Nasal natural killer/T-cell lymphoma mimicking orbital cellulitis: A diagnostic dilemma.

Nasal natural killer/T-cell lymphoma (NK/TCL) is a rare form of malignant non-Hodgkin lymphoma (NHL) with a far more rare involvement of orbit. The orbital involvement has a highly variable clinical presentation. Here, we report one such case of a 40-year-old male patient who presented with swelling in the right upper and lower eyelids with the diminution of vision for 12 days. He had a history of blocked nose for two months. Clinical examination and CT scan of the orbit and paranasal sinuses suggested a diagnosis of right orbital cellulitis with pansinusitis. A combination of intravenous antibiotics was started, and functional endoscopic sinus surgery was done. Histopathology was a suggestive of nasal NK/TCLl NHL. After proper staging, the patient was given chemotherapy and radiotherapy. There was a complete resolution of mass with no recurrence over a follow-up of 10 months.

Efficacy of hypertonic saline in treatment of corneal edema: A randomized crossover trial.

Background: To determine the efficacy of sodium chloride (NaCl) 5% drops in comparison to 6% ointment and study tear Osmolarity as an objective measure correlating with clinical findings in the treatment of corneal edema. Methods: This is a prospective, randomized, interventional, open-label, crossover study of 40 eyes of 40 patients with corneal edema due to Bullous keratopathy and Fuchs endothelial dystrophy. Subjects were divided into 2 groups by simple randomization: group A received NaCl 5% drops and group B received NaCl 6% ointment. Both treatments were administered four times daily for seven days. Subsequently, after a 1-week wash-out period, switch over of treatment was done. Central corneal thickness (CCT) and tear osmolarity were primarily efficacy variables. Results: Baseline parameters were comparable. The median reduction in CCT from baseline (706.7 ± 58.41 µm), at 6 hours with NaCl 5% drops was 23 µm (-27, 74) and that with NaCl6% ointment was 37.5 µm (-7, 85). The reduction in CCT was more with 6% ointment (p = 0.013). The difference in reduction in CCT between two treatments at one week was not statistically significant, although there was a substantial reduction in thickness with each treatment individually. The change in tear osmolarity from the baseline at 2 Hours with both NaCl5% drops and 6% ointment was significant, and it remained so till 6 h. Side-effects such as stickiness, stinginess, blurring, and foreign body sensation were more with 6% eye ointment. Conclusion: Topical NaCl 6% eye ointment in QID dosage is more effective than NaCl 5% drops in the medical management of corneal edema. In patients symptomatically intolerant to ointment, NaCl 5% eye drops may be prescribed as an effective option.

Ophthalmologic manifestations of organoid nevus syndrome: A series of 13 cases.

Background: The organoid nevus syndrome is a rare neurocutaneous syndrome typified by cutaneous sebaceous nevus, seizures and epibulbar choristomas. The condition is associated with multiple ocular abnormalities. Herein, the authors aim to study and report the ophthalmic features of cases diagnosed with organoid nevus syndrome. Methods: The authors retrospectively evaluated the records of patients with the organoid nevus syndrome who had presented to a tertiary care eye hospital in northern India. The ocular features were studied and entered in MS excel and the data were evaluated. Results: Data of 13 patients with the organoid nevus syndrome were found. All 13 patients had cutaneous features in the form of Sebaceous nevus of Jadasson, 8 patients had alopecia of the scalp area, 2 had history seizures and 10 had arachnoid cysts on neuroimaging of the head. All 13 patients had a complex choristoma involving the ocular surface. Conclusions: We conclude that the most common ophthalmologic features associated with organoid nevus are complex choristoma of the bulbar surface, scleral coat calcification and upper eyelid coloboma.

Voriconazole in the treatment of invasive aspergillosis of orbit.

Background: The aim of the study was to evaluate the outcomes of voriconazole in terms of functional recovery and response on imaging in the management of invasive aspergillosis of orbit. Methods: This was a prospective non-comparative interventional study. Diagnosed cases of invasive orbital aspergillosis were studied in a tertiary care hospital. Intravenous voriconazole followed by oral treatment was given. Sinus debridement was done, where needed. The response to treatment was assessed clinically and on radiology. Results: A total of 10 diagnosed cases of invasive orbital aspergillosis were studied. Nine cases (90%) occurred in immunocompetent patients. Predisposing sinus infection was seen in 8 patients (80%). The most common presenting complaint was the protrusion of eye. On voriconazole treatment, there was a statistically significant improvement in vision and extraocular movements from first week onwards (p = 0.01 and p = 0.02, respectively) and reduction in proptosis from second week onwards (p = 0.003). Imaging was done at three months follow-up which revealed a good response to treatment in 90% of patients. All patients tolerated the drug well except one who had transient hepatic dysfunction. The mean follow-up was 5.8 months (range: 3-12 months). There was no recurrence of disease till the last follow-up. Conclusion: Invasive orbital aspergillosis commonly presents as sino-orbital disease, mostly in immunocompetent adult patients. Voriconazole is a safe and effective drug with good short-term clinical outcome.

Primary Ewing sarcoma/primitive neuroectodermal tumors of the kidney: Case series of eight cases from a single center with follow-up details.

Introduction: We aim to share the experience of a single center in the management of eight cases of renal primitive neuroectodermal tumor (PNET) which are uncommon, aggressive tumors. The objectives were to study the presentation of the disease, the treatment offered and its outcomes, and the comparison of the treatment with published literature. Methods: The single-center renal PNET data of all patients were retrospectively reviewed from 2011 to 2022. Renal PNET was seen in eight patients. Minimum follow-up period of 1 year was required. Results: Male-to-female ratio was 7:1. The mean age was 26.5 years. All were locally advanced tumors on presentation. One patient had an inferior vena cava thrombus, one patient had metastases on presentation, and two patients had tumor extending to paranephric space. The diagnosis was made by histopathology supported by immunohistochemistry showing CD99 positivity. All patients were treated with radical nephrectomy, followed by chemotherapy in all and radiotherapy in three patients. Two patients expired at 3½ and 6 years after surgery, the remaining six are alive at a median follow-up period of 34.5 months. Conclusion: Renal PNET is an uncommon renal tumor which is aggressive and requires multimodal therapy for prolonged survival.

Correlation of Levator-Muller's Complex Thickness on Ultrasound Biomicroscopy to Botulinum Toxin A Injection in Thyroid-Related Upper Eyelid Retraction.

OBJECTIVES: To study the thickness of levator palpebra superioris-Muller's muscle complex (LMC) on ultrasound biomicroscopy (UBM) and to correlate with the clinical response to botulinum toxin A (BTA) injection in patients with inactive-stage of thyroid-related upper eyelid retraction (UER). We also studied the correlation of clinical parameters, preinjection with postinjection values. METHODS: This was a prospective, interventional study. Patients with thyroid-related UER who underwent subconjunctival injection of BTA were recruited. Demographic data and clinical details were evaluated. UBM (50 MHz) was done to measure the thickness of LMC. Patient's satisfaction was graded at each follow-up. Follow-up was done at 1 week, 1 month, and 3 months' time intervals. RESULTS: A total of 13 patients were recruited and 26 eyes were divided into two groups; group 1 included eyes with UER (n = 17), and group 2 included eyes without UER (n = 9). There was a statistically significant reduction in margin reflex distance 1 (MRD1) after BTA injection at 1-week, 1-month, and 3-months follow-up with maximum reduction at 1 month. The mean LMC thickness of 26 eyes was 0.96 mm which was found to be significantly more than normal controls. On comparison of mean LMC thickness with the amount of UER and reduction in MRD1, we did not find a significant difference. CONCLUSIONS: Patients with TED have significantly thicker LMC on UBM than controls. Further studies are needed with a larger sample size on the correlation of UBM features of levator aponeurosis with response to BTA injection.

Experience on rhino-orbital mucormycosis from a tertiary care hospital in the first wave of COVID-19: An Indian perspective.

Background: To highlight the clinical presentations and management outcomes of rhino-orbital mucormycosis during first wave of COVID-19 pandemic in North India. Methods: A retrospective observational study. 15 patients with mucormycosis (orbital disease) who presented during short span of 3 months (October-December 2020) in a tertiary-care referral institution were analysed. Results: At presentation, 13 of 15 patients had uncontrolled diabetes. Four had history of COVID-19 infection. All patients had advanced orbital disease with sinusitis; cavernous sinus involvement was in nine and intracranial spread in three patients. Liposomal amphotericin-B was started and prompt orbital exenteration with sinus surgery was performed in 12 patients. All 12 patients survived with an average follow-up of 4.8 months. Conclusion: In the present series, cases with orbital spread of mucormycosis were mostly found in non-COVID uncontrolled diabetics. Exenteration was done in 80% of cases with advanced orbital disease. Prevention and early detection of infection at the stage of sino-nasal involvement might help to prevent spread and/or halt the orbital disease.

Atrial fibrillation in hypertrophic cardiomyopathy: prevalence, clinical impact, and management.

Hypertrophic cardiomyopathy (HCM) is the most common hereditary cardiomyopathy characterized by left ventricular hypertrophy and spectrum of clinical manifestation. Atrial fibrillation (AF) is a common sustained arrhythmia in HCM patients and is primarily related to left atrial dilatation and remodeling. There are several clinical, electrocardiographic (ECG), and echocardiographic (ECHO) features that have been associated with development of AF in HCM patients; strongest predictors are left atrial size, age, and heart failure class. AF can lead to progressive functional decline, worsening heart failure and increased risk for systemic thromboembolism. The management of AF in HCM patient focuses on symptom alleviation (managed with rate and/or rhythm control methods) and prevention of complications such as thromboembolism (prevented with anticoagulation). Finally, recent evidence suggests that early rhythm control strategy may result in more favorable short- and long-term outcomes.

Link between Brugada phenocopy and myocardial ischemia: Results from the International Registry on Brugada Phenocopy.

BACKGROUND: Brugada phenocopies clinical entities that have indistinguishable electrocardiographic (ECG) patterns from true congenital Brugada syndrome. However, they are induced by other clinical circumstances such as myocardial ischemia. The purpose of our study was to examine the clinical features and pathogenesis of ischemia-induced Brugada phenocopy (BrP). METHODS: Data from 17 cases of ischemia-induced BrP were collected from the International Registry (www.brugadaphenocopy.com). Data were extracted from these publications and authors were contacted to provide further insight into each case. RESULTS: Of the patients included in this study, 71% were male. Mean age was 59 ± 11 years (range: 38-76). Type-1 Brugada ECG pattern occurred in 15/17 (88%) of the cases, while a type-2 Brugada ECG pattern was observed in the other 2/17 (12%). In all cases, the Brugada ECG pattern resolved upon correction of the ischemia, indicating ischemia as the inducing circumstance. No arrhythmic events have been detected acutely or during the follow-up. Reported time to resolution ranged from 2 minutes to 5 hours. Provocative challenges using sodium channel blocking agents were performed in 7/17 cases (41%), and all failed to induce a Brugada ECG pattern (BrP Class A). The remaining 10/17 cases (59%) did not undergo provocative testing due to various clinical reasons. CONCLUSIONS: Myocardial ischemia is a commonly reported etiology of BrP. Importantly, this study found no association between BrP induced by myocardial ischemia and sudden cardiac death or malignant ventricular arrhythmias.

Implications for Public Health Regulation if Chevron Deference Is Overturned.

This Viewpoint describes implications for medicine and public health if the US Supreme Court decides to overturn or narrow Chevron deference.

Percutaneous Mitral Valve Interventions and Heart Failure.

Mitral regurgitation (MR) is the most frequent Valvular Heart Disease (VHD) and is an important cause of heart failure. MR can be caused by primary valve abnormality (Degenerative MR/Primary MR) or it can be secondary to cardiomyopathy (Functional MR/Secondary MR). Medical management alleviates symptoms but does not alter the progression of the disease. Current guidelines recommend surgery for moderate-to-severe (Grade > 3) MR in patients with symptoms or evidence of left ventricular dysfunction. Despite current practice guidelines, the majority of patients with severe MR do not undergo surgery. The reasons include high surgical risk from advanced age or multiple comorbidities, and a lack of clear data supporting valve surgery for secondary MR with LV dysfunction. The recent emergence of percutaneous interventional approaches in treating MR has expanded therapeutic options for patients who are at high risk for conventional Mitral Valve (MV) surgery. In this chapter, we will review the novel advancements in the field of percutaneous MV interventions that could potentially become the standard of care for patients with MR and heart failure.

Patent Foramen Ovale Closure in the Setting of Cryptogenic Stroke: A Meta-Analysis of Five Randomized Trials.

BACKGROUND: The clinical benefit of patent foramen ovale (PFO) closure after cryptogenic stroke has been a topic of debate for decades. Recently, 3 randomized controlled trials of PFO closure in patients with cryptogenic stroke demonstrated a significantly reduced risk of recurrent stroke compared with standard medical therapy alone. This meta-analysis was performed to clarify the efficacy of PFO closure for future stroke prevention in this population. METHODS: A systematic literature search was undertaken. Published pooled data from 5 large randomized clinical trials (CLOSE, RESPECT, Gore REDUCE, CLOSURE I, and PC) were combined and then subsequently analyzed. Enrolled patients with cryptogenic stroke were assigned to receive standard medical care or to undergo endovascular PFO closure, with a primary outcome of reduction in stroke recurrence rate. Secondary outcomes included rates of transient ischemic attack (TIA), composite outcome of stroke, TIA, and death from all causes, and rates of atrial fibrillation events. RESULTS: We analyzed data for 3412 patients. Transcatheter PFO closure resulted in a statistically significant reduced rate of recurrent stroke, compared with medication alone. Patients undergoing closure were 58% less likely to have another stroke. The number needed to treat with PFO closure to reduce recurrent stroke for 1 patient was 40. CONCLUSIONS: Endovascular PFO closure was associated with a reduced risk of recurrent stroke in patients with a prior cryptogenic cerebral infarct. Although the absolute stroke reduction was small, these findings are clinically significant, given the young age of this patient population and the patients' lifetime risk of recurrent stroke.

Drug-induced pulmonary arterial hypertension: a review.

Pulmonary arterial hypertension (PAH) is a subgroup of PH patients characterized hemodynamically by the presence of pre-capillary PH, defined by a pulmonary artery wedge pressure (PAWP) ≤15 mmHg and a PVR >3 Wood units (WU) in the absence of other causes of pre-capillary PH. According to the current classification, PAH can be associated with exposure to certain drugs or toxins such as anorectic agents, amphetamines, or selective serotonin reuptake inhibitors. With the improvement in awareness and recognition of the drug-induced PAH, it allowed the identification of additional drugs associated with an increased risk for the development of PAH. The supposed mechanism is an increase in the serotonin levels or activation of serotonin receptors that has been demonstrated to act as a growth factor for the pulmonary artery smooth muscle cells and cause progressive obliteration of the pulmonary vasculature. PAH remains a rare complication of several drugs, suggesting possible individual susceptibility, and further studies are needed to identify patients at risk of drug-induced PAH.

Atrioesophageal Fistula Following Radiofrequency Catheter Ablation of Atrial Fibrillation.

Atrioesophageal fistula (AEF) is a rare but catastrophic complication of catheter ablation of atrial fibrillation (AF), with an incidence of 0.03% to 1.5% per year. We report two cases and review the epidemiology, clinical features, pathogenesis, and management of AEF after AF ablation. The principal clinical features of AEF include fever, hematemesis, and neurologic deficits within 2 months after ablation. The close proximity of the esophagus to the posterior left atrial wall is considered responsible for esophageal injury during ablation and the eventual development of AEF. Prophylactic proton pump inhibitors, esophageal temperature monitoring, visualization of the esophagus during catheter ablation, esophageal protection devices, esophageal cooling, and avoidance of energy delivery in close proximity to the esophagus are some techniques to prevent esophageal injury. Eliminating esophageal injury during AF ablation is of utmost importance in preventing AEF. A high index of suspicion and early intervention are necessary to prevent fatal outcomes. Early surgical repair is the mainstay of treatment.

Duration of dual antiplatelet therapy following drug-eluting stent implantation: A systemic review and meta-analysis of randomized controlled trials with longer follow up.

OBJECTIVE: To evaluate the long term efficacy and safety of long duration DAPT (L-DAPT) compared to short duration DAPT (S-DAPT) after drug-eluting stent (DES) implantation. METHODS: We searched Medline, Embase, and Cochrane Central Register of Controlled Trials (CENTRAL) to identify randomized controlled trials (RCTs) assessing the clinical impact of L-DAPT versus S-DAPT after DES and have mean follow up period of at least 2 years or longer. Primary end point was stent thrombosis (ST). Secondary endpoints were all-cause mortality, cardiac mortality, myocardial infarction (MI), target vessel revascularization (TVR), thrombolysis in myocardial infarction (TIMI) major bleeding and stroke. Event rates were compared using a random effects model. RESULTS: We identified five RCTs in which 19,760 patients were randomized to S-DAPT (N = 9,810) and L-DAPT (n = 9,950), respectively. Compared with L-DAPT, S-DAPT was associated with higher rate of MI (odds ratio [OR] 1.48, 95% confidence interval [CI] [1.04, 2.10]). There were no significant differences between S-DAPT and L-DAPT in terms of all cause mortality, cardiac mortality, ST, TVR or stroke (OR 0.90, 95% CI [0.73, 1.12]; OR 1.02, 95% CI [0.80, 1.30]; OR 1.59, 95% CI [0.77, 3.27]; OR 0.87 95% CI [0.67, 1.14]; and OR 1.08 95% CI [0.81, 1.46], respectively). However, rate of TIMI major bleeding was significantly lower with S-DAPT compared to L-DAPT (OR 0.64, 95% CI [0.41, 0.99]). CONCLUSIONS: In the present analysis of RCTs with longer follow up (2 years or longer), S-DAPT compared with L-DAPT, was associated with higher rate of MI and lower rate of major bleeding without any significant difference in the rates of all cause mortality, cardiac mortality, ST, TVR, and stroke.

Staged versus index procedure complete revascularization in ST-elevation myocardial infarction: A meta-analysis.

BACKGROUND: Complete revascularization of patients with ST-elevation myocardial infarction and multivessel coronary artery disease reduces adverse events compared to infarct-related artery only revascularization. Whether complete revascularization should be done as multivessel intervention during index procedure or as a staged procedure remains controversial. METHOD: We performed a meta-analysis of randomized controlled trials comparing outcomes of multivessel intervention in patients with ST-elevation myocardial infarction and multivessel coronary artery disease as staged procedure versus at the time of index procedure. Composite of death or myocardial infarction was the primary outcome. Mantel-Haenszel risk ratios were calculated using random effect model. RESULTS: Six randomized studies with a total of 1126 patients met our selection criteria. At a mean follow-up of 13 months, composite of myocardial infarction or death (7.2% vs 11.7%, RR: 1.66, 95%CI: 1.09-2.52, P = 0.02), all cause mortality (RR: 2.55, 95%CI: 1.42-4.58, P < 0.01), cardiovascular mortality (RR: 2.8, 95%CI: 1.33-5.86, P = 0.01), and short-term (<30 days) mortality (RR: 3.54, 95%CI: 1.51-8.29, P < 0.01) occurred less often in staged versus index procedure multivessel revascularization. There was no difference in major adverse cardiac events (RR: 1.14, 95%CI: 0.88-1.49, P = 0.33), repeat myocardial infarction (RR: 1.14, 95%CI: 0.68-1.92, P = 0.61), and repeat revascularization (RR: 0.92, 95%CI: 0.66-1.28, P = 0.62). CONCLUSION: In patients with ST-elevation myocardial infarction and multivessel coronary artery disease, a strategy of complete revascularization as a staged procedure compared to index procedure revascularization results in reduced mortality without an increase in repeat myocardial infarction or need for repeat revascularization.

Cardiac molecular imaging to track left ventricular remodeling in heart failure.

Cardiac left ventricular (LV) remodeling is the final common pathway of most primary cardiovascular diseases that manifest clinically as heart failure (HF). The more advanced the systolic HF and LV dysfunction, the worse the prognosis. The knowledge of the molecular, cellular, and neurohormonal mechanisms that lead to myocardial dysfunction and symptomatic HF has expanded rapidly and has allowed sophisticated approaches to understanding and management of the disease. New therapeutic targets for pharmacologic intervention in HF have also been identified through discovery of novel cellular and molecular components of membrane-bound receptor-mediated intracellular signal transduction cascades. Despite all advances, however, the prognosis of systolic HF has remained poor in general. This is, at least in part, related to the (1) relatively late institution of treatment due to reliance on gross functional and structural abnormalities that define the "heart failure phenotype" clinically; (2) remarkable genetic-based interindividual variations in the contribution of each of the many molecular components of cardiac remodeling; and (3) inability to monitor the activity of individual pathways to cardiac remodeling in order to estimate the potential benefits of pharmacologic agents, monitor the need for dose titration, and minimize side effects. Imaging of the recognized ultrastructural components of cardiac remodeling can allow redefinition of heart failure based on its "molecular phenotype," and provide a guide to implementation of "personalized" and "evidence-based" evaluation, treatment, and longitudinal monitoring of the disease beyond what is currently available through randomized controlled clinical trials.

Clinical Application of Biomarkers in Heart Failure with a Preserved Ejection Fraction: A Review.

Heart failure with a preserved ejection fraction (HFpEF) is increasingly prevalent and a leading cause of morbidity and mortality worldwide. HFpEF has a complex pathophysiology, with recent evidence suggesting that an interaction of cardiovascular and noncardiovascular comorbidities (e.g. obesity, hypertension, diabetes, coronary artery disease, and chronic kidney disease) induces an inflammatory state that eventually leads to myocardial structural and functional alterations. Current ACCF/AHA guidelines suggest incorporation of biomarkers along with clinical and imaging tools to establish the diagnosis and disease severity in heart failure (HF). However, the majority of data on biomarkers relating to their levels, or their role in accurate diagnosis, prognostication, and disease activity, has been derived from studies in undifferentiated HF or HF with a reduced EF (HFrEF). As the understanding of the mechanisms underlying HFpEF continues to evolve, biomarkers reflecting different pathways including neurohormonal activation, myocardial injury, inflammation, and fibrosis have a clinical utility beyond the diagnostic scope. Accordingly, in this review article we describe the various established and novel plasma biomarkers and their emerging value in diagnosis, prognosis, response, and guiding of targeted therapy in patients with HFpEF.