A successful surgical repair of anomalous right pulmonary venous connection with Goldenhar syndrome

A ten-year-old girl with Goldenhar syndrome underwent intracardial repair for partial anomalous right pulmonary venous connection into the superior vena cava. In surgical procedure the proximal tract of superior vena cava was reconstructed into double floor, low floor was tract for anomalous pulmonary venous blood drained into left atrium through the atrial septal defect which was made with resection of prinum septum, upper floor was a real tract for superior venous blood. After operation, arrhythmia did not detect. One month after operation, no stenosis along the superior vena cava or right pulmonary vein were evidenced in catheterization. This surgical technique is superior to using baffle for preventing thrombogenesis. The result suggested that this surgical technique is preferable to the reconstruction of baffle in superior vena cava.     

A case of retroperitoneal hemangiopericytoma

We report a case of a huge retroperitoneal tumor in a 67-year-old woman. When the patient was taken to another hospital by ambulance, she had lost consciousness because of hypertension and hypoglycemia and abdominal CT revealed a huge retroperitoneal tumor with deviation of the right kidney and inferior vena cava. After further examinations including ultrasonography, MRI and angiography in our hospital, the tumor was extirpated. The tumor, 22 x 17 x 10 cm in size and 2,580 g in weight was diagnosed as hemangiopericytoma histologically. She has remained well with no evidence of recurrence for 9 months since the operation.     

Thymic carcinoma with tumor thrombus into the superior vena cava

Tumor thrombus into the vena cava have been reported in cases with renal cell carcinoma, thyroid tumor and in those with thymoma. These tumors are frequently invasive and continuous from the main tumor that shows direct vessel wall invasion. Here, we report a case of thymic carcinoma with superior vena cava syndrome, which was caused by a tumor thrombus in the superior vena cava without vessel wall invasion. The main mediastinal tumor did not show innominate vein invasion, and the superior vena cava syndrome was a result of separate tumor thrombus that was free of vessel wall invasion. The tumor thrombus could be removed through a simple venotomy. To prevent stenosis in the superior vena cava and the left innominate vein, we used a pericardial patch to close the venotomy site.     

A case report of intravenous leiomyomatosis of the uterus with extension into the right pulmonary artery

Intravenous leiomyomatosis (IVL) is a rare neoplastic disease which is defined as the extension into venous channels of histologically benign smooth muscle tumors arising either from a uterine myoma or from the walls of a uterine vessel. We report a case of IVL originating from the uterus, growing up in the inferior vena cava, and extending into the right pulmonary artery. The patient was a 42-year-old woman, who was admitted to our hospital with clinical signs of right-side heart failure and syncope in December 1987. Her medical history included a total hysterectomy in August 1986. On examination, two dimensional echocardiography showed a freely floating tumor in the right atrium and right ventricle, which protruded via the inferior vena cava. Cavography confirmed the above findings. Because of the clinical disability caused by the tumor localized in the right heart, emergency operation was performed, extracorporeal circulation was instituted through the femoral vein, the superior vena cava, and the femoral artery. Opening the right atrium confirmed that a white sausage-like mass quite free from the wall extended into the right ventricle, and the head of the tumor was adherent to the right pulmonary artery. After detaching the head of the tumor from the right pulmonary artery, the remaining tumor in the inferior vena cava was also successfully pulled out. The operative specimen was measured 55 cm in length, but the basal end of the tumor was supposed to be left in place. Two months later, the second-stage operation for the remaining tumor was carried out through a median laparotomy.(ABSTRACT TRUNCATED AT 250 WORDS)     

Mitral valve plasty in an adult patient without a right superior vena cava

Persistent left superior vena cava without a right superior vena cava is an extremely rare condition. We report the case of a 65-year-old woman with this condition who underwent mitral valve plasty. During cardiac catheterization, the asymptomatic patient with mitral valve prolapse syndrome was found to have a persistent left superior vena cava without a right superior vena cava. During mitral valve plasty, cardiopulmonary bypass was established using bicaval drainage through the persistent left superior vena cava and the right atrium. A cannula was inserted into the persistent left superior vena cava to provide a large surgical field in the left atrium. We selected a technique that involved direct insertion of an L-shaped cannula into the persistent left superior vena cava and obtained a clear view of the surgical field. Proper assessment of the right superior vena cava is necessary when a persistent left superior vena cava is suspected.     

Mediastinoscopy as an emergency diagnostic procedure in superior vena cava syndrome.

Seven men and one woman (aged 20-70 years) with superior vena cava syndrome underwent diagnostic mediastinoscopy to elucidate the cause, which other, lesser procedures had not identified. Intraoperative frozen-section studies of the biopsy specimens revealed small-cell carcinoma (4 cases), large-cell carcinoma (1), squamous-cell carcinoma (1), large-cell lymphoma (1) and Hodgkin's lymphoma (1). Radiotherapy or chemotherapy was initiated within the following 24 hours in six cases. One of the tumors intraoperatively diagnosed as small-cell carcinoma was subsequently reclassified as lymphocytic lymphoma. Complicating hemorrhage from the right carotid artery required median sternotomy in one case and wound infection occurred in another. There was no mediastinoscopy-related mortality. Mediastinoscopy is useful and reliable in the diagnostic emergency posed by the superior vena cava syndrome.     

Management of superior vena cava syndrome by internal jugular to femoral vein bypass

We report a 30-year-old man with superior vena cava syndrome due to fibrosis from a previously irradiated malignant thymoma. The patient presented 4 years after the initial treatment, after having been lost to follow-up. Investigations revealed total obstruction of the superior vena cava, and right subclavian and right internal jugular vein. The patient underwent an extra-anatomic bypass (ringed polytetrafluoroethylene graft 10-mm diameter) between the left internal jugular vein and the left femoral vein brought in a subcutaneous tunnel over the anterior chest and abdominal wall. Entry to the thoracic cavity was avoided due to extensive fibrotic changes visualized in the computed tomographic chest scan. Follow-up Doppler at 2 months, 6 months, 1 year, and 3 years showed a patent graft. An internal jugular vein to the femoral vein bypass is a simple method for palliation of superior vena cava syndrome.     

Dialysis access-induced superior vena cava syndrome

Vascular thrombosis is a complication of dialysis access and thrombosis of the superior vena cava by indwelling dialysis catheters access can cause superior vena cava syndrome. We describe a case of superior vena cava syndrome resulting from a dialysis access catheter placed in the internal jugular vein. Although surgical intervention is often needed to treat dialysis access-related superior vena cava syndrome this patient required only conservative measures for resolution of the syndrome. In this paper we describe the presentation, diagnosis, and management of this case. A review of dialysis access thrombosis complications and treatment options is also presented.     

Palliative surgery for malignant mediastinal tumor invading the superior vena cava and right atrium

Intra-atrial extension of a superior vena cava (SVC) tumor is rare and it is associated with high mortality rates. An 80-year-old woman presented with malignant mediastinal tumor invading the superior vena cava and right atrium. The unresectable tumor caused uncontrolled SVC syndrome and led to the risk of pulmonary artery tumor thrombosis. Palliative surgery for the intracaval and atrial tumor was performed. Presently, 6 months after surgery, the patient remains free from SVC syndrome and has received effective adjuvant chemotherapy to reduce the size of the tumor.     

Scimitar syndrome with an accessory diaphragm and an absent right superior vena cava

An 8-year-old boy with scimitar syndrome, an accessory diaphragm and an absent right superior vena cava, underwent surgery on March 28, 1983. The scimitar vein was separated from an accessory diaphragm and cut just above the right diaphragm where the vein penetrated. The vein was re-implanted into the right lateral portion of the right atrium and a tunnel was made between the atrial septal defect created in the septum and the site of the implanted vein. The accessory diaphragm was not removed because of the lack of compression on the right lung. At cardiopulmonary bypass, venous cannulae were inserted into the persistent left superior vena cava and inferior vena cava. Because of the absence of the right superior vena cava, the right atrium was not fixed by both cavae so that there was difficulty in intracardiac maneuvers. The patient is doing well 32 months after this treatment.     

Scimitar syndrome with an accessory diaphragm and an absent right superior vena cava

An 8-year-old boy with scimitar syndrome, an accessory diaphragm and an absent right superior vena cava, underwent surgery on March 28, 1983. The scimitar vein was separated from an accessory diaphragm and cut just above the right diaphragm where the vein penetrated. The vein was re-implanted into the right lateral portion of the right atrium and a tunnel was made between the atrial septal defect created in the septum and the site of the implanted vein. The accessory diaphragm was not removed because of the lack of compression on the right lung. At cardiopulmonary bypass, venous cannulae were inserted into the persistent left superior vena cava and inferior vena cava. Because of the absence of the right superior vena cava, the right atrium was not fixed by both cavae so that there was difficulty in intracardiac maneuvers. The patient is doing well 32 months after this treatment.     

Pacemaker implantation in a patient with sick sinus syndrome, complicated with persistent left superior vena cava and absent right superior vena cava; report of a case

A 78-year-old man was referred to our hospital with syncope and palpitation. A Holter electrocardiography (ECG) revealed sick sinus syndrome (SSS), and an enhanced chest computed tomography (CT) scan showed persistent left superior vena cava (PLSVC) and absent right superior vena cava. Myocardial leads and a pacemaker implantation were peformed through left anterior thoracotomy approach. There were only 10 reports of pacemaker implantation in a patient with SSS complicated with PLSVC and absent right superior vena cava in Japan. Open thoracotomy approach was thought to be usuful for these patients.     

A long-term survival case of thymic squamous cell carcinoma, performed complete extirpation with vascular reconstruction of the superior vena cava.

A 59-year-old woman who complained of anterior chest pain exhibited an abnormal shadow on chest X ray and was admitted to our hospital. The chest X ray showed a demarcated tumor at the anterosuperior mediastinum and she was diagnosed as having a mediastinal tumor. After the midsternotomy was performed, the mediastinal tumor derived from the thymic tissues was discovered to have invaded the right upper lung, pericardium and superior vena cava. After excising the tumor, anastomosis between the right brachiocephalic vein and superior vena cava, followed by that between the left brachiocephalic vein and right cardiac auricle was performed using expanded polytetrafluoroethylene-ringed vascular grafts (phi 10 mm) for reconstruction. The tumor was diagnosed as a thymic carcinoma (squamous cell carcinoma) pathologically. After surgery, she was treated by cobalt irradiation. One month and again 3 months after the operation, venography showed patency. The patient has not demonstrated recurrence for 9 years and 6 months.     

A long term survival case of thymic squamous cell carcinoma, performed complete extirpation with vascular reconstruction of the superior vena cava

A 59-year-old woman who complained of anterior chest pain exhibited an abnormal shadow on chest X ray and was admitted to our hospital. The chest X ray showed a demarcated tumor at the anterosuperior mediastinum and she was diagnosed as having a mediastinal tumor. After the midsternotomy was performed, the mediastinal tumor derived from the thymic tissues was discovered to have invaded the right upper lung, pericardium and superior vena cava. After excising the tumor, anastomosis between the right brachiocephalic vein and superior vena cava, followed by that between the left brachiocephalic vein and right cardiac auricle was performed using expanded polytetrafluoroethylene-ringed vascular grafts (? 10 mm) for reconstruction. The tumor was diagnosed as a thymic carcinoma (squamous cell carcinoma) pathologically. After surgery, she was treated by cobalt irradiation. One month and again 3 months after the operation, venography showed patency. The patient has not demonstrated recurrence for 9 years and 6 months.     

Giant benign mesenchymoma of the mediastinum causing superior vena cava syndrome: Report of a case

The surgical resection of a giant primary mediastinal mesenchymoma causing superior vena cava syndrome is herein described. A 53-year-old man was admitted to the hospital complaining of severe dyspnea. A chest roentgenogram showed a large mediastinal mass shadow compressing the superior vena cava. At surgery, a yellow tumor measuring 27×25×12 cm and weighing 3,620 g was resected. The histological diagnosis was benign mesenchymoma. The patient's postoperative course was uneventful with a successful resolution of his superior vena cava syndrome.     

A case of malignant lymphoma of anterior mediastinum requiring superior vena cava reconstruction]

The patient was a 21-year-old female with malignant lymphoma of the anterior mediastinum, which was associated with superior vena cava (SVC) syndrome due to tumor invasion and tracheo-bronchial stenosis due to tumor compression. The bilateral brachio-cephalic vein and superior vena cava were resected with the tumor, and SVC reconstruction with a ringed expanded polytetrafluoroethylene (EPTFE) graft was performed between the right brachio-cephalic vein and SVC, and between the left brachio-cephalic vein and the right appendage. Postoperative anti-coagulant therapy was not performed. Postoperatively, SVC syndrome and the complaint of tracheo-bronchial stenosis disappeared promptly. Venography, which was performed 2 weeks postoperatively, presented patency between the right brachio-cephalic vein and SVC, but the graft between the left brachio-cephalic vein and the right appendage was obstructed. SVC reconstruction using a ringed EPTFE graft is considered to be significant for the improvement of resectability and the relief of clinical symptoms.     

Clinical trials of an intravenous oxygenator in patients with adult respiratory distress syndrome.

In patients with severe adult respiratory distress syndrome, mechanical ventilation may not be able to ensure gas exchange sufficient to sustain life. We report the use of an intravenous oxygenator (IVOX) in five patients who were suffering from severe adult respiratory distress syndrome as a result of aspiration, fat embolism, or pneumonia. IVOX was used in an attempt to provide supplemental transfer of CO2 and O2 and thereby reduce O2 toxicity and barotrauma. All patients were tracheally intubated, sedated, and chemically paralyzed and had a PaO2 < 60 mmHg when the lungs were ventilated with an FIO2 = 1.0 and a positive end expiratory pressure of > or = 5 cmH2O. The right common femoral vein was located surgically, and the patient was systemically anticoagulated with heparin. A hollow introducer tube was inserted into the right common femoral vein, and the furled IVOX was passed into the inferior vena cava and advanced until the tip was in the lower portion of the superior vena cava. IVOX use ranged from 2 h to 4 days. In this group of patients, IVOX gas exchange ranged from 21 to 87 ml x min-1 of CO2 and from 28 to 85 ml x min-1 of O2. One of the five patients survived and was discharged from the hospital. The IVOX transferred up to 28% of metabolic gas-exchange requirements. One patient with a small vena cava showed signs of caval obstruction. Three other patients demonstrated signs of a septic syndrome after the device was inserted.(ABSTRACT TRUNCATED AT 250 WORDS)     

Brain swelling in acute superior vena cava syndrome due to aortic dissection: unusual and lethal manifestation aggravated by induction of general anesthesia

A 32-year-old woman with Marfan syndrome experienced acute superior vena cava syndrome due to aortic dissection. The patient had previously undergone a Bentall operation. The aneurysm from the ascending to the transverse aorta compressed the superior vena cava, the right pulmonary artery, and the trachea. The rare and life-threatening neurological complication in this patient may have been related to brain edema, which was revealed by preoperative computed tomography. The induction of general anesthesia aggravated the symptoms of the superior vena cava syndrome and led to a fatal condition. Additional cannulation in the right subclavian vein was mandatory to alleviate the symptoms because the venous drainage from the upper half of the body created only by the femorofemoral bypass was not adequate. Total arch replacement was performed. The postoperative course was uneventful.     

Intraatrial extension of thyroid cancer: technique and results of a radical surgical approach

An occlusion of the superior vena cava by a tumor thrombus extending into the right atrium was diagnosed in three patients with a follicular thyroid cancer. All patients showed the typical clinical picture of the superior vena cava syndrome. A right parasternal thoracotomy was performed for preparation of the major vessels. The superior vena cava was opened and the entire intravascular tumor thrombus was removed. The cavotomy was closed directly in two patients. In the third patient the left brachiocephalic trunk was resected and reconstructed with a vascular (polytetrafluoroethylene) graft. This patient had bone and brain metastases and an occlusion of the graft 3 months after surgery after anticoagulation was stopped. The other two patients were clinically symptom free without local recurrence 13 and 50 months after surgery. An aggressive surgical approach is justified in grossly invasive thyroid cancer to decrease local recurrence and death rates, to correct the disturbing clinical symptoms of superior vena caval occlusion, and to prevent tumor embolism and the development of distant metastases. By reducing tumor mass, an even better basis for radioiodine treatment can be prepared.     

A case of invasive thymoma displaying endobronchial and endocaval polypoid growth

The hens' egg-sized tumor connecting from anterior mediastinum to the hilus by chest radiography and chest CT was noted in a 63 year old female who complained of facial swelling and cough. The defect of the thumb tip-sized shadow was noted in the superior vena cava by superior vena cavography, and polyp covered with white coating obstructing the right apical bronchus was noted by fibrotic bronchoscopy, and malignant finding was obtained by biopsy of this polyp. After midsternotomy, a mass invaded to the superior vena cava and the right upper lobe was resected, and then superior vena cava was reconstructed by using supported polytetrafluoroethylene. The resected specimen revealed that the tumor extended not only to the pulmonary parenchyma with polypoid growth into the lumina of bronchi but also to the superior vena cava with polypoid growth. The patient was discharged at the postoperative radiotherapy with 40 Gy, and he is still alive free from the disease 3 years and 11 months after operation.