Polycythemia vera is a myeloproliferative disorder characterized by thrombotic complications both in the arterial and venous systems. We report the case of a 55-year-old patient affected by polycythemia vera, presenting with acute superior vena cava syndrome due to thrombosis of the upper part of the superior vena cava. Diagnosis was done clinically and by computed tomography scan and showed an unusual finding: an air bubble trapped in the brachiocephalic venous trunk. The patient underwent emergency surgery. Diagnosis and treatment of the case are discussed.
Polycythemia vera is a chronic myeloproliferative disorder characterized by complicated thrombotic and hemorrhagic events [
A 55-year-old male patient was referred from another hospital, acutely unstable and with signs of superior vena cava (SVC) syndrome. He had a history of polycythemia vera and was on anticoagulation therapy with warfarin and cytoreduction therapy with hydroxyurea.
Physical examination showed that body temperature was
A CT scan showed air bubbles trapped at the level of the venous brachiocephalic trunk (Figure
CT scan showing air bubbles trapped at the level of the venous brachiocephalic trunk.
Due to a rapidly deteriorating condition, no other investigations were attempted. The patient was transferred to the operating room for emergency surgery with a diagnosis of acute SVC obstruction in a patient with polycythemia vera.
At surgery (Figure
Surical findings. (a) The venous brachiocephalic trunk appeared enlarged. (b) The SVC and brachiocephalic vein were clamped. Their junction is opened, and thrombectomy performed. (c) A collection of samples of thrombus. (d) Venous repair using an enlargement patch of GoreTex.
Postoperative course was uneventful. The patient was discharged on postoperative day 8 and remains clinically well at 6-month follow-up.
Polycythemia vera is a myeloproliferative disorder. Patients with such pathology are at risk for thrombotic complications in both the arterial and venous systems [
The diagnosis in this patient was done mainly clinically but also with the use of the CT scan. On the scan a particular finding was present: air bubbles trapped in the brachiocephalic vein, probably arising from intravenous injection before performing the CT scan. The bubbles remained trapped in the brachiocephalic vein due to the obstruction of the upper part of the SVC. The lower part of the SVC was however patent, with flow from the azygos vein.
Upper body oedema of rapid onset and air bubbles trapped in the brachiocephalic trunk, as seen on the CT-scan, should be regarded as suspicious signs for acute vena cava syndrome in patients at risk for venous thrombosis (myeloproliferative disorders), especially in the presence of concomitant venous catheters, and should strongly encourage rapid treatment.
Successful treatment with streptokinase has also been reported. However, in the present case, surgery was mandatoary due to the rapidly deteriorating condition [
Therapy for thrombosis prevention in such patients is still debated. Several platelet antiaggregation medications have been proposed, but with no consensus yet formed [
The authors participated equally in the writing of this manuscript.