Peripheral Retinal Haemorrhages in a Patient with MOG-Associated Optic Neuritis.

A 15-year-old female presented with headaches and bilateral vision loss. Fundoscopic examination revealed bilateral optic nerve oedema as well as peripheral retinal haemorrhages. Magnetic resonance imaging of the brain showed findings consistent with bilateral optic neuritis. The patient was started on high dose intravenous corticosteroids but her vision failed to improve. The presence of retinal haemorrhages raised concern that a vasculitis was underlying her symptoms, prompting an extensive work-up, which was unrevealing. Plasmapheresis was initiated and the patient's vision eventually improved to 20/20 in both eyes. Ultimately, she was found to be positive for myelin oligodendrocyte glycoprotein (MOG) antibodies, consistent with a diagnosis of MOG-associated optic neuritis. The patient's course was typical for MOG-associated optic neuritis but her peripheral retinal haemorrhages were atypical, which created diagnostic uncertainty. It is important to be aware of the possibility of retinal findings in this disease. We also review potential causes for retinal haemorrhages in optic neuritis.

Retinal haemorrhage in infants investigated for suspected maltreatment is strongly correlated with intracranial pathology.

AIM: To test the two prevailing hypotheses regarding the aetiology of infant retinal haemorrhage: (a) traction forces exerted by the lens and/or corpus vitreum on the retina during infant shaking or (b) retinal vessel leakage secondary to intracranial pathology and raised intracranial pressure. METHODS: Comparison of medical findings and reported type of trauma in infants investigated for suspected physical abuse with presence (n = 29) or non-presence of retinal haemorrhage (RH) (n = 119). RESULTS: Intracranial pathology was recorded in 15 (13%) of the non-RH cases and in 27 (97%) of the RH cases (p < 0.0001). All 18 infants with bilateral RH had intracranial pathology. Of 27 infants subjected to witnessed or admitted shaking, two were in the group with RH. One had a single unilateral RH and no intracranial pathology. The other had bilateral RH and intracranial pathology with non-specific white matter changes, acute subdural and subarachnoid haemorrhages, and suspected cortical venous thrombosis. In 15 RH cases, there was no trauma reported and no findings other than RH and intracranial pathology. Accidental blunt head trauma was reported in 7 RH cases. CONCLUSION: The present study indicates that RH in infants is secondary to intracranial pathology of non-specific aetiology.

Dengue induced reversible blindness.

Dengue is spread by the bite of infected Aedes aegypti mosquito. It is usually a self-limiting viral infection but sometimes complicates to mortality. In the last few decades, literature has shown that clinical and biochemical profile of dengue is expanding due to the addition of unusual manifestation day by day. Hereby, we report a young male student suffering from dengue complicated to retinal hemorrhage and severe pancytopenia who recovered near fully on treatment.

Traumatic shaking: The role of the triad in medical investigations of suspected traumatic shaking.

The Swedish Agency for Health Technology Assessment and Assesment of Social Services (SBU) is an independent national authority, tasked by the government with assessing methods used in health, medical and dental services and social service interventions from a broad perspective, covering medical, economic, ethical and social aspects. The language in SBU's reports are adjusted to a wide audience. SBU's Board of Directors has approved the conclusions in this report. The systematic review showed the following graded results: There is limited scientific evidence that the triad (Three components of a whole. The triad associated with SBS usually comprises subdural haematoma, retinal haemorrhages and encephalopathy.) and therefore, its components can be associated with traumatic shaking (low-quality evidence). There is insufficient scientific evidence on which to assess the diagnostic accuracy of the triad in identifying traumatic shaking (very low-quality evidence). Limited scientific evidence (low-quality evidence) represents a combined assessment of studies of high or moderate quality which disclose factors that markedly weaken the evidence. It is important to note that limited scientific evidence for the reliability of a method or an effect does not imply complete lack of scientific support. Insufficient scientific evidence (very low-quality evidence) represents either a lack of studies or situations when available studies are of low quality or show contradictory results. Evaluation of the evidence was not based on formal grading of the evidence according to GRADE but on an evaluation of the total scientific basis.

Insufficient evidence for 'shaken baby syndrome' - a systematic review.

Shaken baby syndrome has typically been associated with findings of subdural haematoma, retinal haemorrhages and encephalopathy, which are referred to as the triad. During the last decade, however, the certainty with which the triad can indicate that an infant has been violently shaken has been increasingly questioned. The aim of this study was to determine the diagnostic accuracy of the triad in detecting that an infant had been shaken. The literature search was performed using PubMed, Embase and the Cochrane Library up to October 15, 2015. Relevant publications were assessed for the risk of bias using the QUADAS tool and were classified as having a low, moderate or high risk of bias according to predefined criteria. The reference standards were confessions or witnessed cases of shaking or accidents. The search generated 3773 abstracts, 1064 were assessed as possibly relevant and read as full texts, and 30 studies were ultimately included. Of these, 28 were assessed as having a high risk of bias, which was associated with methodological shortcomings as well as circular reasoning when classifying shaken baby cases and controls. The two studies with a moderate risk of bias used confessions and convictions when classifying shaken baby cases, but their different designs made a meta-analysis impossible. None of the studies had a low risk of bias. CONCLUSION: The systematic review indicates that there is insufficient scientific evidence on which to assess the diagnostic accuracy of the triad in identifying traumatic shaking (very low-quality evidence). It was also demonstrated that there is limited scientific evidence that the triad and therefore its components can be associated with traumatic shaking (low-quality evidence).

Recognition and nursing management of abusive head trauma in children.

Abusive head trauma (AHT) describes an injury to the head caused by a deliberate impact or shaking by a parent or carer. It can cause significant morbidity and mortality in infants, and is most commonly seen in those aged under 2 years. The initial presentation of AHT can include vague symptoms and the correct diagnosis may be missed by health professionals. Subdural haematoma, brain oedema and retinal haemorrhages are well-known features associated with AHT. However, other conditions such as birth trauma, accidental falls in infants and bleeding disorders can all mimic AHT, thus making its recognition difficult. Suspicion of AHT should lead to initiation of safeguarding procedures alongside organising neurological imaging to identify skull fracture and/or intracranial lesions. This article highlights different aspects of the clinical presentation of AHT and its management. Safeguarding and recognising child abuse is vital and requires every member of the multidisciplinary team to remain vigilant. An illustrative case study is included to highlight some of the challenges that health professionals working in different clinical set-ups are likely to come across while managing an infant with AHT.

Angiographic features and disease outcomes of symptomatic retinal arterial macroaneurysms.

PURPOSE: Symptomatic retinal arterial macroaneurysms (RAM) are primarily investigated by fundus fluorescein angiography after presenting with visual disturbance. The natural history includes spontaneous regression and occasionally occlusion of the arteriole distal to the aneurysm. RAM may be managed conservatively. Interventional treatment options include focal argon laser photocoagulation, Nd:YAG laser hyaloidotomy, and pars plana vitrectomy. The purpose of this study was to elicit the rates of distal vessel occlusion and aneurysm thrombosis in RAM at presentation, and their relevance to the treatment of RAM. Furthermore, visual outcomes were examined. METHODS: Retrospective review of cases of RAM presenting to a tertiary ophthalmology care centre was accomplished in a university teaching hospital. The angiographic features, treatment indications, and visual outcomes in patients with RAM were recorded. Angiographic features noted were distal vessel patency and aneurysm thrombosis at presentation. RESULTS: Ten patients with RAM were identified. Ninety percent had an angiographically patent distal arteriole, with 40 % showing spontaneous thrombosis of the aneurysm sac at presentation. Patients presenting with a spontaneously thrombosed RAM were managed conservatively, those with flow within the aneurysm wall were treated with focal laser, and those with subhyaloid haemorrhage underwent Nd:YAG laser hyaloidotomy. LogMAR visual acuity improved from 0.3 (±0) at presentation to 0.15 (±0.1) in the conservative group, and from 0.78 (±0.23) to 0.24 (±0.18) in those who underwent one intervention. One patient lost vision after multiple RAM. CONCLUSION: Thrombosis within the aneurysm wall is an important feature in deciding to treat RAM, and selective use of interventions improves vision in affected patients.

Approach to the management of pregnancy in patients with pseudoxanthoma elasticum: a review.

Management of pregnancy in patients with rare diseases is often guided by incomplete knowledge because of a lack of high-quality case control studies or single-centre experience. Pseudoxanthoma elasticum (PXE) is an autosomal recessive metabolic disorder that results in calcification of elastic fibres of the skin, retina, and arteries, leading to skin lesions, eventual central visual loss, and potential arterial insufficiency in most patients. It is due to mutations in ABCC6, which encodes the eponymous membrane transport protein. We review the literature on pregnancy in PXE, including the effects of the diseases on pregnancy and its complications, the effect of PXE on the foetus, and the effects of pregnancy on PXE, and conclude that in the majority of pregnancies in women with PXE, the outcome for mother, baby, and the disease is uneventful. We also provide recommendations for managing pregnancy in PXE.

Retinal haemorrhages associated with fatal paediatric infections.

For many physicians, retinal haemorrhages (RHs) in infants and young children remain highly diagnostic of non-accidental (abusive) head trauma. Because clinicians have applied indirect ophthalmoscopy selectively to cases of suspected child abuse, the association between RH and other conditions such as infection, coagulopathy and accidental trauma has encountered habitual bias, creating the potential for iatrogenic misdiagnosis of child abuse. We present an autopsy case series of four children, aged three years old or younger, in whom RHs were detected by post-mortem monocular indirect ophthalmoscopy after the patients had died from infections. We discuss the laterality, number, type and location of RHs in these cases, and summarize proposed mechanisms of RH formation in fatalities from paediatric infection. We demonstrate that many of the ophthalmological findings that have been considered diagnostic of abusive head trauma can also occur in association with infective processes.

Masquerade of Leukemic Infiltration With Cerebral Sinus Venous Thrombosis Causing Papilledema by Asparaginase Therapy.

Acute lymphoblastic leukemia (ALL) is a hematological cancer that can cause ocular tissue involvement. Asparaginase is a chemotherapy regimen that is commonly used in leukemia which could lead to similar ocular manifestations. We report a patient with a history of ALL for seven months on asparaginase therapy and persistent cerebral sinus venous thrombosis (CSVT) with acute venous infarction in the left frontal lobe presented with worsening vision. On examination, he had a visual acuity (VA) of (6/21) in the right eye and (6/60) in the left eye, with a mild left eye abduction limitation. Fundal examination showed bilateral prominent multilayered retinal hemorrhages and papilledema with absence of leukemic infiltration. His chemotherapy regimen was held and a one month follow up was scheduled. Follow up after one month of chemotherapy cessation showed resolution of both VA and fundal exam findings. It is crucial to differentiate between asparaginase toxicity and infiltration of the disease in ALL patients. As this would determine whether the treatment should be continued or suspended.

Bacillary Layer Detachment in Neovascular Age-Related Macular Degeneration: Case Series.

PURPOSE: This study seeks to report the clinical and multimodal imaging findings of eight eyes of seven patients with neovascular age-related macular degeneration (nAMD) who developed bacillary layer detachment (BALAD). Setting/Venue: The patients were analysed at the Western Eye Hospital in London, UK. METHODS: The approaches of this research include clinical examinations and multimodal imaging-based description of cases of nAMD with BALAD. RESULTS: We report multimodal imaging findings of bacillary layer detachment (BALAD) in patients with nAMD. CONCLUSIONS: A bacillary layer detachment was detected in patients with neovascular age-related macular degeneration. This multimodal imaging finding is not commonly described in the literature for this disease.

Predictive value of non-ocular findings for retinal haemorrhage in children evaluated for non-accidental trauma.

PURPOSE: To determine non-ocular examination findings in non-accidental trauma (NAT) patients that are associated with retinal haemorrhage and warrant urgent examination by an ophthalmologist. METHODS: A hospital- and clinic-based retrospective cross-sectional and cohort study of children age 0-12 years who underwent workup for NAT over a span of nine years in a level I trauma and tertiary referral centre. Details of ocular and non-ocular examination and imaging findings at the time of NAT evaluation were collected by chart review. Univariate and logistic regression analysis for association between retinal haemorrhage and non-ocular examination findings was performed. RESULTS: A total of 557 patients with ophthalmology evaluation and 425 without were included in this study. All ages combined, none of the cutaneous signs of trauma or non-skull fractures were associated with retinal haemorrhage on univariate analysis (p > 0.05). By logistic regression, subdural or extra-axial haemorrhage (OR = 16.2; 95% CI [5.11-51.3]), occipital lobe insult (OR = 6.2; 95% CI [1.77-21.6]) and Glasgow coma score (GCS) <15 (OR = 5.8; 95% CI [1.96-17.4]) were significant predictors of retinal haemorrhage. CONCLUSION: Subdural or undistinguished extra-axial haemorrhage, GCS <15, and occipital lobe insult are risk factors for the presence of retinal haemorrhage in patients with suspected NAT and their presence warrant urgent dilated fundus examination by an ophthalmologist. The presence of cutaneous trauma or non-skull bone fractures without the aforementioned risk factors does not warrant ophthalmology evaluation.

Change in Incidence and Severity of Abusive Head Trauma in the Paediatric Age Group Pre- and During COVID-19 Lockdown in the North East of England.

Background: Abusive head trauma (AHT) is currently the accepted terminology that encompasses previously used terms such as non-accidental injury (NAI) or non-accidental head injury (NAHI) and shaken baby syndrome (SBS). It is AHT and its ocular manifestations that ophthalmologists are vital in identifying and reporting. Objectives: To investigate whether there is a change in the incidence or severity of AHT pre- and during COVID-19 lockdown. Participants and Settings: AHT cases reported between March-June 2019 and March-June 2020. Data will be collected from ***** **** ********* NHS Foundation Trust. Methods: A retrospective comparative study. Main Outcome Measures: Comparison of total number of children reported to child protection services pre- and during lockdown.Severity of reported cases.Ophthalmic involvement. Results: Of the pre-lockdown safeguarding referrals, 5/61 (8.19%) had confirmed AHT, and 4/40 (10%) of the during lockdown group were confirmed AHT. The absence of teachers was evident, as in the pre-lockdown group 40% (2) of referrals originated from schools compared to none during the lockdown period. Ophthalmic involvement was not present in any of the pre-lockdown cases and only 50% (2) of the during lockdown cases, with the appropriate proforma only used in one of these cases. Unfortunately, no further statistical testing was meaningful in light of the small sample size. Conclusions: The loss of the early warning detection mechanism provided by schools and health visitors may have contributed to both the change in presentation and severity of cases during the lockdown. There is also a need for ophthalmology and paediatrics to collaborate to ensure AHT cases are thoroughly investigated and documented.

Isolated Ocular Manifestations in Chronic Myeloid Leukaemia.

BACKGROUND: Chronic myeloid leukaemia (CML) presenting with only ocular manifestations either at the initial stage of diagnosis or at relapse is uncommon. We report two cases of CML presenting with isolated visual symptoms. CASE SERIES: The first case is a 21-year-old healthy gentleman who presented with left eye painless loss of vision for a one-week duration. Visual acuity was 6/60 in the left eye and 6/6 in the right eye. There were scattered retinal haemorrhages in both eyes and a sub-macular bleed over the left eye. The full blood count revealed a high white cell count of 134.6 × 109/L. Peripheral blood smear showed hyper-leucocytosis with absolute eosinophilia and basophilia and the presence of blasts suggestive of CML thus chemotherapy was commenced. The second case is a 28-year-old in haematological, molecular, and cytogenic remission from CML for the past two years, presented with left eye painless vision loss for five days duration. Vision in the left eye was counting fingers. There was a large subretinal mass involving the left optic disc. Magnetic resonance imaging of the brain and orbit showed an elliptical orbital mass at the left globe posteriorly with diffuse thickening of the optic nerve. The patient was diagnosed as CML relapsed to the left optic nerve. He underwent intrathecal chemotherapy and orbital irradiation. CONCLUSION: Both these cases are unique since the manifestation of CML was with only ocular features at the time of presentation as per in the first case during the initial diagnosis and in the second case during relapse. This highlights that it is evident that the knowledge of ocular involvement in leukaemia is crucial since the eye is the only organ where leukemic infiltration to nerves and blood vessels can be observed directly. Recognizing fundus changes in leukaemia allows earlier diagnosis and prompt treatment. These case reports highlight the importance of recognizing early fundus changes, which should allow earlier diagnosis and treatment.

Purtscher-like retinopathy after a stent implant for the treatment of carotid aneurysm.

Purtscher retinopathy is an occlusive microangiopathy associated with traumatic causes and usually causes visual impairment. For non-traumatic origins, it is called Purtscher-like retinopathy (PLR). A 26-year-old man reported loss of vision in his left eye after the implantation of a telescoping stent for the treatment of an intracranial carotid aneurysm. The fundus of the eye showed numerous cotton-wool spots and superficial haemorrhages. The spontaneous evolution was favourable, with almost complete resolution, except for an inferior residual scotoma. The appearance of PLR may be associated with the implantation of an endovascular stent for the treatment of intracranial aneurysms.

Shock wave propagation along the central retinal blood vessels.

Retinal haemorrhage is often observed following brain injury. The retinal circulation is supplied (drained) by the central retinal artery (vein) which enters (leaves) the eye through the optic nerve at the optic disc; these vessels penetrate the nerve immediately after passing through a region of cerebrospinal fluid (CSF). We consider a theoretical model for the blood flow in the central retinal vessels, treating each as multi-region collapsible tubes, where we examine how a sudden change in CSF pressure (mimicking an injury) drives a large amplitude pressure perturbation towards the eye. In some cases, this wave can steepen to form a shock. We show that the region immediately proximal to the eye (within the optic nerve where the vessels are strongly confined by the nerve fibres) can significantly reduce the amplitude of the pressure wave transmitted into the eye. When the length of this region is consistent with clinical measurements, the CSF pressure perturbation generates a wave of significantly lower amplitude than the input, protecting the eye from damage. We construct an analytical framework to explain this observation, showing that repeated rapid propagation and reflection of waves along the confined section of the vessel distributes the perturbation over a longer lengthscale.

Recurrent pre-retinal haemorrhages associated with coagulation factor VII deficiency. / Hemorragias prerretinianas de repetición asociadas a déficit del factor VII de la coagulación.

The case concerns a 20 year-old woman with a sudden visual loss in her left eye. In the fundus examination, a focal pre-retinal haemorrhage was observed in the superior temporal vascular branch with accompanying vitreous haemorrhage. There was a favourable spontaneous outcome. Eighteen months later, she presented with a new visual loss in the same eye, showing a sub-hyaloid pre-macular haemorrhage that was satisfactorily drained by hyaloidotomy using a Nd-YAG laser. The patient had also presented with an upper digestive tract haemorrhage and menorrhagia. A haemostasis study was performed that showed a coagulation factor VII deficiency. This is a very uncommon disorder that has not been previously described in association with the appearance of pre-retinal haemorrhages.

Factors related to retinal haemorrhage in infants born at high risk.

PURPOSE: This study aims to determine risk factors for retinal haemorrhage (RH) in high-risk infants. METHODS: A total of 3123 cases with high-risk pregnancy and/or neonatal asphyxia 72 hr after delivery were enrolled into this study. Fundus examinations were performed on newborns utilizing a wide-angle imaging system (RetCam III). Retinal haemorrhage (RH) was classified into three grades. Maternal, obstetric and neonatal parameters from high-risk infants with RH were compared with parameters from infants without RH. RESULTS: Retinal haemorrhage (RH) was found in 550 (18%) of 3123 high-risk infants. Retinal haemorrhage (RH) was classified as grade I (39%), grade II (24%) and grade III (37%). Monocular RH occurred in 37% of cases, while the remaining cases were binocular. Moreover, six cases had vitreous haemorrhage. The following parameters correlated (p < 0.05) with RH in this study: delivery mode (χ2  = 469), gestational age (χ2  = 35), birth weight (χ2  = 18), asphyxia (χ2  = 73), scalp hematoma (χ2  = 55), maternal age (χ2  = 8.9), precipitate labour (χ2  = 120) and delivery times (χ2  = 6.1). Logistic regression analysis indicated that delivery mode and asphyxia were risk factors for RH in high-risk infants, with odds ratios of 0.827 and 2.5, respectively. Gender, intracranial haemorrhage and foetal distress were not correlated with RH in high-risk infants. CONCLUSION: The incidence of RH in high-risk infants was 18%, and delivery mode, and neonatal asphyxia were major risk factors for RH in high-risk infants.

Retinal haemorrhage in infants with pertussis.

AIMS: It has been hypothesised that paroxysmal coughing in infantile pertussis (whooping cough) could produce retinal haemorrhages identical to those seen in abusive head trauma. We aimed to test this hypothesis. METHODS: This is a prospective study of infants hospitalised with pertussis in Auckland, New Zealand, from 2009 to 2014. The clinical severity of pertussis was categorised. All infants recruited had retinal examination through dilated pupils by the paediatric ophthalmology service using an indirect ophthalmoscope. RESULTS: Forty-eight infants with pertussis, aged 3 weeks to 7 months, were examined after a mean of 18 days of coughing. Thirty-nine had severe pertussis and nine had mild pertussis. All had paroxysmal cough, and all were still coughing at the time of examination. No retinal haemorrhages were seen. CONCLUSIONS: We found no evidence to support the hypothesis that pertussis may cause the pattern of retinal haemorrhages seen in abusive head trauma in infants.