An Unusual Presentation of a Swollen Arm

A Case Report

Monica Kidd; Vina Broderick

Disclosures

J Med Case Reports. 2014;8(26) 

In This Article

Discussion

An informal survey of physicians at our family practice teaching clinic revealed only one other case of subclavian thrombosis in the last 16 years, suggesting that in our patient population such presentations are rare. The annual incidence of UEDVT is estimated to be three per 100,000 persons,[7] therefore MSK disorders are a much more common cause of arm pain and paresthesias. The majority of UEDEVTs (80%) are secondary to the use of central venous catheters and pacemakers, or to conditions such as malignancy, surgery, trauma, immobilization, OCP use, pregnancy or ovarian hyperstimulation syndrome; only 20% are believed to have primary causes related to anatomical abnormalities (such as a cervical rib or subclavian stenosis) or so-called "effort thrombosis" (Paget–Schröetter syndrome).[10] It is not uncommon for patients who present with unilateral UEDVT to have bilateral narrow thoracic outlets.[5]

Although surgical decompression and venous angioplasty may be considered for thoracic outlet obstruction,[4,10,11] the typical treatment for primary subclavian vein thrombosis is oral anticoagulation only.[9] In a recent case–control study in the Netherlands, 45 patients with the condition were treated with: (i) oral anticoagulation only, or (ii) thrombolysis followed by anticoagulation, or (iii) thrombolysis followed by transaxillary rib resection. The study showed that although patients undergoing thrombolysis with or without surgery had less pain, swelling and fatigue in the affected limb at 6 weeks, all patients had the same quality of life at a mean follow-up of 57 months; furthermore, rates of recurrence were similarly low (five instances of recurrence across the three groups).[4] Given similar outcomes for each of the treatments, anticoagulation alone would seem prudent in many cases; in fact, recent guidelines favor anticoagulation only over thrombolysis plus anticoagulation (Grade 2C).[9]

Our patient presented with a 3-week history of shoulder pain and paresthesias limited to the ulnar distribution, followed by an acutely swollen and discolored arm plus paresthesias in the ulnar, radial and median distributions. Initially, our differential diagnosis included cellulitis, lymphedema, and neoplastic compression of the subclavian vein. She had no fever or obvious site of infection to suggest cellulitis; she had had no previous surgery or previous episodes of swelling to suggest lymphedema; and she had no risk factors and displayed no constitutional symptoms to suggest a malignancy. A duplex ultrasound confirmed subclavian thrombosis, and an X-ray and CT ruled out both tumor and a cervical rib as causes for the swelling. CT showed subclavian stenosis on her unaffected side, and venography confirmed bilateral thoracic outlet obstruction with stress maneuvers consistent with Paget–Schröetter syndrome.

Although it seemed this primary anatomical anomaly was the probable cause of our patient's clot, we remained uncertain as to the contribution, if any, of her OCP or her cross-training. She was motivated to continue with exercise, and all of her physicians supported this decision once her INR became therapeutic. Her vascular surgeon supported this approach. She was also considering starting a family soon, and we advised her that she may require anticoagulation with low-weight molecular heparin during future pregnancies. We discontinued her compression sleeve at about 1 month, once her swelling and discoloration had resolved, but vascular surgery recommended she resume using it for at least 12 months, perhaps longer, in order to prevent problems with potential post-phlebitic syndrome.

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