Management of Paget-Schroetter Syndrome: a Systematic Review and Meta-Analysis.

OBJECTIVE: Currently, there is no consensus on the optimal management of Paget-Schroetter syndrome (PSS). The objective was to summarise the current evidence for management of PSS with explicit attention to the clinical outcomes of different management strategies. DATA SOURCES: The Cochrane, PubMed, and Embase databases were searched for reports published between January 1990 and December 2021. REVIEW METHODS: A systematic review and meta-analysis was conducted following PRISMA 2020 guidelines. The primary endpoint was the proportion of symptom free patients at last follow up. Secondary outcomes were success of initial treatment, recurrence of thrombosis or persistent occlusion, and patency at last follow up. Meta-analyses of the primary endpoint were performed for non-comparative and comparative reports. The quality of evidence was assessed using the GRADE approach. RESULTS: Sixty reports were included (2 653 patients), with overall moderate quality. The proportions of symptom free patients in non-comparative analysis were: anticoagulation (AC), 0.54; catheter directed thrombolysis (CDT) + AC, 0.71; AC + first rib resection (FRR), 0.80; and CDT + FRR, 0.96. Pooled analysis of comparative reports confirmed the superiority of CDT + FRR compared with AC (OR 13.89, 95% CI 1.08 - 179.04; p = .040, I2 87%, very low certainty of evidence), AC + FRR (OR 2.29, 95% CI 1.21 - 4.35; p = .010, I2 0%, very low certainty of evidence), and CDT + AC (OR 8.44, 95% CI 1.12 - 59.53; p = .030, I2 63%, very low certainty of evidence). Secondary endpoints were in favour of CDT + FRR. CONCLUSION: Non-operative management of PSS with AC alone results in persistent symptoms in 46% of patients, while 96% of patients managed with CDT + FFR were symptom free at end of follow up. Superiority of CDT + FRR compared with AC, CDT + AC, and AC + FRR was confirmed by meta-analysis. The overall quality of included reports was moderate, and the level of certainty was very low.

Acute Myocardial Infarction, Subclavian Vein Thrombosis, and Pulmonary Embolism Secondary to COVID-19-A Case Report.

Introduction: Coronavirus disease 2019 (COVID-19) is an infectious disease caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). The majority of infected patients develop the clinical picture of a respiratory disease, although some may develop various complications, such as arterial or venous thrombosis. The clinical case presented herein is a rare example of sequential development and combination of acute myocardial infarction, subclavian vein thrombosis (Paget Schroetter syndrome), and pulmonary embolism in the same patient after COVID-19. Case presentation: A 57-year-old man with a 10-day history of a SARS-CoV-2 infection was hospitalized with a clinical, electrocardiographic, and laboratory constellation of an acute inferior-lateral myocardial infarction. He was treated invasively and had one stent implanted. Three days after implantation, the patient developed shortness of breath and palpitation on the background of a swollen and painful right hand. The signs of acute right-sided heart strain observed on the electrocardiogram and the elevated D-dimer levels strongly suggested pulmonary embolism. A Doppler ultrasound and invasive evaluation demonstrated thrombosis of the right subclavian vein. The patient was administered pharmacomechanical and systemic thrombolysis and heparin infusion. Revascularization was achieved 24 h later via successful balloon dilatation of the occluded vessel. Conclusion: Thrombotic complications of COVID-19 can develop in a significant proportion of patients. Concomitant manifestation of these complications in the same patient is extremely rare, presenting at the same time, quite a therapeutic challenge to clinicians due to the need for invasive techniques and simultaneous administration of dual antiaggregant therapy combined with an anticoagulant treatment. Such a combined treatment increases the hemorrhagic risk and requires a serious accumulation of data for the purpose of a long-term antithrombotic prophylaxis in patients with such pathology.

Idiopathic upper extremity deep vein thrombosis: A systematic review.

BACKGROUND: Idiopathic upper extremity deep vein thrombosis (UEDVT) management is controversial and ranges from anticoagulation alone to the addition of further interventions such as thrombolysis and decompressive surgery. OBJECTIVES: The objective of this systematic review was to assess the effects of anticoagulation alone compared to anticoagulation with additional interventions such as thrombolysis or decompressive surgery on the incidence of recurrent UEDVT and post-thrombotic syndrome (PTS) in patients with idiopathic UEDVT (including those associated with the oral contraceptive pill). PATIENTS/METHODS: A systematic search was conducted for studies which focused on acute UEDVT treatment defined as therapies starting within 4 weeks of symptom onset. We limited studies to those that recruited 10 or more subjects and involved at least 6 weeks to 12 months anticoagulation alone or together with additional interventions with at least 6-month follow-up. Primary outcomes were symptomatic recurrent radiologically confirmed UEDVT and PTS. Secondary outcomes were symptomatic venous thromboembolism, bleeding and mortality. RESULTS: We found seven studies which reported recurrent UEDVT rates and five that reported PTS rates. All studies were retrospective or cross-sectional. None compared anticoagulation alone to anticoagulation with additional intervention. Study heterogeneity precluded meta-analysis and risk of bias was moderate to serious. Recurrent UEDVT occurred in 0% to 12% post-anticoagulation alone and 0% to 23% post-additional interventions. PTS rates varied from 4% to 32% without severe PTS. Only limited studies reported on our secondary outcomes. CONCLUSION: There is limited evidence behind idiopathic UEDVT management. Prospective comparative studies in this area are essential.

Upper Extremity Deep Vein Thrombosis: Current Knowledge and Future Directions.

Upper extremity deep vein thrombosis (UEDVT) has been increasing in incidence due to the escalating use of central venous catheters such as peripherally inserted central catheters. UEDVT can be primary idiopathic or secondary to pacemaker leads, intravascular catheters or cancer. In comparison to conventional venous thromboembolism such as lower limb deep vein thrombosis or pulmonary embolism the risk factors, investigations, and management are not well defined. We review current evidence in primary and secondary UEDVT, highlighting areas in need of further research. We also explore the entity of venous thoracic outlet syndrome, which is said to be a risk factor for recurrent primary UEDVT and is the rationale behind surgical interventions.

Paget-Schroetter syndrome in athletes: a comprehensive and systematic review.

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.

Effort Thrombosis Presenting as Unilateral "Pumped" Arm in a Climber.

Upper extremity injuries are common among the growing population of climbers. Although conditions affecting musculoskeletal structures are the most common causes of symptoms, a comprehensive differential diagnosis is necessary to avoid the misdiagnosis of high-morbidity conditions in this patient population. We present a case of a climber with acute edema, erythema, and pain of the entire right upper extremity. After confirmation of an upper extremity deep vein thrombosis by ultrasound, and in the absence of secondary causes for his thrombotic process, he was diagnosed with effort thrombosis. The patient was treated acutely with anticoagulation, catheter thrombectomy, direct thrombolysis, and balloon angioplasty followed by surgical decompression of the subclavian vein. Owing to the importance of early diagnosis and initiation of treatment, it is critical to keep disorders affecting the upper thoracic vascular structures in consideration.

Treatment-Related Outcomes in Paget-Schroetter Syndrome-A Cross-Sectional Investigation.

OBJECTIVE: To investigate treatment-related outcomes, namely radiological clot resolution, post-thrombotic syndrome (PTS), and health related quality-of-life (HRQoL) scores, in children with Paget-Schroetter syndrome (PSS) undergoing multidisciplinary management, including anticoagulation and decompressive rib-resection surgery, with or without thrombolytic therapy. STUDY DESIGN: We identified all patients treated for PSS at our institution between the years 2010 and 2017. Baseline clinical and radiologic data were abstracted from medical records. Two validated survey instruments to quantify PTS and HRQoL were mailed to eligible patients. Standard statistical methods were used to summarize these measures. RESULTS: In total, 22 eligible patients were identified; 10 were treated with thrombolysis followed by anticoagulation and rib resection, and 12 were treated with anticoagulation and rib resection alone. Nineteen patients responded to the survey instruments. Median age at deep vein thrombosis diagnosis and survey completion were 16.3 and 20.4 years, respectively. Nineteen of 22 patients had thrombus resolution on radiologic follow-up. Fourteen of 19 survey respondents reported signs/symptoms of PTS of which the majority (12/14) reported mild PTS. Aggregate total, physical, and psychosocial HRQoL scores reported were 90.6, 96.7, and 93.3, respectively. Thrombolytic therapy was not associated with a significant improvement in radiologic, clinical or HRQoL outcomes. CONCLUSIONS: Most patients with PSS had complete thrombus resolution on imaging. Only 11% of survey respondents reported moderate PTS. The entire cohort reported excellent HRQoL scores. The role for thrombolytic therapy in the management of childhood PSS remains incompletely elucidated.

A Case of Paget-Schroetter Syndrome in a Young Male After Lifting Weights

Paget-Schroetter Syndrome (PSS) or effort thrombosis of the axillary-subclavian venous axis is a rare disease affecting healthy young adults which requires a high index of suspicion to diagnose. Management often requires not only anticoagulation but also thrombolysis with first rib resection to prevent recurrence and complications. We present a case of a 31-year-old male who presented to our emergency department with pain and swelling of his left upper limb. He was diagnosed with PSS and underwent; anticoagulation, catheter directed thrombolysis and planned for first rib resection.

Upper Extremity Deep Vein Thrombosis: Symptoms, Diagnosis, and Treatment.

BACKGROUND: Upper extremity deep vein thrombosis (UEDVT) is defined as thrombosis of the deep venous system (subclavian, axillary, brachial, ulnar, and radial veins), which drains the upper extremities. It can be caused by thoracic outlet anatomic obstruction, such as Paget-Schroetter syndrome, (primary) or by central intravenous catheters (secondary). UEDVT may be asymptomatic or present with acute severe pain and arm swelling. Clinical suspicion should be confirmed by diagnostic imaging procedures such as duplex ultrasound, computed tomography scan, or magnetic resonance imaging. UEDVT is managed by anticoagulant treatment. In addition to that, early thrombolysis aimed at preventing post-deep vein thrombosis syndrome and thoracic outlet decompression surgery should be given to patients with primary UEDVT. Anticoagulation without thrombolysis is the treatment of choice for patients with catheter-related thrombosis. Mandatory functioning catheters can remain in place with anticoagulant treatment. All other catheters should be immediately removed. The management of patients with UEDVT requires an experience multidisciplinary team comprised of internists, radiologists, hematologists, and vascular surgeons. Understanding the risk factors for the development of UEDVT, the diagnostic procedures, and the treatment modalities will improve the outcome of those patients.

[Síndrome de Paget-Schrötter: presentación de cuatro casos]. / Paget-Schrötter syndrome: description of four cases.

Deep vein thrombosis (DVT) of the upper limb is a rare entity, estimated to account for 10% of all cases of DVT. Classically, they are classified into primary (idiopathic, due to subclavian vein compression or exercise related) and secondary (cancer, thrombophilia, trauma, shoulder surgery, associated to venous catheters or due to hormonal causes). The Paget- Schrötter syndrome is a primary thrombosis of the subclavian vein in the subclavian-axillary junction, related either to repetitive movements or to exercise; leading to microtrauma in the endothelium with consequent activation of the coagulation cascade. Clinically, it presents abruptly with pain, edema and feeling of heaviness in the affected limb. The treatment varies from thrombolytics and anticoagulation to surgical intervention, depending on the time of evolution. We present four cases of exercise-related subclavian vein thrombosis.

Rivaroxaban withdrawal and rebound hypercoagulability leading to upper extremity deep vein thrombosis: a case report.

As the newer oral anticoagulants (NOACs) are increasingly used in older patients, clinical scenarios when they may need to be discontinued temporarily or indefinitely, may be encountered. Similarly with increasing use of permanent pacemakers and other intra-cardiac devices, there is an increased risk of upper limb venous thrombosis even few years after their insertion. We report a case of a patient with a permanent pacemaker, on rivaroxaban (NOAC) for atrial fibrillation, who developed an upper extremity deep vein thrombosis after its temporary withdrawal following a traumatic acute subdural haematoma. Physicians should be aware of the possibility of rebound hypercoagulability and venous thrombosis soon after the withdrawal of NOACs.

Spontaneous Subclavian Vein Thrombosis in a Healthy Adolescent Cheerleader: A Case of Paget-Schroetter Syndrome.

We present the case of a healthy 13-year-old female adolescent who developed acute progressive swelling and pain in her right upper extremity that was secondary to an acute deep venous thrombosis of her right subclavian vein. Dynamic imaging revealed subclavian vein compression at the junction of the first rib and proximal third of the clavicle consistent with Paget-Schroetter syndrome, also known as effort-related thrombosis. The compressive etiology of her thrombus was most likely related to her cheerleading activity, in which she served as the pyramid base. The patient received multimodal therapy including anticoagulation, mechanical and site-directed thrombolysis, and a first rib resection. This case illustrates that frontline providers should have a high index of suspicion for an upper extremity thrombosis in pediatric patients who present with unilateral arm swelling.

Paget-Schroetter Syndrome in a Baseball Pitcher.

Paget-Schroetter syndrome (PSS) is thrombosis of the deep veins draining the upper extremity due to anatomic abnormalities of the thoracic outlet that cause subclavian compression and subsequent thrombosis, leading to thrombus formation in the subclavian vein. Vigorous arm activity in sports is a known risk factor. Here, we report a case of Paget-Schroetter syndrome in a 31-year-old male non-professional baseball pitcher.

[Upper extremity deep venous effort thrombosis : management of the Paget-Schroetter syndrome in the emergency department]. / Le cas clinique du mois : Thrombose veineuse profonde d'effort du membre supérieur : prise en charge du syndrome de Paget-Schroetter au service des Urgences.

We report a case of upper extremity deep venous effort thrombosis complicating a thoracic outlet syndrome in a 44-year-old patient. The appearance of this complication was sudden. A clinical examination followed by imaging with phlebography leads to a quick diagnosis. Effort thrombosis is a classic example of an entity which, if treated correctly, has minimal long-term sequelae but, if ignored, is associated with significant long-term morbidity. An appropriate medical and interventional care has been implemented as soon as possible. Once primary thrombosis is recognized, catheter-directed thrombolytic therapy is usually successful if initiated early, but often unmasks an underlying problem.

Nous rapportons le cas d'une phlébite d'effort du membre supérieur compliquant un syndrome de la traversée thoraco-brachiale chez un patient de 44 ans. L'apparition de cette complication a été brutale. Un bilan clinique et iconographique par phlébographie a permis de poser le diagnostic rapidement. C'est une affection rare qui suscite une morbidité importante si elle n'est pas traitée de façon adéquate. Une prise en charge médicale et interventionnelle appropriée a été mise en oeuvre dans les plus brefs délais. Nous envisageons les moyens de prise en charge rapide et l'intérêt essentiel de la fibrinolyse in situ en urgence.

Paget-Schröetter Syndrome, a rare cause of subclavian vein thrombosis.

Paget-Schröetter Syndrome, or effort thrombosis of the subclavian vein, is an uncommon condition that affects young, active, healthy individuals. It's a type of Thoracic Outlet Syndrome (TOS), consequence of compression and repetitive injury of the subclavian vein, between the first rib and the clavicle. This paper reports the case of a 27 year-old female presenting with an acutely swollen right upper limb, with no history of trauma or repeated movement of right arm, taking oral contraceptive pills and no history of venous thromboembolic disease. Duplex ultrasound evaluation confirmed right subclavian venous thrombosis with no evidence of cervical rib or other structural abnormalities, diagnosing Paget-Schröetter Syndrome. Following anticoagulation, catheter directed thrombolysis and balloon angioplasty were performed, with no success due to external compression. Therefore, after the correction of the external compression through first rib resection, the luminal flow was restored and the venous obstruction symptoms disappeared. In these case, a prompt treatment was critical for the achievement of the best results.

A Síndrome de Paget-Schröetter, ou trombose de esforço da veia subclávia, é uma entidade rara que surge em indivíduos jovens e saudáveis. É enquadrado na Síndrome do Desfiladeiro Torácico: sinais e sintomas resultantes da compressão e lesão repetida da veia subclávia, entre a primeira costela e a clavícula. Relata-se o caso clínico de uma jovem de 27 anos de idade com edema agudo do membro superior direito, sem história de trauma ou movimentos repetidos do braço direito, sem antecedentes de patologia tromboembólica venosa e medicada com contraceptivo oral. O ecodoppler revelou trombose da veia subclávia direita, sem evidência de costela cervical ou outras anomalias estruturais. Após o início de hipocoagulação, foi realizada venografia, com fibrinólise fármaco-mecânica e angioplastia com balão sem sucesso, devido a compressão extrínseca. Posteriormente, foi submetida a remoção trans-axilar da primeira costela, o que permitiu restabelecimento do fluxo venoso e desaparecimento dos sintomas de compressão venosa. Neste caso, um tratamento atempado foi crucial para o sucesso terapêutico.

Developmental Hemostasis and Management of Central Venous Catheter Thrombosis in Neonates.

Neonatal hemostasis differs qualitatively, but in particular quantitatively, from hemostasis in older children and adults. Nevertheless, hemostasis in healthy neonates is functionally stable with no tendency to bleeding or thrombotic complications. In sick neonates, however, risk factors may disrupt this equilibrium and lead to thrombosis. The most important risk factor is the central venous catheter. Management of neonatal central venous catheter thrombosis is challenging, as no controlled trials have been performed. Therapeutic options include (1) observation and supportive treatment; (2) anticoagulant agents, including low-molecular-weight heparin and unfractionated heparin; (3) thrombolytic agents; and (4) thrombectomy. Prevention of thrombosis with anticoagulation is not advised yet. Careful consideration of the necessity of the catheter and optimal hygienic care are important preventative measures.

Postthrombotic syndrome following upper extremity deep vein thrombosis in children.

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.

Incidence of catheter-related thrombosis in acute leukemia patients: a comparative, retrospective study of the safety of peripherally inserted vs. centrally inserted central venous catheters.

Central venous catheters are a leading cause of upper-extremity deep vein thrombosis. Concomitant severe thrombocytopenia makes anticoagulation for catheter-related thrombosis (CRT) in patients with acute leukemia (AL) a challenge. Incidence of CRT has been reported to be increased in those with peripherally inserted central catheters (PICC) vs. those with centrally inserted ones (CICC). Our objective is to compare the incidence rate of CRT in leukemia inpatients who received either a PICC vs. CICC. We retrospectively reviewed adult inpatients admitted to hematology wards with a new diagnosis of AL and who received either a PICC or a CICC. Baseline patient and catheter characteristics were recorded. Our primary outcome was the incidence rate of CRT in each group. The secondary outcomes included rates of infectious and mechanical complications. Six hundred sixty-three patients received at least one PICC (338) or CICC (325) insertion. A total of 1331 insertions were recorded, with 82 (11.7 %) and 41 (6.5 %) CRT in the PICC and CICC groups, respectively. The incidence rates were 1.89 and 0.52 per 1000 catheter day in the PICC and CICC groups, respectively. A PICC, when compared to CICC, was a significant risk factor for CRT (sHR 2.5, p < 0.0001). The prevalence and incidence rates of CRT in our AL patients were higher than predicted for a general cancer patient population. These rates were higher in the PICC group compared to the CICC group. We recommend careful consideration of thrombotic and bleeding risks of AL inpatients when choosing a central venous catheter.

Paget-Schroetter syndrome: diagnostic limitations of imaging upper extremity deep vein thrombosis.

Paget-Schroetter syndrome is a rare but potentially debilitating condition affecting young, otherwise healthy individuals. This condition, also known as effort thrombosis, is an upper extremity deep vein thrombosis classically caused by anatomical abnormalities compressing the neurovascular structures of the thoracic outlet. The diagnosis is important to emergency medicine providers due to its secondary morbidity and mortality. Common complications affecting these active adults are pulmonary embolism and postthrombotic syndrome. Most patients report a precedent history of vigorous exercise or activity involving the upper extremities. We present a case of a 23-year-old man with redness and swelling of his dominant arm after weightlifting. Previous literature describes Paget-Schroetter syndrome from repetitive activities. The report highlights the limitations of imaging studies in proximal upper extremity deep vein thromboses. The initial selected imaging study, Doppler ultrasound, was negative in our case and was followed by a nondiagnostic computed tomographic venogram. Although ultrasound is the preferred diagnostic imaging modality, it is limited when thrombosis is present in the noncompressible region of the clavicle. Magnetic resonance venogram or computed tomographic venogram is recommended if index of suspicion is high and the ultrasound shows normal results, but these studies are highly dependent on technique, flow, and timing. The eventual diagnosis of axillosubclavian thrombosis was obtained only after specialty consultation and formal venography. This case discusses the limitations of each imaging modality and the importance of a comprehensive clinical approach to this rare diagnosis.

Spontaneous Upper Extremity Venous Thrombosis in a Collegiate Soccer Player: A Case Report.

Spontaneous effort-induced thrombosis is a rare but reported phenomena that was originally described over 100 years ago. The pathogenesis of this thrombosis arises from an abnormality of the thoracic outlet usually combined with a history of physical activity that includes repetitive arm motions, usually of the dominant hand. We present the case of an adolescent patient who presented to a pediatric emergency department with progressive pain, discoloration, and swelling of the shoulder of his nondominant hand. The pain became acutely worse with graying appearance of his arm. The patient was diagnosed with spontaneous thrombosis of the upper extremity extending from the left subclavian vein extending to the axillary vein. Treatment of this patient included aggressive anticoagulation, thrombolysis, and costectomy.