Acute Presentation of Superior Vena Cava Syndrome: A Stepwise Approach to Management
Mark Tran, MD and Michael E. Mahla, M.D.
Department of Anesthesiology, University of Florida College of Medicine
Introduction: Superior vena cava (SVC) syndrome is often due to
obstruction of the SVC by neoplasms and usually has an insidious clinical
course. However, as iatrogenic or benign causes, including the use of
implanted central venous catheters, are increasing in frequency, we may see
more acute and subacute presentations that require a multimodal approach to
therapy.
Case Presentation: We present a 67-year-old white man with a history of
colon adenocarcinoma diagnosed three years ago. His treatment then included
a hemicolectomy, followed by chemotherapy administered via a right
subclavian central venous access infusaport. Plans for removal of the port
were not followed through, and the patient now presented with a three-month
history of increasing head, neck, and shoulder swelling with facial flushing
and occasional dyspnea. Initial workup, including a CT scan of the chest and
abdomen, reported no tumor or lymphadenopathy. A venogram showed clot from
the proximal SVC extending to the atrial junction with compensatory dilation
of the azygous system. The patient was then referred to our hospital for
removal of his port. Anticoagulation had not been initiated. He was first
seen in the outpatient clinic, where he was noted to have significant neck
and shoulder edema, without respiratory distress.
The following day, our encounter with the patient began when he presented to
the emergency room with increasing dyspnea and diaphoresis. On initial
assessment, the patient was noted to be approximately 80 kg and 5 foot 9
inches tall. Vital signs included a blood pressure of 136/87 mmHg, heart
rate of 110 beats/min, and a respiratory rate around 20 breaths/min. The
patient displayed severe orthopnea, and facial, neck, and shoulder edema
with a violet hue discoloration of the upper torso and neck. Airway
examination showed a mouth opening of 2.5 finger-breath, a large tongue with
a Mallampati IV score, and a thick full neck. The combination his symptoms
and the need for further workup and treatment dictated the need to acutely
secure the patient’s airway. After preparing a difficult airway cart at the
bedside, a 22-gauge peripheral intravenous catheter was placed in the foot.
Adequate topical anesthesia was achieved with lidocaine, and successful
awake orotracheal intubation was performed using an adult-size fiberoptic
bronchoscope and a 7.0 ID endotracheal tube. A portable chest x-ray verified
tube position and the presence of a right subclavian infusaport with the tip
in the distal SVC.
A stepwise approach to therapy was required to address the SVC syndrome.
Anticoagulation was begun with heparin. A spiral CT of the chest ruled out
pulmonary embolism, but a clot was seen surrounding the infusaport catheter
filling the majority of the SVC, propagating into the right brachiocephalic
vein. A thin line of contrast was seen entering the right atrium from the
SVC, with numerous collaterals throughout the right shoulder. A venogram
confirmed the above occlusions. An AngioJet thrombectomy system was used
from the level of the right brachiocephalic vein down into the right atrium,
and a recombinant TPA infusion was started. Venograms repeated the following
two days demonstrated a persistent clot and high-grade stenosis. Residual
thrombus in the SVC was treated with catheter-directed chemical thrombolysis
with TPA infusion at the SVC/atrial junction. However, the patient displayed
minimal clinical improvement, and another venogram was done with
intervention including a balloon angioplasty and port removal. Subsequent
imaging revealed marked improvement in the stenosis. The patient tolerated
the procedure well, and was extubated following significant clinical
improvement over the next several days.
Conclusion: While the majority of SVC syndromes take an insidious clinical
course, those that present acutely with airway compromise may benefit from a
stepwise approach to therapy as exemplified in our case.
