2†Pathways to detection of non-infectious childhood uveitis in the UK: findings from the UNICORN cohort study.

INTRODUCTION: Prompt detection of childhood uveitis is key to minimising negative impact. From an internationally unique inception cohort, we report pathways to disease detection.UNICORNS is a national childhood non-infectious uveitis study with longitudinal collection of a standardised clinical dataset and patient reported outcomes. Descriptive analysis of baseline characteristics are reported.Amongst 150 recruited children (51% female, 31% non-white ethnicity) age at detection ranged from 2-18yrs (median 10). In 69%, uveitis was diagnosed following onset of symptoms: time from first symptoms to uveitis detection ranged from 0-739days (median 7days), with longer time to detection for those presenting initially to their general practitioner. Non symptomatic children were detected through JIA/other disease surveillance (16%), routine optometry review (5%) or child visual health screening (1%). Commonest underlying diagnoses at uveitis detection were JIA (17%), TINU (9%, higher than pre-pandemic reported UK disease frequency) and sarcoid (1%). 60% had no known systemic disease at uveitis detection. At disease detection, in at least one eye: 34% had structural complications (associated with greater time to detection - 17 days versus 4 days for uncomplicated presentation).The larger relative proportions of children with non-JIA uveitis reported here increase the importance of improving awareness of childhood uveitis amongst the wider clinical communities. There is scope for improvement of pathways to detection. Forthcoming analysis on the full cohort (251 recruited to date across 33 hospitals and 4 nations) will provide nationally representative data on management and the determinants of visual and broader developmental/well-being outcomes.

Tubulointerstitial nephritis and uveitis in Northern Ireland.

OBJECTIVES: This paper looks at patients with a diagnosis of tubulointerstitial nephritis and uveitis (TINU) presenting to the Northern Ireland regional adult and paediatric uveitis service in the Belfast Health and Social Care Trust. The demographic distribution, treatment required and the visual and renal outcomes of these patients are documented. METHODS: Data were collected retrospectively on 24 patients with TINU using the Northern Ireland Electronic Care Record, central pathology records alongside the adult and paediatric uveitis databases from 2011 to 2021. Patients were categorised into two groups using the Mandeville classification system. Standard Uveitis Nomenclature (SUN) was used to classify the uveitis. RESULTS: The population prevalence is at least 12.6 cases per million based on a population of 1.9 million. Nineteen of 24 cases were definite TINU and five of 24 probable. Seventeen out of 24 had biopsy-positive TIN, all of which met all of the Mandeville clinical diagnostic features required for a definite diagnosis. All but one presented with acute bilateral anterior uveitis. The paediatric cases ranged from age 12 to 18 at age of onset with a mean age of 14. Of the 18 adult onset cases, the age ranged from 20 to 76 years. The mean age of onset for the adult cases was 53 years. Of these patients 71% were female; 42% required second-line immunosuppression for ocular disease. Visual acuity was maintained. Follow-up time ranged from 3 months to 16 years. No patient developed long-term renal impairment. CONCLUSIONS: TINU is a cause of uveitis in both the paediatric and adult populations. In Northern Ireland average age with TINU was older than much of the published literature. Long-term immunosuppression for uveitis may be required as ongoing ocular, rather than renal inflammation seemed to require treatment.

Impact of healthcare strategies on patterns of paediatric sight impairment in a developed population: 1984-2011.

PurposeThe aim of our study was to analyse paediatric sight-impairment trends in Northern Ireland (NI) over a 28-year period to better understand the impact which changes in health-care provision may be having on childhood blindness and to enable us to assess our progress towards achieving the World Health Organisation (WHO) aims.MethodsA database of Certificates of Visual Impairment completed for NI children aged <16 years was used to determine the cause of sight impairment from 1984 to 2011. Causes were classified into preventable or treatable conditions and analysed for trends.ResultsFive hundred and ninety-eight children were registered as having impaired vision over the 28-year period. A total of 22% had preventable or treatable conditions. Optic atrophy was the most common cause responsible for 16% of registrations followed by albinism (12%), cerebral visual impairment (11%), congenital cataract (8%), retinopathy of prematurity (ROP) (8%), and congenital motor nystagmus (7%). The incident rate (per million population aged <16 years) for registerable vision loss due to congenital cataract decreased from 5.89 (CI 2.82-10.83) in 1984-1987 to 2.63 (CI 0.72-6.74) in 2008-2011. For ROP, the incident rate peaked during 2000-2003 at 8.87 (CI 4.85-14.88). Thereafter, there was a statistically significant reduction in incident rate to 1.98 (CI 0.41-5.77) in 2008-2011 (P=0.008).ConclusionsSight-impairment registrations due to preventable or treatable causes have decreased over the past 28 years. This is likely due to better surgical techniques and improved refractive care for conditions such as congenital cataract and ongoing research and treatment protocols for conditions such as ROP. Future advances in this area may help to further reduce the burden of childhood sight impairment and improve quality of life for these patients.

Paediatric intraocular lens implants: accuracy of lens power calculations.

PurposeThis study aims to evaluate the accuracy of lens prediction formulae on a paediatric population.MethodsA retrospective case-note review was undertaken of patients under 8 years old who underwent cataract surgery with primary lens implantation in a regional referral centre for paediatric ophthalmology, excluding those whose procedure was secondary to trauma. Biometric and refractive data were analysed for 43 eyes, including prediction errors (PE). Statistical measures used included mean absolute error (MAE), median absolute error (MedAE), Student's t-test and Lin's correlation coefficient.ResultsThe mean PE using the SRK-II formula was +0.96 D (range -2.47D to +2.41 D, SD 1.33 D, MAE 1.38 D, MedAE 1.55, n=15). The mean PE was smaller using SRK/T (-0.18 D, range -3.25 D to +3.95 D, SD 1.70 D, MAE 1.30 D, MedAE 1.24, n=27). We performed an analysis of the biometry data using four different formula (Hoffer Q, Holladay 1, SRK-II and SRK/T). Hoffer Q showed a smaller MedAE than other formulae but also a myopic bias.ConclusionOur clinical data suggest SRK/T was more accurate in predicting post-operative refraction in this cohort of paediatric patients undergoing cataract surgery. Hoffer Q may have improved accuracy further.

A clinical study of Aicardi syndrome in Northern Ireland: the spectrum of ophthalmic findings.

PurposeAicardi syndrome is a rare disorder, affecting ~1 in 100 000 live births. Chorioretinal lacunae feature alongside agenesis of the corpus callosum and spasms in flexion to make up a diagnostic triad. Recently ophthalmic findings such as microphthalmia and optic disc anomalies have been recognised in association with Aicardi syndrome. This population study aims to determine the presence of ocular findings and identifies some novel associations in these patients.MethodsA retrospective review of charts for seven patients with Aicardi syndrome was carried out.ResultsThe incidence of Aicardi syndrome in Northern Ireland was found to be 1 in 110 000 live births. Four patients who had microphthalmus also had iris abnormalities; two patients with bilateral microphthalmus had partial aniridia and two patients with unilateral microphthalmus had iris coloboma in the same eye. Optic disc abnormalities were found in 11 eyes of six patients. Two patients were found to have areas of fibrovascular proliferation with a thickened white ridge and avascular zone beyond. Both of these patients developed retinal detachments.ConclusionsOur review of patients with Aicardi syndrome in Northern Ireland has revealed some novel clinical findings, including aniridia in two cases. We also found a higher than previously reported rate of excavated disc anomalies of 50% in our cohort. We found two cases of peripheral retinal dysplasia, which has not been previously reported. This finding was associated with microphthalmus and severe optic disc abnormalities, and we feel this warrants early EUA to enable early treatment and hopefully result in better visual prognosis.

Long term functional and structural outcomes of laser therapy for retinopathy of prematurity.

AIMS: To assess the long term functional and structural outcomes of premature babies who received diode laser photocoagulation for threshold retinopathy of prematurity (ROP). METHODS: 25 patients (43 eyes) treated with laser were recalled for assessment at a mean follow up of 11 years. A further seven patients (14 eyes) with subthreshold ROP, which had regressed spontaneously without laser treatment, were also examined. All children underwent distance acuity, near acuity, contrast sensitivity (CS), and colour vision assessments followed by a dilated fundal examination and cycloplegic autorefraction. RESULTS: The laser treated eyes had a mean distance visual acuity of 0.37 logMAR, a mean near visual acuity of 0.39 logMAR, a mean contrast sensitivity of 1.49 log CS units, and a mean spherical equivalent of -2.10D. An unfavourable distance visual acuity outcome occurred in five eyes (13.5%). An unfavourable near visual acuity outcome was also noted in the five eyes (13.5%) with poor distance visual outcome. 7% had an unfavourable structural outcome. On comparison with the control group, there was no significant difference in near acuity, CS, refraction, or colour vision between the two groups. However, there was a statistically significant difference in terms of distance visual acuity (p = 0.03). CONCLUSIONS: Laser treated eyes with favourable structural outcome have a good visual outcome. The results show a long term benefit from diode laser photocoagulation in preserving distance and near vision in eyes with threshold ROP.

RetCam image analysis of optic disc morphology in premature infants and its relation to ischaemic brain injury.

AIMS: To assess optic disc characteristics in premature infants with and without ischaemic brain injury and to evaluate the role of optic disc morphology in dating the injury. METHODS: RetCam fundal images, cranial ultrasounds and magnetic resonance imaging (MRI) of 109 premature infants were analysed. The study cohort was divided into subgroups depending on the presence or absence of periventricular leucomalacia (PVL) and intraventricular haemorrhage (IVH). The control group consisted of infants with normal neuroimaging at term and 2 years of age. Using the image analysis software of the RetCam, optic disc diameter (ODD), optic disc area (ODA), and optic cup area (OCA) were measured at 33-34 weeks gestational age. As serial cranial ultrasonography had been performed, it was possible to date the brain injury in those infants with periventricular white matter (PVWM) damage. RESULTS: Although there was a trend towards reducing ODD, ODA, and OCA with increasing severity of IVH, only the IVH 4 group differed significantly from the controls for these parameters (p = 0.002, p = 0.02, and p = 0.04, respectively). 44.4% of infants with grade 4 IVH had small discs. Only one patient had a large cup in a normal sized disc; this patient had IVH 4. In patients with PVWM damage, the median time of insult was 27 weeks in those with small discs and 28 weeks in those with normal discs. This difference was not significant (p = 0.23). CONCLUSIONS: Premature infants with IVH 4 have an increased incidence of optic nerve hypoplasia. We found no association between disc morphology and timing of brain injury.

Duplication of the optic disc: true or pseudo? A coloboma or not a coloboma?

PURPOSE: To present an unusual case of optic disc pseudoduplication with colobomata. METHODS: Clinical evaluation, fundus photography and literature review. RESULTS: Optic disc duplication is a rare clinical entity. CONCLUSIONS: We report what we believe to be the first case of pseudoduplication of the optic disc with coexistent bilateral optic disc colobomata.

Pregnancy-related papillophlebitis.

PURPOSE: To report a case of pregnancy-related papillophlebitis in a woman with an uncomplicated pregnancy and no identifiable coagulation defect. METHODS: Clinical evaluation and fundus photography. RESULTS: Spontaneous recovery occurred post-delivery without treatment. CONCLUSIONS: Venous thromboembolic events of the eye are a rare complication of pregnancy unless associated with toxemia. This case report of a pregnant patient with papillophlebitis indicates that the natural clinical course of this condition is one of spontaneous resolution and treatment is therefore unnecessary.

Silicone oil-intraocular lens interaction: which lens to use?

AIM: To determine a suitable intraocular lens for implantation in patients at high risk of lens exposure to silicone oil in their lifetime. METHODS: PMMA, AcrySof, AR40, AQUA-Sense, and Raysoft lenses were examined. Each lens was immersed for 5 minute intervals in balanced salt solution (BSS), in stained silicone oil, and again in BSS before being photographed in air and in BSS. Percentage silicone oil coverage of the lens optic was determined. RESULTS: The mean percentage coating (MPC) for the lens biomaterials ranged from 5.2% to 21.5%. The Raysoft lens had significantly less oil coverage when statistically compared with the other lens types (p < 0.001). CONCLUSION: A Raysoft (Rayner) lens is a suitable lens for implantation in patients who are at risk of severe vitreoretinal disease.