Phlegmasia cerulea dolens

Phlegmasia E.JACKSON GILES,

Cerulea M.D.,

Presenled at tbe Annuuf Clinical Session oJ tbe Americun College of Surgeons Second District Cbapter of Texas, May II, 195’6,Corpus Cbristi, Texas.

HLEGMASIA cerulea dolens is a form of of the lower extremities in which there is fulminating venous thrombosis accompanied with marked reflex arterial spasm and by varying degrees of shock. The arterial spasm is so extreme that a diagnosis of arterial embolism is frequently made. Because it appears to be, in all its manifestations, more like an arterial embolus than a thrombophlebitis, it is frequently called “pseudoembolic phiebitis.” Other names for this condition are “blue phlebitis of Gregoire,” “gangrene of venous origin,” and “acute massive venous occlusion of the extremities.” Fifty per cent of these cases progress to gangrene and amputation of the extremity, and one-third end in death. After study of the recent and historical background of this condition, I am firmly convinced that the majority of cases are going unrecognized and are probably mistaken for ordinary thrombophlebitis or phIebothrombosis. History. In 1593 Fabricius Hildanus was the first man to recognize the possibihty of gangrene of venous origin in the extremities. In 1859 and 1894, cases were reported by Hueter and Gaillard. As a clinical entity the condition was described by Tremolieres and Veran in rgzg and by Gregoire in 1938. There were only three case reports in the American literature until DeBakey and Ochsner [?I reported two in 1949. Oaks and Hawthorne [r?] in 1948, and Young and Derbyshire [20] in rg5o reported recovery folIowing ligation of the vena cava. Sympathectomy was not performed in these cases. In 1951 Miles [II] reported a case, to bring the tota up to sixty. He used clot removal, ligation with No. 4 silk, division of the vena cava and sympathetic bIocks. Many cases have been reported since 195 I, and perusal of the literature reveals a

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clelinite difference of opinion as to therapy. More emphasis should be placed on early diagnosis and treatment because of the morbidity as well as mortality associated with phlegmasia cerulea dolens. Phlegmasia cerulea Clinical Manijestations. dolens differs from phlegmasia aIba dolens in that the onset is more sudden and the symptoms more severe. The distinguishing features are a violaceous discoloration of the skin of the affected extremity, accompanied with diminished or absent arterial pulsations and with temperature and sensory alterations. Edema of the leg, thigh and lower trunk is obvious. Lastly, shock of varying degree always accompanies this condition. Of the four cardinal symptoms and signs of the disease-pain, coldness, collapse and cyanosis-the only sign of venous origin is the rapid onset of edema. Haimovici [5] states that phlegmasia ceruIea dolens is preceded in approximately 44 per cent of the cases by phlegmasia alba dolens. It may occur within a few days or after several weeks, and in 56 per cent of the cases it is the initial manifestation. The edema is glossy, tense and woody. Cyanosis is rapid to more dista1 parts first, then becomes absent. The anoxic manifestations are followed in four to eight days with gangrene. Multiple venous occlusions do occur. Of twenty-seven cases reported, two revealed both legs involved. Pathological Physiology. The profound circulatory disturbance simulates arterial occlusion, but pathologic study of amputated Iegs and necropsy specimens has revealed a patent arterial system with associated complete occIusion of the venous channels. The sudden shock in this condition can probabIy best be expIained on the extravasation of serum into the leg, thigh and lower trunk on the affected side. Death in approximately one-third of the cases is considered to be due to profound circulatory collapse. Transient cyanosis and ischemia may

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Giles occur. BIue phlebitis does not necessarily Iead to gangrene, but if present the gangrene is generaIIy moist in type. It appears in the most dista1 parts as a superficia1 bIuish discoloration. BIebs and Iymphangitis appear with poor demarcation, and with infection added the typica picture of wet gangrene is obvious. In approximateIy two-thirds of the cases the dista1 arteries are not paIpabIe. The lower extremities are affected four times as frequentIy as the upper extremities. According to DeBakey and Ochsner [?I, puImonary emboIism occurs in IO per cent of the cases. Etiology. Fontaine and Pereira [4] have shown that gangrene couId be produced in animaIs onIy when the venous return was compIeteIy bIocked. VeaI et al. [19] studied the effect of massive venous occlusion on the arteria1 bIood flow in experimenta work on dogs. The mesenteric vein was Iigated and continuous pressure recordings were made. The cardina1 signs of acute massive venous occIusion were produced. BIood ffow came to a standstiI1 when venous pressure reached its peak. The onIy chance for arteria1 bIood to enter was in the repIacement of ffuid Iost in the tissues. Ochsner and DeBakey [14] beIieve that the most pIausibIe expIanation of the condition is simpIe bIockage of the circuIation by the thrombosis. The fact that the condition progresses to gangrene in spite of antivasospastic measures indicate that vasospasm does not pIay the primary roIe in the genesis of the condition. It may foIIow postpartum thrombophlebitis, chemica1 thrombosis, phIebothrombosis with a shifting thrombus of the vena cava, externa1 or femora1 vein Iigations, postoperative uIcerative coIitis, compIications of surgery, trauma, viscera1 maIignancy, Buerger’s disease, poIycythemia vera or infection. The production of this syndrome depends not onIy on the amount of obstruction but aIso on the suddenness of occurrence. Tberapy. More recent reports continue to advocate conservative therapy directed toward the prevaiIing abnorma1 physioIogica1 process. Since venous engorgement is prominent, VeaI [r9] and his associates have proposed and utiIized with good resuits marked eIevation with manua1 active exercise to the invoIved Iimb to express bIood from the involved Iimb mechanicaIIy. Others have reported good res&s with novocain@ injections of Iumbar sympathetic nerves and gangIia. Vasodilator drugs 430

are advocated, and in the opinion of Davis and Grimes [2] anticoaguIant therapy is the preferred method. Even though previous reports have shown that Iigation of the vena cava is dramatic in its effect, some of the Iater writers have stated that it should not be done unIess puImonary infarcts occur. It is the beIief of most writers that anticoagulant therapy heIps the coIIateraIs. Transfusions of whoIe bIood or pIasma shouId be given immediateIy in those cases in which shock is present. Morphine is necessary to reIieve the pain. It is difFicuIt to seIect the best type of treatment, since good results have been recorded with either conservative measures or operative procedures. MiIes [II] emphasized the physioIogicaI response foIIowing Iigation of the vena cava. Shock disappeared and puIsations in the peripheral vesseIs returned to norma within a very short time. This exceIIent response could not be attributed to the spina anesthetic, because the effect wouid have been noticed soon after the anesthetic was given, and not thirty or forty minutes Iater when the Iigation was done. Ray and Burch [15] reported that vena cava Iigation induced diminished puIsations, and it is a paradoxica1 physioIogica1 response that the puIsations return to norma or become paIpabIe immediateIy after Iigation of the vena cava. The variation in treatment somewhat confuses or disguises the effect of vena cava Iigation. OnIy by recording each individua1 case with immediate observation of the extremity foIIowing Iigation wiI1 we be abIe to determine how consistent are the exceIIent effects. Ligation in this condition is done primariIy to prevent puImonary emboIism, as cIinicaIIy most cases revea1 the thrombus to be we11 into the common iIiac vein and devoid of inff ammatory eIement. It is thus apparent that one can combine a11 the principIes of IogicaI therapy, nameIy, earIy recognition and treatment with Iigation of the vena cava, Iumbar sympathectomy, anticoaguIants, antibiotics, moderate eIevation, moderate active exercise and compression bandages when the patient is aIIowed out of bed. BIood repIacement is essentia1 to prevent death from circuIatory coIIapse. CASE

REPORT

R. D., a sixty-three year oId white man, was seen for the first time at his home on March 15, 1956, at approximately 1:15 P.M. Examination

PhIegmasia

CeruIea DoIens the contents of the abdominal cavity. On exposing the retroperitonea1 area it was obvious that there was no embolus in the aorta or in the common iIiac arteries. The pulsations were present but diminished, and the appearance of the vesseIs was that of vasospasm. The sigmoid coIon was reflected mediaIIy, and the posterior parieta1 peritoneum opened to be reflected aIong with the ureter in a medial direction across the psoas muscle. This exposed the lumbar sympathetic chain on the left which was resected, and the second, third and fourth sympathetic gangIia which were excised. After this, the beIief that an embolus was stiI1 present distal to the common iliac artery almost suggested an exploration distal to the inguinal ligament. However, before this was done, the veins were exposed and a Iarge clot was found Iying in the Ieft common iliac vein as it was crossed by the right common iIiac artery. This area was not visibIe unti1 the fat and areoIar tissue were removed, which were even more dif&uIt to expose in this case because of the edema present in the tissues. When the Ieft common iIiac vein was exposed, it was found to contain a thick, elongated, heavy cIot extending dista1 to the point where it was compressed by the right common iIiac artery. ProximaI to the artery, an examination of the vena cava and the right common iIiac vein revealed no evidence of clotting. Without disturbing the cIot and without opening the vein, umbiIica1 tape was passed about the Iower edge of the vena cava, which was ligated in continuity. OnIy enough of the vena cava was exposed to permit this Iigation. A gaIIbIadder cIamp was used to free the area behind and around the inferior vena cava, and the umbiIica1 tape was drawn beneath the vena cava and tied in pIace. As it was tied, the patient’s genera1 condition was checked. The bIood pressure and puIse did not change with the first appIication of the suture. Therefore the tape was Ieft in pIace since it did not seem to disturb his genera1 condition. However, from the time the vena cava was Iigated and the wound was cIosed with interrupted cotton sutures, examination of the foot reveaIed it to be warm and to have a good puIse in the posterior tibiaI, the dorsaIis pedis, popIitea1 and femora1 vesseIs. The effect of the operation was dramatic, not onIy in increasing the temperature of the leg immediateIy but aIso in reIieving the vasospasm. The cyanosis disappeared rapidIy and

revealed him to be apprehensive but in good spirits. He gave a history of having been on his feet the day before, and that he had had a certain cramping pain in the right caIf at approximately IO:OO P.M. the night before. He gave a history, also, of having had thrombophlebitis in the Ieft leg approximately twelve years previousIy. He had had no acute pain since then, but did have occasiona sweIIing of the Ieft leg if he was up very Iong. He first noticed pain in the Ieft caIf and sweIIing of the left thigh and Ieg at 8:oo A.M. on the day he was first seen. The symptoms grew in severity, and he became increasingly restIess and apprehensive. When seen his face was ashen gray and wet with perspiration and his skin was coId and cIammy. He compIained of severe pain in the Ieft Ieg. There had been no chest pain, dyspnea or hemoptysis. His temperature was normal and his puIse was approximateIy 130, weak and irreguIar. The Ieft Iower extremity was cold, wet, diffuseIy edematous and reddish bIue in coIor. The Ieft dorsalis pedis artery and posterior tibia1 and Ieft popIitea1 puIsations were so diminished that it was questionabIe whether any of them couId be paIpated. Tenderness was present over the course of the Ieft femoral vein. There was a positive Homans sign on the Ieft. By the time the patient couId be gotten to the hospital, with the preoperative diagnosis of a possibIe saddIe emboIus extending into the Ieft common iIiac artery, he was much worse cIinicaIIy and the degree of shock was more profound. The bIood pressure was 70/40 and puIsations had compIeteIy disappeared from the Ieft Iower extremity. ShortIy before he was taken to the operating room it was again possibIe to paIpate, with some question of being positive that minima1 puIsations were present in the Ieft groin and Ieft posterior tibia1 artery. The anesthetist beIieved that the addition of shock required immediate transfusion, because of the extravasation of serum into the greatIy swoIIen Ieft thigh and Ieg. This was done immediateIy foIIowing administration of a splna1 anesthetic. Precaution was necessary not to overIoad the patient, since his past history reveaIed two coronary attacks severa years previousIy. An eIectrocardiogram and x-ray of the chest were within norma Iimits preoperativeIy. A Iong midIine incision in the Iower abdomen, extending four or five fingers above the umbiIicus to the symphysis pubis, was used to expose 43’

GiIes cava is the procedure of choice. Some indications for this Iive-saving procedure are: emboIism despite femora1 vein Iigation, spread of the thrombosis to both femora1 and iIiac veins, faiIure of anticoaguIant therapy to contro1 the spread of the process, sensitivity to dicumarol, advanced thrombosis before the patient is seen, Iesions such as peptic uIcer or tuberculosis where hemorrhage may occur with anticoaguthrombophIebitis and Iants, septic peIvic phIegmasia ceruIea dolens. Some authors state that ligation of the inferior vena cava is not a procedure of eIection because of the Iate deveIopment of undesirabIe sequeIae. Shea and Robertson [r6] reported that of twenty-five patients who had ligation four or more years previousIy, 0nIy sixteen returned to fuI1 activity and the majority had impaired circuIation of the extremities. Bowers patients and Leb [I] foIIowed up thirty-three whose cavae were Iigated one to seven years before demonstrating changes which contraindicated such routine use of the procedure. UnIess thrombosis extends up to or above the renaI veins, the cava may be Iigated in continuity inasmuch as a mechanica bIock is a11 that is considered necessary to prevent puImonary emboIism. The effect of the Iigation shouId be just as good, from the vasospastic reIease, whether the cava is tied or severed. According to Madden [8], Iigation is preferabIe to division because of the technica diffrcuIty of the operation due to the coIIatera1 suppIy and aIso due to the definite intoIerance of bIood Ioss from the circuIation at this site.

the genera1 condition of the patient began to improve remarkabIy. The anxiety and apprehension disappeared, and the p&e and bIood pressure began to improve. The bIood pressure remained at approximately I IO to IZO mm. Hg during the operation, and within a few hours had returned to a normal IeveI. PostoperativeIy a Levin tube was empIoyed and the patient had some feeIing of abdomina1 pressure and sIight nausea. Otherwise, there were no compIaints. The Levin tube was removed at the end of the second day and he was aIIowed to have Iiquids, which nauseated him for twenty-four hours. FoIIowing this, he was given a Iow residue diet and had normal passage of gas and stoo1 within five days. He was kept in bed for thirteen days, and then aIIowed up with elastic bandages appIied to both Iower extremities. During this time he had been treated with anticoaguIants. There has been an adequate foIIow-up for one year, with evidence of good resuIts. COMMENTS

After reaIizing the pathoIogica1 physioIogy which is present, one cannot fai1 to concIude that earIy recognition and treatment wiI1 save Ieg or life. The arteriospasm may be of varying degree and may be of short duration, in which case it wiI1 look Iike an ordinary thrombophIebitis. In the past we have more or Iess recorded those cases which have progressed to CarefuI recordings of those good gangrene. resuIts obtained by therapy within the first eight hours wiI1 be contributory to better care of future cases. If an arteria1 emboIus is best attacked before eight to ten hours have eIapsed, it is natura1 to assume that reIease of this type of occIusion due to vasospasm wiI1 do the most good if it is done within eight to ten hours. In most recorded cases in which surgery was performed after this length of time, the thrombus was found to extend up to or beyond the renaI veins and was found to be both a white and red thrombus. One cannot advocate Iigation of the inferior vena cava for any condition without studying the immediate and Iate resuIts of such a procedure. In most patients whose lives are threatened by a potentia1 puImonary emboIus, treatment may be satisfactory with superficia1 femoral vein Iigation or with anticoaguIants. There remains, however, a limited group of patients for whom Iigation of the inferior vena

SUMMARY I. The patient with phIegmasia ceruIea doIens is offered satisfactory recovery through earIy operative therapy and bIood repIacement. 2. Vena cava Iigation in continuity, with Iumbar sympathectomy and postoperative anticoaguIants is recommended as a satisfactory method of therapy for phIegmasia ceruIea dolens. REFERENCES I. BOWERS, R. F. and LEB,

S. M. Late results of in-

ferior vena cava ligation.

Surgery, 37: 622-628,

1955. 2. DAVIS, 0. G. and GRIMES, 0. F. PseudoemboIic phlebitis (phlegmasia ceruIea dolens); a report of two cases. Am. J. Surg., 86: 480-483, 1953. 3. DEBAKEY, M. and OCHSNER, A. Phlegmasia cerulea dolens and gangrene associated with

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5.

6.

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8. g.

IO. I I.

12.

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thrombophlebitis; case reports and review of the literature. Surgery, 26: 16-29, rg4g. FONTAINE, R. and PEREIRA, S. Obliterations et resections veineuses experimentales; contribution a I’etude de la circmation collaterale veineuse. Rev. de cbir., Paris, 75: 161-200, 1937. HAIMOVICI, H. Gangrene of the extremities of venous origin; review of the literature with case reports. Circulation, r : 225-240, 1950. HAINES, R. D., MCMILLAN, C. D. and STINSON, J. C. Phlegmasia ceruIea doIens. Texas State J. Med., 51: 707-710, 1955. KIRTLEY, J. A., JR., RIDDELL, D. and HAMILTON, E. C. Indications and late resuIts of ligation of the inferior vena cava. Surg., Gynec. @ Obst., 102: 88-89, 1956. MADDEN, J. L. Ligation of inferior vena cava. Ann. Surg., 140: 200, 1954. MANHEIMER, L. H. and LEVIN, L. M. PhIegmasia ceruIea doIens; report of two cases and discussion of pathogenesis. Angiology, 5: 472-478, 1954. MARTIN, P. Phlegmasia ceruIea dolens. &it. M. J., 2: 1351-1353. 1953. MILES, R. M. PhIegmasia cerulea dolens; successful treatment by vena cava ligation. Surgery, 30: 718-721, rg5r. MILLS, E. S. and BENNETTS, R. C. PhIegmasia ceruIea doIens as a cause of gangrene of the iingers. Canad. M. A. J., 72: 917-920, 1955.

DoIens

13. OAKS, W. W. and HAWTHORNE, H. R. PseudoemboIic phlebitis with ligation of the inferior vena cava; a case report. Am. SurgeOn, 127: 1247, 194.8. 14. OCHSNER,A. and DEBAKEY, M. ThrombophIebitis; the roIe of vasospasm in the production of the clinica manifestations. J. A. M. A., I 14: II~123, 1940. 15. RAY, C. T. and BURCH, G. VascuIar responses in man to ligation of the inferior vena cava. Arch. Int. Med., 80: 587-601, 1947. 16. SHEA, P. C., JR. and ROBERTSON, R. L. Late sequeIIae of inferior vena cava Iigation. Surg., Gynec. & Obst., 93: 153, 1951. 17. STREUTER, M. A. and PAINE, J. R. Temporary occlusion of inferior vena cava suggested as a means of treatment in thrombo-embolism requiring cava Iigation. Surgery, 34: 20-27, 1953. 18. TYSON, W. T., JR. and WILSON, H. Acute massive venous thrombosis of the Iower extremity (phIegmasia ceruIea doIens). Am. Surgeon, 18: I 106-1108, 1952. rg. VEAL, J. R., DUGAN, T. J., JAMISON, W. L. and BAUERSFELD, R. S. Acute massive venous occlusion of the lower extremities. Surgery, 29: 355-364, 1951. 20. YOUNG, R. L. and DERBYSHIRE, R. C. Ligation of the inferior vena cava during pregnancy. Ann. Surg., 131: 252, 1950.

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