Consensus ; Humans ; Mediastinal Neoplasms/therapy* ; Superior Vena Cava Syndrome/therapy* ; Vena Cava, Superior

Humans ; Mediastinal Neoplasms/therapy* ; Superior Vena Cava Syndrome/therapy* ; Vena Cava, Superior/surgery*

Objective: To summarize the clinical features, management and outcome of superior vena cava syndrome (SVCS) associated with mediastinal malignancy in children. Methods: Clinical data of 42 children of SVSC associated with mediastinal malignancy in Shanghai Children's Medical Center from January 2015 to December 2021 were collected and analyzed retrospectively. The clinical manifestations, pathological diagnosis, disease diagnosis process, and prognosis were summarized. Results: Among 42 children of SVCS associated with mediastinal malignancy, there were 31 males and 11 females. The age at diagnosis was 8.5 (1.9, 14.9) years. Cough and wheezing (33 cases, 79%), orthopnea (19 cases, 45%) and facial edema (18 cases, 43%) occurred most commonly. T-cell lymphoblastic lymphoma (T-LBL) was the most frequent pathological diagnosis (25 cases, 60%), followed by T-cell acute lymphoblastic leukemia (T-ALL) (7 cases, 17%), anaplastic large cell lymphoma (4 cases, 10%) and diffuse large B-cell lymphoma (2 cases, 5%), peripheral T-lymphoma, Hodgkin lymphoma, Ewing's sarcoma and germ cell tumor (1 case each). Pathological diagnosis was confirmed by bone marrow aspiration or thoracentesis in 14 cases, peripheral lymph node biopsy in 6 cases, and mediastinal biopsy in 22 cases. Twenty-seven cases (64%) had local anesthesia. Respiratory complications due to mediastinal mass developed in 3 of 15 cases who received general anesthesia. Of the 42 cases, 27 cases had sustained remission, 1 case survived with second-line therapy after recurrence, and 14 cases died (2 cases died of perioperative complications and 12 cases died of recurrence or progression of primary disease). The follow-up time was 36.7 (1.2, 76.1) months for 27 cases in continuous complete remission. The 3-year overall survival (OS) and events free survival (EFS) rates of 42 children were 59% (95%CI 44%-79%) and 58% (95%CI 44%-77%) respectively. Conclusions: SVCS associated with mediastinal malignancy in children is a life-threatening tumor emergency with high mortality. The most common primary disease is T-LBL. The most common clinical symptoms and signs are cough, wheezing, orthopnea and facial edema. Clinical management should be based on the premise of stable critical condition and confirm the pathological diagnosis through minimal invasive operation.
Child ; China ; Cough ; Edema ; Female ; Humans ; Male ; Mediastinal Neoplasms/diagnosis* ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma ; Respiratory Sounds ; Retrospective Studies ; Superior Vena Cava Syndrome/therapy*

Vena cava syndrome is caused by central venous obstruction and can be divided into superior vena cava syndrome and inferior vena cava (IVC) syndrome. Symptoms and signs of IVC syndrome vary from no symptoms to lower limb edema, hypotension, and typical venous stasis changes of the lower extremities, such as brownish discoloration of the skin, woody edema, and ulceration. Carbon dioxide pneumoperitoneum, lithotomy-Trendelenburg position, and abdominal obesity could increase intra-abdominal pressure. We report a patient undergoing laparoscopic surgery who showed intrathoracic herniation of peritoneal fat induced by elevated intra-abdominal pressure due to the reasons mentioned above, resulting in IVC syndrome and hypotension perioperatively. The patient was treated with a conservative approach because he was asymptomatic except for hypotension on the first postoperative day.
Carbon Dioxide ; Edema ; Humans ; Hypotension ; Laparoscopy ; Lower Extremity ; Obesity, Abdominal ; Pneumoperitoneum ; Skin ; Superior Vena Cava Syndrome ; Ulcer ; Vena Cava, Inferior

This paper introduced surgical treatment of malignancy-related superior vena cava syndrome. Typical cases were presented with diagnostic radiology results. Authors focused on the main approach to the malignancy-related superior vena cava syndrome of surgery. In order to make it simple for junior doctors to learn and practice, all 4 operation methods were described in details. The writer hopes it would be helpful for all the young thoracic surgeons.
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Humans ; Neoplasm Metastasis ; Superior Vena Cava Syndrome ; diagnostic imaging ; etiology ; surgery ; Thymoma ; complications ; diagnostic imaging ; surgery ; Thymus Neoplasms ; complications ; diagnostic imaging ; surgery ; Vena Cava, Superior ; diagnostic imaging ; pathology ; surgery

Congenital cardiovascular anomalies, such as dextrocardia, persistent left superior vena cava (SVC), and pulmonary artery (PA) sling, are rare disorders. These congenital anomalies can occur alone, or coincide with other congenital malformations. In the majority of cases, congenital anomalies are detected early in life by certain signs and symptoms. A 56-year-old man with no previous medical history was admitted due to recurrent wide QRS complex tachycardia with hemodynamic collapse. A chest radiograph showed dextrocardia. After synchronized cardioversion, an electrocardiogram revealed Wolff-Parkinson-White (WPW) syndrome. Persistent left SVC, PA sling, and right tracheal bronchus were also detected by a chest computed tomography (CT) scan. He was diagnosed with paroxysmal supraventricular tachycardia (PSVT) associated with WPW syndrome, and underwent radiofrequency ablation. We reported the first case of situs solitus dextrocardia coexisting with persistent left SVC, PA sling and right tracheal bronchus presented with WPW and PSVT in a middle-aged adult. In patients with a cardiovascular anomaly, clinicians should consider thorough evaluation of possibly combined cardiovascular and airway malformations and cardiac dysrhythmia.
Adult* ; Arrhythmias, Cardiac ; Bronchi ; Catheter Ablation ; Dextrocardia ; Electric Countershock ; Electrocardiography ; Hemodynamics ; Humans ; Middle Aged ; Pulmonary Artery ; Radiography, Thoracic ; Tachycardia ; Tachycardia, Supraventricular ; Thorax ; Vena Cava, Superior ; Wolff-Parkinson-White Syndrome*

We report a case of a 65-year-old man with Behcet's disease who presented with massive hemoptysis caused by bronchial varices. A computed tomography (CT) scan and bronchoscopy were performed to identify the bleeding site. The CT scan revealed pneumonia and a combined hemorrhage in the right-middle and lower lobes. Massive bleeding was detected during the bronchoscopy and emergency embolization was attempted but angiographic findings were normal. An anteriojugulo-right femoral bypass operation was performed to relieve the tortuous and hypertrophied jugular venous obstruction. However, thrombectomy and thrombolysis followed because of graft thrombosis six days post-surgery. The patient was treated with steroid and high-dose cyclophosphamide therapy for his Behçet's disease, which caused the venous obstructions; the saccular bronchial varices in the right-middle and right lower lobes on bronchoscopy regressed slightly after four cycles of cyclophosphamide therapy
Aged ; Bronchoscopy ; Cyclophosphamide ; Emergencies ; Hemoptysis ; Hemorrhage ; Humans ; Pneumonia ; Superior Vena Cava Syndrome ; Thrombectomy ; Thrombosis ; Tomography, X-Ray Computed ; Transplants ; Varicose Veins*

Superior vena cava (SVC) syndrome refers to a medical emergency resulting from compression of the SVC. It requires early diagnosis and treatment, and is usually caused by malignant tumors; rarely, mediastinal tuberculous lymphadenitis can cause SVC syndrome. Here, we present a case study of an immunocompetent 61-year-old woman who presented with acute onset SVC syndrome and was diagnosed with tuberculous lymphadenitis on thoracotomy; the symptoms resolved with anti-tuberculosis therapy. This unusual case highlights the importance of the differential diagnosis in patients presenting with acute onset SVC syndrome; a timely diagnosis and appropriate treatment lead to complete recovery.
Diagnosis ; Diagnosis, Differential ; Early Diagnosis ; Emergencies ; Female ; Humans ; Lymphadenitis ; Middle Aged ; Superior Vena Cava Syndrome* ; Thoracotomy ; Tuberculosis ; Tuberculosis, Lymph Node* ; Vena Cava, Superior*

Thromboembolic complications (TECs) are clinically important sequelae of nephrotic syndrome (NS). The incidence of TECs in children is approximately 2%-5%. The veins are the most commonly affected sites, particularly the deep veins in the legs, the inferior vena cava, the superior vena cava, and the renal veins. Arterial thrombosis, which is less common, typically occurs in the cerebral, pulmonary, and femoral arteries, and is associated with the use of steroids and diuretics. Popliteal artery thrombosis in children has been described in cases of traumatic dissection, osteochondroma, Mycoplasma pneumoniae infection, and fibromuscular dysplasia. We report of a 33-month-old girl with bilateral iliac and popliteal arterial thrombosis associated with steroid-resistant NS due to focal segmental glomerulosclerosis. Her treatment involved thrombectomy and intravenous heparinization, followed by oral warfarin for 8 months. Herein, we report a rare case of spontaneous iliac and popliteal arterial thrombosis in a young child with NS.
Child* ; Child, Preschool ; Diuretics ; Female ; Femoral Artery ; Fibromuscular Dysplasia ; Glomerulosclerosis, Focal Segmental* ; Heparin ; Humans ; Iliac Artery ; Incidence ; Leg ; Mycoplasma pneumoniae ; Nephrotic Syndrome ; Osteochondroma ; Pneumonia, Mycoplasma ; Popliteal Artery ; Renal Veins ; Steroids ; Thrombectomy ; Thrombosis* ; Veins ; Vena Cava, Inferior ; Vena Cava, Superior ; Warfarin

Endovascular treatment (EVT) including angioplasty and stenting is an effective treatment for superior vena cava (SVC) syndrome. Recurrence of SVC syndrome is mainly caused by tumor progression and occurs in around 20% after EVT, but sometimes venous thrombosis within stent accounts for recurrence of SVC syndrome. Anticoagulation after EVT is still a controversial issue. In our case, a 73-year-old man with SVC syndrome caused by mediastinal metastasis from non-small cell lung cancer underwent endovascular stent followed by anticoagulation with low molecular weight heparin (LMWH), but symptomatic progression due to in-stent thrombosis necessitated the second procedure after two weeks. A total of 4 sessions of endovascular stent and anticoagulation with LMWH, warfarin and rivaroxaban did not induce durable resolution of in-stent thrombosis. Our case suggests refractory in-stent thrombosis could develop despite of anticoagulation after endovascular stent for SVC syndrome.
Aged ; Angioplasty ; Carcinoma, Non-Small-Cell Lung ; Heparin, Low-Molecular-Weight ; Humans ; Neoplasm Metastasis ; Recurrence ; Rivaroxaban ; Stents* ; Superior Vena Cava Syndrome* ; Thrombosis* ; Vena Cava, Superior* ; Venous Thrombosis ; Warfarin