Upper-extremity deep vein thrombosis: limits and frontiers. (Editorial).

Upper-extremity deep vein thrombosis (DVT) is a fairly uncommon syndrome of complex etiology and varied presentation. Its incidence has recently grown, and it has become more widely recognized in diverse clinical settings. Clinical reviews and prospective studies have clarified some aspects of the clinical presentation and setting and have alerted clinicians to the significant morbidity and risk of pulmonary embolization that exist in patients with this condition. Still, upper-extremity DVT remains a source of clinical bafflement to many physicians who, in dealing with patients with this condition, generate a steady stream of questions and referrals to vascular surgeons, hematologists, radiologists, and other specialists with presumed expertise in the diagnosis and management of this disease. From whence came this state of affairs?

In this issue of the Southern Medical Journal, Shah et al' present a review of upper-extremity DVT. They call attention to potentially serious consequences of untreated upper-extremity DVT and to the fact that indwelling catheters are a major cause of upper-extremity DVT, and they recommend that it always be treated with anticoagulation.

One must appreciate that DVT of the upper circulation tends to present and be recognized in three different settings. Effort-induced DVT of the upper extremity, the so-called Paget-Schroetter syndrome, occurs in younger individuals who engage in strenuous exercise such as weightlifting, especially when accompanied by preexistent anatomic abnormalities of the thoracic outlet. Alternatively, it can unexpectedly develop in association with an inherited or acquired thrombotic tendency. Some investigators have concluded on the basis of retrospective reviews that patients with an underlying hypercoagulable state comprise the majority of patients with this condition and therefore have recommended anticoagulation for all patients with upper-extremity DVT, (2) but other experts disagree. (3) Patients with indwelling central venous devices also frequently develop catheter thrombosis, often with thrombus extension beyond the catheter lumen. Fortunately, this variant is usually fairly easy to recognize. Thrombolysis is o ften attempted in these latter situations. Whether routine primary prophylaxis for patients with indwelling central venous devices is indicated is another contentious issue.

The diagnosis of upper-extremity DVT can be complicated and puzzling, with presentations ranging from severe, distressing pain, discoloration, and swelling to subacute discomfort and mild, difficult-to-characterize aching of an upper extremity. Advances in diagnostic modalities have led to an increasing rate of diagnosis; still, many cases are not recognized and treated promptly. Contrast venography is the "gold standard" diagnostic imaging modality, but it is costly and invasive to perform. Noninvasive modalities possess sensitivity and specificity close to that of venography. (4) The American Thoracic Society has developed a set of guidelines for the diagnosis of DVT that conclude that upper-extremity DVT may be diagnosed on the basis of ultrasonography, contrast venography, or magnetic resonance imaging. Compression techniques are used when ultrasonography is performed, but portions of the subclavian vein behind the clavicle cannot be compressed, and thus greater reliance on Doppler evaluation is required. The internal jugular, subclavian, axillary, and brachial veins are generally accessible, but the superior vena cava and brachiocephalic vein are inaccessible or only partially accessible with the use of ultrasonography. (5)

Unfortunately, long-term sequelne of upper-extremity DVT often remain poorly appreciated, leading to widely divergent estimates of morbidity in the literature. Thus, there is no agreement regarding the incidence, significance, and risk of complications. Not surprisingly, one finds widely divergent recommendations in the literature, ranging from surgical embolectomy, thrombolysis, and heparin therapy to symptomatic treatment alone. Although traditionally the incidence of pulmonary embolisms in upper-extremity DVT was thought to be in the 4 to 7% range, reported rates of asymptomatic pulmonary embolus in some series are as high as 36%.6 Clearly, the perceived likelihood of pulmonary embolism and postphlebitic complications has a direct bearing on whether to admit a patient for intravenous anticoagulation therapy or whether to attempt to manage upper-extremity DVT with low molecular weight heparin and rapid coumadinization on an outpatient basis.

What, then, can be agreed on? Certainly, not all cases are the same or need to be treated in the same fashion. The patient's age and the presence of comorbidities, the suspicion or diagnosis of a procoagulant state, or the presence of an indwelling catheter at the site of thrombosis should bear substantially on the choice of treatment. Some cases, such as those brought on by upper-extremity exercise in a young patient with minimal symptoms and signs, may respond well to symptomatic treatment alone and the avoidance of the precipitating causes; others may require aggressive anticoagulation and even thrombolysis.

Given the imperfect state of knowledge of upper-extremity DVT, the art of medicine must perforce trump the science of medicine--for now. It must remain to be hoped that ongoing research, advances in diagnostic imaging, and better case reporting ultimately lead to better characterization of this uncommon but important syndrome so that physicians can base their treatment recommendations on surer footing.

Accepted May 5, 2003.

References

(1.) Shah MK, Burke DT, Shah SH. Upper-extremity deep vein thrombosis. South Med J 2003;96:668-673.

(2.) Ellis MH, Manor Y, Witz M. Risk factors and management of patients with upper limb deep vein thrombosis. Chest 2000;117:43-46.

(3.) Martinelli I, Cattaneo M, Panzeri D, Taioli E, Mannucci PM. Risk factors for deep venous thrombosis of the upper extremities. Ann Intern Med 1997;126:707-711.

(4.) Prandoni P, Polistena P, Bernardi E, Cogo A, Casara D, Verlato F, et al. Upper-extremity deep vein thrombosis: Risk factors, diagnosis, and complications. Arch Intern Med 1997;157:57-62.

(5.) Tapson VF, Carroll BA, Davidson BL, Elliott CG, Fedullo PF, Hales CA, et al. The diagnostic approach to acute venous thromboembolism: Clinical practice guideline--American Thoracic Society. Am J Respir Crit Care Med 1999;160:1043-1066.

(6.) Ault M, Artal R. Upper extremity DVT: What is the risk? Arch Intern Med 1998;158:1950-1952 (letter).

From the Lincoln Medical and Mental Health Center, Bronx, NY, and Weill College of Medicine, Cornell University, New York, NY.

Reprint requests to Mark Levin, MD, Lincoln Medical and Mental Health Center, 234 E. 149th Street, Bronx, NY 10451.

Copyright [C]2003 by The Southern Medical Association

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