Thoracic Outlet Syndrome
TOS definition
is brief and it consists of some anatomical anomaly as a precondition and
causal event what can be long-term physical activity straining the upper limb
and region of upper thoracic aperture or some sudden event in form of trauma in
this region. Clinical background of the symptoms on upper limb is a compression
of neuro-vascular fascicle in upper thoracic aperture. This fascicle consists
of brachial plexus, subclavian artery and vein.
There are three
typical anatomical regions for OTS development – first is a scalene triangle demarcated
by medial and anterior scalene muscle and clavicula, second is costo-clavicular
space between clavicula and first rib with muscle and ligament insertions and
the last one is insertion of small pectoral muscle.
Adopted from Netter´s Atlas of Human Anatomy
In all of these
regions there can be some anatomical anomalies which can be a precondition for
TOS development. Scalene muscle is variable in its insertions and there can be
some tough and thick ligaments causing narrowing of scalene trigonum. A neck
rib is an anomaly when there is a rudimentary rib present and connected to the
last neck vertebra. Its incidence is up to 1 % with much higher portion of females
(7 : 1). This rib is usually present on the left side. The first rib can be
also affected by congenital or development anomalies regarding its size and
shape. Incidence is the same as for the neck rib but there is no difference in
a male and female ratio.
TOS is a disease
of young age and in majority of cases it manifests between 20 and 50 years.
This age period is logical since TOS needs not only anatomical precondition but
also a time of limb straining to develop it manifests in the age of full sport and
work activity after 20 years. The same situation is in case of person with
anatomical precondition there is high possibility that this disease will
manifest most likely till age of 50 years and any later manifestation is
usually because of post-traumatic changes. TOS prevalence is clearly higher for
female gender (up to 70 % of patients are women), the simple reason is higher
incidence of neck rib.
We distinguish
three types of TOS based on which part of neuro-muscular fascicle is affected: neurogenic
TOS, venous TOS and arterial TOS. The most frequent and unfortunately the most
difficult to diagnose and treat is a neurogenic TOS.
Neurogenic TOS
is usually caused by a trauma in neck region, e.g. car crash or fall on
slippery ground. One side straining of the upper limb such as work at
assembling lines leads to micro-trauma in scalene muscle and its fibrotization
has its part in the disease development as well. Anatomical precondition for
this type of TOS is anomaly of scalene triangle, high insertion of brachial
plexus roots and presence of a neck rib.
As mentioned above
trauma to scalene muscle (both chronic micro-trauma or acute trauma) lead to
stretching and rupture of muscle fibers. Consequent to this trauma is a
swelling of the muscle and inflammation causing a compression symptoms in a
form of pain and paresthesia of upper limb, numbness and muscle weakness.
Damaged parts of the muscle will change to scar in time leading to more tough
strains formation and another narrowing of the region. Clinical symptoms are
the same plus there can be occipital headaches and vaso-motoric symptoms (e.g.
Raynaud syndrome).
For venous TOS
manifestation and diagnosis, we have to move one level down to costo-clavicular
space, anatomical anomalies in this region and repeated straining of upper limb
are the cause of venous TOS manifestation. Repeated micro-trauma to the wall of
subclavian vein leads to its fibrotization and stenosis formation which can
ultimately lead to vein thrombosis (in case of e.g. dehydration or holding the
limb in upper position for long time).
Picture shows a
clear difference between upper limbs of young man with acute subclavian vein
thrombosis.
Adopted from Endovascular today: Management of Subclavian Vein Thrombosis With Mechanical Thrombectomy; Frank R. Arko III at. Al.
Etiology of
arterial TOS is similar to neurogenic TOS, mostly rib anomalies and scalene
muscle insertion. If the subclavian artery is compresses there is a
post-stenotic dilatation or even aneurysm developing after the stenosis.
Turbulent flow in aneurysm can cause thrombus formation with distal
embolization or complete aneurysm thrombosis, bot conditions causing acute limb
ischemia. Symptoms are paresthesia and pain during exercise or work, ischemic
skin wounds and palpable whirl in aneurysm position above clavicula.
Key to proper
TOS diagnosis is rigorous patient medical history and consequent physical
examination with test, which can amend and confirm the diagnose. I tis important
to combine all acquired data, because none single diagnostic test is effective
enough for TOS diagnosis confirmation on its own. Important medical history
information is work and sport burden leading to one side limb straining (e.g.
painters, work at assembling line), trauma in neck and shoulder region,
clinical symptoms like limb pain after exercise or occipital headache. Since
the diagnostics and treatment of TOS is difficult patients usually visit
several physicians in search for someone who can help. This is the reason why
we should ask for previous examinations as well.
Differential
diagnostic is rich in case of TOS. In case of neurogenic TOS we have to
consider carpal tunnel syndrome and tennis elbow, injury of rotator calf, neck
intervertebral discs disease and trauma of brachial plexus.
In case of
arterial TOS the symptoms can be similar as for any other ischemic disease like
atherosclerosis, trauma, dissection, some vasculitis and we have to consider
also another sources of embolization such as atrial fibrillation, aortic arch or
stenosis of subclavian artery origin.
If there is a
strong TOS suspicion based on rigorous medical history information it is time
for physical tests. First of them is Adson´s test which starts with palpable
pulsation above radial artery of sitting patient (+hearing of whirl above
clavicula). Than patient rises upper limb and turns head on the same and
opposite side of the limb. Test is positive if the pulsation gets weaker or
when whirl or paresthesia develops. Specificity of the test is not high, because
up to 50 % of healthy population have the positive test.
Second test is
so called EAST (Elevated Arm Stress Test), which is the most precise physical
test. Patient puts his/her arms in „I´m surrender“ position for 3 minutes with
closing and opening of hand. According to symptoms we can even distinguish type
of the TOS. In case of neurogenic TOS patient feels weight, weakness, numbness,
paresthesia from fingers moving proximally. In case of venous TOS there is
cyanosis and forearm vein enlargement. And in case of arterial TOS patient
feels ischemic spastic pain in the hand.
For confirmation
of TOS diagnose based on medical history and physical examination we can also
use imaging methods. Classic X-Ray can easily show bone anomalies in case of
neck and first limb and post-traumatic changes involving clavicula. Imaging of
soft tissue is however limited. The same findings can be acquired using CT or
MR scans. These examinations can rather exclude other causes of the patient’s
problems e.g. stenosis of subclavian artery origin.
Treatment of TOS
differs according to the TOS type. In case of neurogenic TOS the treatment is
most difficult and options limited. Main pillar of neurogenic TOS treatment is
physical therapy in form of guided rehabilitation, stretching and postural
exercise. This treatment can lead to relief of symptoms in up to 30% patients,
others require surgical treatment similar to arterial TOS. In case of venous
TOS the most effective approach in an acute state is a local thrombolysis with
possible PTA and consequent anticoagulation therapy. In case of severe
subclavian vein compression surgical treatment is indicated as well.
In case of
arterial TOS the basic treatment approach is surgical decompression, if the
aneurysm is found its resection and bypass surgery is indicated. In contrary to
vein surgery antiaggregating therapy is needed after this kind of arterial
surgery. To make surgical decompression there are two surgical site accesses –
transaxillar which provides better cosmetic outcome but it doesn´t allow to
reach upper structures and implant bypass and supraclavicular access which is
more technically difficult but it provides better surgical site view and is
feasible for vascular reconstruction.
You can see clear
subclavian artery compressing in costo-clavicular space on angiography image.
Operation site created by supraclavicular access, on the upper right picture is
scalene triangle. On the lower pictures is cut clavicula and subclavian artery
aneurysm, which was resected and replaced with PTFE prosthetic bypass.
archive of 2nd Department of Surgery
As any other
surgery TOS treatment has its specific complications. The most common are
injury of structures during the surgery, pneumothorax or post-operative
lymphorhea.
Treatment effect
of both surgical accesses is comparable. The effect of surgery can not be
assessed by physical tests, rather by data from medical history. The bigger is the
extend of symptoms before surgery and longer the time the worse outcome can be
anticipated. The treatment effect also depends on the etiology of the TOS. The
worst outcomes are after the TOS caused by long-term limb straining, better
outcomes are after treatment of TOS caused by trauma or acute condition.