Pediatric orthopaedic surgeon Kevin Little, MD, didn’t expect to specialize in treating thoracic outlet syndrome (TOS) when he started at Cincinnati Children’s. But in recent years, his practice has seen a rapid increase in adolescents and young adults with this condition and one of its subtypes, Paget-Schroetter syndrome. 

Since 2011, roughly 150 families have traveled from all over the country to Cincinnati Children’s to consult with Little and fellow TOS expert Ann Schwentker, MD, a pediatric plastic surgeon. Other members of the TOS care team include interventional radiologists, hematologists, occupational and physical therapists, and a nurse practitioner.

TOS is a compression disorder that occurs when the brachial plexus or subclavian vessels are compressed proximally between the clavicle and first rib, a cervical rib and/or the anterior and middle scalene muscles, or distally by the pectoralis minor tendon insertion on the coracoid process of the scapula. A small percentage of patients with compression in this area develop Paget-Schroetter syndrome, in which compression in the thoracic outlet leads to thrombosis of the subclavian vein.

Continued Growth of TOS Surgeries

“Ten years ago, TOS was considered a condition mostly affecting middle-aged females. Back then at Cincinnati Children’s, we might have done one TOS decompression surgery a year, mostly for patients born with an extra rib,” says Little, who trained in TOS surgery as part of a fellowship at the Philadelphia Hand Center at Thomas Jefferson University. “But now we are seeing TOS in elite high school and college athletes, particularly females who are involved in repetitive overhead activities such as swimming, softball and volleyball.” 

Since its launch, the TOS program at Cincinnati Children’s has experienced steady growth, improving diagnosis and treatment for the condition. In 2021, Little says, he and Schwentker performed 38 TOS procedures. As of September 2022, 30 procedures have been performed. By the end of the year, the program expects to match 2021 volumes. 

Many pediatric centers lack the experience and expertise needed to diagnose and treat this condition. Little says that, on average, his TOS patients have seen five other specialists before coming to Cincinnati Children’s. Presenting symptoms may include pain, weakness, heaviness, numbness, tingling, swelling and discoloration of the arm, and presence of prominent veins. 

The diagnostic workup includes a physical exam and magnetic resonance imaging/magnetic resonance angiography (MRI/MRA) to view compression of vascular structures with the arms in different positions. Activity modification and physical therapy to decompress the affected area may be successful in patients with neurogenic TOS. Surgery is necessary when nonoperative methods fail or are not indicated, which is the case for about 90% of TOS patients at Cincinnati Children’s.

Prior to surgery, Little says, he and his colleagues collaborate with the Outpatient Pain Management Center to provide a multimodal approach to pain reduction. A combination of psychological strategies, injections and different medications can improve patient discomfort.

“If we can give patients temporary relief, it helps them understand that having surgery is the right decision,” he says. “They can see that we’re making the correct diagnosis. Then, this approach also helps them with a faster recovery.”

Anatomical Considerations in Pediatric Patients

Adults typically undergo an anterior scalenectomy to resolve TOS. But in pediatric patients, a first rib resection is needed as well, due to the significant anatomical compression in this area.

“The space between the clavicle and rib is already tight, which is one reason elite youth athletes are prone to this condition,” Little says. “In addition, some kids are at higher risk for TOS because they have an extra cervical rib, congenitally fused first and second rib or other abnormalities. We have operated on children as young as 6 years old with these anomalies.” 

Within six months of surgery, 85-90% of patients can return to their sport or preoperative activity with a complete resolution of symptoms. One of Little’s patients competed in the 2020 USA Swimming Olympic Trials.

Research to Improve Patient Outcomes

In addition to launching research to identify outcome measures that more specifically relate to TOS, Little and his colleagues at Cincinnati Children’s recently completed a study with MRI. They examined the effectiveness of using the modality to better identify the shoulder’s structural problems with TOS and Paget-Schroetter syndrome. 

By adding MRI images to the surgical process, they’ve accelerated their surgery speed and reduced their complication rate. Currently, they are writing a paper, detailing their findings with 64 patients treated at Cincinnati Children’s.

“We haven’t finalized the correlation to the MRI, but it’s really helping us think of where the problems are structurally. We’re able to better visualize the surgery and understand the pathology,” he says. “Different zones of compression have different symptoms associated with them. This helps us figure out how to treat them better.”