Portal and mesenteric vein and inferior vena cava thrombosis associated with antiphospholipid syndrome

We report a 48-year-old man with thrombosis of the portal and superior mesenteric vein and inferior vena cava associated with primary antiphospholipid syndrome (APS). Primary APS was diagnosed by a positive reaction with anticardiolipin antibody (aCL) and the absence of any evidence suggesting the presence of other disease states known to be associated with aCL. A coeliac angiography showed obstruction of the portal and superior mesenteric vein with prominent collaterals and cavernous transformation. Femoral vein angiography showed total obstruction of the external iliac vein and inferior vena cava, and dilation of the pelvic veins, with contrast medium in the lumbar vein. This case is noteworthy as a report of primary APS accompanied by extensive abdominal and pelvic venous thrombosis.     

Epidural hematoma mimicking transverse myelitis in a patient with primary antiphospholipid syndrome

Antiphospholipid syndrome (APS) is an autoimmune disease, and its most critical pathologic process is thrombosis, which may explain most of the clinical features. Acute management of thrombosis involves immediate anticoagulation. Acute proximal venous thrombosis can be managed with thrombolytic therapy to reduce the long-term complications of the postthrombotic syndrome (pain, swelling, skin discoloration, or ulceration) and perform recanalization of occluded vessels. However, thrombolytic therapies are associated with high risks of bleeding. To our knowledge, this is the first report of epidural hematoma mimicking transverse myelitis after catheter-directed thrombolysis in a patient with primary APS. A 42-year-old male was admitted with sudden onset pain and swelling on left lower extremity. Venography demonstrated multiple thrombi on superficial femoral vein, common femoral vein, common iliac vein, and external iliac vein. Laboratory tests indicated the presence of IgM anticardiolipin antibody. He was diagnosed with primary APS with multiple venous thrombi. He was treated with urokinase (200,000/h) as thrombolytic therapy. After 1 day, he complained both leg weakness and urinary dysfunction. T1- and T2-weighted magnetic resonance images of spine showed about 8 cm-sized mass, suggesting hematoma on the posterior epidural space at thoracolumbar area. Despite the successful evacuation of hematoma, neurologic symptoms persisted and he is now receiving aspirin, warfarin, and physical therapy.     

Recurrent pulmonary embolism from left subclavian thrombosis: a case report

A 38-year-old woman was admitted to our hospital because of pulmonary thromboembolism. Thrombolysis therapy resulted in initial improvement in symptoms and laboratory data. However, 4 months later, pulmonary thromboembolism recurred despite antiplatelet and anticoagulation therapy. Contrast venography and venous ultrasonography of both upper and lower extremities revealed subtotal occlusion and venous thrombosis of the left subclavian vein with collateral vessels, but no evidence of lower extremity venous thrombosis. She had no history of subclavian venous catheterization, neoplasm, hypercoagulability or other predisposing cause of thrombus formation. Operative ligation of the left subclavian vein was performed at the junction with the internal jugular vein. White thrombus was identified within the venous lumen. She was well without recurrent pulmonary thromboembolism or venous insufficiency for 10 months after the operation. Surgical interruption of the subclavian vein may be effective to prevent recurrent pulmonary thromboembolism in patients with recurrent pulmonary thromboembolism due to venous thrombosis of the upper extremity despite therapeutic anticoagulation.     

Stent implantation for iliac compression syndrome with acute deep vein thrombosis: a case report

An 80-year-old woman presented with left lower limb pain and swelling with tenderness over the great saphenous vein. Venography revealed thrombus in the lower leg vein and occlusion of the femoral vein. A temporary vein filter was placed below the renal vein and catheter intervention was performed. The wire was carefully advanced from the left femoral vein to the vena cava. Hard resistance was felt at the proximal iliac vein. After balloon dilation, intravascular ultrasonography showed a very flat lumen at the occlusion site. Stent implantation was performed followed by anticoagulation therapy. Venography at 3 months follow-up showed a widely opened iliac vein. Angiography showed the occlusion site was the crossing point of the right iliac artery and left iliac vein. Stenting for iliac compression syndrome is effective to maintain patency of the vein.     

Deep venous thrombosis of the upper extremity associated with use of the Swan-Ganz catheter

Two patients who developed unilateral neck vein distention following insertion of a Swan-Ganz catheter are described. One patient developed unilateral swelling of the arm used for catheter insertion. Following removal of the Swan-Ganz catheter in this patient, venograms of both upper extremities revealed internal jugular vein thrombosis and subclavian vein thrombosis of the involved extremity. The other patient had thrombosis of the internal jugular veins and subclavian veins bilaterally, as well as superior vena cava thrombosis.     

Central venous catheter-associated thrombosis in severe haemophilia

Significant subclavian vein thromboses associated with indwelling fully implanted (port-a-cath) devices are described in two boys with severe haemophilia A and factor VIII inhibitors. Investigations were prompted by prominent chest wall veins in one case, whereas the thrombosis was a chance finding in the other case during investigation of mechanical dislocation of the catheter tubing. Extensive collateral venous circulations were demonstrated by venography in both instances indicating that the thrombus had been present for some time. Possible contributing factors to the thromboses included desensitization therapy (both patients), high-dose FEIBA (in one patient) and use of lower doses of heparin for line flush than that recommended by some authors. Neither patient had a familial or non-familial predisposition to thrombosis.     

A systemic lupus erythematosus patient with multiple aseptic bone necroses, thrombosis of superior mesenteric artery and anti-phospholipid antibody]

A 38 year old woman with systemic lupus erythematosus (SLE) was admitted because of epigastralgia and fever. The diagnosis of SLE was made 22 years ago based on Raynaud's phenomenon, butterfly rash, hair loss, photosensitivity and positive antinuclear antibody. She had episodes of consciousness disturbance, transient visual disturbance of the left eye, and a necrosis of the left big toe. She underwent artificial arthroplasty of bilateral femoral heads 11 years ago, when multiple aseptic necroses of thirteen bones were found, and when anti-cardiolipin (CL) antibody was found to be positive. An echogram of abdomen suggested an obstruction of superior mesenteric artery (SMA) when she was admitted. Selective angiography revealed a complete obstruction of SMA and splenic artery, and incomplete obstruction of celiac artery. Conservative treatment with urokinase infusion and prednisolone 50 mg/day was not effective, and small intestine and right colon were resected on the 23rd hospital day. The pathological examination showed thrombosis of SMA. There was no evidence of arteritis or atherosclerosis. Anti-CL antibody and lupus anticoagulant were positive on admission, but the level of both anti-DNA antibody and complement was normal. Therefore, it was suggested that the thrombosis was related with anti-phospholipid antibody. The characteristic clinical feature were multiple aseptic bone necroses and thromboses of several arteries. We discussed the relationship of thrombosis and the etiology of multiple bone necrosis in this case with anti-phospholipid antibody.     

Successful stenting of total left subclavian artery occlusion post-coronary artery bypass graft surgery using dual left vertebral artery and left internal mammary artery protection

We report on a 68-year-old male patient (smoker, chronic obstructive pulmonary disease, diabetic for 25 years) who was admitted with acute pulmonary edema 5 months after undergoing coronary artery bypass grafting (left internal mammary artery to left anterior descending artery, saphenous vein graft to posterior descending artery, and sequential saphenous vein graft to obtuse marginal artery). He had no cardiac enzyme leakage and his left ventricular ejection fraction was > 50% on 2-dimensional echocardiography. He proved to have total calcific left subclavian artery occlusion and retrograde flow in both his left internal mammary artery and his left vertebral artery upon left subclavian angiography performed through the left brachial approach. Angiographic vertebral and coronary-subclavian steals were both supported with clinical manifestations. The patient underwent successful stenting to his left subclavian artery using dual protection to his left vertebral artery with filter protection device and to his left internal mammary artery using simple balloon inflation at its mouth before and after each subclavian artery angioplasty step. Three months later, the patient was free from any cardiac or neurologic complaints.     

Is aorto-arteritis a manifestation of primary antiphospholipid antibody syndrome?

A 23 year old female presented with dyspnea on exertion and absent pulses in the left upper limb. She had prior history of two first trimester abortions and pre-eclampsia with premature delivery. A Doppler examination had revealed left subclavian and axillary artery thrombosis for which she had been given warfarin six months previously. She was admitted and investigated. Patient had low positive aCL IgG antibody, positive antibeta2gp1 antibody, negative lupus anticoagulant and negative ANA. Patient had cardiomegaly and her echocardiography showed severe aortic regurgitation, moderate mitral regurgitation and moderate pulmonary artery hypertension with poor ejection fraction with normal aortic root. A diagnosis of primary antiphospholipid antibody syndrome with valvular involvement with dilated cardiomyopathy was entertained. A CT angiogram of the aorta revealed narrowing and irregularity of the aorta and its multiple branches suggestive of type III Takayasu's arteritis. Temporal relationship suggests development of aorto-arteritis secondary to APS but simultaneous presence of both these disorders in this patient cannot be ruled out.     

Chronic pulmonary thromboembolism originating from left forearm deep vein thrombosis: a case report

Upper extremity deep venous thromboembolism has become increasingly common due to the use of subclavian venous access. However, forearm deep venous thrombosis is rare. We report a case of chronic pulmonary thromboembolism originating from left forearm deep venous thrombosis. A 66-year-old woman was admitted to our hospital because of dyspnea, which had worsened over the previous 2 months. Echocardiography showed severe right ventricular enlargement and pulmonary hypertension. Perfusion lung scintigraphy revealed multisegmental perfusion defects. No likely factors responsible for hypercoagulability were found. The patient had a history of left radial fracture and had been treated with external fixation. Venography of the upper and lower extremities revealed total occlusion of the deep veins of the left forearm with collateral vessels, but no evidence of other upper or lower extremity venous thrombosis.     

Venography in axillary-subclavian vein thrombosis.

Nineteen patients with 20 axillary-subclavian vein thromboses were examined by venography. In 70% the axillary and subclavian veins were involved in continuity. The innominate vein was seldom involved, although demonstration of this was difficult. In 50% of the patients the thrombosis was a secondary phenomenon associated with either breast carcinoma, central venous pressure lines, heroin addiction, or cervical rib.     

Catheter interventional therapy in an elderly patient with deep vein thrombosis and a brain tumor

A 92-year-old woman with a brain tumor developed swelling of the left lower extremity. Venography showed considerable thrombi from the left common iliac vein to the femoral vein. Following implantation of a temporary inferior vena cava filter, catheter aspiration therapy and catheter-directed thrombolysis were performed. Venography after 3 days showed disappearance of the thrombi and an improvement in vein flow. A permanent inferior vena cava filter was implanted. Local intensive thrombectomy and thrombolysis by catheter together with a temporary inferior vena cava filter were effective treatments in this elderly patient with deep vein thrombosis.     

Diabetic ketoacidosis complicated by generalized venous thrombosis: a case report and review

Venous thromboembolism is a rarely described complication of diabetic ketoacidosis (DKA). We describe a 21-year-old male patient with poorly controlled type 1 diabetes mellitus who was admitted with DKA, presumably secondary to noncompliance, whose clinical picture was complicated by generalized thrombosis involving multiple venous locations including renal vein, pulmonary vasculature, external iliac and common iliac veins. The patient had no family history of any coagulation disorders and a hypercoagulabilty work-up remained negative. The patient was subsequently anticoagulated with heparin and discharged home on warfarin. To the best of our knowledge, this is the first reported case of multiple venous thromboses occurring as a complication of DKA with no other risk factors. We also reiterate that although rare, venous thrombosis should always be considered as a potential complication of DKA.     

Pulmonary embolism and deep vein thrombosis complicating acute aortic dissection during medical treatment

Acute aortic dissection of Stanford type A with intramural hematoma was diagnosed based on computed tomography (CT) findings in a 60-year-old man. During medical treatment, pulmonary embolism and deep vein thrombosis developed. CT revealed thrombosis in the right pulmonary artery, and 99mTc pulmonary perfusion scintigraphy showed defects in the right lung field. CT showed thrombus in the common iliac vein. An inferior vena caval filter was placed because anticoagulation therapy was contraindicated. A CT scan before discharge showed no thrombus in the pulmonary artery or common iliac vein, but a newly captured thrombus was found inside the filter.     

Deep venous thrombosis and pulmonary thromboembolism associated with a huge uterine myoma--a case report

A 51-year-old woman with a large uterine myoma suffered from acute pulmonary thromboembolism. Venography revealed thrombosis in the right common iliac vein and almost complete obstruction of the left common iliac vein. The ascending lumbar vein showed collateral drainage. Treatment was initiated with continuous intravenous heparin sodium, and a Greenfield filter was inserted to prevent the extension of the pulmonary embolism during and after hysterectomy. After a total hysterectomy, venography revealed restoration of patency in the bilateral common iliac veins, and no flow was seen in the ascending lumbar vein. Thorough clinical examinations failed to identify any other prothrombotic conditions. These results suggest that a large uterine myoma compressed veins in the pelvis, and the resulting impaired blood flow caused deep venous thrombosis and pulmonary thromboembolism.     

Diagnosis and management of subclavian vein thrombosis occurring in association with subclavian cannulation for hemodialysis

With the aim of improving the diagnosis and treatment of subclavian vein thrombosis, associated with subclavian cannulation for hemodialysis, we performed Doppler examination of the subclavian vein and clinical inspection of the ipsilateral arm at every dialysis in 50 consecutive patients who received subclavian hemodialysis catheters over 1 year. Edema of the arm and disappearance of the subclavian vein bruit correctly detected 3 cases of subclavian vein obstruction which were confirmed by X-ray venograms. All 3 cases failed to respond to systemic heparin, but were successfully recanalized within 36 h with continuous local streptokinase infusion at a rate of 10,000 U in 1 ml/h. In 4 other cases of edema of the arm, Doppler examination correctly predicted patency of the vein, also confirmed radiologically. In 2 cases of thrombosis, there was an underlying stenosis of the left innominate vein close to its union with the superior vena cava. These were dilated by balloon angioplasty; the stenosis recurred in both cases without recurrent thrombosis, and the angioplasty was repeated. Doppler surveillance seems to be a promising aid to the detection of subclavian vein thrombosis from hemodialysis catheters. Local streptokinase infusion is effective in treating thrombosis. Underlying venous stenosis should be looked for because this may be at least partly remediable by balloon angioplasty.     

Tissue Doppler echocardiography contribution to the diagnosis of upper extremities venous thrombosis

We present the case of a 35-year-old man with a history of beta-thalassemia complicated with heart failure who was admitted to our department because of right arm painful swelling. A transthoracic echo-Doppler study revealed a mass within the right jugular vein (RJV) lumen and absence of flow within the right subclavian vein (RSV). Subsequently, color tissue Doppler echocardiography clearly demonstrated the intraluminal mass by means of its different color hues as compared with the surrounding vessel wall, further enforcing the suspicion of upper extremity vein thrombosis (UEVT). It is emphasized that tissue Doppler echocardiography, a safe and reproducible method, can contribute to the diagnosis of UEVT.     

Acute thrombosis of subclavian artery during CPR

The case of a 73-year-old man who developed acute thrombosis of the left subclavian artery during CPR is presented. The patient was known to have severe chronic obstructive lung disease, hypertension, coronary artery disease, and severe peripheral vascular disease. He was admitted with ventricular fibrillation. CPR was successful, and the ECG revealed acute extensive anterior and recurrent inferior wall myocardial infarctions. Soon after, acute occlusion of the left subclavian artery was diagnosed. Thrombectomy was performed and circulation was restored to the left upper limb. The patient died 12 hours later from severe bradycardia and asystole.     

A patient with gallbladder cancer with paraaortic lymph node and hepatic metastases who has survived for more than 13 years after the primary extended radical operation

Gallbladder cancer is a disease with poor prognosis, especially when it is associated with distant metastasis. Here we report a rare case of a patient with gallbladder cancer with extensive local and distant lymph node metastases and multiple liver metastases who has survived for more than 13 years through aggressive treatments. A 54-year-old woman developed right upper quadrant pain. Computed tomography (CT) revealed a papillary tumor in the gallbladder. Low-density tumors in segments 4, 5, and 8 of the liver and extensive paraaortic lymph node swelling were observed. She underwent central hepatic bisectionectomy and paraaortic lymphadenectomy. Two months later, hepatic metastases were found in segments 2, 3, 6, and 7, and percutaneous ethanol injection and transcatheter arterial chemoembolization were performed. Twelve months after the first surgery, CT revealed lymph node swelling around the right external iliac artery and behind the left renal vein. Metastatic lymph node dissection and resection and reconstruction of the right external iliac artery and vein with artificial graft replacements were performed. Two months later, CT revealed a paraesophageal lymph node swelling, which was treated by radiotherapy. At present, 13 years after the first surgery, and 11 years after the last radiotherapy, she is alive without any sign of recurrence.     

Primary antiphospholipid syndrome: a cause of fever of unknown origin

Antiphospholipid syndrome (APS) is defined as the occurrence of thrombosis, recurrent miscarriage, or both in association with laboratory evidence of persistent antiphospholipid antibodies. Owing to protean manifestations and laboratory studies, the diagnosis may be difficult. Because the other signs and symptoms of thrombosis are predominant, prolonged fever is not usually the main clinical finding. We describe a patient who presented with fever of unknown origin (FUO) and was found to have thromboses of the splenic vein, the superior mesenteric vein, and the portal vein due to the primary antiphospholipid syndrome. We also reviewed the medical literature (Medline 1966-2001), including the main FUO series of the previous 40 years, and laparotomy series for FUO. We conclude that although very rare, primary APS and thrombosis may present with FUO. APS should be considered in the differential diagnosis of prolonged fever associated with thrombosis.