Axillary-subclavian venous occlusion: morbidity of a nonlethal disease The Peter Gloviczki, M.D., Francis J. Kazmier, M.D. and Larry H. Homer, M.D., Rochester, Minn. To evaluate results of medical and surgical treatment of axillary-subclavian venous oc- clusion, the clinical courses of 95 patients were reviewed. Twenty-three patients had acute axillary-subclavian venous thrombosis, and 72 patients had chronic occlusion. Thirty- four patients with thoracic outlet syndrome and axillary-subclavian occlusion represented 3.5% of the 969 patients treated for thoracic outlet syndrome during the same period. Nonlethal pulmonary embolization from the axillary-subclavian vein occurred in four patients. Sixty percent of patients were asymptomatic or had mild symptoms during strenuous exercise at last follow-up (mean, 5.4 years). Forty-eight of these 56 patients had received anticoagulation during the acute phase of the disease. Twenty-seven percent of patients had symptoms with moderate exercise and I2.6% had symptoms at rest. Thirteen patients had operations, with improvement demonstrable in 10 patients. All five patients who underwent first rib resection for intermittent venous occlusion or for thoracic outlet syndrome after thrombosis occurred on the contralateral side did well. Axillary- subclavian venous occlusion is a nonlethal disease but late sequelae occur in one third of patients. Early anticoagulation appears to be beneficial and, in some patients with con- cominant thoracic outlet syndrome, first rib resection also appears to be helpful. Further data are needed to evaluate results of fibrinolytic treatment, thrombectomy, and venous reconstruction. (J VAsc SuRG 1986; 4:333-7.) The clinical picture of axillary venous thrombosis was described more than a century ago by Sir James Paget and von Schroetter. When compared with ve- nous thrombosis in the lower extremities, its inci- dence is low with only about 2% of all episodes of venous thrombosis occurring in the upper extremi- ties.1 However, the incidence of the disease seems to have increased with the widespread use of diagnostic and therapeutic catheters. 2s The origin is frequently difficult to define and the clinical picture ranges from severe discomfort and edema including the forearm and hand, to few or no symptoms at all. Optimal treatment is still contro- versial and the role of surgery in the management of the disease is often questioned. To clarify the causes and evaluate the morbidity and results of medical and surgical treatment, we retrospectively reviewed the clinical course of 95 con- From the Section of Vascular Surgery, Department of Surgery and Department of Cardiovascular Medicine, Mayo Clinic and Mayo Foundation. Presented at the Tenth Annual Meeting of the Southern Associ- ation for Vascular Surgery, Cerromar Beach, Puerto Rico, Jan. 30-Feb. 1, 1986. Reprint requests: Larry H. Hollier, M.D., Mayo Clinic, 200 Southwest First Street, Rochester, MN 55905. secutive patients with clinical diagnosis of acute or chronic axillary-subclavian venous occlusion. MATERIAL AND METHODS Between Jan. 1, 1976 and March 31, 1985, 95 patients were seen at the Mayo Clinic with the clinical diagnosis of axillary-subclavian venous occlusion. There were 53 male and 42 female patients with a mean age of 42.8 years (range, 15 to 88 years) (Fig. 1). Twenty-three patients had acute axillary-subcla- vian venous thrombosis defined as the presence of symptoms of less than 5 days' duration. Seventy-two patients had chronic venous occlusion. Symptoms of acute venous thrombosis included pain, tightness and discomfort during exercise, edema, cyanosis, an in- creased venous pattern, and tenderness over the ax- illary vein. Gangrene of the extremity developed in one patient. Patients with chronic venous occlusion complained of a heavy feeling of the limb, pain and burning discomfort with exercise, edema, an in- creased venous pattern around the shoulder and up- per arm, and occasionally paresthesia and numbness. The right upper extremity was involved in 51 patients and the left in 43 patients. One patient had docu- mented bilateral axiUary venous occlusion. 333
Transcript
Axillary-subclavian venous occlusion: morbidity of a nonlethal disease
The
Peter Gloviczki, M.D. , Francis J. Kazmier, M.D. and Larry H. H o m e r , M.D. , Rochester, Minn.
To evaluate results of medical and surgical treatment of axillary-subclavian venous oc- clusion, the clinical courses of 95 patients were reviewed. Twenty-three patients had acute axillary-subclavian venous thrombosis, and 72 patients had chronic occlusion. Thirty- four patients with thoracic outlet syndrome and axillary-subclavian occlusion represented 3.5% of the 969 patients treated for thoracic outlet syndrome during the same period. Nonlethal pulmonary embolization from the axillary-subclavian vein occurred in four patients. Sixty percent of patients were asymptomatic or had mild symptoms during strenuous exercise at last follow-up (mean, 5.4 years). Forty-eight of these 56 patients had received anticoagulation during the acute phase of the disease. Twenty-seven percent of patients had symptoms with moderate exercise and I2.6% had symptoms at rest. Thirteen patients had operations, with improvement demonstrable in 10 patients. All five patients who underwent first rib resection for intermittent venous occlusion or for thoracic outlet syndrome after thrombosis occurred on the contralateral side did well. Axillary- subclavian venous occlusion is a nonlethal disease but late sequelae occur in one third of patients. Early anticoagulation appears to be beneficial and, in some patients with con- cominant thoracic outlet syndrome, first rib resection also appears to be helpful. Further data are needed to evaluate results of fibrinolytic treatment, thrombectomy, and venous reconstruction. (J VAsc SuRG 1986; 4:333-7.)
The clinical picture of axillary venous thrombosis was described more than a century ago by Sir James Paget and von Schroetter. When compared with ve- nous thrombosis in the lower extremities, its inci- dence is low with only about 2% of all episodes of venous thrombosis occurring in the upper extremi- ties.1 However, the incidence of the disease seems to have increased with the widespread use o f diagnostic and therapeutic catheters. 2s
The origin is frequently difficult to define and the clinical picture ranges from severe discomfort and edema including the forearm and hand, to few or no symptoms at all. Optimal treatment is still contro- versial and the role of surgery in the management of the disease is often questioned.
To clarify the causes and evaluate the morbidity and results of medical and surgical treatment, we retrospectively reviewed the clinical course of 95 con-
From the Section of Vascular Surgery, Department of Surgery and Department of Cardiovascular Medicine, Mayo Clinic and Mayo Foundation.
Presented at the Tenth Annual Meeting of the Southern Associ- ation for Vascular Surgery, Cerromar Beach, Puerto Rico, Jan. 30-Feb. 1, 1986.
Reprint requests: Larry H. Hollier, M.D., Mayo Clinic, 200 Southwest First Street, Rochester, MN 55905.
secutive patients with clinical diagnosis o f acute or chronic axillary-subclavian venous occlusion.
M A T E R I A L A N D M E T H O D S
Between Jan. 1, 1976 and March 31, 1985, 95 patients were seen at the Mayo Clinic with the clinical diagnosis o f axillary-subclavian venous occlusion. There were 53 male and 42 female patients with a mean age of 42.8 years (range, 15 to 88 years) (Fig. 1).
Twenty-three patients had acute axillary-subcla- vian venous thrombosis defined as the presence of symptoms o f less than 5 days' duration. Seventy-two patients had chronic venous occlusion. Symptoms of acute venous thrombosis included pain, tightness and discomfort during exercise, edema, cyanosis, an in- creased venous pattern, and tenderness over the ax- illary vein. Gangrene of the extremity developed in one patient. Patients with chronic venous occlusion complained o f a heavy feeling of the limb, pain and burning discomfort with exercise, edema, an in- creased venous pattern around the shoulder and up- per arm, and occasionally paresthesia and numbness. The right upper extremity was involved in 51 patients and the left in 43 patients. One patient had docu- mented bilateral axiUary venous occlusion.
333
334 Gloviczki, Kazmier, and Hollier
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C5
25
20
15
10
J A /k I \ I \ I ' ,
I \ I
I I I I I I I I
10 20 30 40 50 60 70 80 Mean age: 42.8 years (Ranges: 15 to 88 years) Age
Fig. 1. Sex and age distribution of 95 patients (male/female ratio, 53:42) with axillary- subclavian venous occlusion. Peak age for incidence of disease in male patients was between 20 and 30 years, reflecting the incidence of effort-related thrombosis in men.
Table I. Origin of axillary-subclavian venous occlusion
No. of patients
Effort thrombosis 36 Thoracic outlet syndrome 34* Central venous access line 10 Previous surgery 9 Trauma 7 Idiopathic 7 Drug-induced (estrogen, vasopressor) 6 Pacemaker implantation 6 Tumor 5 Intravenous injection of contrast medium 2 Progression of acute arterial thrombosis _.L._I
Total 95
*Twenty-eight patients had effort-related thrombosis.
Thirty-six patients had symptoms develop after physical activity and these patients were classified as having effort-related venous thrombosis. The rela- tionship to physical activity was not clear-cut in 5 of the 36 patients. The origin of"effort" venous throm- bosis was related to the following physical activities: lifting or pulling heavy objects (fifteen patients); bas- ketball, baseball, and painting (three patients each activity); tennis (two patients); and softball, rac- quetball, football, golf, wrestling, weightlifting, scrubbing, shoveling snow, swinging rifle, and long bus ride with hand on strap (one patient each ac- tivity).
On the basis of signs and symptoms of neuro- vascular compression at the. thoracic outlet, 28 of these 36 patients were diagnosed as having a thoracic outlet syndrome. Six additional patients had thoracic
outlet syndrome without effort-related venous thrombosis. The 34 patients with axillary-subclavian venous occlusion and thoracic outlet syndrome rep- resent 3.5% of a total of 969 patients seen during the same period for thoracic outlet syndrome. Ten patients had clinical axillary-subclavian venous thrombosis after central venous line placement, but only six patients (0.18%) had manifest venous thrombosis of 3410 patients who underwent pace- maker implantation during this period of observa- tion. In seven otherwise healthy patients in whom a cause could not be established, the disease was called idiopathic. Additional causes of axillary-subclavian venous occlusion are listed in Table I.
The diagnosis was established by history and physical examination, with the use of noninvasive diagnostic methods (venous Doppler, real-time B- mode ultrasound, and venous plethysmography) as well as by CT scan with intravenously administered contrast medium, isotope phlebography, and con- ventional contrast phlebography. Contrast phlebog- raphy was carried out in 51 patients. Other diag- nostic modalities, either alone or in combination, confirmed the diagnosis in the remaining cases.
Treatment for patients who had acute venous oc- clusion included elevation of the extremity, heat, anti- inflammatory drugs, antibiotics, anticoagulation with heparin and/or warfarin, fibrinolytic therapy, and venous thrombectomy. Patients with chronic ve- nous occlusion were advised to limit exercise, elevate the limb, and to use elastic compression (elastic sleeve or intermittent pneumatic compression). Venous re- construction or first rib resection was done in selected
Volume 4 Number 4 October 1986 Axi l lary-subclavian venous occlusion 3 3 5
Table II. Results of 23 patients with acute axillary subclavian venous thrombus
Result
Treatment Good Fair Poor
Physical measures, no 1 ~ 1 - - anticoagulation
Anticoagulation <3 mo 2 2 >/3 mo 13 3 1
Venous thrombectomy 1 ~* - - - - Total 16 6 1
*Same patient.
cases. The chi-square test was used for statistical anal- ysis of the data.
RESULTS
Patients were followed up from the initial acute thrombosis up to an average of 5.4 years (range, 2 weeks to 15 years). Eleven patients died during this period, but on review of the records and autopsy reports supplemented by interviews with the patient's physician and family members, none of the deaths could be attributed to axillary-subdavian venous thrombosis. Pulmonary embolism occurred in six pa- tients and in four of these, the source was clearly the upper extremity (4.2%). Results were classified as good if the patient was asymptomatic or had minimal symptoms after strenuous exercise, fair if there was residual edema and symptoms were present with moderate exercise, and poor if there was discomfort at rest or venous gangrene. Of the 95 patients, 57 (60%) were classified as having a good result; 26 (27%) patients had a fair result and 12 (12.6%) pa- tients had a poor result.
Table II summarizes the results in the 23 patients seen within 5 days after onset of acute axillary-sub- clavian venous thrombosis. Thirteen of 17 patients who received anticoagulant therapy for at least 3 months had a good result. Only four patients in this group demonstrated residual symptoms. One patient who had sustained traumatic axillary-venous throm- bosis underwent successful venous thrombectomy; and although he had residual brachial plexus injury, the circulation of the extremity was essentially normal.
The results noted in the 72 patients with chronic occlusion are presented in Table III. The patients are separated into groups according to the management they received during the acute phase of the disease. Only 35% of those patients who never had antico- agulation had a good result compared with 72% of
Table III. Results of 72 patients with chronic axillary-subclavian venous occlusion
NOTE: Numbers in parentheses indicate number o f patients un- dergoing operation to treat thoracic outlet syndrome. ~Treatment o f acute episode. tStatistical significance in comparison of no anticoagulation ther- apy to anticoagulation of more than or equal to 3 months' du- ration, p < 0.01. :~Amputation for arterial thrombosis.
Table IV. Surgical results of 12 patients (15 extremities) with chronic axillary-subclavian venous occlusion
~Of these patients, one had intermittent venous occlusion, and two others had thoracic outlet syndrome with contralateral venous occlusion t Of t he se patients, one had intermittent venous occlusion and one had thoracic outlet syndrome with contralateral venous occlusion. ~Of these two patients, one had concomitant thrombectomy. §This patient had previously had first rib resection, scalenectomy, and thrombectomy.
those who had received anticoagulation for at least 3 months. This difference was statistically significant (p < 0.01) and was sustained (p < 0.001) if we cal- culated the effectiveness of at least 3 months' anti- coagulation in both the acute and chronic occlusion groups (95 patients). Although we do wish to in- terpret these data cautiously in view of the retro- spective nature of our study, it seems that antico- agulation is effective in decreasing the incidence of late complications of the disease. Two of the patients in this series in whom fibrinolytic treatment with streptokinase was attempted before their referral to Mayo Clinic were treated for residual symptoms.
Twelve patients in the chronic occlusion group underwent operation at some time during the course of their presentation. Six of these patients were op- erated on at the Mayo Clinic and six had previous surgery elsewhere. The results on 15 extremities in
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these 12 patients are shown in Table IV. One patient had documented bilateral venous occlusion and two patients had symptoms of intermittent venous ob- struction without occlusion. Three patients under- went prophylactic first rib resection for symptomatic thoracic outlet syndrome in the face of contralateral venous thrombosis. Nine patients (12 extremities) were believed to have benefited from the procedure, and all five patients who had first rib resection for intermittent venous compression or symptomatic thoracic outlet syndrome did well. None of these patients had venous thrombosis during the follow- up period, although two had residual symptoms. One patient who underwent venous reconstruction was thought to be improved. A saphenous vein graft had occluded in an additional patient who continued to have both pain and significant edema. One additional patient sent to us for treatment of symptoms related to chronic venous occlusion had had unsuccessful thrombectomy and first rib resection in another in- stitution.
DISCUSSION
In 1875 Paget, and later in 1884 von Schroetter, described thrombosis of the axillary vein and Hughes, 6 in a major review of reported cases, intro- duced the eponym Paget-Schroetter syndrome. Thrombosis of the axillary or subclavian vein in otherwise healthy people may be related to injury or effort but may also be spontaneous. The presence of thoracic outlet syndrome favors the development of effort-related venous thrombosis but clearly not all patients who have Paget-Schroetter syndrome have an associated thoracic outlet syndrome. The impor- tance of thoracic outlet compression in producing axillary-subclavian venous occlusion is emphasized by data of Dunant, 7 suggesting that up to 80% of the patients who manifest venous thrombosis had pre- vious symptoms of intermittent venous compression. The overall incidence of venous thrombosis in the upper extremities with thoracic outlet syndrome is still only about 3.5%.
The widespread use of subclavian catheters has increased the incidence and changed the spectrum of these problems. According to Brismar, Hardstedt, and Jacobson, 2 the length of time the catheter is in place rather than the type of catheter itself influences the risk for thrombosis. The risk from pacemaker electrodes appears to be quite low, with manifest upper extremity venous thrombosis developing in only 0.18% of our patients who underwent pace- maker implantation.
The diagnosis can usually be made from history
and physical examination alone. When thrombosis involving the axillary-subclavian vein is acute, the entire arm usually shows edema, including both the forearm and hand. The superficial arm veins are dis- tended and a prominent collateral venous pattern over the anterior chest wall on the affected side is common. Pain and discomfort are consistent findings and tenderness may be elicited over the axillary vein. Noninvasive testing is helpful, although problems in making the correct diagnosis of upper extremity ve- nous thrombosis without the use of contrast phle- bography have been emphasized. 8'9
Pulmonary embolization associated with acute axiUary-subclavian venous thrombosis has been reported s'l° but is fortunately rare. Among our pa- tients, pulmonary embolism clearly related to the up- per extremity thrombosis was nonlethal and noted in only four patients (4.2%). The risk of limb loss is low and the only patient in our series who needed amputation had diabetes mellitus, severe atheroscle- rotic occlusive disease, and acute arterial thrombosis of the upper extremity.
In the acute stage of axillary-subclavian venous occlusion, the problem is usually managed by ele- vating the extremity and using rest, local heat, and systemic anticoagulation. The effectiveness of anti- coagulation in decreasing early symptoms n and late morbidity 12 has been reported, although disappoint- ing results have been published as well. is We believe it is appropriate to maintain anticoagulation with oral warfarin for a 3-month period. During the acute phases of the disease this therapy should prevent fur- ther local extension of thrombosis and help to pre- vent embolization; our data suggest that it decreases both early and late morbidity. Experience with fi- brinolytic therapy here in our own practice or in that noted in the literature has not been sufficient to jus- tify firm conclusions regarding its role in acute ax- illary-subclavian venous thrombosis. 4.s Certainly if the patient is seen early in the course of the disease, fibrinolytic therapy might contribute to decreasing the long-term complications associated with the problem, but more data are needed in this regard.
Surgical treatment of upper extremity venous thrombosis is controversial. The only indication that seems to be accepted by both internists and surgeons is the appearance of intermittent venous compression symptoms caused by thoracic outlet syndrome, s First rib resection in these cases seems to be an effective operation that may alleviate the patient's symptoms and prevent the development of later venous throm- bosis. The role of thoracic outlet decompression in the treatment of those patients who already have sus-
Volume 4 Number 4 October 1986 Axillary-subclavian venous occlusion 337
tained venous thrombosis in the face of a narrow thoracic outlet is less well established. However, we agree with DeWeese s that intermittent obstruction of recanalized subclavian vein or of large first rib collateral vessels caused by narrow thoracic outlet may effectively be decompressed by first rib resection in selected cases. Certainly a decision to perform tho- racic outlet decompression in chronic venous occlu- sion could be made on the basis of other neurovas- cular compression symptoms.
An attempt at thrombectomy seems reasonable in the rare form of phlegrnasia cerulea dolens in- volving the upper extremities, but it must be recalled that almost all reported successful thrombectomies have been performed within 5 days of onset of throm- bosis. In those patients with thrombosis clearly caused by thoracic outlet syndrome, first rib resection would seem appropriate concomitant with throm- bectomy 16 or performed after successful fibrinolytic therapy 7 to prevent recurrent occlusion. Results with venous reconstruction with saphenous vein grafts need further evaluation and are inconclusive at this point.
Axillary-subclavian venous thrombosis is usually a nonlethal disease with a small risk of pulmonary embolization. However, late morbidity is significant, with up to 40% of patients showing residual symp- toms. Systemic anticoagulation in the acute phase of the disease is indicated to prevent extension of throm- bosis and embolism and appears to be beneficial both in the immediate period and in terms of late mor- bidity when continued up to 3 months. Long-term symptoms in patients with associated thoracic outlet syndrome may be alleviated by first rib resection in selected cases. Because of the late complications as- sociated with this condition, a more aggressive man- agement approach in the acute phase seems rational, but data relating to results obtained with fibrinolytic therapy, venous thrombectomy, and/or venous re- construction are still insufficient to advise them as routine treatment.
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