Symptoms of SVCS depend on the severity and rapidness of vessel pathology. Most common symptoms include a sense of fullness in the head, neck and facial swelling, dyspnea, chest pain, cough, dysphagia and distended upper extremities [10]. Tachycardia, hoarseness, stridor, respiratory distress and cerebral edema with ischemia and herniation may be encountered in severe cases. Physical examination can reveal distended neck veins, edema of the upper extremities, while various neurological deficits may be encountered.

• Hodgkin Lymphoma

  Causes were as follows: small cell lung cancer (SCLC) 28.2 %, non-small cell lung cancer 25 %, non-Hodgkin's lymphoma 25 %, metastasis of other malignant tumors 19.4 % and Hodgkin's disease 2.4 %. [ncbi.nlm.nih.gov]

  Lung cancer, usually small cell carcinoma, comprises 75-80% of these cases and non-Hodgkin lymphoma, most commonly diffuse large B-cell lymphoma, comprises 10-15%. [en.wikipedia.org]

  Five patients had non-Hodgkin's lymphoma, two had Hodgkin's disease, two had benign lesions, and one patient was suspected on clinical and radiologic basis to have a lymphoma. [pediatrics.aappublications.org]

• Fatigue

  A 58-year-old man presents with a 2-week history of progressive dyspnea on exertion, neck swelling, decreased appetite, and fatigue. There is no history of syncope or dysphagia. He smoked cigarettes until 5 years ago. [doi.org]

  Abstract Background A 29-year-old woman presented with chest pressure, progressive dyspnea, fatigue, and swelling of her face, neck and upper extremities. [nature.com]

  Chronic cough may cause severe pain, trouble sleeping, dyspnea, or fatigue. The causes of chronic coughing are almost the same as the causes of dyspnea. It may be possible to treat the cause of chronic coughing. [cancer.gov]

  […] relief at 5 days after the first fraction, and after the second fraction 74 % of patients reported that their symptoms had entirely disappeared. 22 % of patients reported treatment-associated nausea, 26 % reported WHO grade I–II dysphagia, 17 % reported fatigue [springerplus.springeropen.com]

• Falling

  In the case of life-threatening SVC obstruction chemotherapy can begin immediately (after the collection of material for histology) from vital indication (so called not fall submission). [telemedicina.med.muni.cz]

  Pape Diadie Fall 2, Mouhamadou Chérif Mboup 2, Khadidiatou Dia 2, Malick Bodian 1 and Modou Jobe 1 Email author Thrombosis Journal 2015 13 :7 https://doi.org/10.1186/s12959-015-0039-z © Sarr et al.; licensee BioMed Central. 2015 Received: 9 June 2014 [thrombosisjournal.biomedcentral.com]

• Malaise

  Table 2 Kishi Scoring system Neurological signs Awareness disorders or coma 4 Visual disorders, headache, vertigo or memory disorders 3 Mental disorders 2 Malaise 1 Thoracic or pharyngeal - laryngeal signs Orthopnea or laryngeal edema 3 Stridor, dysphagia [springerplus.springeropen.com]

• Cough

  We report a case of a middle-aged man who presented with progressive oedema of the upper half of the body, dyspnoea, cough and weight loss for a 1-year duration. He was a non-smoker without prior hospitalisation. [ncbi.nlm.nih.gov]

  The most frequent signs and symptoms were face or neck swelling (82%), upper extremity swelling (68%), dyspnea (66%), cough (50%), and dilated chest vein collaterals (38%). [doi.org]

  They include head, neck and arm oedema, often with cyanosis, headache, confusion and coma, distended subcutaneous veins, cough and hoarseness, and difficulty in breathing and swallowing. [medical-dictionary.thefreedictionary.com]

• Dyspnea

  The most frequent signs and symptoms were face or neck swelling (82%), upper extremity swelling (68%), dyspnea (66%), cough (50%), and dilated chest vein collaterals (38%). [ncbi.nlm.nih.gov]

  Abstract A 31-year-old woman was admitted to our hospital with a sudden onset of chest pain and dyspnea. Echocardiography, chest CT, and chest MRI revealed a huge mass in the right atrium. [jstage.jst.go.jp]

• Hoarseness

  Classic symptoms include edema, plethora, and distended veins of the face, neck, and chest; shortness of breath; cough; headache; and hoarseness. Treatment in the emergency department is mostly supportive, with head elevation, oxygen, and steroids. [ncbi.nlm.nih.gov]

  Severe cases present with hoarseness, laryngeal edema and neurological deficits. [symptoma.com]

  Common childhood SVCS symptoms are similar to what adults experience and may include: Coughing Hoarseness in voice Trouble breathing Chest pain Related Resources Heart Problems More Information National Cancer Institute: Cardiopulmonary Syndromes [cancer.net]

  Social considerations Patients and family members are often frightened and anxious because of the symptoms of SVCS, mainly swelling, trouble swallowing, coughing, and hoarseness. [healthinfo.uclahealth.org]

• Stridor

  Stridor is not present. How should his case be evaluated and managed? Funding and Disclosures No potential conflict of interest relevant to this article was reported. We thank Marilyn L. [doi.org]

  10 Mild or moderate cerebral edema (headache, dizziness) or mild/moderate laryngeal edema or diminished cardiac reserve (syncope after bending) 4 Life-threatening 5 Significant cerebral edema (confusion, obtundation) or significant laryngeal edema (stridor [en.wikibooks.org]

  ▪ Edema- Narrow the lumen of the nasal passages and larynx, potentially compromising the function of the larynx or pharynx- Dyspnea, stridor, cough, hoarseness, and dysphagia. ▪ Cerebral edema can also occur and lead to cerebral ischemia, herniation [slideshare.net]

  The only truly emergent situation in adults is the patient presenting with stridor or other signs of severe airway compromise or the patient with cerebral edema or hemodynamic compromise. [clinicaladvisor.com]

• Pleural Effusion

  European Respiratory Journal 1997 10: 1675-1677; DOI: Abstract Pleural effusion is often a manifestation of the superior vena cava (SVC) syndrome. However, pleural effusion has never been reported to be a cause of the SVC syndrome. [erj.ersjournals.com]

  Chest radiography showed right-sided pleural effusion with an apparent normal superior mediastinum. Contrast-enhanced CT of the chest revealed a right atrial mass extending and completely obliterating to superior vena cava. [ncbi.nlm.nih.gov]

  Chest CT scans showed that 36% of patients had upper airway compression and 44% of patients had pericardial effusions. Sixty-seven percent of patients had pleural effusion, 32% had atelectasis, and 10% had pulmonary embolisms. [oncologynurseadvisor.com]

• Dysphagia

  There is no history of syncope or dysphagia. He smoked cigarettes until 5 years ago. [doi.org]

  Recurrence of SVC syndrome in 13% Toxicity: Minimal, dysphagia most common 26% London (UK)(no dates given) -- SCLC: chemo +/- RT Randomized. 37/366 patients with SCLC enrolled in trial of chemotherapy +/- RT presented with SVC obstruction. 9 relapsed [en.wikibooks.org]

  Symptoms include facial edema, dyspnea, cough, neck distension, hoarseness and dysphagia, while severe cases may present with coma and severe respiratory distress. [symptoma.com]

  An 84-year-old man presented to endocrinology unit with gradually increasing neck mass, dysphagia, and shortness of breath. [endocrine-abstracts.org]

• Chest Pain

  A six months' follow-up revealed abolishment of chest pain and superior vena cava (SVC) syndrome and good prosthetic composite graft function with no recurrence of pseudo aneurysm or dissection. [ncbi.nlm.nih.gov]

  Abstract A 31-year-old woman was admitted to our hospital with a sudden onset of chest pain and dyspnea. Echocardiography, chest CT, and chest MRI revealed a huge mass in the right atrium. [jstage.jst.go.jp]

  Dyspnea at rest, cough, and chest pain were more frequent in the patients with malignancy. Procedures performed for diagnostic or treatment purposes did not increase morbidity or mortality. [doi.org]

• Cyanosis

  It is also associated with cyanosis, plethora, and distended subcutaneous vessels. Lung cancer, including both non-small cell lung cancer and small cell lung cancer, is the most common extrinsic cause of superior vena cava syndrome. [ncbi.nlm.nih.gov]

  The increased venous pressure results in oedema of the head, neck, and arms, often with cyanosis, plethora, and distended subcutaneous vessels. [1] Wilson LD, Detterbeck FC, Yahalom J. [bestpractice.bmj.com]

  They include head, neck and arm oedema, often with cyanosis, headache, confusion and coma, distended subcutaneous veins, cough and hoarseness, and difficulty in breathing and swallowing. [medical-dictionary.thefreedictionary.com]

  Cyanosis and respiratory distress mandates adequate supplementation of oxygen and assisted ventilation in severe cases, while rest, fluid administration, use of diuretics and glucocorticoids is shown to be of great benefit for patients.. [symptoma.com]

• Vascular Disease

  […] heart disease Heart muscle disease ( cardiomyopathy ) Pericardial disease Aorta disease and Marfan syndrome Vascular disease ( blood vessel disease) It’s the leading cause of death in the U.S. [webmd.com]

  Treatment of noncoronary obstructive vascular disease. In: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 11th ed. Philadelphia, PA: Elsevier; 2019:chap 66. [nlm.nih.gov]

  These procedures are generally performed by a trained radiologist or vascular surgeon. [medical-dictionary.thefreedictionary.com]

• Flushing

  With acute superior vena cava obstruction, symptoms include facial and neck swelling, facial flushing, bilateral upper extremity swelling, neurological signs, dyspnea, headache, and cough. [radiopaedia.org]

• Arm Swelling

  Superior vena cava (SVC) syndrome, characterized by facial and arm swelling, is most frequently caused by intrathoracic malignancies. Decompression may be achieved with endovenous stent placement. [ncbi.nlm.nih.gov]

  Subsequently, she developed significant facial and bilateral arm swelling thought to be angioedema and fevers up to 103°F despite restarting antibiotics ( Figure 1 ). [criticalcare.imedpub.com]

  The most common physical findings are facial, neck, and arm swelling and dilated veins in the chest. CT with IV contrast is used to confirm the diagnosis of SVCS; MRI is helpful in cases in which IV contrast is contraindicated. [journals.lww.com]

  Other symptoms are cough, facial swelling, arm swelling, chest pain, dysphagia and hoarseness, cyanosis, cerebral edema, and mental status changes. [clinicaladvisor.com]

  Symptoms may include a combination of the following: coughing difficulty breathing or swallowing hoarseness chest pain coughing up blood swollen veins in the neck or chest arm swelling facial swelling stridor or wheezing nausea lightheadedness red skin [medicalnewstoday.com]

• Edema of the Upper Extremity

  Physical examination can reveal distended neck veins, edema of the upper extremities, while various neurological deficits may be encountered. [symptoma.com]

  The resulting increased venous pressure in the upper body may cause edema of the head, neck, and upper extremities, often associated with cyanosis, plethora, and distended subcutaneous vessels. [rc.rcjournal.com]

• Facial Swelling

  Here, we report a case of facial swelling and severe bilateral pleural effusion secondary to superior vena cava occlusion in a 41-year-old woman. An endovascular venous intervention was attempted initially but failed. [ncbi.nlm.nih.gov]

• Neck Swelling

  The most frequent signs and symptoms were face or neck swelling (82%), upper extremity swelling (68%), dyspnea (66%), cough (50%), and dilated chest vein collaterals (38%). [ncbi.nlm.nih.gov]

• Facial Edema

  RESULTS: The main clinical manifestations were dyspnea (84.7%), facial edema (81.9%), arm edema (22.2%), neck vein distension (25%), hoarseness (12.5%) and facial plethora (5.6%). [e-roj.org]

  Facial edema, dyspnea, cough, dysphagia together with neck and upper extremity distension are most frequently reported. Severe cases present with hoarseness, laryngeal edema and neurological deficits. [symptoma.com]

  Patient with a 4-week history of increasing facial edema and known lung cancer. Chest radiograph of a patient with known superior vena cava syndrome (SVCS) and bronchogenic carcinoma (CA). [emedicine.com]

• Neck Edema

  In summary, facial edema, dilated head, neck, and chest veins, dyspnea, cough, and in some cases, orthopnea are the most common symptoms of SVCS. [rc.rcjournal.com]

  Grading Grade Category Incidence Definition 0 Asymptomatic 10 Radiographic superior vena cava obstruction in the absence of symptoms 1 Mild 25 Edema in head or neck (vascular distention), cyanosis, plethora 2 Moderate 50 Edema in head or neck with functional [slideshare.net]

• Lip Edema

  Unfractionated heparin infusion was started with improvement in her facial and lip edema. Figure 1: Active dermatomyositis. Figure 2: CT chest showing superior vena cava (SVC). [criticalcare.imedpub.com]

  […] or dyspnea 2 Coughing or pleurisy 1 Facial signs Lip edema, nasal obstruction or epistaxis 2 Facial edema 1 Vessel dilation Neck, face or arms 1 Imaging Chest imaging is an important diagnostic tool that can frequently be used to find the abnormality [springerplus.springeropen.com]

• Headache

  A 35-year-old female patient visited our hospital with chief complaints of a 2-week-old headache, chemosis, pulsatile and exophthalmos and the deteriorating ptosis in the right eye. [ncbi.nlm.nih.gov]

  Background: SVCS causes increased venous pressure which can cause intracerebral manifestations including headaches, papilledema and rarely, cerebral edema. [neurology.org]

  Shortness of breath and coughing are quite common symptoms; difficulty swallowing is reported in 11% of cases, headache in 6% and stridor (a high-pitched wheeze) in 4%. [en.wikipedia.org]

• Altered Mental Status

  Stridor, edema, shortness of breath, difficulty swallowing, visual disturbances, headache, and altered mental status were not present. Labs revealed a normal CBC and BMP. [karger.com]

Because SVCS can mimic various conditions, including pericarditis, heart failure, myocardial infarction (if symptoms appear abruptly) and ischemic stroke, the diagnostic workup should start with a full physical examination and proper patient history. Information about comorbidities, history of previous surgical procedures and underlying diseases such as malignant tumors (which may not be diagnosed yet, in which case a high clinical suspicion is necessary) should be obtained in full, whereas physical examination may provide key details in making the diagnosis. These tools, however, may only make an initial diagnosis that needs to be confirmed with imaging studies. Plain radiography may reveal the presence of a tumor, but CT, venography, or MR angiography are recommended methods in confirming the diagnosis [4]. If a tumor is suspected, biopsy with subsequent histopathological evaluation is necessary to determine the subtype. Through these methods, the exact cause may be determined, after which appropriate treatment can be instated.

• Mediastinal Mass

  Anesthesia for patients with a mediastinal mass. Anesthesiol Clin North Am 2001;19:559-79. 2. Levin H, Bursztein S, Heifetz M. Cardiac arrest in a child with an anterior mediastinal mass. Anesth Analg 1985;64:129-30. 3. Keon TP. [joacp.org]

  Here, we report a fascinating case of a young, otherwise healthy, immunocompetent patient that presented to us with superior vena cava syndrome and a mediastinal mass. [ncbi.nlm.nih.gov]

  Anesthesiology 1981; 55: 471–2Keon, TP Goh MH, Liu XY, Goh YS: Anterior mediastinal mass: an anaesthetic challenge. [anesthesiology.pubs.asahq.org]

• Ventricular Hypertrophy

  Ventricular hypertrophy ( Left, Right ) Cerebrovascular diseases Stroke - Transient ischemic attack - Intracranial hemorrhage / cerebral hemorrhage : Extra-axial hemorrhage ( Epidural hemorrhage, Subdural hemorrhage, Subarachnoid hemorrhage ) Intra-axial [wikidoc.org]

• Pleural Effusion

  European Respiratory Journal 1997 10: 1675-1677; DOI: Abstract Pleural effusion is often a manifestation of the superior vena cava (SVC) syndrome. However, pleural effusion has never been reported to be a cause of the SVC syndrome. [erj.ersjournals.com]

  Chest radiography showed right-sided pleural effusion with an apparent normal superior mediastinum. Contrast-enhanced CT of the chest revealed a right atrial mass extending and completely obliterating to superior vena cava. [ncbi.nlm.nih.gov]

  Chest CT scans showed that 36% of patients had upper airway compression and 44% of patients had pericardial effusions. Sixty-seven percent of patients had pleural effusion, 32% had atelectasis, and 10% had pulmonary embolisms. [oncologynurseadvisor.com]

Management of patients initially necessitates supportive measures. Cyanosis and respiratory distress mandates adequate supplementation of oxygen and assisted ventilation in severe cases, while rest, fluid administration, use of diuretics and glucocorticoids is shown to be of great benefit for patients. [4]. The mainstay of therapy, however, is identification and treatment of the underlying cause. In the setting of thrombosis, anticoagulant and thrombolytic therapy consisting of either warfarin or heparin is effective [3], whereas removal of central venous catheters and pacemakers should be considered. Infectious pathogens such as histoplasmosis and tuberculosis require intensive antifungal and antibiotic therapy, respectively. As tumors are the most common cause, appropriate staging of the tumor and adequate therapy consisting of surgery, radiation and/or chemotherapy are vital in eliminating the mass that compresses the superior vena cava. Various techniques for isolated management of SVCS have been described across many studies, with endovascular approaches being recommended as first-line therapy [11]. Insertion of vascular endoprostheses and resection and reconstruction of the venous vessel using autologous tissue are some of the most successful [6] [12] [13].

The prognosis of patients with SVCS directly depends on the underlying cause. If malignancy triggers SVCS, overall prognosis is poor. Studies have established that SVCS is a predictor of poor outcome in patients suffering from non-small cell lung cancer [7]. On the other hand, central venous catheter-induced thrombosis can be resolved quickly, which is why identifying the cause as soon as possible may significantly improve the outcome [9].

SVCS was initially described more than 250 years ago [5], and numerous causes have been described in literature. Studies have shown two pathologic events: extraluminal mechanical compression of the vein that causes narrowing of the vessel lumen and thrombosis [3]. Malignant tumors, most frequently bronchogenic carcinoma, but also lymphoma, chondrosarcoma [6], melanoma and breast cancer are responsible for approximately 85% of all SVCS cases, while mediastinal metastases and enlarged lymph nodes may also compress the superior vena cava [7]. Non-malignant causes are much more uncommon and comprise about 15% of cases. Infectious pathogens such as tuberculosis and histoplasmosis, thymomas, aortic aneurysms, mediastinal fibrosis (that may be a consequence of histoplasmosis), as well as central venous and pacemaker catheters are causative agents in approximately 15% of patients [7]. Recent reports, however, suggest that benign causes may be responsible for up to 40% of cases, primarily because of increasing use of central venous catheters and pacemakers that can lead to thrombosis [2].

Because of the underlying etiologies, SVCS is established to be most common in males between 50-70 years of age with either primary or metastatic tumors of the mediastinum [3]. Certain studies have established that approximately 15,000 individuals develop SVCS in the United States every year [7]. Advanced malignant disease is the single most important risk factor for SVCS, but several other have been established. Right-sided tumors, due to anatomical proximity to the vessel, are more likely to cause SVCS [3]. Placement of central venous catheters [8], as well as implantation of cardiac pacemakers are significant risk factors for SVCS as these methods induce hypercoagulable states that lead to obstruction.

Under physiological conditions, venous blood from the head, upper thorax and the arms drain into the superior vena cava, which eventually terminates in the right atrium, making it one of the most important venous vessels in the body. It is tightly enclosed in the anterior superior mediastinum by several structures [3], including the sternum, the trachea, right mainstem bronchus, thoracic aorta, and both perihilar and paratracheal lymph nodes. In most cases, SVCS stems from either mechanical extraluminal compression by a malignant tumor or thrombosis [3]. In case of compression, narrowing of the vessel lumen occurs, while thrombosis reduces the diameter through which blood can pass, both eventually elevating venous pressures. As a compensatory mechanism, the body redistributes circulation to tributaries of the superior vena cava, such as the azygous vein, in the attempt to reduce vessel overload. As the underlying cause is not resolved, however, severe compression or occlusion eventually triggers the onset of symptoms.

Timely diagnosis of the underlying cause may significantly reduce the rate of SVCS appearance, but directed preventive strategies may be aimed at regular screening of patients who are at risk, such as those with malignant tumors that are known to induce this vascular phenomenon, but also those with central venous catheters and pacemakers.

Superior vena cava syndrome (SVCS) is a clinical entity in which mechanical obstruction of the superior vena cava leads to reduced blood flow. In up to 85% of all cases, SVCS stems from mechanical compression by malignant tumors, while other etiologies include mediastinal fibrosis, use of cardiac pacemakers and central venous catheters that predispose patients to thrombosis [1]. Bronchogenic carcinoma is by far the most common tumor, followed by small-cell carcinomas, non small-cell carcinomas, lymphomas and various other [2]. The superior vena cava is responsible for drainage of the upper portions of the thorax, the arms and the endocranium. In the setting of mechanical compression or thrombosis, there is an increased load on the accessory veins, including vertebral, internal mammary and the thoracic venous system, but the azygous vein is the single most important collateral branch [3]. Eventually, increased venous pressure develops and various symptoms may appear depending on the progression and severity of obstruction. Facial edema, dyspnea, cough, dysphagia together with neck and upper extremity distension are most frequently reported [4]. Severe cases present with hoarseness, laryngeal edema and neurological deficits [3]. Physical examination may reveal distended neck veins, tachycardia, swelling of the face and upper extremities, whereas patients suffering from severe forms may develop respiratory distress, altered mental state, seizures and coma [3]. Although the diagnosis can be made based on clinical criteria, imaging studies such as computed tomography (CT), magnetic resonance imaging (MRI), MR angiography, venography and even plain radiography may be used to confirm the diagnosis and the underlying cause. Since tumors are the most common cause of obstruction, biopsy with subsequent histopathological examination is required to determine the exact tumor subtype. Treatment is aimed at resolving the cause. Radiation, surgery and chemotherapy are used in the case of malignancy, while supportive measures include oxygen therapy and use of diuretics, thrombolytic agents and corticosteroids [3].

Superior vena cava syndrome (SVCS) is a condition in which one of the most important veins, the superior vena cava, is either mechanically compressed or affected by thrombosis, leading to impaired blood flow and appearance of various symptoms. This vein collects blood from the entire head and neck, as well as the upper extremities and the upper part of the chest that reaches the right atrium, from where it travels to the lungs, back to the heart and into the circulation through the aorta. The most common cause of SVCS is a malignant tumor, comprising about 85% of all cases, with lung cancer being the most common type of malignancy. The presence of a tumor causes compression of the vessel and narrows the lumen through which blood can pass. Thrombosis due to placement of central venous catheters (often instated in intensive care patients and many other) or pacemakers can also trigger SVCS, as thromb formation impedes normal blood flow. To compensate for reduced blood flow, the body attempts to redistribute blood via several tributaries of the superior vena cava, but eventually, symptoms such as head and neck swelling, breathing difficulties and chest pain appear. In severe cases, voice changes due to swelling of the larynx, tachycardia and neurological changes may be observed, which requires immediate treatment and identification of the underlying cause. To make a presumptive diagnosis, physical examination can reveal distended neck veins, cyanosis (blue discoloration of the skin due to impaired oxygen delivery of the heart) and swelling of the upper extremities, but imaging techniques such as X-ray, computed tomography (CT scan), venography and a specific form of magnetic resonance imaging (MRI), MR angiography, can be used. If a tumor is suspected, biopsy of the mass is necessary to determine the exact subtype. Treatment depends on the underlying cause, but supportive measures consisting of oxygen therapy, administration of anticoagulants, diuretics, corticosteroids and fluid replenishment are recommended. Surgery, radiation and chemotherapy are used in managing malignant tumors, while various surgical procedures for vessel repair have been described. The condition itself may not be of significant risk for patients, but the underlying diagnosis is cancer in the vast majority of cases, which is why poor outcomes are observed. This condition is most commonly seen in males between 50-70 years and about 15,000 Americans are diagnosed every year with SVCS. For these reasons, screening of patients that are already diagnosed with lung cancer, as well as those that have established risk factors may be an effective treatment strategy in reducing the burden of this condition.

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