Deep thrombophlebitis and pulmonary embolism in thromboangiitis obliterans

Three cases of thromboangiitis obliterans have been presented in which at the reported stages of the disease the manifestations, in contradistinction to the common clinical course of this disease, were primarily those of a deep venous thromboembolic nature rather than occlusive arterial disease. This clinical variant, according to the literature on thromboangiitis, is rare; however, the knowledge of its possible occurrence is important to an early and correct diagnosis of the pulmonary manifestations. Other factors contributing to the confusion and delay in diagnosis of these cases were the relatively late age of the patients at the time of onset of the disease, the absence of clinical evidence of thrombophlebitis at the time of pulmonary embolization and the failure of the arterial occlusive disease to manifest activity simultaneously with the activity of thromboembolic disease.It is possible that the age of these individuals at the time of the greatest activity of the thromboangiitis was a factor contributing to the predominantly venous thromboembolic manifestations. Certainly in general, venous thromboembolic disease is more common in the older age groups. Two of these patients were over fifty years of age at the time of onset of the thromboangiitis and all three were considerably beyond the age group in which the disease is commonly most active clinically.The ages of Case I, fifty-two years, and Case III, fifty-seven years, together with the absence of any active thrombophlebitis or activity of the arterial disease, seemed to necessitate an investigation of the respiratory tract as the possible primary focus of their illness in spite of the knowledge that peripheral vascular disease existed.Pulmonary embolization and infarction occurred repeatedly in all three patients while they were apparently well and ambulatory and at a time when no clinical sign of active deep or even of superficial thrombophlebitis was evident. Iliofemoral thrombophlebitis had occurred in Case I a year prior to the episodes of pulmonary embolization and recurred somewhat over a month after their cessation while in Case II iliofemoral phlebitis was evident three and one-half years before and again two and one-half months before the embolic accidents. Case III never exhibited an iliofemoral thrombophlebitis but a deep thrombosis of the foot and calf veins developed five months after the last pulmonary embolic episode. These observations would tend to confirm the statement of Allen, Barker and Hines² that deep thrombophlebitis of the anterior and posterior tibial veins is common in thromboangiitis but that only rarely is it clinically evident. It appears most likely that the sources of the emboli in all three cases were the calf veins rather than the more usual iliofemoral veins as suggested by Edwards.⁴As stated by Allen, Barker and Hines² clinical evidences of arterial and venous involvement are not necessarily coincident in Buerger's disease. In Cases I and III the fact that they were dissociated increased the difficulty of the differential diagnosis considerably, whereas in Case II in whom arterial occlusion had been recently active, diagnosis of the pulmonary embolic episodes was greatly facilitated by this knowledge.The treatment of the thrombophlebitis and its attendant pulmonary embolization in thromboangiitis obliterans has not been clearly delineated in the literature. Irving Wright⁵ states that there is at present no generally satisfactory treatment for recurrent, migratory thrombophlebitis and that probably the best long term therapeutic procedure is disturbance of the clotting mechanism through the effect of dicumarol, apparently in the hope that protection against embolization will be provided and that the thrombophlebitic process will subside during this period. As regards the procedure of ligation of the larger venous trunks, aside from the general objections to this procedure which have become recently evident,⁶ there is reason to consider the venous congestion produced thereby specifically undesirable in Buerger's disease, in which the limb is already afflicted with an impairment of the circulatory function. Allen, Barker and Hines² have in their experience found that typhoid vaccine fever therapy has an almost specific effect on the superficial thrombophlebitis. If this is true it would be reasonable to expect that it might likewise exert some beneficial effect on deep thrombophlebitis.Case I was particularly instructive as regards the efficacy of the present therapy of the deep thrombophlebitis of Buerger's disease. While the patient was receiving treatment with typhoid vaccine and while the prothrombin time was satisfactorily and continuously elevated through dicumarol, the migratory superficial phlebitis continued to involve new veins and a deep thrombophlebitis occurred. This would lend some support to the view that the pathologic process in thromboangiitis is primarily endothelial and proliferative rather than primarily thrombotic, and that possibly for this reason the phlebitis continued active in spite of the dicumarol effect which, however, was apparently sufficient to prevent the formation of a type of thrombotic material necessary for the liberation of emboli. After the quiescence of the phlebitis the patient underwent a transabdominal bilateral lumbar sympathectomy and a simultaneous vena caval ligation. Subsequent to this procedure a severe thrombophlebitis developed which almost surely involved the anastomotic and collateral veins to such a degree as to produce severe impairment of the venous return and resulted in persistent massive peripheral edema. A similar treatment and outcome had occurred in Case I reported by Kahn.³ The possibility of immediate or long subsequent phlebitis obstructing the remaining venous channels and producing undesirable swelling would appear to be another contraindication to femoral or vena caval ligation. Surprisingly enough, the patient had an excellent result from the operative procedure as regards the arterial circulation of the extremities despite the venous obstruction.Case II has been under continuous dicumarol therapy for four months while ambulant and has had no further thrombophlebitis or pulmonary emboli.Case III was treated with anticoagulants and typhoid vaccine for a two-week period only while active thrombophlebitis was evident and has had no evidence of recurrent thromboembolic disease for five months. Whether one can attribute these results to therapy or to the natural course of the disease is problematic.At present the most logical and satisfactory course of therapy for such major thromboembolic manifestations as occur in Buerger's disease would appear to be a combination of anticoagulant and intravenous typhoid therapy during the active stage of the phlebitis, with continuation of the anticoagulants indefinitely thereafter with the patient ambulant in the hope that the phlebitic process will subside. Other measures, particularly cessation of smoking, which are commonly employed in the treatment of thromboangiitis are, of course, indicated.