The Superior Vena Cava Syndrome: Clinical Characteristics and Evolving Etiology

ArticleinMedicine 85(1):37-42 · February 2006with 69 Reads
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Abstract
Malignancy is the most common cause of the superior vena cava (SVC) syndrome. With the increasing use of intravascular devices, the incidence of the SVC syndrome arising from benign etiologies is increasing. We reviewed the etiology and outcome of 78 patients with SVC syndrome over 5 years. Malignancy was the etiology in 60% of the cases, and bronchogenic carcinoma was the most common malignancy. Small cell and non-small cell lung cancer accounted for 17 (22%) and 19 (24%) cases, respectively, but a higher percentage of patients with small-cell lung cancer developed the syndrome (6% vs 1%). Lymphoma and germ cell tumors were other significant malignant causes (8% and 3% of cases, respectively). An intravascular device was the most common etiology in benign cases (22 of 31 cases; 71%), with fibrosing mediastinitis the second most common benign etiology (6 cases). 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%). 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.
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    Purpose: The aim of this paper is to evaluate the safety and efficacy of endovascular revascularization of malignant superior vena cava syndrome (SVCS) and simultaneous implantation of a totally implantable venous access port (TIVAP) using a dual venous approach. Materials and methods: Retrospectively, 31 patients (mean age 67 ± 8 years) with malignant CVO who had undergone revascularization by implantation of a self-expanding stent into the superior vena cava (SVC) (Sinus XL®, OptiMed, Germany; n = 11 [Group1] and Protégé ™ EverFlex, Covidien, Ireland; n = 20 [Group 2]) via a transfemoral access were identified. Simultaneously, percutaneous access via a subclavian vein was used to (a) probe the lesion from above, (b) facilitate a through-and-through maneuver, and (c) implant a TIVAP. Primary endpoints with regard to the SVC syndrome were technical (residual stenosis < 30%) and clinical (relief of symptoms) success; with regard to TIVAP implantation technical success was defined as positioning of the functional catheter within the SVC. Secondary endpoints were complications as well as stent and TIVAP patency. Results: Technical and clinical success rate were 100% for revascularization of the SVS and 100% for implantation of the TIVAP. One access site hematoma (minor complication, day 2) and one port-catheter-associated sepsis (major complication, day 18) were identified. Mean catheter days were 313 ± 370 days. Mean imaging follow-up was 184 ± 172 days. Estimated patency rates at 3, 6, and 12 months were 100% in Group 1 and 84, 84, and 56% in Group 2 (p = 0.338). Conclusion: Stent-based revascularization of malignant SVCS with concomitant implantation of a port device using a dual venous approach appears to be safe and effective.
  • Article
    Venous obstruction in the cancer population can result in substantial morbidity and, in extreme cases, mortality. While venous obstruction can be caused by both benign and malignant etiologies in this population, the management of malignant venous obstruction as a palliative measure can be somewhat nuanced with respect to nonprocedural and procedural management, both with respect to treatment of the underlying malignancy as well as treatment of venous hypertension, which may be associated with venous thrombosis. Symptom severity, primary malignancy, functional status, and prognosis are all fundamental to the patient workup and dictate both the timing and extent of endovascular intervention. The morbidity and mortality associated with malignant obstructions of central venous structures, specifically the superior vena cava and inferior vena cava, can be significantly improved with endovascular management in appropriately selected patients. Thus, the pertinent literature regarding the clinical presentation, workup, and endovascular management of malignant central venous obstruction syndromes, with directed attention to superior vena cava syndrome and inferior vena cava syndrome, will be reviewed in this article.
  • Article
    Objective: To investigate the feasibility and safety of the peripherally inserted central catheters (PICCs) accessed via the superficial femoral vein in patients with superior vena cava syndrome (SVCS). Methods: From October 2010 to December 2014, 221 cancer patients with SVCS in our center received real-time ultrasound-guidance of the superficial femoral vein inserted central catheters (FICCs) at the mid-thigh. PICC insertion via upper extremity veins had also been investigated in 2604 cancer patients without SVCS as control. The average catheterization time, one-time puncture frequency, catheter duration and complications were compared between two groups. Results: In the FICC group, the mean catheterization time was 31.60 ± 0.15 minutes, one-time puncture frequency was 1.05 ± 0.08, and catheter duration was 168.95 ± 20.47 days. There was no significant difference compared with the upper extremity veins PICC group: 31.11 ± 3.86 minutes, 1.03 ± 0.30, and 173.58 ± 39.81 days, respectively. The major complications included skin allergy to chlorhexidine gluconate (CHG) dressings, exudation, catheter-related infection, catheter occlusions, unplanned catheter withdrawal, venous thrombosis, mechanical phlebitis, and catheter malposition. It is interesting that a higher rate of catheter malposition was observed in the upper extremity veins PICC group than in the FICC group (2.15% vs. 0.00%). There were no significant differences in other complications between the two groups. Conclusions: Real-time ultrasound-guided PICCs accessed via the superficial femoral vein at the mid-thigh is a new modified technique with low complications, which can be a feasible and safe alternative venous access for patients with SVCS.
  • Article
    Unintentional iatrogenic surgical complications can complicate the operative and postoperative courses of paediatric cardiac surgery patients. Unless recognized and treated early, if possible, these complications may lead to a prolonged hospital stay, increased treatment cost, morbidity, and even death. Ventricular Septal Defect (VSD) is the most common congenital heart defect in children, occurring in 50% of all children with congenital heart disease and in 20% as an isolated lesion. Herein, we discuss the development of Superior Vena Cava (SVC) syndrome following surgical repair of VSD in a nine-month-old child. Good clinical suspicion, immediate confirmation with Transthoracic Echocardiography (TTE) and surgical re-exploration led to a good outcome in this patient. If, however, we could have performed an intraoperative Trans Esophageal Echocardiography (TEE), this complication could have been recognized, even before shifting from the operating room. © 2017, Journal of Clinical and Diagnostic Research. All rights reserved.
  • Article
    Obstruction of the superior vena cava (SVC) is an uncommon, but potentially life-threatening condition due to likely development of edema in the head and neck and potential respiratory compromise. Less than half of those affected by SVC syndrome survive more than a year. Obstruction can be from neoplasms or secondary to benign disease. Treatment for most cases of symptomatic SVC syndrome involves placement of a stent to relieve the stenosis. Serious complications such as stent migration, pulmonary embolism, and cardiac tamponade can occur in 5% to 10% of cases, and inadequate imaging of the SVC–atrial junction by fluoroscopy contributes to these problems. The overlapping contrast in the atrium makes it difficult to precisely place the distal end of the stent, potentially allowing for embolization of the stent to occur. We present a case series of 3 patients wherein transesophageal echocardiography was used for guidance of stent placement in the SVC and significantly aided in placement.
  • Article
    The development of medical emergencies related to the underlying disease or as a result of complications of therapy are common in patients with hematologic or solid tumors. These oncological emergencies can occur as an initial presentation or in a patient with an established diagnosis and are encountered in all medical care settings, ranging from primary care to the emergency department and various subspecialty environments. Therefore, it is critically important that all physicians have a working knowledge of the potential oncological emergencies that may present in their practice and how to provide the most effective care without delay. This article reviews the most common oncological emergencies and provides practical guidance for initial management of these patients.
  • Article
    Twenty-two of 53 (42%) patients developed subclavian vein thrombosis (SVT) while receiving chemotherapy for advanced malignancy by an ambulatory pump constant infusion delivery system. The median time to thrombosis was 30 days (range, 5-270 days). Seven patients (36%) were asymptomatic and received no specific anticoagulation. Twelve patients (50%) received heparin followed by coumadin for 2 to 3 months. None of the 19 patients developed complications of the SVT. In three patients (14%), acute SVT progressed to complete or partial superior vena cava syndrome. The use of fibrinolytic therapy was successful in all three patients, and was adequate to lead to complete clinical resolution. Predisposing factors in the patient population included: (1) decreased antithrombin III levels prior to catheter insertion; (2) intrathoracic tumor, possibly creating low flow rates; and (3) improper or suboptimal home management of the catheter. The development of SVT was not related to the chemotherapeutic agent infused or the duration of therapy. The frequency of SVT in this patient population necessitates consideration of prophylactic methods in high-risk patients.
  • Disorders of the mediastinum
    • Dr Park
    • Dj Pierson
    Park DR, Pierson DJ. Disorders of the mediastinum. In: Murray JF, Nadel JA, eds. Textbook of Respiratory Medicine. 3rd ed. Philadelphia: WB Saunders; 2000:2079–2094.
  • Superior vena caval obstruction with a consideration of the possible relief of symptoms by mediastinal decompression
    • W Ehrlich
    • Hc Ballon
    • Ea Graham
    Ehrlich W, Ballon HC, Graham EA. Superior vena caval obstruction with a consideration of the possible relief of symptoms by mediastinal decompression. J Thorac Surg. 1934;3:352.
  • The history of an aneurysm of the aorta with some remarks on aneurysms in general
    • W Hunter
    Hunter W. The history of an aneurysm of the aorta with some remarks on aneurysms in general. Med Obs Soc Phys Lond. 1757;1:323.
  • Article
    We reviewed the records of 28 patients with superior vena cava syndrome (SVCS) between 1973 and 1978 to establish the risk from invasive diagnostic procedures and the therapeutic value of the information obtained. Of 23 patients, this syndrome was the initial manifestation in 18. Bronchoscopy with endobronchial biopsy, thoracentesis, pleural biopsy, lymph node biopsy, and, in one patient, thoracotomy were performed without major complications. Small cell undifferentiated carcinoma of the lung was found in seven of the 18 patients with SVCS as the initial manifestation of their disease. We believe that carefully performed invasive diagnostic procedures can be carried out safely. Furthermore, accurate histological diagnosis assumes major importance with the development of effective chemotherapy for small cell undifferentiated carcinoma of the lung. (JAMA 1981;245:951-953)
  • Article
    Background: Superior vena cava syndrome (SVCS) is a frequent presentation of malignancies involving the mediastinum and can seriously compromise treatment options and prognosis. Stenting of superior vena cava is a well-known but not so commonly used technique to alleviate this syndrome. Patients and methods: Between August 1993 and December 2000 we performed 52 stenting procedures in patients affected by non-small cell lung cancer (NSCLC). Results: Phlebographic resolution of the obstruction was achieved in 100% of cases with symptomatic and subjective improvement in more than 80%. One major complication was observed due to bleeding during anticoagulation. Re-obstruction of the stent occurred in only 17% of the cases, the majority due to disease progression. Improvement of the syndrome allowed hydration necessary for full dose platinum treatment when indicated in patients affected by lung cancer. Conclusions: Stenting of the superior vena cava syndrome is a safe and effective procedure achieving a rapid alleviation of symptoms in almost all patients, and allowing for full dose treatment in lung cancer patients. This procedure could change the traditional poorer prognosis attributed to non-small cell lung cancer patients presenting with this syndrome.
  • Article
    Study objective: Patients with cystic fibrosis (CF) frequently require recurrent courses of IV antibiotics to treat acute exacerbations of their pulmonary disease. Over time, CF patients often lose peripheral access, and indwelling central venous catheters are placed. We attempted to determine the type and incidence of catheter complications so that CF patients could be fully informed of the risks prior to placement of these catheters. Design: The charts of all CF patients who attended the Adult Cystic Fibrosis Clinic of the University of Washington Medical Center from January 1989 through December 1998 were reviewed. Demographic information was obtained along with the type and duration of catheter, type and number of complications, and the use of anticoagulant medication. Measurements and results: Of the 218 CF patients who attended the clinic, 65 patients (30%) had indwelling catheters in place at some time during the study period. A total of 87 catheters were placed into these 65 patients. The total number of catheter-days for first indwelling catheters was 68,220. The total number of catheter-days for all catheters was 75,660 (210 catheter-years). Thirty-five catheter-related complications were identified, occurring in 26 patients. Complications included thrombosis (n = 14), infections (n = 9), mechanical problems (n = 6), pneumothorax (n = 3), superior vena cava syndrome/stenosis (n = 2), and air embolism (n = 1), for an overall complication rate of 0.463/1,000 catheter-days. Conclusion: We conclude that indwelling catheters are relatively safe in patients with CF. Good infection control policies appear to prevent most infectious complications. The most common complication is that of thrombosis, which may be recurrent in some patients. Consideration should be given to prophylactic warfarin therapy despite the potential risk of significant hemoptysis in this patient population.
  • Article
    The treatment of a superior vena caval obstruction associated with a mediastinal mass is a true radiotherapeutic emergency. The heralding signs and symptoms and the morbidity of the syndrome justify beginning therapy before a pathologic diagnosis is established. In a series of 19 patients with superior vena cava syndrome, there was an excellent response to an initial high-dose course of irradiation, consisting of 400 rads midplane for 3 days, then reduced to conventional daily fractionation. It is concluded that rapid high-dose irradiation in the treatment of a superior vena cava syndrome is safe and effective.
  • Article
    Twenty-two of 53 (42%) patients developed subclavian vein thrombosis (SVT) while receiving chemotherapy for advanced malignancy by an ambulatory pump constant infusion delivery system. The median time to thrombosis was 30 days (range, 5–270 days). Seven patients (36%) were asymptomatic and received no specific anticoagulation. Twelve patients (50%) received heparin followed by coumadin for 2 to 3 months. None of the 19 patients developed complications of the SVT. In three patients (14%), acute SVT progressed to complete or partial superior vena cava syndrome. The use of fibrinolytic therapy was successful in all three patients, and was adequate to lead to complete clinical resolution. Predisposing factors in the patient population included: (1) decreased antithrombin III levels prior to catheter insertion; (2) intrathoracic tumor, possibly creating low flow rates; and (3) improper or suboptimal home management of the catheter. The development of SVT was not related to the chemotherapeutic agent infused or the duration of therapy. The frequency of SVT in this patient population necessitates consideration of prophylactic methods in high-risk patients.
  • Article
    A 10 year experience in 66 patients with obstruction of the superior vena cava (SVC) was reviewed. Pathogenesis was malignancy in all except two patients with granulomatous mediastinitis (3%). Venography was especially helpful in determining extent of SVC obstruction and collateral circulation. Only nine patients are still alive; 57 are dead (mean survival, 3.9 months). Patients with upper airway obstruction and/or cerebral edema only survived 1.4 months (P less than 0.001). The two patients with benign obstruction had a new operation to bypass the SVC using a spiral graft of saphlenous vein with complete relief of SVC syndrome up to 26 months. Lymphomas responded to radiation (63% improved), but response in the bronchogenic carcinoma group was variable (42% improved) and was not related to palliative radiation, chemotherapy, or both. Patients receiving full-course mediastinal radiation (more than 5,000 rads) had improved survival (9.9 months). Three patients with bronchogenic carcinoma and severe SVC syndrome (cerebral edema and upper airway obstruction) had excellent palliation using spiral vein bypass graft and postoperative radiation. This experience suggests that treatment of patients with obstruction of SVC based on evaluation of signs, symptoms, and venography may provide extended palliation. If the syndrome is mild, full-course mediastinal radiation is given. If there is cerebral edema or upper airway obstruction, operation to bypass the SVC will relieve SVC syndrome immediately and will allow orderly radiation therapy.
  • Article
    The treatment of a superior vena caval obstruction associated with a mediastinal mass is a true radiotherapeutic emergency. The heralding signs and symptoms and the morbidity of the syndrome justify beginning therapy before a pathologic diagnosis is established. In a series of 19 patients with superior vena cava syndrome, there was an excellent response to an initial high-dose course of irradiation, consisting of 400 rads midplane for 3 days, then reduced to conventional daily fractionation. It is concluded that rapid high-dose irradiation in the treatment of a superior vena cava syndrome is safe and effective.
  • This retrospective review was performed with an intent to clarify several misconceptions associated with superior vena cava syndrome (SVCS). During a 16-yr period, we diagnosed and treated 63 patients with SVCS, including seven (11%) children younger than 10 yr of age. Thirty patients (47.6%) had bronchogenic carcinoma, and 13 (20.6%) had lymphoma. In 43 cases SVCS was the presenting symptom of a mediastinal condition. Forty-one patients underwent diagnostic procedures with no major complications, and diagnosis was obtained in 36. Only six patients had surgical treatment, and 45 had radiation therapy, chemotherapy, or both. There was no mortality associated directly with venous congestion. Symptomatic relief occurred in 80% of treated patients. We conclude that: (1) SVCS per se should not be feared (symptomatic relief is the rule), (2) accurate diagnosis can be achieved with minimal morbidity, and (3) the versatile underlying etiology dictates the outcome that can be improved with appropriate therapy.
  • Article
    Seventy-six cases of superior vena caval obstruction (SVCO) were documented in the period 1970-1980. There were 53 males and 23 females, with a mean age of 61.7 years. The underlying causes were:- Lung cancer, 64/76, 84.2%; Metastatic disease, 4/76, 5.3%; Lymphoma, 4/76, 5.3%; Benign aetiology, 2/76, 2.6%; Undiagnosed, 2/76, 2.6%; 97.4% of the cases were due to malignancy. Of 61 patients on whom follow-up is available, only one is alive. Eighty-two percent of this group died within one year following the onset of SVCO. Autopsies were performed on 17 patients. Nine of these (52.9%) showed invasion of the superior vena cava, 6 (35.3%) showed compression without invasion, and 2 (11.8%) showed neither compression nor invasion following Radiotherapy. Venography was performed on all patients with only one minor complication being recorded.
  • Article
    One hundred and fifty-nine patients with symptoms of superior vena caval obstruction who presented to two major hospitals over a 10-year period, from 1970 to 1979, were reviewed. Lung cancer was the most common histological diagnosis. The most common symptoms were dyspnoea and a feeling of fullness in the head. The most common physical findings were dilatation of the neck or chest wall veins, or oedema of the face and arms. Superior mediastinal widening was the most common radiological abnormality. No significant morbidity was associated with any diagnostic procedure. Only patients with lymphoma had a significantly longer survival period, both from the diagnosis of the disease, and from the onset of the symptoms of superior vena caval obstruction. There is no evidence that superior vena caval obstruction is an absolute medical emergency. Appropriate diagnostic steps should be undertaken to establish the histological diagnosis. The prognosis for some tumour types may be improved by combined modality therapy (chemotherapy plus radiotherapy).
  • Article
    The main cause of superior vena cava syndrome (SVCS) is malignant disease. However, multiple other diseases must be considered in the differential diagnosis and a new entity, SVCS due to central venous catheters, represents an etiology of increasing importance. SVCS due to malignancy should not be considered as a radiotherapeutic emergency. Careful management including invasive procedures such as mediastinoscopy to make a definite diagnosis should be performed when necessary by skilled practitioners, as specific therapy depends on a histologic diagnosis. Irradiation remains the standard treatment for many solid tumours, particularly non-small cell lung cancer. For chemosensitive tumours such as small cell bronchogenic carcinoma or lymphoma, chemotherapy can be recommended as initial treatment, and it is associated with high regression rates of the SVCS. In the case of a recent thrombosis of the superior vena cava, fibrinolytics may be applied as suggested by the experience obtained with central venous catheters.
  • Article
    A totally implanted subclavian venous access system composed of a reservoir and silastic catheter was employed in 92 patients receiving infusion chemotherapy and/or hyperalimentation. The major catheter complication was subclavian or jugular vein thrombosis observed in 15 patients (16%). Thrombosis was observed in the ipsilateral subclavian or jugular vein surrounding the catheter without restricting function, except in two patients with thrombosis in the vein at the end of the catheter. Prophylaxis with low-dose Coumadin was effective in preventing thrombosis in high-risk patients as defined by a history of prior thrombosis. Streptokinase and/or heparin relieved the signs and symptoms of thrombosis, but clot dissolution or reversal of collateral flow was not observed. Explantation of the catheter was not necessary in all patients in that embolic complications of the thrombosis were not observed, and the system was retained and functioned in five patients in spite of the presence of thrombosis around the catheter. Other complications of the implanted system include "pocket" infection, catheter migration, and occlusion. Most complications may be managed without obligate catheter removal.
  • Article
    The cases of 42 patients with malignant superior vena cava (SVC) obstruction were reviewed to evaluate clinical dogmas of prohibitive risk for invasive diagnostic procedures and need for urgent radiotherapy. Thirty-nine had carcinoma (35, bronchogenic and 4, other), and 3 had lymphoma. Lung cancer histology was squamous cell in 11, adenomatous in 10, large cell in 7, and small cell in 7. The SVC obstruction was always symptomatic, usually causing facial or cervical swelling, but there was no instance of SVC obstruction causing life-threatening problems such as cerebral or laryngeal edema. Twenty-two patients underwent bronchoscopy (11 flexible and 11 rigid) prior to radiotherapy without respiratory complications, and diagnostic tissue was obtained in 8. Also prior to radiotherapy, 29 invasive diagnostic procedures were performed: thoracotomy (1), mediastinotomy or mediastinoscopy (11), supraclavicular or scalene node biopsy (15), and percutaneous lung needle biopsy (2). Neither excessive blood loss nor serious complications occurred, and diagnostic tissue was obtained in 22 patients who received individualized therapy. Eight patients had urgent radiotherapy, which delayed diagnosis and specific therapy for two weeks to 6 months. For the 33 patients who underwent radiotherapy after development of the SVC obstruction, the obstruction clinically resolved spontaneously within fourteen days, independently of whether radiotherapy was begun immediately or was delayed. Median survival was 5.0 months and was not influenced by the dose or timing (early or late) of radiotherapy. We reached the following conclusions. First, although a grim prognostic sign, SVC obstruction is rarely life-threatening and typically resolves spontaneously, probably by development of venous collaterals.(ABSTRACT TRUNCATED AT 250 WORDS)
  • Article
    For the past 30 years patients presenting with the superior vena caval syndrome (SVCS) have, as a recommended practice, been treated with radiotherapy without necessarily establishing a tissue diagnosis. This practice has been pursued as it was accepted that the probability that unresectable lung cancer was the cause of the syndrome was high; that SVCS was a condition for which diagnostic procedures carried a high risk; and that SVCS was a life-threatening situation requiring immediate relief. To assess the validity of this practice, the literature since 1934 was reviewed. One thousand nine hundred eighty-six cases of reported SVCS were identified resulting in the emergence of several important facts: (1) small cell lung cancer is currently the leading etiology of SVCS accounting for approximately 40% of all cases due to lung cancer; (2) the experience with performing thoracotomies, mediastinoscopies, bronchoscopies, lymph node biopsies, and venograms as reported in the literature suggests that all of these diagnostic procedures can be performed safely; (3) while the SVCS can cause worrisome symptomatology, there is little reported clinical or experimental evidence that an unrelieved SVCS is life threatening; and (4) patency of the SVCS may not be reestablished after palliative therapy even though signs and symptoms may resolve. Because of therapeutic advances in the treatment of small cell lung cancer, lymphoproliferative disorders, and other malignant etiologies of the SVCS, the findings of this review suggest that a policy of treatment without histologic diagnosis is ill advised.
  • Article
    In a randomised prospective trial of chemotherapy with and without radiotherapy in small-cell carcinoma of the bronchus, 37 of 366 patients presented with obstruction of the superior vena cava at the time of diagnosis. In the study all patients received four cycles of combination chemotherapy over a period of 12 weeks and, provided there was not progressive disease, then received either radiotherapy to the mediastinum and primary tumour followed by eight further courses of chemotherapy or eight cycles of chemotherapy alone. Of the 37 patients presenting with superior vena caval obstruction, nine had relapsed and treatment was stopped during or after the initial four cycles of chemotherapy. Of the remainder, 15 patients received radiotherapy plus chemotherapy and 13 chemotherapy alone. After four cycles of chemotherapy (12 weeks) 21 of the 37 patients had initial complete relief of symptoms secondary to superior vena caval obstruction, 10 had substantial but partial relief, and six had no relief. Twelve patients developed recurrence of superior vena caval obstruction, of whom six had received radiotherapy and four chemotherapy alone; two relapsed in the initial 12 weeks of the study. The median survival of the patients with superior vena caval obstruction allocated at diagnosis to either treatment arm was identical. The survival of patients with obstruction was similar to that of other patients in the main study. Chemotherapy is an effective treatment for superior vena caval obstruction and there appears to be no additional advantage in giving radiotherapy after 12 weeks of cyclical chemotherapy.
  • Complications of indwelling catheters in cystic fibrosis
    • Ml Aitken
    • Mr Tonelli
    Aitken ML, Tonelli MR. Complications of indwelling catheters in cystic fibrosis. Chest. 2000;118:1598–1602.
  • Article
    With the question in mind is superior vena caval obstruction a medical emergency, we reviewed 107 cases of superior vena caval obstruction in adult patients. We sought details of the time duration between the onset of symptoms and the treatment, and examined the complication and survival of patients with this disorder. Fifteen percent of the cases developed from benign causes. In 41 percent there was a previously recognized disease as the etiology. Benign disorders required longer to make the diagnosis. No serious complication resulted from the superior vena caval obstruction itself nor investigative procedures leading to the diagnosis despite, in some cases, a prolonged period between the onset of symptoms and the initiation of therapy. Prognosis and response to treatment were dependent on the underlying cause of the superior vena caval obstruction. Although several cases of tracheal obstruction were included in this series, we did not address the question of whether tracheal obstruction is or is not a medical emergency. No support was found for the notion that superior vena caval obstruction in itself represents a radiotherapeutic emergency.
  • Article
    The Mayo Clinic experience with superior vena cava obstruction during the last 20 years was reviewed. The diagnosis of superior vena cava obstruction is often made at the bedside. Typical symptoms include suffusion, dyspnea, cough, and, less commonly, pain, syncope, dysphagia, and hemoptysis. The most important physical findings are the increased collateral veins covering the anterior chest wall and the dilated neck veins with edema of the face, arms, and chest. The chest x-ray film usually shows widening of the superior mediastinum. Of our 86 cases of superior vena cava obstruction, 67 (78%) were due to malignancy and 19 (22%) to benign causes. The cause of obstruction is usually established by bronchoscopy, open lung biopsy, or biopsy of the superficial lymph node. Radiotherapy remains the standard approach for the treatment of superior vena cava obstruction due to malignant disease. It is of particular interest to note that of the six benign cases resulting from thrombosis of the superior vena cava, three were due to the use of central venous catheters. Physicians should be aware of this association.
  • Article
    We reviewed the records of 28 patients with superior vena cava syndrome (SVCS) between 1973 and 1978 to establish the risk from invasive diagnostic procedures and the therapeutic value of the information obtained. Of 23 patients, this syndrome was the initial manifestation in 18. Bronchoscopy with endobronchial biopsy, thoracentesis, pleural biopsy, lymph node biopsy, and, in one patient, thoracotomy were performed without major complications. Small cell undifferentiated carcinoma of the lung was found in seven of the 18 patients with SVCS as the initial manifestation of their disease. We believe that carefully performed invasive diagnostic procedures can be carried out safely. Furthermore, accurate histological diagnosis assumes major importance with the development of effective chemotherapy for small cell undifferentiated carcinoma of the lung.
  • Article
    During the past 23 1/2 years, 510 infants under 12 months of age had 756 Broviac central venous catheters inserted. At first catheter insertion 51 per cent of patients were less than 1 month old. Catheter function ranged from 3 to 1080 days (mean = 90 days). Sites of insertion were saphenous 85 per cent, external jugular 7 per cent, internal jugular 5 per cent, subclavian 2 per cent, cephalic 0.7 per cent, and transthoracic azygos 0.5 per cent. Eighty-nine patients had malabsorption syndromes, 86 had short bowel syndrome, 74 had intractable diarrhea, and 261 required nutritional support for other reasons. Vena caval thrombosis developed in 35 infants; 23 had inferior vena cava (IVC) occlusion (4.5% at risk); six had isolated superior vena cava (SVC) occlusion (11% at risk), and six had both SVC and IVC thrombosis. No major symptoms or complications resulted from isolated IVC thrombosis, whereas all infants with SVC occlusion developed head and neck swelling, 50 per cent developed pleural effusions, and two died. Each of six infants with combined IVC and SVC occlusion died within 6 months. We conclude that SVC occlusion is a very serious complication in infants receiving total parenteral nutrition (TPN) solutions and that infusion via the IVC has fewer and less serious complications.
  • Article
    The aim of this study was to analyze the features of the superior vena cava syndrome (SVCS) as initial characteristics in small-cell lung cancer: incidence, dissemination of disease, diagnostic procedures, efficacy and toxic effects of chemotherapy, and median survival in patients with SVCS. In a prospective series of 724 patients with biopsy-proved small-cell lung cancer seen during a 6-year period, we reviewed data from patients who also had SVCS. The incidence of SVCS was 87 of 724 at the time of diagnosis. Initial emergency radiation therapy was not used in these patients. Diagnostic procedures in these patients were not associated with mortality. Rapid initiation of intensive chemotherapy, often with heparin therapy, resulted in complete or partial responses in 81% and no response in 12%; data were not evaluable in 7%. Two of these 87 patients died of aplasia within 4 weeks of chemotherapy. Median survival was not significantly different in the patients with SVCS (median, 42 weeks) and without SVCS (median, 40 weeks). A significant increase in initial brain metastases at the time of diagnosis was observed in patients with SVCS (22% vs 11%). Intensive chemotherapy is the first line of therapy in small-cell lung cancer. Histologic diagnostic procedures must be performed in patients with SVCS to adapt the treatment to the underlying cause. Initial emergency radiotherapy, before diagnosis or chemotherapy, does not seem to be useful in these patients. Computed tomography of the brain should be performed routinely in patients with SVCS, and prophylactic brain irradiation could be helpful in such patients. Apparently SVCS is not a poor prognostic factor in treated small-cell lung cancer.
  • Article
    To identify prognostic or treatment factors influencing the response of superior vena cava obstruction (SVCO), time to SVCO recurrence, and overall survival of SCLC patients with SVCO at presentation; and to assess the role of retreatment in patients with SVCO at recurrent or persistent disease. Between January 1983 and November 1993, 76 consecutive patients who had small-cell lung cancer (SCLC) with SVCO were treated in our institution. Analysis was done according to the disease status at diagnosis of SVCO. The first analysis concerned a group of 50 patients who had SVCO at initial presentation. The second analysis concerned a group who had SVCO as a manifestation of persistent or recurrent disease. In the first analysis, 93% had significant improvement in symptoms of SVCO after chemotherapy and 94% after mediastinal radiation. Response is almost universal despite a wide range of radiation fractionation and total dose used. Seventy percent remained SVCO-free before death. Thirty percent developed recurrence of SVCO symptoms 1-16 months (median 8) after the start of initial treatment. Those who received combined chemotherapy and radiation had a longer time to SVCO recurrence (p = 0.018) compared to those who received chemotherapy alone. This effect is mainly seen in limited-stage patients. The presence of SVCO recurrence tends to have an adverse effect on the overall survival (p = 0.077) irrespective of the time when the recurrences occurred (p = 0.296). The median survival of this whole group of 50 patients in the first analysis was 9.5 months, and the 2-year survival was 10%. Stage was strongly predictive of survival (p < 0.001). Sixteen percent (3 of 19) of the patients with limited-stage diseases were long-term survivors (two patients survived 35 months and one survived 70 months). The early mortality from SVCO was 2%. In the second analysis, 85% had previously been treated with chemotherapy alone. The response rate of SVCO in the analysable patients (n = 39) was 77%. There was no significant difference in the response rate of SVCO to treatment comparing patients treated by chemotherapy first or mediastinal radiation first (p = 0.653), but most patients [82% (32 of 39)] received radiation as the initially treatment of SVCO. Ninety-three percent (38 of 41) received mediastinal radiation as a part of their ultimate retreatment regimen, and 68% (28 of 41) received mediastinal radiation as their sole retreatment regimen. Thirty-two percent (13 of 41) received chemotherapy as a part of their ultimate retreatment regimen, and only 7% received chemotherapy alone as their sole retreatment regimen. Eighty-three percent (25 of 30) of those whose SVCO responded remained free of SVCO before death, with a median survival of 3 months after recurrent or persistent disease documented. Chemotherapy or mediastinal radiation is very effective as an initial treatment in SCLC patients with SVCO at presentation and at recurrent or persistent disease. There is no obvious need to use big radiation fraction sizes for the first few radiation treatment as was previously believed. In patients with recurrent or persistent SCLC with SVCO, especially in those who previously received chemotherapy only, we have more experience in incorporating mediastinal radiation as a major component of the palliative regimen with highly effective and durable palliation achieved.
  • Article
    To characterize cranial vena cava thrombosis in dogs with regard to signalment, clinical manifestation, potential inciting causes, treatment, and outcome. Retrospective study. 17 dogs with a cranial vena cava thrombus. Medical and necropsy records were reviewed for signalment, potential causes of thrombus formation, diagnosis, clinicopathologic findings, treatment, and outcome. A signalment predisposition was not found. Ten dogs had cranial vena cava syndrome, and 10 had a pleural effusion. Ten dogs were dyspneic, and 5 had palpable jugular thrombi. Predisposing conditions identified were presumed immune-mediated hematologic disease and corticosteroid administration (6 dogs), sepsis (6), protein-losing nephropathy (2), neoplasia (2), and cardiac disease (1). Central venous catheterization was implicated as a contributing cause. Thrombocytopenia was the most consistent clinicopathologic finding, and ultrasonography was helpful in confirming the diagnosis. Treatment varied, but 15 of the 17 dogs died or were euthanatized within 20 days of clinical manifestation of the thrombus. At necropsy, thrombi were found in other organs, mainly the right atrium, jugular veins, and pulmonary arteries. Prognosis is poor for dogs with cranial vena cava thrombosis associated with clinical signs. Use of central venous catheters should be avoided in dogs with predisposing diseases such as immune-mediated disease, sepsis, protein-losing nephropathy, neoplasia, and cardiac disease.
  • Article
    Superior vena cava (SVC) syndrome is a relatively common complication of lung cancer or lymphoma, and in fact is often the initial manifestation of these diseases. However, benign causes also exist, and physicians should not automatically assume that SVC syndrome is due to cancer. A definitive histologic diagnosis of cancer should be obtained before starting radiotherapy or chemotherapy to treat SVC syndrome.
  • Article
    There has been an evolution in vascular access in the neonate. Newer types of materials and catheters, such as PICC lines and ECMO catheters, are now available. The frequency of line placement has increased, and radiologists now perform many of these procedures. This places the radiologist in the position of not only diagnosing complications, but actually causing them. Knowledge of these complications can help the practitioner avoid them and diagnose them as quickly as possible when they occur.
  • Article
    Patients with cystic fibrosis (CF) frequently require recurrent courses of IV antibiotics to treat acute exacerbations of their pulmonary disease. Over time, CF patients often lose peripheral access, and indwelling central venous catheters are placed. We attempted to determine the type and incidence of catheter complications so that CF patients could be fully informed of the risks prior to placement of these catheters. The charts of all CF patients who attended the Adult Cystic Fibrosis Clinic of the University of Washington Medical Center from January 1989 through December 1998 were reviewed. Demographic information was obtained along with the type and duration of catheter, type and number of complications, and the use of anticoagulant medication. Of the 218 CF patients who attended the clinic, 65 patients (30%) had indwelling catheters in place at some time during the study period. A total of 87 catheters were placed into these 65 patients. The total number of catheter-days for first indwelling catheters was 68,220. The total number of catheter-days for all catheters was 75,660 (210 catheter-years). Thirty-five catheter-related complications were identified, occurring in 26 patients. Complications included thrombosis (n = 14), infections (n = 9), mechanical problems (n = 6), pneumothorax (n = 3), superior vena cava syndrome/stenosis (n = 2), and air embolism (n = 1), for an overall complication rate of 0. 463/1,000 catheter-days. We conclude that indwelling catheters are relatively safe in patients with CF. Good infection control policies appear to prevent most infectious complications. The most common complication is that of thrombosis, which may be recurrent in some patients. Consideration should be given to prophylactic warfarin therapy despite the potential risk of significant hemoptysis in this patient population.
  • Article
    Many patients with previously implanted ventricular defibrillators are candidates for an upgrade to a device capable of atrial-ventricular sequential or multisite pacing. The prevalence of venous occlusion after placement of transvenous defibrillator leads is unknown. The purpose of this study was to determine the prevalence of central venous occlusion in asymptomatic patients with chronic transvenous defibrillator leads. Thirty consecutive patients with a transvenous defibrillator lead underwent bilateral contrast venography of the cephalic, axillary, subclavian, and brachiocephalic veins as well as the superior vena cava before an elective defibrillator battery replacement. The mean time between transvenous defibrillator lead implantation and venography was 45 +/- 21 months. Sixteen patients had more than 1 lead in the same subclavian vein. No patient had clinical signs of venous occlusion. One (3%) patient had a complete occlusion of the subclavian vein, 1 (3%) patient had a 90% subclavian vein stenosis, 2 (7%) patients had a 75% to 89% subclavian stenosis, 11 (37%) patients had a 50% to 74% subclavian stenosis, and 15 (50%) patients had no subclavian stenosis. The low prevalence of subclavian vein occlusion or severe stenosis among defibrillator recipients found in this study suggests that the placement of additional transvenous leads in a patient who already has a ventricular defibrillator is feasible in a high percentage of patients (93%).
  • Article
    Histoplasmosis is an endemic mycosis, which is most prevalent in the Ohio and Mississippi valleys of North America. The causative organism is a dimorphic fungus, Histoplasma capsulatum. Histoplasmosis can present as a self-limited disease or cause life-threatening diseases resulting in considerable morbidity and mortality. Treatment is appropriate in patients with diffuse acute pulmonary infection, chronic pulmonary infection, mediastinal granuloma causing obstruction of important structures, or disseminated infection. Other chronic forms of disease such as fibrosing mediastinitis and broncholithiasis are unresponsive to pharmacologic treatment. Options for therapy include amphotericin B or one of its lipid formulations, and ketoconazole, itraconazole, or fluconazole. Recently, newer antifungal agents have been evaluated in animals models of histoplasmosis. Of these, a new triazole, posaconazole (SCH56592) appears most promising. Generally, amphotericin B or one of the lipid formulations is recommended as initial treatment in patients with more extensive diseases, felt to be ill enough to require hospitalization, and itraconazole for those who have milder illness, or to complete treatment after patients respond to amphotericin B. The role of intravenous formulation of itraconazole for severe histoplasmosis is unknown because studies comparing it with amphotericin B have not been conducted.
  • Article
    Mediastinal fibrosis is the least common, but the most severe, late complication of histoplasmosis. It should be differentiated from the many other less-severe mediastinal complications of histoplasmosis, and from other causes of mediastinal fibrosis. Posthistoplasmosis mediastinal fibrosis is characterized by invasive, calcified fibrosis centered on lymph nodes, which, by definition, occludes major vessels or airways.
  • Article
    The purpose of this study was to evaluate the role of endovascular and open surgical reconstructions in patients with superior vena cava (SVC) syndrome caused by nonmalignant disease. Clinical data from 32 consecutive patients who underwent endovascular or open surgical reconstruction of central veins because of symptomatic benign SVC syndrome between November 1983 and June 2001 were retrospectively reviewed. The study included 17 male and 15 female patients (mean age, 38 years; range, 5-69 years). Presenting symptoms were head fullness (n = 26), dyspnea or orthopnea (n = 23), headache (n = 17), or dizziness (n = 11); physical signs were head swelling (n = 31), chest wall collateral vessels (n = 29), facial cyanosis (n = 18), or arm swelling (n = 17). Etiologic factors included mediastinal fibrosis (n = 19), indwelling catheter (n = 8), idiopathic thrombosis (n = 4), or post-surgery (n = 1). Two patients were heterozygous for factor V Leiden; 1 patient had antithrombin III deficiency. Twenty-nine patients underwent surgical reconstruction with 31 bypass grafts: spiral saphenous vein (n = 20), superficial femoral vein (n = 4), human allograft (n = 1), or expanded polytetrafluoroethylene (ePTFE, n = 6). Eleven patients underwent percutaneous transluminal angioplasty or stenting; 3 primary and 8 secondary endovascular procedures were performed to treat graft stenosis (n = 7) or occlusion (n = 1). There were no early deaths. Five early graft failures in 3 ePTFE grafts and 2 bifurcated vein grafts (thrombosis, n = 4; stenosis, n = 1) were successfully treated with open surgical revision. Over a mean follow-up of 5.6 years (range, 0.4-16.6 years) in surgical patients, 17 additional secondary interventions were performed in 8 patients, 14 endovascular and 3 surgical. Primary, assisted primary, and secondary patency rates of surgical bypass grafts were 63%, 79%, and 85%, respectively, at 1 year, and 53%, 68%, and 80%, respectively, at 5 years. Graft patency was significantly higher in vein grafts compared with ePTFE grafts (P =.02). Mean follow-up after percutaneous transluminal angioplasty or stenting was 3.1 years (range, 1 day-11.7 years). Twelve secondary endovascular interventions were performed in 6 patients (primary group, 3 of 3; secondary group, 3 of 9 grafts in 8 patients) to maintain patency in 11 of 12 reconstructions. Mean follow-up in the entire patient cohort was 5.3 years (range, 0.4-16.6 years). In 79% of patients symptoms had resolved or were significantly improved at last follow-up. Surgical treatment of benign SVC syndrome is effective over the long term, with secondary endovascular interventions to maintain graft patency. Straight spiral saphenous vein graft remains the conduit of choice for surgical reconstruction, with results superior to those with bifurcated vein and ePTFE. Endovascular treatment is effective over the short term, with frequent need for repeat interventions. It does not adversely affect future open surgical reconstruction and may prove to be a reasonable primary intervention in selected patients. Patients who are not suitable for or who fail endovascular intervention merit open surgical reconstruction.
  • Article
    Superior vena cava syndrome (SVCS) is associated to a malignant tumor in more than 90% of cases; being the lung cancer the most frequent (80%). SVCS has a benign cause in less than 5% of cases. Endovascular stenting has been proposed as the primary treatment of choice. We report our experience in SVC recanalization through the use of self-expanding vascular stents as treatment of life-threatening SVCS of benign and malignant etiology. Between January 1994 and April 2002 44 patients with critical SVCS, were treated at the Hospital Italiano de Buenos Aires. Forty nine self-expanding endovascular metallic stents were percutaneously placed in the SVC. Thirty-one (70%) patients were male and 13 (30%) were female. The mean age was 55.6 years (range: 21-77). The etiology of SVCS was malignant in 40 cases and benign in 4. The malignant causes included lung cancer: 37 (37/44 - 92.5%), lymphoma: 1 (2.5%), chondrosarcoma 1 (2.5%), melanoma 1 (2.5%). The benign etiology corresponded to central catheters (N: 2) and post-radiation fibrosis (N: 2). Cavography showed complete occlusion of SVC in 12 cases (27%) and significant partial stenosis in 32 cases (73%). Thrombi associated with tumor stenosis were present in 25 (57%) patients. All procedures were technically successful. No stent migration was observed. Thirty-two patients with malignant tumor ultimately died due to the progression of the disease. Mean survival time was 193 days (range: 25-578). SVCS recurrence was observed on six occasions. In four patients a new stent was placed. Symptomatic improvement was dramatically seen within 24-48 h after stent placement in 40 patients (90.9%) and 83.3% out of the cases (38/44) were symptoms-free during the rest of the disease. Three patients died in the 7 following days. The use of self-expanding vascular endoprostheses in the recanalization treatment of SVC in SVCS due to a malignant or benign etiology offers excellent results with rapid and prolonged remission of symptoms.
  • Article
    Stenting is a relatively new option in the management of superior vena cava obstruction (SVCO), but available data often concern non-malignant and/or various malignant diseases. The aim of this study was to assess the efficacy of vascular stenting as a first-choice treatment in SVCO in the exclusive setting of NSCLC. Retrospective study of NSCLC patients with SVCO treated in the past year. Demographic data, disease characteristics, etiologic and palliative treatment (use of vascular stenting) were recorded as well as treatment outcome and survival. 17 patients were recruited. Eight had vascular stenting while 9 did not. Except for stenting, there was no difference between the two groups (median age 54 years; 80% men; 53% stage IIIB and 47% stage IV). Stenting (median length 60 mm) achieved complete resolution of SVCO more frequently (75 vs. 25%, p = 0.05) and faster (2 vs. 21 days, p = 0.002) without immediate or delayed complication. All patients with stents received anticoagulation therapy. Relapse rate after complete response (33 g, 50%, p = 0.6) was lower and time to relapse (6.5 g, 2 months) was longer for patients undergoing stenting, without reaching statistical significance. Median overall survival was not statistically different (8 and 5 months, p = 0.06). This study demonstrated the effectiveness of vascular stenting for SVCO in NSCLC patients. The high response rate, quick effect and safety of vascular stenting make this palliative treatment a candidate as a potential standard procedure. The results, however, must be confirmed in a prospective randomized trial including quality of life assessment.
  • Article
    The diagnosis of superior vena cava obstruction is based on demonstration of venous collateral circulation. Computed tomography (CT) is the best imaging modality for this disease. Nuclear medicine is also helpful in showing caval flow compromise through scintigraphic venography and abnormal radiotracer accumulation from developed venous collaterals. The authors present a case of superior vena cava obstruction with the two less common venous collateral pathways described in the imaging literature: intrahepatic and right-to-left shunts. These shunts are demonstrated by technetium-99m MAA lung scintigraphy with CT and venographic correlation. Characteristics of these two shunts are discussed with a brief review of the literature.
  • Article
    Fibrosing mediastinitis is a rare, chronic inflammatory process that can cause superior vena cava syndrome, and can mimic malignancy. We present two cases of this disease where surgical resection was not possible and review the treatment options.
  • Article
    Superior vena cava syndrome (SVCS) is a frequent presentation of malignancies involving the mediastinum and can seriously compromise treatment options and prognosis. Stenting of superior vena cava is a well-known but not so commonly used technique to alleviate this syndrome. Between August 1993 and December 2000 we performed 52 stenting procedures in patients affected by non-small cell lung cancer (NSCLC). Phlebographic resolution of the obstruction was achieved in 100% of cases with symptomatic and subjective improvement in more than 80%. One major complication was observed due to bleeding during anticoagulation. Re-obstruction of the stent occurred in only 17% of the cases, the majority due to disease progression. Improvement of the syndrome allowed hydration necessary for full dose platinum treatment when indicated in patients affected by lung cancer. Stenting of the superior vena cava syndrome is a safe and effective procedure achieving a rapid alleviation of symptoms in almost all patients, and allowing for full dose treatment in lung cancer patients. This procedure could change the traditional poorer prognosis attributed to non-small cell lung cancer patients presenting with this syndrome.