Upper Limb Deep Vein Thrombosis

A few months ago I had patient with a suspected Deep Vein Thrombosis, but unusually of the upper limb. As this is a very rare occurrence, and as I have only ever come across this potentially serious complication once before, I thought it would be good to write this case up and take a look at the literature around upper limb DVTs.

What is a DVT?

A thrombosis is a blood clot that occludes or blocks the normal flow of blood through either an artery or vein. A deep vein thrombosis (DVT) as the name implies occur in the deeper veins, and usually in the lower leg. However thromboses can occur anywhere in the body, I once had a patient who had one in the end of her thumb bizarrely. However, upper limb DVTs, in general, are very rare. In fact, it is estimated that only 4-11% of all DVTs are found in the upper limb (ref).
The risks from a DVT are not just from the restriction of blood circulation causing conditions such as compartment syndrome, tissue necrosis and pain, but more seriously there is real risk of death from conditions such as heart failure, stroke and pulmonary embolisms if the clot or parts of it break off and migrate to vital organs of the chest, heart or brain.
There are many causes and risk factors for DVTs, but in the upper limb, there are a few specific ones. Upper limb DVTs do occasionally occur spontaneously and out of the blue, whats called primary DVT, however, the majority, approx 80% of them are caused by some other predisposing factor. These are called secondary DVTs. See the table below for the common causes.

Chart adapted from this paper

As you can see the main causes for primary upper limb DVTs are due to congenital or acquired abnormalities of the thoracic outlet which can compress the neurovascular bundle causing a multitude of symptoms, venous or arterial Thoracic Outlet Syndrome is considered a type of upper limb DVT (ref)
Another cause of primary upper limb DVT is a condition called Paget-Schröegger Syndrome. This is an effort-induced thrombosis usually seen in those who do repeated overhead strenous activity, such as throwing or swimming. These activities can cause a repeated pinching of the subclavian vein between the first rib and the clavicle, causing thickening and narrowing of the veins lumen over time, and can eventually lead to its occlusion. More information on this rare condition can be found here.
However, the most common cause for an upper limb DVT are secondary factors, such as the insertion or removal of central line catheters, surgery or trauma to the neck or shoulder. (ref)

Clinical Presentation

My case of an upper limb DVT was secondary and was seen following surgery for a dislocated left acromioclavicular joint. The patient was a very fit and healthy 28 year old male, semi professional rugby player, with no past medical history.
He suffered a grade 3 ACJ dislocation after a heavy rugby tackle over a year ago. He tried conservative management of this injury with physiotherapy, but continued to suffer with pain and limited shoulder movement and was unable to return to playing.
The patient also reported a history of intermittent distal neural symptoms into his hand and lower arm such as sensory loss, coldness and cyanosis of his hand since the injury. These symptoms could often be eased with neck and shoulder girdle repositioning and was thought to be due to Thoracic Outlet Compression Syndrome triggered by altered positioning of his shoulder girdle following his injury.
Due to the lack of improvement in his symptoms it was decided to surgically stabilise his ACJ with the use of a clavicular hook plate. This was planned to be in situ for approximately 4 months, then to be removed once healing of the joint and ligaments had occurred. This method is the preferred approach for ACJ reconstructions used by the senior shoulder surgeon conducting the operation with very high success rates and low complications.
The operation was routine with no complications reported. The patient had an uneventful over night stay and was discharged the following day with advice on analgesia, use of a sling, and was shown basic passive movements of the shoulder, elbow, wrist and hand to be perfomed regularly through the day.
The patient attended a scheduled outpatient Physio appointment 8 days after surgery with myself.  Upon subjective assessment the patient reported very high levels of progressively worsening diffuse shoulder and upper arm pain over the last 3-4 days with no known trigger or cause. The pain was not eased with analgesia or any re positioning of the arm. He also described a now constant sensory loss in his lower arm and hand in a widespread diffuse pattern.
On examination the wounds were clean and healing well, however the patients upper arm was clearly distended and swollen, it felt hot to touch, the skin was mottled in appearance around the axilla, and the inner aspect if the arm and biceps were all very painful on even light palpation.
Due to these symptoms, a suspicion of either a post operative infection or upper limb DVT was suspected. My physio assessment was therefore halted, and after a telephone consultation with the surgeon an emergancy Doppler US scan was arranged immediately the same day.
Unfortunately the US scan was equivocal due to the radiologist having difficulty in positioning the patients arm due to his pain levels, and so was unable to clearly visualise the subclavian and axillary veins. Reports in the literature have described similar issues with US scans here, and also with the clavicle often shadowing and preventing the full visualisation of these vessels (ref). It is therefore recommended that patients with suspected upper limb DVT whose initial ultrasound is negative but who still have a high clinical suspicion of DVT undergo further testing with a D-dimer blood test, or if available a CT or MRI scan. (ref).

 
Due to availbality it was decided to obtain a D-dimer blood test. This was seen to be very highly elevated, and although false positives are common with D-dimers after surgery, it was decided to start antio-coagulation treatment immediately due to the highly suspicious clinical presentation.
This treatment commenced over a four week period and during this time the swelling and pain reduced in the patients upper arm, and sensation returned to normal. The standard post operative ACJ reconstruction physiotherapy program then resumed and the patient progressed well and made a full recovery after 10 weeks.
Although the removal of the hook plate was delayed by a further two months, it was removed without any further complications and the patient eventually returned to full gym based exercising and rugby training.

Discussion

This is an unusal case of an upper limb DVT in that it occured in a patient without any risk factors, such as history of central line catherisation, obseity, diabetes or any coagulation defects. However one paper has actually reported that upper limb DVTs may be more likely to be found in younger patients with lower BMI (ref).
The cause for this DVT remains unclear, but the symptoms of Thoracic Outlet Compression Syndrome before surgery should be suspected as a potential factor. This could possibly indicate an underlying congential issue, or even some venous lumen scarring and therefore thickening similar in nature to Paget-Schroegger Syndrome, which may have been aggravated by the surgery, or by shoulder girdle immobilisation, or increased depression of the shoulder girdle from pain inhibited neck and shoulder muscles.  All of these factors may have caused further neurovascular compression of the subclavian vessels between the clavicle and ribs and led to the occlusion and the formation of a thrombosis.
As far as I am aware no other cases like this have been reported in the literature of an upper limb DVT after a routine ACJ stabilisation operation. There was no reason to suspect this rare complication would occur in this patient with no pre disposing past medical factors, or with this type of injury, undergoing this type of surgery, therefore the use of prophylactic measures was not indicated. However, the effectivness of any preventative strategies such as anticoagulation prophylaxis for upper limb DVTs is unclear and debatable, and realistically these should only been considered in patients with clear identified risk factors, which this patient did not have (ref).
This case does highlight the need for clinicans to be vigilant and aware of this rare but potenital life threatening condition in those without the usual risk factors. Clinicians need to be able to spot the symptoms and know the appropriate course of action when a upper limb DVT is suspected.
As always thanks for reading
And remember “if in doubt get it checked out”
Adam