I

Fig. 11.1.5 Isotope lymphangiogram showing enhanced uptake of isotope in enlarged left pelvic lymph nodes

Infection

• Filariasis is the commonest secondary cause of lymphoedema and is endemic in many tropical areas, particularly in Indonesia, India and China.

• The worm Wuchereria bancrofti enters the body in mi-crofilarial form through insect bites.

• The worm lodges in lymph nodes, where it matures and starts producing microfilariae which are released into the bloodstream and are thence transmitted back to biting insects, so completing the cycle of reproduction.

• The adult worm induces fibrosis within the lymph node, obstructing lymph flow. Even if the parasite is destroyed, the lymph node changes are irreversible and the lymphoedema does not resolve.

• Elephantiasis also occurs in many tropical areas where filariasis is not endemic, such as parts of East Africa.

• Some of these patients may have tuberculosis, with caseation and destruction of lymph nodes.

• Some may just have repeated episodes of infection and inflammation in the lymph nodes as a result of walk-

ing around barefoot and suffering repeated minor injuries and soft tissue infections. • The recurrent infections induce fibrosis of the lymph nodes, with subsequent lymphatic obstruction.

11.1.4 Symptoms

• Patients note a gradual onset of swelling, starting distally and gradually spreading proximally (Fig. 11.1.2a).

• Lymphoedema is not confined solely to the limbs and some patients may present with genital swelling or with involvement of the intestine or pleura causing diarrhoea, chylous ascites or pleural effusions due to leakage of lymph.

• In the lower limb, where the condition is most common, swelling above the knee is unusual. The swelling subsides at night initially, when the leg is elevated, but through a gradual process of subcutaneous fibrosis the swelling becomes more established and permanent with less pitting on finger pressure.

• In severe cases, particularly those due to megalym-phatics, cutaneous vesicles develop and discharge lymphatic fluid with surrounding excoriation and crusting (Fig. 11.1.6).

• The skin may become coarse and pitted and the patients may become susceptible to repeated episodes of cellulitis, both from infection entering the limb through the damaged skin and from the loss of function of the regional lymphatics in defence against infection.

• Rarely patients with longstanding swelling may develop lymphangiosarcoma in the affected limb, with purple red nodules on the skin.

11.1.5 Diagnosis

11.1.5.1 History and Examination

• A careful medical history and examination will establish the likelihood of many of the conditions listed in Table 11.1.1.

• The history should include enquiry about the age of onset and whether any family members are also affected.

• Secondary causes might be suspected if there is a history of malignant disease or radiotherapy or if the pa

Leg Edema Vesicles
Fig. 11.1.6 Unilateral lymphoedema with skin vesicles and crusting. The patient suffered recurrent episodes of cellulitis in the left leg

tient has travelled to a tropical country, particularly where filaria is endemic.

The patient should be asked about complications, including recurrent cellulitis, discharging vesicles, diarrhoea and whether or not the swelling interferes with quality of life or walking, especially if the thighs are involved and rub together because of the swelling. On examination, the extent of the swelling should be noted and circumference measurements made in relation to fixed bony points, to allow future comparisons.

Regional lymph nodes should be palpated for any enlargement or suspicious features and, in the case of lower limb oedema, the abdomen and pelvis should be carefully palpated for masses, as well as being examined for ascites.

In older patients, the prostate or cervix should be examined for signs of malignancy. The skin of the limb needs to be carefully inspected and the web-spaces between the digits inspected for evidence of fungal infection such as athletes' foot. A finding of nonpitting oedema, particularly if it appears chronic and has developed slowly over a prolonged period of time, raises the clinical suspicion of lymphoedema. Further investigation is needed if the diagnosis is uncertain, to rule out other treatable causes of limb oedema and to aid decision making in the few patients with lymphoedema where surgery is being considered.

11.1.5.2 Laboratory Tests

• Measurement of serum prostate-specific antigen (PSA) may help to rule out prostate malignancy in male patients.

• Liver function tests and creatinine can be used if there is suspicion of hypoproteinaemia, liver or renal fail-

11.1.5.3 Imaging

• Most venous abnormalities can be excluded by Duplex ultrasonography, which may reveal deep or superficial venous reflux and can demonstrate venous obstruction by a lack of flow in, and incompressibility of, a vein.

• Ultrasound, CT or MR scans of the abdomen and pelvis may demonstrate extrinsic compression of the pelvic veins or enlargement of intra-abdominal lymph nodes.

• Image-guided needle aspiration cytology of a mass or an enlarged lymph node is also possible with these modalities and is the method of choice for obtaining histological diagnosis (Fig. 11.1.7), as removal of a node may aggravate the oedema.

• Contrast lymphography has nowadays been replaced by isotope lymphangiography [4] in the routine investigation of lymphatic drainage from the limb.

• A gamma-emitting isotope such as rhenium is injected subcutaneously in both feet and a gamma camera is used to record the speed of uptake of the isotope into the groin and pelvic lymph nodes. Patients with

Fig. 11.1.7 CT-guided needle aspiration of left pelvic mass, which turned out to be metastatic prostate cancer replacing a lymph node. The mass is compressing the left external iliac vein, causing left leg swelling from a mixture of lymphatic and venous obstruction

lymphoedema will show delayed or absent uptake of the isotope on the affected side (Fig. 11.1.4).

• Where a patient is being considered for lymphatic bypass surgery, the more detailed images of lymphatic vessels obtained with contrast lymphography help with the careful case selection needed, but the technique requires skill and patience to undertake open cannulation of a lymphatic vessel on the dorsum of the foot.

11.1.6 Treatment

• Most patients with lymphoedema can be managed conservatively.

• Reconstructive surgical options are very limited, with few patients being suitable and with uncertain results.

• Limb reduction surgery is disfiguring and so is reserved only for the most severely affected patients.

11.1.6.1 Conservative Treatment

• Most patients have modest, nonprogressive limb swelling which does not give rise to complications such as recurrent cellulitis but can cause concern about the cosmetic appearances.

• The mainstay of treatment in such patients is a combination of elevation and compression stockings [11].

Patients should sleep with the limb elevated to assist fluid drainage overnight. Graduated compression stockings need to be applied before getting out ofbed in the morning to try to maintain any reduction in swelling. The stockings need to apply a minimum of 30 mmHg pressure at the ankle.

• If the swelling is confined to the below-knee area then knee-length stockings are acceptable and are more likely to be complied with in men.

• More severe limb swelling may respond to a 2- to 6-week course of complex decompressive therapy, which involves careful skin cleansing, nail care and the application of simple skin creams to avoid infection setting in through skin cracks.

• Specially trained practitioners massage the affected limb using the technique of manual lymphatic drainage and then apply multi-layer compression bandaging each day.

• Patients are encouraged to exercise gently each day and to lose weight.

• Any improvement is maintained after a course of treatment by the use of graduated compression stockings and the course may need to be repeated after 6-12 months.

• A variety of commercially available external compression devices are available, which the patient wears in bed at night. The devices use a pneumatic pump to alternately compress and relax a knee- or thigh-length boot around the leg. Patients use the devices in bed at night and apply compression stockings during the day. Sleep can be difficult with the pump working and there have been no convincing studies of benefit in lymphoedema.

• Other forms of lower-limb pitting oedema may respond better to these devices than the more fixed swelling of lymphoedema.

11.1.6.2 Surgery

• Surgical options fall into two categories, namely operations designed to by-pass lymphatic flow around an obstructed part of the system or those designed to reduce the bulk of the limb. Because the former is not always successful and the latter causes a lot of scarring, such operations are reserved for those with recurrent cellulitis or lymphangitis or those in whom walking is impaired because of the heaviness or the bulk of the limb, which causes the thighs to rub together.

Lymphatic By-pass

• Attempts at lymphatic by-pass have been made by directly anastomosing bivalved lymph nodes to adjacent veins or by microanastomosis of dilated distal lymphatic vessels directly onto adjacent veins but the outcomes of these procedures have been poor.

• An alternative approach is to use pedicles of skin, omentum or mesentery which originate proximal to the lymphatic obstruction and are anastomosed to lymph nodes distal to it.

• Of these the mesenteric bridge has proved most successful, although it only helps around 50% of carefully selected patients [7].

• The procedure is reserved for patients with primary lymphoedema due to proximal obstruction, where the lymphatics above the pelvis and groin are normal and the lymphatics in the thigh are patent within reach of the mesenteric pedicle.

• Only 20% of patients with primary lymphoedema have proximal obstruction of this nature and a proportion of them will have obliterated proximal thigh lymphatics, so the operation is only suitable for a few, carefully selected patients with lymphoedema.

• The mesenteric by-pass is fashioned by resecting a 5-cm segment of terminal ileum on its mesentery, opening it along its antimesenteric border and stripping off the mucosal surface to expose the submucosal lymphatics of the bowel.

• The mesenteric pedicle is then tunnelled down to the groin and the stripped mucosal surface is sutured across the cut surface of bivalved lymph nodes below the obstruction.

• Results are better in younger patients, especially if the limb swelling is not excessive.

Limb Reduction

• In patients where walking is impaired because the thighs rub together and the leg is too heavy to lift or where the limb swelling is excessive and associated with recurrent cellulitis then a limb reduction operation may be considered.

• Several variants are described but only two are in common use today, the Charles reduction and the Homans procedure [9].

• In patients where there is extensive skin damage in the limb below the knee, the Charles reduction replaces all of the skin and subcutaneous fat from the knee to the ankle with split skin grafts to cover the deep fascia overlying the muscle and the tibial periosteum. The cosmetic result is poor, although there is good reduction in limb size.

It is important to retain the patient's skin over the knee itself, so that they avoid kneeling on the thin split skin graft.

It is also more cosmetically acceptable if the skin and subcutaneous tissues at the top and bottom of the denuded area are tapered in towards the skin grafts by undermining the cut edges of skin and excising some of the bulky subcutaneous tissues underneath. The Charles operation in the calf can be combined with a Homans operation in the thigh, or the Homans can be used both above and below the knee if the skin is healthy.

In the Homans operation, the medial side of the thigh or calf is incised vertically, with horizontal T-shaped incisions at the top and bottom of the vertical incision.

Skin flaps are then lifted anteriorly and posteriorly with an underlying layer of subcutaneous fat. It is possible to raise the flaps across to the midline of the limb without damaging the blood supply to the flaps, although care should be taken to preserve any vessels entering the flaps at the limits of their dissection.

The underlying thickened subcutaneous tissue is then excised down to the deep fascia and the skin flaps laid back down on the new fascial bed and sutured back together.

This operation gives a better cosmetic result than the Charles reduction, although the edges of the flaps may undergo necrosis, eventually healing with more prominent scars.

The lateral side of the limb can be tackled in a similar fashion after an interval to ensure full healing of the medial side.

Where debulking of the medial aspect of the thigh is all that is needed, a simpler alternative to the Homans operation is that described by Sistrunk. A vertical wedge of skin and underlying subcutaneous tissue is excised down to muscle fascia and the skin wound is then closed primarily. This may be used to help taper the thigh down towards a Charles excision and skin grafting area of the calf to prevent a pantaloon effect or may be all that is needed where the patient has trouble walking because the thighs rub together. None of these procedures is problem free, with the risk of wound breakdown and excessive scarring.

• They should be viewed as a palliative procedure to achieve a functional result such as improved walking or reduced episodes of cellulitis and they are not indicated for cosmetic reasons alone.

• Surgery should be avoided in patients with secondary lymphoedema due to metastatic disease, as there is often an element of venous obstruction on top of the lymphatic obstruction and the results are poor.

References

1 Brice G, Mansour S, Bell R, Collin JRO, Child AH, Brady AF, Sarfarazi M, Burnand KG, Jeffery S, Mortimer P, Murday VA (2002) Analysis of the phenotypic abnormalities in lymph-oedema-distichiasis syndrome in 74 patients with FOXC2 mutations or linkage to 16q24. J Med Genet 39:478-483

2 Browse NL, Stewart G (1985) Lymphoedema: pathophysiology and classification. J Cardiovasc Surg 26:91-106

3 Burnand K, McGuiness L (2000) Abnormalities of the lymphatic system. In: Morris PJ, Wood WC (eds) Oxford textbook of surgery. Oxford University Press, Oxford, pp 1063-1076

4 Burnand KG, McGuinness CL, Lagattolla NR, Browse NL, El-Aradi A, Nunan T (2002) Value of isotope lymphogra-phy in the diagnosis of lymphoedema of the leg. Br J Surg 89:74-78

5 Fang J, Dagenais SL, Erickson RP, Arlt MF, Glynn MW, Gorski JL, Seaver LH, Glover TW (2000) Mutations in FOXC2 (MFH-1), a forkheadfamily transcription factor, are responsible for the hereditary lymphedema-distichiasis syndrome. Am J Hum Genet 67:1382-1388

6 Ferrell RE, Levinson KL, Esman JH, Kimak MA, Lawrence EC, Barmada MM, Finegold DN (1998) Hereditary lymphedema: evidence for linkage and genetic heterogeneity. Hum Mol Genet 7:2073-2078

7 Hurst PA, Stewart G, Kinmonth JB, Browse NL (1985) Long term results of the enteromesenteric bridge operation in the treatment of primary lymphoedema. Br J Surg 72:272-274

8 Kinmonth JB (1972) The lymphatics. Disease, lymphography and surgery. Arnold, London

9 Kinmonth JB, Patrick JH, Chilvers AS (1975) Comments on operations for lower limb lymphoedema. Lymphology 8:56-61

10 Wolfe JHN (1984) The prognosis and possible cause of severe primary lymphoedema. Ann R Coll Surg Engl 66:251-257

11 Wolfe J (1989) The management of lymphoedema. In: Rutherford RB (ed) Vascular surgery. Saunders, Philadelphia, pp 1648-1677

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