Combined non-surgical treatment for Paget–Schröetter syndrome: a case report

Personal and family history of the patient

A 38-year-old white woman presented with swelling and pain in her right upper limb
that had begun 48 hours previously and that was unrelated to trauma. She was a keen
amateur swimmer and had increased training in the days before the onset of the swelling.
She had a history of bilateral shoulder recurrent luxation during childhood, which
led to an operation on her left shoulder when she was 24-years old. Her history did
not reveal any risk factors for venous thromboembolic disease (VTE): she had not recently
travelled or had prolonged immobilization, nor had there been any recent surgery (the
shoulder surgery occurred 14 years prior and was on her left shoulder, which was not
the site of the swelling and pain that precipitated this case). Furthermore, she had
no family history of thromboembolic disease or thrombophilia and was not taking oral
contraceptive pills. The only historical detail of interest was bilateral recurrent
shoulder dislocation in childhood, although there was no episode prior to the presentation
of this PSS.

Physical examination and other tests

There were no signs of arterial disease; humeral, radial, and ulnar pulses on her
upper limbs were present and good capillary filling was observed without skin changes.
An examination revealed an increased diameter of her right upper limb, as well as
an edema in all her right upper limb and deltopectoral collateral circulation. Upper
limb mobility and sensitivity were normal and preserved, without paresthesia or dysesthesia
that could be suspicious for nerve or arterial compression. Following the protocol
of our center, ultrasound tests were performed to detect deep venous thrombosis (DVT),
to evaluate compression, occupation of light, and color flow, and Doppler ultrasound
was used to assess the phasic flow. The duplex ultrasound revealed a lack of compressibility,
permeability, and phasic flow in the middle third and proximal region of her subclavian
vein; right subclavian vein thrombosis was diagnosed. Computed tomography angiography
(CTA) of her supra-aortic trunks, thorax, and upper extremities confirmed thrombosis
in her right subclavian vein, just below her collarbone and her first rib (Fig. 1). Multiple collateral veins in her right upper limb were observed, which enlarged
the limb compared with her contralateral limb. No other findings of interest were
noted.

Fig. 1. Computed tomography angiography scan of right shoulder

Treatment and follow-up

Venography was performed via her right cephalic vein, confirming thrombosis, a profuse
collateral network, and proximal subclavian vein patency (Fig. 2). With a multi-side-hole catheter placed in the thrombus, a 250,000 IU urokinase
bolus was administered for local fibrinolysis, followed by a continuous perfusion
of 100,000 IU/hour for 24 hours.

Fig. 2. Venography confirmed thrombosis, a profuse collateral network, and proximal subclavian
vein patency

After 24 hours, venography showed partial recanalization of the thrombus and a persisting
moderate residual stenosis (Fig. 3). Percutaneous transluminal angioplasty of the stenosis was performed with a 6×40
mm balloon.

Fig. 3. Venography 24 hours later showing partial recanalization of the thrombus and a persisting
moderate residual stenosis

After a further 24 hours she was showing clinical improvement and was discharged.
During the first week ambulatory treatment with low molecular weight heparin (LMWH)
was provided at a therapeutic dose; for the subsequent 3 months ambulatory treatment
comprised prophylactic doses of LMWH together with an elastic compression sleeve and
physiotherapy. She was advised on preventive measures; she was recommended to abstain
from exercises involving the upper extremity and swimming (identified as the precipitant
element). This was combined with in-hospital and at-home physiotherapy, which provided
advice and education on exercise and lifestyle modifications.

She was followed-up via ambulatory consultation (after 15 days, 1 month, 3, 6 and
12 months, and then yearly) to monitor her return to normal/working life and physical
condition (including eco-Doppler). At the 6-month follow-up, the permeability of her
subclavian vein was examined by ultrasound: clinical improvement had been maintained
with no swelling and no functional impact; duplex ultrasound demonstrated subclavian
vein patency.