MedPix® Case: 5086 - Paget-von Schrotter Syndrome

38.107.191.114

MedPix® Teaching File Case No: 5086

Case Summary: 5086 - || - - || - -

Contributed by: Kevin F. McCarthy ( National Naval Medical Center Bethesda ) - || -

Approved by: Philip A Dinauer ( Civilian Medical Center ) - || -

Diagnosis

Paget-von Schrotter Syndrome

Dx confirmed by

* Not Provided by Author *

Demographic

Age: 17 - Gender: girl -

History

Athletic girl with acute onset of pain and swelling in the right upper arm. No history of trauma.

Exam and Lab

* Not Provided by Author *

Images

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Paget-von Schrotter Syndrome

Findings

Venous occlusion of the right subclavian and axillary veins with large collateral vessels.

Differential

* Not Provided by Author *

Patient Discussion

See factoid.

Treatment and Followup

Pulse infusion with tTissue Plasminogen Activator followed by overnight infusion was performed. No changes were effected.

Disease Topic

Paget-von Schrotter Syndrome

Disease Discussion

The clinical presentation of pain and swelling in the upper extremity due to occlusive thrombosis of the subclavian and axillary veins was given the name Paget-von Shrotter syndrome in 1949 by Hughes in tribute to the work of Sir James Paget in 1875 and von Shrotter in 1884. Sir James Paget first described thrombosis of the subclavian vein while von Schrotter theorized that the clinical presentation was, in fact, due to venous thrombosis. The syndrome is also known as “effort-induced thrombosis” as it usually presents in a dramatic and unexpected manner in young and otherwise healthy patients. Frequent strenuous arm movements from such activities as playing baseball or tennis precipitate the syndrome. The pathophysiology of effort-induced thrombosis is multifactorial. It involves compressive changes in the vessel wall, stasis of blood, and hypercoagulability. The onset of acute pain and swelling is typically in the dominant arm (80%) and worsened with activity. Other causative factors include trauma and the presence of an indwelling central venous catheter.
Ultrasonography is the screening test of choice. It is not very sensitive. If negative, but strong clinical suspicion remains, other imaging modalities should be used.
Venography is the most sensitive and specific study for diagnosis. One should be mindful of the risks of contrast-induced side effects.
Acute thrombosis is treated aggressively with thrombolysis 5 – 7 days after presentation. Conservative measures are associated with greater residual disability. Chronic thrombosis, however, does not respond well to thrombolysis and is better treated with conservative measures or bypass vice thrombectomy. In addition to bed rest and limb elevation this may include anticoagulation with warfarin. Serial venography with balloon angioplasty and/or stenting of the subclavian vein may reduce symptoms in these patients. In the presence of anatomic abnormalities surgery may be a preferred treatment option.
Complications: Pulmonary embolism occurs in 20 – 36% of patients. Other complications include Superior vena cava syndrome, thoracic outlet obstruction, pulmonary hypertension and chronic venous insufficiency.

Reference(s)

* Not Provided by Author *

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