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• W2400100451 abstract "Original ArticlesSuperior Vena Caval Obstruction: King Khalid University Hospital Experience Dahish S. AjarimMD, FRCP(C) Dahish S. Ajarim Address reprints and correspondence to Dr. Ajarim: Department of Medicine (38), King Khalid University Hospital, P.O. Box 7805, Riyadh 11472, Saudi Arabia. From the Department of Medicine, King Khalid University Hospital, Riyadh. Search for more papers by this author Published Online:1 Jan 1992https://doi.org/10.5144/0256-4947.1992.52SectionsPDF ToolsAdd to favoritesDownload citationTrack citations ShareShare onFacebookTwitterLinked InRedditEmail AboutABSTRACTABSTRACTA five year experience, in 21 patients with superior vena caval obstruction (SVCO) was reviewed. SVCO was due to benign conditions in four patients (19%) and malignant tumors in 17 patients (81%). Lymphoma and lung cancer were the most common causes encountered. The most common symptoms and signs were facial swelling, shortness of breath, jugular venous distention, swelling of face and arms, and engorgement of thoracic veins. Benign disorders had a longer duration of symptoms before presentation and required longer time to make the diagnosis than in malignant disorders. No serious complications resulted from the superior vena cava obstruction itself or the investigative procedures leading to the diagnosis. Prognosis and response to treatment were dependent on the underlying cause of SVCO. Chemotherapy was effective in induction of complete regression of SVCO in the majority of patients with underlying malignant disorders. We conclude that SVCO should be approached invasively for diagnosis and tissue diagnosis of the underlying disorder should dictate the appropriate therapy.IntroductionThe superior vena cava (SVC) is the major route for blood return from the upper torso, arms, and head and its anatomical position makes it particularly vulnerable to obstruction because it has a thin wall surrounded by lymph node chains adjacent to the main bronchus and is enclosed in a tight compartment formed by the mediastinum and sternum. Symptoms of superior vena cava obstruction (SVCO) are secondary to systemic congestion and increased pressure. The first case of SVCO was described in 1757 by Hunter [1] as a complication of an aortic aneurysm. But since then, many etiologies for this syndrome have been reported.Over the years, the predominant etiology of this syndrome has changed from benign vascular, fibro tic, and infectious disease to malignant neoplasia such as lung cancer and lymphoma.[2–4] However, approximately 10% to 25% of patients with SVCO presenting in general hospitals have a benign cause of this condition [5–7]. In a recent series reported from radiotherapy and cancer clinics [8, 9], non-malignant diseases as a cause are much less common (0% to 3%).Superior vena caval obstruction syndrome due to cancer has been considered for many decades to bea medical oncological emergency requiring urgent treatment by radiation [8]. However, there is evidence demonstrating that SVCO is not a true emergency [10, 11] and that a histological diagnosis can be obtained without increased risk when biopsy specimens are obtained by skilled surgeons [12].The aim of this report is to review our experience with SVCO at King Khalid University Hospital (KKUH) in Riyadh during the last five years with respect to the clinical presentation, duration of symptoms, etiology, diagnostic procedures and its complications and the overall response to therapy and survival period.PATIENTS AND METHODSThe clinical records of adult patients with SVC obstruction seen and treated at KKUH in Riyadh between September 1985 and August 1990 were reviewed. A clinical diagnosis of SVCO was accepted if there was documentation of venous distention of the superior cava or its tributaries, and edema of the face, neck, arms, or upper torso in the presence of a lung or mediastinal mass and/or there was positive contrast of radionuclide vena cava.Patients were excluded if they did not have documented clinical findings compatible with SVCO or if treatment was given before admission to this hospital. Patients' medical records were reviewed to obtain data about symptoms and signs of SVCO at presentation and its duration, the interval from first medical contact until diagnosis of underlying disorders was made, diagnostic procedures and its complications, radiological investigations, etiology, treatment response and median survival of patients with SVCO. Survival was measured from the date of diagnosis until death and for malignant cases we considered maximum duration of follow-up as the death time if time of death was not known.RESULTSA total of 24 patients were identified for review. Of these, three were excluded from analysis, two because of inadequate clinical documentation of SVCO and one because invasive procedures and treatment were performed elsewhere prior to transfer to our hospital.Presenting symptoms and signs of superior vena caval obstruction for 21 patients are summarized in Table 1. Predominant symptoms for SVCO were facial swelling, shortness of breath, cough and headache. The predominant signs were related to upper compartment venous distention and tissue swelling, neck vein distention, facial swelling and dilated veins of the upper thorax. The median interval from the patient's first recollection of symptoms or signs before seeking medical advice was 24 days (range, 10 to 30 days) for cases with underlying malignant disorders. In contrast, this interval was 105 days (range, 30 to 65) for benign disorders. The median interval from first medical contact until diagnosis of underlying diseases was made ranged from 2 to 20 days (median, seven days) for malignant cases and seven to 210 days (median, 48 days) in benign diseases.Table 1. Presenting symptoms and signs of superior vena caval obstruction in 21 patients.Table 1. Presenting symptoms and signs of superior vena caval obstruction in 21 patients.Etiologies of superior vena caval obstruction are summarized in Table 2. Etiology of SVCO determined as benign disorders was noted in four patients (19%), including thrombosis in three patients and idiopathic mediastinal fibrosis in one patient. The thrombotic case included one patient with Behcet's disease, a case with thrombosis secondary to chronic indwelling catheter and one case of idiopathic thrombosis.Table 2. Etiology of superior vena caval obstruction: our experience compared with other studies.Table 2. Etiology of superior vena caval obstruction: our experience compared with other studies.Etiology was due to malignancy in 17 patients (81%), including five patients with non-small cell lung cancer, two patients with small cell lung cancer, lymphoma in seven patients, and metastatic tumors in three patients (soft tissue sarcoma 1, seminoma 1, histocytosis x 1).Radiological investigation revealed abnormal chest roentograms in all patients with malignant disorders. In contrast all cases with benign disorders had normal chest x-rays. The tumor was located in the right upper lobe in five patients (29%), mediastinum in nine patients (53%), in both locations in three patients (18%) and pleural effusion present in 24% of cases. Contrast venogram was performed in four benign cases and in two cases having malignant disease with no complication. Contrast venogram demonstrated complete occlusion with presence of collateral obstruction in four cases and partial obstruction in one case. Radionuclide scan was done in one case and produced a false-negative result.Diagnostic procedures are summarized in Table 3. Twenty-three invasive procedures were performed. Definite tissue diagnosis was made in 19 patients (17 with malignant disorders, two with benign disorders). No report of prolonged bleeding episodes or fatalities were noted during any procedure or following venipuncture. Two patients underwent mediastinotomy and one patient had exploratory thoracotomy with no substantial blood loss. All patients were extubated within 12 hours.Table 3. Diagnostic procedures in patients with superior vena caval obstruction.Table 3. Diagnostic procedures in patients with superior vena caval obstruction.Of the 21 patients all but three received some type of therapy directed to the superior vena caval obstruction. The treatment was based on the underlying disorders (Table 4). Three out of five patients with non-small cell lung cancer were treated with radiation, 3000 rads in ten fractions, and their symptoms were relieved. The median survival for this group of patients was poor only two months (range, one to four months). Twelve patients were treated with chemotherapy (lymphoma 7, small cell lung cancer 2, germ cell tumors 1, sarcoma 1, histocytosis x 1).Table 4. Results of therapy for SVCO and survival.Table 4. Results of therapy for SVCO and survival.Drugs were chosen according to the underlying malignancy and administered through a freely running intravenous catheter placed in the lower extremities. All of these patients had prompt relief of SVCO symptoms within a median interval of three days (range, one to seven days). The median survival rate for this group of patients was 16 months (range, seven to 60 months). One patient with idiopathic mediastinal fibrosis underwent percutaneous transluminal balloon angioplasty with no response and eventually-underwent bypass surgery of superior vena cava in another hospital with good response. Another patient underwent exploratory thoracotomy and was found to have extensive SVC thrombosis; bypass surgery was not possible. One patient with thrombosis secondary to Hickman's line was treated with an anticoagulant after removal of the line. He had a prompt response but died three months later from the underlying cause. One patient with Behcet's disease had no significant distressing symptoms and no specific treatment was offered except for the underlying disease.DISCUSSIONObstruction of the superior vena cava is mimicked by a few other organic problems. Congestion and edema due to cardiac tamponade, constrictive pericarditis and allergic reactions are occasionally confused with SVCO. Based on the symptoms and clinical findings alone, the diagnosis of superior vena caval obstruction can be made easily when the syndrome has been well established.The true incidence of SVCO may be under recorded in terms of signout diagnoses. This may merely reflect a lack of interest in recording SVCO in terminal cancer patients.The most common symptoms and signs in our study at presentation was facial swelling, shortness of breath, jugular venous distention, swelling of the face and arms, and engorgement of thoracic veins. These are similar to what has been reported by Perez et al [8].The variable which best distinguishes benign from malignant underlying disease is the duration of symptoms prior to presentation. As shown in our experience, and that of others [3,11], it takes a longer time to seek medical advice in benign diseases and this may reflect a rate of occlusion of the vein more rapidly in malignant diseases in comparison with gradual fibrotic compression in benign conditions. Also, the time taken to arrive at a tissue diagnosis correlated with benign causes, a finding which probably follows from the relative ease with which carcinoma is diagnosed as compared with benign disease is frequently a diagnosis made by exclusion. Usually the pulmonary symptoms, signs and chest x-ray abnormalities are absent in benign disorders. We recommended that contrast or radionuclide venocavagram be done only in borderline cases or when surgical intervention is planned.The distribution of etiologies of superior vena caval obstruction in this study, particularly a malignant to benign ratio of 4.25 to 1, is similar to other studies [3–7,11,13] in the literature reported from general hospital populations. In our experience, the underlying malignant disorder was found to be lymphoma (33%), small cell lung cancer (10%), and non-small cell cancer (24%).The lymphoma percentage among the malignant underlying causes of SVCO was higher than what has been reported in many Western studies [11, 13] where bronchogenic carcinoma accounts for approximately 80% of cases. Lymphoma was found in only 8% to 12% of malignant underlying disorders. This could be attributed to the higher relative frequency of lymphomas among Saudi patients, as has been shown in many published epidemiological studies [14–16] about cancer in Saudi Arabia. All benign underlying disorders of SVCO in our report have been reported in previous reports [11, 17].The conventional emergency treatment for SVC obstruction has been radiation therapy, often started prior to the establishment of a definitive histological diagnosis [9,13,18–20], but the current opposing views [8,11,21] do not agree with this approach. Accurate histological diagnosis may be difficult after the initiation of radiotherapy treatment. Furthermore, SVCO is rarely an emergency because patients with SVCO secondary to non-malignant causes have survived as long as 28 years [17]. The ominous nature of SVCO in patients with oncological emergencies is not related to the obstruction in itself, but rather to the underlying malignancy. A definite diagnosis is important since future therapy and prognosis is influenced by the histological nature and anatomical site of the primary lesion. Currently, we have an effective systemic chemotherapy for small cell lung cancer, lymphoma, and germ cell tumor which are common causes of SVCO as shown in our report.It has already been shown in many series [4,22–25] that the combination of chemotherapy is effective as a single modality for resolving symptoms of superior vena caval obstruction.Regression of symptoms and signs of SVCO was observed in all 12 patients who received chemotherapy as induction treatment and within a short period (median interval, three days). This is in accordance with other reports [22–25]. Although the necessity of immediate therapy for superior vena caval obstruction has not been supported by the occurrence of serious neurological complications due to this syndrome, other serious complications of mediastinal entrapment or complications due to progression and spread of the underlying malignancy may justifiably demand urgent therapy, but only after appropriate urgent diagnostic procedures have been carried out. In 21 patients presenting with SVCO without prior diagnosis, we were able to establish etiologies using different diagnostic procedures with no significant reported complications due to procedures, anesthesia or the underlying disease itself. Exact details of tissue diagnosis will take some time to be worked out, but to know whether the tumor is chemosensitive or not may be obtained within a few hours on completion of the procedures.We think survival is mainly related to the nature of the underlying disease and not related to the presence or absence of SVCO. We had few patients available to evaluate the prognostic importance of SVCO, however, it has been shown that there is no significant importance in small cell cancer [24, 25].Surgical bypass of superior vena cava [26, 27] or percutaneous transluminal balloon angioplasty[28] have been shown to have some rules in management of SVCO due to benign or malignant diseases. However, symptomatic therapy using corticosteroids and diuretics has never been demonstrated to be beneficial and therefore was not used in our patients.We conclude that SVCO should be approached diagnostically before initiation of induction treatment. In our experience, an invasive diagnostic workup in these patients is possible without excessive risk. We have confirmed that chemotherapy is an effective induction treatment in patients with SVCO due to chemosensitive tumors and such patients should have a systemic treatment approach rather than local radiation treatment.ARTICLE REFERENCES:1. Hunter W. 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Walpole HT, Lovett KE, Chuang VP, West R, Clements SD. Superior vena cava syndrome treated by percutaneous transluminal balloon angioplasty . Am Heart J. 1988; 115(6):1303–4. Google Scholar Previous article Next article FiguresReferencesRelatedDetails Volume 12, Issue 1January 1992 Metrics History Accepted9 February 1991Published online1 January 1992 ACKNOWLEDGMENTSI would like to thank Ms. Zeny D. Alcid for her secretarial help and Professor Hassan Abu-Aisha, Professor Banji Ayoola, Dr. Mohammed Osman Mekki, and Professor G. Esan for reviewing the manuscript.InformationCopyright © 1992, Annals of Saudi MedicinePDF download" @default.
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