Cause Of Superior Vena Cava Obstruction Research Articles

Recurrent Hematemesis From Downhill Esophageal Varices: A Therapeutic Challenge for Gastroenterologists

Downhill or upper esophageal varices have an etiology that differs from that of the “uphill” varices secondary to portal hypertension. Approximately 0.1% of all cases of variceal hemorrhage are due to downhill varices. The underlying etiology is obstruction of the superior vena cava (SVC) which results in the shunting of blood from the systemic circulation into the esophageal plexus, predominantly the upper two-thirds. The management should be directed to relieve the vascular obstruction. One of the causes of SVC obstruction leading to downhill variceal bleeding is dialysis catheter-associated SVC stenosis. We report the case of a 34-year-old male with hematemesis associated with downhill varices due to chronic SVC obstruction because of a central venous catheter.

Esophageal Varices: Itʼs Not Always an Uphill Battle

Downhill (proximal) esophageal varices (DEV) are a separate and distinct entity from the more common uphill (distal) esophageal varices. DEV are produced when an obstruction of the superior vena cava (SVC) leads to a downward flow of the venous system towards the portal vein. DEV are rarely seen as the cause of an acute upper gastrointestinal bleed and account for about 0.1% of all cases of variceal bleed. While there are many causes of SVC obstruction, catheter-associated SVC stenosis is the most frequently reported etiology in patients presenting with an acute variceal bleed. A 66 year old man with history of end stage renal disease on dialysis complicated by difficult access status post right upper extremity hemodialysis reliable outflow (HeRO) graft presented with melena and hemoglobin of 5.3 gm/dL. He underwent urgent endoscopy that demonstrated 3 columns of large proximal esophageal varices originating from the proximal esophagus and extending distally, one with nipple sign indicative of recent bleeding that was successfully banded. Abdominal ultrasound showed normal liver size and echogenicity. Hepatic enzymes were within normal limits (AST 24 U/L, ALT 25 U/L, and ALP 122 U/L). CT chest revealed no evidence of portal hypertension, however extensive chest wall venous collaterals were noted concerning for central venous stenosis. Vascular surgery was consulted for HeRO graft removal prior to IR stenting of SVC stenosis with no further evidence of bleeding. DEV are a rare form of varices seen as a result of SVC occlusion. Obstruction of the SVC shunts blood from systemic circulation into the esophageal plexus, predominantly the upper two-thirds. Bleeding DEV are most frequently noted in patients with central venous catheters. This is only the second case reported of a patient with SVC stenosis due to HeRO graft leading to DEV. There are no definitive recommendations on how to manage DEV, but addressing the underlying obstruction is the mainstay in treatment. There has been no data to address the use of somatastatin analogues for the use of bleeding DEV, although they would likely be of little benefit as the underlying etiology of DEV is not related to portal hypertension. Endoscopy and banding however do have a role in temporizing active bleeding. There is also currently no evidence on the use of beta-blockers for secondary prevention as relieving the obstruction should be a priority to prevent further bleeding.1810_A Figure 1. Gastroesophageal junction1810_B Figure 2. Proximal to mid-esophagus1810_C Figure 3. Esophageal varices post banding

Surgical Treatment of Benign Superior Vena Cava Syndrome

The obstruction of blood flow through the superior vena cava (SVC) into the right atrium may present as a severe clinical syndrome. One of the benign causes of SVC obstruction is the long-term use of indwelling catheters and wires, increasing the chances of SVC thrombosis. The treatment of the benign SVC syndrome is focused on achieving long-term durability and patency of the superior venocaval system and normal life expectancy. We report the successful surgical management of a patient with severe symptomatic SVC syndrome and emphasize technical details that might be of value in treating this challenging pathologic condition.

Mid- and long-term follow-up experience in patients with malignant superior vena cava obstruction

Superior vena cava obstruction (SVCO) due to mitotic diseases is a serious condition with significant morbidity and mortality. The aim of this study was to examine the follow-up data and demographics of patients with SVCO admitted to the Department of Surgery, Queen Mary Hospital, over a 14-year period. The prospectively entered clinical data of patients with SVCO in Queen Mary Hospital from October 1997 to September 2011 were retrospectively analysed. All patient records were electronically and manually searched. Survival was calculated using Kaplan-Meier survival curves analysis. The Mantel-Cox log-rank test was used to test for statistically significant differences. Demographic data, associated aetiology, intervention and outcome were studied. Only patients with malignant aetiologies were included. A total of 104 patients (81 males and 23 females) were recruited in our study period. Median age at presentation was 65 (range 3-91 years). The median follow-up period was 2 months. The commonest cause of SVCO was bronchogenic carcinoma (71%), followed by extrathoracic malignancies (16%), lymphoma (8%) and thymic malignancy (3%). The mean time from the onset of symptoms to presentation was 34 days. Steroids were prescribed for most (93.9%) of the patients. About half (54.4%) of the patients were given radiotherapy. Only 7 patients had angioplasty and all of them had stents inserted. The overall survival was poor. The mean and median survivals were 8.4 and 1.6 months, respectively. Seventeen percent of patients died in the same hospitalization as for their initial presentations. Younger age (50 years or below; P = 0.000), never smoker (P = 0.012), not using steroids (P = 0.007) and certain primary aetiologies (e.g. lymphoma; P = 0.008) were associated with longer overall survival on univariate analysis. However, on multivariate analysis, none of these factors reached statistical significance. The mean survival for cases with lymphoma, extrathoracic malignancies, bronchogenic tumours and thymic tumours was 80.1, 3.4, 3.1 and 1.8 months, respectively. Angioplasty did not show a statistically significant association with the overall survival. This study, to the best of our knowledge, is the first to study the prognostic factors that may affect survival outcome in malignant SVCO. We showed that in patients with malignant aetiology for SVCO, advanced age (more than 50), history of smoking and use of steroids were statistically significantly associated with a poor outcome. The underlying primary malignant aetiology also has an important prognostic significance. Despite advances in medicine, the prognosis of patients with SVCO is still grave.

Prevalence and Etiology of Focal Liver Opacification in Patients With Superior Vena Cava Obstruction

This study aimed to assess the prevalence and etiologies of focal liver opacification (FLO) in the setting of superior vena cava (SVC) obstruction. An archival search using key words to identify patients with SVC obstruction or severe narrowing and who had computed tomographic scans with intravenous contrast was performed at our institution. Thirty-one patients were included. Focal liver opacification was identified in 9 (29%). The most common direct causes of FLO and SVC obstruction were benign. Focal liver opacification caused by SVC obstruction is relatively common. Focal liver opacification is more commonly associated with benign causes of obstruction such as end-stage renal disease. Identifying FLO is important not only as an indirect sign of SVC obstruction but also must be distinguished from avidly enhancing liver masses. This study also reflects the overall recent increase in benign causes of SVC obstruction.

“Downhill”ʼ Esophageal Varices: Unusual Cause of Dysphagia

Introduction: “Downhill” varices refer to proximal esophageal varices which typically result from superior vena cava (SVC) obstruction resulting reversal of blood flow in the upper esophageal venous plexus. The commonest etiologies of SVC obstruction include lung and thyroid carcinoma, mediastinal fibrosis and metastatic disease. Complication of central venous catheter is becoming a significant cause of SVC obstruction. In contrast to “uphill” varices associated with portal hypertension, downhill varices are usually asymptomatic with only few cases of bleeding reported. Case Description: A 48-year-old African American male with past medical history of AIDS on HAART, ESRD on hemodialysis (for 4 years), hypothyroidism and hypertension presented with progressively worsening dysphagia of 4 weeks duration. The dysphagia is mainly to solid food, which gets stuck at the base of the neck and behind his sternum. This has resulted in loss of appetite and 15 lb unintentional weight loss. He denies pain on swallowing, abdominal pain, nausea, vomiting, regurgitation or hematemesis. He also complained of left upper arm swelling. Physical exam was significant for diffuse left upper arm swelling and left arm arteriovenous graft (AVG). No oral lesions or thrash were seen. Laboratory studies are unremarkable except CD4 count of 131cells/mm3. Endoscopy (EGD) showed several columns of small-medium varicosities in proximal-mid esophagus with normal distal esophagus. No strictures, mass, adherent lesions were seen and biopsy result was unremarkable. The finding is consistent with “downhill” esophageal varices of the upper 1/3 of the esophagus, a condition commonly seen with SVC stenosis. Neck and chest and CT were unremarkable. Fistulogram and superior venacavogram showed patent AV- fistula but narrowed SVC. The SVC had stenotic segment measuring 9.9 mm in diameter above the point of entry of the azygous vein with extensive collateral formation. Angioplasty dilated the narrowing to 13 mm diameter which led to resolution of left arm swelling and dysphagia. Repeat EDG a week later revealed near complete resolution of the downhill varices. The patient continued to improve clinically during his hospital stay and started to eat without difficulty. Discussion: Although bleeding from downhill varices is well described, to our knowledge no case of downhill varices presenting with dysphagia is reported. Our patient's SVC stenosis was a complication of a prior indwelling hemodialysis catheter he had for two years before placement of AV fistula graft. The management is aimed at correcting the cause of the obstruction through angioplasty or stent placement as in our case.

Superior vena cava obstruction caused by ascending aortic pseudoaneurysm as assessed by multi-detector row computed tomography

We describe a case of a 64-year-old man with superior vena cava (SVC) obstruction caused by a huge pseudoaneurysm of the ascending aorta. Pseudoaneurysm of the ascending aorta is an uncommon cause of SVC obstruction. Multi-detector row computed tomography (MDCT) simultaneously clarified the SVC obstruction and coronary artery stenosis.

A primary cardiac sarcoma presenting with superior vena cava obstruction

Superior vena cava (SVC) obstruction leads to a constellation of symptoms and signs that encompass the SVC syndrome. Today, malignancy accounts for 65% of all cases. The most common neoplastic causes are non–small cell lung cancer (50%), small cell lung cancer (25%), lymphoma, and metastasis. Primary cardiac tumors are an extremely rare cause of SVC obstruction. We describe the case of a 48-year-old man who presented with dyspnea, confusion, and facial swelling with cyanosis. The patient developed life-threatening airway obstruction after administration of anxiolytic. The diagnosis of SVC obstruction secondary to a primary cardiac sarcoma was established based on clinical, radiologic, and post-mortem findings. This is one of very few reported cases of a primary cardiac sarcoma causing SVC obstruction.

SUPERIOR VENA CAVA SYNDROME MASKING B-CELL LYMPHOMA: A LIKELY SEQUELA OF THE CHERNOBYL NUCLEAR DISASTER.

Partial or complete obstruction of the thin-walled superior vena cava (SVC) is a relatively rare condition. The patients may present with swelling of the neck and face, headache, dizziness, visual disturbances, stupor, and syncope. The cutaneous veins of the upper chest and lower neck may become dilated and flushing of the face and neck may develop. The most frequent causes of SVC obstruction are divided into two categories: (1) non-neoplastic (mediastinitis, constrictive pericarditis, histoplasmosis, etc) and (2) neoplastic (superior mediastinal tumors). B-cell lymphoma, a subtype of non Hodgkin9s lymphoma, is one of the mediastinal tumors that can cause SVC obstruction. Most common symptoms of B-cell lymphoma are fever with night sweats, weight loss, and fatigue, but rarely it may give a unique presentation of SVC syndrome. A 41-year-old Ukrainian female presented with the history of swelling of the face, neck, left arm, left hemithorax, facial flushing, and generalized body aches. Her past medical history was significant for exposure to radiation during the Chernobyl nuclear disaster. On physical examination, the patient had jugular venous distention with dilated anterior chest wall veins. Chest radiography revealed a widened mediastinum. CT scan of the chest revealed an anterior mediastinal mass with compression of the proximal SVC and left innominate vein. A mediastinotomy was performed and a biopsy of the mass and bone marrow revealed a large B-cell lymphoma infiltrating the bone marrow. The patient was given eight cycles of CHOP therapy (cyclophosphamide, Adriamycin, vincristine, prednisone) with rituximab. With this treatment, the patient went into remission. The etiology of B-cell lymphoma has been considered multifactorial, and exposure to radiation has also been hypothesized as one of the causes. Ionizing radiation exposure may result in multiple neoplastic diseases, including leukemia and cancers of the skin, breast, lung, and thyroid. Although there is no definite etiology, it is very likely in this case that radiation exposure during the Chernobyl nuclear disaster resulted in the B-cell lymphoma in this patient, leading to a unique presentation of SVC syndrome. If a timely diagnosis is made and combination chemotherapy instituted, patients may achieve complete remission and long-term survival is possible.

Endovascular strategies for superior vena cava obstruction

The symptoms of superior vena cava (SVC) obstruction can be extremely debilitating, and current medical and surgical treatments have been only partially successful in providing relief to afflicted patients. Endovascular techniques of venous recanalization have recently shown excellent short-term and mid-term success in patients with both benign and malignant causes of SVC obstruction. Endovascular therapy may be reasonably used as a first-line approach to the palliation of malignant SVC obstruction, and its mid-term patency rates compare favorably with those of modern surgical methods of venous bypass in patients with benign SVC obstruction. In this article, the percutaneous management: of SVC syndrome is discussed, with particular attention to the anatomical considerations and clinical priorities that impact endovascular decision making.

Le syndrome cave supérieur de l'adulte en Tunisie centrale. `A propos de 26 cas

We report 26 cases of superior vena cava obstruction (SVC) observed in Sousse University Hospital (TUNISIA). The diagnosis was confirmed by CT scan and/or superior vena cava angiography. The underlying disease was revealed by SVC on 76.9% of cases. Malignant underlying disease was noted on 61.53% of cases. Non malignant causes of SVC was Behçet disease (4 cases) and mediastinal fibrosis (5 cases). SVC revealed Behçet disease in the 4 cases.