Treatment
TOS Physical Therapy
With an NCV exceeding 60 m/sec, the patient usually improves from conservative physical therapy. Initially, most patients, except those with vascular and motor nerve problems, were treated conservatively with physical therapy. The primary goals of physical therapy are to open up the space between the clavicle and first rib, improve posture, strengthen the shoulder girdle, and loosen the neck muscles. This is accomplished by pectoralis stretching, strengthening the muscles between the shoulder blades, good posture, and active neck exercises, including chin tuck, flexion, rotation, lateral bending, and circumduction.
Most patients with neurologic TOS receive physiotherapy before operative intervention. Such therapy must not be the same as given to patients with the cervical syndrome, which often exaggerates the symptoms of thoracic outlet compression. Proper physiotherapy for thoracic outlet compression includes heat massages, active neck exercises, scalenus anticus muscle stretching, strengthening of the upper trapezius muscle, and posture instruction. Because sagging of the shoulder girdle, common among middle-aged people, is a main etiologic factor in this syndrome, many patients with less severe disease benefit from strengthening the shoulder girdle and improving posture. More than two-thirds of neurogenic TOS patients seen in consultation required no surgical procedure, but improved significantly with conservative management.
The normal NCV across the thoracic outlet is 85m/s. Most patients with a NCV above 60 m/s improve with conservative management. Most patients with a NCV below 60 m/s require surgical resection of the first rib and correction of other bony abnormalities.
Physical therapy is not used in patients with PSS, acute arterial insufficiency or neurological motor problems because of the urgency for immediate interventional therapy in these situations. The primary use for physical therapy and conservative management trials is for primary or recurrent neurologic sensory thoracic outlet syndromes. If the patient develops motor deficits they should have prompt first rib removal and neurovascular decompression.
For Neurological Sensory TOS Symptoms:
1) Conservative Management with improvement of posture
2) Physical Therapy to strengthen shoulder
Upon failure of conservative management and physical therapy, the patient should consider surgical intervention.
Indications for Surgery
The usual indications for surgery in neurological sensory TOS are failure of conservative management and appropriate physical therapy in a patient with a significantly reduced NCV (60 m/s; normal is 85 m/s) and the elimination of other possible etiologies for the symptoms. Patients with motor nerve TOS, and arterial or venous TOS are recommended to have surgery.
Initial surgical therapy involves complete first rib resection, anterior scalenectomy, resection of the costoclavicular ligament, and neurolysis of C7, C8, and TI nerve roots and the brachial plexus through a transaxillary approach for nerve and venous TOS. Various instruments have been developed to expedite the procedure. The first rib with the compressive elements may also be removed using the supraclavicular approach in patients with arterial TOS. This approach, however, requires working through and retracting the brachial plexus and produces a visible scar in women (the preponderant gender with TOS). The posterior thoracoplasty approach for first rib resection may be used for initial therapy, but in our clinic it is reserved for a second procedure and neurolysis of the brachial plexus. Cervical ribs may be removed using any of the approaches described. Dorsal sympathectomy may also be performed with neurovascular decompression through any of the above incisions for sympathetic maintained pain syndrome (SMPS), reflex sympathetic dystrophy (RSD), causalgia, and Raynaud's phenomenon and disease.
TOS can occur in older patients (the oldest reported has been 87 years). However, when nerve compression symptoms occur in patients older than 60, causes such as degenerative or traumatic cervical spine, cardiac, or pulmonary pathologies should be suspected and ruled out.
Surgical Techniques
Transaxillary Resection of the First Rib and Neurovascular Decompression
For primary venous or neurologic TOS, resistant to conservative management, the preferred approach is the transaxillary resection of the first and/or cervical rib with neurovascular decompression. Because most patients are women, the scar is unobtrusive and difficult to visualize; no major muscles are divided, and the cervical or first rib may be removed directly without retraction of the brachial plexus or blood vessels. The wound, however, is deeper, and more light and magnification are required, and greater experience is necessary, than for the other approaches. The costoclavicular ligament in Paget-Schroetter Syndrome is readily available from this approach and difficult to remove from the supraclavicular or posterior approaches. The transaxillary approach is favored because the vein and costoclavicular ligament are best visualized. Also, the neurovascular structures are away from the dissection and do not have to be retracted, thus, minimizing their injury.
The patient is placed in the lateral position with the involved extremity gently supported by forearm traction straps attached to an overhead pulley with 1 to 2 pounds of weight. Two arm holders prevent hyperabduction of the shoulder beyond 90°. After prepping and draping the axilla and arm, a transverse incision is made below the axillary hairline between the pectoralis major and latissimus dorsi muscles. The dissection is carried through the skin, subcutaneous tissue to the chest wall, and extended cephalad to the first rib. Care is taken to prevent injury to the intercostal brachial cutaneous nerve, which passes between the first and second ribs to the subcutaneous tissue in the center of the operative field. With gentle dissection, the neurovascular bundle is identified and its relation to the first rib and both scalene muscles is clearly outlined to avoid injury to these structures.
In most instances of PSS there is a significant inflammatory reaction, which usually “plasters” the neurovascular structures down to the chest wall. This obliterates access to the first rib, requiring careful dissection of the neurovascular structures away from the ribs. Further contributing to the difficulty of surgical dissection is the usual occlusion of the axillary-subclavian vein, eliminating its blue color, which is usually a crucial anatomic landmark in the normal axillary dissection.
The scalenus anticus muscle is divided and resected up into the neck to avoid reattachment to Sibson’s fascia. The lung on the operated side is temporarily collapsed with a double-lumen endotracheal tube to expedite safe dissection. The first rib is dissected subperiostally and carefully separated from the underlying pleura to avoid pneumothorax. The rib is divided and a triangular portion removed, with the vertex of the triangle at the scalene tubercle. The anterior portion of the rib is dissected carefully from the vein, the costoclavicular ligament is divided and resected, and the rib is detached at its sternal cartilaginous junction. All compression bands and adhesions are removed from the axillary subclavian vein, and the anterior venous compartment is thoroughly decompressed.
The posterior segment of the rib is carefully dissected subperiostally from the subclavian artery and brachial plexus posteriorly. The scalenus medius muscle is dissected from the rib. The rib is divided near its articulation with the transverse process of the vertebra. Complete removal of the neck and head of the first rib is achieved with long, specially reinforced double-action Urschel-pituitary and Urschel-Leksell rongeurs. The eighth cervical and first thoracic nerve roots as well as the brachial plexus undergo careful neurolysis. If a cervical rib is present, it is removed and the seventh cervical nerve root is decompressed. Meticulous hemostasis is accomplished. Only the subcutaneous tissues and skin require closure, because no large muscles have been divided. The patient is encouraged to use the arm normally and can usually be discharged from the hospital on the second day after the surgical procedure.
It is preferable to remove the entire first rib, including head and neck, to avoid future bony (or fibrocartilage) regeneration and irritation of the plexus. For recurrent symptoms, removal of incompletely resected or regenerated rib and lysis of adhesions can best be accomplished through the posterior “high thoracoplasty” approach.
Through the transaxillary approach, the vein is one of the landmarks for the dissection of the first rib because of its blue color. In patients with Paget-Schroetter Syndrome, the vein is generally occluded or markedly thickened and there is usually no blue color. It is the same color as the other structures, such as the artery, muscle, etc. Because of this, many surgeons who do not routinely perform this operation have difficulty locating the anatomic structures. This is important to understand, and other landmarks must be used to provide the proper orientation. Extremely long surgeries (up to 8 hours) have been reported by surgeons who cannot seem to find the vein when it is totally occluded. This increases the incidence of nerve injuries.
In most patients that have been operated less than 6 weeks after thrombosis, there is usually a severe inflammatory reaction around the neurovascular structures and first rib. These structures may be "plastered down" to the first rib making the dissection hazardous. Several cases that had previous breast implants placed through the axilla were included in this group of patients. Because of this, structures such as the vein, artery, and brachial plexus may be intimately adherent to the first rib, significantly increasing symptoms and increasing the risk of the operative procedure. This should be suspected in such situations and extra care should be taken not to injure any of the neurovascular structures. If an interval clot recurs or was not relieved by the thromboytic agents, the external venous compression structures are removed (mostly the abnormal costoclavicular ligament), and the clot usually lyses spontaneously recanalyzing the vein. “Roto-rooter” techniques have not been ideal.
Supraclavicular Approach
The supraclavicular approach is reserved for patients with arterial insufficiency or arterial aneurysm who require bypass therapy. A proximal subclavian arterial anastomosis and an infraclavicular brachial or axillary artery anastomosis is necessary in most cases. The disadvantage of this incision is the cosmetic scar being visible in most cases and the necessity of retracting the brachial plexus and blood vessels to remove the first rib. Thus, there is a higher incidence of nerve injury in this approach as compared with the transaxillary technique.
Reoperation for recurrent TOS is best accomplished via the high posterior thoracoplasty approach with a muscle-splitting incision of the trapezius and rhomboids. This provides an excellent approach to remove bone remnants and to the brachial plexus for neurolysis.
Other approaches include the infraclavicular approach and the transthoracic thoracoscopic rib removal as described by Wolf and Associates, as well as combined procedures.
Posterior High Thoracoplasty
The preferred technique for reoperation is the posterior, high thoracoplasty, muscle-splitting incision with removal of first rib stumps, neurolysis of C7, C8, and T1 nerve roots and the brachial plexus and a Dorsal Sympathectomy.
The patient is placed in the lateral position with an axillary roll under the “down” side. The upper arm is placed as for a thoracotomy. An incision is made approximately 6 cm in length with the midpoint at the angle of the scapula. It is halfway between the scapula and the spinous processes. The incision is carried through the skin and subcutaneous tissue down to the trapezius muscle. After dissecting appropriate subcutaneouse flaps, the trapezius and rhomboid muscles are split in the direction of their fibers
The posterior superior serratus muscle is resected and the first rib stump identified by retracting the sacrospinalis muscle medially. Cautery is used to expose the first rib remnant (“stump”) and to open the periostium. A periosteal elevator; or “joker”, is employed to remove the stump subperiosteally. The head and the neck of the rib usually have not been removed in the initial operation. The rib shears are used to divide the rib remnant, and the reinforced Urschel-Leksell and Urschel pituitary rongeurs are employed carefully to remove the head and neck of the rib.
Once the T1 nerve root is identified grossly or with a nerve stimulator, neurolysis is carried out using magnification, a right angle clamp, a knife, and special microscissors. A nerve stimulator may be helpful if extensive scarring is present. Neurolysis is extended to the C7 and C8 nerve roots and to the brachial plexus. All the scar is removed as far forward as necessary so that the nerve roots as well as the upper, middle, and lower trunks of the brachial plexus are free. Care is taken not to injure the long thoracic nerve or any other brachial plexus branch. The axillary subclavian artery and vein are decompressed through the same incision.
The second rib is dissected free and the cautery used to open the periosteum linearly. A 1 cm segment of the rib is resected posteriorly, medial to the sacrospinalis muscle, in order to perform the Dorsal Sympathectomy. (This exposure may also help identify the T1 nerve root.)
After the head and neck of the second rib are removed, the sympathetic chain is identified on the pleura. The stellate ganglion lies in a transverse rather than vertical position.
The lower third of the stellate ganglion is incised sharply (T1) and the gray and white rami communicans are clipped and divided. The T1, T2, and T3 ganglia are removed along with the sympathetic chain using clips on all of the branches. Cautery is employed to effect hemostasis and to minimize sprouting and regeneration of the sympathetic chain. After antibiotic solution is irrigated, Depo-Medrol and Hyaluronic Acid are left on the areas of neurolysis. The wound is closed in layers with interrupted #1 Neurlon in a figure of eight fashion (Tom Jones stitch) in each of the muscle layers. Running and interrupted 2-0 Vicryl sutures are used in the subcutaneous tissue and in the skin. A large round Jackson-Pratt drain is placed in the area of neurolysis through a separate stab wound 2 cm below the inferior part of the incision. Care is taken not to incorporate the drain while closing the muscle layers over the top.
Dorsal Sympathectomy
Historically, the anterior cervical approach to the cervical sympathetic chain has been used. The stellate ganglion lies on the transverse process of C6, and this approach is used primarily by neurosurgeons and vascular surgeons. For hypertension, Smithwick and Urschel popularized the posterior approach, with a longitudinal paraspinal incision with the patient in the prone position. Small pieces of the second ribs are removed, and the sympathetic chain is identified in the usual position. This approach has the advantage of allowing bilateral procedures to be performed at the same time without changing the patient's position. The most common current approach is the transaxillary, trans-thoracic approach, which is performed through the second interspace with a transverse subhairline incision. This is more painful than the other approaches, but with video-assisted thoracoscopy it can be performed with minimal discomfort.
The approach most frequently used for TOS and Dorsal Sympathectomy is the transaxillary approach, during the first rib resection. This causes minimal pain and combines two procedures with a low morbidity rate. Video assistance is also used frequently with this approach.
If symptoms recur, they happen, on average, in 3 years (range 6 months to 25 years). This is most likely from sprouting, or failure to strip the artery of its sympathetic nerves. This complication seems to occur less often if the bed of the sympathetic chain is cauterized after dorsal sympathectomy. It can also be explained by high circulating concentrations of catecholamines.