Case Presentation:
We present a case of a 36 year old non-smoking female with history of hypothyroidism, two spontaneous abortions (with negative hypercoagulability workup), no recent travel, or OCP use, who came to the ED with three days of left arm pain, swelling, and bluish discoloration. Patient noticed dull pain of left forearm and arm, 5/10 in intensity at commencement, worsening to 9/10 at presentation, aggravated by movement. Her alarming inability to lift her arm to wash her hair prompted her arrival. Blue discoloration, swelling, tenderness to palpation and inability to abduct arm was noted. Patient denied direct trauma, but was busy shifting homes, and carrying heavy boxes repeatedly over three weeks. Ultrasonography revealed extensive DVT of subclavian, axillary and brachial veins. Patient was started on tPA by infusion catheter began at 1mg/hr, with 7 mg bolus. 24 hours later, reassessment revealed 50% thrombolysis. EKOS catheter was introduced with tPA at 1mg/ hour for 12 hours more, resulting in 100% successful thrombolysis. The greater than 75% narrowing at the junction of first rib and clavicle was dilated. She was discharged on three month Apixaban therapy with one month follow-up for Dynamic Venography and subsequent decompression surgery.
Discussion: Rarely, primary venous thrombosis results by compression of subclavian vein at thoracic outlet due to abnormalities of the first rib, clavicle, costoclavicular ligament or anterior scalene muscle. History reveals repeated overhead arm movements, with venous compression, fibrosis, stasis and thrombosis. Most present with acute pain, swelling following strenuous exertion, and classic aggravation with overhead arm elevation. Edema of the affected extremity, with cyanosis of the hand and fingers is often seen. Dilated collateral veins over upper chest and proximal extremity, Urschel’s sign, is particularly seen with underlying chronic venous stenosis. Ultrasonography is the best initial investigation, with venography recommended when inconclusive. Patients presenting within two weeks of symptom onset undergo thrombolysis, as in this case, to restore patency and minimize endothelial injury. Discharge with anticoagulation for four to six weeks to allow for healing is followed by definitive treatment with thoracic outlet decompression surgery. Although there is limited data on the management of PSS, angioplasty or stenting is strongly discouraged due to high rate of thrombosis. At least three to six months of anticoagulation is required following initial event.
Conclusions:
Effort thrombosis is a rare but significantly debilitating condition seen in otherwise healthy young individuals which can be effectively treated if recognized early and managed appropriately.