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Venous thrombosis of the upper limbs
(Portuguese PDF version)

Waldemy Silva^*

* Retired professor, Universidade de Pernambuco (UFPE), Recife, PE, Brazil.

 

Correspondence:
Waldemy Silva
Rua Samuel Pinto, 90/202
CEP 50050-240 - Recife, PE, Brazil
Tel: +55 (81) 3221.1634
Fax: +55 (81) 3221.1634
E-mail: waldemysilva@terra.com.br

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ABSTRACT

Clinical course of 52 patients with axillary and/or subclavian vein thrombosis was reviewed. In the author's opinion, up to the present time we have no evidence of strain in the pathogenesis of this topographic vein thrombosis. The term Paget-Schrötter syndrome can be used when a thrombus is present, as these authors have suggested. With regard to the patients whose clinical picture is supported by an extrinsic compression on the venous trunks, another syndrome must be considered, such as the thoracic outlet syndrome. For the determination of a clinically suspected deep venous thrombosis, phlebography is the gold standard. The optimal treatment for the axillary-subclavian venous occlusion remains to be established, but the anticoagulant therapy has the preference. The efficacy of in situ thrombolytic effect is contested in medical publications. A direct surgical access for thrombectomy can be made only under special conditions.

Key-words: venous thrombosis, thoracic outlet syndrome, phlebography.

J Vasc Br 2005;4(4):371-82

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In 1965 I wrote a thesis to apply for professor for the discipline of Vascular Surgery at the Faculdade de Medicina da Universidade de Pernambuco (UFPE), called "Paget-Schrötter syndrome: clinical and phlebographic study".¹

At that time, I made a reference to the fact that Paget had described his two cases as gouty phlebitis, with clinical data attributed to a "thrombosis of the main veins of the upper limb".²

Similarly, I cited and described in a literal translation the clinical observation made by Schrötter,³ which I transcribe here, due to the different references I have seen in some publications. "I want to report here a rare, clear case of right subclavian vein thrombosis. A 42-year-old man, currently a painter, who did not have a major disease, in 1884 noticed that his right arm suddenly swelled, without an evident cause, constantly and progressively, according to the patient's opinion, maybe due to a distension occasioned by the efforts he used to make at that time as a worker in a railroad. The skin turned red, and there was an insensitivity until the hand. The situation was indeed to be feared, once Billroth recommended the limb amputation. Little by little the swelling reduced, and a clear dilation of the veins could be seen. The vessels appeared more clearly in the right upper extremity and they were, so to speak, rolled like calibrated pencils. The veins, dilated and sinuous, are in the shoulder edges at the height of the third and fourth ribs and go towards the anterior wall of the thorax on the mentioned side and are distributed over the sternum, in a network that goes thinner and swells at each coughing fit. The fact that the jugular veins, the other neck veins and the veins located behind the shoulder were not dilated gives rise to the exact prediction of a thrombosis of the subclavian vein in the confluence with the cephalic vein. Finally, he affirms: "Once the other causes cannot be determined, we have the fact that the thrombosis was probably established due to a particular stretching of the venous wall, due to a given muscle distension".

As can be inferred from the description above, the author did not have concrete data to confirm the origin of the thrombosis. He only proposed the hypothesis of an effort made 7 years ago, suggested by the patient himself, having produced a stretching of the venous wall. The picture included in the publication is highly suggestive of a subclavian vein thrombosis (Figure 1).

Figure 1 - Picture of the original publication by Schrötter,³ 7 years after the thrombosis. Dilated veins of the shoulder, absence of edema

TERMINOLOGY

The term we have been using for some time for the morbid condition we are reporting is venous thrombosis of the upper limb, specifying if the location is axillary or subclavian, or even axillary-subclavian, if this is the case.

Yoshida⁴ prefers the term deep venous thrombosis of upper limbs (DVTUL), more accordingly to the current knowledge, due to its physiopathological analogy with the DVT of lower limbs.

Hughes⁵ performs a wide literature review, reporting clinical and pathological data of 320 cases, making reference to the probable causes of the disease. He categorically affirms: "The more or less fast installation of a venous obstruction of the upper limb in a normal individual represents a syndrome and, in the absence of a opinion consensus with regard to the etiology and pathology, we suggest to adopt the term Paget-Schrötter syndrome". Everything indicates that he was the first author to use such term (Table 1).

Table 1 - Terminology adopted by several authors

Term	Author	Year
Gouty phlebitis	Paget2	1875
Subclavian vein thrombosis	Schrötter3	1901
Traumatic venous thrombosis	Heinecke6	1911
Thrombophlebitis par effort	Lenormant7	1912
Thrombosis due to effort	Rosenthal8	1912
Subacute edema of the upper limb	Delbet9	1913
Traumatic venous stasis	Cadenat10	1920
Primary venous thrombosis	Gould & Patey11	1928
Primitive thrombosis of the axillary vein	Corrêa Netto12	1933
Claudicatio venosa intermittens	Löhr13	1933
Thrombophlebitis dite par effort	Olivier & Léger14	1941
Paget-Schrötter syndrome	Hughes5	1949
Syndrome of the obstacle to the venous reflux	Tagariello15-17	1951
Paget-Schrötter syndrome	Silva1	1965
Deep venous thrombosis of the lower limbs	Yoshida4	2002

CLINICAL STATUS

The clinical status is the base of the diagnosis of venous thrombosis of upper limbs.

Arm, forearm and hand edema, preceded or not by pain in the internal aspect of the arm or axilla, with dilated veins or even well defined circulation in the shoulder, in the deltoid-pectoral space and in the thoracic wall characterize the blockage of the venous circulation. The absence of lymphangitis in the early stage serves as an excellent guidance for the differential diagnosis. The feeling of weight reported by the patient is a consequence of the edema volume, as well as the discomfort and even a certain degree of functional impotence, which avoids the movement of the limb. A skin modification may occur, particularly cyanosis of the fingers in certain positions. The elevation of the limb may improve or worsen the symptoms.

Vasomotor symptoms and quite uncomfortable paresthetic sensations may also be associated. During the clinical examination, the palpation of the arm and axilla may cause pain, especially if the location of the thrombus is axillary.

Muscular tenderness is the rule.

On the contrary to the DVT of lower limbs, which can be asymptomatic, the thrombosis of upper limbs is rich in clinical signs and symptoms.

The patients frequently report an effort, which they consider abnormal, or a trauma, which is considered the cause of the disease.

Predisposing factors, such as neoplasias or systemic diseases, can be identified during the search for associated morbid conditions.

In spite of considering rare the cases of pulmonary embolism in patients with venous thrombosis of upper limbs, Maffei et al.,¹⁸ in a study performed at the Pathology Department of the Faculdade de Medicina de Botucatu (SP), verified, in a review of 998 autopsies, that 7.9% of cases of pulmonary thromboembolism had their origin in upper limb veins.

ETIOPATHOGENY

The classic Virchow triad remains until now the fundamental reference so that a thrombosis may be established. A lesion of the endothelium, followed by venous stasis, is associated with alterations in the coagulation factors, which lead to thrombogenesis.

Put it that way, everything seems to be very simple. Actually, despite several researchers, many questions are still without an answer, since the mechanisms involved in the process have not been totally cleared.

The finding of thrombosis in the axillary-subclavian venous segment in young people, with good organic constitution, in the absence of risk factors, suggested the possibility that indirect traumas, or an effort considered abnormal, could be the cause of the disease.

There are several factors reported in the literature as possible determiners of thrombosis, so that a cause and effect relation can be established, since the nature of the effort is different, such as lifting a heavy weigth,^6,19 lifting a 100-kg bag,²⁰ placing a wagon on the tracks,²¹ ringing a church bell.²²

Sports activities have also been reported with a certain frequency: volleyball,^8,23 tennis,^24-26 swimming,^26-28 basketball,²⁹ baseball,^24,26,30 equitation,¹³ spiroball,¹ and martial arts.⁴

Physical efforts related to everyday professional activities are also mentioned: lifting a ledger book,³¹ after answering the telephone,³² conducting an orchestra³³ and typing at a typewriter.²⁵

Regarding our cases, three presented compression and subclavian venous thrombosis with a large callus (one case) and pseudarthrosis (two cases), in a clavicle fracture.

As a rule, we were able to correlate the venous, thrombogenic or compressive alterations with muscle, bone or ligament structures.

We still support, as we did in the conclusion of our thesis, the point of view that "the antecedent of effort or trauma does not explain, for itself, the etiopathogeny of this morbid condition" and that "from the etiopathogenic, clinical, phlebographic and anatomopathological perspective, we do not see a difference between the venous thrombosis of upper limbs and lower limbs".¹

French-speaking authors have a motive to use the term soit disant, and English-speaking authors to use the term so-called, to classify this type of thrombosis.

On the other hand, we must acknowledge the participation of iatrogenic factors, which influence the triggering of the thrombosis, such as the prolonged venous catheterization, each time more frequent, the use of a pacemaker or another aggressive instrument, as well as the presence of inflammatory or neoplastic processes, which affect the lymph nodes, or even tumors of the mediastinum, which are able to compress the superior vena cava or the intrathoracic venous trunks.

DIAGNOSIS

The clinical data are the key elements for the diagnosis of a DVT of upper limbs.

If there is a reference to a physical effort or recent trauma, it should be registered for a more detailed analysis during the anamnesis.

It is clear that the examination must be detailed, searching for systemic diseases that might be associated with the thrombosis.

Complementary tests

A thoracic radiography, which can be easily performed at any urban center, may provide an orientation for the aspect of the mediastinum, lungs, soft tissues and skeleton.

Phlebography - it is the most efficient method to locate the venous obstruction, defining the characteristics of the thrombosis, whether it is adhesive or embolic, as well as the most affected vein, whether the axillary or the subclavian. The type of collateral circulation may give an idea of the thrombus extension (Figures 2 and 3). The contrast might show the fluctuating thrombus in the lumen of the superior vena cava (Figure 4). It is worth remembering that, to contrast the innominate veins, direct affluent veins of the superior vena cava, the injection of the contrast must be bilateral and simultaneous (Figure 5). One of the disadvantages is that it is an invasive procedure, able to produce a reaction in the venous wall, which is already affected by the thrombosis. On the other hand, the examination may be contraindicated for patients with renal insufficiency, due to the nephrotoxicity of the contrast substance. The other disadvantage is technical. It is not possible to obtain a good phlebographic image by injecting any vein of the arm or forearm. The injection must be preferentially made by puncture, followed by catheterization, of the median basilic vein at the level of the elbow bend, so that the contrast may follow through the basilic vein, in the arm, which ends in the initial segment of the axillary vein or in the brachial veins, thus reaching the contrast in the subclavian vein. In case the injection is made in the affluent veins of the cephalic vein or in itself, the filling of the main veins will not be obtained, since it goes through the lateral edge of the biceps to enter the terminal segment of the axillary vein or above it, in the subclavian vein. This statement is based on a phlebographic study that we performed in 55 normal individuals and in 30 patients with clinical suspicion of venous obstruction.^34,35 We also verified the number of "false images" due to the opening and closure of the subclavian vein valve during forced inspiration and expiration, at the moment of image taking. We based ourselves on the publications by Tagariello,^15-17 who, in the phlebographic interpretation, created the terms false images "of stop" and "of stenosis", whereas other authors attributed them to extrinsic compressions.

Figure 2 - Phlebography: thrombosis of the right axillary vein. The collateral veins "in bridge" affect the subclavian vein

Figure 3 - Phlebography: right axillary-subclavian thrombosis. Collateral circulation directed to the neck, contrasting the internal jugular vein

Figure 4 - Thrombosis of the subclavian and internal jugular veins. A: edema of the arm; B: phlebographic aspect; C: autopsy material, with fluctuating thrombus in the superior vena cava

Figure 5 - Phlebography of the superior vena cava system by simultaneous bilateral injection. A: normal phlebography; B: thrombosis of the innominate venous trunks and left internal jugular

 

Eco-Doppler - this noninvasive method has been more used, due to its great contribution to the diagnosis of DVT of lower limbs. For upper limbs, the technique is similar, but there are certain limitations with regard to the veins located below the clavicle, i.e., at the terminal part of the subclavian vein, as well as to the innominate veins, located inside the thorax. Nevertheless, it presents an exceptional advantage: it can be used several times, given the good acceptance by the patient. Moreover, it is nonaggressive and it does not require a contrast substance. On the other hand, it demands the participation of a highly skillful professional, who knows the regional anatomy well, above all the vascular region, in order to establish the difference between the dilated trunk and collateral veins, identify the internal jugular vein, as well as being alert to flow variations related to respiratory movements. To have a good quality examination, which allows a reliable interpretation, there is also the need of sophisticated equipment, which limits the method to advanced medical centers. As Yoshida⁴ stresses: "The duplex scanning technique, with real-time imaging and spectral analysis, is more accurate than the continuous wave ultrasound Doppler for the assessment of patency of upper limb veins". And he insists: "For being totally noninvasive and accurate, this method has been replacing the phlebography for the diagnosis of the DVTUL".

Some authors even affirm that: "The color Doppler ultrasound is an important part of the assessment of upper limb veins. It might help to identify any blood vessels that may be difficult to be identified with the standard sonography".³⁶

TREATMENT

Anticoagulant therapy

Soon after the diagnostic confirmation, i.e., the evidence of DVT of upper limb trunk veins, specifically the axillary and/or subclavian vein, the anticoagulant therapy must be started with unfractioned heparin, endovenously and with doses identical to those used for the lower limb DVT, corrected by the TTPA. The oral anticoagulant, in this case the sodium warfarin, must be started along with the heparin, at a dose of 10 mg in the first 3 days, maintaining 5 mg/day on subsequent days, until 3 months, under laboratory control. Ameli et al.,³⁷ from the University of Toronto, studied 20 patients with axillary DVT, initially treated with heparin and soon after with cumarin for 3 months and followed-up for 42 months. After this period, only five patients presented a slight discomfort by making great efforts. They finish by stating that the excellent results obtained make the use of fibrinolytic unjustifiable, as well as the resection of the first rib or venous thrombectomy. Gloviczki et al.,³⁸ from the Mayo clinic, followed the evolution of 95 patients; 23 with severe axillary-subclavian venous occlusion and 72 with chronic venous occlusion, who were reviewed to evaluate the results of the medical and surgical treatment. They verified that 36 had effort venous thrombosis, 34 concurrently had venous thrombosis and thoracic outlet syndrome and 20 had venous occlusion due to catheterization. In the follow-up, 57 patients (60%) were asymptomatic. Of these, 48 were treated with anticoagulants during the acute stage of the disease. The best results occurred with those who received the anticoagulation for, at least, 3 months. Twenty-six patients (27%) presented residual edema and complaints during moderate exercises and 12 (12.6%) had a certain degree of discomfort. They consider the immediate systemic anticoagulation, indicated to prevent the extension of the thrombosis and the embolism. They believe that the axillary-subclavian DVT is a non-lethal disease, with low risk of pulmonary embolism, but with a high incidence of late sequelae.

Fibrinolytic

Despite having used the streptokinase in patients with acute arterial thrombosis, lower limb DVT and even pulmonary embolism, when the fibrinolysis center was inaugurated at the Serviço do Professor Romero Marques at the Hospital das Clínicas da UFPE, in Recife, in 1971, we did not have the opportunity to use it for upper limb DVT. Steed et al.³⁹ used the streptokinase in seven patients with subclavian or axillary thrombosis, due to catheterization in a forearm vein, under phlebographic control, before and after the injection. In spite of a remarkable improvement of clinical symptoms, the complete dissolution of the thrombus was not obtained. AbuRahma et al.⁴⁰ performed a comparative study between anticoagulant drugs and fibrinolytics in 10 patients with axillary DVT, followed-up for a period of 36 months. The first six patients received the conventional treatment. The dissatisfaction with the result, after 1 year, made them choose the thrombolytic therapy with streptokinase in two new cases and with urokinase in other two cases. Three patients treated with thrombolytic agents had complete dissolution of the thrombus and elimination of symptoms. In conclusion, they believe that the thrombolytic therapy is the choice treatment for the effort thrombosis, since the diagnosis is made within 7 days of symptoms onset.

Fibrinolytic and decompressive surgery

Following a line of reason that there is always a venous decompression of the subclavian vein associated with an effort thrombosis, Molina²⁶ performed the section of the tendon of the anterior scalene muscle and of the subclavian muscle, which is retracted in order to resect the first rib.

A few minutes before, during a phlebography, he placed a catheter with the tip inside the thrombus and infused urokinase for 12 to 24 hours. Once the complete dissolution of the thrombus was obtained, he performed the decompression surgery.

From a total of 28 patients, six were followed-up for 5 days, considered in the acute stage; seven in the subacute stage, 6 days to 2 weeks after the event and; 15 in the chronic stage. In 15 patients, an angioplasty was performed, under the form of a venous patch.

The decompression was obtained, with restoration of the normal diameter of the vein, in all patients who were in the acute or subacute stage, and in 86% of those in the chronic stage.

In another publication,⁴¹ the author emphasizes the need of an emergence surgical intervention for similar cases. With a population of 65 cases, he identified three categories: acute group (less than 1 week of evolution), which consisted of 14 patients; subacute group (between 1 and 2 weeks), with four patients and; chronic group (more than 2 weeks), with 47 patients.

The standardized treatment was: use of urokinase followed by heparin; soon after, the decompressive surgery, i.e., the resection of the first rib and subclavian and anterior scalene muscles, through anterior and subclavicular approach.

The results were the following: for acute cases, 100% of good results. The angioplasty with patch was necessary in subacute, chronic and recurrent cases. This procedure was 100% efficient, with a stenosis lower than 2 cm. Of this series, 14 patients were considered impossible to be submitted to a surgery.

With regard to the long-term follow-up (more than 7 years), he affirmed that, for the 17 initial patients who were submitted to angioplasty, the control was made by phlebography in order to verify the vein patency. He added that he is currently using only the ultrasound, as a result of the excellent quality of the examination. He ends his report categorically: "One definite conclusion of all these studies is that the surgical intervention is always necessary to repair the obstruction. In our experience over the past years, it became evident that the emergence treatment is the only way to avoid the chronic fibrotic obliteration of the subclavian and axillary veins".

Thrombolysis and venous stent

Méier et al.⁴² performed the treatment and control of the results of 11 patients with Paget-Schrötter syndrome, from October 1992 to December 1995. The diagnosis was confirmed by phlebography and the established protocol was the thrombolysis with urokinase. The duration and adjustment of the perfusion velocity were determined by a vascular surgeon and an interventionist radiologist. The stents were placed when the patients had a residual stenosis of, at least, 50% after the percutaneous angioplasty with transluminal balloon.

The thrombosis was associated with an effort or trauma in four cases. The fracture of the stent occurred in two patients, in which the resection of the first rib was not made. In the late follow-up, eight out of 11 patients were asymptomatic.

Of the three remaining patients, one presented limited thrombosis; in the other two an early extensive rethrombosis was diagnosed, and the symptomatology was so significant that it avoided their activities. The authors highlight that the fracture of the stent is likely to occur in case the resection of the first rib is not made.

Venous thrombectomy

It is even curious the fact that the venous thrombectomy was performed for the first time in upper limb veins. Everything indicates that it was Schepelmann,⁴³ in 1910, the first one to operate a 22-year-old student with mitral insufficiency and history of edema of the left upper limb, with a 5-day duration. He thought it was a case of lymphadenitis with lymphangitis, and being uncertain about the diagnosis, he decides to indicate a surgical intervention.

By performing it, he verifies the presence of thrombosis of the brachial, axillary and subclavian veins. "The vein walls seemed to be intact, but thin as a sheet of paper, making it possible to see, by transparence, the clots inside them". After incising the vein wall, he removed the clots, suturing it afterward. Three weeks later, the patient presented symptoms of small pulmonary embolisms and, within 5 weeks, an axillary cord and development of the collateral circulation could be seen. The author classified this case as a thrombosis with a rare etiology, the cause being possibly due to "a violent effort that the patient made when he tried to stop a wild horse".

In 1937, Läwen⁴⁴ performed an axillary thrombectomy in a case of venous thrombosis of the upper limb, with a large edema and a clear arterial spasm and with a 3-day evolution. The result was considered excellent, and the author comments: "if the regression of the edema can be explained by the reestablishment of the circulatory flow, the elimination of arterial phenomena is probably due to the removal of the intravascular foreign body". He was still enthusiastic about the procedure, and published, in the following year, eight cases of thrombosis of the femoral and external iliac vein, with excellent outcome in six patients.⁴⁵

Tuchmann⁴⁶ wrote his Ph.D. thesis based on the observation of 56 patients at the Surgical Clinic A, from Strasbourg, submitted to venous thrombectomies performed by Professor Fontaine. In only seven patients, the topography was of the upper limb; three of them were axillary, two were subclavian and two axillary-subclavian, all confirmed by phlebography.

In a critical article, published as an offprint of the Therapeutische Umschau, by the Publishing House Hans Huber, from Berna, in 1962, Fontaine & Tuchmann⁴⁷ made a circumstanced report of the "role of thrombectomy in the therapeutic of deep venous thrombosis in its acute stage". In spite of accepting and praising the anticoagulant therapy - particularly the heparin - they do not believe that it is sufficient to control a femoro-iliac or iliac-cava venous thrombosis in its evolution, to avoid a pulmonary embolism. That is why they insist on the surgical treatment. "Similarly they think, as personal experience, that the phlegmasia cerulea must be systematically operated, once it is diagnosed".

Rollo⁴⁸, when referring to the indication, makes an appreciation that I consider very appropriate, by stressing as "advantages of the thrombectomy with regard to the clinical treatment, the immediate resolution of the venous obstruction, the faster relief of pain, the prevention of pulmonary embolism and the preservation of venous valves, by reducing the risk of the post-thrombotic syndrome".

In our series, we performed axillary thrombectomy in two patients and thrombectomy, followed by short phlebectomy of the subclavian venous segment, with great parietal alteration and adhesive thrombosis in another patient (Figure 6). During a follow-up that lasted for more than 5 years, the patients did not present complaints or residual edema. Nevertheless, there was still collateral circulation in the shoulder and in the deltoid-pectoral space. In one case of subclavian DTV, with propagation to the internal jugular vein and fluctuating thrombus in the superior vena cava, we could not remove the obstruction of the jugular vein. The death occurred in the first 24 hours, and the patient presented convulsions. The autopsy confirmed the presence of cerebral infarction. We remember that the extraction of thrombi was performed with choledochal clamps, Randall type, followed by aspiration. At that time, the Fogarty catheter did not exist.

Figure 6 - A: photos of the patient before and 5 years after the thrombectomy and segmental phlebectomy; B: phlebography with image of the axillary thrombosis; C: operative piece

POPULATION

From 1962 to 1995, 52 patients with the clinical diagnosis of venous thrombosis of upper limbs were assessed; 40 came from the Vascular Surgery Service at the Hospital das Clínicas of the UFPE (Recife, Brazil) and 12 from a private clinic.

Of this series, 32 cases were confirmed by phlebography (61.5%), eight (15.4%) by subclavian thrombosis and catheter obstruction for parenteral feeding, three (5.8%) by traumatic lesion of the subclavian vein, associated to the fracture of clavicle with pseudarthrosis, one case (1.9%) with histopathologic diagnosis of subclavian valve hypertrophy and eight patients (15.4%) with large edema and collateral circulation suggesting venous obstruction, with significant reduction of clinical signs and symptoms, with the use of anticoagulants, but without the phlebographic control (Table 2).

Table 2 - General status with 52 cases

Diagnosis	Cases	%
DVT confirmed by phlebography	32	61.5
DVT due to venous catheterization	8	15.4
DVT due to extrinsic compression	3	5.8
Fracture of a clavicle with a large callus (one case)
Fracture with pseudarthrosis (two cases)
DVT and hypertrophy of the subclavian valve	1	1.9
Edema with no DVT confirmation by phlebography	8	15.4
Total	52	100

 

The mortality rate in our series was high (seven cases), but the causa mortis was related to the associated disease, mainly to a malignant neoplasia, and not to the venous thrombosis itself. The deaths were confirmed by autopsy; in one case, there was cerebral infarction, with an extensive thrombosis of the internal jugular vein (Table 3).

Table 3 - Deaths

Causa mortis*	Cases
Axillary-subclavian, tibiofibular and popliteal DVT (pancreatic cancer, pulmonary embolism)	1
Axillary and femoro-popliteal DVT (pancreatic cancer, cachexia)	1
Innominate trunk DVT (pancreatic cancer, peritoneal metastasis)	1
Axillary-subclavian and internal jugular DVT (cerebral infarction)	1
Axillary DVT, phlegmasia cerulea (bladder cancer, cachexia)	1
Innominate trunk DVT (aneurysm of the thoracic aorta)	1
Post-delivery axillary and iliac-cava DVT (pulmonary embolism)	1
Total	7

* Confirmed by autopsy.

 

Considerations on the cases of unconfirmed venous thrombosis.

We have already mentioned that the authors, because they could not identify the venous thrombosis, despite having a suggestive clinical status, introduced several terms, according to the interpretation given to the fact.

If, for some of them, the anatomical data were used to explain the location of the thrombosis, which in turn would explain the clinical status, for others the supposed compression determined by such structures was enough to explain the whole syndrome.

The misinterpretation of certain phlebographic aspects also made them admit venous compression between the clavicle and the first rib, which were not confirmed by the surgery.

It is surprising to verify that the suggested "intermittence" motivated an association of ideas so strong in the imagination of its supporters that the compression factor started to be "intermittent".

The notion of "intermittence" seems to be, at first sight, justified to characterize the syndrome. However, it does not resist a deep analysis, particularly when we take into consideration the alterations that occur in the venous circulation of the limb due to any type of obstruction. It is understandable that, if the blood flow is affected and the patient demands from this system a function that it is not able to perform well, the symptoms - the edema above all - can reappear.

We have never been able to validate if a compression can determine, by a nearly vascular mechanism, a "syndrome of intermittent venous obstruction", and so we question its existence, once the arguments presented so far are not convincing.

COMMENTS AND CONCLUSIONS

As it has already been stressed, the term deep venous thrombosis (DVT) of upper limbs, used by Yoshida,⁴ seems to be the most adequate to characterize the acute venous obstruction of the axillary and subclavian veins.

Similarly, the eponymy Paget-Schrötter syndrome presupposes the existence of a thrombus in the mentioned veins. Even when it affects young and apparently healthy people, with or without antecedent of effort and with no associated thrombogenic conditions, we consider the terminology adequate, particularly because the two authors attributed the clinical status to a venous thrombosis. As to the effort, they only suggested it.

The diagnosis must be based on the triad pain, edema and collateral venous circulation, whose characteristics are easy to identify.

The imaging ultrasound, especially the color eco-Doppler, is the examination to be requested as soon as possible. Based on its result, in my opinion, the phlebography must be performed, even if the anticoagulation is already established. I also consider necessary the hospitalization and the laboratory data, as an urgent matter, taken into account the velocity with which the thrombus adhesiveness is processed.

The basic therapeutic conduct should be the use of unfractioned heparin, by continuous endovenous access and under laboratory control, in order to allow the dosage correction.

The oral anticoagulant should be employed along with the heparin and maintained for 6 months.

As I see it, the in situ thrombolytic therapy is a modality that should be more frequently applied. In these cases, soon after the phlebography, the catheterized vein would serve for the infusion, and the dosage and velocity adjustment would be established, by common agreement, by a vascular surgeon and an interventionist radiologist. Unfortunately, the current tests of coagulation control do not assure the success of the thrombus dissolution nor do they safely signal the risk of bleeding, including in the systemic infusion.

Although I have no experience, I confess I have no attraction to the use of stent for repairing a residual stenosis, as suggested by some.

With regard to the venous thrombectomy, I still have some hope, once the diagnosis is established until the first week, and the phlebography shows the embolic or adhesive aspect of the thrombus.

Concerning the thrombosis due to permanent catheterization, whether in the subclavian or jugular vein, in most cases the simplest and most efficient conduct is the removal of the catheter.

Finally, there is an aspect I would like to highlight. I do not agree with the inclusion, in the classifications of the thoracic outlet syndrome (TOS), of subgroups related to supposed arterial and mainly venous compressions.

From the anatomical perspective, the subclavian vein passes beyond the anterior scalene muscle, is well protected by the clavicle and, during movements of limb hyperabduction, the first rib gets a little closer to it, besides the costoclavicular ligament being located at a medial position.

It is clear that the form of facing the problem changes according to the specialist who is approaching the issue.

There is no doubt that our specialty is well defined because it has its own means of diagnosis and treatment. Since the focus of the morbid condition is a venous thrombosis, the vascular surgeon should be in charge of the therapeutic attitude.

REFERENCES

1. Silva W. Síndrome de Paget-Schrötter. Estudo clínico e flebográfico [tese de livre-docência]. Recife: UFPE, 1965.

2. Paget J. Clinical lectures and essays. London: Longmans, Green & Co.; 1875.

3. Schrötter L. Erkrankungen der Gefäss. In: Nothnagel H. Spezielle pathologie und therapie. Alfred Hölder-Wien, X Band, II Theil; 1901. p. 533-534.

4. Yoshida WB. Trombose venosa profunda de membros superiores. In: Maffei FHA, Lastoria S, Yoshida WB, Rollo HA. Doenças vasculares periféricas. 3ª ed. São Paulo: Medsi; 2002. p. 1433-1440.

5. Hughes ES. Venous obstruction in the upper extremity (Paget-Schrötter's syndrome). A review of 320 cases. Surg Gynec Obst. 1949;88:89-127.

6. Heinecke H. Uber thrombosen an der oberen Extremität. Zentralbl Chir. 1911;38:110-16.

7. Lenormant C. Les thromboses veineuses par effort. Presse Méd. 1912;20:761-3.

8. Rosenthal WJ. Ueber thrombose an der oberen Extremität nach Anstrengungen. Deutsche Ztschr Chir. 1912;117:405-24.

9. Delbet P. Oedéme subaigu du membre supérieur. Bull Mém Soc Chir de Paris. 1913;39:1001-5.

10. Cadenat FM. Les thrombo-phlébites du membre supérieur. Paris Méd. 1920;10:253-9.

11. Gould EP, Patey DH. Primary thrombosis of the axillary vein: a study of eight cases. Br J Surg. 1928;16:208-13.

12. Corrêa Netto A. Trombose primitiva da veia axilar. Folia Clin Biol. 1933;5:89-94.

13. Löhr W. Die claudicatio venosa intermittens der oberen Extremität. Ein kritischer beitrag zur sogenannten traumatischen thrombose der vena axillaris und subclávia. Arch Klin Chir. 1933;176:701-34.

14. Olivier C, Léger L. Les thrombo-phlébites "par effort" du membre supérieur. Paris: Masson & Cie; 1941.

15. Tagariello P. Interpretazione delle immagini "da arresto" e "da stenosi" nella flebografia della succlavia. Acta Chir Patavina. 1951;7:103-8.

16. Tagariello P. Value of phlebography in the diagnosis of intermittent obstruction of the subclavian vein. J Int Coll Surg. 1952;17:789-801.

17. Tagariello P. Le sindromi da ostacolato scarico venoso dell'arto superiore. Bologna: Casa Editrice Cappelli; 1954.

18. Maffei FH, Falleiros ATS, Venezian LA, Franco MF. Contribuição ao estudo da incidência e anatomia patológica do tromboembolismo pulmonar em autópsias. Rev Ass Méd Brás. 1980;26:7-10.

19. Winterstein O. Ueber gefässverletzungen mit beiträgen zum traumatisschen segmentären arterienspasmus und zur "traumatisschen" thrombose der vena subclavia. Schweiz Med Wchnschr. 1925;55:360-5.

20. Cottalorda J. La thrombo-phlébite par effort. Lyon Chir. 1932;29:169-90.

21. Grimault L, Dantlo R. Thrombophlébite dite "par effort" de la veine axillaire. Bull Mém Soc Nat Chir. 1924;50:118-21.

22. Pinelli L. Contributo clinico allo studio della trombo-flebite detta "spontanea" o "de sforzo". Chir Org Movimento. 1933;17:537-48.

23. Ofstun MS, Merendino KA. Bilateral obstruction of the subclavian veins. Case report and review of the literature. Am J Surg. 1961;101:803-7.

24. Sampson JJ. An apparent causal mechanism of primary thrombosis of axillary and subclavian veins. Am Heart J. 1943;25:313-27.

25. Suhler A. Contribution à l'étude des thrombophlébites du membre supérieur dites "par effort" . Thése de Strasbourg; 1961.

26. Molina JE. Surgery for effort thrombosis of the subclavian vein. J Thorac Cardiovasc Surg. 1992;103:341-6.

27. De Takats G. Vascular sugery. Philadelphia: W. B. Saunders Co.; 1959. p. 258.

28. Vogel CM, Jensen JE. "Effort" thrombosis of the subclavian vein in a competitive swimmer. Am J Sports Med. 2002;30:708-12.

29. Cãmara Z, Ferreira Jr O. Síndrome de Paget-Schroetter. Boletim do Centro de Estudos do HSE. 1952;4:141-6.

30. Difelice GS, Paletta Jr GA, et al . Effort thrombosis in the elite throwing athlete. Am J Sports Med. 2002;30:708-12.

31. Guyot J, Jeanneney G. Thrombophlébite dite "par effort" de la veine axillaire. Bull Mém Soc Chir. 1923;49:231-6.

32. Huard P. Un cas de thrombo-phlébite du membre supérieur révelée par un effort. Résection du segment veineux thrombosé et denudation artérielle. Guérison. Bull Mém Soc Nat Chir. 1933;59:1406-12.

33. De Bakey M, Ochsner A, Smith MC. Primary thrombosis of the axillary vein. New Orleans: M & SJ. 1942;95:62-70.

34. Silva W. Flebografia dos membros superiores: técnica e resultados. Rev Bras Cardiovasc. 1969;5:1-24.

35. Silva W. Phlébographie des membres supérieurs. Phlébologie. 1970;23:309-10.

36. Baidle TR, Letourneau JG. Arm swelling. In: Bluth EJ, Arger PH, Benson CB, et al . Ultrasonography in vascular diseases. 1st ed. New York: Thieme; 2001. p. 69-82.

37. Ameli FM, Minas T, Weiss M, Provan JL. Consequences of "conservative" conventional management of axillary vein thrombosis. Can J Surg. 1987;30:167-9.

38. Gloviczki P, Kazmier FJ, Hollier LH. Axillary-subclavian venous occlusion: the morbidity of a nonlethal disease. J Vasc Surg. 1986;4:333-7.

39. Steed DL, Teodori MF, Peitzman AB, et al. Streptokinase in the treatment of subclavian vein thrombosis. J Vasc Surg. 1986;4:28-32.

40. AbuRahma AF, Sadler D, Stuart P, Khan MZ, Boland JP. Conventional versus thrombolytic therapy in spontaneous (effort) axillary-subclavian vein thrombosis. Am J Surg. 1991;161:459-65.

41. Molina JE. Need for emergency treatment in subclavian vein effort thrombosis. J Am Coll Surg. 1995;181:414-20.

42. Meier GH, Pollak JS, Rosenblatt M, et al. Initial experience with venous stents in exertional axillary-subclavian vein thrombosis. J Vasc Surg. 1996;24:974-83.

43. Schepelmann E. Demonstration eines patienten mit thrombose der linken vena subclavia seltener aetiologie. München Med Wchnschr. 1910;57:2444-5.

44. Läwen A. Ueber thrombektomie bei venenthrombose und arteriospasmus. Zentralbl F Chir. 1937;64:961-8.

45. Läwen A. Thrombektomie als frühoperation bei durchblutungsstörung durch akute massive thrombose der vena femoralis und vena iliaca externa. Zentralbl f Chir. 1938;65:2139-46.

46. Tuchmann L. La thrombectomie dans la thrombose veineuse aiguë. These presentée pour le doctorat en medicine. 1961;12.

47. Fontaine R, Tuchmann L. La place de la thrombectomie dans la thérapeutique des thromboses veineuses profondes à leur phase aiguë .Therapeutische Umschau, ed Hans Huber, Bern. 1962;11:462-9.

48. Rollo HA. Trombectomia venosa. In: Maffei FHA, Lastoria S,Yoshida WB, Rollo HA. Doenças vasculares periféricas. 3ª ed. São Paulo: Medsi; 2002. p. 1427-1431.