Management of lymphoodema Physical methods
The patient should elevate the foot above the level of the hip when sitting, elevate the foot of the bed when sleeping and avoid prolonged standing. Various forms of massage are effective at reducing oedema. Single- and multiple-chamber intermittent pneumatic compression devices ate also useful. In most clinics the mainstay of therapy is correctly fitted graduated compression hosiery. Pressures exceeding 50 mmHg at the ankle may be required to control oedema. Below-knee stockings are usually sufficient. The patient should put the stocking on first thing in the morning when the leg is at its least swollen. General advice regarding exercise and weight reduction, if necessary, is sensible.
Diuretics are of no value in pure lymphoedema. Their use is associated with side effects including electrolyte disturbance. The hydroxyrutosides are reported to be beneficial, as are the coumadins, but there are no scientifically robust data to support their use. Antibiotics should be prescribed promptly for cellulitis; penicillin V 500 mg four times daily for streptococcal infection and flucloxacillin 250 mg four times daily for staphylococcal infection are suitable. In severe cases there should be no hesitation in admitting the patient to hospital, elevating the limb and administering antibiotics intravenously. Antibiotics should be continued for at least 7 days or until all signs and symptoms have abated. Erythromycin is a reasonable alternative for those who are allergic to penicillin. In patients who suffer recurrent spontaneous episodes of cellulitis, long-term prophylactic antibiotic therapy may be indicated. Fungal infection (tinea pedis) must be treated aggressively; topical clotrimazole 1 per cent or miconazole 2 per cent used regularly is sufficient in most cases, but in refractory situations systemic griseofulvin 250—1000 mg daily may be required. The feet must be dried after washing and the skin kept clean and supple with water-based emollients to prevent entry of bacteria.
Only a small minority of patients with lymphoedema benefits from surgery. Operations fall into two categories: bypass procedures and reduction procedures.
In less than 2 per cent of patients with primary lymphoedema, lymphangiography will demonstrate proximal lymphatic obstruction in the ilio-inguinal region with essentially normal distal lymphatic channels. In theory at least, such patients might benefit from lymphatic bypass. A number of methods has been described, including the omental pedicle, the
skin bridge (Gillies), anastomosing lymph nodes to veins (Neibulowitz), the ileal mucosal patch (Kinmonth) and, more recently, direct lymphovenous anastomosis with the aid of the operating microscope. Although the last two techniques do appear to lead to significant improvement in about 50 per cent of patients, it is not possible to predict which patients will benefit. The procedures are technically demanding, not without morbidity and there is no controlled evidence to suggest that these procedures produce a superior outcome to best medical management alone.
Limb reduction procedures
These are indicated when a limb is so swollen that it interferes with mobility and livelihood. These operations are not ‘cosmetic’ in the sense that they do not create a normally shaped leg and are usually associated with significant scarring. Four operations have been described.
Sistrunk. A wedge of skin and subcutaneous tissue is excised and the wound closed primarily. This is most commonly employed to reduce the girth of the thigh.
Homan. Skin flaps are elevated and subcutaneous tissue is excised from beneath the flaps, which are then trimmed to size to accommodate the reduced girth of the limb and closed primarily. This is the most satisfactory operation for the calf. The main complication is skin flap necrosis. There must be at least 6 months between operations on the medial and lateral sides of the limb and the flaps must not pass the midline. This procedure has also been used on the upper limb but is contraindicated in the presence of venous obstruction or active malignancy.
Thompson. One denuded skin flap is sutured to the deep fascia and buried beneath the second skin flap (the so-called buried dermal flap). This procedure has become less popular as pilonidal sinus formation is common, the cosmetic result is no better than that obtained with Homan’s procedure and there is no evidence that the buried flap establishes any new lymphatic connection with the deep tissues.
Charles. This operation was initially designed for filariasis and involved excision of all the skin and subcutaneous tissues down to deep fascia with coverage using split skin grafts. This leaves a very unsatisfactory cosmetic result and graft failure is not uncommon. However, it does enable the surgeon to reduce greatly the girth of a massively swollen limb.
Other excisional surgery
Scrotal, penile and labial lymphoedema may be highly symptomatic causing embarrassment, preventing intercourse and impeding micturition. Minor swelling may be treated with support hosiery hut severe swelling is best treated with excisional surgery. Lymphoedema of the eyelid may be treated by lid reduction.
Chylous ascites and chylothorax
The diagnosis may be obvious if accompanied by lymphoedema of an extremity, especially if the latter is associated with vesicles. However, some patients develop chylous ascites and/or chylothorax in isolation, in which case the diagnosis can be confirmed by aspiration and the identification of chylomicrons in the aspirate. Cytology for malignant cells should also be carried out. CT scan may show enlarged lymph nodes, and CT with guided biopsy, laparoscopy or even laparotomy and biopsy may be necessary to exclude lymphoma or other malignancy. Lymphangiography may indicate the site of a lymphatic fistula which can be surgically ligated. Even if no localised lesion is identified, it may be possible to control leakage at laparotomy or even remove a segment of affected bowel. If the problem is too diffuse to be corrected surgically, a peritoneal venous shunt may be inserted, although occlusion and infection are important complications. Medical treatment comprising the avoidance of fat in the diet and the prescription of medium chain triglycerides (which are absorbed directly into the blood rather than via the lymphatics) may reduce swelling. Chylothorax is best treated by pleurodesis with either bleomycin, talc, pleural stripping or tetracycline. In some cases this leads to death from lymph-logged lungs as the excess lymph has nowhere to drain.
Filariasis is the most common cause, with chyluria occurring in 1—2 per cent of cases 10—20 years after initial infestation. It usually presents as painless passage of milky white urine, particularly after a fatty meal. The chyle may clot leading to renal colic and hypoproteinaemia may result. A clot forms in the urine on standing which does not dissolve on shaking with an equal amount of ether. The urine contains chylomicrons, and oral ingestion of fat with Sudan Red turns the urine pink. Chyluria may also be caused by ascariasis, malaria, tumour and tuberculosis, and the differential diagnosis includes gross pyuria, phosphaturia and caseous material from tuberculosis. Intravenous urography and/or lymphangiography will often demonstrate the lymphourinary fistula. Treatment includes a low-fat and high-protein diet, increased oral fluids to prevent clot colic, and laparotomy and ligation of the dilated Iymphatics. Attempts have also been made to sclerose the lymphatics either directly or via instrumentation of the bladder, ureter and renal pelvis.
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