A rare case of bilateral cystic nephroma associated with embryonal rhabdomyosarcoma of the penile urethra.

Bilateral cystic nephroma is an extremely rare benign renal neoplasm. Here we present a case of bilateral cystic nephroma in a four month old boy who subsequently developed embryonal rhabdomyosarcoma of the urethra. Both tumors were successfully treated. To our knowledge this is the first reported case of this association which could be related to Dicer-1 mutation.

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.

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.

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.

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.

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.

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.

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.

Hyaline globules in fine-needle aspiration smears of salivary gland neoplasms.

Most salivary gland neoplasms can be accurately diagnosed on fine-needle aspiration cytology. Few cases present with overlapping cytomorphological features, so accurate distinction in these cases may be difficult. We describe a case of pleomorphic adenoma that had a close resemblance to adenoid cystic carcinoma on smears due to presence of numerous hyaline globules and bare nuclei. Careful analysis of cellular details along with corroborative clinical evidence clinched the correct diagnosis. This article discusses cytological features of salivary gland tumours in which hyaline globules can be seen on smears.

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.