Pediatric superior vena cava syndrome: An evidence-based systematic review of the literature.

Superior vena cava syndrome (SVCS) results in vascular, respiratory, and neurologic compromise. A systematic search was conducted to determine the prevalence of pediatric SVCS subtypes and identify clinical characteristics/treatment strategies that may influence overall outcomes. Data from 101 case reports/case series (142 patients) were analyzed. Morbidity (30%), mortality (18%), and acute complications (55%) were assessed as outcomes. Thrombosis was present in 36%, with multi-modal anticoagulation showing improved outcome by >50% (P = 0.004). Infant age (P = 0.04), lack of collaterals (P = 0.007), acute complications (P = 0.005), and clinical presentation may have prognostic utility that could influence clinical decisions and surveillance practices in pediatric SVCS.

Clinical and radiological grading of superior vena cava obstruction.

BACKGROUND: Superior vena cava obstruction (SVCO) is commonly caused by neoplastic venous compression and presents with typical symptoms and signs. Its clinical severity presumably depends on the degree of obstruction and the adequacy of venous collateral formation. OBJECTIVES: The development of novel clinical and radiological scoring systems based on the postulate that a reproducible relationship exists between the degree of SVCO, the presence of collateral circulation and the extent of clinical symptoms. METHODS: We prospectively evaluated consecutive cases of acute and subacute SVCO with a newly developed clinical scoring system, which is based on easily detectable clinical symptoms and signs of SVCO. In parallel, we recorded and scored the degree of SVCO and the extent of collaterals visible on contrast-enhanced computed tomography (CT). RESULTS: Thirty-four cases of SVCO were evaluated: 8 (23.5%) were clinically mild, 16 (47%) moderate and 10 (29.5%) severe. Lung cancer was the underlying histological diagnosis in 94% of cases. Radiologically, 53% had complete SVCO. A well-developed collateral system was found in 14 (41%). A scoring system subtracting a 'collateral score' from an 'obstruction score' showed a significant correlation with the clinical score (r = 0.75, p < 0.01). CONCLUSIONS: Clinical severity of SVCO depends upon the degree of SVCO and is ameliorated by collateral formation. The novel clinical scoring system can predict the underlying CT features in SVCO and may be valuable in the bedside assessment of SVCO severity.

[Technical points of venous reconstruction for disorders of the superior vena cava].

In recent years, the number of patients with disorders of the superior vena cava due to mediastinal tumors such as malignant thymoma, teratoma and hilus type of lung cancer is now increasing in Japan. For those patients, we have tried to do venous reconstruction for the disorders of the superior vena cava (SVC) due to malignant mediastinal tumors. By these combined surgical procedures such as resection of malignant tumor and SVC reconstruction, curability and longevity of the life were apparently recognized. However, it is very important to select patients of which resection of tumor and venous reconstruction can be carried out by several kinds of examinations such as computed tomography (CT), magnetic resonance angiography (MRA) and venography. There are 3 kinds of reconstruction such as direct suture and patch grafting, as well as bypass grafting with ringed e-PTFE (expanded-polytetrafluoloethylene) prosthetic graft except for endovascular interventions of which number of patients is still few. On the other hand, bypass grafting is also effective for high intracranial pressure with syncope for the patients whose tumors could not be resected, because of huge tumor and diffuse invasion.

Superior vena cava obstruction: a venographic classification.

Analysis of venacavograms in 27 patients with superior vena caval obstruction revealed the following four patterns of venous collateral return: type I, partial obstruction (up to 90% stenosis) of the superior vena cava with patency of the azygos vein; type II, near-complete to complete obstruction (90-100%) of the superior vena cava with patency and antegrade flow through the azygos vein and into the right atrium; type III, near-complete to complete obstruction (90-100%) of the superior vena cava with reversal of azygos blood flow; type IV, complete obstruction of the superior vena cava and one or more of the major caval tributaries, including the azygos system. These patterns correlate well with the patients' clinical courses and can be used to identify patients who are at risk of developing cerebral and airway compromise and therefore would benefit from superior vena cava bypass surgery.