Thoracic Outlet Syndrome Stephen Etheredge, MD, San Francisco, California Bruce Wilbur, MD, San Francisco, California Ronald J. Stoney, MD, San Francisco, California
Symptoms of vascular insufficiency resulting from compression of the subclavian vessels as they exit the thoracic outlet are rare. Failure to recognize those symptoms may result in prolonged disability or loss of the extremity. We are reporting our experience treating 11 patients, 5 with arterial complications and 6 with venous insufficiency. There were 10 male patients and 1 female patient ranging in age from 16 to 53 years, representing 15 per cent of all patients requiring surgical treatment of the thoracic outlet syndrome over the past 17 years at the University of California, San Francisco (UCSF). Arterial Complications
Five patients were referred to UCSF for ischemic symptoms of the forearm, hand, or fingers. The mqst common history was the relatively sudden onset of discomfort, coolness, and numbness in the hand or fingers. Exertional fatigue of the hand or forearm was noted by three patients. Reports of the original examination of these patients $dicatkd that all had cyanosis and coolness in one or more digits but that four of the five had palpable wrist pulses. The wrist pulses in one of these patients subsequently disappeared before our examination. In the patient with the most severe ischemia, the absence of palpable pulses at the original examination prompted embolectomy of the brachial and radial arteries. Failure From the Department of Surgery, University of California, San Francisco, California. Reprint requests should be addressed to Stephen Etheredge, MD, Dapartment of Surgery, University of California, San Francisco, California 94143. Presented at the 50th Annual Meeting of the Pacific Coast Surgical Association, Yosemite National Park, California, February 19-22, 1979.
Volume 138, July 1979
to relieve severe ischemia led to his referral to UCSF. At our initial evaluation, all five patients had evidence of unilateral ischemia of the digits, usually manifest as embolic, focal ischemia (Figure 1). A widened subclavian pulse was palpated in one patient and subclavian bruits were heard in three patients with the arm in a neutral position. Obliteration of pulses was demonstrated in all five patients during at least one of the three classic positional maneuvers (hyperabduction, military brace, or Adson’s manetiver) . Routine chest and cervical spine films demonstrated anomalous cervical ribs in two patients and hypoplastic first ribs in the remaining three patients. Subclavian arteriography demonstrated a subclavian arterial lesion in each patient. Arteriography with the patient’s arm in the common exaggerated positions demonstrated arterial compression but was not necessary to reach a diagnosis in these patients. Poststenotic subclavian arterial aneurysms were demonstrated in four patients and focal atherosclerosis with a discrete ulcer crater was discovered in the fifth patient (Figure 2). Runoff films revealed scattered embolic occlusions of the forearm vessels in three patients, including occlusion of the radial and ulnar arteries in the patient with severe ischemia (Figure 3). Operation. A supraclavicular approach to the subclavian artery was used in all patients, combined with an infraclavicular incision for distal control in one patient and with partial claviculectomy for greater exposure in another. All anomalous osseous structures were resected, including cervical ribs in
175
Etheredge et al
Figure 1. Focal ischemia of the fourth fingertip secondary to emboli.
two patients and hypoplastic first ribs in three. Cervical sympathectomy that included removal of the stellate ganglion and the second and third thoracic ganglia was performed in all patients. Four of the five patients underwent resection of the poststenotic dilatation with reconstruction by primary anastomosis (one), prosthetic graft (one), or arterial autograft (two). The fifth patient had focal atherosclerosis and ulceration that was removed by endarterectomy. The patient presenting with severe ischemia and absent wrist pulses was not relieved of symptoms with rib resection and arterial recon-
struction. He underwent a second procedure, a saphenous vein interposition graft from the axillary artery to the interosseous artery. Results (Table I). Postoperative follow-up period extends from 1 to 17 years. Three patients presenting with early signs of microembolization, who were treated with sympathectomy, resection of anomalous rib, and vascular reconstruction, have had no recurrent emboli and remain asymptomatic 2,3, and 17 years later. The patient who initially had wrist pulses but lost them before our initial evaluation still has mild “arm claudication” but no progression of symptoms. His arm is totally functional and his activity minimally limited. The bypass graft occluded in the single patient who was diagnosed late and failed to respond to the usual therapy; he eventually required a forearm amputation and has successfully been rehabilitated with a prosthesis. Comments. Most symptoms in patients with thoracic outlet syndrome originate in the brachial plexus compression. Fewer than 5 per cent of patients evaluated have vascular sequelae due to outlet compression [1,2]. Most descriptions of arterial complications of thoracic outlet syndrome list either subclavian arterial occlusion or excessive vasoconstriction, rather than embolization, as the cause of symptoms. Subclavian arterial occlusion was not present in any of our five patients. Initial signs of embolization with focal digital changes may account for diagnostic confusion with Raynaud’s syndrome.
Figure 2. Left subciavian arteriogram demonstrating compression by the cervical rib, mild poststenotic dilatation, and deep ulceration (arrow).
The American Journal of Surgery
Thoracic
Outlet Syndrome
Figure 3. Forearm “runoff” arteriogram demonstrating obstruction of the ulnar and radial arteries ( arrows). Note also the large filling defect in the interosseous artery.
Complete investigation is warranted in these patients with unilateral complaints of coolness, pallor, or pain. Careful physical examination with attention to signs of microembolization, digital arterial occlusion, obliteration of wrist pulses, increased size of the subclavian artery, or a subclavian artery bruit may establish a diagnosis of arterial injury with resultant microemboli. As stressed by others [3], positional testing with obstruction of the radial pulse in the various exaggerated positions does not permit a diagnosis of thoracic outlet syndrome to be made but does detect compression of vascular structures in that given position. Series have reported positional testing to be positive in 60 to 90 per cent [4,5] of asymptomatic patients.
TABLE I
As emphasized by Roos [6], the demonstration of cervical ribs that impinge on the vessels, hypoplastic first ribs that often join the second rib at the insertion of the anterior scalene muscle, and the elongated transverse process of the seventh cervical vertebra associated with type 2 congenital bands will aid in the diagnosis of arterial compression. Chest and cervical spine films demonstrated bony abnormalities in all of our patients. Arteriography remains the most specific and informative diagnostic test for chronic arterial wall changes associated with significant compression injury. Our present experience suggests that arteriograms should be obtained in any patient with symptoms of ischemia of the arms that could be caused by embolization. Because of the possible
Thoracic Outlet Arterial Compression Pathologic Condition
Mechanism of lschemia
Vascular Operation
Result
Follow-Up Period (yr)
Patient
Cause
ER
HYPOplastic first rib Cervical rib
Poststenotic aneurysm
Microemboli
Excision of aneurysm, autogenous arterial graft
Excellent
Poststenotic aneurysm
Microemboli
Forearm amputation
3.5
CD
HYPOplastic first rib
Poststenotic aneurysm
Microemboli
(1) Excision of prosthetic graft; (2) attempted saphenous axillary to interosseous artery bypass Excision, reanastomosis
Good
2
HR
HYPOplastic first rib Cervical rib
Poststenotic aneurysm
Microemboli
Excision, autogenous graft
Excellent
1.5
Focal atherosclerosis with ulceration
Microemboli
Thromboendarterectomy
Excellent
1
JD
MC
l
l
17
All patients had resection of first rib and cervical rib, if present, and all patients underwent cervical sympathectomy.
Volume 139,July 1979
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Etheredge et al
Figure 4. Phlebogram demonstrating thrombosis of the right axillary and subclavian veins (arrow) and prominent collateral veins.
presence of a friable mural lesion in the subclavian artery, the catheter for the arteriogram should not be advanced beyond the origin of the subclavian artery. Arteriography with the arm in exaggerated positions is useful in demonstrating the location and extent of arterial compression, but films with good penetration, exposed in the anteroposterior projection with the arm in a neutral position, were adequate for diagnosis in all five patients. Careful scrutiny and
subtraction techniques are often necessary to delineate minimal arterial wall changes. Films of the arteries of the forearm document distal embolic occlusion. The goals of surgical therapy are excision of the anatomic abnormality responsible for the compression injury of the arterial wall, removal of the source of microemboli with appropriate vascular reconstruction, and improvement of arterial blood flow to
Figure 5. Phfebogram demonstrating extrinsic compression of the subclavian vein in the costoclavicular space with perivenous fibrosis.
178
The American Journal of Surgery
Thoracic
the ischemic region by cervical sympathectomy. These three goals can be achieved from the supraclavicular approach. The functional results in our patients were inversely proportional to the degree of ischemia that the repetitive embolization had produced before operation. Early recognition, evaluation, and therapy should prevent the progression and functional loss such as occurred in two of our patients.
vious surgical procedures in the supraclavicular fossa (Figure 5). Prominent collaterals were present in the four patients with subclavian venous occlusion. Surgery. Transaxillary, extraperiosteal resection of the first rib was employed in five of the six patients in this group. In addition, venous thrombectomy was attempted through the supraclavicular approach in the only patient with symptoms of recent onset (2 weeks). Excision of perivenous fibrosis freed the subclavian vein in the patient with extrinsic compression. The sixth patient underwent resection of a tumor of the first rib (osteochondroma), second rib, and medial clavicle from an anterior approach to relieve compression of the subclavian vein. Results (Table II). Follow-up period ranged from 6 months to 4 years. Of the four patients with thrombosis of the subclavian vein, three are asymptomatic. Postoperatively occlusion of the subclavian vein recurred in the single patient who underwent venous thrombectomy. He continues to have minimal signs of edema with a slight increase in symptoms on exercise. The patient with resection of the first rib for tumor is completely asymptomatic, whereas the other patient with external compression is improved. Comment. Thrombosis of the axillary and subclavian veins (Paget-Schroetter syndrome) was first described by Paget in 1875 and by Van Schroetter in 1884. Thrombosis occurs in patients with congestive heart failure, mediastinal tumors, and chronic indwelling subclavian vein catheters. It also commonly occurs in laborers, painters, and truck drivers, hence the name “effort thrombosis.” Generally, there must be changes in the venous endothelium in addition to venous stasis for thrombosis to occur. Patients with structural abnormalities of the thoracic outlet have the mechanism necessary for these chronic compressive changes.
Venous Insufficiency
Six patients were referred to UCSF for symptoms of upper extremity venous hypertension. All were males engaged in manual labor or strenuous upper extremity exercise and in all symptoms developed in the dominant arm. In four of the patients arm edema, a feeling of heaviness, and slight cyanotic discoloration developed over a period of days. The same symptoms developed in the remaining two patients but over a longer period. Three of the six patients also complained that symptoms worsened after arm exercise. On examination, all six had edema of the involved arm, which was 1 to 8 cm larger in circumference than the uninvolved arm, and distended collateral veins particularly across the shoulder, chest, and supraclavicular region. Only one patient had a tender palpable cord in the axilla. Arterial pulses diminished or disappeared in all during positional testing. Routine chest and cervical spine films revealed no anatomic abnormalities except in the one patient with a tumor of the first rib. Phlebography, performed in all six patients, demonstrated subclavian venous thrombosis in four patients (Figure 4). Subclavian venous compression was present in the patient with a tumor of the first rib and in another with perivenous fibrosis presumably resulting from pre-
TABLE II
Outlet Syndrome
Thoradc Outlet Venous Compression Duration
Patient
Cause
Symptoms
(mo)
Follow-Up Period (yr)
Good
4
2 3
Excellent Excellent
1.5 0.5
1.5 yr
Transaxillary first rib resection
Excellent
1.5
1
Transaxillary first rib resection
Good
2
3
Supraclavicular resection of clavicle and first and second ribs
Excellent
1.5
Effort thrombosis
Arm edema
1
SF AF
Effort thrombosis Effort thrombosis
MW
Effort thrombosis
NG
Stenosis postoperatively, perivenous fibrosis Stenosis, osteochondroma of first and second ribs
Arm edema, tingling Arm edema, worse with exercise Arm edema, worse with exercise Arm edema, pain in axilla Arm edema
Volume 139,July 1979
Result
Transaxillary first rib resection, attempted venous thrombectomy Transaxillary first rib resection Transaxillary first rib resection
JG
SM
Operation
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Etheredge et al
Figure 6. A, artist’s drawing of subclavian vein compression in the costoclavicular space. 6, artist’s drawing demonstrating adequate decompression of the thoracic outlet by resection of the first rib with d&articulation of the costochondral joint.
An accurate diagnosis of occlusion of the subclavian vein is not difficult to make, but often the patient delays in seeking medical attention. Only one of our patients was seen by us less than 1 month from the onset of symptoms. Therapy for thrombosis of the subclavian vein has included anticoagulation, venous thrombectomy [ 71, and resection of the first rib together with venous thrombectomy [8] when diagnosed within days of its occurrence. Therapy for late consequences of thrombosis of the subclavian vein includes elastic bandages and avoidance of prolonged exercise or limb dependency. Adams and DeWeese [9] reported a single case in which initial thrombectomy failed to restore venous patency and eliminate the symptoms of venous outflow occlusion. Claviculectomy, performed 9 months after the initial symptoms, relieved exercise-induced venous hypertension, presumably by relieving clavicular compression of a large “first rib bypass venous collateral.” Similarly four of the patients in this series underwent first rib resection 2 weeks to 18 months after thrombosis of the subclavian vein. Decompression of the thoracic outlet with increased flow through the deep venous collaterals appears to have been a factor in the resolution of symptoms of venous hypertension in three patients and improvement in the fourth. The transaxillary approach to the first rib should offer the least risk of interruption of the venous collaterals. The first rib is the common denominator in the compressive areas of costocoracoid ligament and first rib, subclavian muscle and first rib, or clavicle and first rib (Figure 6A). Resection of the first rib with particular care to disarticulate the rib
180
at the costochondral joint should maximally enlarge the anterior portion of the thoracic outlet and relieve compression of the venous collaterals in the costoclavicular space (Figure 6B). All four patients with occlusion of the subclavian vein improved after decompression of the thoracic outlet, Subjective assessment alone has been obtained in these patients. Objective data such as limb plethysmography or venous outflow velocity have not been recorded. The clinical results lead us to recommend thoracic outlet decompression for those patients with symptomatic venous hypertension of a chronic nature. Summary
Eleven patients with vascular sequelae of thoracic outlet syndrome were operated on at the University of California, San Francisco, during the past 17 years. Five patients presented with episodes of ischemia of the arm and hand secondary to microemboli released from subclavian arterial lesions produced by chronic compression at the thoracic outlet. Treatment consisted of arterial reconstruction, removal of the compressive structure, and cervical sympathectomy to relieve or lessen distal ischemia. Four of the five patients had good or excellent results; one patient required amputation of the forearm. The results were inversely proportional to the extent of distal arterial embolic occlusions present at the time of surgical treatment. Six patients presented with symptoms of chronic venous hypertension. Four of the six had subclavian
The American Journal of Surgery
Thoracic
venous thrombosis and were treated by transaxillary resection of the first rib to decompress the collateral veins within the costoclavicular space. All four were symptomatically improved. Two patients had venous hypertension due to extrinsic compression of the subclavian vein. One patient became asymptomatic and the other was markedly improved after resection for external compression. In this small series transaxillary resection of the first rib has resulted in symptomatic improvement in chronic venous hypertension of the arm. References 1. Urschel HC Jr, Paulson DL, McNamara JJ: Thoracic outlet syndrome. Ann Thorac Surg 6: 1, 1968. 2. Roos DB: Experience with first rib resection for thoracic outlet syndrome. Ann Surg 173: 429, 1971. 3. Roes DB: Thoracic outlet and carpal tunnel syndromes, p 605. Vascular Surgery (Rutherford RB, ed). Philadelphia, WB Saunders, 1977. Raaf J: Surgery for cervical rib and scalenus anticus syndrome. JAMA 157: 219, 1955. Wright IS: Neurovascular syndrome produced by hyperabduction of the arms. Am Heart J 29: 1, 1945. Roos DB: Congenital anomalies associated with thoracic outlet syndrome. Arch Surg 132: 771, 1976. Drapanas T, Curran WL: Thrombectomy in the treatment of “effort” thrombosis of the axillary and subclavian veins. J Trauma 6: 107, 1966. 8. DeWeese JA, Adams JT, Gaiser DL: Subclavian venous thrombectomy. Circulation 41 (suppl): 158, 1970. 9. Adams JT, DeWeese JA: “Effort” thrombosis of the axillaty and subclavian vein. J Trauma 11: 923, 1971.
Discussion Louis L. Smith (Loma Linda, CA): Dr. Etheredge and his associates have focused on a small but very important group of patients with thoracic outlet compression, namely, those in whom arterial and venous complications have developed. The association of arteriosclerotic arterial stenosis and peripheral embolization is well understood in aortoiliac and carotid bifurcation disease. The investigators stress the importance of this mechanism in causing acute and chronic symptoms of the arm in patients with the thoracic outlet syndrome. Indeed, all five patients with arterial occlusive disease were thought to have symptoms on the basis of this mechanism. The investigators stressed the importance of a careful physical examination combined with arteriography in delineating disease in the subclavian artery as well as in the “runoff” circulation. Early diagnosis should prevent functional or anatomic loss in the involved arm. The investigators employ cervical sympathectomy routinely in the surgical management of patients with arterial involvement. Is this essential? Have they seen any complications such as Horner’s syndrome associated with this procedure? Our experience with subclavian venous occlusion associated with thoracic outlet compression parallels that of
Volume
138, July 1979
Outlet Syndrome
the investigators. We too recommend resection of the first rib for decompression after acute subclavian venous occlusion. Scientific demonstration of the value of this treatment is admittedly lacking. Hopefully, the investigators will continue studies including venous pressure measurements, limb plethysmography, and venous outflow velocity to clarify this point. Samuel N. Etheredge (Oakland, CA): I wonder if the investigators have noticed whether any physical type of person is more prone to the warning signs and symptoms, not necessarily to the eventual bad complications. Through the years I have noticed a distinct tendency among the male and female persons affected. The male is one who goes in for weightlifting and body building. I do not see a great many of these persons in the office, but over the years, of the number I have seen, 50 per cent had complaints that we thought were related to signs of the shoulder girdle syndrome. The women have been heavy breasted, many of whoti were not careful about the type of support they wore. A high percentage of persons with complaints of this nature have been those who have an extra drag that increases the tension effect at the costoclavicular angle. As regards claviculectomy, certainly in any problem case, particularly in those in which we have not shown by X-ray film any significant prominence of abnormality of the first rib and certainly not a cervical rib, if the vascular lesion is unusual, such as an aneurysm, claviculectomy performed extraperiosteally is excellent.. It is a big mistake to strip it out and leave the periosteum, because another clavicle is going to form, which may be a rough and irregular one. However, claviculectomy, performed properly, certainly lays the whole field wide open. I have done this procedure in relatively young, pretty women, and it is not disfiguring. Ronald J. Stoney (closing): Dr. Smith has questioned the need for cervical sympathectomy in patients with arterial insufficiency. Since the ischemic lesion is due to occlusion of small arterioles by microemboli, which evoke meager collateral circulation, loss of nutritive skin blood flow is a concern. Also, these small arteries are under sympathetic control, and vasodilatation resulting from sympathectomy augments skin blood flow as we know from experience with ischemia of the leg. Horner’s syndrome is anticipated after cervical sympathectomy, which in our practice includes removal of the stellate ganglion as well as the upper thoracic ganglion. There has been no concern from patients we have treated in that manner, and virtually all have some evidence of lid drooping, particularly when fatigued. This is the only, and for the most part trivial, side effect of sympathectomy. Subclavian venous compression injury resulting in thrombosis is a particularly challenging problem when seen in the chronic form. This occurred in all but one of our patients. Enlargement of the thoracic outlet to improve collateral flow through the first rib bypass collateral veins, as originally suggested by Adams and DeWeese, is an important consideration. Pre- and postoperative studies to
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et al
this are necessary, as Dr. Smith has so correctly pointed out, and we plan in future patients to attempt to document this response. Dr. Sam Etheredge asked about the habits and sex of these patients with venous thrombosis. We had only one female patient among the six patients with venous thrombosis, and she did not demonstrate any unusual physical characteristics that would alter the thoracic outlet anatomy. All the male patients were physically active, and document
182
their effort thrombosis developed in the dominant arm. In one patient, the effort that precipitated the thrombosis was a rock wall climb here in Yosemite Valley. We only used claviculectomy in one patient with arterial compression, to expose the entire subclavian and proximal axillary artery. In all the remaining patients, an arterial or venous supraclavicular approach or transaxillary resection of the first rib, respectively, was effective and avoided traversing potentially important venous collaterals.
The American Journal of Surgery
Symptoms of vascular insufficiency resulting from compression of the subclavian vessels as they exit the thoracic outlet are rare. Failure to recognize those symptoms may result in prolonged disability or loss of the extremity. We are reporting our experience treating 11 patients, 5 with arterial complications and 6 with venous insufficiency. There were 10 male patients and 1 female patient ranging in age from 16 to 53 years, representing 15 per cent of all patients requiring surgical treatment of the thoracic outlet syndrome over the past 17 years at the University of California, San Francisco (UCSF). Arterial Complications
Five patients were referred to UCSF for ischemic symptoms of the forearm, hand, or fingers. The mqst common history was the relatively sudden onset of discomfort, coolness, and numbness in the hand or fingers. Exertional fatigue of the hand or forearm was noted by three patients. Reports of the original examination of these patients $dicatkd that all had cyanosis and coolness in one or more digits but that four of the five had palpable wrist pulses. The wrist pulses in one of these patients subsequently disappeared before our examination. In the patient with the most severe ischemia, the absence of palpable pulses at the original examination prompted embolectomy of the brachial and radial arteries. Failure From the Department of Surgery, University of California, San Francisco, California. Reprint requests should be addressed to Stephen Etheredge, MD, Dapartment of Surgery, University of California, San Francisco, California 94143. Presented at the 50th Annual Meeting of the Pacific Coast Surgical Association, Yosemite National Park, California, February 19-22, 1979.
Volume 138, July 1979
to relieve severe ischemia led to his referral to UCSF. At our initial evaluation, all five patients had evidence of unilateral ischemia of the digits, usually manifest as embolic, focal ischemia (Figure 1). A widened subclavian pulse was palpated in one patient and subclavian bruits were heard in three patients with the arm in a neutral position. Obliteration of pulses was demonstrated in all five patients during at least one of the three classic positional maneuvers (hyperabduction, military brace, or Adson’s manetiver) . Routine chest and cervical spine films demonstrated anomalous cervical ribs in two patients and hypoplastic first ribs in the remaining three patients. Subclavian arteriography demonstrated a subclavian arterial lesion in each patient. Arteriography with the patient’s arm in the common exaggerated positions demonstrated arterial compression but was not necessary to reach a diagnosis in these patients. Poststenotic subclavian arterial aneurysms were demonstrated in four patients and focal atherosclerosis with a discrete ulcer crater was discovered in the fifth patient (Figure 2). Runoff films revealed scattered embolic occlusions of the forearm vessels in three patients, including occlusion of the radial and ulnar arteries in the patient with severe ischemia (Figure 3). Operation. A supraclavicular approach to the subclavian artery was used in all patients, combined with an infraclavicular incision for distal control in one patient and with partial claviculectomy for greater exposure in another. All anomalous osseous structures were resected, including cervical ribs in
175
Etheredge et al
Figure 1. Focal ischemia of the fourth fingertip secondary to emboli.
two patients and hypoplastic first ribs in three. Cervical sympathectomy that included removal of the stellate ganglion and the second and third thoracic ganglia was performed in all patients. Four of the five patients underwent resection of the poststenotic dilatation with reconstruction by primary anastomosis (one), prosthetic graft (one), or arterial autograft (two). The fifth patient had focal atherosclerosis and ulceration that was removed by endarterectomy. The patient presenting with severe ischemia and absent wrist pulses was not relieved of symptoms with rib resection and arterial recon-
struction. He underwent a second procedure, a saphenous vein interposition graft from the axillary artery to the interosseous artery. Results (Table I). Postoperative follow-up period extends from 1 to 17 years. Three patients presenting with early signs of microembolization, who were treated with sympathectomy, resection of anomalous rib, and vascular reconstruction, have had no recurrent emboli and remain asymptomatic 2,3, and 17 years later. The patient who initially had wrist pulses but lost them before our initial evaluation still has mild “arm claudication” but no progression of symptoms. His arm is totally functional and his activity minimally limited. The bypass graft occluded in the single patient who was diagnosed late and failed to respond to the usual therapy; he eventually required a forearm amputation and has successfully been rehabilitated with a prosthesis. Comments. Most symptoms in patients with thoracic outlet syndrome originate in the brachial plexus compression. Fewer than 5 per cent of patients evaluated have vascular sequelae due to outlet compression [1,2]. Most descriptions of arterial complications of thoracic outlet syndrome list either subclavian arterial occlusion or excessive vasoconstriction, rather than embolization, as the cause of symptoms. Subclavian arterial occlusion was not present in any of our five patients. Initial signs of embolization with focal digital changes may account for diagnostic confusion with Raynaud’s syndrome.
Figure 2. Left subciavian arteriogram demonstrating compression by the cervical rib, mild poststenotic dilatation, and deep ulceration (arrow).
The American Journal of Surgery
Thoracic
Outlet Syndrome
Figure 3. Forearm “runoff” arteriogram demonstrating obstruction of the ulnar and radial arteries ( arrows). Note also the large filling defect in the interosseous artery.
Complete investigation is warranted in these patients with unilateral complaints of coolness, pallor, or pain. Careful physical examination with attention to signs of microembolization, digital arterial occlusion, obliteration of wrist pulses, increased size of the subclavian artery, or a subclavian artery bruit may establish a diagnosis of arterial injury with resultant microemboli. As stressed by others [3], positional testing with obstruction of the radial pulse in the various exaggerated positions does not permit a diagnosis of thoracic outlet syndrome to be made but does detect compression of vascular structures in that given position. Series have reported positional testing to be positive in 60 to 90 per cent [4,5] of asymptomatic patients.
TABLE I
As emphasized by Roos [6], the demonstration of cervical ribs that impinge on the vessels, hypoplastic first ribs that often join the second rib at the insertion of the anterior scalene muscle, and the elongated transverse process of the seventh cervical vertebra associated with type 2 congenital bands will aid in the diagnosis of arterial compression. Chest and cervical spine films demonstrated bony abnormalities in all of our patients. Arteriography remains the most specific and informative diagnostic test for chronic arterial wall changes associated with significant compression injury. Our present experience suggests that arteriograms should be obtained in any patient with symptoms of ischemia of the arms that could be caused by embolization. Because of the possible
Thoracic Outlet Arterial Compression Pathologic Condition
Mechanism of lschemia
Vascular Operation
Result
Follow-Up Period (yr)
Patient
Cause
ER
HYPOplastic first rib Cervical rib
Poststenotic aneurysm
Microemboli
Excision of aneurysm, autogenous arterial graft
Excellent
Poststenotic aneurysm
Microemboli
Forearm amputation
3.5
CD
HYPOplastic first rib
Poststenotic aneurysm
Microemboli
(1) Excision of prosthetic graft; (2) attempted saphenous axillary to interosseous artery bypass Excision, reanastomosis
Good
2
HR
HYPOplastic first rib Cervical rib
Poststenotic aneurysm
Microemboli
Excision, autogenous graft
Excellent
1.5
Focal atherosclerosis with ulceration
Microemboli
Thromboendarterectomy
Excellent
1
JD
MC
l
l
17
All patients had resection of first rib and cervical rib, if present, and all patients underwent cervical sympathectomy.
Volume 139,July 1979
177
Etheredge et al
Figure 4. Phlebogram demonstrating thrombosis of the right axillary and subclavian veins (arrow) and prominent collateral veins.
presence of a friable mural lesion in the subclavian artery, the catheter for the arteriogram should not be advanced beyond the origin of the subclavian artery. Arteriography with the arm in exaggerated positions is useful in demonstrating the location and extent of arterial compression, but films with good penetration, exposed in the anteroposterior projection with the arm in a neutral position, were adequate for diagnosis in all five patients. Careful scrutiny and
subtraction techniques are often necessary to delineate minimal arterial wall changes. Films of the arteries of the forearm document distal embolic occlusion. The goals of surgical therapy are excision of the anatomic abnormality responsible for the compression injury of the arterial wall, removal of the source of microemboli with appropriate vascular reconstruction, and improvement of arterial blood flow to
Figure 5. Phfebogram demonstrating extrinsic compression of the subclavian vein in the costoclavicular space with perivenous fibrosis.
178
The American Journal of Surgery
Thoracic
the ischemic region by cervical sympathectomy. These three goals can be achieved from the supraclavicular approach. The functional results in our patients were inversely proportional to the degree of ischemia that the repetitive embolization had produced before operation. Early recognition, evaluation, and therapy should prevent the progression and functional loss such as occurred in two of our patients.
vious surgical procedures in the supraclavicular fossa (Figure 5). Prominent collaterals were present in the four patients with subclavian venous occlusion. Surgery. Transaxillary, extraperiosteal resection of the first rib was employed in five of the six patients in this group. In addition, venous thrombectomy was attempted through the supraclavicular approach in the only patient with symptoms of recent onset (2 weeks). Excision of perivenous fibrosis freed the subclavian vein in the patient with extrinsic compression. The sixth patient underwent resection of a tumor of the first rib (osteochondroma), second rib, and medial clavicle from an anterior approach to relieve compression of the subclavian vein. Results (Table II). Follow-up period ranged from 6 months to 4 years. Of the four patients with thrombosis of the subclavian vein, three are asymptomatic. Postoperatively occlusion of the subclavian vein recurred in the single patient who underwent venous thrombectomy. He continues to have minimal signs of edema with a slight increase in symptoms on exercise. The patient with resection of the first rib for tumor is completely asymptomatic, whereas the other patient with external compression is improved. Comment. Thrombosis of the axillary and subclavian veins (Paget-Schroetter syndrome) was first described by Paget in 1875 and by Van Schroetter in 1884. Thrombosis occurs in patients with congestive heart failure, mediastinal tumors, and chronic indwelling subclavian vein catheters. It also commonly occurs in laborers, painters, and truck drivers, hence the name “effort thrombosis.” Generally, there must be changes in the venous endothelium in addition to venous stasis for thrombosis to occur. Patients with structural abnormalities of the thoracic outlet have the mechanism necessary for these chronic compressive changes.
Venous Insufficiency
Six patients were referred to UCSF for symptoms of upper extremity venous hypertension. All were males engaged in manual labor or strenuous upper extremity exercise and in all symptoms developed in the dominant arm. In four of the patients arm edema, a feeling of heaviness, and slight cyanotic discoloration developed over a period of days. The same symptoms developed in the remaining two patients but over a longer period. Three of the six patients also complained that symptoms worsened after arm exercise. On examination, all six had edema of the involved arm, which was 1 to 8 cm larger in circumference than the uninvolved arm, and distended collateral veins particularly across the shoulder, chest, and supraclavicular region. Only one patient had a tender palpable cord in the axilla. Arterial pulses diminished or disappeared in all during positional testing. Routine chest and cervical spine films revealed no anatomic abnormalities except in the one patient with a tumor of the first rib. Phlebography, performed in all six patients, demonstrated subclavian venous thrombosis in four patients (Figure 4). Subclavian venous compression was present in the patient with a tumor of the first rib and in another with perivenous fibrosis presumably resulting from pre-
TABLE II
Outlet Syndrome
Thoradc Outlet Venous Compression Duration
Patient
Cause
Symptoms
(mo)
Follow-Up Period (yr)
Good
4
2 3
Excellent Excellent
1.5 0.5
1.5 yr
Transaxillary first rib resection
Excellent
1.5
1
Transaxillary first rib resection
Good
2
3
Supraclavicular resection of clavicle and first and second ribs
Excellent
1.5
Effort thrombosis
Arm edema
1
SF AF
Effort thrombosis Effort thrombosis
MW
Effort thrombosis
NG
Stenosis postoperatively, perivenous fibrosis Stenosis, osteochondroma of first and second ribs
Arm edema, tingling Arm edema, worse with exercise Arm edema, worse with exercise Arm edema, pain in axilla Arm edema
Volume 139,July 1979
Result
Transaxillary first rib resection, attempted venous thrombectomy Transaxillary first rib resection Transaxillary first rib resection
JG
SM
Operation
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Figure 6. A, artist’s drawing of subclavian vein compression in the costoclavicular space. 6, artist’s drawing demonstrating adequate decompression of the thoracic outlet by resection of the first rib with d&articulation of the costochondral joint.
An accurate diagnosis of occlusion of the subclavian vein is not difficult to make, but often the patient delays in seeking medical attention. Only one of our patients was seen by us less than 1 month from the onset of symptoms. Therapy for thrombosis of the subclavian vein has included anticoagulation, venous thrombectomy [ 71, and resection of the first rib together with venous thrombectomy [8] when diagnosed within days of its occurrence. Therapy for late consequences of thrombosis of the subclavian vein includes elastic bandages and avoidance of prolonged exercise or limb dependency. Adams and DeWeese [9] reported a single case in which initial thrombectomy failed to restore venous patency and eliminate the symptoms of venous outflow occlusion. Claviculectomy, performed 9 months after the initial symptoms, relieved exercise-induced venous hypertension, presumably by relieving clavicular compression of a large “first rib bypass venous collateral.” Similarly four of the patients in this series underwent first rib resection 2 weeks to 18 months after thrombosis of the subclavian vein. Decompression of the thoracic outlet with increased flow through the deep venous collaterals appears to have been a factor in the resolution of symptoms of venous hypertension in three patients and improvement in the fourth. The transaxillary approach to the first rib should offer the least risk of interruption of the venous collaterals. The first rib is the common denominator in the compressive areas of costocoracoid ligament and first rib, subclavian muscle and first rib, or clavicle and first rib (Figure 6A). Resection of the first rib with particular care to disarticulate the rib
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at the costochondral joint should maximally enlarge the anterior portion of the thoracic outlet and relieve compression of the venous collaterals in the costoclavicular space (Figure 6B). All four patients with occlusion of the subclavian vein improved after decompression of the thoracic outlet, Subjective assessment alone has been obtained in these patients. Objective data such as limb plethysmography or venous outflow velocity have not been recorded. The clinical results lead us to recommend thoracic outlet decompression for those patients with symptomatic venous hypertension of a chronic nature. Summary
Eleven patients with vascular sequelae of thoracic outlet syndrome were operated on at the University of California, San Francisco, during the past 17 years. Five patients presented with episodes of ischemia of the arm and hand secondary to microemboli released from subclavian arterial lesions produced by chronic compression at the thoracic outlet. Treatment consisted of arterial reconstruction, removal of the compressive structure, and cervical sympathectomy to relieve or lessen distal ischemia. Four of the five patients had good or excellent results; one patient required amputation of the forearm. The results were inversely proportional to the extent of distal arterial embolic occlusions present at the time of surgical treatment. Six patients presented with symptoms of chronic venous hypertension. Four of the six had subclavian
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venous thrombosis and were treated by transaxillary resection of the first rib to decompress the collateral veins within the costoclavicular space. All four were symptomatically improved. Two patients had venous hypertension due to extrinsic compression of the subclavian vein. One patient became asymptomatic and the other was markedly improved after resection for external compression. In this small series transaxillary resection of the first rib has resulted in symptomatic improvement in chronic venous hypertension of the arm. References 1. Urschel HC Jr, Paulson DL, McNamara JJ: Thoracic outlet syndrome. Ann Thorac Surg 6: 1, 1968. 2. Roos DB: Experience with first rib resection for thoracic outlet syndrome. Ann Surg 173: 429, 1971. 3. Roes DB: Thoracic outlet and carpal tunnel syndromes, p 605. Vascular Surgery (Rutherford RB, ed). Philadelphia, WB Saunders, 1977. Raaf J: Surgery for cervical rib and scalenus anticus syndrome. JAMA 157: 219, 1955. Wright IS: Neurovascular syndrome produced by hyperabduction of the arms. Am Heart J 29: 1, 1945. Roos DB: Congenital anomalies associated with thoracic outlet syndrome. Arch Surg 132: 771, 1976. Drapanas T, Curran WL: Thrombectomy in the treatment of “effort” thrombosis of the axillary and subclavian veins. J Trauma 6: 107, 1966. 8. DeWeese JA, Adams JT, Gaiser DL: Subclavian venous thrombectomy. Circulation 41 (suppl): 158, 1970. 9. Adams JT, DeWeese JA: “Effort” thrombosis of the axillaty and subclavian vein. J Trauma 11: 923, 1971.
Discussion Louis L. Smith (Loma Linda, CA): Dr. Etheredge and his associates have focused on a small but very important group of patients with thoracic outlet compression, namely, those in whom arterial and venous complications have developed. The association of arteriosclerotic arterial stenosis and peripheral embolization is well understood in aortoiliac and carotid bifurcation disease. The investigators stress the importance of this mechanism in causing acute and chronic symptoms of the arm in patients with the thoracic outlet syndrome. Indeed, all five patients with arterial occlusive disease were thought to have symptoms on the basis of this mechanism. The investigators stressed the importance of a careful physical examination combined with arteriography in delineating disease in the subclavian artery as well as in the “runoff” circulation. Early diagnosis should prevent functional or anatomic loss in the involved arm. The investigators employ cervical sympathectomy routinely in the surgical management of patients with arterial involvement. Is this essential? Have they seen any complications such as Horner’s syndrome associated with this procedure? Our experience with subclavian venous occlusion associated with thoracic outlet compression parallels that of
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the investigators. We too recommend resection of the first rib for decompression after acute subclavian venous occlusion. Scientific demonstration of the value of this treatment is admittedly lacking. Hopefully, the investigators will continue studies including venous pressure measurements, limb plethysmography, and venous outflow velocity to clarify this point. Samuel N. Etheredge (Oakland, CA): I wonder if the investigators have noticed whether any physical type of person is more prone to the warning signs and symptoms, not necessarily to the eventual bad complications. Through the years I have noticed a distinct tendency among the male and female persons affected. The male is one who goes in for weightlifting and body building. I do not see a great many of these persons in the office, but over the years, of the number I have seen, 50 per cent had complaints that we thought were related to signs of the shoulder girdle syndrome. The women have been heavy breasted, many of whoti were not careful about the type of support they wore. A high percentage of persons with complaints of this nature have been those who have an extra drag that increases the tension effect at the costoclavicular angle. As regards claviculectomy, certainly in any problem case, particularly in those in which we have not shown by X-ray film any significant prominence of abnormality of the first rib and certainly not a cervical rib, if the vascular lesion is unusual, such as an aneurysm, claviculectomy performed extraperiosteally is excellent.. It is a big mistake to strip it out and leave the periosteum, because another clavicle is going to form, which may be a rough and irregular one. However, claviculectomy, performed properly, certainly lays the whole field wide open. I have done this procedure in relatively young, pretty women, and it is not disfiguring. Ronald J. Stoney (closing): Dr. Smith has questioned the need for cervical sympathectomy in patients with arterial insufficiency. Since the ischemic lesion is due to occlusion of small arterioles by microemboli, which evoke meager collateral circulation, loss of nutritive skin blood flow is a concern. Also, these small arteries are under sympathetic control, and vasodilatation resulting from sympathectomy augments skin blood flow as we know from experience with ischemia of the leg. Horner’s syndrome is anticipated after cervical sympathectomy, which in our practice includes removal of the stellate ganglion as well as the upper thoracic ganglion. There has been no concern from patients we have treated in that manner, and virtually all have some evidence of lid drooping, particularly when fatigued. This is the only, and for the most part trivial, side effect of sympathectomy. Subclavian venous compression injury resulting in thrombosis is a particularly challenging problem when seen in the chronic form. This occurred in all but one of our patients. Enlargement of the thoracic outlet to improve collateral flow through the first rib bypass collateral veins, as originally suggested by Adams and DeWeese, is an important consideration. Pre- and postoperative studies to
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this are necessary, as Dr. Smith has so correctly pointed out, and we plan in future patients to attempt to document this response. Dr. Sam Etheredge asked about the habits and sex of these patients with venous thrombosis. We had only one female patient among the six patients with venous thrombosis, and she did not demonstrate any unusual physical characteristics that would alter the thoracic outlet anatomy. All the male patients were physically active, and document
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their effort thrombosis developed in the dominant arm. In one patient, the effort that precipitated the thrombosis was a rock wall climb here in Yosemite Valley. We only used claviculectomy in one patient with arterial compression, to expose the entire subclavian and proximal axillary artery. In all the remaining patients, an arterial or venous supraclavicular approach or transaxillary resection of the first rib, respectively, was effective and avoided traversing potentially important venous collaterals.
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