Progressive Device Failure at Long Term Follow Up of the Nellix EndoVascular Aneurysm Sealing (EVAS) System.

OBJECTIVE: High rates of midterm failure of the Nellix EndoVascular Aneurysm Sealing (EVAS) System resulted in device withdrawal from the UK market. The study aim was to report long term Nellix EVAS outcomes and management of a failing device. METHODS: A retrospective review of EVAS procedures at a tertiary unit was performed. Device failure was defined as a triad of stent migration, stent separation, and secondary sac expansion, or any intervention for type 1 endoleak, device rupture, or explant. RESULTS: 161 (male n = 140, female n = 21) patients with a median follow up of 6.0 (IQR 5.0-6.6) years were included. Freedom from all cause mortality estimate at six years was 41.5%. There were 70 (43.5%) device failures with a freedom from device failure estimate at six years of 32.3%. Failure was the result of sac expansion (n = 41), caudal stent migration (n = 36), stent separation (n = 26), and secondary AAA rupture (n = 15). A substantial number of type 1 endoleaks was present (1a n = 33, 1b n = 11), but the type 2 endoleak rate was low at 3.7%. Some 36 (22.4%) patients required re-intervention. Twenty-one patients underwent explant with no 30 day deaths. Six patients underwent Nellix-in-Nellix application (NINA) with one early death from bowel ischaemia and one patient who died later from non-aneurysm related cause. Two NINA patients have ongoing sac expansion and two have had thrombosis of a Nellix limb or visceral stent. Proximal embolisation was only successful in one of six cases. CONCLUSION: The long term failure rate of Nellix EVAS is high. All patients with a device must be informed and be enrolled in enhanced surveillance. EVAS explant is an acceptable technique with favourable outcomes. Management by open explant, if the patient is fit, should be considered early and offered to those with device failure.

Ligation Alone Versus Immediate Revascularization for Femoral Artery Pseudoaneurysms Secondary to Intravascular Drug Use: A Systematic Review and Meta-Analysis.

BACKGROUND: Femoral artery pseudoaneurysms (FA-PSAs) remain a common vascular aneurysmal pathology associated with intravascular drug use (IVDU). To date no internationally agreed consensus regarding optimal surgical management of FA-PSAs exists. The aim of this systematic review and meta-analysis was to determine the optimal surgical treatment of FA-PSAs associated with IVDU. METHODS: A systematic search was undertaken following PRISMA (Preferred Reporting Items for Systematic reviews and Meta-Analyses) guidelines identifying original studies reporting outcomes of ligation-debridement and/or excision-revascularization of FA-PSAs secondary to IVDU. Outcomes of interest were 30-day mortality, incidence of amputation at 12 months, chronic limb threatening ischemia (CLTI) at any follow-up appointment, reintervention, and bleeding. RESULTS: A total of 39 cohort studies describing 1,217 FA-PSA operative outcomes met inclusion criteria, 993 (81.6%) treated by ligation-debridement and 224 (18.4%) by excision-revascularization. The incidence of 30-day mortality was 0.8% (n = 8) and 1.3% (n = 3) in the ligation-debridement and excision-revascularization groups, respectively, with only one study reporting mortality in both groups. This meta-analysis found no difference in amputation (8.89% vs. 8.03%, odds ratio (OR) 0.74 95% confidence interval (CI) 0.35-1.56, P = 0.42, 11 studies) or CLTI (21.5% vs. 12.4%, OR 1.24 95% CI 0.35-4.38, P = 0.74, 9 studies) after ligation and debridement compared with excision and revascularization. There was a higher incidence of reintervention (24.7% vs. 10.6%, OR 0.31 [95% CI 0.16, 0.62], P = 0.0009, 13 studies) and rebleeding (7.1% vs. 1.6%, OR 0.61 [95% CI 0.16, 2.38], P = 0.48, 5 studies) after excision and revascularization compared with ligation alone. CONCLUSIONS: For treatments of IVDU-related FA-PSAs, this study suggests no significant difference in association of mortality, incidence of amputation, or CLTI with ligation-debridement or excision-revascularization, but a significantly higher reintervention rate and greater rebleeding rate for revascularized patients.

Bladder-Drained Pancreas Transplantation: Urothelial Innate Defenses and Urinary Track Infection Susceptibility.

BACKGROUND: Pancreas transplantation restores insulin secretion in type 1 diabetes mellitus. The graft also produces exocrine secretions that can be drained enterically (enteric drainage [ED]) or via the bladder (bladder drainage [BD]). We suggest that in BD transplants, such secretions destroy bladder innate immunity, specifically host defense peptides/proteins (HDPs), which increases patient susceptibility to recurrent urinary tract infections (rUTIs). MATERIALS AND METHODS: BD and ED patient records were reviewed retrospectively for UTIs. Urine samples from ED and BD transplant recipients were analyzed for pH, the HDPs ß-defensin 2 (HBD2) and lipocalin-2, and amylase concentrations. In vitro, bacterial growth curves and antimicrobial assays were used to evaluate the effects of pH, HBD2, and HBD2 + pancreatic digestive enzymes (pancreatin) on uropathogenic Escherichia coli (UPEC) survival and growth. RESULTS: Urinalysis revealed a significant difference in pH between the BD and ED cohorts (7.2 ± 0.8 versus 6.7 ± 0.8; P = 0.012). Urinary HDPs were measured and BD, but not ED, lipocalin-2 concentrations were significantly decreased compared with those of diabetics awaiting transplant (P < 0.05). In vitro, an alkaline environment, pH 8.0, concomitant with the urine of the patient who underwent BD transplantation, significantly reduced UPEC growth (P < 0.05); addition of pancreatin to the growth medium was associated with a significant increase (P < 0.001) in growth rate. Antimicrobial data suggested significant UPEC killing in the presence of HBD2 (P < 0.01), but not in the presence of HBD2 + pancreatin (>12,500 amylase units). CONCLUSIONS: These in vivo and in vitro data suggest that BD pancreatic exocrine secretions inactivate the bladder innate defenses, which facilitate UPEC growth and underpins the increased susceptibility of patients who underwent BD pancreas transplantation to rUTIs.

Primary diffuse large B cell lymphoma of the base of tongue: a rare entity.

Primary lymphomas of the oral cavity are uncommon and of the tongue even rarer. It is more common in the masticatory mucosa than the lingual and buccal mucosa. We describe a 63 year old male who presented with dysphagia & change in voice. Computed tomography revealed a 4.8 × 3.7 cm mass localized to the base of tongue. He underwent biopsy and IHC studies & was diagnosed as having diffuse large B cell lymphoma. The patient was successfully treated with wide excision of the lesion and is undergoing chemotherapy now. Although oral lymphoma of tongue is very uncommon, it should always be considered in differential diagnosis of various benign and malignant lesions in this region. A proper clinical evaluation and histopathologic as well as immunohistochemical evaluation of biopsy specimen are required to establish the diagnosis and for further management. This is one of the few cases of non-Hodgkin's lymphoma of the base of tongue being reported from India.

Comparative Study of Ki-67 Labeling Index Quantification by Eye-rolling, Manual Count, and Digital Image Analysis; An Approach with Caution.

Background and Objective: An accurate Ki-67 labeling index assessment is critical for managing a few tumors, like breast carcinomas and neuroendocrine tumors. We aimed to determine the degree of agreement between digital image analysis (DIA) and eye-rolling assessment (EE) and DIA and manual count (MC) for Ki-67 LI scoring. Methods: A total of 120 cases (both tru-cut biopsies and resected specimens) were selected during the study period from the institutional database, wherein the Ki-67 labeling index was performed. The selected cases were divided into two groups, i.e., breast neoplasms and other neoplasms. The correlation between DIA and EE and DIA and MC for Ki-67 LI scoring was calculated in both groups. Results: A total of 113 cases were analyzed for Ki-67 LI by three different methods (EE, MC, and DIA); 7 cases were rejected due to poor image quality. Ki-67 LI scoring by DIA and EE was highly correlated in both study groups with a Spearman's rank correlation coefficient of 0.809 (P=0.01) and 0.904 (P=0.01), respectively. Correlation between DIA and MC methods was also found to be almost perfect in both study groups with a Spearman's rank correlation coefficient of 0.974 (P=0.01) and 0.955 (P=0.01), respectively. Conclusion: ImmunoRatio is a free web-based digital image analysis application that can be used for Ki-67 LI assessment with considerable reliability and reproducibility. Yet, it carries a few limitations and demands a careful approach and final confirmation by an expert.

Understanding the current acute aortic syndrome (AAS) pathways-The Collaborative Acute Aortic Syndrome Project (CAASP) protocol.

BACKGROUND: Acute aortic syndrome (AAS) is an emergency associated with high peri-hospital mortality rates. Variable clinical presentation makes timely diagnosis challenging and such delays in diagnosis directly impact patient outcomes. AIMS AND OBJECTIVES: The aims of the Collaborative Acute Aortic Syndrome Project (CAASP) are to characterise and evaluate the current AAS pathways of a cohort of hospitals in the UK, USA and New Zealand to determine if patient outcomes are influenced by the AAS pathway (time to hospital admission, diagnosis and management plan) and demographic, social, geographic and patient-specific factors (clinical presentation and comorbidities). The objectives are to describe different AAS pathways and time duration between hospital admission to diagnosis and management plan instigation, and to compare patient outcomes between pathways. METHODS: The study is a multicentre, retrospective service evaluation project of adult patients diagnosed on imaging with AAS. It will be coordinated by the UK National Interventional Radiology Trainee Research (UNITE) network and Vascular and Endovascular Research Network (VERN) in conjunction with The Aortic Dissection Charitable Trust (TADCT). All AAS cases diagnosed on imaging between 1st January 2018 to 1st June 2021 will be included and followed-up for 6 months. Eligibility criteria include aortic dissection (AD) Type A, Type B, non A/B, penetrating aortic ulcer, and intramural haematoma. Exclusion criteria are non-AAS pathology, acute on chronic AAS, and age<18. This project will evaluate patient demographics, timing of presentation, patient symptoms, risk factors for AD, physical examination findings, timing to imaging and treatment, hospital stay, and mortality. Univariate and multivariate analysis will be used to identify predictors associated with prolonged time to diagnosis or treatment and mortality at 30 days.

Understanding genomic medicine for thoracic aortic disease through the lens of induced pluripotent stem cells.

Thoracic aortic disease (TAD) is often silent until a life-threatening complication occurs. However, genetic information can inform both identification and treatment at an early stage. Indeed, a diagnosis is important for personalised surveillance and intervention plans, as well as cascade screening of family members. Currently, only 20% of heritable TAD patients have a causative mutation identified and, consequently, further advances in genetic coverage are required to define the remaining molecular landscape. The rapid expansion of next generation sequencing technologies is providing a huge resource of genetic data, but a critical issue remains in functionally validating these findings. Induced pluripotent stem cells (iPSCs) are patient-derived, reprogrammed cell lines which allow mechanistic insights, complex modelling of genetic disease and a platform to study aortic genetic variants. This review will address the need for iPSCs as a frontline diagnostic tool to evaluate variants identified by genomic discovery studies and explore their evolving role in biological insight through to drug discovery.

Liposarcoma of tongue: A case report.

ABSTRACT: Liposarcoma (LS) is one of the most common malignant tumors. However, oral LS is an extremely rare lesion that is often clinically misdiagnosed as a benign lesion because of its asymptomatic and indolent clinical course. In oral cavity, the tongue is the most frequent site for its occurrence.

A State-of-the-Art Review of Intra-Operative Imaging Modalities Used to Quality Assure Endovascular Aneurysm Repair.

Endovascular aortic aneurysm repair (EVAR) is the preferred method for elective abdominal aortic aneurysm (AAA) repair. However, the success of this technique depends greatly on the technologies available. Intra-operative imaging is essential but can come with limitations. More complex interventions lead to longer operating times, fluoroscopy times, and greater contrast doses. A number of intra-operative imaging modalities to quality assure the success of EVAR have been developed. A systematic literature search was performed with separate searches conducted for each imaging modality in the study: computed tomography (CT), digital subtraction angiography (DSA), fusion, ultrasound, intra-operative positioning system (IOPS), and non-contrast imaging. CT was effective at detecting complications but commonly resulted in increased radiation and contrast dose. The effectiveness of DSA can be increased, and radiation exposure reduced, through the use of adjunctive technologies. We found that 2D-3D fusion was non-inferior to 3D-3D and led to reduced radiation and contrast dose. Non-contrast imaging occasionally led to higher doses of radiation. Ultrasound was particularly effective in the detection of type II endoleaks with reduced radiation and contrast use but was often operator dependent. Unfortunately, no papers made it past full text screening for IOPS. All of the imaging techniques discussed have advantages and disadvantages, and clinical context is relevant to guide imaging choice. Fusion and ultrasound in particular show promise for the future.

Pyoderma Gangrenosum with Splenic Abscess- A Rare Association.

Pyoderma gangrenosum is a rare, chronic neutrophilic dermatosis of unknown etiology. The classical clinical feature of pyoderma gangrenosum is a pustule or plaque that rapidly progresses to a painful, necrotic ulcer with undermined violaceous margins. Pyoderma gangrenosum may be associated with underlying inflammatory bowel diseases, hematological malignancies, or rheumatologic disorders in 50-70% of the cases. The visceral involvement by pyoderma gangrenosum is rare. Sterile neutrophilic infiltrates in organs other than the skin are uncommon systemic manifestations of neutrophilic dermatoses, but have occasionally been reported. We report a case of a 38-year-old female with pyoderma gangrenosum and visceral involvement manifesting as splenic abscess.

Proton Pump Inhibitor Induced Subacute Cutaneous Lupus Erythematosus: A Case Series of 7 Patients and Brief Review of Literature.

BACKGROUND: Subacute cutaneous lupus erythematosus (SCLE) manifests with erythematous, nonscarring, annular, or papulosquamous plaques. Proton pump inhibitors (PPIs) are increasingly being incriminated in its causation, but reports of similar nature from India are lacking. AIMS: To describe the characteristics of seven patients with SCLE induced by PPIs and to review the published cases in order to provide a better perspective of the association. MATERIALS AND METHODS: We describe seven patients of PPI-induced SCLE, seen over a period of 6 years. We also review the literature for additional data on PPI-induced SCLE. The selected publications were reviewed, and relevant clinical and laboratory data were extracted. RESULTS: Of the total seven cases, there were four males and three females with a mean age of 60.2 ± 5.5 years (range 53-70 years). Nine episodes of PPI-induced SCLE were recorded in the seven patients. Of the initial episodes, esomeprazole was implicated in four, pantoprazole in two, and rabeprazole in one patient. Latency period ranged from 2 weeks to 1 year (mean 11.4 ± 16.2 weeks). Morphology was described as annular scaly plaques in six and papulosquamous in one. Antinuclear antibodies and anti-Ro antibodies were positive in all patients. Naranjo probability scale was used in all patients; two were categorized as definite and five as probable. Treatments included drug withdrawal in six patients, topical steroids in one, systemic corticosteroids in all seven, and hydroxychloroquine in one patient, used alone or in combinations. Complete remission was achieved in six cases, while one had partial remission. LIMITATION: Retrospective nature of this study and limited number of patients. CONCLUSION: PPIs can trigger SCLE.

Isolated pancreatic myeloid sarcoma: A potential mimicker of pancreatic adenocarcinoma.

Myeloid sarcoma (MS) is an extramedullary proliferation of immature myeloid cells which may occur as a progression of myelodysplastic syndrome (MDS), myeloproliferative neoplasm (MPN), or myelodysplastic syndrome/myeloproliferative neoplasm (MDS/MPN) and as acute myeloid leukemia (AML) relapse. Rarely may it be de novo. Lymph nodes, skin, lungs, intestine are the commonly involved sites. However, an isolated pancreatic MS is seldom reported in the literature. Herein, we report one such case which was misdiagnosed as pancreatic adenocarcinoma on the clinico-radiological examination which misled us away from preoperative diagnostic sampling, and a Whipple pancreaticoduodenectomy was performed. Histopathological examination in conjunction with immunohistochemistry revealed the final diagnosis of isolated MS of the pancreas. We emphasize that although rare, a clinical suspicion along with preoperative histopathological examination may lead to early diagnosis, targeted management, and a better clinical outcome in such cases.

Diagnostic Accuracy of Calculated Tumor Volumes and Apparent Diffusion Coefficient Values in Predicting Endometrial Cancer Grade.

Background: Magnetic resonance imaging (MRI) has been shown to be an accurate imaging technique for the preoperative assessment of local staging of endometrial cancer and for evaluating the depth of myometrial invasion. Materials and Methods: This was a single-center retrospective study performed on patients with histopathologically proven endometrial carcinoma who underwent an MRI examination of the pelvis between October 2017 and May 2020. Results: In the present analysis, mean apparent diffusion coefficient (ADC) values for each histologic grade were 0.72 ± 0.13 × 10-3 mm2/s (G1), 0.76 ± 0.17 × 10-3 mm2/s (G2), and 0.74 ± 0.12 × 10-3 mm2/s (G3), respectively, showing no significant correlation between ADC values and tumor grade (P = 0.73). Overall, ADC minimum was significant in differentiating grades of endometrial carcinoma (P = 0.02) with the ability to differentiate Grade I and II lesions (P = 0.01). A mean tumor volume of 25.2 cc could differentiate low-grade tumors (Grade I and Grade II) from high-grade tumors (Grade III) with a sensitivity and specificity of 88% and specificity of 89%. The tumor volume/uterine volume ratio (TV/UV) differentiates high-grade tumors from low-grade tumors (P < 0.001), however, no significant difference in the ratio was observed among Grade I and II lesions (P = 0.48). The area under the curve of tumor volume was 0.875 (95% confidence interval 0.0-1.00) (P = 0.001), indicating that tumor volume was an effective tool for distinguishing high-grade and low-grade endometrioid adenocarcinomas. The corresponding sensitivity and specificity were 88.0% and 89.0%, respectively. Conclusion: Preoperative noninvasive radiological assessment for tumor volume, TV/ UV or tumor volume/uterine volume is important surrogate markers for preoperative prognostication of endometrial carcinoma.