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Superior Vena Cava Thrombosis

Superior vena cava thrombosis is a potentially life-threatening clinical entity that mainly arises from the use of central venous catheters that may promote thrombosis and obstruction of this major vessel. Swelling of the head and neck, flushing, cyanosis, respiratory distress, and neurological deficits comprise the clinical presentation. The initial diagnosis can be made on clinical grounds, but imaging studies such as multidetector computed tomography (MDCT), magnetic resonance imaging (MRI), and sometimes standard contrast venography, are used to confirm superior vena cava thrombosis.


Presentation

Superior vena cava thrombosis has a potential to result in superior vena cava syndrome (SVCS). SVCS is a well-known disorder that stems from the obstruction of the superior vena cava. By far, the most common cause of SVCS are malignancies - carcinomas of the lungs, lymphoma, mediastinal tumors, or metastatic deposits [1] [2] [3] [4] [5] [6]. Thrombotic events in the superior vena cava constitute the significant proportion of the remaining SVCS cases [1] [4]. The presence of indwelling intravascular devices is the main predisposing factor to superior vena cava thrombosis [1] [2]. In fact, virtually any condition that causes mechanical obstruction of blood flow through this vessel can have prothrombotic effects [2]. The clinical presentation of superior vena cava thrombosis focuses on the head and neck area, with main complaints being a sense of head fullness accompanied by flushing and swelling of the face and neck [3] [4] [7]. Respiratory manifestations such as dyspnea and stridor are not uncommon, particularly when thrombosis develops suddenly [4] [8]. Other findings include prominent dilation of veins on the neck, the trunk, and the upper extremities, cyanosis, and neurological deficits [4]. Embolization is a rare but important and possibly life-threatening complication that may occur in superior vena cava thrombosis [2] [7].

Pseudotumor
  • Patients in the first group develop hydrocephalus and those in the second develop a pseudotumor-like syndrome.[ncbi.nlm.nih.gov]
  • Possible complications include pulmonary embolus, thrombophlebitis, airway edema, pseudotumor cerebri, superior sagittal sinus thrombosis, coma, and death.[westjem.com]
Internal Bleeding
  • Thrombolysis with rt-PA was not associated with cutaneous or internal bleeding and was partially effective (improvement of the clinical picture, disappearance of the right atrium thrombus, superior vena cava still occluded).[ncbi.nlm.nih.gov]
Vascular Disease
  • You are more likely to have vascular disease as you get older.[icdlist.com]
  • Continued Other Vascular Diseases Your circulatory system is made up of the vessels that carry blood to every part of your body. Vascular disease includes any condition that affects your circulatory system.[webmd.com]
  • Signs and symptoms include a nonproductive cough, breathing difficulty, cyanosis, central nervous system disorders, and edema of the conjunctiva, trachea, and esophagus. superior vena cava syndrome Superior vena cava obstruction, SVC obstruction Vascular[medical-dictionary.thefreedictionary.com]
  • Nonmalignant causes include mediastinal fibrosis; vascular diseases (eg, aortic aneurysm, large-vessel vasculitis); infections such as histoplasmosis, tuberculosis, syphilis, and actinomycosis; benign mediastinal tumors such as teratoma, cystic hygroma[mdedge.com]
  • VASCULAR DISEASE MANAGEMENT 2017;14(7):E157-E159.[vasculardiseasemanagement.com]
Acne Vulgaris
  • In the follow-up, the patient developed recurrent, painful orogenital ulcers and acne vulgaris-like skin eruptions. HLA tissue typing was HLA B-51. The diagnosis of Crohn's disease associated with Behçet's disease was made.[ncbi.nlm.nih.gov]
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]
Communicating Hydrocephalus
  • Abstract The authors report an unusual case of superior vena cava thrombosis in an infant who subsequently developed communicating hydrocephalus; they also review previously reported cases of dural sinus hypertension, and separate them into two groups[ncbi.nlm.nih.gov]

Workup

A thorough clinical and imaging assessment is necessary to make the diagnosis of superior vena cava thrombosis. A properly obtained patient history can point to an existing condition that could predispose to the symptoms observed during the physical examination. Furthermore, a localized head and neck symptomatology with distended venous vessels are highly suggestive of an ongoing process in the superior vena cava, especially in the presence of a central venous catheter. When clinical suspicion is raised, imaging studies should be employed as soon as possible. Until recently, conventional venography was the gold standard for the evaluation of superior vena cava pathology [5] [6] [8]. The necessity for non-invasive and more sophisticated methods has resulted in the replacement of conventional venography with computed tomography (CT) and magnetic resonance imaging (MRI) [1] [8]. In fact, multidetector CT (MDCT) is able to delineate the exact changes occurring in this vessel and is sufficient to make the diagnosis of superior vena cava thrombosis. Moreover, it also obtains crucial information for choosing the optimal therapeutic approach [1]. In addition, CT and MRI angiography are described as potentially useful studies [5].

Mural Thrombus
  • Abstract This is the first case report to our knowledge of superior vena cava syndrome and lethal, massive pulmonary embolus associated with a noniatrogenic right atrial mural thrombus.[ncbi.nlm.nih.gov]

Treatment

  • The clinical features of superior vena cava syndrome disappeared completely 3 d after treatment. No complications were observed and radiological investigations showed blood flow through the thrombus after treatment.[ncbi.nlm.nih.gov]
  • Patients need to know what to report to their oncology team members so that early treatment can be initiated.[ncbi.nlm.nih.gov]
  • This case report represents the successful treatment of superior vena cava thrombosis with urokinase in a patient with an indwelling Hickman catheter.[ncbi.nlm.nih.gov]
  • Ultrasonography of the pleura and thoracic vasculature was invaluable in making the diagnosis and monitoring the resolution of this condition during treatment.[ncbi.nlm.nih.gov]
  • The development of superior mediastinal widening is a valuable sign of SVC thrombosis and its recognition may allow earlier diagnosis and treatment of this condition.[ncbi.nlm.nih.gov]

Prognosis

  • As for the prognosis, based on longer series, 5 the most important factors to keep in mind are the state of the disease and tumor histology.[archbronconeumol.org]
  • Early detection improves prognosis and is based on clinical data and imaging studies. We describe 8 cases where the SVCS was the first manifestation of neoplastic diseases.[ncbi.nlm.nih.gov]
  • Prognosis The prognosis depends on the underlying cause of SVCS. In cases of SVCS caused by lung cancers, the prognosis is generally rather poor since SVCS does not generally occur until the later stages of these diseases.[medical-dictionary.thefreedictionary.com]
  • However, the ultimate prognosis is of course associated with the underlying condition.[patient.info]

Etiology

  • The etiologies of venous thrombosis and thrombogenetic effect of chronic lead exposure are discussed.[ncbi.nlm.nih.gov]
  • Other etiologies were appropriately ruled out and the thrombosis was initially attributed to Crohn's disease. In the follow-up, the patient developed recurrent, painful orogenital ulcers and acne vulgaris-like skin eruptions.[ncbi.nlm.nih.gov]
  • KEYWORDS: Brachiocephalic veins; catheterization, central venous/adverse effects; vena cava, superior; venous thromboembolism/diagnosis/etiology/prevention & control/therapy[ncbi.nlm.nih.gov]
  • Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10coded.com]
  • Hypercoagulable states have also become an important etiology for SVC syndrome. Although SVC obstruction is mostly related to malignant process but many different causes have also been described.[romj.org]

Epidemiology

  • 3] Synonyms and Keywords: SVC syndrome; superior vena cava obstruction; SVC obstruction; superior mediastinal syndrome; SVCS; SVCO Overview Historical Perspective Pathophysiology Causes Differentiating Superior Vena Cava Syndrome from other Diseases Epidemiology[wikidoc.org]
  • […] obstruction of the superior vena cava (SVC) ( 1 ): 60% due to malignant causes 20–40% thrombosis or nonmalignant causes Usual course: acute; usually 2 to 4 weeks from onset of symptoms to diagnosis Synonym(s): superior mediastinal syndrome; SVC obstruction Epidemiology[unboundmedicine.com]
  • In children, the most common causes are T-cell acute lymphoblastic leukaemia and non-Hodgkin's lymphoma (T-cell lymphoblastic lymphoma). [ 5 ] Epidemiology Gender predilection reflects aetiology, with more males than females due to the prevalence of lung[patient.info]
  • From Wikibooks, open books for an open world Jump to navigation Jump to search Superior Vena Cava Syndrome Histologic diagnosis is essential for choosing appropriate therapy Epidemiology [ edit ] 15,000 cases in US per year Caused by invasion/compression[en.wikibooks.org]
  • Epidemiology Etiology 60%-85% of cases of SVC syndrome are due to intrathoracic malignancies, with the remainder due to benign causes. NSCLC accounts for 50% of all malignant causes, followed by by SCLC (25% of malignant cases) and NHL (10%).[clinicaladvisor.com]
Sex distribution
Age distribution

Pathophysiology

  • Causes malignancy ( 90% of cases), most commonly 5 : lung carcinoma lymphoma central venous catheters pacemaker wires fibrosing mediastinitis l uetic aneurysm Behcet disease Pathophysiology In long-standing cases with 60% or more stenosis, collateral[radiopaedia.org]
  • ., Ph.D. [2] Maria Fernanda Villarreal, M.D. [3] Synonyms and Keywords: SVC syndrome; superior vena cava obstruction; SVC obstruction; superior mediastinal syndrome; SVCS; SVCO Overview Historical Perspective Pathophysiology Causes Differentiating Superior[wikidoc.org]
  • […] superior mediastinal syndrome; SVC obstruction Epidemiology Predominant age: all ages, less commonly children and young adults (16 to 30 years) Predominant sex: male female Incidence 15,000 new cases per year in the United States ( 1 ) Etiology and Pathophysiology[unboundmedicine.com]
  • Pathophysiology 11. Pathophysiology Right lung. Lymph nodes. Mediastinal structures. Thrombosis of blood within SVC. 12. Collateral veins may arise from the azygos, internal mammary, lateral thoracic, paraspinous, and esophageal venous systems .[slideshare.net]
  • The pathophysiology of BD remains largely obscure.[thrombosisjournal.biomedcentral.com]

Prevention

  • KEYWORDS: Brachiocephalic veins; catheterization, central venous/adverse effects; vena cava, superior; venous thromboembolism/diagnosis/etiology/prevention & control/therapy[ncbi.nlm.nih.gov]
  • Many conditions can cause the blood to clot too much or prevent blood clots from dissolving properly.[icdlist.com]
  • Similarly, a filter in the superior vena cava may prevent pulmonary embolism from an upper extremity thrombus.[aafp.org]
  • No preventive measures known Commonly Associated Conditions Malignancy: Common: lung, lymphoma, and metastatic breast Less common: germ cell tumors, thymoma, and mesothelioma Hyperthyroidism Infections: tuberculosis, histoplasmosis, syphilis -- To view[unboundmedicine.com]
  • Antiemetics may be provided as needed to prevent nausea and vomiting. If a patient has been started on steroids, the steroids should be tapered slowly, depending on the patient's condition.[emedicine.medscape.com]

References

Article

  1. Sheth S, Ebert MD, Fishman EK. Superior vena cava obstruction evaluation with MDCT. AJR Am J Roentgenol. 2010;194(4):W336-346.
  2. Lepper PM, Ott SR, Hoppe H, et al. Superior vena cava syndrome in thoracic malignancies. Respir Care. 2011;56(5):653-666.
  3. Otten TR, Stein PD, Patel KC, Mustafa S, Silbergleit A. Thromboembolic disease involving the superior vena cava and brachiocephalic veins. Chest.2003;123(3):809-812.
  4. Nunnelee JD. Superior vena cava syndrome. J Vasc Nurs. 2007;25(1):2-5.
  5. Santra A, Nandi S, Mondal S, Chakraborty S. Superior Vena Cava Syndrome due to Thrombosis: A Rare Paraneoplastic Presentation of Bronchogenic Carcinoma. Iran J Med Sci. 2016;41(4):354-358.
  6. Shaikh I, Berg K, Kman N. Thrombogenic Catheter-Associated Superior Vena Cava Syndrome. Case Rep Emerg Med. 2013;2013:793054.
  7. Nascimbene A, Angelini P. Superior Vena Cava Thrombosis and Paradoxical Embolic Stroke due to Collateral Drainage from the Brachiocephalic Vein to the Left Atrium. Tex Heart Inst J. 2011;38(2):170-173.
  8. Cohen R, Mena D, Carbajal-Mendoza R, Matos N, Karki N. Superior vena cava syndrome: A medical emergency? Int J Angiol. 2008;17(1):43-46.

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Last updated: 2017-08-09 12:43