Mediastinal lymphangioma causing superior vena cava syndrome
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203 Hellenic Journal of Surgery 2012; 84: 3
Mediastinal Lymphangioma Causing Superior Vena Cava Syndrome Case Report A. Kourtesis Received 03/02/2012 Accepted 16/03/2012
Abstract
Case presentation
Background: Mediastinal tumours in children are most likely malignant. They can compress the great vessels or the aerodigestive tract causing dysphagia and stridor. Displacement of the cardiovascular structures by a rapidly growing mediastinal mass may cause haemodynamic instability. Case presentation: The case of an upper mediastinalneck lymphangioma in a 10-year-old male patient is described herein. This congenital condition manifested as superior vena cava syndrome. The diagnosis was suspected following imaging studies. The lesion was excised and the diagnosis was confirmed with histological analysis. Conclusions: Mediastinal masses are asymptomatic or potentially life-threatening conditions. Lymphangioma may occasionally cause superior vena cava syndrome. Surgical excision is recommended for larger lesions, for diagnostic and treatment purposes.
A ten-year-old male patient presented with acute onset of superior vena cava syndrome. The child was delivered by caesarian section following a fullterm pregnancy, and the mother’s past medical history was free of any disease. Family history was significant for rheumatic fever and purpura on the father’s side and hyperthyroidism treated with thyroxin on the mother’s side. The patient presented a rapid onset of head and neck swelling, without dyspnoea. His vital signs were stable and physical examination revealed a palpable neck mass. Chest radiography displayed widening of the superior anterior mediastinum. An ultrasound study confirmed the presence of a cystic mass in the mediastinum measuring 10x14 cm; the jugular veins were patent without evidence of thrombus. A computerized tomography of the chest demonstrated displacement of the trachea, the superior vena cava, and the thymus by the mass. Intravenous steroid and endoxan treatment was commenced empirically, due to suspicion of lymphoma. The patient’s
Key words:
Mediastinal lymphangioma, Superior vena cava syndrome
Introduction Superior vena cava syndrome is a rare clinical condition in childhood. It is a potentially life-threatening condition that presents acutely and merits immediate treatment. Lymphangioma of the mediastinum and neck is a benign lesion, though it can cause obstruction of the superior vena cava (1, 2). Mediastinal tumours in children are most likely malignant. When located in the thoracic inlet, they can compress the great vessels or the aerodigestive tract causing dysphagia and stridor. Displacement of the cardiovascular structures by a rapidly growing mediastinal mass may cause haemodynamic instability.
A. Kourtesis (Corresponding author) - Cardiac Surgery Department ‘Aghia Sophia’ Children’s Hospital of Athens, Greece e-mail: [email protected]
Fig.1 MRI demonstrating the mass
204 Hellenic Journal of Surgery 2012; 84: 3
Mediastinal Lymphangioma Causing Superior Vena Cava Syndrome
Fig.
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