USE OF HAND-HELD OPTICAL COHERENCE TOMOGRAPHY DURING RETINOPATHY OF PREMATURITY SCREENING DEMONSTRATES AN INCREASED OUTER RETINA FROM EARLY POSTMENSTRUAL AGE IN PRETERM INFANTS WITH RETINOPATHY OF PREMATURITY.

PURPOSE: To identify structural markers of active retinopathy of prematurity (ROP) in foveal and parafoveal retinal layers using hand-held optical coherence tomography. METHODS: Hand-held optical coherence tomography images (n = 278) were acquired from a prospective mixed cross-sectional longitudinal observational study of 87 participants (23-36 weeks gestational age; n = 30 with ROP, n = 57 without ROP) between 31 and 44 weeks postmenstrual age excluding treated ROP and features of cystoid macular edema. Six retinal layer thicknesses from the fovea to the parafovea were analyzed at five locations up to 1,000 µ m, temporally and nasally. RESULTS: The mean outer retinal thickness during active ROP increased at the fovea and parafovea from postmenstrual age 33 weeks to 39 weeks ( P < 0.001), whereas the parafoveal inner nuclear layer and retinal nerve fiber layer reduced ( P < 0.001). Outer retinal thickness at the fovea from 33 weeks to 39 weeks postmenstrual age was consistently thicker in infants with ROP across all levels of prematurity (gestational age). CONCLUSION: Increased foveal and parafoveal outer retina measured using hand-held optical coherence tomography shows potential as a marker for ROP screening.

POTENTIAL UTILITY OF FOVEAL MORPHOLOGY IN PRETERM INFANTS MEASURED USING HAND-HELD OPTICAL COHERENCE TOMOGRAPHY IN RETINOPATHY OF PREMATURITY SCREENING.

PURPOSE: To investigate dynamic foveal morphology with postmenstrual age, in preterm infants with and without retinopathy of prematurity using hand-held optical coherence tomography, adjusting for gestational age (GA) and birthweight (BW). METHODS: Prospective mixed cross-sectional/longitudinal observational study of 87 participants (23-36 weeks GA; n = 30 with, n = 57 without retinopathy of prematurity) using hand-held optical coherence tomography images (n = 278) acquired between 31 weeks and 44 weeks postmenstrual age excluding treated retinopathy of prematurity. Measurements included foveal width, area, depth, central foveal thickness, maximum slope, and parafoveal retinal thickness at 1,000 µm nasal and temporal to the central fovea. RESULTS: Retinopathy of prematurity was significantly correlated with only foveal width in either GA or BW adjusted statistical models. In contrast, severity of prematurity (GA, BW) correlated with foveal area (P < 0.005), depth (P ≤ 0.001), and slope (P < 0.01), although central foveal thickness (P = 0.007) and parafoveal retinal thickness (P < 0.001) correlated with GA, but not with BW. CONCLUSION: Foveal width is independent of GA and BW with potential in retinopathy of prematurity screening assessment using hand-held optical coherence tomography. Foveal morphology could be graded in prematurity during development, with possible implications for future management of preterm infants.

Retinal development in albinism: a prospective study using optical coherence tomography in infants and young children.

BACKGROUND: Retinal development normally involves migration of the inner retinal layers away from the fovea, migration of the cone photoreceptors into the fovea, and elongation of the photoreceptors over time. This process is arrested prematurely in albinism. However, because retinal development continues at least until the age of 4 years, when development arrests in albinism is uncertain. In this study we outlined the time course of retinal development in children with albinism. METHODS: We studied 44 children with a diagnosis of albinism and 223 control participants. All participants were aged between 0 and 6 years. We obtained 219 mixed cross-sectional and longitudinal optical coherence tomography examinations in the albinism group and compared them with 558 control examinations. Retinal layer segmentation was performed with ImageJ software. Generalised linear mixed regression modelling was used to analyse group differences in retinal development. FINDINGS: In the albinism group, inner retinal layer migration from the fovea was delayed and arrested prematurely, resulting in a significantly thicker central macular thickness than in the control group (p<0·0001). Whereas the central macular thickness increased with age in the control group, in the albinism group it initially decreased with age as a result of continuing regression of the inner retinal layers (p=0·041). The perifoveal retinal thickness was significantly decreased in albinism from a reduction of both inner (p<0·0001) and outer (p<0·0001) retinal layer thicknesses. There was evidence that the photoreceptor layers across the fovea were elongating in albinism, albeit at a reduced rate, compared with the control group. This difference was most apparent for the foveal photoreceptor inner segment (p=0·001). INTERPRETATION: Our findings show that perturbations exist in several aspects of retinal development including the migration and differentiation of the neuronal cells within the retina. We showed continuing regression of the inner retinal layers and elongation of the photoreceptor layers suggesting residual plasticity of the developing albino retina. This finding is important because treatment at the earliest stages of the condition might normalise retinal development and optimise vision. FUNDING: UK Medical Research Council (grant number MR/J004189/1), Ulverscroft Foundation, National Eye Research Centre, Nystagmus Network UK.

Predicting outcome in acute heart failure, does it matter?

BACKGROUND: Acute heart failure (AHF) is a cardiac condition with multiple aetiologies that carries a high fatality rate. The development of heart failure following a coronary event has a poor prognosis. The main aim of this study was to predict in-hospital and long-term prognosis of AHF based on co-morbidities, physical and paraclinical findings at the time of admission. METHODS: The charts from 366 AHF patients, admitted for the first time during a 4-year period at a major university hospital, were reviewed. In addition to the demographic information, a comprehensive social and medical history was obtained. Presenting symptoms and physical findings were also recorded along with admission values of laboratory and myocardial injury markers. Echocardiographic examination was performed and recorded. Angiographic findings were only recorded in patients with acute coronary ischaemia. Univariate and multivariate analyses were performed to examine the relative contribution of clinical factors on in-hospital and long-term mortality. RESULTS: Preceding acute coronary syndrome (STEMI/NSTEMI) was the strongest predictor of in-hospital death for AHF (OR 3.74; 1.94-7.22, P = 0.001). Multivariate binary logistic regression identified that older age, female gender presence of acute myocardial infarction (higher cTNI or higher CKMB levels), lower blood pressure, lower LVEF and higher WBC were independently associated with long-term mortality. CONCLUSION: AHF following an acute coronary syndrome carries a higher fatality rate. Higher levels of cardiac injury markers and white blood cell counts may present as a poor prognostic indicator secondary to an acute ischaemic event.

Severity of cystoid macular oedema in preterm infants observed using hand-held spectral domain optical coherence tomography improves weekly with postmenstrual age.

OBJECTIVE: To investigate the relationship between cystoid macular oedema (CMO) measured in preterm infants using hand-held spectral domain optical coherence tomography (HH SD-OCT), with gestational age at birth (GA), birthweight (BW), diagnosis of retinopathy of prematurity (ROP) and the presence or absence of the external limiting membrane (ELM). METHODS: We conducted a prospective mixed cross-sectional/longitudinal observational study of 112 participants (23 to 36 weeks GA; n = 25 with, and n = 87 without, CMO). Retinal images were acquired using 344 HH SD-OCT (n = 66 with and n = 278 without, CMO) between 31 to 44 weeks postmenstrual age (PMA). CMO type ('fovea' and 'dome') was measured using thickness, width, area and peak. RESULTS: CMO was observed in 22.9% of preterm infants, and 19.2% of images. The mean values for  thickness, width, area and peak of 'dome' CMO were 128.47 µm (SD +/- 34.23), 3624.45 µm (SD +/- 1323.03), 0.49 mm2 (SD +/- 0.28) and 279.81 µm (SD +/- 13.57) respectively. The mean values for  thickness, width, area and peak of 'fovea' CMO were 64.37 µm (SD +/- 17.11), 2226.28 µm (SD +/- 1123.82), 0.16 mm2 (SD +/- 0.11) and 95.03 µm (SD +/- 26.99) respectively. Thickness, area width and peak were significantly greater for 'dome CMO compared with 'fovea' CMO (P < 0.0001 for thickness, area and peak; P < 0.01 for width). Area and width significantly decreased with PMA for 'dome' and 'fovea' CMO (p = 0.0028; p < 0.001 respectively). No association was found between the presence of ROP and the detection of CMO or detection of CMO with absence of ELM. CONCLUSIONS: HH -OCT in preterm infants demonstrates that the severity of CMO appearance improves each week for both fovea and dome CMO.

An unusual case of keratoconus.

We report a rare case of severe keratoconus and acute corneal hydrops in a 6-year-old girl. We describe the management of this challenging case and the outcome.

Fourth cranial nerve palsy and bilateral acute retinal necrosis following human herpesvirus 6 infection of the central nervous system.

Acute retinal necrosis (ARN) is a rare, potentially blinding condition typically affecting immunocompetent individuals. It is defined by the clinical triad of vitreous inflammation, occlusive vasculopathy, and progressive retinal necrosis, usually located in peripheral retina with circumferential extension. Varicella zoster virus (VZV), herpes simplex virus (HSV), Epstein-Barr virus (EBV) and occasionally cytomegalovirus (CMV) are the common causative agents of ARN. Reports of human herpesvirus 6 (HHV6) infection of the central nervous system (CNS) associated with ocular inflammatory disease are extremely rare. We here report the case of a 22-year-old immunocompetent male who presented with acute bilateral ARN and fourth nerve palsy, following HHV6 infection of the CNS and EBV infectious mononucleosis.

Primary trabeculodysgenesis in association with neonatal Marfan syndrome.

We present the clinical and ophthalmological findings in two infants with neonatal Marfan syndrome (nMFS) and primary trabeculodysgenesis (PT). Fibrillin 1 (FBN1) mutations were confirmed in both cases. Numerous eye anomalies have been recognized in infants with nMFS, but PT has not been reported previously. Our report expands the phenotype of nMFS, and highlights the importance of early and careful ophthalmological assessment of these infants.