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Lymphology 31 (1998) 15-20

Department of Pediatrics (MB,SW), North Shore University Hospital, Manhasset, and LymphedemaTherapy (BBL), Woodbury, New York, USA

In a single lymphedema treatment facility,128 consecutive patients with lower limblymphedema were retrospectively analyzed forthe development ofgenital edema. Thepatients were separated for analysis on thebasis of who used or did not use compressivepump therapy. Of the 128 patients with lowerlimb lymphedema, 75 received no pumptherapy, and 53 used pumps. Of the 75 whodid not use pump compression, only 2 hadgenital edema. Of the 53 patients who usedpump compression, 23 patients developedgenital edema after pump therapy (p<.0001).The incidence ofgenital edema was unaffected

.by age, sex, grade or duration of lymphedema,whether lymphedema was primary orsecondary, whether a single or sequentialpump was used, the pressure level applied, orduration or hours per day ofpump therapy.

Compressive pump therapy for lower limblymphedema produces an unacceptably highincidence ofgenital edema.

Lymphedema is a chronic, debilitatingdisorder. Untreated, primary and secondarylymphedema tends to worsen with time (1),with a multitude of clinical and psychologicalcomplications. These include limb immobility,loss of function, pain, paresthesias, recurrentinfections, skin ulcerations, lympho­cutaneous fistulae, genital edema, and rarelyangiosarcoma (2,3). Other disabilities includeimpaired performance of common householdand work tasks, inability to wear normal

clothing, and difficulty with sexual relations.Accordingly, adequate treatment of lymphe­dema is recommended.

The gradation of lymphedema has beendefined by the International Society ofLymphology (4). Grade 1 has minimalfibrosis, edema pits on pressure, and reduceson limb elevation; Grade 2 has overt fibrosis,non-pitting edema and fails to reduce withlimb elevation; Grade 3 has marked trophicchanges characterized by the expression"elephantiasis." Current treatment forlymphedema includes: Complex Lymphe­dema Therapy (CLT) (5-13), benzopyronedrugs (2,13), surgery (6,14), and pneumaticcompression pumps (15-24). Early phase(Grade 1) lymphedema responds to elevationand use of compression garments. Operativeprocedures include debulking, lymphatic orlympho-nodal anastomosis to veins, omentaltransplantation and lymphatic transplants(6) but morbidity is high and long-termeffectiveness is questionable. Moreover, someoperations (e.g., micro-surgical shunts) areavailable only in a few specialized centers(6,14).

External pneumatic compression devicesor "pumps" are commonly used to treatperipheral lymphedema. Several clinicalreports show that pumps reduce lymphe­dema; however, the clinical outcomes varywidely (15-24). Maintenance of edemareduction depends on the continued use ofpumps and/or compression garments (19).

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The amount of improvement in swelling aftertherapy appears related to degree of fibrosiswith the lower grades of lymphedemaresponding more favorably (23). Complica­tions of pump treatment include cellulitis andarterial insufficiency with occasional tissuenecrosis and even limb amputation (24,25).Onset of edema in previously normal regionsand increased fibrosis proximal to the cuff ofthe pump has been described (3,6,7).However, there is no demographic dataavailable as to what proportion of patientsare treated by pumps in the generallymphedema population, nor of the results orcomplications. This report examines theincidence of genital edema in 128 consecutivepatients with lower limb lymphedema whowere evaluated and treated at one facility.

CLINICAL EXPERIENCE

Subjects

The presence of genital edema wasstudied retrospectively in 128 consecutivepatients with lower extremity lymphedema ina single treatment facility. The demographicsof the study groups are illustrated in Table 1,

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and are similar to previous series (7-13,18-21)thereby demonstrating the study patients didnot manifest unusual clinical features. Eachpatient was interviewed and examined by atleast two of the authors. Before evaluation,each patient completed an information form.Moreover, a complete medical history wasobtained without emphasizing any relation­ship between genital edema and pump usage.

Statistical Analysis

Chi square tests were mainly used withYale's correction or Fisher's exact tests. Forage and duration, student T and Mann­Whitney tests were utilized.

Each set of data was analyzed for a)whether the outcome in a patient treated witha pump was affected by age, sex, duration oflymphedema, grade, unilateral/bilateralleglymphedema, single or sequential pump,pump pressures, hours per day of usage,length of time of pump therapy, or whetherlymphedema was primary or secondary; b)whether pump therapy was related to the .incidence of genital edema; c) whether pumptherapy correlated to any factors in a) orcorrelated with the incidence of genital edema.

TABLE 1Demographics of Patient Population With Lymphedema (LE)

Treated With and Without a Pump

Variable Pump No Pump p-values

Males 14(11%) 18 (14%) 0.76Females 39 (30%) 57 (45%)

Lymphedema (LE)Age (years) 49.1 (18.8)* 46.2 (21.2)* 0.82Duration (years) 12.6 (11.4)* 13.2 (10.6)* 0.85Grade 1 6 (5%) 20 (16%) 0.06Grade 2/3 47 (37%) 55 (16%)Primary 45 (35%) 28 (22%) 0.28Secondary 30 (23%) 25 (20%)Unilateral 34 (27%) 33 (26%) 0.025Bilateral 19 (15%) 42 (33%)

*Mean±SD

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. TABLE2Occurrence of Genital Edema (GE) in 128

Consecutive Patients with Lower LimbLymphedema

TABLE 3, Occurrence of Genital Edema (GE)Mter Different Types of Compression

Pumps Used to Treat Lower LimbLymphedema*

GE No Pump PumpGE Single Sequential

(-) 73 (97%) 30 (57%)(-) 12** (43%) 16** (57%)

(+) 2 (3%)* 23 (43%)** (+) 14t (37%) 24t (63%)

*p=<0.0001;*Not statistically significant; **5 patientsused both types of pumps; t8 patients used

**No genital edema before pumpingboth types of pumps

RESULTS

The initial 128 consecutive patientspresenting with lower extremity lymphedemawere examined by the authors for evidence ofgenital edema. Of the 75 patients who did notuse pumps, there were only 2 (3%) withgenital lymphedema (Table 2), one of whom(a 2-month old female) had vulvar edemasince birth. Of the 53 patients who usedpumps, genital edema developed in 23 (43%).The difference was highly significant(p<.0001). Each patient reported that genitaledema was absent before compressive pumputilization, that it developed subsequently,and persisted after discontinuance ofcompression pumping. The incidence ofgenital edema was not influenced by sex, age,duration of lymphedema, if primary orsecondary or the grade of lymphedema. Theoccurrence of bilateral lower limblymphedema in the pump treated patients(15%) compared with the non-pump treatedpatients (33%) was significantly different(p=O.025). However, when this difference wasanalyzed as a factor in production of genitaledema, it was not significant (p=0.31).

The development of genital e<;lema afteruse of an external pump was not affected bythe type of compression pump utilized,whether single or sequential, the pressure

gradient (mmHg) used for treatment, theduration (months) of pump therapy, or thehours per day of pump treatment (Tables 3-6).

DISCUSSION

Patients with peripheral lymphedemaoften display multiple disabilities bothorganic and psychologic and those withgenital edema have major psycho/sexualsymptoms including severe depression.Scrotal, penile and vulval edema are oftenirreversible, respond poorly to treatment,and often are the site of externallympho­cutaneous fistulae, and, accordingly,represent a potentially serious complicationof lower extremity lymphedema.

Consideration of the basic anatomy ofthe lymphatic system illustrates the non­physiologic mechanism of pump treatment.Kubik demonstrated that the lymphaticsystem is made up of many regional"lymphotomes," which have minimalcollateral communications (26). Lymph fluidfrom these lymphotomes drains throughincreasingly larger lymphatic vessels to enterregional lymph nodes. If these lymph nodesare blocked, excised or fibrosed, the onlylymphatic pathways remaining aremarginally effective lymphatic collaterals.These collaterals can theoretically be opened

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TABLE 4Duration of Pnmp Therapy (Months) in

Lower Limb Lymphedema Correlated lvithOCcurrence of Genital Edema (GE)*

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TABLE 5Lack of Correlation of

Pnmp Pressure Gradient (mmHg) WithOccurrence of Genital Edema (GE)*

GE <1 1-3 4-12 12+ GE <40 40-80 >80 Unknown

(-) 8 (27%) 6 (13%) 5 (17%) 11 (33%)

(+) 6 (26%) 6 (26%) 3 (13%) 8 (35%)

*Not statistically significant

by Complex Lymphedema Therapy (CLT)(5-13) as suggested by lymphoscintigraphicimaging (3,7).

External pump compression may beeffective in "low protein" venous edemawhere tissue fluid is forced directly back intothe bloodstream. However, in lymphedema,the excess fluid is simply displaced into adja­cent lymphotomes. If this region is drained byobstructed or extirpated nodes, it seldom canaccommodate the excess lymphatic fluid andsurplus interstitial protein is left behind (27)as suggested by ragioimaging tracers (27,28).Whereas the flow of fluid may be enhanced,the residual protein in the tissues remainsand increased colloid;li osmotic pressureexerted by the proteins in the tissues causesthe edema to recur. Continual utilization of apump and/or high compression garments areneeded to restrict the recurrence of edemafluid. Often this approach is impractical anddoes not circumvent the problem caused bythe more proximal lymphatic blockade.Moreover, sustained high external pumppressures may damage remaining intactlymphatic vessels. Thus, pump pressures over45 mmHg m~y damage the initial lymphatics(29,30). Increased interstitial protein isassociated with progressive fibrosis and"chronic inflammation" so characteristic oflymphedema (2,31).

(-) 2 (9%) 7 (30%) 5 (22%) 9 (39%)

(+) 4 (13%) 8 (27%) 7 (23%) 11 (37%)

*Not statistically significant

Zelikovski, developer of the sequentialpump, Lymphapress, claims that a pump ismost useful in Grade 1 lymphedema andthat Grades 2 or 3 are less ideally suited forcompressive pump therapy (23). In thisinvestigation, 102 of 128 or 80% of thepatients had Grade 2 or 3 lymphedema.

Nowadays, a non-operative, safe, non­invasive treatment known as ComplexLymphedema Therapy (CLT) is commonlyused effectively to reduce peripherallymphedema (5-13,32,33). CLT consists ofmanual compression, external compressivebandaging, and specific physical therapyexercises. The pattern of both the lymphaticdrainage and the follow-up physical therapyexercises is based on specific collateralpathways determined for each individual atthe time of consultation. Factors to beconsidered to determine the collateralpathways include surgical scars (which mayblock lymph flow), areas of lymph nodaldissection, areas of skin and subcutaneoustissue fibrosis and risk for edema in thecontralateral limb. The regional lymphaticanatomy and collateral pathways betweenlymph drainage areas have been described byKubik (3). The successful reduction oflymphedema through CLT is based on thehypothesis of increasing lymphatic drainagethrough opening of collateral circulation from

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*Not statistically significant

the obstructed lymphotomes into normallyfunctioning lymphotomes. Unlike pumptherapy which has, to be utilized on acontinual basis, after a single course of CLT(averaging 5 days per week for 4 weeks),edema reduction is not only maintained,but further improves in compliant patients.Compliance consists of wearing compressivegarments and performing special physicaltherapy exercises 15-20 minutes, twice daily.

Lymphedema of the genital area is moredifficult to control because of the problem ofachieving adequate external compression tothis anatomical area. These sites are thusdifficult to bandage-wrap without causinguntenable functional problems with activitiesof daily living. At our facility, in conjunctionwith CLT, limited success has been achievedin reducing genital lymphedema. Patients arefitted with compression bike shorts over theirleg compression bandages. A good athleticsupporter for scrotal or penile edema in themales and a sanitary pad for edema of thevulva provides increased compression to theseareas. Special breathing exercises withmanual pressure in the abdominal and pelvicareas during forceful exhalation, mayincrease lymphatic flow and help reducesuprapubic and genital lymphedema. Patientsare taught how to perform these exercises inthe supine position with their legs elevated on

TABLE 6Lack of Correlation of

Hours per Day Compression WithOccurrence of Genital Edema (GE)*

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GE

(-)

(+)

6 (20%)

5 (22%)

1-4

16 (53%)

11 (48%)

5-12

8 (27%)

7 (30%)

a wedge and the buttock and genital areaselevated on pillows and rolls. Whereaspatients and therapists report a noticeabledecrease in the suprapubic and genitallymphedema utilizing these techniques,objective confirmation of edema reductionby measurement is problematic. Subjectively,patient complaints of pelvic "throbbing,""bursting," "congestion," and tolerance forsitting, standing and walking are improvedby compression treatment.

In patients with lower limb lymphedema,utilization of pneumatic compressive pumpsare significantly associated with the likelyd~velopment of genital edema. If pump treat­ment is to be administered for lower limblymphedema, one must give major consi­deration to the development of genital edemaespecially when an alternative, effective, safe,non-invasive option is available, namely, CLT.

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