CHAPTER 21 - Venous and Lymphatic Disease Richard M.

Green Kenneth Ouriel

VENOU D! EA E
Venous Anatomy and Physio"o#y Lower extremity veins can be divided into three types: superficial, deep, and perforatin veins !"i . #$%$&. 'he systemic veins contain approximately two%thirds of the circulatin blood volume under relatively low pressure, and venous flow from the lower extremities must overcome ravity and intraabdominal pressure to return blood to the ri ht ventricle. 'he initial force produced by the left ventricle is reduced throu h the capillary bed to a pressure of about $( mm) in the venules. 'he calf muscles provide an additional pump function as they compress deep veins within an unyieldin fascial compartment. *roximal flow is assured by the presence of the delicate but stron venous valves, which prevent reflux. The supe$%icia" &enous system is composed of the reater and lesser saphenous veins !G+, and L+,& and lies above the investin fascia. 'he G+, be ins in the dorsum of the foot and ascends cephalad anterior to the medial malleolus. -t runs alon the medial aspect of the le , crossin the .nee /oint 0 to $1 cm dorsal to the medial ed e of the patella. 'he saphenous nerve accompanies the vein from the foot to the upper thi h, where it penetrates the fascia and enters the subsartorial canal alon the superficial femoral artery. 'he G+, pierces +carpa2s fascia in the midthi h and enters the fossa ovalis in the roin, 3 cm lateral and inferior to the pubic tubercle. 'he L+, be ins laterally from the dorsal venous arch, courses posterior to the lateral malleolus, and enters the popliteal vein between the medial and lateral heads of the astrocnemius muscle. 'he sural nerve lies lateral to the G+,. The deep &eins are primarily responsible for lower extremity venous return. 'hese veins follow the course of the ma/or arteries and share their names. -n the lower le the veins are paired and /oin at the .nee to form the popliteal vein, which continues throu h the adductor hiatus to become the superficial femoral vein. 'he latter is /oined by the deep femoral vein in the upper thi h to become the common femoral vein, which becomes the external iliac vein as it enters the pelvis beneath the in uinal li ament. 4umbers of valves increase with distance from the heart, thou h the vena cava and common iliac veins are valveless. 5ach valve is based within a dilated sinus of the vein, which .eeps the valve cusps away from the walls and promotes rapid closure when flow ceases. ,alves are the focal point of most of the patholo y of venous thrombosis because their sinuses are where the initial thrombus forms, and the loss of valvular function after recanali6ation of a thrombus produces venous insufficiency !"i . #$%#&. 7utopsies su est that it is more common for thrombi to ori inate in the veins of the soleus and then propa ate proximally, but there is evidence that primary thrombosis of the femoral and iliac venous tributaries occurs as well. 'here also are a number of venous sinuses within the substance of the soleus muscle, which empty into the posterior tibial vein, and in the astrocnemius muscle, which empty into the popliteal vein. 'hese sinuses are critical to the function of the calf muscle pump.

'he perforatin or communicatin veins connect the superficial venous system with the deep and direct flow internally from the superficial veins in all areas of the lower extremity except the foot, where the opposite occurs. 'he perforatin veins are so named because they penetrate the fascia of the lower le to connect the superficial and deep systems. 'he perforators ad/acent to the medial malleolus often are responsible for the development of stasis ulcers at that level when they become incompetent. 8oyd2s perforator connects the G+, to the deep veins $1 cm below the .nee. 9oc.ett2s perforators connect the posterior arch vein with the posterior tibial vein and often become varicose. 'he )unterian perforator connects the G+, to the superficial femoral vein: its incompetence accounts for many thi h varicosities when the saphenofemoral /unction is competent. ;hen a person is in the supine position, lower extremity venous return is primarily dependent on the respiratory cycle. -ntraabdominal pressure increases as the diaphra m descends durin inspiration and the external pressure on the external iliac veins decreases venous return. ,alve closure prevents si nificant reflux durin inspiration. 'he converse occurs durin expiration. ,enous return is increased as intraabdominal pressure is decreased with upward movement of the diaphra m. ;hen a person is in the upri ht position, venous flow is dependent on the contractile force of the heart, static fillin pressure, and ravity. 'he expiratory enhancement of venous flow is insufficient, and the calf muscle pump is necessary to overcome the hydrostatic forces. 7 sin le contraction of the calf muscles can empty <1 percent of the blood pooled in the tibial veins and muscular sinuses. 'he standin venous pressure in the foot veins is 01 to =1 mm) while standin and falls to 31 mm) durin ambulation !see "i . #$%##&.

Deep Vein Th$om'osis

Etio"o#y of >eep ,ein 'hrombosis 'hree factors are primarily responsible for the development of a thrombus within a vein: abnormalities of blood flow, abnormalities of blood, and in/ury to the vessel wall. 'he occurrence of this process in a nontraumati6ed vein was reco ni6ed by Rudolf ,irchow, who introduced the term thrombosis in $0(<. tasis 7lthou h stasis alone is not sufficient, it is the most important factor in the development of deep vein thrombosis !>,'&. 'he main event in the formation of a venous thrombus is the eneration of thrombin in areas of stasis. 'his leads to platelet a re ation and fibrin formation. ;hen contrast medium is in/ected into the veins of the lower extremities of a bedridden patient, it may remain in venous valve sinuses for as lon as an hour, confirmin the poolin effect in the soleal veins. *rimary and secondary vortices are produced beyond the valve cusps, the favored location for the formation of a thrombus, and trap red cells to form the early nidus for thrombus formation. 5arly thrombi attach to normal endothelium and consist of loosely pac.ed red cells within a fibrin networ. accompanied by a variable number of leu.ocytes. 'he propa ation of the thrombus depends on the relative balance between activated coa ulation and fibrinolysis. More commonly, in about <1 percent of patients the thrombus propa ates without interruptin flow and develops a lon floatin ?tail@ that is more susceptible to brea.in loose from its tenuous anchor within the valvular sinus. -t is the latter seAuence of events that is the most dan erous aspect of the

disorder, because ma/or pulmonary embolism can and does occur without premonitory si ns or symptoms at its point of ori in. 'his process can be in under eneral anesthesia in the operatin room but usually reAuires other contributin factors such as shoc., infection, trauma, or con estive heart failure. 7 in , obesity, pre nancy, and mali nant disease also are important ris. factors. Endothe"ia" Dama#e 'he role of endothelial in/ury is Auestionable. -t appears that it is neither a necessary nor a sufficient condition for thrombosis. ;ith the exceptions of hip arthroplasty and central venous catheters, there is little evidence that ross or microscopic venous in/ury has a role in venous thrombo enesis. Routine histolo ic examination of veins containin thrombus usually fails to show an inflammatory response consistent with vessel wall in/ury. -t is possible that hypoxic or biochemical in/ury has a role, but definitive evidence is lac.in . Hype$coa#u"a'i"ity 7bnormalities of the blood include aberrations of the clottin and fibrinolytic systems. +tasis and in/ury alone are not sufficient to cause thrombosis experimentally in the absence of low levels of activated coa ulation factors. *atients who present at an early a e with spontaneous venous thrombosis, who have a stron family history of >,', or who develop recurrent venous thromboembolisms are usually considered ?prothrombotic@ or ?hypercoa ulable.@ 'hese conditions are listed in 'able #$%$. 7ctivated protein 9 resistance !7*9%R& is a common hereditary condition that results in decreased efficacy of the natural anticoa ulant protein 9. -t is transmitted as an autosomal dominant trait, and =1 percent of the cases are because of a mutation in factor , !factor , Leiden mutation&. 'he syndrome was initially described in youn patients with venous thrombosis but may also have a role in hypercoa ulability after arterial reconstruction. 7ntithrombin --- deficiency often is associated with unexplained arterial thrombosis. -t is found more often in patients with serum albumin levels less than B.1 CdL. *atients with antithrombin --- deficiency present as a resistance to heparin. 'ypically, heparin is iven and no increase in the partial thromboplastin time !*''& is noted. -n this settin , immediate anticoa ulation can be achieved by providin substrate !fresh fro6en plasma& in addition to heparin and then conversion to coumarin derivatives. 'he antiphospholipid syndrome !7*+& is another hypercoa ulable state with recurrent thrombotic events and antibodies directed a ainst phospholipids. 'here are primary !no associated autoimmune disease& and secondary forms. 7*+ occurs in youn er patients rather than in atherosclerotic populations, and very few of these patients smo.e. 7ntiphospholipid antibodies, which include anticardiolipin, are bein reco ni6ed with increasin freAuency in association with a variety of thrombotic disorders. 'heir association with unexplained /uvenile >,' indicates that screenin for antiphospholipid antibodies should be included in the wor.up of any unexplained thrombosis. One% third of patients with systemic lupus erythematosus have antiphospholipid antibodies. ;omen with 7*+ often have a history of spontaneous abortions. 'he dia nosis is su ested in a patient with an appropriate history and a spurious elevation of the *''. *atients on estro en therapy for postmenopausal

replacement, birth control, or chemotherapy are at increased ris. for venous thrombosis. 'he association between venous thrombosis and cancer was first su ested by 7rmand 'rousseau in $0(< and often has been confirmed in postmortem studies. -n a series reported by 7der.a and associates, B3 percent of otherwise healthy patients with idiopathic >,' were found to have mali nant disease dia nosed an avera e of #3 months later. -ncreased li.elihood of cancer in these patients was associated with a e over <( years, anemia, and eosinophilia. 'he earliest%onset mali nancies were found within $ year and usually occurred in the pelvic or ans and breast. *atients presentin with a thrombotic episode at a youn a e or those with previous events should be screened for hypercoa ulability. Routine screenin should include measurements of prothrombin time, activated partial thromboplastin time, hematocrit level, white blood cell count, sedimentation rate, and platelet count. Measurements of homocysteine levels, antiphospholipid antibodies, protein 9 and protein +, antithrombin ---, activated protein 9 resistance, platelet a re ation, and mutant factor , should be done in very hi h%ris. patients. +creenin is difficult once anticoa ulation has be un. "or instance, coumarin derivatives interfere with measurements of proteins 9 and + and the functional assay for activated protein 9 resistance, heparin reduces circulatin levels of antithrombin ---, and antiplatelet dru s may produce false ne atives when testin for heparin%induced thrombocytopenia. e(uence o% Patho"o#y 'he venous lumen is most often recanali6ed after an episode of >,'. 'his process is a result of spontaneous lysis and involves a complex series of cellular and humoral processes. Or ani6ation of the thrombus be ins at the attachment 6one as endothelial cells activate thrombus%bound plasmino en. 'his results in enlar in poc.ets within the thrombus and eventual fra mentation. 'he clot itself under oes softenin and contraction durin this process, with the potential to restore the venous lumen. +erial studies usin duplex ultrasono raphy show that the process of recanali6ation be ins by day D in rou hly (1 percent of thromboses and is uniformly observed by =1 days. Recurrent thrombotic events compete with recanali6ation early in the course of a >,'. 'his encompasses those patients with propa ation of clot in previously uninvolved areas, thromboses in another extremity, and rethrombosis of a partially recanali6ed se ment. 'he incidence of these recurrences is reduced tenfold when patients are iven adeAuate anticoa ulation therapy for a B%month period. C"inica" )ani%estations 'he site of venous obstruction determines the level at which swellin is observed clinically !"i . #$%B&. 9alf vein thrombosis is locali6ed to one or more of the three ma/or named veins below the .nee. 9alf tenderness is freAuently present, but because the thrombi are rarely completely obstructive and the veins are paired, swellin is not a universal findin . "emoral vein thrombosis usually is associated with swellin of the foot and calf. -liofemoral venous thrombosis represents the most extensive form of >,' and usually is associated with tenderness in the roin and swellin of the entire le . Ma/or venous thrombosis involvin the deep venous system of the thi h and pelvis produces a characteristic presentation of pain and extensive pittin edema. 'he

extremity may have bluish discoloration !phle masia cerulea dolens& or blanchin !phle masia alba dolens, or ?mil. le @&. 'he latter usually occurs in association with pre nancy. Other mechanical factors that can affect the left iliac vein include compression from the ri ht iliac artery, an overdistended bladder, and con enital webs within the vein. 'hese factors are responsible for the observed 3:$ preponderance of left versus ri ht iliac vein involvement. *hle masia cerulea dolens occurs when the venous thrombosis pro resses and impedes most of the venous return from the extremity. 'here is dan er of limb loss from cessation of arterial flow. "ortunately, this occurrence is rare. Loss of sensory and motor function and venous an rene are li.ely unless an a ressive approach is implemented to remove the thrombus and restore blood flow. 'his condition almost always occurs with advanced mali nant disease. Dia#nosis Only 31 percent of patients with venous thrombosis have any clinical si ns of the disorder. )omans2s test is performed by dorsiflexin the foot. -t is considered positive for >,' if the patient complains of calf pain. "alse% positive clinical si ns occur in more than B1 percent of patients studied. ,enous duplex ultrasono raphy has rele ated other noninvasive tests, such as radioactive%labeled fibrino en scans and all types of plethysmo raphy, to historical interest. -n some centers duplex scans have replaced contrast veno raphy as the best dia nostic test for >,'. 7ccuracy rates above =1 percent have been consistently reported for venous duplex exams. -ndications for duplex venous scans include patients with pulmonary emboli, patients with extremity pain or swellin , and patients at increased ris. for developin a >,'. 'he latter roup includes those with trauma, /oint replacement, other ma/or sur eries, prolon ed immobili6ation, and .nown hypercoa ulability states. 'here are three essential phases to the venous duplex scan: !$& thrombus visuali6ation, !#& vein compressibility, and !B& venous flow analysis. 7ccuracy is dependent on the examiner2s s.ill. 'hrombus may be difficult to visuali6e in its acute form, and the addition of color flow ima in facilitates the identification of nonoccludin clots. 'hrombus echo enicity increases with a e of clot. ,enous compressibility is determined by placin the probe directly over the vein and applyin entle pressure while observin under 8%mode ima in !"i . #$%3&. ,eins filled with thrombus do not collapse with this maneuver. ,enous flow assessment evaluates the respiratory phasicity and response to external extremity compression. *ersistent lac. of a flow si nal indicates total obstruction. 7 ne ative scan performed by a well% trained ultrasono rapher is sufficient to rule out a >,' of the lower extremity. 'he role of veno raphy has been diminished by the advances in ultrasound technolo y. 4onetheless, the in/ection of contrast material for direct visuali6ation of the venous system of the extremity remains the most accurate method of confirmin the dia nosis of venous thrombosis and the extent of involvement. 'he main indication for its use in the dia nosis of an acute >,' is a nondefinitive duplex scan. -n/ection usually is made into the foot while the superficial veins are occluded by tourniAuet, and a supplemental in/ection into the femoral veins may be reAuired to visuali6e the iliofemoral system !"i . #$%(&. *otential false%positive examinations may result from external compression of a vein or washout of the contrast material from venous flow from collateral veins.

P$ophy"a*is 8ecause the first manifestation of a >,' may be a fatal pulmonary embolus, some form of prophylaxis is indicated in hi h%ris. patients. *atients older than D1 years of a e, those with previous thromboembolism, mali nant disease, paralysis, multiple trauma, or lower extremity /oint sur ical procedures have a very hi h ris. for >,'. *rophylactic measures are directed toward alterin blood coa ulability or eliminatin or reducin venous stasis. 5fforts to reduce stasis include elastic compression stoc.in s, intermittent external le compression, le elevation, and early ambulation. -ntermittent pneumatic le compression is the most effective measure. -t reduces stasis and increases fibrinolytic activity with virtually no side effects. 'he pneumatic boots can be applied in the operatin room to minimi6e the ris. of venous thrombosis be innin under eneral anesthesia and are of proven efficacy in patients under oin total .nee replacement, radical prostatectomy, or operations where ad/uvant anticoa ulation therapy is contraindicated. *harmacolo ic prophylaxis includes low%dosa e unfractionated heparin !E")&, ad/usted%dose heparin, low%molecular% wei ht heparin !LM;)&, warfarin !international normali6ed ratio #.1FB.1&, and dextran D1. *rophylactic low%dosa e subcutaneous E") that does not alter the clottin profile has been extensively tested and is safe and effective in moderate%ris. patients. 7 (111%unit dose is iven subcutaneously # h preoperatively and then every $# h postoperatively for < days. 'his provides protection for most hi h%ris. roups with the exception of those under oin orthopaedic or urolo ic procedures. )i her% ris. patients reAuire ad/usted% dose E") with the activated partial thromboplastin time !7*''& held in the upper normal ran e. 8oth re imens are associated with an increased incidence of wound hematomas. 'he studies comparin the prophylactic use of LM;) and E") have concluded that there is little difference between the two dru s. LM;) is ten times more costly than E"), however. 'here are ood data to support the use of preoperative oral anticoa ulant therapy with coumarin derivatives in hi h%ris. patients. ;hen iven the ni ht before operation, warfarin anticoa ulation is achieved within B to 3 days. 'he ?two%step@ or minidose warfarin re imen is desi ned to circumvent the delay in anticoa ulation. ;arfarin is started at a dose of $ m $3 days before operation to prolon the prothrombin time !*'& by # or B seconds. 'his procedure increases the ris. of hemorrha e, and because of the added difficulties of laboratory control of prothrombin time, there has not been widespread acceptance of this approach. 7 national tas. force on prophylaxis for patients under oin hip sur ery recommends warfarin or ad/usted%dose heparin to prolon the 7*'' to the upper normal ran e. 'he administration of dextran, which produces a variety of effects on platelets and clottin factors, has been demonstrated to reduce the incidence of detectable thrombi. -t too can produce hemorrha ic problems, aller ic reactions and, in older patients, con estive heart failure. Recommendations for prophylaxis are listed in 'able #$%#. )edica" T$eatment 'he approach to mana ement of the patient with >,' is based on three ob/ectives: minimi6in the ris. of pulmonary embolism, limitin further thrombosis, and

facilitatin resolution of existin thrombi to avoid the postthrombotic syndrome. 'he traditional treatment places the patient at bed rest with the foot of the bed elevated 0 to $1 inches. -ntravenous E") is administered, and oral warfarin is started when the patient2s 7*'' is in a satisfactory ran e. 7s the pain, swellin , and tenderness resolve over a ( to D day period, ambulation is permitted with elastic stoc.in support. +tandin still and sittin should be prohibited to avoid increased venous pressure and stasis. *atients with lar e thrombus loads are candidates for fibrinolytic a ents in an attempt to preserve venous valvular competence. 'he practice of mandatory bed rest and hospitali6ation has been challen ed by the encoura in results of outpatient treatment of >,' with LM;). Anticoa#u"ation 'he foundation of therapy for >,' is adeAuate anticoa ulation, initially with heparin and then with coumarin derivatives for prolon ed protection a ainst recurrent thrombosis. Enless there are specific contraindications, heparin should be administered in an initial dose of $11 to $(1 unitsC. intravenously. )eparin is an acid mucopolysaccharide that neutrali6es thrombin, inhibits thromboplastin, and reduces the platelet release reaction. -t may be administered by continuous or intermittent intravenous doses re ulated by whole blood clottin time or 7*''. Recurrent episodes of thromboembolism are $( times more common in patients with inadeAuate anticoa ulation treatment within the first #3 hours. 8leedin complications can be minimi6ed by doses of heparin that prolon the laboratory clottin determinations by about twice the normal time. 9ontinuous intravenous infusion re ulated by an infusion pump minimi6es the total dose reAuired for control and is associated with a lower incidence of complications and no loss of effectiveness. 'hrombocytopenia is the most common complication of heparin therapy and is estimated to occur in $ to ( percent of patients receivin the dru . Enli.e other dru % induced thrombocytopenias, heparin%induced thrombocytopenia often is associated with thromboembolic complications from antibody%mediated platelet activation. 'he paradox of thrombosis occurrin in a patient receivin heparin was first described in $=(0 by ;eismann and 'obin. 'owne and associates described the ?white clot@ syndrome, a peripheral vascular complication of heparin therapy in $=D=. )eparin% induced thrombocytopenia !)-'& represents the prodrome to the thrombotic syndrome !)-''+& that occurs in $ in #111 patients who receive more than #1,111 -E of E") per day for more than ( days, $ in ( patients with )-', and $ in B patients who have heparin%dependent antiplatelet antibodies. *atients who develop )-''+ have a mortality rate ran in from #( to BD percent resultin from diffuse uncontrolled clottin with limb ischemia and or an infarction. 'here are no .nown factors that predict ris.. >evelopment of thrombocytopenia from heparin is independent of sex, a e, blood type, amount of heparin iven, type of heparin, and route of administration. )-' has been documented after minimal heparin dosa es such as those received with -.,. flushes and heparin% coated indwellin catheters. 'wo forms of )-' exist. 'ype -, the most freAuent, is mild !platelet counts G$11,111CmmB&, reverses despite continuation of heparin, is due to a direct pro% a re ant effect of heparin, and is not associated with thromboses. -n contrast, 'ype -- )-' is severe !platelet counts H$11,111CmmB&, resultin from antibodies bindin to a platelet%heparin complex that leads to platelet activation and a re ation and is often associated with arterial or venous thromboses. 'he thrombocytopenia typically

occurs after ( days of heparin therapy but can occur earlier in patients who have a prior exposure to heparin. 'ype -- )-' reAuires the immediate withdrawal of all heparin. 'he most important advanta e of the LM;)s over E") is their superior pharmaco.inetic properties, allowin their use without laboratory monitorin . LM;) preparations have been compared with E") for the acute treatment of >,' in $B well% desi ned trials. *ooled results from these studies show that LM;)s administered subcutaneously are as effective and safe as E") but have the advanta e of the potential for home treatment and do not reAuire laboratory monitorin . 'hese advanta es may offset the increased cost of LM;). Oral administration of anticoa ulants is be un shortly after initiation of heparin therapy. 'here is a ris. in ivin coumarin derivatives to a patient who is not already anticoa ulated with heparin. 'he coumarin derivatives bloc. the synthesis of the vitamin K%dependent clottin factors and inhibit vitamin K carboxylation of proteins 9 and +. 'hese latter proteins are naturally occurrin anticoa ulants that function by inhibitin activated factors , and ,---. 7 vitamin K anta onist potentially can create a hypercoa ulable state before achievin its anticoa ulant effect because the half%lives of proteins 9 and + are shorter than the half%lives of the other clottin factors. )eparin should be continued for the 3 to ( days reAuired to achieve full anticoa ulation with coumarin derivatives. >ata from prospective studies indicate that the level of anticoa ulation with coumarin derivatives are effective at an international normali6ed ratio of #.1 to B.1. )i her levels are not more effective and are associated with a hi her incidence of bleedin complications. 7dministration of fresh fro6en plasma usually can normali6e the prothrombin time and control hemorrha ic complications. 9oumarin derivatives cross the placenta and should not be used durin pre nancy. 7fter an episode of acute >,', anticoa ulation therapy should be maintained for a minimum of B months: some investi ators favor < months for treatment of thrombi in the lar er veins. Many dru s interact with coumarin derivatives !e. ., barbiturates&, and it is essential that a routine for re ular monitorin of prothrombin time be established after the patient leaves the hospital. Th$om'o"ysis 7nticoa ulant therapy is desi ned to prevent recurrent thromboembolism. -deally, a treatment would be available with the potential to eliminate the thrombus and maintain valvular function. 7 number of trials have been performed comparin thrombolysis with standard anticoa ulant therapy. 9omplete clearin of thrombus was noted in 3( percent of patients treated with thrombolytic a ents, compared to 3 percent of those treated with heparin. 'his seems to translate into a lon %term improvement in venous function. *opliteal valve incompetence was documented in DD percent of those patients who did not have clearin , compared to a = percent incidence in those with complete lysis. 'hese a ents have no advanta e over heparin in the treatment of recurrent venous thrombosis or thrombosis that has existed for more than D# h, and they are contraindicated in postoperative or posttraumatic patients. 'here are more bleedin complications with thrombolytic treatment, and this approach is reserved for those patients with clot in the common femoral and iliac

venous systems. -n a prospective study of #= patients with thrombosis involvin the popliteal veins, with or without involvement of calf veins, Ka..ar compared hemodynamic and clinical results in patients receivin (%day treatment with heparin or strepto.inase, followed by a <% month course of a coumarin derivative. Overall, at #%year follow%up they found more than half of the limbs to have evidence of the postthrombotic syndrome. 9linically, $3 percent of patients had no symptoms, #1 percent had severe symptoms, and the remainder demonstrated mild to moderate chan es. 4o difference was seen between patients receivin heparin or strepto.inase. 'hese studies were done with systemic infusion of lytic a ents. +tudies evaluatin the use of catheter%directed thrombolysis have had encoura in results in selected areas and are discussed in detail in the section +ubclavian ,ein 'hrombosis.

u$#ica" App$oaches
Ope$ati&e Th$om'ectomy 'here are very few indications for operative thrombectomy because catheter%directed thrombolysis is so effective in treatin iliofemoral venous thrombosis. 'he procedure is reserved for limb salva e in the presence of phle masia cerulea dolens and impendin venous an rene and in patients with a contraindication to thrombolysis. 'he direct sur ical approach to remove thrombi from the deep veins of the le uses the common femoral vein and is facilitated by the use of a "o arty venous balloon catheter and an elastic wrap for mil.in the extremity !"i . #$%<&. Results are improved when the extent of thrombus is documented preoperatively, when completion phlebo raphy is performed to assure complete clot removal, when a small arteriovenous fistula is constructed to maintain hi h blood flows in the iliac vein, and when anticoa ulation therapy is iven over prolon ed periods. 5arly results in (D patients treated in this fashion reported by 5inarsson and associates showed patency of the iliofemoral se ment by veno raphy in <$ percent, and D( percent had a ood clinical result. Measurement by venous function, however, usin plethysmo raphy and foot volumetry, showed normal results in only #= percent. 'he use of arteriovenous fistulas after iliofemoral thrombectomy or reconstruction of the venous system is controversial. Most of the experience has been accumulated in 5urope, where it is believed to reduce the incidence of early rethrombosis. 'he two most commonly used sites are the femoral trian le and the an.le. 7fter sur ery on the iliofemoral system, an )%shaped fistula can be established easily by anastomosin a branch of the saphenous vein end%to%side to the proximal portion of the superficial femoral artery. 7t the an.le, the posterior tibial artery may be anastomosed to the posterior tibial vein or the reater saphenous vein. 'wo problems have led to the reluctance of some sur eons to adopt this procedure: the fear of dama in functionin valves distal to the fistula and the reAuirement for a second operation to close the fistula. "istulas usually are closed B to 3 months postoperatively, and problems with incompetent valves distal to the fistula have not been reported. 'wo steps durin primary venous reconstruction simplify operative closure of the fistula later. 'he fistula is made distal to the venous reconstruction, avoidin dama e to this area at reoperation, and a li ature is wrapped around the fistula and left in the subcutaneous tissue, where it can be found under local anesthesia. Obliteration of the fistula percutaneously by a detachable balloon has been described. Vena Ca&a" !nte$$uption

,ena caval interruption also is indicated when there is a contraindication to or failure of anticoa ulation therapy !'able #$%B&. 5arly operations in which one or both common femoral veins were li ated were associated with hi h recurrence rates and a hi h incidence of seAuelae because of stasis in the lower extremity. 9ontrol of the inferior vena cava for li ation reAuired a laparotomy and added the adverse effect of a sudden reduction in cardiac output under eneral anesthesia. 'his effect, coupled with stasis seAuelae and recurrent embolism throu h dilated collateral veins, led to efforts to compartmentali6e the vena cava by means of sutures, staples, and external clips in order to provide filtration without occlusion !"i . #$%D&. 8ecause these procedures reAuired eneral anesthesia and laparotomy, the next lo ical step was to devise a transvenous approach that could be performed under local anesthesia. 'he Mobin%Eddin ?umbrella@ unit was inserted from the /u ular vein and positioned under fluoroscopic control below the renal veins. 'he incidence of vena cava occlusion was D1 percent, and fatal embolism sporadically occurred with device mi ration. 'he Greenfield cone%shaped filter was developed to maintain patency after trappin emboli. 'his is possible because of the eometry of the cone, which collects emboli in its apex and retains perimeter flow. *reservation of flow avoids stasis and facilitates lysis of the embolus !"i . #$%0&. -t can be inserted percutaneously from either the /u ular vein or the femoral vein. 'he rate of recurrent embolism with this device has been 3 percent over $# years of follow%up. -ts lon %term patency rate in excess of =( percent allows it to be placed above the renal veins when necessary for embolism control, such as when there is a thrombus within the renal veins or the vena cava. 'here are a number of proprietary devices available for percutaneous insertion that are eAually successful in preventin pulmonary embolism. upe$%icia" Th$om'oph"e'itis 'he term thrombophlebitis should be restricted to a disorder of the superficial veins characteri6ed by a local inflammatory process that usually is aseptic !"i . #$%=&. *atients present with a painful swellin and erythema alon the course of a superficial vein. 'he cause of thrombophlebitis in the upper limb usually is acidic fluid infusion or prolon ed cannulation. -n the lower extremity it is often associated with varicose veins and may coexist with >,'. -ts association with the in/ection of contrast material can be minimi6ed by washout of the contrast material with heparini6ed saline. 'he dia nosis usually is obvious. >uplex scans are very accurate in confirmin the dia nosis and should be performed especially when swellin is present to rule out a concomitant deep venous problem. +ymptoms usually last for # to B wee.s. 4onsteroidal anti% inflammatory a ents provide si nificant pain relief. *atients should not be .ept at bed rest. 7ctivity should be encoura ed with the extremity in external elastic support. -f the thrombus extends into the saphenofemoral /unction, the patient should have the saphenous vein disconnected from the common femoral vein or under o full anticoa ulation therapy. Th$om'oph"e'itis )i#$ans 'hrombophlebitis mi rans, a condition of recurrent episodes of superficial thrombophlebitis, can be associated with visceral mali nancy, 8uer er2s disease, the hypercoa ulable states, systemic colla en vascular disease, and blood dyscrasias.

-nvolvement of the deep veins and the visceral veins also has been described. 'he presence of this condition should alert the clinician to search for an underlyin condition. u'c"a&ian Vein Th$om'osis +ubclavian venous thrombosis !+,'& may be associated with an anatomic abnormality at the thoracic outlet, may be related to the placement of a central venous catheter, or may occur in a hypercoa ulable patient. -f left untreated, #( to D3 percent of affected patients will have some limitations in activity, as many as $# percent will have a pulmonary embolus, and $ percent will die. 'hose cases related to a thoracic outlet abnormality ma.e up 1.( percent to $.( percent of all venous thromboses and often are associated with strenuous activities. 'hese cases are referred to as the *a et% +chroetter syndrome or ?effort thrombosis.@ 9atheter%induced thrombosis is an increasin ly common event because of the more freAuent use of central veins for access, nutrition, chemotherapy, and monitorin . +creenin veno raphy in patients with central venous catheters demonstrates that BB to <1 percent have thrombus in the axillosubclavian se ments. 9linically evident +,' develops in B percent of these patients. *atients with +,' present with a bluish, swollen arm and a pattern of upper extremity venous hypertension. 9ollateral veins usually are visible around the shoulder and chest wall. *atients typically describe an achin pain that is exacerbated by exercise. 'he color duplex scan has virtually replaced contrast veno raphy in the dia nosis of lower extremity venous thrombosis, but the opposite is true in the upper extremity. ,eno raphy has a reater dia nostic accuracy when performed with the catheter in the basilic vein. 7 typical +,' is shown in "i . #$%$1. >ata support a role for conventional anticoa ulation in all patients with +,' for prophylaxis a ainst pulmonary embolism and for reduction of residual symptoms. 7 ressive sur ical therapy focused on clot removal is no lon er recommended. -nstead, physically active patients with +,' dia nosed in the acute phase should under o catheter%directed thrombolysis. 9urrent protocols recommend uro.inase delivered via a catheter placed throu h the thrombus. 'his approach is successful in more than (1 percent of patients and ideally identifies the anatomic cause of the thrombosis !"i . #$%$$&. 7lthou h the timin of correction is debated, most sur eons a ree that extrinsic and extrinsic venous lesions should be a ressively treated. 'his means eliminatin the compression at the costoclavicular space by a transaxillary first rib resection or a medial claviculectomy. 'he former is used when a direct approach to the vein is not reAuired: the latter is used when the vein reAuires repair. !n%e$io$ Vena Ca&a" Th$om'osis 'hrombosis of the inferior vena cava can result from tumor invasion or propa atin thrombus from the iliac veins. 'umors of the vena cava are rare and are usually mali nant and have a poor pro nosis. 'hey may be primary, such as a leiomyosarcoma, or secondary, such as a hypernephroma and a retroperitoneal sarcoma. +ymptoms and si ns depend on the se ment of vena cava affected by the tumor and the de ree of obstruction of ad/acent or ans. -nvolvement of the suprahepatic cava may cause the 8udd%9hiari syndrome. 'he dia nosis can be made with a variety of ima in modalities, includin ma netic resonance ima in !MR-&, computed tomo raphy !9'&, ultrasono raphy, and contrast cavo raphy.

'he most common cause of vena cava obstruction is iatro enic, resultin from li ation, plication, or insertion of partially occludin caval devices. 7ny caval filtration device can become totally occluded by a trapped massive thrombus, causin sudden reduction in venous return and cardiac output. -n a patient with .nown prior pulmonary embolism, it is a rave error to ascribe the resultin hypotension to recurrent pulmonary embolism and treat the patient with vasopressor a ents. -n this situation, the cause of the hypotension may be functional hypovolemia that can readily be confirmed by measurement of central venous pressure. 'hrombosis of the renal vein can result from extension of vena caval thrombosis, but it is most li.ely to occur in association with the nephrotic syndrome. -t can be a source of thromboembolism and has been treated successfully by suprarenal placement of the Greenfield filter. Visce$a" Venous Th$om'osis Mesenteric venous thrombosis is an uncommon but often fatal cause of intestinal ischemia. *atients present with nonspecific abdominal complaints. *ain out of proportion to abdominal findin s is a common occurrence. Enli.e arterial ischemia, pro ression is slow. >elay in dia nosis is freAuent. Laboratory findin s are not usually helpful. 7bdominal 9' scannin is the best dia nostic tool for demonstratin thrombus in the superior mesenteric vein. 9olor duplex scans also are accurate, especially if performed early in the course of the disease. 'he event may be spontaneous and idiopathic or associated with hypercoa ulability, pancreatitis, trauma, cirrhosis, splenome aly, or infection. *olycythemia is the most common associated condition. 'he oal of treatment is to prevent intestinal necrosis, or, if bowel necrosis has already occurred, to resect the nonviable areas. 7nticoa ulation therapy with heparin is the mainstay of medical mana ement. *atients with local or diffuse peritonitis should under o abdominal exploration. *ortal vein thrombosis can occur in the neonate, usually secondary to propa atin septic thrombophlebitis of the umbilical vein. 9ollateral development leads to the occurrence of esopha eal varices. -n the adult, thrombosis of the portal, hepatic, splenic, or superior mesenteric vein can occur spontaneously but usually is associated with hepatic cirrhosis or a hypercoa ulable state. )epatic vein thrombosis !8udd%9hiari syndrome& usually produces massive hepatome aly, ascites, and liver failure. -t can occur in association with a con enital web, endophlebitis, or polycythemia vera. +ome success has been reported usin a direct approach to the con enital webs, but the usual treatment is a side%to%side portacaval shunt to allow decompression of the liver. 'he development of pelvic sepsis after abortion, tubal infection, or puerperal sepsis can lead to septic thrombophlebitis of the pelvic veins and septic thromboembolism. Li ation of the ovarian vein and vena cava has been the traditional treatment, but the emphasis should be on draina e or excision of the abscesses and appropriate antibiotic therapy. -t also is appropriate to use the Greenfield filter in this situation because it is inert stainless steel and avoids the development of an intraluminal abscess that can occur after li ation of the vena cava, as demonstrated experimentally by *eyton and associates in $=0B.

Pu"mona$y Th$om'oem'o"ism *ulmonary embolism is the third leadin cause of death from cardiovascular events, second only to myocardial infarction and stro.e. 5stimates of the mortality in the Enited +tates alone ran e from (1,111 to #11,111 per annum. -t may be the most common form of preventable hospital death. -t is estimated that ( of every $,111 adults under oin ma/or sur ery will die from massive pulmonary embolism. ,irchow first reco ni6ed the association between the venous thrombosis and pulmonary embolus after performin autopsies in D< patients with antemortem thrombi obstructin their pulmonary arteries. -t also became obvious in the early reports by patholo ists that pulmonary embolism could be well tolerated by some patients who then died of other causes. 'he full spectrum of the disorder ran es from asymptomatic minor embolism to sudden death from massive embolism. 7utopsy studies consistently demonstrated a $1 to $( percent incidence of fatal pulmonary embolism until the $=D1s. ;ith a ressive prophylaxis, the incidence has been reduced to < percent. C"inica" )ani%estations 'he si ns and symptoms of an embolic episode depend primarily on the ma nitude of embolus and, to a lesser extent, on the cardiopulmonary status of the patient. Less than BB percent of patients with documented pulmonary embolism show clinical si ns of venous thrombosis. 'he dia nosis is unsuspected in the ma/ority of patients who die of pulmonary embolism. 'he vast ma/ority of patients suddenly develop chest pain or dyspnea. Other early symptoms may include tachypnea, diaphoresis, and mar.ed anxiety. )emoptysis is an uncommon si n, and when present it usually occurs late in the course of the disease and represents pulmonary infarction. Ob/ectively, the patient with ma/or embolism usually shows tachycardia, an increased pulmonary second sound, cyanosis, prominent /u ular veins, and varyin de rees of collapse. Less commonly, there may be whee6in , a pleural friction rub, splintin of the chest wall, rales, low% rade fever, ventricular allop, and wide splittin of the pulmonic second sound. 'he incidence of these findin s found in the Ero.inase *ulmonary 5mbolism 'rial is shown in 'able #$%3. 'he differential dia nosis includes esopha eal perforation, pneumonia, septic shoc., and myocardial infarction. 8ecause all these entities are life% threatenin , it is mandatory that an orderly approach be formulated to confirm or re/ect the wor.in dia nosis. Laboratory studies in eneral are not very helpful in the differential dia nosis, althou h a white blood cell count of less than $(,111CmmB mi ht be su estive when a pulmonary infiltrate is present to help rule out pneumonitis. Dia#nostic tudies 5lectrocardio raphy 'he primary value of the electrocardio ram !59G& is to rule out a myocardial infarction. -n the presence of a pulmonary embolus, the 59G may have si ns of ri ht ventricular overload such as the +$, IB, 'B pattern. 'his only occurs in $< percent of patients with documented pulmonary embolism. More commonly, the 59G has nonspecific +' and ' wave chan es that are nondia nostic. Chest Radio#$aphy

'he primary value of the chest radio raph is to exclude other dia nostic possibilities, such as pneumonia, pneumothorax, esopha eal perforation, or con estive heart failure. 7lthou h central vascular enlar ement, asymmetry of the vascular mar.in s with se mental or lobar ischemia !;estermar.2s si n&, or pleural effusion may su est pulmonary embolism, they are rarely sufficient to establish a dia nosis. 'he chest radio raph is critical in the interpretation of a lun scan, because any radio raphic density or evidence of chronic lun disease ma.es a perfusion defect less li.ely to represent a pulmonary embolism. *ulmonary infarction as a conseAuence of embolism is a rare findin . A +ed#e-shaped density usua""y is seen on the chest radio raph !"i . #$%$#&. A$te$ia" ,"ood -ases )ypoxemia with *aO # of less than <1 mm) is hi hly su estive of pulmonary embolism, especially when the chest radio raph does not show any other pulmonary patholo y. 'he low *aO # is believed to be a result of shuntin by overperfusion of nonemboli6ed lun and a widened alveolar% arterial oxy en radient from reduced cardiac output. 'he reduction in arterial *9O # that follows ma/or embolism is the most discriminatin findin because hypoxemia is present in several disorders li.ely to be misdia nosed as massive embolism !e. ., septic shoc.&. -f hypoxemia and hypocapnia are not present, the dia nosis of ma/or embolism in the severely ill patient is unli.ely, and an alternative dia nosis should be sou ht. Cent$a" Venous P$essu$e Low central venous pressure !9,*& virtually excludes pulmonary embolism as the primary cause of the hypotension because massive embolism almost always is accompanied by ri ht ventricular overload and elevated ri ht atrial pressures. 5levated ri ht ventricular fillin pressures may be transient, however, as hemodynamic accommodation occurs, and in subacute or chronic embolism the central venous pressure may be normal. Lun# can 'he most commonly used dia nostic test is the perfusion lun scan. 7 normal scan rules out a ma/or pulmonary embolus, but an abnormal scan does not ensure the dia nosis. -n a nonhypotensive patient with a normal chest radio raph, the lun scan is a valuable screenin test that has increasin validity as the si6e of the perfusion defect approaches lobar distribution !"i . #$%$B&. +maller peripheral perfusion defects are more difficult to interpret because pneumonitis, atelectasis, or other ventilation abnormalities alter pulmonary perfusion. ;hen the ventilationC perfusion lun scan is interpreted as hi h probability, the dia nostic accuracy as compared to pulmonary an io raphy is =< percent. Pu"mona$y A$te$io#$aphy +elective pulmonary arterio raphy is the most accurate method of confirmin the presence of pulmonary emboli and should be performed for any eAuivocal ventilationCperfusion scan. 'he procedure is invasive, reAuirin passa e of a cardiac catheter into the pulmonary artery for in/ection of a bolus of contrast medium. 7 series of radio raphs that outline areas of decreased perfusion and usually show fillin defects or the rounded trailin ed e of impacted emboli !"i . #$%$3& is obtained. +trai ht cutoffs of the smaller pulmonary arteries are more difficult to interpret, particularly if there is associated chronic lun disease that obliterates pulmonary

vessels. 'he procedure can be performed with low ris., althou h pulmonary hypertensive and cardiac patients are at hi hest ris. for this type of study, which usually carries a 1.B to 1.( percent mortality rate. 7voidance of in/ection of contrast medium into the main pulmonary artery minimi6es the complications and mortality rates. 7dditional useful information is obtained before contrast in/ection by measurement of pulmonary arterial pressures. 7 normal pulmonary an io ram excludes the dia nosis of pulmonary embolism in acutely ill patients. Pathophysio"o#y -t is estimated that 0( to =1 percent of all pulmonary emboli ori inate from the veins of the lower extremity, and the remainder arise from the ri ht side of the heart or other veins. Once the embolus has lod ed and interrupted pulmonary blood flow, the ratio of re ional ventilation to perfusion increases, and the lun responds by bronchoconstriction to reduce wasted ventilation. 'his response is mediated by a local reduction in 9O#output because it can be prevented by ventilation with increased concentration of 9O#. 'he bronchoconstriction is exacerbated by the release of serotonin from platelets adherent to the embolus. 'he ability of heparin to inhibit the release of serotonin adds further /ustification to the early use of this dru . Other vasoactive a ents, such as histamine and prosta landins, may have a role, but the net effect is a reduction in si6e of peripheral airways, reduced lun volume, and reduced static pulmonary compliance. 'he hypoxemia that characteri6es ma/or embolism is thou ht to be due to a ventilation% perfusion imbalance secondary to the ventilation chan es described above, althou h the findin s in some patients resemble true arteriovenous shuntin . 7lthou h there may be some improvement in *aO #after supplemental oxy en is administered, the effects usually are minimal. 'he pulmonary vascular and cardiac effects of embolism are a direct conseAuence of the de ree of occlusion of the pulmonary vascular bed. 'he loss of more than B1 percent of the vascular tree is reAuired to be in to elevate mean pulmonary artery !*7& pressure, and usually more than (1 percent occlusion is reAuired to reduce systemic pressure. T$eatment Anticoa#u"ation 'he hemodynamic variables previously described provide a means of classification of patients that uses four rades of severity and is a useful uide to therapy and pro nosis !'able #$%(&. 'he minor de rees of embolism usually can be mana ed with anticoa ulants alone with a satisfactory outcome. 9ontinuous%infusion heparin is the initial treatment, in a dosa e desi ned to prolon the partial thromboplastin time to at least twice normal !approximately $(1 unitsC . &. Most clinicians also be in oral anticoa ulation therapy to allow several days2 overlap of the dru s as prothrombin time is extended into the therapeutic ran e. 7deAuate anticoa ulation stops the pro ression of thrombosis and is associated with a recurrence rate of less than ( percent. .i'$ino"ytic The$apy 5mboli typically under o dissolution as a result of the active fibrinolytic mechanism in the pulmonary circulation. 7ctivation of plasmino en to plasmin, which is found in hi h concentration in the pulmonary circulation, promotes this fibrinolytic effect. "ibrinolytic a ents have been administered to increase the rate of lysis after pulmonary embolism/ Tissue p"asmino#en acti&ato$ 0t-PA1 wor.s more rapidly than u$o2inase3 but both are costly and are associated with a hi h incidence of bleedin

complications. -ndications for fibrinolytic treatment include any critically ill patient with a pulmonary embolus. 8leedin complications can be reduced by ta.in a careful neurolo ic history to eliminate patients with any brain patholo y, minimi6in venous and arterial punctures, stoppin heparin administration, and identifyin any other potential causes of bleedin . 'he advanta e of thrombolytic therapy may well be to improve the ultimate resolution of ma/or thromboembolism, as demonstrated by +harma and associates. 'heir follow% up studies in patients treated with uro.inase or strepto.inase showed a better restoration of pulmonary%capillary blood volume and diffusin capacity at # wee.s than in patients treated with heparin and anticoa ulants alone. 'he reason for the continued improvement at $ year was not clear but was believed to be related to more complete early resolution of the embolic condition, allowin more effective natural lytic processes, or to more complete clearance of peripheral venous thrombi, preventin silent recurrent embolism. 'herefore, the patient who is not in shoc. and who has no clear contraindication should be treated with a fibrinolytic a ent. Vena Ca&a" !nte$$uption -n some patients, anticoa ulants cannot be used because of associated problems !e. ., peptic ulcer disease&, and mana ement must be directed toward a mechanical means of protection a ainst recurrent embolism as outlined earlier !see 'able #$%B&. Other patients, in whom anticoa ulation appears to be adeAuate, sustain recurrent embolism and become candidates for sur ical intervention. 'he third indication is when there has been a complication of anticoa ulant therapy, forcin it to be discontinued and leavin the patient with untreated >,'. 7nother indication for a vena caval filter is protection a ainst recurrent embolism in a patient who has sustained massive pulmonary embolism reAuirin open or catheter embolectomy. -n these patients, in spite of a satisfactory embolectomy of the pulmonary circulation, the ori inal focus of venous thrombosis remains untreated and recurrent embolism is li.ely. 'here are two additional relative indications for a vena caval filter in a patient with active or recent >,'. One is the hi h% ris. patient over 31 years of a e who is obese and has a serious associated medical illness !e. ., heart disease&, mali nant disease, or a history of previous embolism and who under oes a ma/or abdominal or vascular procedure. 'he final relative indication is the patient in whom 31 to (1 percent of the vascular bed has been occluded !ma/or& and who would most li.ely not be able to tolerate additional emboli, particularly if there is associated cardiac or pulmonary disease. Pu"mona$y Em'o"ectomy 'he direct sur ical approach to pulmonary embolism can be traced bac. to 'rendelenbur !$=10&, who demonstrated the feasibility of pulmonary embolectomy experimentally but had no successes clinically. -t remained for his pupil Kirschner !$=#3& to confirm the possibility of embolectomy by a successful clinical outcome. 8ecause this procedure was attempted without circulatory support usin a direct approach to the pulmonary artery at thoracotomy, the number of survivors was very small, and the first successful case in the Enited +tates was not reported until $=(0 by +teenbur . 'he first successful open embolectomy durin cardiopulmonary bypass was reported by +harp in $=<#. +ince then, partial bypass support has also been used for the patient in shoc.. Local anesthesia is used, and the femoral artery and vein are

cannulated for venoarterial bypass. 'he eAuipment is fully portable !"i . #$%$(&, and patients can be supported durin pulmonary arterio raphy and then transported to the operatin room, where they can tolerate eneral anesthesia and sternotomy much better while bein maintained on partial cardiopulmonary bypass. 5mer ency pulmonary embolectomy rarely is indicated but should be considered in any patient with an acute pulmonary embolism who appears preterminal. 'hese patients rarely survive the trip to the an io raphy suite and operatin room, but when they do, the operation is probably not necessary if fibrinolytic therapy is available. >ocumentation of the dia nosis of massive pulmonary embolism by pulmonary arterio raphy is mandatory because the clinical dia nosis often is incorrect. 'he initial approach to patients who have transient collapse or persistent systemic hypotension should include full heparini6ation and administration of inotropic dru s, if necessary, to support the circulation while the dia nosis is confirmed. -soproterenol !3 m in $111 mL of (J dextrose in water& is useful initially because of its bronchodilatin and vasodilatin effects and its positive inotropic cardiac effect. -t may provo.e arrhythmias, however, necessitatin the use of dopamine. -n the patient who responds to heparin and does not reAuire vasopressors for systemic pressure or urine output, careful monitorin is essential to determine whether anticoa ulation and fibrinolysis will control the disorder. Open pulmonary embolectomy carries a mortality rate in the ran e of (1 percent, however, and uncontrollable pulmonary hemorrha e may follow open restoration of pulmonary perfusion. 7n alternative approach usin local anesthesia has been su ested by Greenfield and associates for transvenous removal of pulmonary emboli. 7 cup device attached to a steerable catheter is inserted in the /u ular or the femoral vein, and the cup is positioned under fluoroscopy ad/acent to the embolus seen on arterio raphy !"i . #$% $<&. 'he position is verified by in/ection of contrast medium throu h the catheter. 'hen syrin e suction is applied to aspirate the embolus into the cup, where it is held by suction vacuum as the catheter and captured embolus are withdrawn. 9linical experience with the techniAue in B# patients demonstrated that emboli could be extracted in #= of them !=$ percent& with an overall survival rate of D< percent. 5mboli could not be removed when they had been impacted for more than D# h or if the patient suffered cardiac arrest at the time of an io raphy, in which case open embolectomy was reAuired. *lacement of a Greenfield vena caval filter after removal of sufficient emboli to produce near% normal hemodynamics protected the patients from recurrent embolism. Pu"mona$y Hype$tension and Th$om'oem'o"ism *ulmonary emboli may accumulate radually over a prolon ed period if they fail to under o lysis and obliterate the pulmonary vascular bed. 'he clinical picture in this case is one of chronic cor pulmonale because si nificant pulmonary hypertension results from chan es in the pulmonary vascular bed. 'he presentation may be subtle with only dyspnea or syncope on exertion, but there is a loud *# and ri ht%sided strain on the electrocardio ram. 'he seAuence also may occur unaccompanied by si nificant respiratory symptoms, and this may explain the cause in some of the patients considered to have primary pulmonary hypertension. ;hen the dia nosis is made, there is limited life expectancy, but the patient may benefit from a vena caval filter to prevent additional embolism even if the disorder is primary pulmonary hypertension, as reported by Greenfield and associates. 'he rationale for this is that they ultimately

develop ri ht heart failure, predisposin to pulmonary embolism that is lethal even if small. ;hen acute cardiopulmonary decompensation occurs in these patients after embolism, they are not ood candidates for embolectomy because of fixation of the older thrombi to the pulmonary arterial wall. 'hey should be classified separately !chronic& and mana ed by lon %term anticoa ulation therapy, or in some cases should be considered for open pulmonary thromboendarterectomy or heart%lun transplantation. Recurrent thromboembolic pulmonary hypertension produces exertional dyspnea and si ns of ri ht heart strain with cor pulmonale. ;ith further pro ression of ri ht heart overload, tricuspid insufficiency may develop. 'his disorder may be difficult to distin uish from primary pulmonary hypertension, althou h the latter is more li.ely to be found in women under #1 years of a e without a history of >,'. +evere pulmonary hypertension is a serious problem and usually limits the life expectancy to less than # years from dia nosis. Open thrombectomy for chronic occlusion was first performed by 7llison and associates in $=(0 and remains a possibility for improvin pulmonary blood flow. "or a patient to be eli ible for this procedure, the occlusion must involve the proximal portion of the pulmonary arterial tree, and the distal bed must be patent. 'he physiolo ic basis for continued distal patency after proximal occlusion is bronchial arterial collateral flow. 'he procedure also has a si nificant mortality, but this has been decreasin with reater experience and identification of ris. factors. >aily and associates performed pulmonary thromboendarterectomy on $#D patients under deep hypothermic circulatory arrest with a mortality rate of $#.< percent. "or the ma/ority of patients with severe pulmonary hypertension, however, the outloo. is poor unless they receive maximum protection from recurrent embolism, which in the authors2 experience has reAuired anticoa ulation therapy and vena caval filter placement. "i . #$%$D presents an al orithm for the mana ement of pulmonary embolism. Va$icose Veins ,aricose veins are the most common vascular disorder affectin human bein s. 7 definition of a varicose vein that lays the roundwor. for a unified theory of causation was iven by 9arl 7rnoldi, who described them as ?any dilated, elon ated, or tortuous vein, irrespective of si6e.@ Etio"o#y 'here are four factors that affect the development and pro ression of varicose veins: heredity, female sex hormones, ravitational hydrostatic force, and hydrodynamic muscular compartment forces. 7 familial tendency toward the development of varicosities may be the most important predisposin factor. "emale sex hormones also have a profound effect on the lower extremity superficial veins. ,aricose veins are common occurrences in pre nancy, usually appearin in the first trimester !D1 to 01 percent& when the corpus luteum is secretin pro esterone. *ro esterone is .nown to inhibit smooth muscle contractility and increase venous distensibility. 'hese effects are maximal on the first day of the menstrual cycle, when the effects of pro esterone are amplified by estro en. )ydrostatic forces produce venous dilatation from the wei ht of the blood column transmitted throu h incompetent valves. 'he other force

is exerted by the contractin muscles on ad/acent veins via the perforatin system. 'hese forces re ularly exceed $(1 mm) . C"inica" )ani%estations 'he usual distribution of varices is below the .nee in branches of the reater saphenous system !"i . #$%$0&. 'he symptoms associated with varicose veins are nonspecific achin and heaviness of the le s that can be attributed to the con estion and poolin of blood in the enlar ed superficial venous system. 'hese symptoms worsen as the day pro resses, reAuirin the patient to rest with le elevation to obtain relief. 9alf%len th elastic stoc.in support in the ran e of #1 to B1 mm) may provide symptomatic relief for those whose vocations reAuire lon periods of standin or sittin . 7lthou h mild edema may occur from varicosities alone, it usually reflects additional incompetence of the deep or perforatin venous system and other medical conditions, such as cardiac or renal failure. 7ssociated ni ht crampin of the le s may be helped by the administration of Auinine sulfate, which reduces muscular irritability. Dia#nosis 'he 'rendelenbur test is useful in distin uishin between primary varicose veins and the more serious condition of varicosities secondary to underlyin deep venous disease. -n the 'rendelenbur test the limb is elevated to evacuate the veins, then pressure by hand or tourniAuet is applied to the saphenofemoral /unction !"i . #$%$=&. ;ith the patient standin , the lower le is observed for the rate of fillin of the varicosities. Gradual fillin occurs in normal patients when the perforatin veins are competent. Rapid fillin occurs if the perforators are incompetent. 'he second phase of the test consists of release of the pressure to see if the upper thi h varices fill rapidly, indicatin incompetence of the saphenofemoral valve. 'here are four possible results of this test. 7 ne ative%ne ative result occurs when there is only radual fillin in the distal one%third of the le with compression in place and only continued slow fillin when the compression is released. 'his indicates that the valves of the perforatin veins !phase -& and superficial veins !phase --& are competent. -n a ne ative%positive result, the release of compression is followed by a rapid fillin of the saphenous vein, indicatin that its valves are incompetent. 7 positive%ne ative result indicates that the perforatin veins are incompetent, but the superficial veins are competent. 7 positive%positive result indicates that both systems have valvular incompetence. 'hese principles have been refined by the use of color%flow duplex scannin . 9han es in the direction of flow are detected by chan es in color, and venous valves may be seen on the rey% scale ima e. -n the *erthes test, a tourniAuet is placed around the upper le and the patient is instructed to wal.. -f the varicose veins disappear, the deep venous system is patent and the perforatin veins are competent. -f pain occurs with wal.in , the deep system is obstructed and the superficial system represents the ma/or source of venous outflow. -t would be a serious error to excise superficial veins under these circumstances. +eAuential tourniAuets also may be used to define and isolate areas of incompetent perforatin veins !Ochsner%Mahorner test&. T$eatment 'he ma/ority of patients can be mana ed by conservative methods, but if these fail to control symptoms or if additional complications of venous stasis develop, such as

dermatitis, bleedin , thrombosis, or superficial ulceration, the patient may become a candidate for more a ressive mana ement. 'he two methods of treatment currently used are in/ection sclerotherapy and ablative sur ery. +clerotherapy should be reserved for those patients who do not have evidence of axial saphenous reflux. Lar e varices of the thi h also should be removed sur ically because they are sub/ect to superficial thrombophlebitis after sclerotherapy and are often associated with lar e perforatin veins. -n/ection sclerotherapy destroys the endothelium of the vein and promotes its obliteration by scarrin . *ressure must be applied to the vein after in/ection of the sclerosant to prevent thrombus formation and later recanali6ation. 'he techniAue for in/ection involves placement of the needle and syrin e with the patient standin followed by elevation of the le , in/ection of the a ent, and banda e compression of the area for # to B wee.s. +odium chloride #B.3J is the a ent most prefer, but a wide selection is available. 9urrent indications for sclerotherapy include superficial venules !H$ mm&, varicosities $ to B mm in diameter, postoperative residual veins, small con enital vascular malformations of venous predominance, bleedin varices, and lar e varices around an ulcer. 'he most common complications include hyperpi mentation, s.in necrosis, pain, anaphylaxis, and mattin . 'he oals of operative treatment are the elimination of the hydrostatic forces of saphenous reflux, the removal the hydrodynamic forces of perforatin vein reflux, and the eradication of the varicosities in as cosmetic a manner as possible. 5ach case must be planned thorou hly because routine strippin of the reater saphenous vein from roin to an.le usually is not reAuired. *atients with saphenous reflux should have roin%to%.nee strippin . -t is unnecessary to strip the below%the%.nee portion of the vein unless it is varicose. +tab avulsion of vein clusters, which are mar.ed preoperatively, supplements the strippin . Removal of the reater saphenous vein reAuires its detachment from the common femoral vein and li ation of its tributaries at the saphenofemoral /unction !"i . #$%#1&. -f an an.le incision is made, care must be ta.en to avoid in/ury to the saphenous nerve. 8leedin can be reduced by the use of a tourniAuet and le elevation durin the strippin . 'he incisions made for stab avulsion of varices are $ to # mm in len th and are oriented in the s.in lines. 5cchymosis is the most common complication after operations for varicose veins. 'he incidence can be reduced by carefully placed elastic support. Recurrences are usually due to incompetence at the roin or in the midthi h from perforatin veins. Ch$onic Venous !nsu%%iciency 9hronic venous insufficiency or the postthrombotic syndrome develops in approximately (1 percent of the patients with deep venous thrombosis. -t is estimated that there are (11,111 patients in the Enited +tates with venous ulcers. )omans noted in $=$D that ?overstretchin of the vein walls and destruction of the valves upon which the mechanism principally depends brin about a de ree of surface stasis which obviously interferes with the nutrition of the s.in and subcutaneous tissues.@ -t is now .nown that recanali6ation of the deep veins results in valvular incompetence, which in turn results in a lon column of blood that transmits pressures of over $11 mm) to the venules, causin the development of abnormal capillaries. 'hese new vessels have an increased permeability to fibrino en and red blood cells. Lymphocyte and

macropha e recruitment occurs in response to extravasated protein. *ericapillary cuffin , an attempt by the endothelial cells to limit extravasation, occurs and results in widened endothelial ap /unctions. 'he result is thic.enin and lipodermatosclerosis of the subcutaneous tissues that produce a characteristic ?brawny@ edema. 'he loss of red cells results in hemosiderin deposits, producin the characteristic pi mentation. 7 deterioration of mononuclear cell function is associated with chronic venous insufficiency. 7 decreased capacity for lymphocyte and monocyte proliferation in response to various challen es translates into poor or prolon ed wound healin . ;hen the distal perforatin veins become incompetent, there is additional pressure, with s.in atrophy leadin ultimately to necrosis and chronic stasis ulceration !"i . #$%#$&. Dia#nosis 'he dia nosis of chronic venous insufficiency usually is made by inspectin the le . *hysical findin s do not provide information about the presence, extent, or location of valvular incompetence or obstruction. >uplex scannin is the most reliable method of identifyin valvular incompetence and venous obstruction. ,enous valvular incompetence is identified easily with the duplex scanner. 'he vein to be studied is identified with 8%mode ima in . ;hile the velocity spectrum is displayed, various maneuvers are performed to reverse the normal peripheral%to% central radient. Retro rade flow is indicated by an inverted spectrum and a chan e in color from blue to red. Reflux is evaluated in the roin by havin the patient perform a ,alsalva maneuver. 7 period of reversed flow exceedin $.( s is considered abnormal. Manual compression is used above and below the vein in Auestion. +ome prefer to examine the veins for reflux while the patient is standin . 7 pneumatic cuff is placed at various levels be innin at ( cm below the vein in Auestion. 'he velocity spectrum is recorded continuously as the cuff is inflated and deflated. 4ormal valves close rapidly in response to temporary flow reversal. *erforatin veins are studied with the patient in the reversed 'rendelenbur position. *erforators are identified as veins arisin from the superficial veins and penetratin the deep fascia into the muscular compartment. Outward flow with calf compression indicates valvular incompetence. ,enous valves are identified with 8%mode ima in . 4ormal valves are thin and mobile: diseased valves are shortened and thic., often with attached echo enic material. ,enous obstruction is identified with the same techniAues used to dia nosis acute venous thrombosis. 7fter the vein is identified with 8% mode ima in , its patency is assessed by its compressibility and >oppler spectrum. ;ith partial occlusion or incomplete recanali6ation, there will be an encroachment on the flow ima e that no lon er fills the entire vein. 9ollateral veins will be seen, and that findin is particularly useful in distin uishin between acute versus chronic occlusions. 7nother distin uishin feature of chronic occlusion is the shrun.en si6e of the vein when compared to the distention seen in acute >,'. 'he physiolo ic response to venous reflux can be measured. 7 needle is placed in a dorsal foot vein and secured in place. 'he patient2s venous pressures can be determined in the restin and active state. 'he pressure in the standin position is sli htly hi her than the hydrostatic force of a column extendin from the atrium to the foot. Restin pressures in patients with and without venous insufficiency are similar. -n contrast to normal patients, who reduce their distal venous pressure with wal.in ,

patients with the postthrombotic syndrome ain no benefit from their muscle pump and their pressure increase !"i . #$%##&. -f there has been failure of recanali6ation with persistent obstruction, the increase in blood flow with exercise may increase venous hypertension to produce ischemic pain referred to as venous claudication. Nonope$ati&e )ana#ement 'he oals of treatment are to alleviate symptoms, heal ulcerations, and prevent ulcer recurrences. 'he vast ma/ority of patients can be mana ed nonoperatively. 7lthou h the mechanism of benefit is un.nown, compression therapy is the most important aspect of patient mana ement. 9onflictin hypotheses include a reduction in ambulatory venous pressure, improvements of the microcirculation of the s.in and subcutaneous tissue, and increase in the pressure of the subcutaneous tissue that reduces the lea.a e of fluid from the capillaries. 'he latter is the most plausible because cutaneous metabolism may improve after fluid resorption, allowin an enhanced diffusion of oxy en and other nutrients. 'he initial treatment of patients with venous ulceration should include a period of strict bed rest to reduce edema. +ystemic antibiotics are iven for the surroundin cellulitis. 5lastic stoc.in s are fitted when the edema has subsided. +urroundin areas of dermatitis are treated with topical steroids. *atients are then instructed to wear the elastic stoc.in s for life. 'wo pairs are prescribed to allow for daily launderin of alternate pairs. Elcer recurrence is $< percent in compliant patients, but lon % term compliance is difficult to achieve with patients who are reluctant to wear the stoc.in s after their ulcer is healed. +ome physicians prefer the paste au6e boot !the Enna boot& durin the ulcer healin phase. 'his dressin contains calamine, 6inc oxide, lycerin, sorbitol, elatin, and ma nesium aluminum silicate. *atients whose ulcers fail to heal after prolon ed outpatient care reAuire hospitali6ation. Ope$ati&e )ana#ement *atients selected for operation have severe, disablin symptoms and a history of recurrent ulceration despite a ressive medical therapy. 9andidates for operation should under o ascendin and descendin veno raphy in addition to duplex scans and ambulatory venous pressures. 'hese tests provide data allowin an individuali6ed treatment plan that addresses specific areas of obstruction or reflux. Pe$%o$ato$ Vein Li#ation )ealin of chronic stasis ulcers is not li.ely unless the perforatin veins responsible for the ulcer are identified and li ated. 'he typical location of these veins is posterior and superior to the medial malleolus. Li ation of the perforator vessels should be the initial procedure for recurrent ulceration. 'reatment failure occurs in $1 percent of patients despite vi orous medical therapy, includin support stoc.in s, le elevation, wound care, and patient education. 'hese patients should be considered for venous reconstruction. Venous Reconst$uction 'he present attitude of most sur eons toward venous reconstruction is critical and pessimistic. 'he venous system, unli.e the arterial system, tends to recanali6e, thus ma.in it more difficult to Auantitate the obstruction and identify the patient who may benefit from venous reconstruction. >ale estimated that the percenta e of patients

with chronic venous insufficiency who could benefit from reconstruction was $ to # percent. *rimary valvular dysfunction can be treated by valvuloplasty. 'he valve most suitable to direct valve repair usually is the most proximal valve in the superficial femoral vein. 7fter >,', most patients have scarred and thic.ened valves that do not lend themselves to this type of reconstruction. Kistner, after studyin #11 limbs with ascendin and descendin veno raphy, found #0 that could be treated by valve repair, and D# percent had an excellent result. -n this procedure, floppy, incompetent valves are tethered a ainst the vein wall or shortened usin interrupted 0%1 monofilament suture !"i . #$%#B&. Recent technical advances have allowed this procedure to be done under direct visuali6ation usin an an ioscope. Results of this procedure are difficult to interpret because the operations are often combined with saphenous vein strippin and perforator li ation. Most investi ators have reported improvement in symptoms for prolon ed periods in approximately <1 to 01 percent of the patients. >irect repair is not possible for postthrombotic valvular dysfunction. 'he two reco ni6ed options for sur ical candidates are transposition of a deep femoral or saphenous vein valve or transplantation of a valve%bearin se ment of the axillary vein to the superficial femoral or popliteal vein !"i . #$%#3&. Results are not as ood as those achieved by valvuloplasty for primary incompetence. 'aheri and cowor.ers described << patients with ood results in D0 percent. -n this series, B$ patients had postoperative veno rams, #0 of these were found to have valvular competence. 7 number of other investi ators report symptomatic relief in (1 to =# percent and ulcer recurrence in < to (3 percent of patients. Most of these patients had ood results initially: however, at $ year, a hi h proportion of the affected limbs had reverted to their preoperative condition. 8er an and collea ues have pointed out that for venous valve sur ery to be successful, it usually must be accompanied by saphenous vein strippin and perforator li ation. 'he difficulty in identifyin patients who could benefit from these procedures was put into perspective by >ale, who, after # years of investi atin , failed to identify a roup of patients who would benefit from venous valve transplantation or valvuloplasty. )usni found that venous reconstruction fails in three situations: when the bypass raft is too small in caliber, when venous hypertension is mild to moderate !less than 01 percent of the standin venous pressure&, and when a thrombectomy or endophlebectomy has to be performed before anastomosis. -n these patients who are at hi h ris. for failure, he has recommended a distal arteriovenous fistula. 7pproximately one%third of patients with chronic venous insufficiency have a predominant obstructive component because of inadeAuate recanali6ation after a >,'. *atients typically complain of swellin and pain on ambulation. 'he pain often is described as burstin , but patients with valvular incompetence refer to their pain as achin . 8ecause the pain of chronic venous obstruction reAuires the patient to be off his or her feet to obtain relief, it is referred to as venous claudication. 7mbulatory venous pressure measurements document the dia nosis, and veno raphy identifies the site of obstruction. ,enous obstruction of the iliofemoral venous system can be bypassed by a saphenous vein cross%over raft, first described by *alma and 5speron in $=(0. 'he procedure consists of isolatin the normal contralateral saphenous vein and dividin it distally.

'he vein is tunneled suprapubically and anastomosed to the contralateral femoral vein, distal to its obstruction. -n $=0# >ale described (= patients who had the *alma bypass with excellent results in <B percent, ood results in $D percent, and a failure rate of #1 percent. )usni, in $=0B, and +mith and 'rimble, in $=DD, reported similar results. 'he saphenous vein cross% over raft enerally has been accepted as useful: however, the natural history of iliac vein occlusion is recanali6ation, and very few patients with iliofemoral thrombosis become candidates for sur ery. Ese of the saphenous vein for popliteal%to%femoral vein bypass was described in $=(3 by ;arren and 'hayer with ood results in $1 of $3 patients. 'his operation provides the muscle pump system a means of emptyin the calf by bypassin the occluded superficial femoral vein. ;ith rich collateral veins in the thi h, identifyin the patient with an obstructed superficial femoral vein who mi ht benefit from the saphenous%to% popliteal vein bypass is difficult. 'he saphenous vein is dissected free below the .nee and anastomosed to the popliteal vein, which is obstructed proximally. )usni has populari6ed this procedure and has reported the outcome in #D patients with a ood result in <B percent. >ale reported ood results in $1 patients !<1 percent&, and +mith and 'rimble, in a collected series of (= patients, reported ood results in D< percent. 'he ma/ority of iliofemoral thromboses occur on the left side. 'his is attributed to the ri ht iliac artery compressin the left iliac vein as it crosses the fifth lumbar vertebra. ,arious autopsy series and operative studies have documented the presence of left iliac vein webs and scarrin in patients who have had iliofemoral thrombosis. 'here was interest in this problem in the $=<1s by 9alnan and associates and by 9oc.ett and 'homas, who advocated sur ical correction of these lesions. >ale reviewed ei ht such patients identified by veno raphy and subseAuently operated on four, trimmin out anterior webs or scar tissue and usin a venous patch for closure. 'wo of the patients had excellent results: edema developed later in one patient, and a fourth patient had a complicated postoperative course, complainin of excruciatin pain and postoperative swellin . >ale recommended operation only for the patient whose symptoms are severe and who accepts the operation with the understandin that the results are not predictable. +mith and 'rimble have followed B1 patients with this problem and have operated on $3, with an 0( percent postoperative improvement rate. 9oc.ett and 'homas, conversely, found the results unsatisfactory, and after operatin on B1 patients usin several different methods they recommended abandonin the procedure. Venous T$auma ,enous in/uries of the extremities usually are associated with arterial in/uries because of their anatomic proximity. >irect li ation of in/ured superficial veins is appropriate treatment except when they are the sole remainin venous draina e of the extremity, which mandates their repair. 'reatment of in/uries of the deep veins chan ed dramatically as a result of the military experience in Korea and ,ietnam, as reported by Rich and associates. -t was well demonstrated that li ation of ma/or extremity veins resulted in hi her rates of disability and limb loss than repair or replacement by auto enous vein se ments. 'he concept of primary repair of venous in/uries by suture vein patch or vein raft interposition has been extended to civilian in/uries by 7 arwal and associates with favorable results. 'he rationale for primary repair is based in part on the adverse hemodynamic effects in the first D# h after ma/or venous li ation. 9urrent recommendations are that repair of the common femoral and

popliteal veins should be done whenever possible. Repair of the superficial femoral vein is controversial.

L4)PHAT!C AND L4)PHEDE)A

Anatomy and Physio"o#y 'he exact ori in of lymphatic vessels is a matter of disa reement amon embryolo ists. 'he ori inal theory of +abin traced the ori in from the venous system, while )untin ton and Mc9lure su ested that lymphatics form by fusion of mesenchymal spaces or clefts, which has been labeled the centripetal theory. 8y the sixth wee. of estation, there are paired lymph sacs in the nec. and lumbar areas, and at the ei hth wee., there is a retroperitoneal lymph sac with a developin cisterna chyli. 'hese systems develop communicatin channels that ultimately form the thoracic duct by mer er of the ri ht lymphatic duct with the left across the fourth to sixth thoracic vertebrae, which then drains into the left subclavian vein. +maller lymphatic ducts persist that drain into the ri ht subclavian vein. >evelopmental arrest or abnormalities can result in primary hypoplasia or absence of ducts and lymph nodes. 7bnormal rowth of /u ular lymph sacs can produce unilocular or multilocular lymph cysts termed cystic hy romas. 'hese cysts also may be found in the axilla, mediastinum, retroperitoneum, or intestinal mesentery. )yperplastic chan es may occur to produce lymphan iomas with or without other vascular malformations. 'he function of the lymphatic system be ins with lymphatic capillaries, which collect fluid and protein from the extravascular spaces. -n addition to the protein that cannot be reabsorbed by the venules, red blood cells, bacteria, and other lar er particles can be evacuated only throu h the lymphatics. 'his permeability is facilitated by the absence of a basement membrane beneath the lymphatic endothelial cells. 'he lymphatic capillaries are found beneath the epidermis in the superficial dermis. 'hese vessels drain into valved channels in the deep dermis and subdermal tissues, formin lar er channels that follow the vascular pathways superficial to the deep fascia. 7lthou h lymphatics can be found in the intermuscular fascia, they are absent in muscles, tendon, cartila e, brain, and cornea. Lymph is transported by afferent vessels to re ional lymph nodes that vary in si6e accordin to their function and activity. ;ithin the medullary sinuses of the node, circulatin lymphocytes are replaced and initial contact of forei n material with the immune system is made. 5fferent lymph leaves the node via hilar channels, which are less numerous than the afferent channels that enter the convex side of the node. -n addition to direct thoracic duct draina e into the subclavian vein, there are other lymphovenous communications within nodes and in peripheral vessels. 9entral lymphatic flow is promoted by the lymphatic valves, muscular contractions in lar er ducts, respiration, arterial pulsation, and external massa e. 'he main function of the lymphatic system is to clear the interstitial spaces of excess water and particulate matter. Lymphedema 9lassification of Lymphedema

'he ori inal classification of 7llen was into two types, one in which there was no .nown cause, and the other secondary to a .nown disease or disorder. 'he primary lymphedemas were called con enital when present at birth and praecox when there was onset in childhood. Kinmonth added the term tarda for when the onset was not until later life. ;ith the advent of lympho raphy it became possible to classify the primary lymphedemas structurally into hyperplasias and hypoplasias. 'he ori inal classification as proposed by Kinmonth has lar ely been abandoned for the more simplified version presented in 'able #$%<. 'he con enital lymphedemas are hypoplastic in =# percent of cases. 'heir sub roups are defined by lympho raphy and behave differently. 'hose with distal hypoplasia have a mild, nonpro ressive form of the disorder provided that their proximal pathways are normal. Most of these patients are women and notice the onset after puberty. -n proximal hypoplasia, the lymphedema is more extensive, involvin the entire extremity, and it occurs eAually amon males and females. 'he combination of proximal and distal hypoplasia shows features of both roups and tends to be pro ressive. 'he primary hyperplastic lymphedemas are uncommon !0 percent&, and those with bilateral hyperplasia usually can be reco ni6ed by diffuse capillary an iomata on the lateral sides of the feet. Lympho raphy shows dilated lymphatics with normal valves, in contrast to the findin s in the me alymphatic roup, in which no valves can be seen. -n this latter roup, chylous reflux may produce chylometrorrhea, s.in vesicles, or chyluria. 'he most common cause of secondary lymphedema in this country is mali nant disease metastatic to lymph nodes. +ur ical removal of nodes, especially when combined with radiation therapy that produces lymphatic fibrosis, is another common cause. -n tropical and subtropical countries, filariasis is the most common cause of secondary lymphedema, producin the typical appearance of elephantiasis. Other infective and chemical a ents, such as silica, can enter the lymphatic system via barefoot wal.in and cause fibrosis of lymphatics and lymph nodes. 9linical Manifestations Lymphedema is a clinical dia nosis and should be restricted to situations where other causes of edema have been excluded or a specific lymphatic abnormality has been demonstrated. 'he presence of bilateral dependent ?pittin @ edema usually indicates a renal or cardiac etiolo y. Other enerali6ed hypoproteinemias may be seen in malnutrition, cirrhosis, and protein%losin enteropathy, or they may be idiopathic. 7ller ies or hereditary causes are unusual. -n unilateral edema, venous disease is the most li.ely cause and can be reco ni6ed by the examinations described in the previous section. 'he patient with lymphedema complains of swellin and fati ue. Limb si6e increases durin the day and decreases at ni ht but is never normal. -t is important to determine whether there is a family history of primary lymphedema and whether the patient has visited any countries where filariasis is endemic. 'he presence of wei ht loss and diarrhea su ests small bowel lymphan iectasia. On examination, lymphedema is characteristically firm and rubbery but nonpittin . Lymph vesicles may be present containin fluid of hi h protein concentration. 9omplications of lymphedema such as

infection, cellulitis, erythema, and hyper.eratosis may be present. -t is important to document limb si6e to identify isolated limb i antism and the Klippel%'rKnaunay syndrome that may have hypoplastic lymphatics in addition to venous abnormalities, capillary nevus, and limb elon ation. 'he patient should be examined for upper extremity and enital lymphedema, hydroceles, and amelo enesis imperfecta. >ia nostic +tudies >ye -n/ection Lymphatics can be visuali6ed by dye in/ection in the extremities and mesentery, and also by in estion of cream or mil. to visuali6e intestinal lacteals and ma/or ducts. 7 hi hly diffusible dye such as patent blue, introduced by )udac. and McMaster, or s.y blue dye, recommended by 8utcher and )oover, can be in/ected in 1.# mL amounts subcutaneously into each interdi ital web. Massa e of the s.in and movement of the /oints usually defines a networ. of fine intradermal lymphatics !"i . #$%#(&. -f the collectin vessels are obstructed or inadeAuate, the dye diffuses throu h the dermal lymphatics to produce a marbled appearance called ?dermal bac.flow.@ Lympho raphy 'he techniAue of lympho raphy was developed by Kinmonth, who demonstrated that it was possible to cannulate the lymphatics visuali6ed by dye in/ection and then in/ect contrast medium !Lipiodol&. 'his is a meticulous and tedious procedure that may reAuire eneral anesthesia, as proposed by Kinmonth. -f the lymphatics in the foot are not usable, it is possible to cannulate lymphatics ad/acent to roin nodes or to in/ect the node directly. ;ith adeAuate visuali6ation, the lymphatics in the extremity will be identified, often as parallel trac.s that are of uniform si6e and bifurcate as they proceed proximally in contrast to the venous system. 4ormally, there is some dilatation at the level of the valves. Radionuclide Lymphatic 9learance Radionuclide scannin usin human serum albumin labeled with radioactive iodine or technetium ==m colloid has been used to monitor lymphatic clearance by serial scannin . 7lthou h the techniAue is simpler than standard lympho raphy, it has ma/or disadvanta es because of the ha6iness of the scan, radiation dosa e, and distribution of the radionuclide into the extracellular fluid, ma.in calculations of clearance dependent on le volume. 7nalysis of 'issue "luid 'issue fluid or lymph can be aspirated or collected from a tube in the subcutaneous tissues but contributes little to the dia nosis of lymphedema. 9haracteristically, lymphedema fluid has a protein content of more than $.( CdL, in contrast to that of edema fluid from venous hypertension, which usually is less. 'he ratio of albumin to lobulin also is hi her in lymphedema fluid than in plasma, which is helpful in the presence of an inflammatory exudate in which the protein content is hi h but the albumin%to% lobulin ratio is normal. Mana ement +upportive 'reatment 'here are si nificant anatomic and physiolo ic limitations to the treatment of lymphedema. "rom the standpoint of physiolo y, the removal of fluid is not as

effective as in edema of other causes because of the residual protein in lymphedema. -n addition, from an anatomic standpoint, the development of fibrosis produces irreversible chan es in the subcutaneous tissues. 'herefore, the options are limited and the primary ob/ectives remain for control of edema, maintenance of healthy s.in, and avoidance of the complications of cellulitis and lymphan itis. 'he initial ob/ective of control of edema can be approached by elevation and the use of seAuential pneumatic compression boots to massa e the le . 'hese treatments can be done at home with eAuipment rented for this purpose. Once the le has reached optimal si6e, the patient should be fitted with firm elastic stoc.in s as described earlier for venous insufficiency. 'he stoc.in s should be removed at ni ht and the foot of the bed elevated to maintain the pressure radient from le to ri ht atrium. Massa e therapy was used in the treatment of lymphedema as early as $00# and has current advocates. 'he onset of redness, pain, and swellin usually si nifies early cellulitis or lymphan itis, which can be reco ni6ed by red strea.in up the le . 'he usual causative or anism is staphylococcus or beta%hemolytic streptococcus, which must be treated vi orously, usually with intravenous antibiotics. -n the absence of treatment, the infection may obliterate more lymphatics and produce constitutional si ns of fever, malaise, nausea, and vomitin . 7nother freAuent complication is ec6ema, which usually will respond to hydrocortisone cream. 7ntifun al a ents may be necessary, topically and systemically, for chronic infections, particularly between the toes. -n contrast to the stasis edema of venous insufficiency, ulceration is unusual, althou h fissures and lymph fistulas can develop and reAuire sur ical excision. 'he secondary lymphedemas may lend themselves to treatment of the underlyin disorder, such as usin diethylcarbama6ine for filariasis or appropriate antibiotics for tuberculosis or lympho ranuloma venereum. -n rare cases of lon %standin secondary lymphedema, such as in the arm after radical mastectomy, a lymphan iosarcoma may develop, appearin as a raised blue or reddish nodule. +atellite tumors and early metastases may develop if it is not reco ni6ed and widely excised. Operative 'reatment Only $( percent of patients with primary lymphedema become candidates for operative treatment, which usually is directed to reducin le si6e. 'he indications for operation are related to functional rather than cosmetic improvement, because the appearance of the extremity even after a successful procedure will still be abnormal and show extensive scarrin . 'he best results are obtained when the bul. of the extremity has severely impaired movement or when there have been recurrent attac.s of cellulitis. 7lthou h some efforts have been made to develop techniAues to improve lymphatic draina e, most of the established procedures consist of excisional operations. 'hree of the excisional procedures were based on the incorrect assumption that the deep fascia acted as a barrier to lymphatic draina e, and the efforts of Kondoleon and associates to excise fascia or insert a dermal flap into muscle proved ineffective in improvin lymphatic draina e. 'he ori inal procedure devised by 9harles consistin of wide excision of lymphedematous tissue followed by s.in raftin still is useful when the overlyin s.in is in poor condition, as in elephantiasis. 'he procedure used

most often, however, is Kinmonth2s modification of )omans2s procedure, in which s.in flaps are raised to allow excision of the underlyin subcutaneous tissues. 'he most lo ical, albeit technically demandin , approach has been directed to establishin lymphaticovenous anastomoses. -nitial efforts in this area were made by 4ielubowic6 and Ols6ews.i, who divided a lymph node, removed the pulp under ma nification, and sutured the node capsule with its afferent lymphatics into a vein. 'his procedure is more suitable for secondary lymphedema than primary, in which the disorder lies in the lymphatic channels themselves. 7nother promisin techniAue of direct lymphovenous connection was developed by 9ordeiro and modified by >e ni, who used a special needle for insertion of lymphatic vessels directly into veins and fixed them there by a sin le suture. Esin this techniAue, "ox and associates treated ei ht secondary and $# primary lymphedema patients, with follow%up as lon as 3 years. Good results were obtained in two of four postmastectomy lymphedemas, with poor results in the other two, who had postoperative lymphan itis. 4ine of $$ patients with primary lymphedemas had ood functional results, allowin them to resume normal activity. 'he authors recommend lon %term preoperative anti%inflammatory and antimicrobial therapy to avoid postoperative lymphan itis. -t is difficult to evaluate the results of such procedures when combined with resectional operations and in the absence of postoperative lympho raphy to demonstrate patency of the anastomoses. )owever, the deleterious effects of lymphan io raphic contrast on lymphatics were well demonstrated by O28rien and associates, who measured limb volume after lymphan io raphy in $11 patients and found that B# percent had a si nificant increase in le volume and $= percent developed lymphan itis. 'herefore, it seems advisable to use lymphan io raphy only for dia nostic studies and not for pre% or postoperative evaluation until safer contrast material becomes available. 7dditional efforts to combine resectional operations with microlymphovenous anastomoses as reported by O28rien and +hafiroff may offer some bri hter prospects for improvement of these debilitatin disorders. !8iblio raphy omitted in *alm version& 8ac. to 9ontents