Title:A Long Time History of a Mediastinal Fibrosis with Triple Vascular Stenosis
Volume: 14
Issue: 3
Author(s): Laura Peretti, Pierre Vaillant, Yves Billon, Olivier Menard and Angelica Tiotiu*
Affiliation:
- Pulmonology Department, University Hospital, Nancy 54000,France
Keywords:
Endovascular stenting, mediastinal stenosis, triple vascular stenosis, surgery, endovascular treatment.
Abstract: Mediastinal fibrosis is a rare disease of diverse etiologies, but sometimes idiopathic. Its
evolution is progressive and its complications depend on the structures it compresses. Vascular
compressions may lead to superior vena cava syndrome, and pulmonary hypertension. Surgery and
endovascular treatment may be effective, but often need to be repeated. Systemic treatments,
including corticosteroids, have variable responses.
We present an original case of mediastinal fibrosis, compressing the superior vena cava, the right
pulmonary artery, and upper right pulmonary vein. For a 6 year follow up, the patient did not present
pulmonary hypertension but only superior vena cava syndrome which was effectively treated by
endovascular stenting. Systemic corticosteroids were not very effective for the improvement of
symptoms. Later, the patient was treated with chemotherapy and immunosuppressive drugs for acute
myeloid leukaemia. His respiratory, cardiovascular and imaging exams revealed stability which led us
to reflect on the possible impact of the immunosuppressive therapy on the evolution of this
mediastinal fibrosis.
