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Dr Bistritz is a pediatric nurse practitioner, Montefiore New Rochelle Hospital, New Rochelle, New York.
A 15-year-old female presents to the emergency department of a community hospital with acute onset of duskiness in her left arm.
A 15-year-old female presents to the emergency department (ED) of a community hospital with acute onset of duskiness in her left arm. She was sitting in class when she noted the sudden color change in her arm accompanied by stiffness and swelling. She did not report any pain in the left arm and there were no symptoms in her right arm. She has never had any similar episodes in the past and has had no recent trauma to her arm or neck. She is not taking any medications and has no known allergies. The patient is on the school softball team and earlier had been practicing at a batting cage.
On presentation to the ED, the patient’s vital signs included a temperature of 98.4°F; heart rate of 67 beats per minute; respiratory rate of 18 breaths per minute; blood pressure of 106/55 mm Hg; and oxygen saturation of 100% on room air. Physical exam of the left upper extremity revealed a full range of motion with mild stiffness, capillary refill of <2 seconds, mild decrease in skin temperature as compared with the right arm, and mild cyanosis. All pulses were 2+ and equal, and radial and ulnar compression tests were both normal. No crepitus or deformity existed of the affected limb. There was full range of motion of elbow and wrists, with intact tendon reflexes. The remainder of the physical exam was within normal limits.
The girl’s family reported a history of leg clots in her mother and maternal grandmother, but denied a family history of autoimmune disease.
Laboratory studies revealed a normal complete blood count, prothrombin time, partial prothrombin time, and comprehensive metabolic panel. The international normalized ratio (INR) was 1.04 (normal, 0.70-1.10) and a D-dimer test was obtained. The D-dimer results were elevated at 292 D-DU ng/mL (0-230 D-DU ng/mL). A duplex ultrasound was ordered (Figure 1) and the patient was transferred to a tertiary care center for further treatment.
Pallor and upper extremity edema in a young adult needs to be evaluated in the context of chronicity of symptoms (Table).1-5 A long history of swelling would be more consistent with a lymphatic or arterial abnormality. Congenital or posttraumatic anomalies would not generally present acutely. Additionally, mediastinal masses, causing venous compression, can present as unilateral edema in the absence of thrombosis but it is more likely to cause bilateral arm and facial swelling. The acute onset of swelling is almost always venous in nature and typically is related to venous thrombosis. Ultrasound imaging can confirm the presence of a thrombus in the venous system.
Based upon the acute presentation in this patient, the diagnosis of upper extremity deep-vein thrombosis (UEDVT) was believed to be the most likely etiology. The presence of thrombus in the central venous system was confirmed by ultrasound.
Although UEDVT is unusual in a young person, it was important to confirm this first as delay in treatment can lead to increased morbidity and loss of function and future productivity. To pinpoint the etiology, further imaging and consultation with vascular surgery was required.
Duplex ultrasonography of the patient’s left upper extremity revealed an occlusive thrombus involving the subclavian, axillary, and brachial vein to the level of the mid-arm (Figure 2). Further imaging with computed tomography (CT) angiography revealed compression of the neurovascular structures by the first rib.
The patient was admitted for management of her left UEDVT. Therapy with enoxaparin was initiated urgently while awaiting evaluation by vascular surgery. On hospital day one, left upper extremity thrombolysis and balloon angioplasty were performed. The procedures were uncomplicated and complete recannulation was achieved. A hypercoagulable workup was unremarkable. Genetic testing also was performed. The enoxaparin dose was titrated over the next 3 days and the patient was discharged on enoxaparin twice daily. Instructions were given to suspend all repetitive upper extremity activities.
Deep-vein thrombosis in the upper extremity accounts for only 2% to 10% of all thrombotic events and is an unusual finding in a patient without previous trauma or damage to the area.1,2 This finding triggered further investigation to ascertain the etiology of the thrombus.
Thoracic outlet syndrome (TOS) is a term used to describe signs and symptoms that arise from compression of the neurovascular bundle (including the brachial plexus, the subclavian artery, and subclavian vein) by various structures in the area just above the first rib and behind the clavicle, within the confined space of the thoracic outlet.3 There are 3 types of TOS: neurogenic, from brachial plexus compression; venous, from subclavian vein compression; and arterial, from subclavian artery compression. The symptoms noted with each type of compression are distinct. Compression of the brachial plexus causes upper extremity numbness and weakness; venous compression causes deep vein thrombosis and extremity swelling; and arterial compression causes distal thromboembolism, arm pain with exertion, or arterial thrombosis.4
Compression in the thoracic outlet area can result from a combination of congenital abnormalities, injuries, and physical activities. Variations of thoracic outlet anatomy can be congenital or acquired. Congenital examples include anomalous ribs and fibrocartilaginous bands, whereas acquired may arise from chronic inflammatory changes, masses, or development of bony malformation secondary to trauma.5
Venous TOS is the second most common type after neurogenic and accounts for 5% of the cases. Upper extremity DVT can be divided into primary and secondary etiologies. Primary is also known as Paget-Schroetter syndrome or effort thrombosis. The symptoms typically occur in young patients who are engaged in sports activities or whose professions require them to perform vigorous repetitive exertion of the upper extremities, usually with the arms above shoulder level. The repetitive motion produces recurrent venous compression, which leads to perivascular fibrosis, focal stenosis, and subsequent thrombosis.6 Secondary UEDVT is related to a predisposing factor, such as previous peripherally inserted central catheters (PICC lines), subclavian vein cannulation, or malignancy.7,8
Upper extremity edema is the hallmark of venous TOS. The diagnosis is supported by the demonstration of stenosis or occlusion of the corresponding subclavian vessel in a patient with the appropriate clinical history. Ultrasonography is the imaging test of choice because it is inexpensive and noninvasive. Duplex ultrasound is highly specific and sensitive for venous thrombosis of the axillary and brachial veins. Nonthrombotic stenosis of the subclavian vein can be hard to detect with ultrasound as the affected potion of the subclavian vein is behind the clavicle. Evaluation of a predisposing anatomic anomaly is important to identify so that earlier invasive therapy can be considered to diminish recurrence and improve long-term prognosis.
To identify the point of vascular compression, CT studies can help to reveal the relationship of the vascular structures to the surrounding muscles and bone.9 However, venous compression is only present when the arm is in the abducted position. A CT scan performed with the arm in the resting position may underestimate the degree of narrowing at the thoracic outlet. An arm venogram in the abducted and nonabducted position can reliably confirm the presence of significant vascular compression.10
Treatment of subclavian thrombosis with catheter-directed thrombolysis is currently accepted as the preferred initial treatment. Subsequent anticoagulation for 1 to 3 months following successful thrombolysis is recommended to allow endothelial healing and resolution of inflammation.11 Because of the risk of reocclusion, decompression surgery in patients with vascular TOS is advocated. The choice of the procedure depends on the anatomic anomaly; however, resection of the first rib is the treatment of choice.5,12,13 Patients with venous TOS who experience successful thrombolysis and subsequent decompression have 5-year secondary vein patency rates greater than 95% with successful clinical outcomes.12,13
The patient in this case is currently undergoing 3 months of anticoagulation therapy and at its conclusion she is scheduled for surgery to resect the first rib. All genetic testing was negative. She is asymptomatic and has full function of her upper extremities.
It is estimated that 60 million children aged 6 to 18 years are currently participating in organized athletic activity.12 Among these athletes, approximately 2.6 million children are seen in EDs for sports-related injuries each year.14 Although more commonly injuries present as sprains, fractures, and concussions, clinicians should also be alert for heat-related illnesses and repetitive motion injuries.15
Venous thrombosis related to the thoracic outlet impingement in this case was triggered by the repetitive swinging motion of batting, but may present more subtly in pitchers and tennis players. Considering TOS in the differential of UEDVT can reveal an underlying anomaly that requires prompt attention.
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14. Centers for Disease Control and Prevention. Nonfatal traumatic brain injuries related to sports and recreation activities among persons ≤ 19 years-United States, 2001-2009. MMWR Morb Mortal Wkly Rep. 2011;60(39):1337-1342.
15. National Institute of Arthritis and Musculoskeletal and Skin Diseases. Childhood sports injuries and their prevention: a guide for parents with ideas with kids. NIH Pub. 2006. 06-4821.