chapter 21 venous & lymphatic

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CHAPTER 21 - Venous and Lymphatic Disease

Richard M. Green

Kenneth Ouriel

VENOU D!EAE

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 riht ventricle. 'he initial force produced by the left ventricle is reduced

throuh 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+ beins 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 ede

of the patella. 'he saphenous nerve accompanies the vein from the foot to the upper

thih, where it penetrates the fascia and enters the subsartorial canal alon the

superficial femoral artery. 'he G+ pierces +carpa2s fascia in the midthih and enters

the fossa ovalis in the roin, 3 cm lateral and inferior to the pubic tubercle. 'he L+

beins 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

throuh the adductor hiatus to become the superficial femoral vein. 'he latter is

/oined by the deep femoral vein in the upper thih to become the common femoral

vein, which becomes the external iliac vein as it enters the pelvis beneath the inuinal

liament. 4umbers of valves increase with distance from the heart, thouh the vena

cava and common iliac veins are valveless. 5ach valve is based within a dilated sinusof 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 patholoy 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 suest that it is more common for thrombi to

oriinate in the veins of the soleus and then propaate 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.

9ocett2s 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 thih 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

diaphram descends durin inspiration and the external pressure on the external iliac

veins decreases venous return. alve closure prevents sinificant reflux durin

inspiration. 'he converse occurs durin expiration. enous return is increased asintraabdominal pressure is decreased with upward movement of the diaphram. ;hen

a person is in the upriht 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 sinle 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 reconi6ed by

Rudolf irchow, who introduced the term thrombosis in $0(<.

tasis

7lthouh 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 plateletareation 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 paced

red cells within a fibrin networ accompanied by a variable number of leuocytes.

'he propaation of the thrombus depends on the relative balance between activated

coaulation and fibrinolysis. More commonly, in about <1 percent of patients the

thrombus propaates without interruptin flow and develops a lon floatin ?tail@ that

is more susceptible to breain loose from its tenuous anchor within the valvularsinus. -t is the latter seAuence of events that is the most danerous aspect of the



disorder, because ma/or pulmonary embolism can and does occur without

premonitory sins or symptoms at its point of oriin. 'his process can bein under

eneral anesthesia in the operatin room but usually reAuires other contributin

factors such as shoc, infection, trauma, or conestive heart failure. 7in, obesity,

prenancy, and malinant 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 thromboenesis. Routine histoloic 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 lacin.

Hype$coa#u"a'i"ity

7bnormalities of the blood include aberrations of the clottin and fibrinolyticsystems. +tasis and in/ury alone are not sufficient to cause thrombosis experimentally

in the absence of low levels of activated coaulation factors. *atients who present at

an early ae with spontaneous venous thrombosis, who have a stron family history of

>', or who develop recurrent venous thromboembolisms are usually considered

?prothrombotic@ or ?hypercoaulable.@ '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 anticoaulant 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 hypercoaulability 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 anticoaulation 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 hypercoaulable state with recurrent

thrombotic events and antibodies directed aainst phospholipids. 'here are primary

!no associated autoimmune disease& and secondary forms. 7*+ occurs in youner

patients rather than in atherosclerotic populations, and very few of these patients

smoe. 7ntiphospholipid antibodies, which include anticardiolipin, are bein

reconi6ed 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 worup 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 dianosis is suested in a patient with an appropriate history and aspurious elevation of the *''. *atients on estroen therapy for postmenopausal



replacement, birth control, or chemotherapy are at increased ris for venous

thrombosis.

'he association between venous thrombosis and cancer was first suested by

7rmand 'rousseau in $0(< and often has been confirmed in postmortem studies. -n a

series reported by 7dera and associates, B3 percent of otherwise healthy patientswith idiopathic >' were found to have malinant disease dianosed an averae of

#3 months later. -ncreased lielihood of cancer in these patients was associated with

ae over <( years, anemia, and eosinophilia. 'he earliest%onset malinancies were

found within $ year and usually occurred in the pelvic orans and breast.

*atients presentin with a thrombotic episode at a youn ae or those with previous

events should be screened for hypercoaulability. 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 areation, and mutantfactor should be done in very hih%ris patients. +creenin is difficult once

anticoaulation has beun. "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

drus may produce false neatives 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. Orani6ation of the thrombus beins at the attachment 6one as endothelial

cells activate thrombus%bound plasminoen. 'his results in enlarin pocets within

the thrombus and eventual framentation. 'he clot itself underoes softenin and

contraction durin this process, with the potential to restore the venous lumen. +erial

studies usin duplex ultrasonoraphy show that the process of recanali6ation beins

by day D in rouhly (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 propaation of clot in previously

uninvolved areas, thromboses in another extremity, and rethrombosis of a partially

recanali6ed sement. 'he incidence of these recurrences is reduced tenfold when patients are iven adeAuate anticoaulation 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 thih and pelvis produces a characteristic presentation of pain and extensive pittin edema. 'he



extremity may have bluish discoloration !phlemasia cerulea dolens& or blanchin

!phlemasia alba dolens, or ?mil le@&. 'he latter usually occurs in association with

prenancy. Other mechanical factors that can affect the left iliac vein include

compression from the riht iliac artery, an overdistended bladder, and conenital

webs within the vein. 'hese factors are responsible for the observed 3:$

preponderance of left versus riht iliac vein involvement.

*hlemasia cerulea dolens occurs when the venous thrombosis proresses and

impedes most of the venous return from the extremity. 'here is daner of limb loss

from cessation of arterial flow. "ortunately, this occurrence is rare. Loss of sensory

and motor function and venous anrene are liely unless an aressive approach is

implemented to remove the thrombus and restore blood flow. 'his condition almost

always occurs with advanced malinant disease.

Dia#nosis

Only 31 percent of patients with venous thrombosis have any clinical sins of the

disorder. )omans2s test is performed by dorsiflexin the foot. -t is considered positivefor >' if the patient complains of calf pain. "alse% positive clinical sins occur in

more than B1 percent of patients studied. enous duplex ultrasonoraphy has

releated other noninvasive tests, such as radioactive%labeled fibrinoen scans and all

types of plethysmoraphy, to historical interest. -n some centers duplex scans have

replaced contrast venoraphy as the best dianostic 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 sureries,

proloned immobili6ation, and nown hypercoaulability 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 sill. 'hrombus may be difficult to visuali6e in its acute form, and the

addition of color flow imain facilitates the identification of nonoccludin clots.

'hrombus echoenicity increases with ae of clot. enous compressibility is

determined by placin the probe directly over the vein and applyin entle pressure

while observin under 8%mode imain !"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

sinal indicates total obstruction. 7 neative scan performed by a well% trainedultrasonorapher is sufficient to rule out a >' of the lower extremity.

'he role of venoraphy has been diminished by the advances in ultrasound

technoloy. 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 dianosis of venous thrombosis and the extent of involvement. 'he main

indication for its use in the dianosis 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 fromvenous 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 hih%ris patients. *atients older than D1 years of

ae, those with previous thromboembolism, malinant disease, paralysis, multiple

trauma, or lower extremity /oint surical procedures have a very hih ris for >'.*rophylactic measures are directed toward alterin blood coaulability or eliminatin

or reducin venous stasis.

5fforts to reduce stasis include elastic compression stocins, 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 beinnin under eneral

anesthesia and are of proven efficacy in patients underoin total nee replacement,

radical prostatectomy, or operations where ad/uvant anticoaulation therapy is

contraindicated.

*harmacoloic prophylaxis includes low%dosae unfractionated heparin !E")&,

ad/usted%dose heparin, low%molecular% weiht heparin !LM;)&, warfarin

!international normali6ed ratio #.1FB.1&, and dextran D1. *rophylactic low%dosae

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 hih%ris roups with the exception of those

underoin orthopaedic or uroloic procedures. )iher% ris patients reAuire ad/usted%

dose E") with the activated partial thromboplastin time !7*''& held in the upper

normal rane. 8oth reimens 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 drus. LM;) is ten times

more costly than E"), however.

'here are ood data to support the use of preoperative oral anticoaulant therapy with

coumarin derivatives in hih%ris patients. ;hen iven the niht before operation,

warfarin anticoaulation is achieved within B to 3 days. 'he ?two%step@ or minidose

warfarin reimen is desined to circumvent the delay in anticoaulation. ;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 hemorrhae, and becauseof 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 underoin hip surery recommends warfarin or ad/usted%dose heparin to

prolon the 7*'' to the upper normal rane. '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 hemorrhaic

problems, alleric reactions and, in older patients, conestive heart failure.

Recommendations for prophylaxis are listed in 'able #$%#.

)edica" T$eatment

'he approach to manaement 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 rane. 7s the pain, swellin, and tenderness resolve

over a ( to D day period, ambulation is permitted with elastic stocin support.

+tandin still and sittin should be prohibited to avoid increased venous pressure andstasis. *atients with lare thrombus loads are candidates for fibrinolytic aents in an

attempt to preserve venous valvular competence. 'he practice of mandatory bed rest

and hospitali6ation has been challened by the encourain results of outpatient

treatment of >' with LM;).

Anticoa#u"ation

'he foundation of therapy for >' is adeAuate anticoaulation, initially with heparin

and then with coumarin derivatives for proloned protection aainst 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 reducesthe platelet release reaction. -t may be administered by continuous or intermittent

intravenous doses reulated by whole blood clottin time or 7*''. Recurrent

episodes of thromboembolism are $( times more common in patients with inadeAuate

anticoaulation 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 reulated 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. Enlie 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 ranin from #( to BD percent resultin from diffuse uncontrolledclottin with limb ischemia and oran infarction. 'here are no nown factors that

predict ris. >evelopment of thrombocytopenia from heparin is independent of sex,

ae, blood type, amount of heparin iven, type of heparin, and route of administration.

)-' has been documented after minimal heparin dosaes such as those received with

-.. flushes and heparin% coated indwellin catheters.

'wo forms of )-' exist. 'ype -, the most freAuent, is mild !platelet counts

$11,111CmmB&, reverses despite continuation of heparin, is due to a direct pro%

areant 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 areation and isoften 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 advantae of the LM;)s over E") is their superior

pharmacoinetic properties, allowin their use without laboratory monitorin. LM;) preparations have been compared with E") for the acute treatment of >' in $B

well% desined trials. *ooled results from these studies show that LM;)s

administered subcutaneously are as effective and safe as E") but have the advantae

of the potential for home treatment and do not reAuire laboratory monitorin. 'hese

advantaes may offset the increased cost of LM;).

Oral administration of anticoaulants is beun shortly after initiation of heparin

therapy. 'here is a ris in ivin coumarin derivatives to a patient who is not already

anticoaulated 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 anticoaulants that function byinhibitin activated factors and ---. 7 vitamin K antaonist potentially can create

a hypercoaulable state before achievin its anticoaulant 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 anticoaulation with

coumarin derivatives.

>ata from prospective studies indicate that the level of anticoaulation with coumarin

derivatives are effective at an international normali6ed ratio of #.1 to B.1. )iher

levels are not more effective and are associated with a hiher incidence of bleedin

complications. 7dministration of fresh fro6en plasma usually can normali6e the

prothrombin time and control hemorrhaic complications. 9oumarin derivatives cross

the placenta and should not be used durin prenancy. 7fter an episode of acute >',

anticoaulation therapy should be maintained for a minimum of B months some

investiators favor < months for treatment of thrombi in the larer veins. Many drus

interact with coumarin derivatives !e.., barbiturates&, and it is essential that a routine

for reular monitorin of prothrombin time be established after the patient leaves the

hospital.

Th$om'o"ysis

7nticoaulant therapy is desined to prevent recurrent thromboembolism. -deally, a

treatment would be available with the potential to eliminate the thrombus andmaintain valvular function. 7 number of trials have been performed comparin

thrombolysis with standard anticoaulant therapy. 9omplete clearin of thrombus was

noted in 3( percent of patients treated with thrombolytic aents, 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 aents have no advantae 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 thisapproach 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, Kaar compared

hemodynamic and clinical results in patients receivin (%day treatment with heparin

or streptoinase, 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

chanes. 4o difference was seen between patients receivin heparin or streptoinase.

'hese studies were done with systemic infusion of lytic aents. +tudies evaluatin the

use of catheter%directed thrombolysis have had encourain results in selected areas

and are discussed in detail in the section +ubclavian ein 'hrombosis.

u$#ica" App$oachesOpe$ati&e Th$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 salvae in the presence of phlemasia cerulea dolens and

impendin venous anrene and in patients with a contraindication to thrombolysis.

'he direct surical approach to remove thrombi from the deep veins of the le uses

the common femoral vein and is facilitated by the use of a "oarty venous balloon

catheter and an elastic wrap for milin the extremity !"i. #$%<&. Results are

improved when the extent of thrombus is documented preoperatively, when

completion phleboraphy is performed to assure complete clot removal, when a small

arteriovenous fistula is constructed to maintain hih blood flows in the iliac vein, and

when anticoaulation therapy is iven over proloned periods. 5arly results in (D

patients treated in this fashion reported by 5inarsson and associates showed patency

of the iliofemoral sement by venoraphy in <$ percent, and D( percent had a oodclinical result. Measurement by venous function, however, usin plethysmoraphy

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 trianle and the anle. 7fter surery 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 anle, the posterior tibial artery may be anastomosed to the

posterior tibial vein or the reater saphenous vein. 'wo problems have led to thereluctance of some sureons to adopt this procedure: the fear of damain 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 damae to this area at

reoperation, and a liature 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 anticoaulation therapy !'able #$%B&. 5arly operations in which one or both

common femoral veins were liated were associated with hih recurrence rates and a

hih incidence of seAuelae because of stasis in the lower extremity. 9ontrol of the

inferior vena cava for liation reAuired a laparotomy and added the adverse effect of a

sudden reduction in cardiac output under eneral anesthesia. 'his effect, coupled withstasis seAuelae and recurrent embolism throuh 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 loical

step was to devise a transvenous approach that could be performed under local

anesthesia. 'he Mobin%Eddin ?umbrella@ unit was inserted from the /uular 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

miration.

'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

/uular 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 proloned 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 dianosis usually is obvious. >uplex scans are very accurate in confirmin

the dianosis and should be performed especially when swellin is present to rule outa concomitant deep venous problem.

+ymptoms usually last for # to B wees. 4onsteroidal anti% inflammatory aents

provide sinificant pain relief. *atients should not be ept at bed rest. 7ctivity should

be encouraed 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 undero full anticoaulation therapy.

Th$om'oph"e'itis )i#$ans

'hrombophlebitis mirans, a condition of recurrent episodes of superficial

thrombophlebitis, can be associated with visceral malinancy, 8uerer2s disease, thehypercoaulable states, systemic collaen 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 anatomicabnormality at the thoracic outlet, may be related to the placement of a central venous

catheter, or may occur in a hypercoaulable 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 mae up 1.( percent to $.( percent of all venous thromboses and

often are associated with strenuous activities. 'hese cases are referred to as the *aet%

+chroetter syndrome or ?effort thrombosis.@ 9atheter%induced thrombosis is an

increasinly common event because of the more freAuent use of central veins for

access, nutrition, chemotherapy, and monitorin. +creenin venoraphy in patients

with central venous catheters demonstrates that BB to <1 percent have thrombus in the

axillosubclavian sements. 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 venoraphy in the dianosis of

lower extremity venous thrombosis, but the opposite is true in the upper extremity.

enoraphy has a reater dianostic accuracy when performed with the catheter in

the basilic vein. 7 typical +' is shown in "i. #$%$1.

>ata support a role for conventional anticoaulation in all patients with +' for

prophylaxis aainst pulmonary embolism and for reduction of residual symptoms.

7ressive surical therapy focused on clot removal is no loner recommended.

-nstead, physically active patients with +' dianosed in the acute phase should

undero catheter%directed thrombolysis. 9urrent protocols recommend uroinase

delivered via a catheter placed throuh the thrombus. 'his approach is successful in

more than (1 percent of patients and ideally identifies the anatomic cause of the

thrombosis !"i. #$%$$&. 7lthouh the timin of correction is debated, most sureons

aree that extrinsic and extrinsic venous lesions should be aressively 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 approachto 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 propaatin

thrombus from the iliac veins. 'umors of the vena cava are rare and are usually

malinant and have a poor pronosis. 'hey may be primary, such as a

leiomyosarcoma, or secondary, such as a hypernephroma and a retroperitoneal

sarcoma. +ymptoms and sins depend on the sement of vena cava affected by the

tumor and the deree of obstruction of ad/acent orans. -nvolvement of the

suprahepatic cava may cause the 8udd%9hiari syndrome. 'he dianosis can be made

with a variety of imain modalities, includin manetic resonance imain !MR-&,computed tomoraphy !9'&, ultrasonoraphy, and contrast cavoraphy.



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 stroe. 5stimates of the mortality in the

Enited +tates alone rane 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 underoin ma/or surery will die from massive pulmonary embolism.

irchow first reconi6ed 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 patholoists that pulmonary embolism could be well tolerated by some

patients who then died of other causes. 'he full spectrum of the disorder ranes 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 aressive prophylaxis, the incidence has been

reduced to < percent.


'he sins and symptoms of an embolic episode depend primarily on the manitude 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 sins

of venous thrombosis. 'he dianosis 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

mared anxiety. )emoptysis is an uncommon sin, 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 /uular veins, and varyin derees 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 findins found in the Eroinase *ulmonary

5mbolism 'rial is shown in 'able #$%3.

'he differential dianosis includes esophaeal 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 worin

dianosis. Laboratory studies in eneral are not very helpful in the differential

dianosis, althouh a white blood cell count of less than $(,111CmmB miht besuestive when a pulmonary infiltrate is present to help rule out pneumonitis.

Dia#nostic tudies

5lectrocardioraphy

'he primary value of the electrocardioram !59G& is to rule out a myocardial

infarction. -n the presence of a pulmonary embolus, the 59G may have sins of riht

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 chanes that are nondianostic.

Chest Radio#$aphy



'he primary value of the chest radioraph is to exclude other dianostic possibilities,

such as pneumonia, pneumothorax, esophaeal perforation, or conestive heart

failure. 7lthouh central vascular enlarement, asymmetry of the vascular marins

with semental or lobar ischemia !;estermar2s sin&, or pleural effusion may

suest pulmonary embolism, they are rarely sufficient to establish a dianosis. 'he

chest radioraph is critical in the interpretation of a lun scan, because anyradioraphic density or evidence of chronic lun disease maes a perfusion defect less

liely 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

radioraph !"i. #$%$#&.

A$te$ia" ,"ood ases

)ypoxemia with *aO # of less than <1 mm) is hihly suestive of pulmonary

embolism, especially when the chest radioraph does not show any other pulmonary

patholoy. 'he low *aO # is believed to be a result of shuntin by overperfusion of

nonemboli6ed lun and a widened alveolar% arterial oxyen radient from reduced

cardiac output. 'he reduction in arterial *9O # that follows ma/or embolism is themost discriminatin findin because hypoxemia is present in several disorders liely

to be misdianosed as massive embolism !e.., septic shoc&. -f hypoxemia and

hypocapnia are not present, the dianosis of ma/or embolism in the severely ill patient

is unliely, and an alternative dianosis should be souht.

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 riht ventricular overload and elevated riht atrial pressures.

5levated riht 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 dianostic test is the perfusion lun scan. 7 normal scan

rules out a ma/or pulmonary embolus, but an abnormal scan does not ensure the

dianosis. -n a nonhypotensive patient with a normal chest radioraph, 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 isinterpreted as hih probability, the dianostic accuracy as compared to pulmonary

anioraphy is =< percent.

Pu"mona$y A$te$io#$aphy

+elective pulmonary arterioraphy 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 passae of a cardiac

catheter into the pulmonary artery for in/ection of a bolus of contrast medium. 7

series of radioraphs that outline areas of decreased perfusion and usually show fillin

defects or the rounded trailin ede of impacted emboli !"i. #$%$3& is obtained.

+traiht 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, althouh pulmonary

hypertensive and cardiac patients are at hihest 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 anioramexcludes the dianosis of pulmonary embolism in acutely ill patients.

Pathophysio"o#y

-t is estimated that 0( to =1 percent of all pulmonary emboli oriinate from the veins

of the lower extremity, and the remainder arise from the riht side of the heart or

other veins. Once the embolus has loded and interrupted pulmonary blood flow, the

ratio of reional 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 therelease of serotonin adds further /ustification to the early use of this dru. Other

vasoactive aents, such as histamine and prostalandins, 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

thouht to be due to a ventilation% perfusion imbalance secondary to the ventilation

chanes described above, althouh the findins in some patients resemble true

arteriovenous shuntin. 7lthouh there may be some improvement in *aO #after

supplemental oxyen is administered, the effects usually are minimal. 'he pulmonary

vascular and cardiac effects of embolism are a direct conseAuence of the deree of

occlusion of the pulmonary vascular bed. 'he loss of more than B1 percent of the

vascular tree is reAuired to bein 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

pronosis !'able #$%(&. 'he minor derees of embolism usually can be manaed with

anticoaulants alone with a satisfactory outcome. 9ontinuous%infusion heparin is the

initial treatment, in a dosae desined to prolon the partial thromboplastin time to at

least twice normal !approximately $(1 unitsC &. Most clinicians also bein oralanticoaulation therapy to allow several days2 overlap of the drus as prothrombin

time is extended into the therapeutic rane. 7deAuate anticoaulation stops the

proression of thrombosis and is associated with a recurrence rate of less than (

percent.

.i'$ino"ytic The$apy

5mboli typically undero dissolution as a result of the active fibrinolytic mechanism

in the pulmonary circulation. 7ctivation of plasminoen to plasmin, which is found in

hih concentration in the pulmonary circulation, promotes this fibrinolytic effect.

"ibrinolytic aents have been administered to increase the rate of lysis after

pulmonary embolism/ Tissue p"asmino#en acti&ato$ 0t-PA wors more rapidly thanu$oinase3 but both are costly and are associated with a hih incidence of bleedin



complications. -ndications for fibrinolytic treatment include any critically ill patient

with a pulmonary embolus. 8leedin complications can be reduced by tain a

careful neuroloic history to eliminate patients with any brain patholoy, minimi6in

venous and arterial punctures, stoppin heparin administration, and identifyin any

other potential causes of bleedin.

'he advantae 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 uroinase or streptoinase showed a better

restoration of pulmonary%capillary blood volume and diffusin capacity at # wees

than in patients treated with heparin and anticoaulants 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 aent.

Vena Ca&a" !nte$$uption

-n some patients, anticoaulants cannot be used because of associated problems !e..,

peptic ulcer disease&, and manaement must be directed toward a mechanical means

of protection aainst recurrent embolism as outlined earlier !see 'able #$%B&. Other

patients, in whom anticoaulation appears to be adeAuate, sustain recurrent embolism

and become candidates for surical intervention. 'he third indication is when there

has been a complication of anticoaulant therapy, forcin it to be discontinued and

leavin the patient with untreated >'. 7nother indication for a vena caval filter is

protection aainst 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 oriinal focus of

venous thrombosis remains untreated and recurrent embolism is liely.

'here are two additional relative indications for a vena caval filter in a patient with

active or recent >'. One is the hih% ris patient over 31 years of ae who is obese

and has a serious associated medical illness !e.., heart disease&, malinant disease, or

a history of previous embolism and who underoes 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 liely not be able to

tolerate additional emboli, particularly if there is associated cardiac or pulmonary

disease.

Pu"mona$y Em'o"ectomy

'he direct surical 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 usedfor 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 arterioraphy and then transported to the

operatin room, where they can tolerate eneral anesthesia and sternotomy much

better while bein maintained on partial cardiopulmonary bypass.

5merency pulmonary embolectomy rarely is indicated but should be considered inany patient with an acute pulmonary embolism who appears preterminal. 'hese

patients rarely survive the trip to the anioraphy suite and operatin room, but when

they do, the operation is probably not necessary if fibrinolytic therapy is available.

>ocumentation of the dianosis of massive pulmonary embolism by pulmonary

arterioraphy is mandatory because the clinical dianosis often is incorrect. 'he initial

approach to patients who have transient collapse or persistent systemic hypotension

should include full heparini6ation and administration of inotropic drus, if necessary,

to support the circulation while the dianosis 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 provoe

arrhythmias, however, necessitatin the use of dopamine. -n the patient who respondsto heparin and does not reAuire vasopressors for systemic pressure or urine output,

careful monitorin is essential to determine whether anticoaulation and fibrinolysis

will control the disorder. Open pulmonary embolectomy carries a mortality rate in the

rane of (1 percent, however, and uncontrollable pulmonary hemorrhae may follow

open restoration of pulmonary perfusion.

7n alternative approach usin local anesthesia has been suested by Greenfield and

associates for transvenous removal of pulmonary emboli. 7 cup device attached to a

steerable catheter is inserted in the /uular or the femoral vein, and the cup is

positioned under fluoroscopy ad/acent to the embolus seen on arterioraphy !"i. #$%

$<&. 'he position is verified by in/ection of contrast medium throuh the catheter.

'hen syrine 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 anioraphy, 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 proloned period if they fail to

undero lysis and obliterate the pulmonary vascular bed. 'he clinical picture in this

case is one of chronic cor pulmonale because sinificant pulmonary hypertension

results from chanes in the pulmonary vascular bed. 'he presentation may be subtle

with only dyspnea or syncope on exertion, but there is a loud *# and riht%sided strain

on the electrocardioram. 'he seAuence also may occur unaccompanied by sinificant

respiratory symptoms, and this may explain the cause in some of the patients

considered to have primary pulmonary hypertension. ;hen the dianosis 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 riht 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 manaed by lon%term anticoaulation therapy, or in some cases should

be considered for open pulmonary thromboendarterectomy or heart%luntransplantation.

Recurrent thromboembolic pulmonary hypertension produces exertional dyspnea and

sins of riht heart strain with cor pulmonale. ;ith further proression of riht heart

overload, tricuspid insufficiency may develop. 'his disorder may be difficult to

distinuish from primary pulmonary hypertension, althouh the latter is more liely to

be found in women under #1 years of ae without a history of >'. +evere

pulmonary hypertension is a serious problem and usually limits the life expectancy to

less than # years from dianosis.

Open thrombectomy for chronic occlusion was first performed by 7llison andassociates in $=(0 and remains a possibility for improvin pulmonary blood flow. "or

a patient to be eliible for this procedure, the occlusion must involve the proximal

portion of the pulmonary arterial tree, and the distal bed must be patent. 'he

physioloic basis for continued distal patency after proximal occlusion is bronchial

arterial collateral flow. 'he procedure also has a sinificant 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 anticoaulation therapy and vena caval filter placement.

"i. #$%$D presents an alorithm for the manaement of pulmonary embolism.

Va$icose Veins

aricose veins are the most common vascular disorder affectin human beins. 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, elonated, or tortuous

vein, irrespective of si6e.@

Etio"o#y'here are four factors that affect the development and proression 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 prenancy, usually appearin in the first trimester !D1 to 01

percent& when the corpus luteum is secretin proesterone. *roesterone 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 proesterone

are amplified by estroen. )ydrostatic forces produce venous dilatation from the

weiht of the blood column transmitted throuh incompetent valves. 'he other force



is exerted by the contractin muscles on ad/acent veins via the perforatin system.

'hese forces reularly exceed $(1 mm).


'he usual distribution of varices is below the nee in branches of the reater

saphenous system !"i. #$%$0&. 'he symptoms associated with varicose veins arenonspecific achin and heaviness of the les that can be attributed to the conestion

and poolin of blood in the enlared superficial venous system. 'hese symptoms

worsen as the day proresses, reAuirin the patient to rest with le elevation to obtain

relief. 9alf%lenth elastic stocin support in the rane of #1 to B1 mm) may

provide symptomatic relief for those whose vocations reAuire lon periods of standin

or sittin. 7lthouh 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 niht crampin of the les may

be helped by the administration of Auinine sulfate, which reduces muscular irritability.

Dia#nosis'he 'rendelenbur test is useful in distinuishin 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 thih varices fill

rapidly, indicatin incompetence of the saphenofemoral valve. 'here are four possible

results of this test. 7 neative%neative 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

neative%positive result, the release of compression is followed by a rapid fillin of

the saphenous vein, indicatin that its valves are incompetent. 7 positive%neative

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. 9hanes in the direction of flow are detected by chanes in color, and

venous valves may be seen on the rey% scale imae.

-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 walin, 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 manaed 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 aressive manaement.

'he two methods of treatment currently used are in/ection sclerotherapy and ablative

surery. +clerotherapy should be reserved for those patients who do not have

evidence of axial saphenous reflux. Lare varices of the thih also should be removedsurically because they are sub/ect to superficial thrombophlebitis after sclerotherapy

and are often associated with lare 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 syrine with the patient standin followed by elevation of the le, in/ection of the

aent, and bandae compression of the area for # to B wees. +odium chloride #B.3J

is the aent 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 conenital vascular malformations of venous

predominance, bleedin varices, and lare varices around an ulcer. 'he most commoncomplications include hyperpimentation, sin 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 thorouhly because routine strippin of the reater saphenous vein from

roin to anle 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 mared

preoperatively, supplements the strippin.

Removal of the reater saphenous vein reAuires its detachment from the common

femoral vein and liation of its tributaries at the saphenofemoral /unction !"i. #$%#1&.

-f an anle incision is made, care must be taen 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 lenth and

are oriented in the sin 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

midthih 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 deree of surface stasis which

obviously interferes with the nutrition of the sin 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 havean increased permeability to fibrinoen and red blood cells. Lymphocyte and



macrophae 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 thicenin and lipodermatosclerosis

of the subcutaneous tissues that produce a characteristic ?brawny@ edema. 'he loss of

red cells results in hemosiderin deposits, producin the characteristic pimentation. 7

deterioration of mononuclear cell function is associated with chronic venousinsufficiency. 7 decreased capacity for lymphocyte and monocyte proliferation in

response to various challenes translates into poor or proloned wound healin. ;hen

the distal perforatin veins become incompetent, there is additional pressure, with

sin atrophy leadin ultimately to necrosis and chronic stasis ulceration !"i. #$%#$&.

Dia#nosis

'he dianosis of chronic venous insufficiency usually is made by inspectin the le.

*hysical findins 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 imain. ;hile the velocity spectrum is

displayed, various maneuvers are performed to reverse the normal peripheral%to%

central radient. Retrorade flow is indicated by an inverted spectrum and a chane 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 beinnin 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 imain. 4ormal valves are

thin and mobile diseased valves are shortened and thic, often with attached

echoenic material.

enous obstruction is identified with the same techniAues used to dianosis acute

venous thrombosis. 7fter the vein is identified with 8% mode imain, its patency is

assessed by its compressibility and >oppler spectrum. ;ith partial occlusion orincomplete recanali6ation, there will be an encroachment on the flow imae that no

loner fills the entire vein. 9ollateral veins will be seen, and that findin is

particularly useful in distinuishin between acute versus chronic occlusions. 7nother

distinuishin feature of chronic occlusion is the shrunen si6e of the vein when

compared to the distention seen in acute >'.

'he physioloic 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

slihtly hiher 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 walin,



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 manaed nonoperatively. 7lthouh

the mechanism of benefit is unnown, compression therapy is the most important

aspect of patient manaement. 9onflictin hypotheses include a reduction in

ambulatory venous pressure, improvements of the microcirculation of the sin and

subcutaneous tissue, and increase in the pressure of the subcutaneous tissue that

reduces the leaae of fluid from the capillaries. 'he latter is the most plausible

because cutaneous metabolism may improve after fluid resorption, allowin an

enhanced diffusion of oxyen and other nutrients.

'he initial treatment of patients with venous ulceration should include a period ofstrict bed rest to reduce edema. +ystemic antibiotics are iven for the surroundin

cellulitis. 5lastic stocins are fitted when the edema has subsided. +urroundin areas

of dermatitis are treated with topical steroids. *atients are then instructed to wear the

elastic stocins 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

stocins 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 manesium aluminum silicate. *atients whose

ulcers fail to heal after proloned outpatient care reAuire hospitali6ation.

Ope$ati&e )ana#ement

*atients selected for operation have severe, disablin symptoms and a history of

recurrent ulceration despite aressive medical therapy. 9andidates for operation

should undero ascendin and descendin venoraphy 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 liely unless the perforatin veins responsible

for the ulcer are identified and liated. 'he typical location of these veins is posteriorand superior to the medial malleolus. Liation of the perforator vessels should be the

initial procedure for recurrent ulceration. 'reatment failure occurs in $1 percent of

patients despite viorous medical therapy, includin support stocins, le elevation,

wound care, and patient education. 'hese patients should be considered for venous

reconstruction.

Venous Reconst$uction

'he present attitude of most sureons toward venous reconstruction is critical and

pessimistic. 'he venous system, unlie the arterial system, tends to recanali6e, thus

main it more difficult to Auantitate the obstruction and identify the patient who may

benefit from venous reconstruction. >ale estimated that the percentae 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 thicened valves that do not lendthemselves to this type of reconstruction. Kistner, after studyin #11 limbs with

ascendin and descendin venoraphy, found #0 that could be treated by valve repair,

and D# percent had an excellent result. -n this procedure, floppy, incompetent valves

are tethered aainst 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 anioscope. Results of this procedure are difficult

to interpret because the operations are often combined with saphenous vein strippin

and perforator liation. Most investiators have reported improvement in symptoms

for proloned periods in approximately <1 to 01 percent of the patients.

>irect repair is not possible for postthrombotic valvular dysfunction. 'he tworeconi6ed options for surical candidates are transposition of a deep femoral or

saphenous vein valve or transplantation of a valve%bearin sement 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 coworers

described << patients with ood results in D0 percent. -n this series, B$ patients had

postoperative venorams, #0 of these were found to have valvular competence. 7

number of other investiators 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 hih proportion of the affected limbs had reverted to

their preoperative condition. 8eran and colleaues have pointed out that for venous

valve surery to be successful, it usually must be accompanied by saphenous vein

strippin and perforator liation. 'he difficulty in identifyin patients who could

benefit from these procedures was put into perspective by >ale, who, after # years of

investiatin, 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 hih 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 dianosis, and venoraphy 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 procedureconsists 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 surery.

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 thih, identifyin the patient

with an obstructed superficial femoral vein who miht 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 +mithand '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

riht 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 9ocett and

'homas, who advocated surical correction of these lesions. >ale reviewed eiht such

patients identified by venoraphy 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. 9ocett and

'homas, conversely, found the results unsatisfactory, and after operatin on B1

patients usin several different methods they recommended abandonin the

procedure.

Venous T$aumaenous in/uries of the extremities usually are associated with arterial in/uries because

of their anatomic proximity. >irect liation of in/ured superficial veins is appropriate

treatment except when they are the sole remainin venous drainae of the extremity,

which mandates their repair. 'reatment of in/uries of the deep veins chaned

dramatically as a result of the military experience in Korea and ietnam, as reported

by Rich and associates. -t was well demonstrated that liation of ma/or extremity

veins resulted in hiher rates of disability and limb loss than repair or replacement by

autoenous vein sements. '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

7arwal 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 venousliation. 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 oriin of lymphatic vessels is a matter of disareement amon

embryoloists. 'he oriinal theory of +abin traced the oriin from the venous system,

while )untinton and Mc9lure suested 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 eihth wee, there is a retroperitoneal lymph sac with a developin cisterna

chyli. 'hese systems develop communicatin channels that ultimately form the

thoracic duct by merer of the riht lymphatic duct with the left across the fourth to

sixth thoracic vertebrae, which then drains into the left subclavian vein. +mallerlymphatic ducts persist that drain into the riht subclavian vein.

>evelopmental arrest or abnormalities can result in primary hypoplasia or absence of

ducts and lymph nodes. 7bnormal rowth of /uular lymph sacs can produce

unilocular or multilocular lymph cysts termed cystic hyromas. 'hese cysts also may

be found in the axilla, mediastinum, retroperitoneum, or intestinal mesentery.

)yperplastic chanes may occur to produce lymphaniomas with or without other

vascular malformations.

'he function of the lymphatic system beins 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 larer particles can

be evacuated only throuh 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

larer channels that follow the vascular pathways superficial to the deep fascia.

7lthouh lymphatics can be found in the intermuscular fascia, they are absent in

muscles, tendon, cartilae, brain, and cornea.

Lymph is transported by afferent vessels to reional 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 forein 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 drainae 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 larer

ducts, respiration, arterial pulsation, and external massae. 'he main function of the

lymphatic system is to clear the interstitial spaces of excess water and particulate

matter.

Lymphedema

9lassification of Lymphedema



'he oriinal 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 conenital 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 lymphoraphy it became possible to classify the

primary lymphedemas structurally into hyperplasias and hypoplasias. 'he oriinalclassification as proposed by Kinmonth has larely been abandoned for the more

simplified version presented in 'able #$%<.

'he conenital lymphedemas are hypoplastic in =# percent of cases. 'heir subroups

are defined by lymphoraphy and behave differently. 'hose with distal hypoplasia

have a mild, nonproressive 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

proressive.

'he primary hyperplastic lymphedemas are uncommon !0 percent&, and those with

bilateral hyperplasia usually can be reconi6ed by diffuse capillary aniomata on the

lateral sides of the feet. Lymphoraphy shows dilated lymphatics with normal valves,

in contrast to the findins in the mealymphatic roup, in which no valves can be

seen. -n this latter roup, chylous reflux may produce chylometrorrhea, sin vesicles,

or chyluria.

'he most common cause of secondary lymphedema in this country is malinant

disease metastatic to lymph nodes. +urical 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 aents, such as silica, can enter the lymphatic system via

barefoot walin and cause fibrosis of lymphatics and lymph nodes.

9linical Manifestations

Lymphedema is a clinical dianosis 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 etioloy. Other enerali6ed hypoproteinemias may be seen inmalnutrition, cirrhosis, and protein%losin enteropathy, or they may be idiopathic.

7lleries or hereditary causes are unusual. -n unilateral edema, venous disease is the

most liely cause and can be reconi6ed by the examinations described in the

previous section.

'he patient with lymphedema complains of swellin and fatiue. Limb si6e increases

durin the day and decreases at niht 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 weiht loss and

diarrhea suests small bowel lymphaniectasia. On examination, lymphedema is

characteristically firm and rubbery but nonpittin. Lymph vesicles may be presentcontainin fluid of hih protein concentration. 9omplications of lymphedema such as



infection, cellulitis, erythema, and hypereratosis may be present. -t is important to

document limb si6e to identify isolated limb iantism and the Klippel%'rnaunay

syndrome that may have hypoplastic lymphatics in addition to venous abnormalities,

capillary nevus, and limb elonation. 'he patient should be examined for upper

extremity and enital lymphedema, hydroceles, and ameloenesis imperfecta.

>ianostic +tudies

>ye -n/ection

Lymphatics can be visuali6ed by dye in/ection in the extremities and mesentery, and

also by inestion of cream or mil to visuali6e intestinal lacteals and ma/or ducts.

7 hihly diffusible dye such as patent blue, introduced by )udac and McMaster, or

sy blue dye, recommended by 8utcher and )oover, can be in/ected in 1.# mL

amounts subcutaneously into each interdiital web. Massae of the sin 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 throuh

the dermal lymphatics to produce a marbled appearance called ?dermal bacflow.@

Lymphoraphy

'he techniAue of lymphoraphy 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 tracs 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. 7lthouh the techniAue is simpler than standard lymphoraphy, it has ma/or

disadvantaes because of the ha6iness of the scan, radiation dosae, and distribution

of the radionuclide into the extracellular fluid, main 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 dianosis 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 hiher in lymphedema fluid than in plasma, which is helpful in the

presence of an inflammatory exudate in which the protein content is hih but the

albumin%to%lobulin ratio is normal.

Manaement

+upportive 'reatment

'here are sinificant anatomic and physioloic limitations to the treatment oflymphedema. "rom the standpoint of physioloy, 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 chanes in the subcutaneous tissues. 'herefore, the options are limited

and the primary ob/ectives remain for control of edema, maintenance of healthy sin,

and avoidance of the complications of cellulitis and lymphanitis.

'he initial ob/ective of control of edema can be approached by elevation and the use

of seAuential pneumatic compression boots to massae 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 stocins as described

earlier for venous insufficiency. 'he stocins should be removed at niht and the

foot of the bed elevated to maintain the pressure radient from le to riht atrium.

Massae therapy was used in the treatment of lymphedema as early as $00# and has

current advocates.

'he onset of redness, pain, and swellin usually sinifies early cellulitis or

lymphanitis, which can be reconi6ed by red streain up the le. 'he usualcausative oranism is staphylococcus or beta%hemolytic streptococcus, which must be

treated viorously, usually with intravenous antibiotics. -n the absence of treatment,

the infection may obliterate more lymphatics and produce constitutional sins of

fever, malaise, nausea, and vomitin. 7nother freAuent complication is ec6ema, which

usually will respond to hydrocortisone cream. 7ntifunal aents 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, althouh

fissures and lymph fistulas can develop and reAuire surical 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 lymphoranuloma venereum. -n rare cases of lon%standin secondary

lymphedema, such as in the arm after radical mastectomy, a lymphaniosarcoma may

develop, appearin as a raised blue or reddish nodule. +atellite tumors and early

metastases may develop if it is not reconi6ed 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 abnormaland show extensive scarrin. 'he best results are obtained when the bul of the

extremity has severely impaired movement or when there have been recurrent attacs

of cellulitis. 7lthouh some efforts have been made to develop techniAues to improve

lymphatic drainae, 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 drainae, and the efforts of Kondoleon and

associates to excise fascia or insert a dermal flap into muscle proved ineffective in

improvin lymphatic drainae. 'he oriinal procedure devised by 9harles consistin

of wide excision of lymphedematous tissue followed by sin raftin still is usefulwhen the overlyin sin is in poor condition, as in elephantiasis. 'he procedure used



most often, however, is Kinmonth2s modification of )omans2s procedure, in which

sin flaps are raised to allow excision of the underlyin subcutaneous tissues.

'he most loical, albeit technically demandin, approach has been directed to

establishin lymphaticovenous anastomoses. -nitial efforts in this area were made by

4ielubowic6 and Ols6ewsi, who divided a lymph node, removed the pulp undermanification, 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 >eni,

who used a special needle for insertion of lymphatic vessels directly into veins and

fixed them there by a sinle suture. Esin this techniAue, "ox and associates treated

eiht 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 lymphanitis. 4ine of $$ patients

with primary lymphedemas had ood functional results, allowin them to resume

normal activity. 'he authors recommend lon%term preoperative anti%inflammatoryand antimicrobial therapy to avoid postoperative lymphanitis.

-t is difficult to evaluate the results of such procedures when combined with

resectional operations and in the absence of postoperative lymphoraphy to

demonstrate patency of the anastomoses. )owever, the deleterious effects of

lymphanioraphic contrast on lymphatics were well demonstrated by O28rien and

associates, who measured limb volume after lymphanioraphy in $11 patients and

found that B# percent had a sinificant increase in le volume and $= percent

developed lymphanitis. 'herefore, it seems advisable to use lymphanioraphy only

for dianostic 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 brihter prospects for improvement of these debilitatin disorders.

!8iblioraphy omitted in *alm version&

8ac to 9ontents

chapter 21 venous & lymphatic

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