Latest Treatments for Lymphedema

Lymphoedema Clinics Keyworker role within the clinic

Adequate provision will need to be made to offer existing and newly diagnosed patients with lymphoedema a maintenance service. Comprehensive assessment of patients with lymphoedema unique to their needs. Measurable tools will be included within the assessment process to monitor clinical effectiveness and contribute to ongoing audit of the service. These will include 4 cm cylindrical limb volume measurements, skin and subcutaneous tissue score, pain and psychological visual analogue scales (VAS), distribution of swelling, function and movement. Screening tools for onward referral to the lymphoedema specialist for intensive treatment are also implemented. Due to the chronic nature of lymphoedema a key-worker is in a position to monitor long-term care and refer onwards to the appropriate specialist as necessary. The key-worker has not undertaken specialist treatment interventions, therefore is not in a position to offer intensive treatment sessions, e.g. manual lymphatic drainage (MLD)...

A Management of Upper Extremity Lymphoedema with Microsurgical Lymphovenous Anastomosis

Initially, the oedema had a rhizomelic location. It was hard to the touch without pitting oedema. There were no dystrophic or dyschromic skin lesions, except for signs of acute reticular erysipeloid lymphangitic attacks caused by Gram-positive Staphylocci infections promoted by lymph stasis. A lymphan-gioscintigram was performed, which showed features compatible with lymphatic circulatory impairment in the left arm (Fig. 47a.2). This was followed by lym-phangio-magnetic resonance imaging (MRI) of the left arm and hemithorax, which showed no signs of locoregional relapse of breast cancer but confirmed lymph stasis, predominantly in the epifascial compartment. In addition, it showed dilated medial arm lymphatic collectors interrupted at the proximal third of the arm. Finally, echo-Doppler of the left subclavian and axillary venous axis was performed. This did not demonstrate any venous dysfunction. A diagnosis of chronic secondary lymphoedema of the left arm following breast cancer...

Managing Lymphoedema A Clinic Approach

Lymphoedema is a progressive chronic swelling of the limbs and the body that occurs as a result of an inadequate compromised lymphatic system. Lymphoedema is classified as either primary or secondary Primary lymphoedema Is due to underdevelopment of the lymphatic system. Three different types have been recognised. - Congenital lymphoedema (Milory's disease) Present at or within a year of birth. It is more common in males and more likely to be bilateral and involve the whole leg. - Lymphoedema praecox Appears during puberty and is the most common form of primary lymphoedema. It is more common in females and more likely to be unilateral and only extends to the knee. - Lymphoedema tarda Appears later in life, i.e. from 30 to 40 onwards. It is important that when lymphoedema develops for the first time in later life, an underlying malignancy is excluded. Secondary lymphoedema Occurs when the lymphatic system becomes impaired following surgery and or radiotherapy (as in cancer treatment),...


The lymphatic system is a significant pathway for the drainage of fluid, large protein molecules and white blood cells from the interstitial spaces within capillary beds. Lymphatic capillaries lie in the dermis and subcutaneous fat, in the fascial planes between muscles and in perivascular tissues. Lymphatic vessels transport lymph from these areas through regional lymph nodes towards the thoracic duct. Lymph returns to the venous circulation via the termination of the thoracic duct at the left internal jugular vein close to its junction with the left subclavian vein. The system plays a major immunologi-cal role, with the regional lymph nodes processing antigens presented to them by white blood cells from the peripheries. Antigen-specific lymphocytes proliferate in the lymph nodes and are then released into the circulation via the main lymph ducts and also through small lym-phovenous connections within the lymph nodes themselves. Impaired drainage of lymph from a limb, usually due to...

Primary Lymphedema

It has been proposed that all cases of primary lymphedema are due to an inherited abnormality of the lymphatic system, sometimes termed congenital lymphatic dysplasia. However, it is possible that many sporadic cases of primary lymphedema occur in the presence of a (near) normal lymphatic system and are actually examples of secondary lymphedema for which the triggering events have gone unrecognized. These might include seemingly trivial (but repeated) bacterial or fungal infections, insect bites, barefoot walking (silica), DVT, or episodes of superficial thrombophlebitis. In animal models, simple excision of lymph nodes or trunks leads to acute lymphedema that resolves within a few weeks, presumably due to collater-alization. In animals, the human condition can only be mimicked by inducing extensive lymphatic obliteration and fibrosis. Even then, there may be considerable delay between the injury and the onset of edema. Primary lymphedema is much commoner in the legs than the arms....

Granuloma Inguinale Donovanosis

A vulvar nodule breaks down, forming a painless, beefy red, highly vascular ulcer with fresh granulation tissue without regional lymphadenopalhy. Lymphatic obstruction can result in marked vulvar enlargement. Chronic scarring can lead to lymphatic obstruction.

Sex Chromosome Aneuploidy

Turner syndrome (45 X) affects approximately 1 in 2,500 live-born females. Features include reduced stature, webbed neck, lymphedema of the ankles and wrists (especially at birth), diminished spatial perception, shield-shaped chest with widely spaced nipples, and sterility as a result of gonadal-dysgenesis. Although Turner syndrome is relatively uncommon among live births, it is found in approximately 2 of conceptions. Thus, more than 9-9 of Turner conceptions are spontaneously aborted. Among those who survive to term, mosaicism-is quite common (i.e., the presence of more than one genetically distinct cell type). The most common mosaicism in Turner syndrome is the presence of some 45 X cells and some normal 46,XX cells. Turner females with 45,X 46,XY mosaicismare highly susceptible to gonadoblastorna. Mosaicism is an occasional feature of many chromosome abnormalities (Down syndrome, trisomies 13 and 18) and is sometimes associated with milder expression of the disease.

Clinically Significant or Cytologically Confirmed Groin Nodes

Lower extremity or penoscrotal lymphedema seen in nearly one third of patients. In light of this, a lot of attention has recently focused on reduction of morbidity by modification of the surgical procedure. reported no skin necrosis, infection, or deep vein thrombosis, and only moderate lymphedema in four patients at a follow up of 6 to 104 months in his series of 10 patients. At the Tata Memorial Hospital, we practice routine excision of the skin overlying the inguinal nodal area in all patients undergoing radical ilioinguinal lymphadenectomy, even when the skin is not infiltrated by the nodal disease and we perform immediate reconstruction using a tensor fascia lata myocutaneous flap or anterolateral thigh flap 70,77 . We have had no major problems of skin loss or wound breakdown since the time we began employing this procedure. In addition, the incidence of lower extremity lymphedema has also been significantly reduced with a long follow-up in these patients. With the majority of...

Environmental Strictures

Balanitis Xerotica Obliterans

Although direct radiation to the genitalia is uncommon, occasionally a patient will present with a small (2- to 3-cm), superficial, noninvasive, malignant lesion of the glans penis or coronal sulcus, who elects small-field electron beam radiotherapy vs partial penectomy. Although there are generally few problems with these patients, others who have had direct radiation exposure to the penis can present with progressive gangrene of the genitalia, requiring debridement and, in some cases, partial penectomy. In addition, radiotherapy to the pelvis can cause chronic lymphedema, with the patients often presenting with hydroceles as well. In contrast to direct radiation exposure, chronic lymphedema is considered a highly treatable condition, usually involving excision of the lymphedematous tissue and primary reconstruction.

Lymphogranuloma Venereum

The iliac or perirectal nodes and may present as lower back or abdominal pain. Involvement of the iliac lymph nodes can result in pelvic adhesions. Complications include rectal strictures, stenosis, perianal abscesses, fistulas, proctocolitis, and perianal outgrowths of lymphatic tissue. If untreated, the disease can progress to genital elephantiasis, chronic lymphatic obstruction, worsening strictures and fistulas, or a frozen pelvis.

Soft Tissue Infections

GAS may invade the epidermis and subcutaneous tissues, resulting in local swelling, erythema, and pain.17 The skin becomes indurated and, in contrast to erysipelas, is a pinkish color. Patients with lymphedema secondary to lymphoma, filariasis, or surgical node dissection (mastectomy, carcinoma of the prostate, etc.) are predisposed to development of GAS cellulitis, as are those with chronic venous stasis and superficial dermatophyte infection of the toes. Saphenous vein donor site cellulitis may be due to group A, C, or G streptococci. Cellulitis associated with a primary focus (e.g., an abscess or boil) is more likely caused by S. aureus. Aspiration of the leading-edge and punch biopsy yield a causative organism in 15 and 40 of cases, respectively. Patients respond quickly to penicillin, though in some cases where staphylococcus is of concern, nafcillin or oxacillin may be a better choice, or one may need cover for methicillin-resistant S. aureus (MRSA) infection (discussed later in...

Complications of Ilioinguinal Lymphadenectomy

Wherein to avoid damage to the vessels of the groin region that run parallel to the inguinal ligament and lie in the fat of the superficial layer of the superficial fascia, dissection is done beneath this layer (deep to Scarpa's fascia), he saphenous nerve is preserved and the sartorius is left in situ so as not to disturb the collateral lymphatic drainage. He reported no skin necrosis, infection, or deep vein thrombosis, and only moderate lymphedema in four patients at a follow up of 6 to 104 months in his series of 10 patients. At the Tata Memorial Hospital, we practice routine excision of the skin overlying the inguinal nodal area in all patients undergoing radical ilioinguinal lymphadenectomy, even when the skin is not infiltrated by the nodal disease and we perform immediate reconstruction using a tensor fascia lata myocutaneous flap or anterolateral thigh flap 70,77 . We have had no major problems of skin loss or wound breakdown since the time we began employing this procedure....

Atrophic Vaginitis And Serosanguineous Discharge

The answer is a. (DiSaia, 5le, pp 41-42.) Lichen sclerosus was formerly termed lichen sclerosus et atrophicus, but recent studies have concluded that atrophy does not exist. Patients with lichen sclerosus of the vulva tend to be older they typically present with pruritus, and the lesions are usually white with crinkled skin and well-defined borders. The histo-logic appearance of lichen sclerosus includes loss of the rete pegs within the dermis, chronic inflammatory infiltrate below the dermis, the development of a homogenous subepithelial layer in the dermis, a decrease in the number of cellular layers, and a decrease in the number of melanocytes. Mechanical trauma produces bullous areas of lymphedema and lacunae, which are then filled with erythrocytes. Ulcerations and ecchymoses may be seen in these traumatized areas as well. Mitotic figures are rare in lichen sclerosus, and hyperkeratosis is not a feature. While a significant cause of symptoms, lichen sclerosus is not a...

Definition and Pathophysiology

Lymphedema may be defined as abnormal limb swelling due to the accumulation of increased amounts of high-protein ISF secondary to defective lymphatic drainage in the presence of (near) normal net capillary filtration (Szuba and Rockson, 1997). In order for edema to be clinically detectable, the ISF volume has to double. About 8L of lymph is produced and, following resorption in lymph nodes, about 4L enters the venous circulation. In one sense, all edema is lymphedema in that it results from an inability of the lymphatic system to clear the ISF compartment. However, in most types of edema this is because the capillary filtration rate is pathologically high and overwhelms a normal lymphatic system, resulting in the accumulation of low-protein edema fluid. By contrast, in true lymphedema, capillary filtration is normal and the edema fluid is relatively high in protein. Both mechanisms frequently coexist, as in patients with CVI. Lymphedema can result from lymphatic aplasia, hypoplasia,...

Treatment Of Systemic Nematodes

Diethylcarbamazine, a piperazine derivative, is well absorbed orally and has a half-life of 8 hours. The parent drug and its metabolites are excreted through the kidney. Side effects include those attributable directly to the drug and those that result from the release of parasite antigens and Wolbachia lipopolysaccharide. Side effects include nausea, vomiting, anorexia, headache, malaise, weakness, arthralgias, and, rarely, acute psychotic reactions. In patients with W bancrofti or B. malayi infection, localized swelling or nodules may develop along lymphatics during treatment, or there may be transient lymphedema or hydrocele formation. Diethylcarbamazine is no longer used in patients with onchocerciasis because it can elicit the Mazzotti reaction, which is characterized by hypotension, pruritus, fever, tachycardia, wheezing, chorioretinitis, and uveitis secondary to the release of microfilarial antigens and Wolbachia lipopolysaccharide. Life-threatening encephalopathy has been...

Clinical Features

Disseminated cutaneous leishmaniasis or tegumentary (nodular disseminated) leishmaniasis, tends to cover the entire body, but mucosae involvement is infrequent. It is caused by several species and subspecies L. m. amazonensis, L. aethiopica and in Venezuela by L. mexicana (L. m. pifanoi). It usually involves the exposed areas, the pinna of the ears, cheeks, ciliary regions and extremities. The folds and hairy skin are usually free of involvement (Fig. 46.4). It is characterized by firm, gray-red, smooth or verrucous nodules and plaques that can ulcerate. Lymphedema, lymphadenopathy, malaise and fever may be present. Mucocutaneous leishmaniasis (American leishmaniasis or espundia) is commonly limited to South America. It affects youngsters and is caused by L. braziliensis braziliensis, L. aethiopica and L. mexicana. There is a primary cutaneous lesion at an exposed site, generally on the lower extremities. It is nodular and can ulcerate or assumes a...

B Dengue Fever Dandy Fever Breakbone Fever

For the febrile and local inflammatory occurrences, bed rest is indicated. For secondary infections (abscesses over inflamed nodes), antibiotics should be administered. A palliative measure used for orchitis (inflammation of the testis), epididymitis (inflammation of the elongated cordlike structure along the posterior border of the testis), and scrotal lymphedema, a suspensory bandaging may be used. Mild edema of a limb may be treated with rest, elevation, and by an elastic stocking. In cases of elephantiasis (inflammation and enlargement of the part), the surgical removal of the breast, scrotum or vulva is easy and usually satisfactory. Limb elephantiasis is not easy and meets with disappointing results. The drug of choice is diethylcarbamazine which is given orally 3 times daily after meals for 21-28 days. The dosage is usually 2 mg of citrate per body weight. The microfilaria are destroyed however, there is only limited action on the adult worms. If the victims...

Subacute Bacterial Endocarditis

Etiologic agents in adults Haemophilus influenzae infection is common in children. Patients with chronic venous stasis and lymphedema of any cause (lymphoma, filariasis, post-regional lymph node dissection, radiation therapy) are predisposed recently, recurrent sap he nous-vein donor-site cellulitis was found to be attributable to group A, C, or G streptococci.

Specific Discussion

The chest x-ray shows large pulmonary arteries, and this, coupled with the clinical scenario, is consistent with primary pulmonary hypertension (PPH). As mentioned in the general discussion, this entity is due to an unknown cause. The physical sign most likely to be present would be a loud P2, and right heart catheterization would confirm the high pulmonary artery (PA) pressures. Patients with PPH may give a history of syncopal episodes. A loud A2 is heard in systemic hypertension, and rib notching is classically seen on the x-ray in coarcta-tion of the aorta. Right arm swelling is seen with either a localized vascular or lymphatic obstruction such as postradiation, malignancy, or superior vena cava syndrome. This patient does not exhibit any of these signs. Since the hilar shadows are of vascular nature, mediastinoscopy or bronchoscopy would not be indicated and in fact may be dangerous if PA pressures are very high.

Prosthetic Valve Endocarditis

Cellulitis is an acute spreading infection of the skin that predominandy affects deeper subcutaneous tissue. Group A streptococci and S. aureus are the most common etiologic agents in adults H. influenzae infection is common in children. Patients with chronic venous stasis and lymphedema of any cause (lymphoma, filariasis, post-regional lymph node dissection) are predisposed recently, recurrent saphenous-vein donor-site cellulitis was found to be attributable to group A, C, or G streptococci.

Lyme Disease

Imaging Lymphangiogram partial lymphatic obstruction at iliac level. Discussion Lymphatic filariasis is a chronic disease that is due to lymphatic obstruction and is caused by several types of filarial roundworms, mainly Wuchereria bancrofti and Brugia malayi it is transmitted by female mosquito bites. .Also known as elephantiasis.

The Lymphatic System

The distribution of fluid and protein between the vascular and ISF spaces depends on hydrostatic and oncotic pressures (Starling's forces), together with the relative impermeability of the blood capillary membrane to molecules over 70 kd. In healthy subjects there is net capillary filtration, which is removed by the lymphatic system. Small particles enter the initial lymphatics directly larger particles are phagocy-tosed by macrophages and transported through the lymphatic system intracellularly. Lymph flows against a small pressure gradient due to transient increases in interstitial pressure secondary to muscular contraction and external compression, the sequential contraction and relaxation of lymphangions, and the prevention of reflux due to valves. Lymphangions respond to increased lymph flow in much the same way as the heart responds to increased venous return in that they increase their contractility and stroke volume. Transport in the main lymph ducts also depends on...


It is usually possible to diagnose and manage lymphedema purely on the basis of the history and examination, especially when the swelling is mild and there are no apparent complicating features. In patients with severe, atypical, and multifactorial swelling, investigations may help confirm the diagnosis,inform management, and provide prognostic information. A full blood count, urea and electrolytes, creatinine, liver function tests, chest radiograph, and blood smear for microfilariae may be indicated. Direct lymphangiography involves the injection of contrast medium into a peripheral lymphatic vessel and subsequent radiographic visualization of the vessels and nodes. It remains the gold standard for showing structural abnormalities of larger lymphatics and nodes. However, it can be technically difficult, is unpleasant for the patient, may cause further lymphatic injury, and has largely become obsolete as a routine method of investigation. Indirect lymphangiog-raphy involves the...

Nonsurgical approach

Lymphoedema is an excess of water, large proteins and waste material, which has accumulated in the tissues. This is an ideal environment for bacteria to manifest. In some cases, the lymph nodes may have been removed, therefore, the local immune system may be compromised in the swollen area. All of these factors contribute to the increased risk of developing an AIE (cellulitis). Taking care of your skin, minimises the increased risk of infection, which often accompanies lymphatic disorders. To strengthen the muscles of the limb to help avoid any muscle wastage often seen in long-standing lymphoedema. It is advisable to wear rubber gloves to aid application making it much easier to grip and adjust the garment. Compression garments are tight, therefore quite difficult to apply. If you have problems, contact your lymphoedema therapist as they may have some aids, which may ease application. You will be supplied with two sleeves from your lymphoedema therapist, one to wear and one to wash....

Clinical Evaluation

The LM component in this patient was treated only with complex decongestive therapy (CDT) in order to prevent full development of lymphedema. The infiltrating ET form of LM detected together with the ET form of VM has been shown to put extra burden on the marginally normal lymph-conducting system on lymphoscinti-graphic evaluation. Therefore, continuous surveillance for aggressive preventive measurement of local to systemic cellulitis along this ET form of LM lesions is mandated.

Turner Syndrome Xo

Turner syndrome occurs in 1 5000 newborns. It has a sporadic occurrence. Usually it is the paternal X chromosome that is missing. All patients have short stature and gonadal dysgenesis. Findings include a tendency to obesity, congenital lymphedema, broad (shield) chest, and widely spaced nipples. Patients have a low posterior hairline and a webbed neck (pterygium coli). Skeletal manifestations include cubitus valgus and hip dislocation. Skin findings include narrow hyperconvex nails, pigmented nevi, and a tendency to form keloids. Bicuspid aortic valves, coarctation of the aorta, and valvular aortic stenosis are cardiac anomalies. Horseshoe kidneys may be seen. Occasionally, patients have blue sclera, cataracts, mental retardation, Crohn disease, or thyroid disorders. The congenital lymphedema resolves. Patients do not achieve an adolescent growth spurt, reaching a final height of 55 inches. Estrogen replacement is indicated.

Clinical Assessment

In most cases the diagnosis of primary or secondary lymphedema can be made, and the condition differentiated from other causes of a swollen limb, on the basis of history and examination without recourse to complex investigation. Unlike other types of edema, lymphedema characteristically involves the foot. The contour of the ankle is lost through infilling of the sub-malleolar depressions a buffalo hump forms on the dorsum of the foot, the toes appear square due to confinement of footwear, and the skin on the dorsum of the toes cannot be pinched due to subcutaneous fibrosis (Stemmer's sign). Lymphedema usually spreads proximally to knee level and less commonly affects the whole leg. In the early stages, lymphedema pits, and the patient reports that the swelling is down in the morning. This represents a reversible component to the swelling, which can be controlled. Failure to do so allows fibrosis, dermal thickening, and hyperkeratosis to occur. In general, primary lymphedema progresses...

Clinical Picture

Erythema Neck

This disease manifests on the skin and in the eyes. Skin subcutaneous lesions occur on or near the joints, on the head and upper trunk, but may also occur on the buttocks, in the sacrococcygeal region and the lower extremities (onchocercomas). The lesions are cystic and contain adult filariae they are spherical or oval, are asymptomatic and may perforate the skull. On the trunk and extremities so-called lichenified onchodermatitis is characterized by dry skin, ictiosiform, lichenification, pigmentation, scaling or hyperkeratosis (saurio skin) and pruritus. Saurio skin is the most constant characteristic, and in some occasions it is limited to only one extremity (sowdah). Later on there is atrophy, especially of lesions of the buttocks, with loss of elasticity, marked skin folds, alopecia and anhidrosis (Fig. 49.1). There is a form known as onchocercal dyschromia with alternating areas of depigmented and normal or hyperpigmented skin around the follicles (Leopard skin). It predominates...

Stage V

Clinical instrumental diagnostic staging of lymphoedema. positive in more than 80 per cent of cases, with an even better outcome in patients operated upon precociously (at stages II and III see Fig. 47a.7). The incidence of lymphangitic attacks decreases significantly after microsurgery. The reduction in oedema volume obtained by microsurgery is seen immediately after operation (within the first three postoperative days), and a further decrease in lymphoedema is also observed at medium- and long-term follow-up, particularly between the first and fifth years after operation. From the fifth year onwards, the clinical condition of the limb tends to remain stable with time, even more than 10 years after surgery. Lymphangioscintigraphy can document objectively that the flow through the venous graft parallels the clinical improvement over the 10-year period 36 . Q4 Turning to the prevention of secondary lymphoedema, early identification of high-risk patients (such as those...


Surgery benefits only a small minority of patients with lymphedema. Operations fall into two categories bypass procedures and reduction procedures. The rare patient with proximal ilioinguinal lymphatic obstruction and normal distal lymphatic channels might benefit, at least in theory, from lymphatic bypass. A number of methods have been described including the omental pedicle, the skin bridge (Gillies), anastomosing lymph nodes to veins (Neibulowitz), CHRONIC VENOUS INSUFFICIENCY,VARICOSE VEINS, LYMPHEDEMA, AND ARTERIOVENOUS FISTULAS

Other Filariasis

Most cases are asymptomatic, without the presence of microfilariae. Acutely there can be adenitis, centrifugal lymphangitis and recurrent fever. Lymphangitis of the genitals is not uncommon. The disease is generally recurrent. Chronic disease is characterized by adenolymphoceles, varicose veins, lymphedema and elephantiasis.