Thoracic outlet syndrome in children and young adults.

OBJECTIVES: Thoracic outlet syndrome has been well described in the population between 25 and 40 years of age, and is less frequently reported in those in the first two decades of life. The objective of this study was to review results with onset of TOS in the first two decades of life to determine type of presentation and outcomes from surgical intervention. METHODS AND MATERIALS: Charts of all patients in the first two decades of life, operated on for TOS between 1994 and 2006 were reviewed with follow-up by clinic visit and phone survey to assess the patients' current level of activity and relief from symptoms. RESULTS: Twelve patients were identified (13 operations), with a mean age of 16.8 years. Acute ischemic symptoms were the initial presentation for 38%, venous TOS in 24%, and neurogenic symptoms in 38%. All patients had symptom relief with surgery with a mean time to resolution of 10.9 weeks. All patients remained symptom free or improved at follow-up. CONCLUSIONS: Vascular TOS is much more common in TOS presenting in the first two decades of life. Surgical intervention for TOS in this population results in long-lasting symptom relief and should be considered for all subtypes of patients.

Inactivation of thrombin by the aortic endothelium.

The aim of the investigation was to clarify the uptake of thrombin on vascular endothelium and the inhibition of thrombin on the endothelium in the presence or absence of plasma. Segments of porcine aorta were used. Thrombin was labelled with 125I and its enzymatic activity was assayed amidolytically using a specific chromogenic substrate. After exposure to the endothelium both enzymatic activity and radioactivity disappeared from the thrombin solution and were recovered as surface bound activities. The enzyme activity confined to the endothelium rapidly disappeared in the presence of plasma but no activity was recovered in the plasma. The surface confined radioactivity, however, decreased slowly and was quantitatively recovered in the plasma. In the absence of plasma, i.e. in the presence of a balanced Ringer's solution, only slow disappearance of thrombin enzymatic activity occurred although the rate of disappearance was higher than that of release of radioactivity. It is concluded that thrombin, taken up on the endothelium, is almost instantaneously inhibited by an interaction mechanism with plasma and then released in an inactive state. The endothelium itself, however, seems to slowly inhibit bound thrombin and then release it.

Thrombin activity appearing on the vessel wall after trauma.

Thrombin activity was assayed on the aortic surface of rabbits after soft tissue trauma or endothelial injury caused by a balloon catheter. The animals were sacrificed by exsanguination 20 minutes or 3 hours after either type of trauma. Thrombin amidolytic activity on the luminal surface of the aorta was measured by exposing it to a synthetic chromogenic substrate. Thrombin activity appeared on the aortic endothelium 3 hours but not 20 minutes after soft tissue trauma. Heparin prevented the appearance of thrombin activity completely only if given just before the trauma. After endothelial injury, thrombin activity appeared on the vascular surface after 3 hours but not after 20 minutes. Thrombin activity appearing on the endothelium after soft tissue trauma may explain posttraumatic thrombotic events. The thrombin activity could, however, also be directed towards activation of protein C in which case an anticoagulant effect is obtained. Thrombin appearing after endothelial injury may enhance reactivity on the damaged vessel wall.

Interaction of thrombin and factor Xa with bovine vascular endothelial cells, smooth muscle cells and rat hepatoma cells.

The aims of the present investigation were to characterize the binding and inhibition of thrombin and factor Xa to bovine vascular endothelial cells (EC), bovine smooth muscle cells (SMC), and rat hepatoma cells (RHC), and to evaluate the effects of plasma constituents on their inhibition. The enzymatic activities of bovine thrombin and factor Xa were assayed using chromogenic substrates. After 10 min incubation with the cells, thrombin activity in the solution had decreased by about 20% and was subsequently recovered on the cell surfaces. When the cells with the surface-bound thrombin were incubated with defibrinogenated plasma or antithrombin III (AT-III) for 30 sec only about 10% and 20-40%, respectively, of the initial activity could be recovered. In similar experiments with factor Xa, initial activity in the solution had decreased by 10% after 10 min incubation, and was subsequently recovered from the cell surfaces. After 30 sec incubation with AT-III, no cell surface-bound factor Xa activity was detected, whereas 10% of the bound factor Xa activity was recovered after incubation with defibrinogenated plasma. It is concluded that thrombin and factor Xa are taken up and inhibited by EC, SMC and RHC cell surfaces in similar ratios, suggesting that cell surface-mediated inhibition of clotting factors is not restricted to vascular wall cells. The inactivation of factor Xa was dependent on AT-III, however, the inactivation of thrombin was further promoted by an additional unidentified plasma constituent.

Influence of nicotine and cotinine on the expression of plasminogen activator activity in bovine aortic endothelial cells.

The effects of nicotine and its major metabolite, cotinine, were evaluated on the secretion of plasminogen activator (PA) and plasminogen activator inhibitor (PAI) in cultured bovine aortic endothelial cells. Both compounds increased PA secretion, determined by 125I fibrin plate assay, in a time- and dose-dependent manner. Maximum effects after 24 hr incubation were observed for nicotine at 10(-8) M and for cotinine at 10(-7) M, which corresponded to about 2.6-fold increases over control for both compounds. The pharmacological PA stimulation required both RNA and protein syntheses, as evidenced by inhibition by actinomycin D and cycloheximide. Both control and treated cells produced multiple forms of PA, as evaluated by SDS-PAGE zymography, and a single form of PAI, as evidenced by reverse fibrin autography. Although activities of all species of PA were enhanced by nicotine and cotinine, these compounds had no significant effects on the release of PAI. These results thus suggest that nicotine and cotinine may have fibrinolytic activity in vivo.

Uptake and inactivation of thrombin on rabbit aortic endothelium studied with two different substrates.

The endothelium is an important compartment for uptake and inhibition of thrombin. The amount of enzymatically active bound thrombin can be detected with both small synthetic substrates and with aid of fibrinogen as substrate. The present study was designed to investigate the relation between endothelially bound thrombin with amidolytic activity towards a synthetic substrate (S-2238) and thrombin capable of converting fibrinogen by measuring generation of fibrinopeptide A (FPA). The luminal surfaces of rabbit aortae (2 cm2) were exposed in vitro to thrombin (0.625-5.0 NIH units/ml). Thrombin disappeared from the solution and a certain fraction was recovered on the surface. There was a linear relationship between the amount of thrombin on the surface and the concentration of thrombin in the incubation mixture. Approximately one third of the thrombin measured with S-2238 was also able to cleave fibrinogen. After incubation with defibrinogenated plasma almost total inhibition of fibrinogen splitting activity occurred within 30 sec. The inhibition of the amidolytic activity was less complete. When endothelially bound thrombin was exposed to plasma much less FPA was generated than in a fibrinogen solution. A minor fraction of endothelially bound thrombin was inhibited also upon incubation with Tyrode without recovery of any enzymatic activity in the solution. The results indicate that a fraction of thrombin bound to the endothelium has retained enzymatic activity and that a fraction of the enzymatically active thrombin is capable of converting fibrinogen. Inhibition of thrombin enzymatic activity occurs rapidly upon exposure to plasma. The endothelium itself has a minor inhibitory effect also in the absence of plasma.(ABSTRACT TRUNCATED AT 250 WORDS)

The vascular endothelium as an inhibitor of thrombin.

Uptake and inhibition of thrombin on the endothelium of porcine aorta was studied in vitro. The thrombin was labelled with 125J and its enzyme activity was measured with an amidolytic assay. After exposure to the aorta both the enzyme activity and radioactivity disappeared from the solution and were recovered as surface bound activities. The rate of inhibition of the surface bound thrombin was determined in presence or absence of plasma. In presence of plasma the endothelially confined enzymatic activity was rapidly inhibited, no enzymatic activity was recovered in plasma. The surface bound radioactivity, however, decreased slowly and was recovered in plasma. In absence of plasma only slow inhibition took place. It is concluded that thrombin taken up on the endothelium is rapidly inhibited there by an interaction with plasma and then released in an inactivated state. An alternative conclusion, based on the anticoagulant and antithrombotic actions of the endothelial fibrin lining is briefly discussed.

Uptake and inhibition of thrombin by the vascular wall.

Thrombin activity and inhibition were assayed on the aortic surface of dogs and pigs. After sacrifice, the aortae were excised and thrombin activity was measured with an amidolytic assay. A small thrombin activity was found on the endothelium. Heparinization of the animals lowered endothelial thrombin activity. After exposure of the aortic endothelium to a thrombin/albumin solution in vitro, thrombin activity disappeared from the solution and was recovered on the surface. De-endothelialized vessels took up more thrombin than those with intact endothelium. Endothelium confined thrombin was rapidly inactivated when exposed to plasma. A slow inactivation was seen upon exposure to a modified Ringer's solution. Thrombin confined to de-endothelialized aortae, i.e. to medial structures, was always inactivated at a slow rate irrespective as to whether it was exposed to plasma or Ringer's solution. It is concluded that endothelium and subendothelium bind thrombin and subsequently inactivate it. The inactivation proceeds faster on endothelium when exposed to plasma. The possible role of glycosaminoglycans is discussed.

Effect of glycosaminoglycans and antithrombin III on uptake and inhibition of thrombin by the vascular wall.

Thrombin binds to and is inactivated by the endothelium. The inactivation is potentiated by plasma. The present investigation was designed to clarify the role of vessel wall glycosaminoglycans (GAG) and plasma antithrombin III (AT III) in the inactivation and binding of thrombin by endothelium. Thrombin was shown to bind to vascular endothelium and artificial surfaces containing GAG:s. The binding could be inhibited on both types of surfaces by pretreating them with protamine. Thrombin bound to endothelium was rapidly inactivated in the presence of plasma but only slowly if the plasma was replaced by AT III, AT III-depleted plasma or a balanced salt solution. It is concluded that thrombin binds to vessel wall GAG:s and is inactivated by the endothelium. Potentiation of the inhibition of the endothelially bound thrombin by plasma is dependent upon presence of AT III but an additional plasma factor is also required.

Isolated iliac artery aneurysms in patients with or without previous abdominal aortic aneurysm repair.

BACKGROUND: Isolated iliac artery aneurysms (IAA) in patients with or without previous abdominal aortic aneurysm (AAA) repair are rare. We wanted to compare the presentation, distribution, treatment, outcome and patterns of subsequent aneurysm formation in these patients. METHODS: We retrospectively reviewed patients with isolated IAA over a 10-year period. Patients with primary isolated IAA (group 1) were compared with patients who presented with IAA after previous AAA repair (group 2). RESULTS: There were 23 patients in each group. Demographics and comorbidities were similar. No aneurysms were detected outside of the iliac system in group 1; 22% of patients in group 2 had other aneurysms. The mean time after AAA repair to IAA diagnosis was 8.8 +/- 3.2 years for operated on patients. The in-hospital mortality was 0% for elective cases and 50% for emergency cases for both groups. Three patients in group 2 (13%) developed new aneurysms during follow-up, whereas the only new aneurysm in group 1 was a contralateral IAA. CONCLUSIONS: Patients with new IAA after AAA repair have a greater tendency to develop further aneurysms in other sites, synchronously or metachronously. The time to detection of new IAA after AAA repair is at least 5 years in most cases. In both groups, a quarter to a third of patients present with rupture, with a resultant mortality of 30% to 50%, whereas those operated on electively have minimal morbidity and almost no mortality.

Acute ischemia of the extremities in a metropolitan area during one year.

All acute extremity ischemias during one year (1980) in the greater Stockholm area (population 1.5 million) were evaluated. One hundred and thirty eight cases were found. The incidence figures varied from 0.4/100,000 in the age group 20 to 30 years to 182/100,000 in the ages above 90 years. 81% of the cases were classified as emboli and 19% as acute thrombosis. Over all mortality was 19.5%. In the embolism group mortality was 21%, amputation rate 28% and limb salvage rate 51%. Limb salvage rate was significantly higher in patients receiving heparin therapy (61%) as compared to those who received no heparin (32%). Delay of treatment also caused an increase in amputation rate. Patients with acute thrombosis had a lower mortality (12%) and an amputation rate of 42%. It is concluded that the results of acute extremity ischemia may be less favourable than previously reported when results from institutions not specialised in vascular surgery are also included. Among other possible factors explaining the high amputation rate may be the proportion of older patients.

The small abdominal aortic aneurysm: the eternal dilemma.

In order to evaluate morbidity and mortality after elective resection of abdominal aortic aneurysms (AAA) as it relates to aneurysm size, a retrospective review of 111 elective aneurysmectomies over a 5 year period was undertaken in a VA population. Thirty seven AAA's measured < 5 cm in diameter and 74 were > or = 5 cm by CT scan. Patients with small AAA (S-AAA) were significantly younger (mean 64 years) than those with large AAA (L-AAA) (mean 69 years) (p < 0.003). Both groups were similar with respect to prevalence of cardiovascular, pulmonary and renal disease. Aortic cross-clamping time was significantly shorter in L-AAA, possibly because those with S-AAA had a higher prevalence of associated occlusive disease requiring more femoral anastomoses (p < 0.04). Postoperatively six patients (8%) had a myocardial infarction (MI) in the L-AAA group and four (5%) of these died. In contrast no patient with S-AAA suffered a postoperative MI. The rates of non-cardiac complications and length of hospital stay were not significantly different between the two groups. However, the patients with L-AAA stayed longer in ICU (p < 0.05) and the overall combined morbidity rate was significantly higher in this group (p < 0.02). Our results suggest that resection of S-AAA upon diagnosis in acceptable risk patients appears to be the safest overall therapeutic plan.

Acute thrombosis and embolism of the extremities: factors influencing the result of treatment.

Eighty-five cases treated for acute nontraumatic ischemia of the extremities have been reviewed. Eighty per cent were diagnosed as emboli and the remaining 20% were considered to have acute thrombosis. The most common embolism source was the heart and the most common heart disease causing embolism was atrial fibrillation. Seventy patients were treated surgically (80 surgical procedures) and 14 conservatively. All surgical patients received pre-, per- and postoperative anticoagulants. Limb-salvage rate was 76% in patients with emboli and 60% in the thrombosis group. The amputation rate was significantly higher in the thrombosis group. The overall mortality was 12% and was slightly but not significantly higher in the embolism group. It is concluded that acute arterial emboli should be treated rapidly by embolectomy. Acute thromboses carry a higher amputation rate if treated by thrombectomy alone and thus reconstruction should be considered. The relatively low mortality rate in the present material could be due to the routine use of anticoagulants.

Septic false aneurysms. Report of three cases.

Three cases of septic false aneurysms are reported. They appeared as palpable pulsating masses shortly after severe septic episodes and were diagnosed by angiography. Two aneurysms were located in the common iliac artery and one in the superficial femoral artery. Operation was performed under antibiotic coverage and included ligation of the artery and excision of the aneurysm. Immediate reconstruction was performed in one case, in the two remaining, such reconstruction was abstained from since intraoperative ankle pressures were judged adequate. In one case reconstruction was performed later to relieve claudication. All patients survived with functioning extremities.

Acute nontraumatic extremity ischaemia in Sweden. A one-year survey.

An attempt was made to evaluate the results of treatment for acute nontraumatic extremity ischaemia in Sweden during one year. A questionnaire was sent to all surgical units, and 61% replied. Of the total 586 evaluated cases, 497 were classified as embolism and 89 as acute thrombosis. Patient age strongly influenced results in both groups as regards limb salvage and mortality rates. The site of embolic occlusion also influenced mortality, with greatly heightened rate in aortic occlusion. Delay of operation for more than 12 hours after onset of symptoms was associated with increase in amputation rate and mortality. Adequate heparin therapy significantly improved results after embolectomy, but had no such effect after surgical treatment of thrombosis. The amputation rate was higher after acute thrombosis than after embolism. The authors conclude that patient age should be considered in comparisons between different case series of acute ischaemia, that embolus site and time of surgery are important determinants of mortality and amputation rate, and that heparin significantly improves results of embolectomy.

The vascular endothelium as an inhibitor of thrombin.

Uptake and inhibition of thrombin on the endothelium of porcine aorta was studied in vitro. The thrombin was labelled with 125J and its enzyme activity was measured with an amidolytic assay. After exposure to the aorta both the enzyme activity and radioactivity disappeared from the solution and were recovered as surface bound activities. The rate of inhibition of the surface bound thrombin was determined in presence or absence of plasma. In presence of plasma the endothelially confined enzymatic activity was rapidly inhibited, no enzymatic activity was recovered in plasma. The surface bound radioactivity, however, decreased slowly and was recovered in plasma. In absence of plasma only slow inhibition took place. It is concluded that thrombin taken up on the endothelium is rapidly inhibited there by an interaction with plasma and then released in an inactivated state. An alternative conclusion, based on the anticoagulant and antithrombotic actions of the endothelial fibrin lining is briefly discussed.