Abstract
I am a 17-year-old girl and was diagnosed with venous thoracic outlet syndrome (vTOS) last year. It took 7 months and numerous visits with different doctors to receive the accurate diagnosis of vTOS, and I ultimately had a first rib resection and venogram. Reasons for the delay in my diagnosis are (1) vTOS is hard to diagnose, especially in adolescents, (2) the relation between a fever and swelling, which was later attributed to a blood clot, was overlooked, and (3) initial scans and tests were not in the right areas or were reviewed by people unfamiliar with vTOS. Raising awareness of vTOS can help pediatricians, orthopedists, and vascular surgeons diagnose and treat vTOS in earlier stages, which will help patients be treated sooner and lower the chances of permanent blockage of their vein, while also lessening the stress, fear, and frustration of a long period without a diagnosis and action plan.
Keywords
Introduction
Thoracic outlet syndrome (TOS) occurs when there is compression of vessels or nerves in the thoracic outlet, an area that comprises the first rib, scalene muscles, and the clavicle. 1 There are 3 different variants of TOS, which are neurogenic, venous, and arterial, although neurogenic is by far the most common, representing 95% of TOS cases. Typical causes of TOS include trauma and repetitive overhead movement, and common symptoms include pain, numbness, tingling, vein blockage, blood clots, and swelling of the arm. 2 TOS is a rare condition, and venous TOS (vTOS) is even more rare—approximately 1 person per 100,000 presents with vTOS. 3 Although vTOS does occur in children and teenagers, the diagnosis is often missed or delayed because pediatric physicians may not consider that symptoms are due to this disorder and instead suspect simple muscle strains. 4
The repetitive overhead movement of my left arm, caused by competitive swimming and violin, led to the development of vTOS and a blood clot, which left scarring and severe occlusion of my subclavian vein, the main vein beneath the collarbone.
I first experienced TOS symptoms during a swim practice when my arm swelled significantly, causing significant pain. Shortly after, I had a fever, prompting me to see my pediatrician. The pediatrician thought the fever and swelling were likely unrelated and said to rest my arm for a couple of days. I then returned to the pool; however, my arm continued to swell every time I swam. I next saw a sports medicine orthopedist, who suspected triceps tendonitis and prescribed rest and physical therapy (PT).
I saw a PT for several months but had no relief in the pool, at which point I had a magnetic resonance imaging (MRI). However, the MRI only looked at osseous structures (the skeletal system), tendons, muscles, and tissue, and it is very difficult to spot TOS with an MRI. I also had two ultrasounds that month, looking for vessel compression and deep vein thrombosis in my left upper extremity. The results showed normal compression for my jugular, subclavian, and axillary veins, and TOS was not identified.
By this time, I had become frustrated, scared, and stressed because no one knew how to fix the problem, or even what it was, and I was in one of the biggest cities in the country with as many experts as any patient could wish for.
It was not until five months after the onset of symptoms that an angiography, imaging to visualize the inside of veins, of my upper extremity with and without intravenous contrast, showed a narrowing of the left subclavian vein arms down, and abrupt cutoff at the proximal bilateral subclavian veins arms up, in addition to prominent collateral, or detour, veins. The next month, a computed tomography scan suggested I had scarring from a resolved blood clot. At this point, it was clear my condition was a vascular issue, and I began seeing vascular surgeons. I made appointments with several vascular surgeons to get different points of view regarding treatment.
All the vascular surgeons I saw were very experienced, yet there were differences in views and recommendations: (1) conservative management with no surgery; (2) remove my first rib and see if symptoms resolve; (3) remove my first rib and have a venalysis, a dissection of the vein to remove fibers and scar tissue, in the same surgery; (4) rib resection and venous reconstruction; (5) rib resection and 2 weeks later, a venogram, imaging of the vein with contrast, followed by a brief balloon inflation to relieve the narrowing.
The differing pathways and surgeon opinions were overwhelming. I ultimately chose a recognized TOS expert who advised a first rib resection with a follow-up venogram. At that point, 6 months after my symptoms began, my subclavian vein was chronically occluded, so the likelihood of reopening and widening the vein was uncertain.
After the rib resection, my vein was patent, but I still had severe stenosis (about 90% narrowing) with multiple collateral veins draining into the jugular veins. However, after the venogram procedure two weeks later, I was relieved to learn that I had a widely patent subclavian vein with minimal flow through my collaterals, meaning my veins were working normally for the first time in seven months.
While the outcome for me ultimately was successful, my experience shows the importance of obtaining a proper diagnosis as early as possible to improve chances of a successful outcome and decrease the stresses that come along with every major medical journey.
Key Factors for Healthcare Providers to Consider
vTOS can be challenging to diagnose because (1) vTOS is rare, especially in pediatric cases, and symptoms often resemble those of other injuries, (2) fever and swelling as an indication of a blood clot can be overlooked, and (3) tests and scans may not immediately show vTOS.
vTOS is rare and symptoms resemble more common injuries. One reason vTOS is difficult to diagnose is that symptoms vary among patients and can be similar to symptoms of other conditions, such as triceps tendonitis and complex regional pain syndrome, especially for adolescents, as many physicians believe more common sports injuries and conditions are causing symptoms. Intense swelling in the arm, together with a fever, may be considered unrelated. While vascular surgeons were quicker to consider swelling and a fever to indicate a blood clot, other physicians—who will likely be seen first—may think the symptoms unrelated, resulting in delays before appropriate scans are ordered. Scans and tests may not show vTOS. Issues with the scans and tests can prolong the time it takes to diagnose vTOS. First, scans need to be done in the right location. My initial scans were too localized, focusing solely on the upper extremity and not the thoracic outlet region. Second, interpreting TOS usually requires experience, and sonographers may fail to identify it.
Recommendations for Patients Experiencing TOS Symptoms
For people with symptoms without a diagnosis, I recommend the following:
Be slow to rule out any condition: My physical therapist early on said I may have TOS, but when I relayed this to the sports medicine orthopedist, I was told TOS was unlikely. Extensive imaging: When getting scans, I recommend seeking extensive scans to not miss the region that is actually causing symptoms. Multiple opinions: Additionally, if feasible, seeing multiple doctors can be beneficial because it allows a patient to get various points of view, and can be especially important to hear new viewpoints if a patient is not making progress with their diagnosis.
If a patient has already been diagnosed with vTOS, I recommend the following:
Press for a quick appointment: When making appointments with vascular surgeons, a patient should indicate if they have vTOS or feel at risk of a blood clot, because otherwise, a patient could wait weeks or months for an appointment. Blood thinners: Research blood thinners and ask a physician if they could benefit. I did not learn about blood thinners until 6 months after the onset of symptoms and the blood clot. Specific surgical methods: Research the different ways surgeons approach first rib resections if that is a part of the treatment plan, as the location of the scar will vary. Opening of the vein post rib resection: Surgeons recommend a venography or venogram. Some did not mention this in the clinical visit and may have stopped after the first rib resection, which would have left me with tight stenosis >90% in my subclavian vein. The venogram was vital in opening my vein and relieving symptoms.
Conclusion
Since TOS is a rare condition, and vTOS even more rare, it is vital to raise awareness so that people with TOS and their physicians have the best chance of opening their vein before a permanent blockage and lifelong symptoms. While vascular surgeons are aware of the condition, pediatricians and orthopedists see many patients who have other, more common conditions with similar symptoms. As a result, it can take months to even get to vascular surgeons, and they may well have different views and recommendations even then. My experience with vTOS was a long and painful journey, which I hope to share with physicians and sonographers, as well as people experiencing vTOS symptoms, so that more people can have improved outcomes.
Declaration of Conflicting Interest
The author declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
