Intravitreal injections as a leading cause of acute postoperative endophthalmitis-a regional survey in England.

BACKGROUND: To evaluate the characteristics, treatment patterns and outcomes of acute postoperative endophthalmitis. METHODS: Patients presenting with acute postoperative endophthalmitis between January 2017 to December 2019 were identified from hospital records in this multicentre retrospective cohort study. Clinical records were reviewed for visual acuity (VA) at various timepoints, cause of endophthalmitis, microbiological results, treatments and complications. RESULTS: Forty-six eyes of 46 patients were included. Intravitreal injections were the leading cause of acute postoperative endophthalmitis (n = 29; 63%), followed by cataract surgery (n = 8; 17%), vitreoretinal surgery (n = 7; 15%), and secondary intraocular lens insertion (n = 2, 4%). The absolute risk of endophthalmitis was 0.024% (1:4132) for intravitreal injections, 0.016% (1:6096) for cataract surgery, and 0.072% (1:1385) for vitreoretinal surgery. The majority of patients (n = 38; 83%) had better VA at 6 months compared to presentation, although fewer (n = 13; 28%) maintained similar or better VA compared to before the precipitating surgery. Twenty-four cases yielded positive culture results, of which staphylococcus epidermidis was the most commonly isolated organism. Microbiological yield was not associated with better final visual outcomes. Patients who underwent therapeutic vitrectomy (n = 15; 33%) had poorer VA at presentation, but subsequently achieved visual outcomes comparable to those who received medical treatment alone. There was no difference in time to presentation, visual outcome and retinal detachment rates among the different causative procedures. CONCLUSION: Intravitreal injections were the most common cause of endophthalmitis in our region, primarily because of their higher frequency compared to other intraocular procedures. In this cohort, the primary procedure had no effect on presentation, management or visual outcomes.

User-acceptability of an automated telephone call for post-operative follow-up after uncomplicated cataract surgery.

BACKGROUND: Innovative technology is recommended to address the current capacity challenges facing the NHS. This study evaluates the patient acceptability of automated telephone follow-up after routine cataract surgery using Dora (Ufonia Limited, Oxford, United Kingdom), which to our knowledge is the first AI-powered clinical assistant to be used in the NHS. Dora has a natural-language, phone conversation with patients about their symptoms after cataract surgery. METHODS: This is a prospective mixed-methods cohort study that was conducted at Buckinghamshire Healthcare NHS Foundation Trust. All patients who were followed up using Dora were asked to give a Net Promoter Score (NPS), and 24 patients were randomly selected to complete the validated Telephone Usability Questionnaire (TUQ) as well as extended semi-structured interviews that underwent thematic analysis. RESULTS: A total of 170 autonomous calls were completed. The median NPS score was 9 out of 10. The TUQ (scored out of 5) showed high rates of acceptability, with an overall mean score of 4.0. Simplicity, time saving, and ease of use scored the highest with a median of 5, whilst 'speaking to Dora feels the same as speaking to a clinician' scored a median of 3. The main themes extracted from the qualitative data were 'I can see why you're doing it', 'It went quite well actually', 'I just trust human beings I suppose'. CONCLUSION: We found high levels of patient acceptability when using Dora across three acceptability measures. Dora provides a potential solution to reduce pressure on hospital capacity whilst also providing a convenient service for patients.

The Clinical Profile and Long-Term Outcome of Children with Membranous Nephropathy, and the Evaluation of Anti-Phospholipase A2 Receptor Antibody Immunohistochemistry in Kidney Biopsy.

Introduction: Idiopathic membranous nephropathy (iMN) is a rare cause of nephrotic syndrome in children (1%-7%). Anti-phospholipase A2 receptor (PLA2R) antibody positivity in kidney biopsy is observed in 52%-78% of adults and 45% of children with iMN. The objectives of the study are to analyze the clinical profile and outcome of membranous nephropathy in children, to assess the prevalence of anti-PLA2R immunohistochemistry (IHC) in kidney biopsy, and to correlate their presence with disease characteristics. Methods: We are reporting a single-center retrospective study conducted in pediatric nephrology division. Clinical data and outcome parameters of children with membranous nephropathy were analyzed. PLA2R IHC was performed in kidney biopsy specimens retrospectively. Results: We analyzed 43 children with membranous nephropathy (MN) from a single center. 18 (42%) had idiopathic MN (iMN). PLA2R IHC was performed in kidney biopsy specimens in 14/18 (78%) patients with iMN and 7/9 (78%) non-lupus secondary membranous nephropathy (SMN) patients. The most common cause of SMN was lupus nephritis in 16 patients (64%). The mean estimated glomerular filtration rate (eGFR) at onset was 156 ± 81 ml/min/1.73m2. The sensitivity and specificity of PLA2R IHC in diagnosing pediatric MN was 50% and 57%, respectively; positive and negative predictive values were 70% and 36%, respectively. At the final follow-up, chronic kidney disease stage 5 (CKD 5) developed in 2/14 (14.3%) iMN patients. Conclusions: IHC PLA2R staining of glomerular tissue is a useful diagnostic marker of IMN. Though PLA2R prevalence is lower in children, its role in guiding treatment needs further exploration.

Is MDCT an accurate tool to differentiate between benign and malignant etiology in diffuse peritoneal disease?

PURPOSE: There is often considerable overlap of imaging findings in benign and malignant peritoneal diseases. We evaluated patients with diffuse peritoneal disease, to assess the diagnostic value of MDCT in predicting benign or malignant etiology in patients with unknown etiology, by analyzing the various patterns of involvement, particularly tuberculosis (TB) vs malignancy. METHODS: One hundred and thirty-six patients with diffuse peritoneal disease who had abdominal CT and subsequently underwent omental biopsies were included in the study. Peritoneal, mesenteric and omental involvement by disease was evaluated on CT using specific parameters. The presence of lymphadenopathy, ascites, scalloping of organs, involvement of liver and spleen, were also compared between benign and malignant conditions using histopathology as the gold standard. RESULTS: In 136 patients, 72 benign and 64 malignant pathologies were classified as per histopathology. Higher age (p < 0.001), increasing omental thickness (mean 25.2 mm, p = 0.004), omental caking (p < 0.001), > 10 mm mesenteric/peritoneal nodules (p < 0.03), visceral scalloping (p = 0.001), free ascites (p = 0.003), serosal involvement (p = 0.004) and bilateral pleural effusion (p = 0.02) were associated with malignant etiology. Mesenteric thickening/stranding (p = 0.02), mesenteric adenopathy (p < 0.001), necrotic nodes (p = 0.02), splenomegaly (p = < 0.001) and higher attenuation (> 20HU) of ascitic fluid (p < 0.001) were associated with benign etiology. The presence of mesenteric thickening or stranding (p = 0.01), splenomegaly (p = 0.02), higher ascitic fluid attenuation > 20HU (p = < 0.01), mesenteric adenopathy (p < 0.01), necrotic nodes (p = 0.03) favored tuberculosis. CT had diagnostic accuracy (79.3, 86.7%), sensitivity (79.2, 74.6%) and specificity (79.4, 97%) for observers 1 and 2, respectively (Kappa 0.713). CONCLUSION: Contrast-enhanced MDCT has good sensitivity, specificity and accuracy in differentiating benign and malignant etiologies of diffuse peritoneal disease. Multiple common parameters can be used to differentiate between tuberculous peritonitis and peritoneal carcinomatosis.

Papillorenal syndrome: a systemic diagnosis not to be missed on funduscopy.

A 45-year-old man presented to the ophthalmology department with visual symptoms in his left eye. Almost two decades ago, he required a renal transplant for focal segmental glomerular sclerosis and a detailed enquiry revealed a strong family history of renal and ocular disease. Fundus examination demonstrated significant optic disc dysplasia in his left eye and optical coherence tomography showed intraretinal fluid bilaterally. The diagnosis of papillorenal syndrome was suspected and genetic testing identified a heterozygous pathogenic variant in the PAX2 gene c.76dupG, p.Val26Glyfs*28, confirming the diagnosis. The patient was treated conservatively, and his vision eventually improved and stabilised. His renal disease and transplant were concurrently monitored by nephrologists. In this case, history-taking and ophthalmic examination raised suspicion of this rare systemic condition, which led to genetic testing and molecular confirmation of the diagnosis. We therefore highlight this case to raise awareness of papillorenal syndrome, which has significant systemic implications and also impacts familial screening and genetic counselling.

The Role of Frozen Section in the Rapid Diagnosis of Severe Cutaneous Adverse Drug Reactions.

CONTEXT: Early diagnosis is the mainstay in the management of severe cutaneous adverse reactions (SCARs) to drugs. AIMS: To study the role of frozen section in the rapid diagnosis of SCARs and the impact on outcome of the affected patients. SETTINGS AND DESIGN: A single-blind, hospital-based study was conducted from December 2014-July 2016. METHODS AND MATERIAL: We biopsied 32 adults with SCARs diagnosed by clinical features and standard criteria. The histopathological features seen on frozen sections were compared to that of paraffin blocks. The impact of rapid diagnosis on the clinical outcome was studied in toxic epidermal necrolysis (TEN), Stevens-Johnson syndrome (SJS), drug rash with eosinophilia and systemic symptoms (DRESS) and acute generalized exanthematous pustulosis (AGEP). STATISTICAL ANALYSIS: Z test was used to compare two proportions. Kappa statistic, sensitivity, specificity, positive predictive value, and negative predictive value of the frozen section diagnosis were calculated in TEN/SJS and DRESS using MedCalc software. RESULTS: Frozen and paraffin sections were done in TEN/SJS spectrum (13), DRESS (17), and AGEP (2). The sensitivity, specificity and kappa values for frozen section diagnosis in SJS/TEN and DRESS were 91.7%, 95%, 0.867 and 94.4%, 100%, 0.937 respectively. The concordance between frozen and paraffin section diagnosis was 100% in TEN, SJS, DRESS and AGEP. All the 6 patients with TEN and 2 with AGEP survived. Taking the worst-case scenario, the mortality in SJS was 28.6%. The mortality among patients with DRESS was 11.8%. CONCLUSIONS: Frozen section helps in the rapid diagnosis and early treatment of SCARs and differentiates it from diseases that mimic it.

Restructuring Wet Age-Related Macular Degeneration Services During the COVID-19 Pandemic to Allow Social Distancing Outpatient Clinics (SDOC).

BACKGROUND: COVID-19 has had a major impact on health-care provision. Social distancing will impact the organization of outpatient clinics (OCs) and require general restructuring of health care. METHODS: Our retinal team participated in a structured fact-finding session to implement social distancing of patients and staff in wet age-related macular degeneration (wAMD) clinics. Clinic flow and performance were continually reviewed and improved. A retrospective audit of all wAMD follow-up appointments was conducted for 4 weeks from the start of the UK lockdown. A search for clinical guidance regarding retinal services was performed on the homepages of international professional bodies. The guidelines were compared to the implemented changes in our wAMD social distancing OCs (SDOCs) and potential risk examined. RESULTS: The changes in clinic setup to achieve SDOCs are described. The average total time spent in the clinic area by each patient has reduced by 27%. The audit concluded that 65% of patients needed a treatment interval of 4-7 weeks after their appointment, 17% at either 8 or 9 weeks, and 18% at 10 weeks or beyond. The UK, Australian-New Zealand, US, and German professional ophthalmology bodies have published divergent guidelines, but all recommended a continuation of anti-VEGF injections. CONCLUSION: Health-care provision will change and hospitals and outpatient facilities will have to adapt to the COVID-19 epidemic. We describe a clinic setup (SDOCs) that minimizes risk to patients and staff, while maintaining the ability to treat each patient and their disease individually.

Secondary Bacterial Infection of a Solitary Cysticercus Granuloma.

The most common presenting form of neurocysticercosis in the Indian subcontinent is a solitary cysticercus granuloma (SCG). Patients with typical SCGs almost never require any form of surgical intervention. Herein, we report an extremely rare case of bacterial superinfection of a left frontal SCG in a 23-year-old female, resulting in severe perilesional edema and mass effect. The patient had to undergo an emergency left decompressive hemicraniectomy and excision of the infected granuloma. Serum enzyme-linked immunoelectrotransfer blot (EITB) for cysticercal antibodies was positive and histopathological examination of the lesion revealed a cysticercus. The culture of the pus from within the lesion grew vancomycin-resistant Enterococcus spp. for which she was treated with linezolid for 6 weeks. At 6 months follow-up, she had residual motor dysphasia, right homonymous hemianopia, and right hemiparesis but was steadily improving. Secondary bacterial infection of an SCG is very uncommon and can be devastating. A high index of suspicion is therefore required when there is disproportionate perilesional edema and mass effect.

Nephropathic cystinosis presenting with uveitis: Report of a "Can't See, Can't Pee" situation.

Nephropathic cystinosis is a rare autosomal recessive lysosomal disease characterized by accumulation of pathognomonic cystine crystals in renal and other tissues of the body. Cystinosis is caused by mutant cystinosin, the cystine transport protein located in lysosomal membranes, leading to systemic deposits of cystine and resultant end organ damage. Cystinosis is rarer in Asians than Caucasians with only a handful of cases reported from India to date. Due to its extreme rarity and clinically insidious presentation in contrast to the infantile form, the diagnosis of juvenile nephropathic cystinosis is frequently delayed or overlooked. Moreover, routine processing and sectioning of paraffin embedded tissues dissolves cystine crystals, making it difficult to diagnose this condition on light microscopic examination alone, mandating electron microscopic (EM) analysis of renal biopsies for an accurate diagnosis of this condition. We describe a case of juvenile nephropathic cystinosis presenting with uveitis and photophobia in a 17-year-old Indian male, diagnosed after EM examination of the patient's renal biopsy for evaluation of nephrotic syndrome. While highlighting the diagnostic utility of EM, we describe a few histopathologic clues which can prompt inclusion of EM analysis of renal biopsies in this setting.

Unmasking and successful management of light chain deposition disease of kidney in pregnancy: a complex case, mirroring the complex needs of pregnancy with kidney disease in India.

Pregnancy offers a precious window of opportunity to diagnose previously undetected or new onset kidney diseases in emerging countries like India, where access to medical, educational and health care facilities are not equitably distributed across varied sections of society. We report a case of a 33 year-old primi gravida who had a successful pregnancy following what was initially considered to represent preeclampsia at 38 weeks of gestation, in whom a subsequent kidney biopsy for persistence of pregnancy-related acute kidney injury (Pr-AKI) revealed light chain deposition disease (LCDD). The etiological evaluation of LCDD led to the detection of an underlying plasma cell dyscrasia which was treated effectively with chemotherapy and autologous stem cell transplant. In this report, we explore the hitherto uncharted pathophysiological relationship between LCDD and pregnancy-related kidney injury by transmission electron microscopic (TEM) studies of endothelial injury in this setting, and underscore the benefits of medical care in a multidisciplinary environment which yielded gratifying results in preservation of maternal kidney health and fetal outcome.

Silicone oil mimicking pathology on CT imaging.

An 81-year-old man was taken to Accident & Emergency after a fall. CT brain imaging demonstrated a well-defined, homogenous, hyperdense mass in the posterior segment of the left eye. Reported as vitreous haemorrhage, an urgent ophthalmological assessment was instigated.Detailed history revealed previous vitreoretinal procedures for multiple retinal detachments. Ophthalmological examination confirmed presence of silicone oil in this eye.The appearance of silicone oil in the eye has become an increasingly prevalent finding given its continued use in complex vitreoretinal procedures since 1962. Clinicians regularly interpreting orbital, facial and brain imaging need to be aware of these imaging appearances to prevent diagnostic confusion.

Gastric Glomus Tumour Misdiagnosed as Gastric Carcinoid: An Unfamiliar Entity with Aids to Diagnosis and Review of Literature.

Glomus tumour (GT) is a rare mesenchymal tumour of the stomach with Gastrointestinal Stromal Tumour (GIST), leiomyoma and schwannoma being far more common and comprising more than 90% of all gastric mesenchymal tumours. As glomus bodies are located in the peripheral parts of the human body, these tumours are peripherally located, classically the subungual region, hands, feet and trunk. While being evaluated for renal problems, a middle aged lady was incidentally found to have a gastric tumour. This was submucosal in location and was excised by a wedge resection and reported elsewhere as carcinoid tumour. The patient came to our hospital for further management. The biopsy was reviewed here and the modified diagnosis given was GT, confirmed by panel of immunohistochemistry. Two years after regular clinical follow up the patient is free of disease or any distant metastasis. In this paper the authors discuss the potential pitfalls, differential diagnoses and diagnostic clues that help in diagnosing this gastric tumour.

Late-onset acute graft-versus-host disease mimicking hand, foot, and mouth disease.

Acute skin graft-versus-host disease (GVHD) classically presents as a pruritic erythematous maculopapular rash. We describe a patient who underwent allogeneic hematopoietic stem cell transplantation and presented with a hand foot and mouth disease like clinical presentation. Histopathology was suggestive of acute GVHD. This case is being reported to make dermatologists aware of this unusual presentation of GVHD.

Idiopathic choroidal neovascularisation in pregnancy: treatment options and a successful outcome.

Choroidal neovascularisation (CNV) is a major cause of visual loss and treatment options aim to halt progression and stabilise vision. We describe a 29-year-old woman who presented with blurred vision and distortion in her left eye while 26 weeks pregnant. She was diagnosed with idiopathic CNV and multiple treatment options were discussed. The patient did not want to undertake any risks from having an anti vascular endothelial growth factor agent during pregnancy. Therefore on discussion with the obstetric team, she elected to have early delivery at 32 weeks followed by a course of intravitreal bevacizumab. Subsequently there was resolution of symptoms and intraretinal and subretinal fluid. CNV is uncommonly seen in pregnancy and there remains no consensus on treatment. We describe the third reported case of idiopathic CNV in pregnancy. Careful patient counselling and close liaison between the ophthalmology and obstetric teams are necessary in this condition to obtain a safe outcome while maximising vision.

TRANSCLERAL DRAINAGE OF SUBRETINAL FLUID, ANTI-VASCULAR ENDOTHELIAL GROWTH FACTOR, AND WIDE-FIELD IMAGING-GUIDED LASER IN COATS EXUDATIVE RETINAL DETACHMENT.

PURPOSE: To evaluate the safety and efficacy of combined transscleral drainage of subretinal fluid (SRF) with intravitreal bevacizumab and laser photocoagulation in the management of advanced Coats disease (Stage 3) with exudative retinal detachment. DESIGN: Retrospective interventional case series. METHODS: Retrospective case review of eight eyes in eight children with advanced Coats disease manifested as total or subtotal retinal detachment. All eyes initially underwent surgical drainage of exudative SRF followed by intravitreal injection of bevacizumab and laser photocoagulation. Patients were subsequently followed up for up to 60 months. RESULTS: In all eyes, after SRF drainage and administration of one to two intravitreal injections, SRF was completely eliminated. Patients required up to four sessions of laser photocoagulation. Retinal detachment consequently reduced with all patients showing total retinal reattachment and resolution of the subretinal exudates. At the last follow-up, no patient showed recurrent SRF and no ocular complications related to bevacizumab nor evidence of further disease progression were noted. CONCLUSION: The authors present a new therapeutic approach that allows for the first time successful treatment of advanced cases of exudative retinal detachment in Coats disease without the need for vitrectomy. Transscleral drainage of SRF accompanied by anti-vascular endothelial growth factor injection and laser photocoagulation appears to be successful in halting progression of advanced Coats disease with exudative detachment and a less invasive approach when compared with conventional management.

Harlequin Ichthyosis: Prenatal Diagnosis of a Rare Yet Severe Genetic Dermatosis.

Harlequin Ichthyosis (HI) is an extremely rare genetic skin disorder. It is the most severe type of ichthyosis. It is characterized by thickened, dry, rough and armor like plates of skin with deep cracks in between. Alternative names for HI include- keratosis diffusafetalis, ichthyosis congenital, icthyosis fetalis, harlequin fetus and icthyosis congenital gravior. It is an autosomal recessive disorder with the majority of affected individuals being homozygous for mutation in the ABCA 12 gene. This condition presents with a wide range of severity and symptoms. Affected neonates usually do not survive beyond first few days of life. We are presenting prenatal diagnosis of a case of this rare condition.