Case Presentation: An otherwise healthy 36 year-old presented to the emergency room with increasing pain and swelling of her left upper extremity, progressively worsening over the last week. Physical examination showed significantly enlarged left upper extremity with tenderness and erythema. Further evaluation showed presence of left subclavian, internal jugular vein DVT. A CT-angiogram performed for mild dyspnea on exertion revealed concomitant right lower lobe segmental pulmonary embolism. The patient denied any recent trauma, surgery, intravascular lines. She denied any history of malignancy and family history of veno-thromboembolic disease. Hypercoagulable work up was negative as well. Patient was a hair dresser by occupation and it was felt that her UEDVT was secondary to Effort thrombosis due to strenuous and repetitive activity of the upper extremity in the setting of a cervical rib. She was started on anticoagulation and referred to vascular surgery for thrombolysis evaluation. She was also sent to sports medicine specialist as part of multimodal approach required to appropriately manage this uncommon presentation.
Discussion: Upper extremity deep venous thrombosis (UEDVT) accounts for only 5-10% of total cases of DVT. These cases are increasing in incidence due to increased used of intravenous catheter use. Other predisposing factors include malignancy, thrombophilic states etc. We present here a rare case of an UEDVT secondary to effort thrombosis (“Paget-Schroetter Syndrome”) in otherwise healthy young female with no usual predisposing factors.Effort thrombosis is a rare and unusual primary cause of UEDVT seen mainly in athletes and in physically active individuals with unusual strenuous use of the arm and shoulder. It is believed that the retroversion, hyperabduction and extension of the arm involved in strenuous activities imposes strain on the subclavian vein leading to micro-trauma of the endothelium and activates the coagulation cascade. Our patient’s occupation as a hair dresser predisposes her to similar mechanism due to repeated arm extension movements. While Doppler ultrasound remains a preferred initial test, MR venography has highest sensitivity and specificity among all non-invasive diagnostic modalities. It is important to note that anticoagulation alone remains suboptimal in the management of effort thrombosis. Multimodal approach including catheter-directed thrombolysis, vascular surgeries if indicated, thoracic decompression surgery if present followed by rehabilitation is recommended. This aggressive approach mitigates the risk of an unacceptably high incidence of residual symptoms, disability and recurrent VTE reported with conservative treatment.
Conclusions: While effort thrombosis has been described widely in literature, this is a rare presentation in a hair dresser. It emphasizes the importance of occupational history in cases of VTE. In addition, given the rarity of the condition, and the paucity of literature, it is important for physicians to recognize the importance of the multimodal approach as management strategy for effort thrombosis.