Key words
Paget-Schroetter syndrome - thrombosis par effort - thoracic inlet syndrome - vascular
thoracic outlet syndrome
Schlüsselwörter
Paget-von-Schroetter-Syndrom - Par-effort-Thrombose - Thoracic-inlet-Syndrom - vaskuläres
Thoracic-outlet-Syndrom
Introduction
The possibility of a subclavian vein thrombosis in the context of a thoracic inlet
syndrome (TIS) should always be considered in young otherwise healthy patients, who
have a sudden onset of arm swelling (especially with activities involving movements
above the head, such as lifting weights) or otherwise inexplicable pulmonary emboli
[25].
In addition to the swelling, clinical examination reveals a cyanotic discolouration
of the skin and an increase in prominent veins over the upper arm, shoulder and chest.
The signs and symptoms are often dramatic and associated with pain [10]. There is a striking difference in the findings on the two sides. The condition
may be misdiagnosed as an allergic reaction to an insect bite or topical medication.
Muscle spasm or tears also have to be ruled out in the individual case.
The diagnosis is usually made with colour-coded duplex ultrasonography. Further cross-sectional
modalities (computed tomography (CT) and magnetic resonance imaging (MRI)) may also
be used [7]. Venography of the arm is the basis for catheter-directed thrombolysis [Fig. 1a, b]) [10].
Fig. 1 a Venography with catheter-directed revascularisation and b evidence of bilateral compression on abduction/elevation in an upright position.
Once the diagnosis has been confirmed, there are five possible treatment options:
-
Purely conservative with anticoagulation and compression therapy
-
Local thrombolysis with subsequent anticoagulation and compression therapy
-
Local thrombolysis with subsequent surgical decompression of the upper thoracic outlet
-
Surgical decompression of the upper thoracic outlet with subsequent anticoagulation
and compression therapy
-
Surgical decompression of the upper thoracic outlet and secondary recanalisation of
the venous thrombosis
When considering purely conservative treatment analogous to that given for a deep
vein thrombosis of the pelvic or leg veins [8], it must be remembered that these particular patients are all young otherwise healthy
people. The risk of a potential lysis-induced complication is therefore extremely
low in comparison with thrombolysis performed in other indications. When there is
evidence of a compression syndrome, relieving the stress on the externally compromised
structures is the only permanent treatment that does not necessitate substantial changes
in everyday life. Long-term anticoagulation at any dose is contraindicated or should
not be used without at least a critical appraisal in all forms of contact sport and
in athletes with a potential risk of injury. Insurance provisions for professional
athletes also have to be taken into consideration.
In a competitive athlete, the cause of thrombosis in TIS can mainly be attributed
to repeated mechanical compression of the vein between the clavicle and first rib.
Besides muscular endurance training and the simultaneous anatomical pressure exerted
in the costoclavicular space, a common secondary factor is repetitive chronic strain
with the associated risk of injury and development of progressive fibrosis [20]. In competitive athletes, the outbreak of the disease may therefore occur in particular
after activities involving abduction, lifting or vigorous movements of the arm. The
average age of onset is between 25 and 35 years of age. It affects men almost twice
as frequently as women [10]. Depending on the severity of the condition, the symptoms may be positional or persistent.
If the condition becomes chronic, a collateral circulation develops over the chest
wall with time ([Fig. 2]). Pulmonary embolism with dyspnoea and dizziness may also occur in up to 20 % of
cases [5].
Fig. 2 Typical clinical picture of a marked collateral circulation in a functional position.
In a special form of the disease that is seen in some patients, the typical swelling
can occur without thrombotic occlusion at various positions of the arm on applying
external compression. This particular variant is known as McCleery’s syndrome, named
after the person who first described it in 1951 [17].
Methods
The present article is based on a presentation given at the 59th Annual Meeting of the German Phlebology Society in Stuttgart (2017).
We analysed the literature on the basis of a PubMed search from January 1980 to 31
May 2018. The search terms (medical subject headings, MeSH) were Paget-Schroetter
syndrome, thrombosis par effort, thoracic inlet syndrome and vascular thoracic outlet
syndrome. In addition, we included similar articles. The search yielded 2086 hits.
The results were analysed for content and further search constraints were subsequently
added in an advanced search strategy with the Boolean operators ‘and’ and ‘or’. The
literature references that remained were checked for subject value. Further constraints
were added with the filters ‘randomized control trial’ and ‘clinical queries’. We
took into account only articles published in English or German or with at least a
relevant abstract. We compared the data and results obtained with our own experiences.
Results
We did not find any randomised controlled trials that specifically addressed primary
(idiopathic) venous thrombosis of the upper limb. Our search yielded only statements
on very heterogeneous causes of upper limb thrombosis due mainly to the insertion
of catheter material or in association with cancer. After the further exclusion of
publications with a predominantly clinical neurogenic or arterial TOS, only case reports
and analyses of small TIS case series remained. The largest-scale publication followed
32 patients over a period of ten years [24]. By further narrowing the search strategy to deep vein thrombosis of the arm in
athletes, we found 51 publications. We found publications that specifically cited
TIS in athletes in only a further 11 cases [3]
[4]
[6]
[9]
[11]
[14]
[18]
[19]
[23]
[26]. There were no randomised controlled trials that covered the whole topic.
Out of 276 patients with TOS treated in their own practice (1997–2007), Melby et al.
[24] analysed the outcomes in 29 male and 3 female athletes with thrombosis par effort
of the upper arm. The right arm was affected in 26 cases (81 %). Seven patients were
professional athletes. The interval between the time when the symptoms were first
noticed and the diagnosis of venous thrombosis confirmed by venography was 20.2 ± 5.6
days (median 4.5 days, range 1–120 days). Preliminary ultrasound examinations were
carried out in 21 patients (66 %). However, the findings gave false negatives in six
patients (sensitivity 71 %). Twenty-six patients (81 %) had previously undergone catheter-directed
thrombolysis elsewhere. Twelve patients (46 %) from this group had also had balloon
angioplasty. One patient had also had a stent inserted. In each case, there was overlapping
anticoagulant therapy. A further six patients (19 %) had the primary diagnostic investigation
and treatment in-house. All patients were offered secondary surgical decompression
of the upper thoracic outlet, irrespective of the time that had already elapsed, although
it should ideally be performed within three weeks. Twelve patients had initially received
no such recommendation for surgery from the medical facility that first treated them.
Due to persistent symptoms (with five cases of recurrent thrombosis despite anticoagulation),
these patients were finally transferred and underwent surgery after a considerable
delay (163.3 ± 29.2 days). All patients were operated on by the same surgeon following
the same surgical strategy (combination of supraclavicular and infraclavicular access)
with full scaleneotomy, plexus neurolysis and removal of the 1st rib. Complete venolysis was carried out in each case. Fourteen patients (44 %) with
residual venous stenosis or an operable occlusion opted for a patch plasty or a saphenous
vein graft (with a peripheral arteriovenous fistula). The stent that had been inserted
previously had fractured. Seven patients needed early revision surgery: three occlusions
of the vein graft, two haematomas that required evacuation, one haemothorax in need
of drainage and a lymphatic fistula. All 32 patients were able to resume their sporting
activities. Even though the outcome was ultimately the same, the inpatient stay was
significantly prolonged in patients with delayed and complicated treatment (re-thrombosis).
In the remaining publications, the authors mostly presented a case report on one of
their own patients combined with a literature search on the clinical signs, symptoms
and treatment of this medical condition. In the more recent literature, we found recommendations
for aggressive invasive therapy, with good results [4]
[11]
[19]
[23]
[26].
In our own patient population in 2013–2016, we treated 184 inpatients with a compression
syndrome of the upper thoracic outlet. Surgery was performed on 88 of these patients.
Fifty-two patients had a vascular TOS; 12 patients were treated for a purely venous
TIS. This specific patient group included five active professional athletes (two surfers,
one footballer, one canoeist and one handball player). All of these patients were
men ([Table 1]).
Table 1
Patient characteristics, diagnosis, treatment, and outcome.
|
Patient
|
Sex
|
Age
|
Sport
|
Side
|
Time until imaging
|
Pulmonary embolism
|
Thrombolysis/angioplasty
|
Transaxillary decompression
|
Complications
|
Duration of hospital stay
|
Competitive sport resumed
|
|
N. S.
|
M
|
28
|
Football
|
Left
|
3.5 months
|
yes
|
+ / +
|
+
|
–
|
11 days
|
5 months
|
|
M. H.
|
M
|
28
|
Handball
|
Left
|
1 month
|
no
|
+ / +
|
+
|
–
|
8 days
|
5.5 months
|
|
J. H.
|
M
|
24
|
Surfing
|
Left
|
1 year
|
yes
|
±
|
+
|
–
|
26 days
|
6 months
|
|
M. S.
|
M
|
35
|
Surfing
|
Left
|
1 week
|
yes
|
±
|
+
|
–
|
10 days
|
4.5 months
|
|
J. L.
|
M
|
25
|
Canoeing
|
Right
|
2 months
|
no
|
±
|
+
|
–
|
12 days
|
5.3 months
|
We followed the modified treatment schedule given below [5]
[13]
[22].
After the initial diagnostic work-up, our athletes underwent catheter-directed lysis
with urokinase over a maximum of 36 hours. We first gave a bolus dose of 100 000 IU
followed by 100 000 IU/ hour via an infusion pump, with a simultaneous administration
of unfractionated heparin (usually beginning with 1000 IU/hour). The heparin dose
was adjusted according to the monitored coagulation status. In two patients, who initially
had undergone only a partial recanalisation with residual stenosis, an additional
balloon angioplasty was subsequently performed. Decompression surgery was carried
out within a few days to six weeks after the procedure ([Fig. 3]). In addition to the necessary surgical vasolysis and neurolysis, we recommend the
complete removal of the cervical/first rib to prevent residual or recurrent problems
([Fig. 4]). All patients were treated with a direct oral anticoagulant (DOAC) and arm immobiliser/
Gilchrist bandage to prevent sudden movements until such time as the definite operation
with transaxillary rib resection was performed. Postoperative care was always adapted
to the individual situation. Patient-controlled analgesia (PCA) via a pump was used
for 2–3 days after the surgical decompression and was then switched to oral analgesia
with an individually determined dose of gabapentin, diclofenac and/or Novalgin. Restricted
personal training, e. g. on a treadmill, and rehabilitation measures were also possible.
In accordance with the literature, anticoagulation was continued for 6–12 weeks [8]. A return to unrestricted training or team training took 4–6 months ([Fig. 5]). We have not so far had any complications or recurrent thrombosis in our patient
population.
Fig. 3 Transaxillary access showing the anatomy. The vertical arrow points to the subclavius
and the horizontal arrow indicates the anterior scalene muscle that has already been
divided.
Fig. 4 Resection of the 1st rib. All anatomical muscle and ligament variants that could
potentially compress the vessels are removed, with the exception of the pectoralis
minor muscle.
Fig. 5 Individual follow-up and advice on convalescence, for example, by WhatsApp.
A comparison of our results with the data available in the literature is difficult
and, in our opinion, would not be justified. The reason for this is that not only
the timing, but also the range of treatment for the required comparison was extremely
variable. We did not link any conventional open venous reconstruction to this patient
population.
Discussion
Deep vein thrombosis in the shoulder girdle has a serious impact on health and sporting
activities, especially in competitive athletes. Besides the usual risk factors (Virchow’s
triad), the congenital narrowing of anatomical structures, such as ligaments, muscle
fibres and cervical ribs, generally combined with additional triggers (posture, accident,
muscle mass) or taking anabolic steroids must also be considered. The literature also
points to the considerable time spent travelling to competitions and repeated localised
vascular trauma with particular force and impact.
The treatment goals are to achieve a rapid resolution of symptoms, to lower the risk
of pulmonary embolism (some 20–30 %) and to prevent a recurrence of the thrombosis
[1]
[10]
[15]. As this patient group consists of young active athletes, purely conservative measures
of anticoagulation and compression are not the treatment of choice in most cases.
With this type of treatment, these patients would have to forgo their customary sporting
activities because of the increased risk of bleeding (contact sports) and compensate
for the long periods when they are out of action – making it difficult to resume their
active lifestyle. We therefore recommend an active therapeutic approach. For this,
a rapid diagnosis is of key importance, which is to be followed by venography and
catheter-directed thrombolysis combined with subsequent decompression surgery. Based
on the literature and the results in our own patients, the majority of patients, who
have successful local lytic therapy and surgical treatment within the first two weeks,
have better long-term results [10]
[25]. However, the publications do not uniformly assess the precise timing of the operation
[5]
[10]
[12]. Lytic therapy alone without subsequent surgery carries a residual thrombotic risk
of 30–70 %. Stents should also be avoided. The timing of surgery varied between immediately
and an interval of up to three months. Surgical decompression consisted mainly of
the transaxillary removal of the first rib with an Atkin’s procedure [5], with or without venous reconstruction.
Successful catheter-directed thrombolysis followed by anticoagulation alone and no
surgical decompression carries a risk of recurrence as high as 25 % [16]
[25].
Excellent long-term results have been achieved in specialised centres after combination
treatment with thrombolysis and surgical decompression. The five-year patency rate
is between 80 % and 90 % [13]. In addition to a rapid diagnosis, prompt referral to an experienced specialist
team is essential to achieve the expected good outcome. The longer the occlusion is
present, the more complicated the chances of success, even with the necessary expertise.
The experts’ consensus-based standard treatment for TIS consists of rapid thrombolysis,
anticoagulation and surgical decompression [2]
[13]
[20]
[21]. This combination represents the only causal treatment for the condition.
Another major factor in assessing the quality of treatment is the use of a defined
follow-up examination and care protocol, such as the one we have established ourselves
[10].
In summary, rapid targeted diagnostic investigation and interdisciplinary treatment
in an experienced unit makes it possible for these patients to be treated successfully.
