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Background: In recent years, knowledge about cancer associated thrombosis has evolved considerably., Methods: Practical guidelines were drafted on the initiative of the INNOVTE FCRIN Network, led by the French Speaking Society of Respiratory Diseases (SPLF), by a coordinating group, a writing group, and a review group, with the involvement of different scientific societies practicing in various settings. The method followed the "Clinical Practice Guidelines" process of the French National Authority for Health (HAS)., Results: After a literature review, guidelines were formulated, improved, and then validated by the working groups. These guidelines addressed multiple aspects of the disease and management from the data of available clinical trials and observational studies : epidemiology, initial treatment, treatment duration, extended treatment, recurrent thrombosis, central venous catheter thrombosis, incidental thrombosis, treatment in case of thrombocytopenia., Conclusion: These evidence-based guidelines are intended to guide the practical management of patients with cancer associated thrombosis., Competing Interests: Declaration of Competing Interest I. Mahé: Grants: BMS, Pfizer; Consulting fees : BMS/Pfizer, Leo-Pharma, Sanofi, Aventis, Astra-Zeneca; Support for attending meeting: BMS/Pfizer, Leo-Pharma. P. Girard: Payment or Honoria for lectures: Leo-Pharma, MBS/Pfizer, Bayer; Support for attending meeting: Leo-Pharma; L. Bertoletti: Grants: MSD, Bayer; Payment or Honoria for lectures: Leo-Pharma, MBS/Pfizer, MSD, Viatris; Support for attending meeting: Leo-Pharma, MBS/Pfizer, MSD, Viatris. S. Laporte: Consulting fees: Ferring; Payment or Honoria for lectures: Pfizer, Lilly. F Couturaud : Grants: Bayer, BMS, Pfizer, GSK, Astra, Leo-Pharma, MSD, Roche; Payment or Honoria for lectures: Bayer, BMS, GSK, Astra, Leo-Pharma, Sanofi, MSD; Support for attending meeting: Astra, MSD. P. Mismetti : None O. Sanchez : Consulting fees: Bayer, BMS/Pfizer, Leo-Pharma, Sanofi/Aventis, Boehringer, Inari, Boston Scientifics, MSD; Payment or Honoria for lectures: Bayer, BMS/Pfizer, Leo-Pharma, Sanofi Aventis, Boehringer, Inari, Boston Scientifics, MSD, Viatris; Support for attending meetings and/or travel: Oxyvie, BMS/Pfizer, (Copyright © 2023 The Authors. Published by Elsevier Masson SAS.. All rights reserved.)

Objective: Upper extremity (UE) deep vein thrombosis (DVT) is a common and increasing complication in hospitalized patients. The objective of the present study was to determine the prevalence, treatment strategies, complications, and outcomes of UE-DVT., Methods: We performed a retrospective single-institution study of patients with a diagnosis of UE-DVT from January 2016 through February 2018 (26 months). Patients aged ≥18 years who had been admitted to the hospital and who had had positive UE duplex ultrasound findings for acute UE-DVT were included in the present study. The outcomes were in-hospital mortality, major bleeding, pulmonary embolism (PE), and recurrent UE-DVT., Results: Among 63,045 patients admitted to the hospital, 1000 (1.6%) had been diagnosed with UE-DVT. Of 3695 UE venous duplex ultrasound examinations performed during the study period, almost one third (27.0%) were positive for acute UE-DVT. The mean age was 55.0 ± 17.2 years, and most patients were men (58.3%), white (49.2%), and overweight (mean body mass index, 29.4 ± 10.3 kg/m 2 ). The most affected vein was the right internal jugular vein (54.8%). Most of the patients (96.9%) has been receiving venous thromboembolism prophylaxis or anticoagulation therapy at the diagnosis. Most patients (77.8%) had had an intravenous device (IVD) in place at the diagnosis. Most of the patients (84.4%) were treated with anticoagulation therapy in the hospital but only one half (54.5%) were discharged with anticoagulation therapy. In-hospital mortality was 12.1% unrelated to UE-DVT, major bleeding occurred in 47.6% of the patients during hospitalization (fatal bleeding, 1%), PE was diagnosed in 4.8% of the patients, and 0.7% were fatal. Recurrent UE-DVT occurred in 6.1% of the patients. On multivariable analysis, the risk of death was increased by older age, cancer, intensive care unit admission, concomitant lower extremity DVT, and bleeding before the UE-DVT diagnosis. The presence of an IVD increased the risk of PE and the risk of recurrent UE-DVT. The risk of major bleeding was increased by the presence of an IVD, female sex, and concomitant lower extremity DVT., Conclusions: UE-DVT is a common complication in hospitalized patients (1.6%). Consequent acute PE and recurrent DVT remain important complications, as does bleeding. It is unclear whether standard thromboprophylaxis effectively protects against UE-DVT. More studies dedicated to UE-DVT are required to provide appropriate guidance on prophylaxis and treatment., (Copyright © 2021 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: Post-thrombotic syndrome (PTS) is one of the main complications that occurs after venous thrombosis. There are few data on the proportion of patients that will develop upper extremity PTS (UE-PTS) after upper extremity venous thrombosis (UEVT). The main objective of the study was to assess the prevalence of PTS in a UEVT cohort and to identify predictive factors of UE-PTS., Methods: This study included patients with a history of proximal or arm UEVT, diagnosed on duplex ultrasound examination, between January 1, 2015, and December 31, 2017, in a university hospital. After UEVT, each patient was evaluated by a prospective standardized recording of clinical manifestations and duplex ultrasound examination in case of upper limb symptoms. UE-PTS was defined as a modified Villalta score of 4 or higher., Results: Ninety-two patients were included; 68 (73.9%) had deep vein thrombosis (DVT) and 24 (19.2%) arm superficial vein thrombosis. Thirteen patients had PTS (14.1%), 12 (17.6%) in the DVT group and 1 (4.2%) in the superficial vein thrombosis group. There was a history of DVT in 92.3% of the cases of PTS. PTS was more frequent in patients with strokes with limb movement reduction (P = .01). On multivariate Cox analysis, a history of stroke (hazard ratio, 5.4; 95% confidence interval, 1.46-20.22; P = .01) was predictive of UE-PTS., Conclusions: UE-PTS occurred in 14.1% of cases after UEVT. Stroke with a decrease in limb movement was a predictor of developing PTS. Diagnostic criteria should be established for UE-PTS and prospective studies are needed to improve the description and management of UE-PTS., (Copyright © 2021 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Phlegmasia cerulea dolens (PCD) is a rare condition characterized by deep venous thrombosis with gangrene. It can result in critically severe edema that affects the blood supply to the limbs. PCD generally occurs in the lower rather than upper extremity. We herein present a case report of upper extremity PCD and discuss thrombophilia secondary to low protein S activity as the main cause. Catheter-directed thrombolysis via the occluded end of the artery may be one of the best treatment methods for PCD., (Copyright © 2021. Published by Elsevier Inc.)

Background: Paget-Schroetter syndrome (PSS) is a rare condition of axillosubclavian vein thrombosis often seen in athletes with a history of repetitive external rotation and abduction of the shoulder. The purpose of this review was to analyze the literature and characterize PSS in the athletic population, including risk of PSS by sport. We also provide a comprehensive review of PSS to inform clinicians on the pathophysiology, detection, and management of the condition., Methods: Four databases were reviewed to identify cases of PSS occurring in athletes. Data on patient demographics, reported sport, diagnosis, treatment, management, return to sport, and complications were extracted and analyzed by 2 independent reviewers., Results: Of the 123 cases of PSS identified, baseball and weight lifting had the highest incidence (26.8% and 19%, respectively), followed by swimming, football, and basketball. The average return to sport was 4.7 months, and 26.7% of subjects reported complications, most commonly pulmonary embolism., Conclusion: In athletes presenting with upper extremity pain and swelling with a history of playing baseball or weight lifting, PSS should be higher on a clinicians differential diagnosis list. Swimmers, football, and basketball players are less likely to present with PSS but are still more likely than other types of athletes to develop the condition. Clinician awareness of PSS in athletes is critical to avoid delays in treatment and misdiagnosis, and to allow for a timely return to sport with minimal complications., (Published by Elsevier Inc.)

Introduction: Central venous catheters related thrombosis (CRT) insertion has been shown to increase the risk of venous thromboembolism, particularly pulmonary embolism (PE). Nevertheless, deaths cased due to PE have been rarely reported., Patient Concerns: A central venous catheter was introduced through the right jugular vein during the operation due to severe septic shock from a 57-year-old male patient. Two days after surgery, the hemodynamics was stable. On the 7th day, and low molecular weight heparin calcium (4100 units, once a day) was added for anticoagulation to prevent venous thromboembolism. On the 15th day, during the process of central venous catheter removal, the patient suddenly lost consciousness, suffered cardiac arrest, and received emergency cardiopulmonary resuscitation., Diagnosis: Jugular venous catheter-associated thrombosis and fatal PE. An acute bedside ultrasound showed a thrombus drifting with the blood stream in the right jugular vein. The lower section of the xiphoid process by echocardiography showed decreased systolic amplitude of the right atrium and right ventricle, widened and fixed inferior vena cava, and no variation with respiration. Para-sternal left ventricular long axis section showed that the right ventricular outflow tract was significantly extended, and the contraction amplitude of the anterior and posterior walls of the left ventricle decreased. Left ventricular short axis section indicated a right ventricle enlargement and ventricular septum deviation of left ventricle, showing "D" sign. Apical 4-chamber view showed that the right ventricular ratio increased and the contractile capacity decreased., Interventions: One and a half million units of urokinase were immediately given trough intravenous drip., Outcomes: Twenty minutes after thrombolysis, the patient's autonomic heart rhythm was recovered, but continued to suffer from hypotension and coma, followed by multiple organ failure, and died 50 hours later., Conclusion: Recent clinical practice guidelines recommend against the routine use of any anticoagulant thromboprophylaxis in patients with central venous catheters, but for patients at particularly high risk for CRT, consideration can be given to using higher doses of anticoagulant as prophylaxis, although there are virtually no data to support this approach.

BACKGROUND Risk factors for venous thromboembolism can include a combination of genetic, anatomic, and physiologic factors, some of which are modifiable. Patients presenting to the hospital with venous thromboembolism may have multiple risk factors that require testing beyond the initial admission labs and hypercoagulability screening panel. CASE REPORT We describe a right-handed patient who lifts weights for exercise, who presented with pulmonary infarcts and clot in the right superior vena cava/subclavian vein. These were due to a combination of 1) an acquired hypercoagulability from minimal change disease and 2) dynamic anatomic narrowing of the subclavian vein, which is known as Paget-Schroetter syndrome. Despite normal serum levels of antithrombin, protein C and S, his serum albumin was low, which prompted workup for proteinuria. Testing revealed nephrotic range proteinuria as well as dynamic occlusion of the right subclavian vein on magnetic resonance venography only when the patient lifted and externally rotated his arms. CONCLUSIONS This case report highlights the need for a thorough history and physical examination, as well as additional testing in some patients beyond the initial admission laboratory tests and screening panel for hypercoagulability. Tests could include diagnostic imaging testing with provoking maneuvers, which can help elucidate dynamic physiology. Such testing, when appropriate, can help to inform the treatment plan and prevent recurrent thromboses.

Introduction: There is a paucity of data on patients with deep vein thrombosis (DVT) in upper extremities., Materials and Methods: The COMMAND VTE Registry is a retrospective multicenter registry enrolling 3027 consecutive patients with acute symptomatic venous thromboembolism (VTE) in Japan. The current study population included 2498 patients with upper or lower extremities DVT., Results: There were 74 patients (3.0%) with upper extremities DVT and 2424 patients with lower extremities DVT. Patients with upper extremities DVT more often had active cancer (58%) and central venous catheter use (22%). The proportion of concomitant pulmonary embolism at diagnosis was lower in patients with upper extremities DVT than in those with lower extremities DVT (14% and 51%, P < 0.001). Discontinuation of anticoagulation therapy was more frequent in patients with upper extremities DVT (63.8% and 29.8% at 1-year, P < 0.001). The cumulative 3-year incidence of recurrent VTE was not different between the 2 groups (9.8% and 7.4%, P = 0.43). After adjusting confounders, the risks of upper extremities DVT relative to lower extremities DVT for recurrent VTE remained insignificant (HR 0.94, 95%CI 0.36-2.01, P = 0.89)., Conclusions: The prevalence of patients with DVT in upper extremities was 3.0% in the current large-scale real-world registry. Patients with DVT in upper extremities more often had active cancer at diagnosis and central venous catheter use as a transient risk factor for VTE, and less often had concomitant PE. Patients with DVT in upper extremities had similar long-term risk for recurrent VTE as those with DVT in lower extremities despite shorter duration of anticoagulation., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Background: Internal jugular venous thrombosis (IJVT) is an uncommon condition rarely diagnosed in the outpatient setting. IJVT carries significant morbidity and mortality and must be considered in the differential diagnosis for new-onset neck pain and swelling, especially in the emergency setting. Paget-Schroetter syndrome (PSS), or primary thrombosis secondary to effort, is an uncommon, likely under-recognized etiology of thrombosis. We report a case of PSS extending from the right subclavian vein into the right internal jugular vein, suspected based upon patient history and physical examination and confirmed by point-of-care ultrasound (POCUS). We then review the presentation, causes, and diagnostic standards for PSS., Case Report: We present a case of a 79-year-old man who presented to the Emergency Department with acute right-sided neck pain 1 day after playing the saxophone for 4 h the prior evening. POCUS confirmed Paget-Schroetter syndrome, or primary effort thrombosis of the internal jugular vein. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: To our knowledge, this is the first documented case of PSS resulting from venous stasis with prolonged Valsalva maneuver and vascular trauma with activity of playing the saxophone. The significance of this case is the unusual etiology of a rare presentation and the ability to diagnose this condition quickly and accurately with POCUS., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Introduction: There is limited data on the occurrence of complications in patients with upper extremity deep vein thrombosis (UEDVT)., Aims: We aimed to determine the frequency of post-thrombotic syndrome (PTS), thrombosis recurrence and major bleeding (MB) in patients with UEDVT., Material and Methods: We conducted a systematic review of the literature including studies from 1970 onwards. We included observational studies, randomized trials, or cases series including >20 patients. We calculated pooled proportions using a random effects model. Subgroup analyses according to etiology and treatment modality were conducted., Results: A total of 62 studies comprising 3550 patients were included. The pooled proportions for PTS and recurrence were 19.4% (95% CI 11.3-27.6) and 7.5% (95% CI 4.1-10.9), respectively. With a mean follow up of 6 months, the proportion of PTS was higher in patients with primary (unprovoked) UEDVT compared to secondary, whereas recurrence was higher in secondary UEDVT. PTS was more frequent in patients treated with anticoagulation alone compared to thrombolysis or surgical decompression. The pooled proportion for MB was 5.0% (95% CI 0.3-9.7) after anticoagulation alone and 3.8% (95% CI: 2.4-5.8%) after thrombolysis and/or surgery., Conclusions: This study suggests that UEDVT is associated with significant rates of PTS and recurrence and its treatment has a relatively low risk of major bleeding. Differences exist depending on etiology and treatment modality., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Competing Interests: Competing interests: None declared.

A young man presented with the severe right upper limb swelling following a heavy weight lifting that was thought to be caused by a biceps tendon rupture. However, subsequent investigations confirmed the diagnosis of Paget-Schroetter syndrome that was associated with an incidental pulmonary embolism. The patient underwent a successful thrombolysis followed by a surgical thoracic outlet decompression. Overall, the patient has made a good recovery., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Background: This study aims to evaluate the efficacy and safety of ultrasound-accelerated catheter-directed thrombolysis (UACDT) in the treatment of upper extremity deep vein thrombosis (UEDVT)., Methods: We conducted a prospective, observational cohort study of consecutive patients with acute UEDVT with low-dose UACDT using the Ekosonic® Endovascular System (EKOS Corporation, Bothell, WA, USA) at a single center from September 2012 until October 2014. Overall, sixteen patients (11 males and 6 females, age range 18 to 70 years, mean age, 45.6 years) were included in the study protocol. The primary efficacy outcome was complete thrombus clearance. The primary safety outcomes were recurrence of thrombosis within the follow-up visit and major bleeding within 96 hours of the procedure initiation., Results: The median tissue plasminogen activator (tPA) dose for all patients in our study was 16.81±2.51 mg (range 15 to 28 mg) and the median infusion time was 15 hours. Complete thrombus clearance was achieved in 11 (68.8%) patients, and partial clearance was detected in 3 (18.8%) patients. Of 16 patients with UEDVT, two had gastrointestinal bleeding, and two had puncture site bleeding., Conclusions: This prospective study demonstrates effectiveness and safety of ultrasound accelerated thrombolysis in patients with UEDVT.

Background: To assess subclavian vein (SCV) patency and long-term functional outcomes following surgical decompression of the thoracic outlet (SDTO) for Paget-Schroetter Syndrome (PSS)., Methods: Between January 1978 and January 2013, we identified 33 patients with PSS who underwent SDTO. Demographic, clinical and radiological data were extracted from electronic databases and patient records. All patients were invited to update their follow-up data during dedicated outpatient visits between October and December 2013. Outcome measures included long-term SCV patency and clinical success rates during follow-up. Clinical success was defined as the combined absence of functional symptoms and patient's ability to maintain normal professional activities at final follow-up. The QuickDASH score was also determined., Results: The study population comprised 17 men and 16 women (mean age 34 years; range: 14-53 years) with PSS. Diagnosis was reached by venography (29 cases) or duplex scan (4 cases). SDTO was performed via the transaxillary route (25 cases) or using the combined supra-infraclavicular approach (8 cases). The procedure was carried out within 10 days in 13 patients (early-group), and between 30 to 120 days in the remaining 20 patients (late-group). The former had SCV recanalization obtained actively by thrombolysis (3 cases), thrombectomy (9 cases) or endovenectomy followed by patch venoplasty (1 case). The latter were maintained under chronic oral anticoagulation to allow SCV recanalization. There was neither postoperative death nor major bleeding complications. At a median follow-up of 240 months, 11 SCV remained patent in the early group, while in the other there was 3 re-occlusions, 4 residual stenoses and 5 chronic SCV occlusions. Clinical success was achieved in 73% of patients for the whole cohort, but was significantly better in patients operated on in the early stages (100% vs. 55%; P=0.005). The mean Quick Disabilities of the Arm, Shoulder, and Hand Score was 3.5 (95% CI: 1.5-5.4) in the early-group and 17.3 (95% CI: 8.4-26.2) in the late-group (P=0.01)., Conclusions: Our data shows that long-term functional outcomes and SCV patency remained better in PSS patients who underwent early SDTO and active SCV recanalization techniques.

The study highlights pulmonary embolism and deep vein thrombosis by methylene tetrahydrofolate reductase (MTHFR) deficiency-related hyperhomocysteinemia occurring in rare locations of left veins superior to the heart extensively. A 59-year-old white man with history of leg pain, smoking, weight loss, benign prostatic hyperplasia, lipoma and panic attack presented with shortness of breath and chest pain for 2 days precipitated by not feeling well for months. The diagnostic workup revealed pulmonary embolism and deep vein thrombosis in the left subclavian vein which extended throughout the left brachiocephalic vein to the superior vena cava and left jugular vein. Further workup showed moderate hyperhomocysteinemia with normal levels of vitamin B6, B12 and folic acid. Methylene tetrahydrofolate reductase genetic study found the patient to be homozygous for G677T variant. He was started on low-molecular-weight heparin and was discharged on oral anticoagulant. No recurrent thrombotic episodes were witnessed after 4 months of follow-up after discharge.

Upper extremity deep vein thrombosis is a relatively rare condition which has thus far not attracted much interest in the literature. The differences in its aetiopathology, different diagnostic modalities and the current standard of care are discussed in this article.

We describe the case of a 23-year-old serviceman on overseas deployment who presented with a painful, swollen arm. Investigations showed an upper extremity deep vein thrombosis (UEDVT) of the right arm with an associated asymptomatic pulmonary embolism, which was treated with warfarin anticoagulation. Further investigation identified positional obstruction at the thoracic outlet, and the patient was diagnosed with Paget-Schroetter syndrome. He underwent elective resection of the first rib, and has now returned to normal duties. After review of the literature on UEDVT, it is suggested that in this military patient, the occurrence of an anatomical variant put him at risk of upper limb venous thrombosis, which was probably potentiated by the occupational factor of carrying a rifle. The external compression of the subclavian vein from the rifle butt and hypertrophied muscles, in addition to the anatomical variation, caused repetitive microtrauma of the vessel intima, which precipitated venous thrombosis., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/)

A 28-year-old male was referred to our emergency department with a swollen left arm after intensive exercise. Physical examination revealed distension of the superficial veins of his left arm and chest. Duplex ultrasound confirmed the diagnosis of Paget-Schroetter syndrome. He was successfully treated with thrombolysis and first rib resection.

Occlusive central line-related complications are not infrequent in children undergoing cancer therapy, but are generally not associated with life-threatening complications. Thrombosis of the superior vena cava (SVC) is rarely described in such patients, and downhill esophageal varices have been described in children and adults as a complication of altered SVC blood flow. The management of patients with SVC thrombosis and associated varices is complicated by the need to treat the thrombus weighed against bleeding risk. We present a 14-year-old adolescent with a history of acute leukemia and central line-related complications, including SVC thrombosis with subsequent formation of downhill esophageal varices. Conservative management consisting of anticoagulation alone resulted in resolution of the varices with no bleeding complications.

A 59-year-old woman was admitted three times over a six-month period with recurrent upper extremity deep venous thrombosis (UEDVT). It was determined that this patient was suffering from an unusual presentation of Paget-Schröetter syndrome secondary to a 20-year-old non-union of a midshaft clavicle fracture. Following thrombolysis the patient underwent resection and plate fixation of the clavicle fracture non-union. Despite the anatomic proximity of the subclavian vessels to the clavicle, vascular complications from fracture are rare. Treatment of midshaft clavicle fractures is often non-operative. Non-union rates are generally less than 10%, and easily treated secondarily without complication. Clavicular pseudo-arthroses from trauma have been implicated in the development of the thoracic outlet syndromes, however, onset 20 years after fracture has never before been reported., (© The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.)

Despite its relatively estimated high occurrence, the characterization of pediatric upper extremity deep vein thrombosis (UE-DVT) and of UE postthrombotic syndrome (PTS) is still lacking. We investigated the occurrence, characteristics, and predictors of UE-PTS in a cohort of children with objectively confirmed UE-DVT. Patients were analyzed in 3 groups according to DVT pathogenesis and neonatal status: primary (G1), secondary neonates (G2neonates), and non-neonates (G2non-neonates). A total of 158 children (23 G1, 25 G2neonates, and 110 G2non-neonates) were included. The most common triggering factors were effort-related (87%) in G1 and central lines in G2neonates (100%) and in G2non-neonates (92%). PTS scores ≥1, as per the Modified Villalta Scale, were identified in 87% of primary patients, 16% of G2neonates, and 49% of G2non-neonates. Survival analysis showed that the time to PTS score ≥1 significantly differed among group (log-rank test P < .0001). A multivariable logistic regression showed that DVT pathogenesis and imaging-determined degree of thrombus resolution at the end of therapy were independent predictors of a PTS score ≥2. In conclusion, pediatric UE-PTS frequency and severity depend on UE-DVT pathogenesis (primary/secondary) and, within the secondary group, on patient's age. Line-related UE-PTS has a more benign course, particularly in neonates., (© 2014 by The American Society of Hematology.)

Patient: Male, 16., Final Diagnosis: Effort thrombosis (Paget-Schroetter Sydnrome)., Symptoms: Swollen arms., Medication: -., Clinical Procedure: -., Specialty: Metabolic Disorders and Diabetics., Objective: Rare disease., Background: Thrombotic events in otherwise healthy pediatric patients are rare. In patients presenting with limb swelling, thrombosis must be considered in the differential diagnosis. In pediatric patients with thrombosis, there has been wide variability in the rates of associated thrombophilia. Many pediatric patients may instead have other contributors such as venous catheters or physical activity., Case Report: We present a case of bilateral upper extremity deep venous thrombi in a previously healthy 16-year-old male. The patient presented with swelling and pain in both arms after several days of weight-bearing exercise. Following emergency department evaluation with ultrasound and laboratory testing, the patient was diagnosed with effort thrombosis - also known as Paget-Schroetter syndrome - and rhabdomyolysis., Conclusions: This case of Paget-Schroetter syndrome is distinguished by elevation in creatine kinase and transaminases. While these findings can be due to physical exertion and effort, effort thrombosis is not classically associated with laboratory abnormalities except an elevated D-dimer. The significance of these laboratory test result abnormalities remains unclear. Given the rarity of effort thrombosis, further epidemiological study is warranted to determine if these laboratory findings are seen in other cases, and, if so, what implications they may have for management and prognosis.

Objective: To determined the current incidence and acute complications of asymptomatic central venous catheter (CVC)-related deep venous thrombosis (DVT) in critically ill children., Study Design: We performed a prospective cohort study in 3 pediatric intensive care units. A total of 101 children with newly inserted untunneled CVC were included. CVC-related DVT was diagnosed using compression ultrasonography with color Doppler., Results: Asymptomatic CVC-related DVT was diagnosed in 16 (15.8%) children, which equated to 24.7 cases per 1000 CVC-days. Age was independently associated with DVT. Compared with children aged <1 year, children aged >13 years had significantly higher odds of DVT (aOR, 14.1, 95% CI, 1.9-105.8; P = .01). Other patient demographics, interventions (including anticoagulant use), and CVC characteristics did not differ between children with and without DVT. Mortality-adjusted duration of mechanical ventilation, a surrogate for pulmonary embolism, was statistically similar in the 2 groups (22 ± 9 days in children with DVT vs 23 ± 7 days in children without DVT; P = .34). Mortality-adjusted intensive care unit and hospital lengths of stay also were similar in the 2 groups., Conclusion: Asymptomatic CVC-related DVT is common in critically ill children. However, the acute complications do not seem to differ between children with and without DVT. Larger studies are needed to confirm these results. Future studies should also investigate the chronic complications of asymptomatic CVC-related DVT., (Copyright © 2013 Mosby, Inc. All rights reserved.)

Background: Patent foramen ovale (PFO) has been associated with cryptogenic stroke, particularly in young adults. However, the source of particles leading to cerebral embolism remains frequently unknown despite comprehensive evaluation., Objective: To report and comment on therapeutic options for 2 patients with acute ischemic strokes, PFO, and venous access related thrombosis, sources of paradoxical embolism, from Boston Medical Center stroke database., Case Descriptions: Case 1. A 71-year-old man presented with brain magnetic resonance imaging (MRI) confirmed acute cerebellar infarction. Echocardiography showed a PFO and thrombotic material at the tip of a peripherally inserted central catheter (PICC) line in the superior vena cava (SVC) prolapsing into the right atrium (RA). Case 2. A 64-year-old woman with end-stage renal disease and PFO presented with brain MRI confirmed acute parietal lobe infarction. Three days prior to her stroke, she had thrombectomy and venoplasty of an arterio-venous (AV) dialysis graft followed by a post-thrombectomy fistulogram that showed persistent thrombotic material at the venous site., Conclusions: PFO associated with large venous access site thrombosis was the most likely mechanism of stroke in both cases. Local thrombosis at sites of large venous access may be an overlooked source of paradoxical embolism in patients with PFO as well as a preventable cause of stroke in critically ill patients., (Copyright © 2011 by the American Society of Neuroimaging.)

Background: Subclavian vein thrombosis (SVT) is usually caused by vigorous activity or extensive use of the upper extremity. Patients are tested for hypercoagulability if they present with a spontaneous clot unassociated with such activity. The objective of this study was to determine the prevalence of hypercoagulability in patients undergoing first rib resection and scalenectomy presenting with SVT., Methods: Using a prospectively maintained database from August 2003 through June 2011, patients were retrospectively reviewed for hypercoagulability testing and clinical outcomes., Results: One hundred forty-three patients (79 females and 64 males; mean [range] age, 32 [16-71] years) presented with SVT, of whom 55 patients (43 females and 12 males; mean age, 32 [16-61] years) had undergone hypercoagulable testing. Fourteen patients (25.5%) (12 females and 2 males; mean age, 27 [16-46] years) had an abnormal hypercoagulable profile. A factor V Leiden mutation was present in 6 patients, protein S deficiency in 4, a plasminogen-activator inhibitor-1 (PAI-1) deficiency in 2, and 1 patient each with protein C deficiency, anticardiolipin antibodies, factor VII mutation, factor II mutation, and antiphospholipid antibodies. Immediate and long-term postoperative vein patency was similar to patients without hypercoagulability. Patients were placed on lifelong anticoagulation therapy if they had a PAI-1, protein C, or protein S deficiency., Conclusions: Patients with hypercoagulability do as well with first rib resection and scalenectomy for SVT as those without hypercoagulability. In our patient subset, more females were tested owing to a history of spontaneous thrombosis and an increased incidence of hypercoagulable disorders. Because of our findings, we believe younger women with SVT should undergo hypercoagulable testing to identify the need for long-term anticoagulation therapy.

Cardiac implantable electronic device (CIED) leads can cause central venous stenosis (CVS). In addition, these devices can get infected. Both are critically important considerations in patients with chronic kidney disease (CKD) for at least two reasons: (i) central veins serve as the final pathway should these patients need an arteriovenous access to provide dialysis therapy; and (ii) the presence of renal failure increases the risk of CIED infection. In this analysis, we investigated the prevalence as well as the degree of chronic kidney disease in patients harboring a CIED. Patients undergoing CIED removal were evaluated from 2001 to 2011. The patients were categorized into CKD stage I-V based on National Kidney Foundation-Dialysis Outcomes Quality Initiative guidelines. A total of 503 patients underwent CIED removal. Demographic characteristics revealed that 30% had hypertension, 44% were diabetics, 77% had coronary artery disease, and 84% suffered from congestive heart failure. Ninety percent (452/503) of the patients had CKD (stage I = I9 [4.2%], stage II = 189 [41.8%], stage III A = 96 [21.2%], stage III B = 59 [13.0%], stage IV = 45 [9.9%], and stage V = 44 [9.7%]). Overall, 148 (32.7%) patients (stage III B, stage IV, and stage V) of 452 had advanced renal failure. The results of this study reveal that one-third of CIED patients undergoing device removal have advanced chronic kidney disease., (© 2012 Wiley Periodicals, Inc.)

Upper extremity deep-vein thrombosis (UEDVT) is common and can cause important complications, including pulmonary embolism and post-thrombotic syndrome. An increase in the use of central venous catheters, particularly peripherally inserted central catheters has been associated with an increasing rate of disease. Accurate diagnosis is essential to guide management, but there are limitations to the available evidence for available diagnostic tests. Anticoagulation is the mainstay of therapy, but interventional treatments may be considered in select situations. The risk of UEDVT may be reduced by more careful selection of patients who receive central venous catheters and by use of smaller catheters. Herein we review the diagnosis, management and prevention of UEDVT. Due to paucity of research, some principles are drawn from studies of lower extremity DVT. We present a practical approach to diagnosing the patient with suspected deep-vein thrombosis of the upper extremity.

We describe a novel arteriovenous graft configuration in the abdominal wall for hemodialysis in a 51-year-old woman with sickle cell disease. Upper extremity access sites were exhausted, and intrathoracic central veins occluded. Because of diminished quality of the left groin due to scar tissue from previous infected access, inadequate vasculature, and the presence of functional femoral catheter in the right groin with common iliac vein stenosis, we decided to create an arteriovenous graft from the left common iliac artery to the inferior vena cava. Adequate thrill and uneventful postoperative recovery was observed. At 4 months, the patient has been successfully using her graft., (Copyright © 2012 Society for Vascular Surgery. Published by Mosby, Inc. All rights reserved.)

Background: To determine the impact of the postthrombotic syndrome (PTS) on quality of life after primary upper extremity deep venous thrombosis (UEDVT)., Patients and Methods: Twenty-five patients with a history of primary UEDVT, treated with anticoagulation alone, and twenty healthy controls were retrospectively identified and prospectively assessed for health-related quality of life (SF-36 and VEINES-QOL-questionnaire) and upper extremity functional impairment (DASH-score). Presence of PTS was classified according to the modified Villalta-score. Comparisons between patients and controls and between patients with and without PTS were performed using Fisher`s exact test (categorical variables) and Mann-Whitney-U-test (continuous variables)., Results: According to the modified Villalta-score, 32 % of the patients suffered from mild to moderate PTS. None of the patients developed severe PTS. Compared to healthy control subjects, patients with a history of primary UEDVT reported on considerably worse health-related quality of life and significantly stronger upper extremity functional impairment. Within the cohort of patients with UEDVT, subjects with PTS had a significantly reduced quality of life and a more severe functional limitation., Conclusions: Quality of life and functional performance are impaired in patients with a history of conservatively treated primary UEDVT. Impairment is most pronounced in patients with mild to moderate PTS occurring in every third patient.

Upper extremity deep vein thrombosis (UEDVT) is an increasingly important clinical entity with potential for considerable morbidity, especially pulmonary embolism (PE). Here, the authors report a fatal case of the massive PE after spinal surgery, along with the UEDVT of superior vena cava (SVC).

Objectives: Dialysis-dependent patients often have central venous drainage complications. In patients with functioning arm arteriovenous fistula, this may result in venous hypertension, arm oedema and vascular access failure. Percutaneous angioplasty and stent implantation might be inadequate to resolve these issues. In these cases, new access can potentially be created with anastomosis to the subclavian vein, iliac vein or vena cava or by making a veno-venous graft to bypass the thrombosis. The aim of this study was to assess the utility of unusual bypasses in vascular access in patients with the central vein thrombosis., Materials: A total of 49 patients were treated. The mean number of previous vascular access surgery procedures was 7.6 (3-17). We performed 19 axillo-iliac, 14 axillo-axillary bypasses and 16 conduits from the arm fistula to the jugular (nine conduits) or subclavian (seven conduits) vein for haemodialysis purposes., Results: All fistulas except one were used for haemodialysis. One patient died before the first use of the fistula. At 12 months, the primary, primary assisted and secondary patency rates were 85.4%, 89.6% and 95.8%, respectively. The follow-up period ranged from 1 to 84 months., Conclusion: Unusual grafts are an efficient option as a permanent vascular access for haemodialysis purposes in patients with central vein occlusion., (Copyright © 2011 European Society for Vascular Surgery. Published by Elsevier Ltd. All rights reserved.)

Upper-extremity deep venous thrombosis is less common than lower-extremity deep venous thrombosis. However, upper-extremity deep venous thrombosis is associated with similar adverse consequences and is becoming more common in patients with complex medical conditions requiring central venous catheters or wires. Although guidelines suggest that this disorder be managed using approaches similar to those for lower-extremity deep venous thrombosis, studies are refining the prognosis and management of upper-extremity deep venous thrombosis. Physicians should be familiar with the diagnostic and treatment considerations for this disease. This review will differentiate between primary and secondary upper-extremity deep venous thromboses; assess the risk factors and clinical sequelae associated with upper-extremity deep venous thrombosis, comparing these with lower-extremity deep venous thrombosis; and describe an approach to treatment and prevention of secondary upper-extremity deep venous thrombosis based on clinical evidence., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Thrombosis is a well known complication of subclavian vein catheterization. As collateral circulation develops, consequences are usually limited to the fact that this vein is no longer usable as an access route. However, one of the possible complications of a superior vena cava thrombosis is the development of a chylothorax. We describe an infant developing a chylothorax caused by a SVC thrombosis after subclavian vein catheterization for parenteral nutrition. The chylothorax did not resolve following conservative management, but was successfully treated by surgical ligation of the thoracic duct.

Background: Although the incidence of upper extremity deep venous thrombosis (UEDVT) diagnoses has increased, anticoagulation therapy for UEDVT remains inconsistent and of variable duration. This study sought to analyze our institution's current treatment practices for UEDVT and assess the risk for subsequent pulmonary embolism (PE)., Methods: Between April 2005 and July 2007, 200 consecutively encountered patients with UEDVTs were identified in the Peripheral Vascular Laboratory. Then, UEDVT location and sonographic characteristics, patient demographics, anticoagulation treatment, and PE incidence and mortality were examined., Results: Among the 200 patients with UEDVT, 156 (78%) had UEDVTs identified as clearly acute or acute on chronic, based on sonographic appearance. In all, 85% of the patients were symptomatic (n = 171). Among the patients, 71 (36%) had documented malignancy, 58 (29%) were postoperative or suffering from trauma, and 52 (26%) were obese (body mass index: >30). In addition, 153 (76%) had associated current or previous indwelling lines or leads. A total of 73 patients (36%) were put on anticoagulation therapy for variable periods. Younger age of the patient, duplex evidence of an acute deep venous thrombosis, and involvement of multiple named upper extremity venous segments were independent predictors of the decision to initiate anticoagulation therapy for patients with UEDVT. Two patients (1%) suffered PE, most likely the consequence of their UEDVTs. An additional two patients with UEDVT treated with coumadin died months after hospital discharge from intracranial bleedings after minor falls., Conclusion: Currently, the clinical decision to initiate anticoagulation therapy for patients with UEDVT is most associated with the documented acute nature of the deep venous thrombosis by duplex ultrasonography, involvement of multiple venous segments, and younger age of the patient. The incidence of PE attributable to previously documented UEDVT is very small (1%), regardless of anticoagulant therapy. Anticoagulation therapy for UEDVT is most likely to be best suited to address the symptoms of UEDVT; its proposed use to decrease the very small risk of PE may be rarely indicated, and must be carefully weighed with the risks associated with therapeutic anticoagulation in this patient population., (Copyright © 2011. Published by Elsevier Inc.)

The number of patients with end-stage renal failure requiring dialysis keeps increasing every year. Many of these patients also suffer from peripheral arterial disease. We report the case of a middle age woman receiving dialysis who had undergone amputation of both lower limbs and suffered multiple deep venous thrombosis. Therefore, peripheral accesses for venous dialysis were not available. A catheter had to be implanted right into the right atrium.

Central venous catheter (CVC)-related thrombosis is a frequently occurring complication and may cause significant morbidity in patients with cancer. The aim of this review is to discuss the main studies that examined whether a state of thrombophilia increases the risk of CVC-related thrombosis in patients with cancer. The studies were retrieved by an extensive Medline search. Patients with cancer with a CVC and a factor V Leiden mutation have a higher risk of developing CVC-related thrombosis than patients with cancer having a CVC without the mutation. The scarce information available suggests hyperhomocysteinemia to be a risk factor for CVC-related thrombosis. For other congenital thrombophilia factors, the available data are too limited to allow for any definitive conclusions to be made. Because the clinical implications of all these findings remain to be clarified, routine screening of patients with cancer having a CVC for thrombophilia cannot yet be recommended on the basis of the studies discussed.

Thrombosis of the superior vena cava leads to obstruction of venous outflow of the head and upper extremities and causes severe clinical symptoms. The management of SVC syndrome depends on aetiology and acuity at clinical presentation and ranges from conservative medical treatment to bypass surgery. Endovascular treatment can provide rapid relief of symptoms and substantial clinical improvement independent of aetiology. We report a case of successful interventional treatment in a patient with catheter-induced SVC thrombosis and present a review of the literature., (Crown Copyright © 2010. Published by Elsevier B.V. All rights reserved.)