Painless acrocyanosis: Paget-Schroetter syndrome secondary to thoracic outlet obstruction from muscle hypertrophy

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American Journal of Emergency Medicine

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American Journal of Emergency Medicine 34 (2016) 1323.e1-1323.e3

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Painless acrocyanosis: Paget-Schroetter syndrome secondary to thoracic outlet obstruction from muscle hypertrophy?


Upper extremity deep vein thrombosis is an uncommon and often- times overlooked phenomenon. The presentation may be subtle, especially in the young, otherwise healthy demographic. Review of the literature focuses on secondary causes, most commonly involving sicker patients with multiple underlying comorbidities and in the context of invasive venous catheters. Here, we report a case of a young woman who presented with an extensive upper extremity deep vein thrombosis secondary to thoracic outlet obstruction after just a few days of strenuous exercise.

A 24-year-old Caucasian woman presented for evaluation of her arm “turning blue out of nowhere.” She was in her normal state of health until 2 days before arrival, when she noted her left arm swelling and cyanotic. Initially limited to the distal forearm, it progressed to include the entire extremity to the shoulder. The edema worsened as well. No pain or parasthesias were reported at any time. She denied any trauma or history of similar. There were no symptoms in the other extremities. Furthermore, she denied chest pain, dyspnea, cough, or fevers and denied any recent travel or hospitalizations. Medical, surgical, and social histories were negative. Medications included Sprintec daily for 4 years and acetaminophen as needed. She is a full-time college student and recently joined the swim and track teams. There was no personal or fam- ily history of thromboembolic disease, malignancy, vasculitis, or Raynaud phenomenon. She is right-hand dominant. Vital signs were within refer- ence range. Physical examination revealed a well-appearing female with a benign presentation except a cyanotic left upper extremity to the shoul- der with pitting edema (Fig. 1A and B). Sensations, active and passive range of motion, deep tendon reflexes, and capillary refill were intact. Compartments were soft. There were no petechiae, purpura, or erythema. A duplex venous ultrasound was obtained, showing a partially occluding echogenic thrombus in the left subclavian, axillary, and proximal superficial basilic veins (Fig. 2A-C). The jugular, brachial, antecubital, cephalic, ulnar, and radial veins showed compression, flow, and patency. The arteries showed patent flow as well. (Notably, an ultrasound was performed by the emergency medicine resident at bedside using a SonoSite Edge before the official study, clearly visualizing the occlusive thrombus as well as lack of compression in

the distal subclavian and axillary segments.)

Laboratory data, including a complete blood count, basic metabolic profile, Coagulation studies, and a urine pregnancy test, were within

? There has been no utilization of equipment, drugs, drugs, or grants for this case report. This article has not been presented yet.

normal limits. D-Dimer was 0.91 ug/mL (reference, b 0.49). Blood pressures in both arms were equal. Once the extent of the venous clot was known, a cat scan of the chest with pulmonary embolism protocol was done, showing soft tissue inflammation surrounding enlarged sub- clavian and axillary veins, without evidence of pulmonary embolism, aortic dissection, or any other acute intrathoracic pathology.

The patient was diagnosed with a fairly extensive acute Deep vein thrombosis of the left subclavian and axillary veins secondary to thoracic outlet syndrome from muscle hypertrophy and started on a heparin drip. Before initiation of anticoagulation, a hypercoagulable panel was drawn for completeness. Given the Clot burden, vascular sur- gery and interventional radiology were consulted from the emergency department (ED) but recommended no immediate interventions. The following day, interventional radiology performed an upper extremity venogram and venocavogram, showing complete occlusion of the sub- clavian, basilic, and axillary veins. Alteplase and AngioJet thrombolysis with transluminal angioplasty were instituted. Notably, they found functional occlusion and pinpoint narrowing at the level the first rib with extrinsic compression at the undersurface of the subclavian vein coinciding with the first rib. She was discharged on hospital day 2 with rivaroxaban, with instructions to follow-up with vascular surgery for evaluation of first rib resection.

Of note, she returned 1 month later with left Arm pain. Her rivaroxaban had been decreased from 30 mg daily to 20 mg daily the week prior, and she was scheduled for rib resection. Review of systems and examination were unimpressive. Her oral contraceptives had not been restarted. A repeat ultrasound showed no clots. She was pre- scribed diazepam and morphine for thoracic outlet obstruction, with resolution of symptoms in about an hour.

Upper extremity DVT (UE-DVT) in young, healthy individuals is an uncommon disease. An informal survey of local ED physicians reported no similar cases in over 20 years. They constitute 11% to 14% of all DVTs. Primary thrombosis is either idiopathic or secondary to an obstructive pathology (Paget-Schroetter syndrome or effort-related thrombosis) [1-15].

Paget-Schroetter syndrome is brought on by recent heavy strenuous activity (eg, rowing or weightlifting) in otherwise healthy individuals. The coagulation cascade is activated from repetitive trauma to the inti- ma. Persistent obstruction of the subclavian vasculature (ie, thoracic outlet) leads to fibrosis, and high-intensity athletic activity can lead to muscle hypertrophy and form a mechanical obstruction. Cervical and first ribs, as well as abnormally long transverse processes, have also been implicated. Usually, the dominant arm is affected. Upon further inquiry, she admitted to initiating an intense Training regimen for her school teams 2 weeks before symptom onset, including weightlifting and rowing. Interestingly, the nondominant arm was affected.

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Image of Fig. 1

Fig. 1. A and B, Clinical photographs showing edematous and cyanotic left upper extremity as compared to the right, taken before any intervention.

There is controversy regarding working up idiopathic UE-DVT for underlying thrombophilic disorders. Some experts recommend testing for protein C, S, and antithrombin deficiencies; Factor V Leiden; pro- thrombin gene mutation; hyperhomocysteinemia; and antiphospholipid antibody syndrome. Clinical yield is generally low; incidence/prevalence data of underlying Hypercoagulable states in this population are limited.

Secondary causes are much more common, largely due to placement of central venous catheters (eg, long-term chemotherapy, parenteral nutrition), hemodialysis access, pacemakers, or underlying malignancy. Central venous catheters were found to be the most powerful indepen- dent predictor for UE-DVT development and accounts for up to 55% of all cases. Mechanical obstruction by the catheter and direct injury to the vessel wall are commonly cited etiologies. Risk factors are similar to Venous thromboembolic disease elsewhere. Of note, she fell into the moderate-to-severe risk group when applying Wells’ and Geneva scores.

As with lower extremity deep vein thromboses (LE-DVTs), patient may report none or very mild symptoms, including a low-grade fever. Neurovascular compromise from thoracic outlet obstruction compressing the brachial plexus or subclavian artery may lead to

parasthesias, numbness, or compromised perfusion. With an extensive clot causing superior vena cava (SVC) syndrome, patients may report extremity and facial edema, visual complaints, and dyspnea. Jugular ve- nous distention, varicosities and collateralization, and supraclavicular fullness may also be present. Short- and long-term complications are common. Risk of pulmonary embolism ranges from 9% to 36%. Postthrombotic syndrome, SVC syndrome, septic thrombophlebitis, thoracic duct obstruction, and future loss of vascular access can be problematic.

The rapidity, ease, accuracy, and noninvasive nature of ultrasonogra- phy makes it the ideal initial Diagnostic modality, with sensitivity and specificity for different modes (compression, Doppler, color flow) well over 90%. The major limitation is difficulty evaluating the proximal sub- clavian vein because of the overlying clavicle, with misdiagnosis rates as high as 18%. Contrast venography affords better visualization and is the criterion standard. However, it mandates radiation and contrast exposure. magnetic resonance angiography provides the most informa- tion, is safe and noninvasive, and can be used as an alternative to venog- raphy. Both computed tomography and magnetic resonance imaging are also excellent modalities for evaluating potential underlying

Image of Fig. 2

Fig. 2. A to C, Duplex venous ultrasound of the left subclavian, axillary, and basilic veins.

A.B. Norinsky et al. / American Journal of Emergency Medicine 34 (2016) 1323.e11323.e3

pathology (eg, mass or adenopathy). Light reflection rheography, phleborheography, and impedance plethysmography are also options,


Alexander B. Norinsky, DO? James Espinosa, MD

but oftentimes not readily available.

The current guidelines from the American College of Chest Physi- cians recommend managing UE-DVT in the same manner as LE-DVT, that is, anticoagulation with heparin or fondaparinux with bridge to warfarin (level I evidence). A minimum of 3 to 6 months is usually recommended, although there is a paucity of evidence for the optimum duration. As yet, there have been no studies for the efficacy of novel oral anticoagulant agents. Thrombolytics have been shown to improve results and decrease long-term complications. Catheter-directed intravenous thrombolytics are preferred over systemic thrombolytics, with minimal risk of clinically significant hemorrhage (of note, our pa- tient experienced a brief episode of clinically insignificant hematuria after thrombolytics, which resolved on its own without intervention). Although studies have evaluated very specific populations, it is generally held that the best candidates are young, otherwise healthy patients with clots less than 2 weeks old or those with symptomatic SVC syndrome. Continuous infusion of tissue plasminogen activator for 8 hours is the usual schedule, although other agents and Infusion protocols (eg, “pulse-spray”) have been studied with positive results. Success rates are usually determined by the extent of clot burden and the clot freshness, ranging from 100% (if initiated within a few days) to as low a 22% to 25%. Mechanical thrombectomy can be used in addi- tion to thrombolytics; the very limited literature available supports its efficacy. A superior vena cava filter can be considered if there is a contra- indication to anticoagulation and thrombolytics, although that too is fraught with multiple complications.

surgical interventions are limited and focus on decompression and

rectifying the underlying obstruction after anticoagulation/thrombolytics have been given. Options include clavicular or first rib resection, removal of the costoclavicular ligament, anterior scalenectomy, venolysis, and/or Surgical thrombectomy. Surgical interventions are regionalized nationally. Repeat ultrasonography or venography in the neutral and military positions at set intervals is recommended to visualize resolution of

vein compression.

We report of a young, healthy female on long-term oral contra- ceptives who developed an extensive UE-DVT secondary to thoracic outlet obstruction after strenuous exercise. This case highlights the need to maintain a broad differential, especially when encountering uncommon complaints and diagnoses in the ED, as well as emphasiz- ing the ease and efficiency of using bedside ultrasonography for evaluating extremity VTE. There has been a movement recently for discharging acute uncomplicated LE-DVTs with novel oral anticoag- ulant agents; whether the same future holds for UE-DVTs remains to be seen.

Department of Emergency Medicine, Rowan University SOM Kennedy

University Hospital, Stratford, NJ, USA

?Corresponding author. Department of Emergency Medicine, Rowan University SOM Kennedy University Hospital, 18 East Laurel Road

Stratford, NJ 08084. Tel.: +1 646 241 5695

E-mail address: [email protected]

Majid Kianmajd, DO

Department of Surgery, Rowan University SOM Kennedy University

Hospital, Stratford, NJ, USA

Francesca DiLeonardo, MD

Department of Emergency Medicine, Rowan University SOM Kennedy

University Hospital, Stratford, NJ, USA


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