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.
Drugs
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.
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
Thompson.
One denuded skin flap is sutured to the deep fascia and buried beneath the
second skin flap (the so-called buried dermal flap) (Fig.
17.16). 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 (Fig. 17.17). 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.
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.