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

Nossair, Fadi; Schoettler, Peter; Starr, Joanne P.; Chan, Anthony; Kirov, Ivan I.; Paes, Bosco; Mahajerin, Arash

doi:10.1002/pbc.27225

Cited by 33 publications

(36 citation statements)

References 17 publications

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“…A prospective study conducted by Hartmann et al 31 In our study, 72% were extremely preterm and 81% had vena cava syndrome, which in the neonate period is associated with a higher rate of complications due to limited physiological reserve, leading to increased morbidity and mortality in these patients. 52 Furthermore, in 33% of the cases Staphylococcus aureus, one of the most deadly agents in developing countries and a master manipulator of the human coagulation system, 53 was isolated in blood cultures.…”

Section: Discussionmentioning

confidence: 99%

Thrombolytic therapy in preterm infants: Fifteen‐year experience

Grizante-Lopes

Garanito

Celeste

et al. 2020

Pediatric Blood & Cancer

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Objective To report a single‐center experience with thrombolytic therapy using recombinant tissue plasminogen activator (rt‐PA) in preterm neonates with severe thrombotic events, in terms of thrombus resolution and bleeding complications. Study design This retrospective study included 21 preterm neonates with severe venous thrombotic events admitted to the neonatal intensive care unit, identified in our pharmacy database from January 2001 to December 2016, and treated with rt‐PA until complete or partial clot lysis, no‐response or bleeding complications. Our primary outcome was thrombus resolution. Results Twenty‐one preterm neonates were treated with rt‐PA for an average of 2.9 cycles. Seventeen patients (80.9%) had superior vena cava thrombosis and superior vena cava syndrome. All patients had a central venous catheter, parenteral nutrition, mechanical ventilation, and sepsis. Fifteen patients (71.4%) were extremely preterm, 11 (52.4%) were extremely low birth weight, and seven (33.3%) were very low birth weight. The patency rate was 85.7%, complete lysis occurred in 11 (52.4%) patients, and partial lysis in seven (33.3%). Minor bleeding occurred in five (23.8%) patients, three patients (14.2%) had clinically relevant nonmajor bleeding events, and major bleeding occurred in six (28%) patients. Conclusion In this study, the rate of thrombus resolution in preterm neonates treated with rt‐PA were similar to the percentages reported in children and adolescents, with a high rate of bleeding. Therefore, rt‐PA thrombolytic therapy should only be considered as a treatment option for severe life‐threatening thrombosis in premature neonates for whom the benefits of the thrombolytic treatment outweigh the risks of bleeding.

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Section: Discussionmentioning

confidence: 99%

Thrombolytic therapy in preterm infants: Fifteen‐year experience

Grizante-Lopes

Garanito

Celeste

et al. 2020

Pediatric Blood & Cancer

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“…With a morbidity of 30% and a mortality of 18%, thromboembolic disorders have a profound impact on public health; they can cause pulmonary embolism (6-8%) and postthrombotic syndrome (12-19%) and have a significant percentage of recurrence (10-20%). (18) In this scenario, and taking into account that treatment in the pediatric population has not yet been clearly established, it is important to make a risk-benefit comparison to define the best therapy for each patient based on antithrombotic and anticoagulant therapeutic options, especially for the neonatal population. In this sense, it is necessary to stress the importance of conducting therapeutic research in this age group to support the therapies described in medical practice, since the lack of evidence has led to the need to extrapolate valid recommendations in the adult population without adequate consensus.…”

Section: Discussionmentioning

confidence: 99%

Fibrinolytic therapy in newborns with superior vena cava syndrome. Case report

et al. 2020

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Introduction: Superior vena cava syndrome is described as the obstruction of blood flow through the superior vena cava. The literature reports that the incidence of this pathology varies from 1 case in every 650 inhabitants and 1 case in every 3 100 inhabitants. Since this condition is very rare in the pediatric population, no clear figure has been reported regarding its incidence in children. The use of a central venous catheter in newborns is a risk factor for this condition, as it may cause a thrombus due to the inflammatory reaction against the device. Therefore, it is necessary to initiate anticoagulation management and remove the catheter.Case presentation: Premature male newborn, (31.4 weeks gestation), with acute respiratory distress syndrome, early neonatal sepsis, pneumonia, necrotizing enterocolitis on 2 occasions, intestinal obstruction due to adhesions and intestinal volvulus. At 90 days of age, he presented thrombosis of the superior vena cava without involvement of the jugular and subclavian vein junction in the right atrium. Anticoagulant management was started, but given his unfavorable evolution, a multidisciplinary medical board was held to assess the risks, benefits, and treatment options in this age group. It was decided to start intracavitary tissue plasminogen activator treatment associated with mechanical thrombectomy and angioplasty of the superior vena cava. Due to the difficulty of conducting clinical trials in this population and the rates of major bleeding complications obtained with thrombolytic therapies, there is very little information available on the use of tissue plasminogen activator in newborns. For this reason, alteplase is seldom considered as the therapy of choice. However, in patients with life-threatening thrombosis, such as the present case, the results obtained in adults could be extrapolated in search of a favorable outcome.Conclusions: Fibrinolytic therapy is a way to reduce the size of the thrombus, but it dramatically increases the risk of bleeding; consequently, these patients must be strictly monitored. In pediatric populations, due to the diameter of the blood vessels, thrombectomy is difficult to perform; additionally, recurrent thrombosis and the need for transfusion of blood products are frequent.

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“…5 One of the most common forms of presentation is Superior Vena Cava Syndrome, whose clinical features may include head and neck edema, dyspnea, cough, neurological symptoms, pleural effusion, or chylothorax. 6 Congenital stenosis of the IVC is also very rare (0.15% of the general population) and may present with an array of clinical findings, ranging from a complete absence of symptoms to lifethreatening conditions, such as portal hypertension or hepatic cirrhosis. 7 Spontaneous lower extremity deep venous thrombosis, leg In this patient, in spite of a not quite impressive clinical presentation, a severe systemic venous drainage stenosis was found, with major involvement of the SVC, mild involvement of the IVC, and otherwise normal intracardiac anatomy.…”

Section: Discussionmentioning

confidence: 99%

Congenital systemic venous drainage obstruction: A case report

et al. 2020

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Background Vena cava anomalies are rare congenital defects due to incorrect development during fetal life, ranging from minor asymptomatic anatomic variations to complex life‐threatening abnormalities. Echocardiography plays a fundamental role in the diagnosis, with advanced imaging techniques allowing detailed anatomic delineation. Invasive cardiology techniques are a promising therapeutic approach, but surgery is probably the best option when diffuse compromise of the systemic veins is present. Case Report An 8‐month‐old infant presenting episodes of labial cyanosis and a failure to thrive was diagnosed with severe superior vena cava (SVC) stenosis at the right atrium ostium, with decompression via azygos vein and mild inferior vena cava (IVC) stenosis. The patient was referred for surgery, with IVC enlargement and removal of a fibromuscular tissue band on the anterior aspect of the SVC ostium. At a 6‐month follow‐up, the patient is asymptomatic, with excellent surgical outcome. To the authors' knowledge, only two cases of congenital SVC stenosis have been previously described.

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

Cited by 33 publications

References 17 publications

Thrombolytic therapy in preterm infants: Fifteen‐year experience

Thrombolytic therapy in preterm infants: Fifteen‐year experience

Fibrinolytic therapy in newborns with superior vena cava syndrome. Case report

Congenital systemic venous drainage obstruction: A case report

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