Primary thrombosis of the subclavian vein at the costoclavicular junction. The formation of an axillo-subclavian vein thrombosis results from endothelial trauma, often as a result of repetitive activity of the upper limbs.
Incidence 1-2/100,000; average early 30s; male predominance (2:1); right sided (correlated with hand dominance); 60-80% direct association with vigorous exercise or activity involving the upper extremities
Thoracic outlet syndrome (TOS) comprises three variants. Neurogenic (NTOS), venous (VTOS) and arterial (ATOS). VTOS is further divided into intermittent/positional venous; obstruction, secondary subclavian vein thrombosis (e.g. catheters/pacemaker leads); and primary ‘effort thrombosis’ (Paget-Schroetter Syndrome)
Classification of thoracic outlet syndrome
Basic classification of thoracic outlet syndrome. Intermittent positional venous obstruction is felt to be a precursor of effort thrombosis if not recognized and treated. The exact incidences of these conditions is unknown, but their relative frequencies are shown, most common to least common, reading left to right. TOS, Thoracic outlet syndrome. [Illig KA, Doyle AJ. J Vasc Surg. 2010 Jun;51(6):1538-47 [Open Access]]
Algorithm for treatment of patients with partial or complete effort thrombosis
Algorithm for treatment of patients with partial or complete effort thrombosis. The best initial procedure (more accurately, the chances of success) is defined by the duration of symptoms, whereas the subsequent method of thoracic outlet decompression is defined by the status of the residual vein and residual symptoms. Timing of decompression is not defined in our protocol, although we believe decompression should immediately follow thrombolysis. [Illig KA, Doyle AJ. J Vasc Surg. 2010 Jun;51(6):1538-47 [Open Access]]
History of Paget-Schroetter Syndrome
1816 – Jean Cruveilhier (1791-1874) provided an early description in his 1816 doctoral dissertation ‘Essai sur l’anatomie pathologique en général, et sur les transformations et productions organiques en particulier‘
J’ai plusieurs fois rencontré des veines remplies dans toute leur étendue de caillots trrès-consistans, très-adhérens à la membrane interne, sans que j’aie pu soupçonner d’autre cause de ce phénomène…une fois toutes les veines du bras remplies de pus solide, et la membrane interne très-rouge chez un individu vigoureux… Le bras avoit acquis un grand volume; tout le tissu cellulaire sous-cutané étoit infiltré.
“I have several times seen veins filled in all their extent with very consistent clots, very adherent to the internal membrane, without my having been able to suspect any other cause of this phenomenon… once all the veins of the arm filled solid, and the inner membrane very red in a fit, healthy individual… the arm had acquired a large volume; all subcutaneous cellular tissue was infiltrated. “
1858 – Sir James Paget described ‘some affections of voluntary muscles as a consequence of excessive exercise’ with reference to four cases of enlargement of the upper limb appearing to be associated with ‘dilated or varicose state of the veins’
In another case, in a soldier 27 years old, there was great enlargement of all the muscles of the right arm and shoulder, and such fulness of all its subcutaneous veins, and such a feeling of weight and tightness when it hung down, that I supposed the axillary vein must be obstructed. However, with rest in the Hospital, and the occasional application of leeches, the muscles gradually diminished, and the veins be came less filled; and finally, during an attack of scarlatina, they returned to nearly their natural state.Paget, Medical Times and Gazette. March 1858
1875 – Paget described in greater detail two of these cases in the chapter “gouty phlebitis” in his collection of Clinical lectures and essays. This is generally taken as the first description of effort thrombosis, despite a clear reference to the original publication of the cases from March 1858.
In strong contrast with cases of widely diffused phlebitis, are those in which a single small portion of a great vein becomes obstructed…
A man about 50 years old, thin, and moderately muscular, and usually healthy, observed, during a September, that his right arm was growing larger, and, as he thought, stronger, and fitter for work…I found the upper arm two inches more in circumference than the left, and the whole limb enlarged in the same proportion.
With the help of the hot douche, warmth, and friction, the swelling of the arm very gradually subsided; and, as it did so, the cord-like feeling of the obliterated axillary vein became more distinct. A year elapsed before the vein regained its completely natural condition.Paget, ‘Gouty Phlebitis’ 1875
1884 – Leopold Von Schrötter postulated that the upper limb swelling in otherwise fit and healthy individuals resulted from direct damage to the vein caused by stretching that occurred with muscular strain of exercise.
1948 – English surgeon, ESR Hughes termed the condition Paget-Schroetter syndrome
The association of acute venous stasis and a healthy person constitutes a syndrome which, in the absence of unanimity of opinion as regards the aetiology or pathology, might well be called the ‘Paget-Schrotter syndrome’.Hughes ESR, 1948
- Jean Cruveilhier (1791-1874)
- James Paget (1814–1899)
- Leopold Von Schrötter (1837-1908)
- Rudolph Matas (1860 – 1957)
- Paget-Schrötter syndrome; Paget-von-Schrötter syndrome (Hughes 1949)
- Effort thrombosis
- Traumatic thrombosis of the axillary vein
- Intermittent venous claudication (Lohr, 1933; Harms, 1938)
- Venous stasis (Hauge, 1942)
- Cruveilhier J. Essai sur l’anatomie pathologique en général, et sur les transformations et productions organiques en particulier. 1816
- Paget J. On some affections of voluntary muscles III. Medical Times and Gazette. 1858: 260-261
- Paget J. Gouty Phlebitis. In: Clinical lectures and essays. 1875: 305-307
- Schrötter L. Erkrankungen der Gefässe In: Specielle Pathologie und Therapie v.15:2 (1884) Prof Dr H. Nothnagel: Wien, Hölder
- Matas R. Primary Thrombosis of the Axillary Vein Caused by Strain, Am. J. Surg. 1934; 24(3): 642-666
- Hughes ESR. Venous obstruction in the upper extremity (Paget- Schroetter’s syndrome). Br J Surg. 1948; 36: 155-63.
- Hughes ES. Venous obstruction in the upper extremity; Paget-Schroetter’s syndrome; a review of 320 cases. Surg Gynecol Obstet. 1949; 88(2): 89-127.
- Illig KA, Doyle AJ. A comprehensive review of Paget-Schroetter syndrome. J Vasc Surg. 2010; 51(6): 1538-47
- Top 100 Ultrasound – Ultrasound Case 085
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