Bilateral Post-typhoid Retinitis: A Case Report

Retinitis following typhoid fever is an immune-mediated reaction that is rarely reported in literature. We present a case of a 20-year-old pregnant female who presented with progressive loss of vision in both eyes for three weeks. She had completed treatment for enteric fever 4 weeks before. Presenting vision was 6/36 in right eye and 3/60 in left eye. Fundus examination showed retinitis with retinal haemorrhages and macular stars in both eyes. After approval from gynaecologist, oral steroid was given. Vision improved significantly to 6/6 in right eye and 6/12 in left eye after 1.5 months of treatment. There was resolution of haemorrhages, exudates and stellate maculopathy. Hence, post-typhoid immune-mediated retinitis is rare sequelae of typhoid fever which can be treated with good visual outcome using oral steroids.

Is external hydrocephalus a possible differential diagnosis when child abuse is suspected?

Background Criteria for diagnosing abusive head trauma (AHT) or “shaken baby syndrome” are not well defined; consequently, these conditions might be diagnosed on failing premises. Methods The authors have collected a total of 28 infants, from the US (20) and Norway (8), suspected of having been violently shaken, and their caregivers had been suspected, investigated, prosecuted or convicted of having performed this action. Among 26 symptomatic infants, there were 18 boys (69%) and 8 girls (31%)—mean age 5.1 month, without age difference between genders. Results Twenty-one of 26 symptomatic children (81%) had a head circumference at or above the 90 percentile, and 18 had a head circumference at or above the 97 percentile. After macrocephaly, seizure was the most frequent initial symptom in 13 (50%) of the symptomatic infants. Seventeen (65%) of the symptomatic infants had bilateral retinal haemorrhages, and two had unilateral retinal haemorrhages. All infants had neuroimaging compatible with chronic subdural haematomas/hygromas as well as radiological characteristics compatible with benign external hydrocephalus (BEH). Conclusions BEH with subdural haematomas/hygromas in infants may sometimes be misdiagnosed as abusive head trauma. Based on the authors’ experience and findings of the study, the following measures are suggested to avoid this diagnostic pitfall: medical experts in infant abuse cases should be trained in recognising clinical and radiological BEH features, clinicians with neuro-paediatric experience should always be included in the expert teams and reliable information about the head circumference development from birth should always be available.

EFFECT OF MRIDWEEKADI KASHAYA SEKA IN RETINAL HAEMORRHAGES ASSOCIATED WITH NON-PROLIFERATIVE DIABETIC RETINOPATHY – A CLINICAL STUDY

Diabetic retinopathy (DR) is a leading cause of acquired vision loss in middle-aged and elderly people globally. In modern science, other than the meticulous control of diabetes there is no proven non-invasive management for the prevention or cure of Diabetic retinopathy. In this study, mild to moderate Non-proliferative diabetic retinopathy (NPDR) with retinal haemorrhages is considered as a Timira (symptomatically) and as Abhishyanda (considering etiopathogenesis) with Kapha-pitta predominance. Mridweekadi kashaya, predominantly Kapha Pitta samana, was selected for the study to be used as Seka. Method: The study design was Interventional- pre and post evaluation without control, sample size fixed as 30 eyes. Mridweekadi kashaya was used as Seka for 21 days, twice daily. Fundus photographs were taken prior to commencement of Seka, on the 22nd day and then on 30th and 60th day after completion of the procedure. Change in extent of retinal hemorrhages were assessed as visualized in Fundus photographs and direct ophthalmoscopy. Change in visual acuity was assessed by LogMar Visual acuity chart and change in contrast sensitivity by Pelli-Robson contrast sensitivity chart consecutively, prior to the treatment, on the 10th day, 22nd day and then on 30th and 60th day after completion of procedure. Statistical analysis was done using Wilcoxon signed rank test and Paired t test according to the variable. Result: Control in retinal haemorrhages associated with NPDR and improvement in visual acuity and contrast sensitivity. Conclusion: Mridweekadi kashaya seka is effective in controlling retinal haemorrhages associated with NPDR.

Haemorrhagic Retinopathy Associated with Zieve Syndrome

Zieve syndrome, a scarcely reported case is a triad of jaundice, haemolytic anaemia and hyperlipidaemia that develops secondary to alcohol-induced liver injury. Herein, a case of 23-year-old young male with history of steatohepatitis is reported who presented with diminution of vision in both eyes. On fundoscopy, there were extensive retinal haemorrhages involving fovea. Workup was consistent with haemolytic anaemia with no source of active bleeding. The patient was managed with supportive treatment. No specific ocular treatment was given. However, the vision gradually improved during follow-up. A rare association of zieve syndrome with haemorrhagic retinopathy is described here. Moreover, zieve syndrome has been described in the literature, mostly in non-English language case studies and is still under-recognised and under-reported. Diagnosis should be made quickly to avoid unnecessary invasive diagnostic interventions.

MO002OPHTHALMOLOGICAL EFFECTS OF ROXADUSTAT IN THE TREATMENT OF ANAEMIA IN CHRONIC KIDNEY DISEASE PATIENTS ON DIALYSIS IN A PHASE 3, RANDOMISED, DOUBLE-BLIND, ACTIVE-COMPARATOR CONVERSION STUDY

Background and Aims Roxadustat is an orally administered hypoxia-inducible factor prolyl hydroxylase inhibitor (HIF-PHI) approved in China for the treatment of dialysis-dependent (DD) and non–dialysis-dependent chronic kidney disease (CKD) anaemia, and in Japan for the treatment of DD CKD anaemia. An acceptable safety profile as well as noninferiority of roxadustat to darbepoetin alfa (DA), measured by haemoglobin (Hb) change from baseline (BL), was shown in patients (pts) with DD CKD in a Japanese phase 3, randomised double-blind, double-dummy study (CL-0307). Some nonclinical data suggest that HIF stimulation may promote angiogenesis, thereby increasing the risk of certain retinal pathologies. As such, this report focuses on a prospective analysis of ophthalmological/retinal-related events observed during the 24-week study period of the aforementioned phase 3 study. Method Japanese pts with DD CKD were randomised 1:1 to receive roxadustat three times weekly or DA once weekly; doses were titrated to maintain Hb between 10–12 g/dL. Ophthalmological/retinal-related outcomes were evaluated in pts who received ≥1 dose of study drug (SAF) and included emergence of adverse events assessed by the investigator and ophthalmological findings based on assessments performed by centralised grading. Ophthalmic imaging (colour fundus photography, optical coherence tomography) and assessment of visual acuity were performed at BL, Week 12, and Week 24 or at study discontinuation according to Ophthalmic Image Acquisition Guidelines. Each investigator evaluated the ophthalmic images and assessed the clinical relevance of any changes. Central evaluation of ophthalmological examination results was then performed by two independent specialists who were blinded to study treatment. During central evaluation, the reviewers assessed ophthalmic images in accordance with the Safety Independent Ophthalmology Review Charter. If graders’ results disagreed, adjudication was performed by a blinded, independent grader who did not participate in the primary review of the patient data. Grading results and visual acuity were analysed descriptively. Results In the SAF, a total of 302 pts were randomised to receive either roxadustat (n=150) or DA (n=152). At BL, previous or concurrent retinal vascular disorders were present in 41.3% (62/150) and 37.5% (57/152) of pts in the roxadustat and DA groups, respectively; 32.7% (49/150) and 27.6% (42/152) of pts presented with a history of both diabetes mellitus and retinal vascular disorders, respectively. The mean (SD) duration of exposure to study drug was 146.7 (45.8) days in the roxadustat group and 154.7 (37.4) days in the DA group. The proportion of pts with new or worsening retinal haemorrhages during the treatment period was 32.4% (n=46/142) with roxadustat and 36.6% (n=53/145) with DA. In a subgroup analysis of pts with no retinal haemorrhage at BL, the proportion of pts with new retinal haemorrhages was 19.1% (n=18/94) with roxadustat and 25.0% (n=24/96) with DA during the treatment period; in pts with ≥1 retinal haemorrhage at BL, the proportion of pts with new or worsening retinal haemorrhages was 58.3% (n=28/48) with roxadustat and 59.2% (n=29/49) with DA during the treatment period. From BL, no clinically meaningful changes in visual acuity or retinal thickness were found in either treatment group. Similarly, from BL, no clinically meaningful changes were found in the proportion of pts exhibiting intra-/sub-retinal fluid, hard exudates, or cotton wool spots in either treatment group. Conclusion These findings suggest that, during the 24-week treatment period, DD CKD pts treated with roxadustat were not at an increased risk of ophthalmic abnormalities—including retinal haemorrhages or increased retinal thickness—compared with pts treated with DA.

Neonatal Eye Screening for 203 Healthy Term Newborns Using a Wide-Field Digital Retinal Imaging System

Purpose: This study aimed to determine the proportion and types of ocular abnormalities detected in healthy term newborns and also the risk factors associated with retinal haemorrhages.Method: This cross-sectional study comprised of 203 participants, all healthy term newborn infants in the Obstetrics and Gynaecology ward at Hospital Kuala Lumpur over a six months period. The examination list includes external eye examination, red reflex test, and fundus imaging using a wide-field digital retinal imaging system (Phoenix Clinical ICON Paediatric Retinal Camera) by a trained Investigator. The pathologies detected were documented. The results were compared with similar studies previously published in the literature.Results: Total ocular abnormalities were detected in 34% infants. The most common finding was retinal haemorrhage in 29.6% infants, of which 53.3% occurred bilaterally. Spontaneous vaginal delivery (SVD) remained the greatest risk factor which has nearly 3.5 times higher risk of newborns developing retinal haemorrhage compared to Lower Segment Caesarean Section (LSCS). There was a 6% increased likelihood of developing retinal haemorrhage for every 1 minute increment in the duration of 2nd stage of labour. Conclusion: Universal eye screening for all newborns using a wide-field digital imaging system is possible, safe and useful in detecting posterior segment disorders. The most common abnormality detected is retinal haemorrhage. Although most resolve spontaneously, a longitudinal study is needed to study the long term effect of retinal haemorrhages in these infants.

Henle fibre layer haemorrhage: clinical features and pathogenesis

BackgroundTo describe the clinical presentation and characteristic imaging features of deep retinal haemorrhages primarily located in the Henle fibre layer (HFL) of the macula. The spectrum of aetiologies and a comprehensive theory of pathogenesis are presented.MethodsThis is a retrospective, multicentre case series evaluating eyes with retinal haemorrhage in HFL. Clinical features, underlying aetiology, systemic and ocular risk factors, visual acuity, and multimodal imaging including fundus photography and cross-sectional and en face optical coherence tomography (OCT) are presented.ResultsRetinal haemorrhages localised to HFL in 33 eyes from 23 patients were secondary to acute blunt trauma to the head (n=2), eye (n=1) and trunk (n=1), ruptured intracranial aneurysm (Terson’s syndrome, n=3), general anaesthesia (n=1), epidural anaesthesia (n=1), hypertension with anaemia (n=1), decompression retinopathy (n=1), postvitrectomy with intraocular gas (n=1), retinal vein occlusion (n=7), myopic degeneration (n=2), macular telangiectasia type 2 (n=1), and polypoidal choroidal vasculopathy (n=1). Defining clinical features included deep retinal haemorrhage with feathery margin and petaloid pattern radiating from the fovea. OCT demonstrated characteristic hyper-reflectivity from the haemorrhage delineated by obliquely oriented fibres in the Henle layer. Spontaneous resolution of HFL haemorrhage occurred after 3 months in 15 patients with follow-up.ConclusionThe characteristic petaloid-shaped, deep intraretinal haemorrhage with a feathery margin localised to HFL is associated with various disorders. The terminology ‘Henle fiber layer hemorrhage (HH)’ is proposed to describe the clinical and OCT findings, which may result from abnormal retinal venous pressure from systemic or local retinovascular disorders affecting the deep capillary plexus or from choroidal vascular abnormalities.

The use of pan-retinal photocoagulation to treat recurrent vitreous haemorrhage with neovascularisation in the context of Epstein syndrome: an MYH9-related disorder

A female patient presented with stable chronic thrombocytopaenia with large platelets, sensorineuronal deafness and renal impairment. Her treatment was refractory to intravenous immunoglobulins (IVIG) and steroids for a putative diagnosis of immune thrombocytopaenic purpura (ITP). She underwent genetic testing which revealed a MYH9 mutation in-keeping with a diagnosis of Epstein Syndrome. Subsequently to this she developed globally constricted fields on Goldmann visual field testing. MRI pituitary was unremarkable but she was diagnosed with a pituitary microprolactinoma secondary to raised prolactin in the blood responsive to carbegoline therapy. She subsequently developed retinal haemorrhages and recurrent vitreous haemorrhages due to neovascularisation. Fluorescein angiography revealed the extent of the neovascularisation and microvascular ischaemia. She underwent pan-retinal photocoagulation (PRP) to treat the ischaemic stimulus which resulted in regression of the new vessels and cessation of vitreous haemorrhages. There are no previous reported cases of microvascular retinal disease in the literature in the context of Epstein Syndrome, and this is the first report of successful treatment with PRP.