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

OBJECTIVE: Vena cava superior syndrome comprises various symptoms of compression of vena cava superior. The results of increased venous pressure in the upper body may cause edema of the head and neck associated with cyanosis, plethora and 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 Pediatric Hematology-oncology, Erciyes University Faculty of Medicine, Kayseri,Turkey, from January 2010 to December 2017. Diagnostic procedures included hematological investigations, chestradiography, thoracic computed tomography, echocardiography and 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- lymphoblastic leukemia 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/kg dexamethasone). Furthermore, the patient with anaplastic round cell sarcoma received emergency radiotherapy. No patients died because of Vena cava superior syndrome.
CONCLUSION: Vena cava superior syndrome is a medical emergency that requiresurgent treatment. Vena cava superior syndrome studies in children are rare. In this retrospective study,we found that the most common cause of Vena cava superior syndrome was Hodgkin’s lymphoma different from literature.

Mediastinal Tümörlü Çocuklarda Vena Cava Superior Sendromu: Tek Merkez Deneyimi

GÝRÝÞ ve AMAÇ: Vena cava superior sendromu, vena cava superiorun çeþitli kompresyon belirtilerini içerir. Vücudun üst kýsmýnda artmýþ basýncýn sonucunda siyanozla beraber baþ ve boyunda ödem, plethore ve yüzeyel damarlarda belirginleþmeye sebep olabilir. Vena cava superior sendromunun çocuklarda nadir olmasý nedeniyle bu çalýþmayý planladýk.
YÖNTEM ve GEREÇLER: Retrospektif çalýþma, Erciyes Üniversitesi Týp Fakültesi Pediatrik Hematoloji-Onkoloji Anabilim Dalý'nda Ocak 2010-Aralýk 2017 tarihleri arasýnda mediastinal tümörü olan çocuklarda yapýldý. Taný yöntemleri arasýnda hematolojik incelemeler, göðüs radyografisi, göðüs tomografisi, ekokardiyografi ve lenf nodu veya mediastinal biyopsi sayýlabilir.
BULGULAR: Mediastinal tümör tanýsý alan 41 hastadan 19’ unda (% 46.3) Vena cava superior sendromu bulgularýnýn olduðu saptandý. Geriye dönük tanýlara bakýldýðýnda 7’ si (% 37) Hodgkin Lenfoma, 6’sý (% 32) Non-Hodgkin Lenfoma, 4’ü (%21) T hücreli Akut Lenfoblastik Lösemi, 1’i (%5) nöroblastom, 1’i (%5) anaplastik round cell sarkom idi. 19 hastanýn tümünde yüzde þiþlik, venöz geniþleme, mediastinal geniþleme görüldü. Tüm hastalar intravenöz kortikosteroid tedavisi aldý (0.6 mg/kg deksametazon). Ayrýca anaplastik round cell sarkom tanýsý alan bir hasta radyoterapi aldý. Vena cava superior sendromu nedeniyle ölen hastamýz olmadý.
TARTIÞMA ve SONUÇ: Vena cava superior sendromu acil tedavi gerektiren bir týbbi acil durumudur. Çocukluk yaþ gurubu Vena cava superior sendromu çalýþmalarý nadirdir. Bu retrospektif çalýþmada, Vena cava superior sendromunun en sýk nedeni literatürden farklý olarak Hodgkin Lenfoma olarak saptanmýþtýr. (NCI-2019-0004.R1)