Forty seven year old, black female, admitted with subarachnoid hemorrhage grade
III. See CT brain below:

A four-vessel angiogram on admission to the emergency room showed multiple cerebral aneurysms and was transferred to our institution. Her past medical history included untreated arterial hypertension and chronic renal failure. On the second day, she was intubated due to respiratory distress. The same day, the patient underwent clipping of 2 of her 5 aneurysms (anterior communicating and right middle cerebral) without complications.

The above changes were associated with these physical changes:

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1. What is your diagnosis?

2. What is the relation of this entity to syphilis?

3. How are the findings of the CT (on the 15th) associated with this entity?

4. What are the potential complications its therapy?

Answers

1. What is your diagnosis?

A/ This patient has a superior vena cava (SVC) syndrome. On the second day of the admission to the hospital, the patient already required new intravenous access due to clots in the central line placed in the left subclavian on arrival. A massive thrombosis, of the subclavian veins with cephalic and caudal extension to the jugulars and SVC veins respectively, was diagnosed by Doppler ultrasound later on.

The most common clinical symptoms and signs as result of the obstruction are dyspnea, headache, blurry vision, venous congestion of the face and neck with or without cyanosis of the upper body, and facial and upper extremity edema.

2. What is the relation of this entity to syphilis?

A/ The relation is historic and etiological. It was first reported in 1757, in a patient with syphilitic aortic aneurysm. Until the mid 70's, the latter remained one of the main causes of the syndrome along with histoplasmosis and thyroid goiter. Today, the most frequent etiology is malignancy. Among which, lung cancer is the most frequent (> 50% of the cases) followed by malignant lymphoma.

3. How are the findings of the CT (on the 15th) associated with this entity?

A/ Venous obstruction to the drainage of the cerebral blood flow has serious repercussions on the cerebral circulation and blood volume, rising intracranial pressure (ICP) and leading to brain ischaemia.

Almost two weeks after the clipping of the aneurysm, the patient had a change in mental status and respiratory distress requiring mechanical ventilation. This deterioration was associated with the swelling of the right upper extremity, neck, and face (Doppler ultrasound had showed thrombosis the previous day). A CT brain revealed an infarct in the MCA territory opposite to the clipped aneurysm despite that there was no cerebral vasospasm by transcranial Doppler, signs of previous ischaemia by Xenon CT brain scan, or abnormalities in the particular vascular territory by digital substraction angiography (DSA). A ventriculostomy placed to aid in the monitoring of the ICP and therapeutic
he drainage of cerebrospinal fluid, showed an ICP above 40 mmHg.

4. What are the potential complications of its therapy?

A/ The therapeutic modalities are directed to the etiology of the SVC syndrome. The main therapeutic interventions in patients with malignancies are high-dose steroids, to decrease edema and inflammation produced by the tumor, followed by radiation therapy. In patients with lung cancer and non-Hodgkin's lymphoma, combination chemotherapy has shown to be more effective than radiation alone.

In the group with SVC syndrome secondary to thrombosis, tissue plasminogen activator, urokinase, or streptokinase have been recommended. Unfortunately, anticoagulation in a patient with subarachnoid hemorrhage is contraindicated due to the potential for intracranial bleeding. Thrombectomy is not a good alternative because thrombosis recurs despite the necessary adjunctive anticoagulation. Thrombolytic therapy has a success rate up to 70 to 80 %.

Recommended Reading

Ahmed N, Payne RF. Thrombosis after central venous cannulation. Med J Aust 1976;

1:217-20

Birdwell BG, et al. Pseudotumor cerebri: a complication of catheter-induced subclavian vein thrombosis. Arch Intern Med 1994; 154:808-11.

Brima B, et al. Diagnosis of thrombosis by catheter phlebography after prolonged central venous catheterization. Ann Surg 1981; 194:779-83.

Capek P, Cope C. Percutaneous treatment of superior vena cava syndrome. AJR 1989; 152: 183.

Donayre CE, et al. Pathogenesis determines late morbidity of axillosubclavian vein thrombosis. Am J Surg 1986; 152:179-84.

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Haire WD, et al. Thrombotic complications of silicone rubber catheters during autologous marrow and peripheral stem cell transplantation: prospective comparison of Hickman and Groshong catheters. Bone Marrow Transplant 1991; 7:57-9.

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Stephens PH, et al. Superior sagittal sinus thrombosis after internal jugular vein cannulation. Br J Anaesth 1991; 67:476.

Trottier SJ, et al. Femoral deep venous thrombosis associated with central venous catheterization: Results from a prospective, randomized trial. Crit Care Med 1995; 23:52-9.