistent component of retinal astrocytic damage in AQP4-IgG+ NMOSD. Longitudinal studies are necessary to determine if OPL and ONL are damaged in late disease due to retrograde trans-synaptic axonal degeneration and whether outer retinal dysfunction occurs despite any measurable structural correlates.

Clinical Features and Outcomes of Patients with Orbital Schwannoma.

PURPOSE: To report the clinical features, treatment, and outcome of 11 patients with orbital schwannoma in Korean patients. METHODS: The medical records of 11 orbital schwannoma patients treated between April 2007 and April 2021 were retrospectively reviewed. The demographic data, clinical characteristics, radiological features, and outcomes were reviewed. RESULTS: The mean age at the time of diagnosis was 49.00 ± 14.45 years. The most common initial symptom was ocular protrusion (n = 7), and other symptoms were decreased visual acuity ( n = 5), restriction of eye movement ( n = 4), swelling ( n = 3), and pain ( n = 2). Locations of tumors were superomedial; followed by the orbital apex and inferolateral. The most common shape seen in our patients was beads like multilobulated appearance; followed by a round, oval, fusiform, and dumbbell shape. MRI of T1-weighted revealed isointense or hypointense, whereas the T2-weighted indicated hyper or isointense lesion. Five patients had optic neuropathy at presentation, and 1 of them showed improved vision after surgery. Complete or incomplete excision was performed for all. Surgical complications include decreased vision and paraesthesia. There has been no recurrence to date. CONCLUSIONS: Orbital schwannoma is a rare disease and it is difficult to distinguish it from other orbital tumors because the initial symptoms are nonspecific. Differential diagnosis by combining the shape, location, and contrast enhancement findings seen on computed tomography and magnetic resonance imaging can be helpful in surgical treatment. Complete excision gives the best results without recurrence, but if the patient is in an inaccessible location, only surgery to reduce the volume can satisfy the patient without recurrence.

Analysis of Clinical Characteristics and Prognosis of Patients With Periocular Malignant Melanoma in Korea.

OBJECTIVE: To report the clinical features of 20 patients with malignant melanoma and to evaluate the survival and prognosis of patients with malignant melanoma in Korea. METHODS AND ANALYSIS: The medical records of 20 patients with malignant melanoma treated between March 2004 and March 2020 were reviewed retrospectively. The demographic data, clinical characteristics, and outcomes were also reviewed. Outcome measures included local recurrence, metastasis, and tumor-related mortality. Prognostic factors associated with recurrence, metastasis, and survival were analyzed using a Cox proportional hazards model. RESULTS: Among the 20 patients with periocular malignant melanoma, 4 (20%) showed local recurrence during follow-up (6.61 ±â€Š6.36 years). The 1-, 5-, and 10-year recurrence rates were 5%, 10.3%, and 24.1%, respectively. 10 (50%) patients developed distant metastasis, mainly involving lung, brain, parotid gland and spine. The 1-, 5-, and 10-year rates of metastasis were 5%, 10.9%, and 35.7%, respectively. Significantly fewer metastases were detected following initial extensive surgical excision (P = 0.04). 8 (40%) patients died of malignant melanoma. The main risk factor for mortality was tumor thickness (HR: 3.88, P < 0.01). Based on Kaplan-Meier survival estimates, the 1-, 5-, and 10-year tumor-related survival rates were 75.8%, 55.6%, and 55.6%, respectively. CONCLUSION: Tumor thickness is a significant prognostic factor affecting the survival rate. It is important to reduce the metastatic rate via extensive resection without leaving any residual tumor in the margin during surgery.

Blepharoptosis among Korean adults: age-related prevalence and threshold age for evaluation.

BACKGROUND: To evaluate the prevalence of blepharoptosis among Korean adults and the characteristics of blepharoptosis patients, and to determine an appropriate age threshold for recommending blepharoptosis evaluation. METHODS: The Korean National Health and Nutrition Examination Survey (KNHANES-V) was conducted in 2010-2012. We extracted data on 17,878 Korean adults aged more than and equal to 19 years included in KNHANES-V, and determined blepharoptosis prevalence according to age, to determine the cutoff age for recommending blepharoptosis evaluation. We also determined the possible association between blepharoptosis and obesity parameters, such as body mass index (BMI) and waist circumference (WC). RESULTS: There was astrong association between older age and the prevalence of blepharoptosis. The cutoff age for recommending blepharoptosis evaluation was 63 years for males, 70 years for females, and 66 years for all patients. Patients with a high BMI and large WC had a higher prevalence of blepharoptosis in all age groups except for those aged over 80 years. The association of blepharoptosis with BMI according to age group showed that in the 50-59 and 60-69 years age groups, blepharoptosis prevalence and BMI were higher. However, in the 70-79 and 80-89 years age groups, extremely obese patients (BMI > 30) showed a decreased blepharoptosis prevalence. CONCLUSIONS: Moderate to severe blepharoptosis can result in poor visual function and exacerbate headaches and depression, leading to decreased quality of life. This study proposed an appropriate age threshold for recommending evaluation of patients with blepharoptosis among the general population of Korea.

A Metastatic Neuroendocrine Eyelid Carcinoma Originating From the Ampulla of Vater.

Tumor metastasis to the eyelids is very rare. The authors report the first case of a neuroendocrine tumor that originated from the ampulla of Vater in the gastrointestinal tract and metastasized to the eyelid. The patient with a hospice care was referred to the authors' eye clinic after presenting with a palpable mass on the left eyelid and blinking discomfort. He was previously diagnosed with a neuroendocrine tumor arising from the ampulla of Vater. The eyelid tumor was simply removed to relieve discomfort, and histological and immunophenotypic evaluation of the resected eyelid mass showed a poorly differentiated neuroendocrine tumor with large cell types, similar to specimens from the duodenum.

CD34 + tumours of the orbit including solitary fibrous tumours: a six-case series.

BACKGROUND: To report six cases of CD34+ fibroblastic mesenchymal tumours, which are uncommon neoplasms in the orbit. CASE PRESENTATION: Six patients presenting with proptosis and palpable mass who were later diagnosed with fibrous solitary tumours, fibrous histocytoma or haemangiopericytoma in the orbit were included. All patients received radiologic examinations and surgical excision for histopathology and immunohistochemistry examinations. Five patients had no recurrence after a minimum follow-up of 12 months. One patient (case 6) experienced recurrence twice, and had debulking surgeries each time. At present, the patient still has remnant tumour in the orbit, but no growth has been detected during the past two years. The tumour size will be closely monitored. CONCLUSIONS: Even though fibroblastic tumours are rarely found in the orbit, they can present as a palpable mass with proptosis. Complete surgical excision is important for long-term prognosis, and immunohistochemical study is helpful for confirming pathologic diagnosis.

Surgical Outcomes of Deep Superior Sulcus Augmentation Using Acellular Human Dermal Matrix in Anophthalmic or Phthisis Socket.

Patients with anophthalmic or phthisis socket suffer from cosmetic problems. To resolve those problems, the authors present the surgical outcomes of deep superior sulcus (DSS) augmentation using acellular dermal matrix in patients with anophthalmic or phthisis socket. The authors retrospectively reviewed anophthalmic or phthisis patients who underwent surgery for DSS augmentation using acellular dermal matrix. To evaluate surgical outcomes, the authors focused on 3 aspects: the possibility of wearing contact prosthesis, the degree of correction of the DSS, and any surgical complications. The degree of correction of DSS was classified as excellent: restoration of superior sulcus enough to remove sunken sulcus shadow; fair: gain of correction effect but sunken shadow remained; or fail: no effect of correction at all. Ten eyes of 10 patients were included. There was a mean 21.3 ±â€Š37.1-month period from evisceration or enucleation to the operation for DSS augmentation. All patients could wear contact prosthesis after the operation (100%). The degree of correction was excellent in 8 patients (80%) and fair in 2. Three of 10 (30%) showed complications: eyelid entropion, upper eyelid multiple creases, and spontaneous wound dehiscence followed by inflammation after stitch removal. Uneven skin surface and paresthesia in the forehead area of the affected eye may be observed after surgery. The overall surgical outcomes were favorable, showing an excellent degree of correction of DSS and low surgical complication rates. This procedure is effective for patients who have DSS in the absence or atrophy of the eyeball.

Suggestion of response evaluation criteria in patients with ocular adnexal mucosa-associated lymphoid tissue lymphoma (OAML).

Ocular adnexal mucosa-associated lymphoid tissue lymphoma (OAML) has been recognized as most common primary orbital malignancy. However, little was known about the response criteria for OAML. Our aim was to suggest response criteria for nonconjunctival OAML; the response evaluation of which using the conventional response criteria is inappropriate. A retrospective chart review of 34 eyes from 30 patients diagnosed with nonconjunctival OAML was conducted, focusing on the change in tumor size based on linear bi-dimensional and three-dimensional methods in magnetic resonance imaging (MRI) of the orbit. The maximum tumor response period of each case was investigated, and the expected optimal response period was calculated using regression analysis. In 30 evaluable patients, the median time taken for the maximum tumor response was 6 months (range, 3-18). More than 75 % of patients attained maximal tumor response in 6 months after initial therapy for follow-up period, the median value of which was 30 months (range, 15-77). Based on the regression analysis, it took 4.7 months for the maximum diameter (2r) of tumor to decrease by 50 % of initial lesion size. We cautiously suggest that optimal response could be defined as 50 % reduction of the maximum diameter in 6 months since the treatment was initiated, and that only observation without additional therapy is enough for nonconjunctival OAML, if optimal response is achieved.

Change in the antioxidative capacity of extraocular muscles in patients with exotropia.

BACKGROUND: We compared the oxidative stress and antioxidant capacities of the medial rectus muscles (MRMs) between the patients with constant exotropia and control subjects. METHODS: A total of 40 MRMs from patients with constant exotropia and 40 MRMs from normal donor eyes (controls) were assessed. Neuronal nitric oxide synthase (nNOS), superoxide dismutase (SOD), and catalase levels were compared between the two groups using western blot analysis. In addition, the lipofuscin accumulation level was compared between the exotropic and control groups. RESULTS: According to western blot analysis, the nNOS level was significantly higher in the exotropic group than in the control group (P < 0.05). On the other hand, a catalase expression level was higher in the control group than in the exotropic group (P < 0.05). The SOD1 level did not show a significant difference between the two groups. The relative lipofuscin fluorescence intensity was higher in the exotropic group than in the control group (P < 0.001). CONCLUSIONS: Based on these findings, the MRMs of patients with exotropia had a redox imbalance status. Further study is needed to investigate whether this imbalance in antioxidant capacity is present in the extraocular muscles of patients with other strabismus.

Surgical outcomes of acellular human dermal grafts for large conjunctiva defects in orbital implant insertion.

PURPOSE: We report the surgical outcomes of acellular human dermal allografts for repair of large conjunctiva defects in primary or secondary orbital implant insertion. METHODS: This retrospective chart review examined 12 eyes in 12 patients who underwent primary or secondary orbital implant insertion using Medpor® and acellular human dermal allografts to decrease conjunctival tension. Only patients with at least 6 months follow-up were included. The procedure was successful if there was no exposure until the last eye-clinic visit and the patient could wear a prosthesis. It failed if additional surgery was needed to correct the orbital implant exposure. RESULTS: The mean follow-up was 24.5 (range 6-54) months. Seven patients had primary orbital implant insertion, and five had secondary orbital implant insertion with acellular human dermal allografts. All enrolled patients had successful outcomes without exposure of the central conjunctiva. Two underwent additional surgery to correct fornix contracture and regain a cosmetically good prosthesis. Of these, one patient received additional acellular human dermal grafts and had a fair outcome. The other patient ultimately required fornix reconstruction with an autologous graft using oral mucosa. CONCLUSION: Orbital implant insertion with acellular human dermis grafts had good surgical outcomes. The procedure could be effective for patients with insufficient conjunctival tissue to cover an orbital implant and a high probability of developing orbital implant exposure if tension was created by excessive direct conjunctival closure.

No thermal tool using methods in endoscopic dacryocystorhinostomy: no cautery, no drill, no illuminator, no more tears.

The purpose of this study was to evaluate the surgical outcome of the endoscopic dacryocystorhinostomy (DCR) without thermal tools such as cautery, drill and illuminator. The study is a retrospective analysis of patients in a tertiary care unit for oculoplastic surgery. The participants enrolled into the study are a retrospective series of 127 consecutive endonasal DCRs performed between January 2008 and March 2011. The surgical procedure in this conventional endoscopic transnasal DCR involved a manual osteotomy of the frontal process of the maxilla and removal of the lacrimal bone by punch without illuminator, cauterization and drill. We evaluated the result of the manual Endo-DCR technique without cauterization or drilling-assisted technique. Data of 127 eyes were reviewed. Full success was achieved in 90.5 % (115/127) of manual Endo-DCR technique with an average follow-up period of 6 months. Our study appears to show favorable results compared to other previously published outcomes including Endo-DCR surgery with thermal equipments. No thermal tool methods in endonasal DCR can achieve a good surgical success rate. Therefore, the newest tools, cauterization, drilling or illumination, are generally not necessary for endoscopic dacrycystorhinostomy.

A case of congenital orbital fibroepithelial polyp.

Fibroepithelial polyp (FEP) is a benign tumor of mesodermal origin, composed of varying amounts of stroma covered by epithelium. The skin and the genitourinary tract are the most common sites of FEP. Uncommon locations of FEP include the inferior turbinate of the nasal cavity, bronchi, and the pharynx. To date, there has been no report of orbital FEP. The authors describe a patient with congenital orbital FEP arising from the lateral canthal area. This congenital FEP was completely excised successfully through an anterior orbitotomy without any complications, with no evidence of recurrence after 25 months.

Lacrimal gland fistula after cosmetic lateral canthoplasty.

BACKGROUND: We report a case of lacrimal gland fistula formation after cosmetic lateral canthoplasty in a young Asian woman. PATIENT: A 34-year-old woman, who twice underwent lateral canthoplasties of both eyes with additional upper eyelid blepharoplasty, developed clear fluid discharge from a small draining tract near the lateral canthus after being operated on with cosmetic lateral canthoplasty. Assuming that lacrimal gland fistula has developed, we differentiated the tract from the lacrimal gland using a Bowman probe and performed lacrimal gland fistulectomy, which resolved the discharge, leaving no complications. CONCLUSIONS: To our knowledge, this is the first case of lacrimal gland fistula after cosmetic lateral canthoplasty, and surgeons performing this procedure should be aware of lacrimal gland herniation and fistula tract formation, especially in patients who have undergone multiple eyelid surgeries.

Association between age-related cataract and blepharoptosis in Korean adults: a population-based study.

Cataract and blepharoptosis are both commonly encountered ophthalmic problems in older adults. Since they share similar risk factors, it is plausible that there may be an association between the two conditions. We examined data from the Korean National Health and Nutrition Examination Survey (KNHANES) 2010-2012 to determine if there is an association between age-related cataract and blepharoptosis. Multivariable adjusted logistic regression analysis was conducted to examine the odds ratio (OR) and 95% confidence interval (CI) for association of each specific type of cataract with presence of blepharoptosis. Of the 10,387 eligible participants, 4782 (46.0%) had cataract and 1419 (15.8%) had blepharoptosis. There were more participants with blepharoptosis in the cataract group, compared with those in the no cataract group. After adjusting for potential confounders, participants with blepharoptosis had a higher risk of total cataract (OR: 1.557, 95% CI 1.201-2.019) and nuclear subtype cataract (OR: 1.305, 95% CI 1.050-1.620). Blepharoptosis was associated with significantly higher odds of cataract in obese participants when compared with non-obese participants (p for interaction = 0.0236). Our study revealed a positive association between age-related cataract and blepharoptosis; it suggests that thorough ophthalmic assessment is needed when assessing patients who are planning cataract or blepharoptosis surgery.

Nationwide trends in the incidence of orbital lymphoma from 1999 to 2016 in South Korea.

BACKGROUND/AIMS: Lymphomas are the most frequent neoplasm of the orbit. However, the epidemiology of orbital lymphomas is not well reported. This study aimed to provide a population-based report on the epidemiology of orbital lymphomas and measure the trends in the incidence of orbital lymphoma cancer in South Korea. METHODS: Nationwide cancer incidence data from 1999 to 2016 were obtained from the Korea Central Cancer Registry. Age-standardised incidence rates and annual percent changes were calculated according to sex and histological types. The analysis according to the Surveillance, Epidemiology, and End Results summary stage classifications was performed from 2006 to 2016. Survival rates were estimated for cases diagnosed from 1999 to 2016. RESULTS: A total of 630 patients (median age: 54 years) with orbital lymphoma in the orbital soft tissue were included in this study. The age-standardised incidence rates increased from 0.03 to 0.08 per 100 000 individuals between 1999 and 2016, with an annual percent change of 6.61%. The most common histopathological type of orbital lymphoma was extra marginal zone B cell lymphoma, accounting for 82.2% of all orbital lymphomas during 1999-2016, followed by diffuse large B cell lymphoma (9.2%). Five-year, 10-year and 15-year overall survival (OS) of orbital lymphoma was 90.8%, 83.8% and 75.8%, respectively. OS showed a significant decrease as age increased and no significant differences between men and women. CONCLUSION: The incidence rate of orbital lymphoma is very low in South Korea. However, the incidence rate has increased over the past years. Orbital lymphomas have a worse prognosis as age increases.

Nationwide Trends in the Incidence and Survival of Eyelid Skin Cancers in Korea.

PURPOSE: This study provides a population-based report on eyelid skin cancer epidemiology showing trends in its incidence and survival in South Korea. METHODS: For this population-based epidemiological study, nationwide cancer incidence data were obtained from the Korea Central Cancer Registry, covering the entire population. Age-standardised incidence rates (ASRs) and annual per cent changes (APCs) were calculated according to sex, histological types, stage, and date of diagnosis. The 5-year relative survival rates (RSRs) were estimated for patients diagnosed between 1993 and 2016. RESULTS: The ASR increased from 0.27 per 100,000 population in 1999 to 0.61 in 2016, with an APC of 4.94%. The most common histopathological type was basal cell carcinoma (BCC), accounting for 67.5% of all eyelid skin cancers during 1999-2016, followed by sebaceous gland carcinoma (SGC, 10.7%), and squamous cell carcinoma (SCC, 10.6%). The number of localised cancer diagnoses tended to increase, compared to that of distant cancer. The 5-year RSR was nearly 100% when considering all eyelid skin cancers together. When considering them separately, the 5-year RSR of BCC was nearly 100% throughout, whereas for SGC and SCC, it gradually increased from 94.2% and 87.7% (1993-1995), respectively, to nearly 100% (2011-2016) for both. Melanoma had the lowest survival, although its survival has increased markedly since 1993. CONCLUSIONS: Nationwide data used in this study showed an increase in incidence and survival rates of eyelid skin cancers in Korea. BCC was the most predominant type, and its proportion among all eyelid skin cancer cases has increased.

Visual outcomes of proton beam therapy for choroidal melanoma at a single institute in the Republic of Korea.

We evaluate the ocular effects of proton beam therapy (PBT) in a single institution, in Korea, and identify factors contributing to decreasing visual acuity (VA) after PBT. A total of 40 patients who received PBT for choroidal melanoma (2009‒2016) were reviewed. Dose fractionation was 60‒70 cobalt gray equivalents (CGEs) over five fractions. Complete ophthalmic examinations including funduscopy and ultrasonography were performed at baseline and at 3, 6, and 12 months after PBT, then annually thereafter. Only patients with at least 12 months follow-up were included. During the follow-up, consecutive best-corrected visual acuity (BCVA) changes were determined, and univariate and multivariate logistic regression analyses were performed to identify predictors for VA loss. The median follow-up duration was 32 months (range: 12‒82 months). The final BCVA of nine patients was > 20/40. The main cause of vision loss was intraocular bleeding, such as neovascular glaucoma or retinal hemorrhage. Vision loss was correlated with the tumor size, tumor distance to the optic disc or fovea, maculae receiving 30 CGEs, optic discs receiving 30 CGEs, and retinas receiving 30 CGEs. Approximately one-third of PBT-treated choroidal melanoma patients with good pretreatment BCVA maintained their VA. The patients who finally lost vision (VA < count fingers) usually experienced rapid declines in VA from 6‒12 months after PBT. Tumor size, tumor distance to the optic disc or fovea, volume of the macula, and optic discs or retinas receiving 30 CGEs affected the final VA.

Serum neurofilament light chain levels as a biomarker of neuroaxonal injury and severity of oxaliplatin-induced peripheral neuropathy.

We set out to determine the usability of serum neurofilament light chain (sNfL), serum glial fibrillary acidic protein (sGFAP), and retinal parameters by using optical coherence tomography (OCT) as reliable biomarkers of the progression of oxaliplatin-induced peripheral neuropathy (OIPN). Forty-three patients scheduled to undergo oxaliplatin-based chemotherapy at the National Cancer Center of Korea between June 2018 and October 2019 were prospectively assessed at baseline, 3 months, and 6 months of chemotherapy. Patients were assessed on clinical scales and underwent OCT, sNfL, and sGFAP level measurement at each follow-up visit. By applying the National Cancer Institute-Common Toxicity Criteria (NCI-CTC), OIPN was classified as grade 1 in 12 (28%) patients, grade 2 in 25 (58%), and grade 3 in 5 (12%) at 6 months of chemotherapy. sNfL levels increased during oxaliplatin administration, while serial sGFAP levels or retinal parameters did not change. Patients with grade-3 OIPN showed significantly higher mean sNfL levels than patients with grade 0-2 OIPN at 6 months of treatment. At 4-6 months after completion of chemotherapy, sNfL levels were significantly reduced compared to the levels at 6 months of chemotherapy. Monitoring of sNfL during chemotherapy can indicate ongoing neuroaxonal injury and the severity of OIPN.

Cohort profile: a collaborative multicentre study of retinal optical coherence tomography in 539 patients with neuromyelitis optica spectrum disorders (CROCTINO).

PURPOSE: Optical coherence tomography (OCT) captures retinal damage in neuromyelitis optica spectrum disorders (NMOSD). Previous studies investigating OCT in NMOSD have been limited by the rareness and heterogeneity of the disease. The goal of this study was to establish an image repository platform, which will facilitate neuroimaging studies in NMOSD. Here we summarise the profile of the Collaborative OCT in NMOSD repository as the initial effort in establishing this platform. This repository should prove invaluable for studies using OCT to investigate NMOSD. PARTICIPANTS: The current cohort includes data from 539 patients with NMOSD and 114 healthy controls. These were collected at 22 participating centres from North and South America, Asia and Europe. The dataset consists of demographic details, diagnosis, antibody status, clinical disability, visual function, history of optic neuritis and other NMOSD defining attacks, and OCT source data from three different OCT devices. FINDINGS TO DATE: The cohort informs similar demographic and clinical characteristics as those of previously published NMOSD cohorts. The image repository platform and centre network continue to be available for future prospective neuroimaging studies in NMOSD. For the conduct of the study, we have refined OCT image quality criteria and developed a cross-device intraretinal segmentation pipeline. FUTURE PLANS: We are pursuing several scientific projects based on the repository, such as analysing retinal layer thickness measurements, in this cohort in an attempt to identify differences between distinct disease phenotypes, demographics and ethnicities. The dataset will be available for further projects to interested, qualified parties, such as those using specialised image analysis or artificial intelligence applications.