Vena cava superior syndrome in the children with mediastinal tumors: Single-center experience

Abstract

OBJECTIVE: Vena cava superior syndrome comprises various symptoms of compression of vena cava superior. The resultsof increased venous pressure in the upper body may cause edema of the head and neck associated with cyanosis, plethoraand distended subcutaneous vessels. Vena cava superior syndrome is rare in childhood. Therefore, we planned this retrospective study.METHODS: The retrospective study was carried out on the children with mediastinal tumors in the Department of PediatricHematology-oncology, Erciyes University Faculty of Medicine, Kayseri,Turkey , from January 2010 to December 2017. Diagnostic procedures included hematological investigations, chestradiography, thoracic computed tomography, echocardiographyand lymph node or mediastinal biopsy.RESULTS: In this study, 19 (five were female) of 41 patients with mediastinal tumors had Vena cava superior syndrome.Diagnosis included Hodgkin’s lymphoma in seven (37%), non-Hodgkin’s lymphoma in six (32%), acute T- lymphoblasticleukemia in four (21%), neuroblastoma and anaplastic round cell sarcoma in one each respectively. All of the 19 patients’facial swelling, venous distention and mediastinal widening. All patients received intravenous corticosteroids (0.6 mg/kgdexamethasone). Furthermore, the patient with anaplastic round cell sarcoma received emergency radiotherapy. No patientsdied because of Vena cava superior syndrome.CONCLUSION: Vena cava superior syndrome is a medical emergency that requiresurgent treatment. Vena cava superiorsyndrome studies in children are rare. In this retrospective study,we found that the most common cause of Vena cava superiorsyndrome was Hodgkin’s lymphoma different from literature.