Phlegmasia cerulea dolens

Phlegmasia cerulea dolens

Phlegmasia Cerulea Dolens* NEOPITO L. ROHLES, M.D.,BENEDICT R. WALSKE, M.D.,ANDW. Tucson, Arizona From the Veterans Administration A rizona Hospital...

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Phlegmasia Cerulea Dolens* NEOPITO L. ROHLES, M.D.,BENEDICT R. WALSKE, M.D.,ANDW. Tucson, Arizona

From the Veterans Administration A rizona



* Presented at the Eighteenth

Vol. 112,Nowember



readily explained by the pathophysiology of the thrombotic process within the venous system of the involved extremity. In the past several years the treatment for this disease began to crystallize. Some forms of treatment such as exercise [Z] and massage of the extremity [3] are being abandoned since they are associated with a high mortality from pulmonary thromboembolism [4]. Conservative treatment with intravenous heparin, sympathetic block, and leg elevation should be reserved for preoperative stabilization while replacement fluid and plasma therapy are being instituted. Fortunately, emergency thrombectomy is receiving wide acceptance as the treatment of choice for these patients, with very gratifying results. Salvage of numerous limbs from either frank gangrene or the sequelae of chronic venous insufficiency has been achieved by early surgical treatment. Phlegmasia cerulea dolens disease is frequently associated with malignant diseases, trauma, infection, and postoperative and postpartum states. This report will be of four patients seen by us in a two year period. It was prompted by the excellent result obtained by emergency thrombectomy on three of the patients when close communication was made between the surgeon and internist early in the course of the disease. One patient who subsequently required amputation was seen late, when gangrene was already present. This patient was treated by sympathetic blocks, anticoagulants, and elevation of the extremity; results were disastrous to the involved extremity, and the patient eventually had an amputation to the mid-thigh. (Table I.)


cerulea dolens is one of the infrequent but most severe forms of venous thrombotic disease. This form of phlebitis is known by a number of names, most of which are descriptive of its clinical findings; such terms as “blue phlebitis,” pseudoembolic phlebitis, ” “acute massive venous occlusion,” and “gangrene of venous origin” have been encountered in the literature. The pathogenesis of this disease has been the subject of much dispute. The basic pathologic condition is total or near total occlusion of the venous bed of the involved extremity. The circulatory changes and in most cases impairment of arterial flow of the involved extremity are mechanical and develop subsequent to massive intravenous thrombotic obstruction. Vasospasm, if it occurs and which many believe is the primary cause of the disease, plays a secondary role. The increase in capillary pressure and filtration distal to the proximal thrombus produces massive edema of the involved extremity with subsequent loss of extracellular fluid, mainly plasma, resulting in hypovolemic shock. This subsequently causes a fall in arterial pressure and rapidly increases tissue pressure within the involved extremity. The difference in this pressure across the wall of the arterial tree falls below a critical value and causes a complete closure of the vessels and cessation of arterial flow results. Burton [1] has defined this phenomenon as “critical closing pressure.” The clinical features of the disease are severe excruciating pain, massive edema, bluish purple discoloration, shock, and eventual gangrene if proper treatment is not instituted. These are




Annual Meeting of the Southwestern April 18-21, 1966. 693

A sixty-nine

year old white male patient

Surgical Congress, Las Vegas, Nevada,

Robles, Walske, and Wilcox









67 54





Duration of symptoms (hr.) 12 8 12 30










Associated Conditions

Thrombectomy Thrombectomy Thrombectomy Sympathetic blocks; anticoagulants

Good Excellent Excellent Poor

Arteriosclerotic heart disease Carcinoma of colon Multiple fractures Pneumonia

was admitted to the Veterans Administration Hospital with sudden onset of severe pain of the left leg of twelve hours’ duration and progressive swelling and bluish discoloration of the leg for eight hours. He had arteriosclerotic cardiac disease with fibrillation and was receiving 0.1 mg. digitoxin daily and 50 mg. hydrochlorothiazide daily. Physical examination revealed cardiomegaly by percussion and extrasystoles on auscultation. There was dark, reddish blue discoloration of the entire left leg below the inguinal ligament, with a marked swelling of the leg. The skin was cold and no femoral or popliteal pulses were palpable. Intravenous heparin was given and an emergency thrombectomy performed. Thrombi were extracted both proximally and distally until a good flow of blood was obtained from both ends of the venotomy. Several hours after the procedure, the swelling and discoloration began to subside. The leg felt warmer and the femoral pulse became palpable. Twenty-four hours later heparin therapy was reinstituted and after ten days [email protected] was substituted as the anticoagulant. The patient had an uneventful postoperative period. There was minimal residual swelling and discomfort on ambulation and this was controlled readily with elastic stockings. CASE II. A sixty-seven year old white man was admitted to the Veterans Administration Hospital on May 29, 1964 because of marked swelling of the left leg of about eight hours’ duration. Several hours prior to admission the patient noticed a bluish discoloration of the left leg and he complained of severe pain. Physical examination showed marked edema and bluish discoloration of the entire left leg. The femoral and popliteal arterial pulsation was weaker on the left than the right, and the pedal pulse was barely palpable. His blood pressure was 150/70 mm. Hg, pulse rate 92 per minute, white blood cell count 7,500 per cu. mm., hemoglobin 14 gm. per cent, and hematocrit 44 per cent. Emergency exploration of the femoral vein was performed with spinal anesthesia. Numerous fresh and organized thrombi were extracted proximally and distally until good flow was obtained from both sides of the vein. The venotomy was closed and the patient tolerated the procedure

well. Intravenous heparin was started five hours postoperatively. The swelling lessened almost immediately after the thrombectomy, and pulses over the artery were noticed to be stronger three hours later. Three days later the patient complained of slight pain in the posterior part of the chest. An electrocardiogram suggested pulmonary embolism. Roentgenograms of the chest revealed no definite abnormality. Four days after operation the left leg was the same size as the right. A report from another hospital was obtained showing that the patient had had resection of the colon for adenocarcinoma one year previously. Gradual ambulation was tolerated without difficulty, and the patient was discharged nineteen days after operation on regular doses of heparin. He was readmitted four weeks later with recurrent chest pain. The electrocardiogram was again suggestive of pulmonary embolism. There was an infiltrate in the right lower part of the chest. A peripheral venous pneumoangiogram revealed a lobar block in the artery in the lower lobe of the right lung. Both main pulmonary arteries were enlarged. There was severe venospasm encountered at the time of the angiogram and node encroachment of the superior vena cava. The patient’s course gradually went downhill and he died on September 30, 1964. Postmortem examination showed metastatic carcinoma of the right diaphragm. There were multiple small pulmonary thromboemboli throughout both lung fields and a partial block of the artery in the right lower lobe. CASE III. A fifty-four year old white man was admitted to the Veterans Administration Hospital because of multiple fractures (including fracture of the right femur) sustained after an automobile accident. He was convalescing satisfactorily when hemoptysis and pain in the right leg developed. He was placed on heparin. On June 29, 1965 at 7 : 00 P.M., however, nurses noted discoloration of the left leg. By midnight the left thigh and leg were cyanotic and swollen, with weakness of femoral pulse. The next morning, surgical consultation was obtained, a diagnosis of phlegmasia cerulea dolens was made, and

American Journal of Surgery





thrombectomy under general anesthesia was performed. The condition in the leg started to improve within four hours, and swelling and cyanosis of the left leg disappeared. Heparin was started six hours postoperatively and was continued for six weeks. There were no noticeable sequelae in the leg operated upon. CASE IV. X sixty-nine year old white man was admitted to the hospital because of pain and swelling of the right leg for three days. Physical examination showed tenderness and slight swelling of the right leg as well as Homans’ sign. The pedal pulses were diminished but present. The patient was started on therapeutic doses of Coumadin. The following day spiking temperatures developed; the diagnosis was pneumonia and the patient was treated with antibiotics. Two da.ys after admission the leg and foot became more swollen with bluish red discoloration associated with extreme pain. A consultation with the Surgical Service was made thirty hours later. The gangrenous changes on the toes and foot were so far advanced (Fig. 1) that thrombectomy was not performed. After demarcation of the gangrene was more pronounced an amputation to the mid-thigh was carried out. Comment: This case represents the typical course of phlegmasia cerulea dolens when prolonged conservative therapy is inadequate and surgical consultation is delayed. COMMENTS

Massive edema, violaceous discoloration of the skin, excruciating pain, shock, and some degree of arterial insufficiency are the classic manifestations of phlegmasia cerulea dolens. In the presence of these signs and symptoms, early emergency thrombectomy [5,6] appears to be the treatment of choice. Conservative treatment in the form of intravenous heparin, sympathetic nerve block, and elevation of the involved extremity can be performed while fluid and plasma replacement are being instituted in preparation for surgery and in cases where the general condition of the patient does not permit an operation. Intravenous heparin administered for at least fourteen days postoperatively also appears indicated. With the better understanding of the altered hemodynamics within the vascular and tissue spaces, there is increasing evidence that an emergency thrombectomy is the treatment of choice for phlegmasia cerulea dolens. The normal hemodynamits at the capillary level as illustrated in Figure 2 shows that the normal hydrostatic pressure is approximately 8 cm. Hz0 greater than the Vol. 112, Nouembev


FIG. 1.

Gangrenedue to massive venous thrombosis.

colloid osmotic pressure and is responsible for the formation of extracellular fluids. The reverse is true at the venule side where the osmotic pressure is almost 8 cm. Hz0 greater than the hydrostatic pressure and the extracellular fluid is reabsorbed. (Fig. 2A.) With the massive thrombus formation within the venous system, the hydrostatic pressure on the venule side increases tremendously, thereby neutralizing the osmotic pressure, so that very little, if any, of the extracellular fluid (plasma) is reabsorbed. (Fig. 2B.) This explains the massive edema of the involved extremity. The characteristic violaceous discoloration is due to the engorgement of the smallest venous tributaries and the associated extravasation of red blood cells. The excruciating pain is mainly due to stretching of the sensory nerve endings as a result of the massive edema. Shock, which is a common finding with this condition, is secondary to trapping of fluid within the involved extremity and/or to pulmonary thromboembolism. lt has been estimated that as much as 55 per cent of the blood volume can be lost into the third space within a period of eight hours after onset of massive venous occlusion [7]. With this predominant loss of plasma volume in experimental animals, there is an associated 48 per cent rise in hematocrit above the original value. Some degree of arterial insufficiency is probably a constant feature of the condition ; however, in a certain number of patients it is more pronounced, which explains the early appearance of gangrene. The cause of impairment of the arterial flow has been investigated by numerous workers [8,9]. It is still not clear what role arterial spasm plays in the pathogenesis of this condition, but in all proba-





and Wilcox


FIG. 2. A, normal hemodynamics at capillary level. B, altered hemodynamics secondary to phlegmasia cerulea dolens. ECF, extracellular fluid; HP, hydrostatic pressure; OP, osmotic pressure.

bility it is a secondary one. The role of venospasm is still less well understood and has not yet been studied adequately. Vasko and Brockman [7], after creation of massive venous occlusion in experimental animals, found the tissue pressure increased secondary to massive edema. This is associated by a decrease in arterial pressure due to loss of effective circulating volume. The pressure difference across the wall of the arteries decreases and when it reaches a critical level, the arteries collapse with cessation of flow and gangrene follows. This was termed by Burton [I] in 1951 as “critical closing pressure.” Management of phlegmasia cerulea dolens has evolved from conservative medical management to thrombectomy, which has resulted in a significant improvement in immediate and later results. Elevation of the involved extremity to relieve venous engorgement is simple, but should be at least 8 inches to accomplish its purpose; however, for this to be effective there must be only incomplete venous obstruction by the thrombus so that venous return through collateral pathways can be increased. The use of heparin and other anticoagulants in venous thrombotic disease has a definite place, but caution should be exercised on total dependence on these drugs in treating these conditions. There have been many cases reported in the literature of phlegmasia cerulea dolens developing in patients on adequate levels of anticoagulants. Heparin is the anticoagulant of

choice and should be administered for at least two to three weeks before switching to the coumarin derivatives for prolonged anticoagulant therapy after recovery from phlegmasia cerulea dolens. Fasciotomy should probably be reserved as an accessory method of treatment if edema persists after thrombectomy. This procedure relieves the tissue pressure, thus decreasing the pressure difference across the wall of the artery and allowing a greater arterial flow. One report from South Africa has shown a good result with this form of treatment [IO]. Thrombectomy is probably the most definitive form of treatment for phlegmasia cerulea dolens since it removes the basic pathologic process, that of massive venous thrombosis. Our own experience in three of four patients with phlegmasia cerulea dolens on whom we performed thrombectomies gave very gratifying results. The earlier the thrombectomy is performed, the better the results. Judgment must always be tempered by the general condition of the patient when a major surgical procedure such as thrombectomy may have an undesirable result for the patient who is desperately ill. With these patients conservative measures should be the treatment of choice until he has improved sufficiently to undergo thrombectomy. Fogarty has developed a catheter for venous thrombectomy which appears to be an ingenious instrument. If free venous return, both proximally and distally, is obAmerican


of Surwy


Phlegmasia Cerulea IIolens tained after thrombectomy little reason in compromising

there seems to be

the unobstructed venous return achieved by the procedure by a proximal ligation of a major venous channel unless subsequent thromboemboli occur or unless the pulmonary vascular reserve is already greatly compromised at the time of the thrombectomy. SUMMARY

Severe excruciating pain, massive edema, bluish purple cyanosis, and diminished arterial flow to an extremity are the clinical manifestations of phlegmasia cerulea dolens. It is frequently associated with malignant disease, trauma, infection, and postoperative and postpartum states. The basic pathologic process is massive obstructive venous thrombosis and the treatment of choice is early thrombectomy. If the general condition of the patient is poor, conservative measures such as elevation of the involved extremity, intravenously administered heparin, and sympathetic nerve blocks are of help while preparing the patient for the operation. Our experience with four patients who had phlegmasia cerulea dolens is reported. Three of these patients received thrombectomy early with good results and amputation was necessitated in one patient seen late. One of the patients treated by thrombectomy died subsequently from recurrent pulmonary throm-

Vol. 112, November 1966

boemboli which in retrospect were discovered to be present soon after thrombectomy. REFERENCES 1. BURTON, 11. C. On the physical equilibrium










of small blood vessels. ;Im. /. Physiol., 164: 319. 1951. MOSER, M.. BARIN, S. M., COTTS. G. W.. and PRAXDONI,A. G. Acute massive venous occlusion; report of a case successfully treated with exercise. Ann. Int. _\I&., 40: 361, 1954. HERSHEY, C. D. and SNYDER, R. E. Thrombophlebitis with ischemia and gangrene. Surgery, 31: 296, 1953. MCDONALD, S. T., PERSON, N. W., and TAYLOR, L. M. Thrombophlebitis cerulea dolens; treatment by passive exercise with production of pulmonary embolus. Arch. Surg., 80: 350, 1960. FOGARTY, T. J., CRANLEY, J. J., KRAUSE, R. J., STRASSER, E. J., and HAFNER, C. D. Surgical management of phlegmasia cerulea dolens. Arch. Surg., 86: 256, 1963. BROCKMAN,S. K. and VASKO, J. S. Observations on the pathophysiology and treatment of phlegmasia cerulea dolens with special reference to thrombectomy. Am. J. Surg., 109: 485, 1965. VASKO, J. S. and BROCKI\IAN, S. K. Massive venous occlusion of the lower extremity; an experimental study. 5’. Forum, 13: 233, 1964. BURCH, J. E., DEBAKEY, M., and SODEMAN,W. A. Effects of venous pressure on volume pulsation. Proc. Sot. Exper. Biol. 6 Med., 42: 858, 1939. SAVAGE, J. P. The role of reflex spasm in the pathogenesis of venous ischemia. Surg. Gynec. & Obst., 113: 47, 1961. CYWES, S. and Louw, J. H. Phlegmasia cerulea dolens; successful treatment by relieving fasciotomy. Surgery, 51: 169, 1962.