Validation of the East London Retinopathy of Prematurity algorithm to detect treatment-warranted retinopathy of prematurity: a cohort study.

AIM: To validate the East London Retinopathy of Prematurity algorithm (EL-ROP) in a cohort of infants at risk of developing retinopathy of prematurity (ROP). METHODS: The EL-ROP algorithm was applied retrospectively to routinely collected data from two tertiary neonatal units in England on infants eligible for ROP screening. The EL-ROP recommendation, to screen or not, was compared with the development of treatment-warranted ROP (TW-ROP) for each infant. The main outcome measures were (1) EL-ROP's sensitivity for predicting the future development of TW-ROP and (2) potential to reduce ROP screening examinations. RESULTS: Data from 568 infants were included in the trial. The median (IQR) birth weight (g) was 875 (704 - 1103) and gestational age (weeks) was 27.0 (25.4 - 29.0). Maternal ethnicity was black (33%) and non-black (67%). 58(10%) developed TW-ROP and in every case this was predicted by the EL-ROP algorithm. It's sensitivity was 100% (95% CI 94-100%) specificity: 44% (95% CI 39-48%) positive predictive value: 17% (95%CI 16-18%), negative predictive value: 100%. CONCLUSIONS: EL-ROP has been validated in a cohort of infants from two tertiary neonatal units in England. Further validation is required before its clinical usefulness can be assessed.

Periosteal fixation of the medial and lateral recti for large-angle incomitant exotropia.

PURPOSE: To describe the evolution of a surgical technique for the correction of large-angle incomitant exodeviations. METHODS: Retrospective review of an interventional case series from 2005 to 2019 in a single centre, with analysis of surgical procedure, prism diopter (PD) deviations and complications. RESULTS: Thirty-one patients underwent surgery at an average age of 42 years (range 4-75 years) for minimal medial rectus function, mostly from oculomotor nerve palsy (23/31; 74%). The mean pre-operative exodeviation was 75 PD (range 30-200PD). Sixteen patients (52%) had undergone previous strabismus surgery. Thirty-eight operations were performed in which the medial rectus insertion was anchored to the periosteum of the posterior lacrimal crest via a retrocaruncular transconjunctival approach. The ipsilateral lateral rectus (LR) was disinserted and fixed to lateral orbital tissue in 29/38 (76%) operations, injected with botulinum toxin in 5, recessed in 2 and had already undergone maximal LR recession in 2. In all but the first 8 operations, temporary limbal sutures were passed through the eyelids to maximally adduct the globe post-operatively. At last follow-up (mean 24 months; range 2-130), the mean reduction in exodeviation was 49PD (range 10-80) and overall residual deviation was 26PD (range 80PD base-in to 14PD base-out). The 5 LR toxin procedures had a mean reduction of 22PD (range 10-40). Seven patients had persistent diplopia, one a transient corneal erosion and one caruncle suture exposure 4 years after surgery. CONCLUSION: Large-angle exodeviations can be markedly improved by bi-rectus fixation. This approach is both safe and effective and can be performed in complex patients with multiple previous procedures.

Retinopathy of prematurity treatment in the UK: trends in neonatal anaesthetic support and location of treatment from a national surveillance study.

The aim of this study is to evaluate current anaesthetic practice for retinopathy of prematurity (ROP) interventions in the UK. We collected the data from the 12-month prospective British Ophthalmic Surveillance Unit study carried out in 2013/2014 that were analysed with regard to type of anaesthesia used for primary ROP procedures and the hospital department in which treatment took place. A total of 327 cases of treated ROP from 55 different UK units were reported in the study. Type of anaesthesia used during treatment was available for 324 (99.1%) cases and the treatment location in 316 (96.6%). Overall, 266 (89.3%) laser treatments and 13 (50.0%) of primary intravitreal injections were performed with the neonate intubated, using intravenous sedation (IVS) in 158 (59.4%) and the remainder, under general anaesthesia (GA). Two hundred thirteen (67.4%) of all ROP procedures took place in the neonatal unit. GA was used in 98 (95.1%) of theatre cases compared with 19 (8.9%) of cases treated in the neonatal unit. Three (0.9%) neonates suffered significant respiratory distress during or immediately after laser treatment.Conclusion: This survey suggests that the preference in UK units is to undertake ROP laser treatment in the neonatal unit with the neonate intubated and sedated intravenously. Those babies treated in the operating theatre are more likely to receive GA. In the surveyed year, half of the neonates receiving intravitreal injections as sole primary therapy was intubated; the reason for this could not be elucidated from the responses. Adverse respiratory reactions during or after laser treatment affected fewer than 1% of the neonates in this study. What is Known: • Prior to the introduction of intravitreal anti-VEGF, almost all ROP treatments in the UK were performed under general anaesthetic (GA). • The technique of intravitreal injection is described using topical anaesthesia and was thought to be changing anaesthesia preferences for ROP treatment. What is New: • Half of the neonates receiving primary anti-VEGF injection in the UK were treated under intravenous sedation or GA. • The increasing use of primary anti-VEGF treatment has not influenced trends in anaesthetic practice in the UK since the last review 10 years ago.

Medical Management of Children With Congenital/Infantile Cataract Associated With Microphthalmia, Microcornea, or Persistent Fetal Vasculature.

PURPOSE: To report the surgical outcomes of children with cataract associated with microphthalmia, microcornea, or persistent fetal vasculature (MMP) and children with isolated cataract. METHODS: The study included 111 children (cataract associated with MMP: n = 25) who underwent cataract surgery at younger than 16 years. Exclusion criteria were duration of follow-up less than 5 years, intraindividual differences in age at surgery, eye disorders other than MMP, secondary cataract, and syndromal diseases. Main outcome measures were proportion of eyes with glaucoma and best corrected visual acuity (BCVA). Both groups were dichotomized by age at surgery (early intervention group: ≤ 48 days). Descriptive analysis was performed throughout. RESULTS: Median age at surgery was 3.9 months for cataract associated with MMP and 23.3 months for isolated cataract. The median (interquartile range [IQR]) duration of follow-up was 95.9 months (range: 76.0 to 154.5 months). In children with bilateral cataract associated with MMP, the proportion of eyes with final BCVA worse than 0.3 logMAR was similar regardless of age at surgery (early intervention: 80%, later intervention: 78%). In bilateral isolated cataract, the proportions were 56% and 33%, respectively. Children with cataract associated with MMP had a high prevalence of glaucoma (28%). Glaucoma prevalence was lower in the later intervention group. CONCLUSIONS: In the presence of MMP, early cataract surgery is associated with a high risk of post-lensectomy glaucoma, but does not offer the benefit of better visual outcomes. [J Pediatr Ophthalmol Strabismus. 2019;56(1):43-49.].

Botulinum toxin chemodenervation for childhood strabismus in England: National and local patterns of practice.

BACKGROUND: Botulinum toxin injection chemodenervation is a well-established intervention for adult strabismus, and has also been recognised as an effective alternative to routine incisional surgery for paediatric disease. We aimed to investigate the temporal patterns of practice, indications and outcomes of chemodenervation for paediatric strabismus at national and tertiary centre level. METHODS: Retrospective study using routinely collected patient data: Hospital Episode Statistics (HES) data were used to identify children undergoing non-incisional strabismus procedures in England from 2007 to 2016. Single-centre retrospective data on children undergoing botulinum toxin injections (Dysport® 2.5 units/ 0.1ml) as an isolated intervention (not involving incisional procedures) was undertaken to identify indications and outcomes. Successful outcome was defined as deviation <11 prism dioptres (PD). RESULTS: Between 2007 and 2016, there was no increase in the proportion of childhood strabismus involving non-incisional procedures. Amongst 150 children undergoing chemodenervation for strabismus within the tertiary centre, the most common diagnoses were acute onset esotropia (n = 34), infantile esotropia (n = 16) and consecutive exotropia (n = 15). Median age at injection was 8.5 years (range 0.9-15 years), and median follow up 12 months (6 months-11 years). Success rates differed by diagnosis, from 66% (non or partially accommodative esotropia) to 0% (congenital cranial disorders). Adverse events were seen in 62/150, 41%, most commonly transient ptosis (39%, n = 58). Overcorrection was seen in 14/119, 13%. Mild subconjunctival haemorrhage (n = 2) was the only other adverse event. CONCLUSIONS: Botulinum toxin for childhood strabismus has an acceptable safety profile, and considerable potential therapeutic benefit. However, nationally there has been no increased uptake of chemodenervation non-incisional procedures. Further prospective studies are necessary to understand the predictors of outcome within the separate clinical subgroups, to guide clinical decision making.

Mobile femtosecond laser platform for pediatric cataract surgery.

PURPOSE: To describe the use of a mobile femtosecond laser platform in assisting paediatric cataract surgery. METHODS: A mobile femtosecond laser was brought into the operating room and calibrated on the day of the surgery. After general anesthesia is induced, the femtosecond laser was docked onto the eyes with a liquid-filled interface, without any perioperative adaptations or additional surgical procedures. An anterior capsulotomy was created with the femtosecond laser, followed by conventional cataract extraction and intraocular lens implantation. RESULTS: Five eyes of 3 children with congenital cataracts were treated with this technique. Docking and capsulorhexis were successful in all cases. No perioperative or intraoperative complications were noted in any of the cases. At median follow-up of 15 months (range 6-18 months), all patients had improved best-corrected visual acuity. CONCLUSIONS: Using the mobile femtosecond laser platform, a perfectly sized anterior capsulotomy could be created with high precision and accuracy in paediatric cataract cases, while ensuring that perioperative care for the children undergoing the procedure was not compromised.

Treatment trends for retinopathy of prematurity in the UK: active surveillance study of infants at risk.

OBJECTIVES: To estimate the incidence of severe retinopathy of prematurity (ROP) requiring treatment and describe current treatment patterns in the UK. DESIGN: Nationwide population-based case ascertainment study via the British Ophthalmic Surveillance Unit and a national collaborative ROP special interest group. Practitioners completed a standardised case report form (CRF). SETTING: All paediatric ophthalmologists providing screening and/or treatment for retinopathy in the UK were invited to take part. PARTICIPANTS: Any baby with ROP treated or referred for treatment between 1 December 2013 and 30 November 2014, treated with laser, cryotherapy, vascular endothelial growth factor (VEGF) inhibitor or vitrectomy/scleral buckling, or a combination. MAIN OUTCOME MEASURE: Incidence of ROP requiring treatment. RESULTS: We received 370 CRFs; 327 were included. Denominator from epidemiological data: 8112 infants with birth weight of <1500 g. The incidence of ROP requiring treatment was 4% (327/8112, 95% CI 3.6% to 4.5%). Median gestational age was 25 weeks (IQR 24.3-26.1), and median birth weight 706 g (IQR 620-821). Median age at first treatment was 80 days (IQR 71-96). 204 right eyes (62.39%) had type 1 ROP, and 27 (8.26%) had aggressive posterior ROP. Infants were also treated for milder disease: 9 (2.75%) right eyes were treated for type 2 ROP, and 74 (22.63%) for disease milder than type 1 with plus or preplus, which we defined here as 'type 2 plus' disease. First-line treatment was diode laser photoablation of the avascular retina in 90.5% and injection of VEGF inhibitor in 8%. CONCLUSIONS: ROP treatment incidence in the UK is 2.5 times higher than previously estimated. 8% of treated infants receive intravitreal VEGF inhibitor, currently unlicensed. Research is needed urgently to establish safety and efficacy of this approach. Earlier treatment and increasing numbers of surviving premature infants require an increase in appropriate eye care facilities and staff. TRIAL REGISTRATION NUMBER: NCT02484989.

Is strabismus the only problem? Psychological issues surrounding strabismus surgery.

BACKGROUND: Strabismus, which has been estimated to occur in 4% of the adult population, can have a negative psychosocial effect on an individual's quality of life. While correction of strabismus may improve quality of life, this is not always the case, even if realignment surgery is clinically successful. Surgeons need to understand patients' expectations of postsurgical outcomes and the impact surgery has on their psychosocial well-being. METHODS: This was a prospective cross-sectional study of 220 adult patients undergoing strabismus surgery at Moorfields Eye Hospital NHS Foundation Trust. Clinical assessments were performed before surgery and again 2 weeks and 3 months after surgery. Standardized psychological questionnaires were completed preoperatively and 3 and 6 months postoperatively. RESULTS: Of 220 patients included in the study (mean age, 45 years), 54% were female, 81% were white, and 58.6% had a concomitant deviation. Prior to surgery, 24% of study participants were experiencing clinical levels of anxiety; 11% were suffering clinical levels of depression. Quality of life improved and anxiety and depression were reduced after surgery. Approximately 6% of the sample regretted having undergone surgery either at 3 or 6 months after treatment. CONCLUSIONS: It is important to identify patients who are experiencing significant psychosocial distress and to assess their postsurgical expectations in order to improve the outcomes of strabismus surgery. Presurgical psychosocial support should be considered for these patients.

What do patients with strabismus expect post surgery? The development and validation of a questionnaire.

AIMS: To develop and validate a short questionnaire to assess patients' expectations about outcomes post strabismus surgery. METHODS: Questionnaire items were extracted from previous literature and reviewed by a multidisciplinary team. A cross-sectional study was then undertaken with 220 adult patients due to undergo strabismus surgery. Participants completed the 17-item questionnaire. Scale structure was explored using principal component analysis (PCA), and the subscales analysed in relation to demographic and clinical characteristics and psychosocial well-being in order to establish validity. RESULTS: PCA revealed a 3-factor solution for the Expectations of Strabismus Surgery Questionnaire (ESSQ): (a) intimacy and appearance-related issues, (b) visual functioning, (c) social relationships. This 3-factor solution explained 59.30% of the overall variance in the ESSQ. Internal consistency, content and nomological and concurrent validity were considered acceptable. CONCLUSIONS: Patients with strabismus have high expectations about their postsurgical outcomes. This questionnaire provides a useful tool to assess the expectations patients have about their surgery, whether these expectations change over time and how they impact on postsurgical outcomes.

Prescribed computer games in addition to occlusion versus standard occlusion treatment for childhood amblyopia: a pilot randomised controlled trial.

BACKGROUND: Amblyopia ("lazy eye") is the commonest vision deficit in children. If not fully corrected by glasses, amblyopia is treated by patching or blurring the better-seeing eye. Compliance with patching is often poor. Computer-based activities are increasingly topical, both as an adjunct to standard treatment and as a platform for novel treatments. Acceptability by families has not been explored, and feasibility of a randomised controlled trial (RCT) using computer games in terms of recruitment and treatment acceptability is uncertain. METHODS: We carried out a pilot RCT to test whether computer-based activities are acceptable and accessible to families and to test trial methods such as recruitment and retention rates, randomisation, trial-specific data collection tools and analysis. The trial had three arms: standard near activity advice, Eye Five, a package developed for children with amblyopia, and an off-the-shelf handheld games console with pre-installed games. We enrolled 60 children age 3-8 years with moderate or severe amblyopia after completion of optical treatment. RESULTS: This trial was registered as UKCRN-ID 11074. Pre-screening of 3600 medical notes identified 189 potentially eligible children, of whom 60 remained eligible after optical treatment, and were enrolled between April 2012 and March 2013. One participant was randomised twice and withdrawn from the study. Of the 58 remaining, 37 were boys. The mean (SD) age was 4.6 (1.7) years. Thirty-seven had moderate and 21 severe amblyopia. Three participants were withdrawn at week 6, and in total, four were lost to follow-up at week 12. Most children and parents/carers found the study procedures, i.e. occlusion treatment, usage of the allocated near activity and completion of a study diary, easy. The prescribed cumulative dose of near activity was 84 h at 12 weeks. Reported near activity usage numbers were close to prescribed numbers in moderate amblyopes (94 % of prescribed) but markedly less in severe amblyopes (64 %). Reported occlusion usage at 12 weeks was 90 % of prescribed dose for moderate and 33 % for severe amblyopes. CONCLUSIONS: Computer-based games and activities appear acceptable to families as part of their child's amblyopia treatment. Trial methods were appropriate and accepted by families.

Factors associated with quality of life and mood in adults with strabismus.

BACKGROUND/AIMS: To explore the factors associated with the mood and quality of life (QoL) of patients with strabismus due to undergo realignment surgery. METHODS: A cross-sectional study was undertaken with adult patients. Along with demographic, clinical and psychosocial process variables, the Hospital Anxiety and Depression Scale and AS-20 QoL measures were administered. Regression models were used to identify the factors associated with QoL and mood. RESULTS: Of the 220 participants, 11% were experiencing clinical levels of depression, and 24% clinical anxiety. This is in line with other forms of facial disfigurement but higher than other chronic diseases. Although mood and QoL were associated with age and diplopia, it was beliefs and cognitions which were more consistently associated with well-being. This included feelings of social anxiety and avoidance, a belief that strabismus has negative consequences, poor understanding of strabismus, social support, fear of negative evaluation and the perceived visibility of their condition. CONCLUSIONS: Psychosocial rather than clinical characteristics were identified as determinants of well-being in this population. It is important for clinicians planning surgery to be aware of these factors which could influence outcomes. Longitudinal studies need to be conducted to explore the direction of causality before interventions to improve well-being are developed and evaluated.

The impact of strabismus on quality of life in adults with and without diplopia: a systematic review.

Strabismus affects approximately 4% of the adult population and can cause substantial physical disturbance and changes to appearance. This article aims to examine the impact of strabismus in adults both with and without diplopia, focusing primarily on quality of life (QoL). We highlight the value of measuring QoL, assess the ways in which it can be measured, and the impact the disease, diplopia, and surgery have on the patient. QoL differs for strabismus patients based on their diplopia status. Patients with diplopia tend to have more concerns relating to functional QoL, whereas patients without diplopia have primarily psychosocial concerns. Two diplopia-specific questionnaires have been designed to assess QoL and the perceived severity of symptoms. Further research is needed to identify the variables which influence QoL so that appropriate support can be given to all patients with strabismus to improve their QoL.

Role of inferior rectus botulinum toxin injection in vertical strabismus resulting from orbital pathology.

INTRODUCTION: We describe the uses and efficacy of botulinum toxin injection to the inferior rectus muscle in vertical strabismus resulting from orbital pathology. METHODS: A retrospective review of patients undergoing inferior rectus botulinum toxin injection between 1982 and 2006 for vertical strabismus due to orbital pathology. RESULTS: There were 13 patients identified; 6 with idiopathic orbital inflammatory syndrome (IOIS) including myositis, 3 with previous orbital wall fractures, 1 post-optic nerve sheath Schwannoma resection, 1 with lymphoma, 1 with metastasis, and 1 post-superior ophthalmic vein hemorrhage. A beneficial effect with inferior rectus botulinum toxin was obtained in 9/13 (69%) patients (mean follow-up 14 months). Resolution or improvement occurred in 4/6 (67%) patients with IOIS. Resolution was also obtained in the patient with previous optic nerve sheath Schwannoma resection, and the patient who sustained the superior ophthalmic vein hemorrhage. Improvement was noted in both the patient with lymphoma and in the patient with a metastasis. However, in patients with orbital wall fractures, only 1 of 3 patients obtained improvement. Benefit appeared independent of the size of vertical deviation, with 4/6 (67%) showing improvement with a pre-toxin angle 4-12 PD, and 5/7 (71%) improving with a pre-toxin angle 16-25 PD. Eleven patients received only 1 injection, one patient received 2, and another had an ongoing course of 8 injections. DISCUSSION: Vertical strabismus secondary to a range of orbital conditions, particularly inflammatory, often can be successfully managed by inferior rectus botulinum toxin injections.

The use of amniotic membrane for the management of fibrosis in complex strabismus surgery.

ABSTRACT Postoperative adhesion formation following complex strabismus surgery limits surgical outcomes. The benefits of amniotic membrane transplant (AMT) are widely recognized in ophthalmology. There are only published isolated case reports regarding its use in strabismus surgery. The limitation of these reports is that they describe use of AMT in cases of restrictive strabismus resulting from periocular surgery and indirect involvement of the extraocular muscles. In our series, the amniotic membrane was used to manage complex strabismus surgery. In this retrospective case series we describe surgical management with AMT in 8 strabismus cases between 2007 and 2010 in a tertiary referral center. Of these, 6 cases resulted from complex and repeat strabismus procedures to the extraocular muscles and 2 cases were a result of periocular surgery. Descriptive methodology was used. Outcomes examined were postoperative objective improvement in angle of deviation, subjective improvement in patient symptoms, and need for further interventions. Mean follow-up period was 15 months. All patients had multiple strabismus surgery in the past with a mean number of 3.2 ± 1.5 procedures (range 1-5). The indication for surgery using amniotic membrane graft was in 7 cases a large hypotropia with inferior conjunctival fibrosis and in one case a large consecutive horizontal deviation. The mean angle of vertical deviation was reduced from 32.1 ± 13 prism diopters (PD) preoperatively to 19.75 ± 11.4 PD postoperatively, with better outcomes in cases where AMT was used early in the surgical plan. Six out of the eight cases had objective improvement also consistent with improvement in the binocular field of vision. This is the largest reported case series on amniotic membrane grafts for strabismus surgery complicated by fibrosis and especially cases of consecutive hypotropia. Our outcomes indicate that AMT is beneficial, but we suggest it be used in the early stages of the surgical management of these cases.

Relationships between maternal ethnicity, gestational age, birth weight, weight gain, and severe retinopathy of prematurity.

OBJECTIVE: To develop an algorithm that allows advanced identification of infants requiring treatment for retinopathy of prematurity (ROP). STUDY DESIGN: A retrospective observational study was performed at 2 tertiary neonatal units serving a multiethnic population in the UK, using data on 929 infants eligible for ROP screening. The relationships between study variables and the risk of developing ROP requiring treatment were analyzed using multiple logistic regression. RESULTS: After applying exclusion criteria, data from 589 infants were analyzed; of these, 57 required laser treatment. The proportion of treated infants was 5.9% of those born to black mothers, 9.39% of those born to white mothers, and 12.8% of those born to Asian mothers (P = .047). Multiple logistic regression showed that gestational age, birth weight, maternal ethnicity, and early weight gain were predictors for the development of ROP requiring treatment, with maternal ethnicity having greater predictive power compared with early weight gain. We developed an algorithm for predicting the development of ROP requiring treatment with sensitivity, specificity, and positive and negative predictive values of 100%, 65.7%, 23.8%, and 100%, respectively. CONCLUSION: Gestational age, birth weight, early weight gain, and maternal ethnicity are important predictors for the development of ROP requiring treatment. In a multiethnic population, an algorithm to predict development of ROP requiring treatment should include maternal ethnicity. If confirmed through prospective studies, this algorithm could reduce the number of opthalmologic examinations performed for ROP screening.

Prevalence of retinal hemorrhages in critically ill children.

BACKGROUND: Retinal hemorrhages (RHs) with encephalopathy and subdural bleeding are considered suggestive of abusive head trauma (AHT). Existing studies describing RH focus on AHT and have potential selection bias. We undertook a prospective observational study to define the prevalence, distribution, and extent of RH in critically ill children. METHODS: From February 2008 to December 2009, emergency intensive care admissions ≥ 6 weeks of age underwent dilated retinal examination by either a pediatric ophthalmologist or RetCam (retinal photograph) imaging after written informed consent. Patients with suspected or proven AHT, penetrating eye trauma, and elective admissions were excluded. RESULTS: The prevalence of RH was 15.1% (24/159; 95% confidence interval [CI]: 9.5%-21%); 16/24 (66%) mild, and 2/24 (8%) moderate. Severe multilayered RH were seen in only 6 patients (3.7%), 3 with myeloid leukemia and sepsis, 2 with severe accidental head injury, and 1 with severe coagulopathy secondary to late onset hemorrhagic disease of newborn. There was no detectable impact of age, gender, seizures, coagulopathy or cardiopulmonary resuscitation on prevalence of severe multilayered RH; however, sepsis (odds ratio: 3.2; 95% CI: 1.3-8.0, P = .018) and coagulopathy (odds ratio: 2.8; 95% CI: 1.2-7.0, P = .025) were significantly associated with any RH. Only admission diagnosis was independently associated with severe multilayered RH on logistic regression. CONCLUSIONS: RHs were seen in critically ill children with a prevalence of 15.1% (24/159); however, most were mild. Severe multilayered RH resembling those described in AHT were rare (6/24) and were only seen in patients with fatal accidental trauma, severe coagulopathy, sepsis with myeloid leukemia, or a combination of these factors.