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LYMPHOEDEMA OF OTHER SPECIAL SITES
Genital lymphoedema
Minor scrotal and penile lymphoedema can be tolerated without specific treatment, although support garments may be helpful. Severe scrotal oedema is best treated by excisional reduction surgery in which a large central segment is excised from the scrotum, preserving the spermatic cords and testicles. The flaps are then primarily sutured using an absorbable material and the scrotum is drained. Mobilization of the testes with gentle abrasion of their surfaces may encourage adhesions to form, allowing lymph to drain via the testicular lymphatics, aiding the scrotal reduction.
The penis may be reduced by simple excisional procedures, combined with circumcision if necessary. Alternatively the skin and subcutaneous tissue can be stripped off the deep fascia and split skin grafts applied (a Charles operation of the penis). Both scrotal and penile reduction operations produce gratifying results for surgeon and patient.
Massive labial swelling can also be treated by excisional procedures.
Eyelids and upper limb
Eyelid swelling can be treated by lid reduction. Arm swelling can be treated by a Homan's type of limb reduction, which can be performed on both the inner and outer sides of the upper limb. Patients with postmastectomy oedema must be assessed carefully to ensure that the venous drainage is satisfactory and to be certain that there is no evidence of recurrent axillary nodal disease. Both venous obstruction and recurrent malginancy are contraindications to arm reduction. Postoperatively an elasticated sleeve should be worn to try to prevent recurrent swelling.
Liposuction
Liposuction has been used to remove subcutaneous fat in patients with mild lymphoedema. Anecdotal successes have been achieved but the cosmetic results are variable and the procedure should be used with caution.
Chylous reflux
Some patients have dilated (almost varicose) valveless megalymphatics which allow the reflux of lymph (often chyle) against the expected direction of flow. These dilated lymphatics often end in cutaneous vesicles which are visible in the skin or which may rupture into body cavities such as the pleura, peritoneum, kidney, bladder, uterus, and vagina. Rupture results in the accumulation of lymph or chyle in the relevant cavity (chylothorax, hydrothorax, chylous ascites, chyluria) and chylous discharge on to the skin surface or mucosa can also occur. Accumulation of chyle in the pleural and peritoneal cavities produces severe symptoms, and patients often become dyspnoeic and very distended. Patients with megalymphatics often also have a protein-losing enteropathy which can cause weight loss and exacerbate accumulation of fluid in the body cavities and tissues. This results from leakage of lymph from the mucosal surface of the bowel; associated lymphatic leakage from the serosal surface may exacerbate the accumulation of ascites.
The diagnosis of chylous ascites or chylothorax must first be confirmed by aspiration of the fluid, which is then tested for chylomicrons. The condition may be suspected if there is pre-existing lymphoedema of the extremities and it is especially likely if vesicles and lymphatic leakage are present. However, in quite a few patients the condition develops de novo. Chylothorax and chylous ascites must be distinguished from malignant ascites or a malignant effusion: cytological examination of the aspirate may help to exclude or confirm the presence of malignant cells. CT scan and ultrasound can demonstrate the presence of moderate or severe enlargement of the abdominal or mediastinal lymph nodes which suggests the possibility of a lymphoma or secondary malignant spread. Guided biopsy, laparoscopy, or laparotomy may be necessary to confirm these diagnoses. Contrast lymphography demonstrates lymphadenopathy, filling defects, or the presence of megalymphatics and is indicated if the diagnosis remains in doubt. Contrast lymphography may also demonstrate a lymphatic leak which can be surgically sealed.
Lymphoedema associated with megalymphatics rarely requires reduction surgery, but the complications of lymphatic vesicles, recurrent infections, lymphatic discharge on to the skin, chylous ascites, chyluria, and chylothorax often demand treatment. Leakage of chyle or lymph may be prevented by ligating or under-running the dilated lymphatic channels, but this carries the risk of lymphatic obstruction which will worsen the limb swelling. Despite this many patients benefit from ligation of dilated lymphatics, and sealing off of any obvious site of fistulation.
If a patient with chylous ascites or chylothorax has no obvious leak on the lymphangiogram, chromium chloride studies and a barium study of the small bowel may provide useful information before a laparotomy is performed. At laparotomy the posterior abdominal wall over the main lymphatic pathways must be carefully inspected for the presence of lymphatic leakage, and the whole of the intestine should be examined. If the surface of the small bowel is grossly abnormal and leaking lymph, the involved or most abnormal segment should be resected. If this simple approach fails, consideration must be given to shunting the ascites back into the venous system using a LeVeen or Denver shunt. Although these shunts often work well in patients with refractory ascites, chyle often blocks the plastic tubing, or the valve, and produces an early occlusion of the shunt. Many patients improve with simple avoidance of fat and prescription of medium chain triglycerides combined with diuretics.
Chylothorax may respond to aspiration but often recurs and is best prevented by some form of pleurodesis induced with talc, tetracycline, bleomycin, or pleural stripping. After these procedures some patients die from water- or lymph-logged lungs as the lymphatics draining the lung become obstructed when they are no longer able to empty into the pleural cavity. Nevertheless many patients with severe problems as the result of megalymphatics can be helped by some of the procedures outlined above. Cutaneous vesicles may be simply excised or touched with the diathermy or cautery, but they tend to recur. Recurrent infections should be treated by a prolonged course of broad-spectrum antibiotics.
Lymphangioma circumscriptum
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