Cancer of the Vagina - Lymphoedema
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Contents

bulletWhat is Lymphoedema?
bulletAnatomy and physiology
bulletLymph
bulletLymph vessels
bulletLymph nodes
bulletLymph circulation
bulletPathophysiology
bulletDiagnosis
bulletTreatment
bulletComplex lymphoedema therapy
bulletManual lymphatic drainage
bulletSkin care
bulletExercises
bulletCompression
bulletPneumatic compression
bulletElevation
bulletLaser treatment
bulletPharmacological management
bulletPsychosocial support
bulletSurgical management
bulletReferences

What is Lymphoedema?

Lymphoedema occurs when the lymphatic system has a reduced ability to transport protein rich fluids out of the tissue spaces and the inability of phagocytic cells (macrophages) to remove abnormally accumulated proteins as a result of damage to the lymphatic system or inadequate formation of the lymphatic system 2.

There is currently very little written in the literature about lymphoedema occurring following cancer and treatment of cancer affecting the female gynaecological tract. However, we do know that cancer and its treatment can cause damage to the lymph system and in some cases, result in lymphoedema. The incidence of lower limb lymphoedema following surgery and irradiation to the pelvic nodes has been reported as high as 12.5% 1.

Anatomy and Physiology.

The role of the lymphatic system is to 

bulletCollect and remove proteins from the interstitial spaces;
bulletTransport fats from the digestive system;
bulletAct as part of the body's immune defence system by filtering abnormal cells and producing macrophages and lymphocytes 3 

It is made up of:

bulletLymph
bulletLymphatic vessels
bulletLymph nodes
bulletLymphatic organs
bulletSpleen
bulletTonsils
bulletThymus gland.

Lymph

Lymph is clear, watery fluid inside the lymphatic vessels. It is generally low in protein concentration in most areas of the body, but the lymph draining from the liver and small intestine has a higher concentration of lymph. Lymph is very similar to interstitial fluid - the fluid outside of the lymphatics in the cell spaces - which also has a low protein concentration. Blood plasma differs slightly from these fluids due to its high protein concentration.

Lymph Vessels

The lymph system differs from the blood circulatory system in that it is not a continuous loop but is a blind ended system starting in the tissue spaces. It is made up of lymphatic capillaries (or lacteals for those which drain the small intestine) which attach to cells by tiny filaments. These capillaries consist of a single layer of flat endothelial cells which are arranged like large overlapping plates. These can be opened widely by the tiny filaments attached to surrounding cells to allow large molecules such as proteins to pass into the lymphatics. These capillaries have no valves. Pre-lymphatic channels exist distally to the lymph system but are not lymphatic vessels.

Similar to the venous system, the lymphatic capillaries then flow into deep lymphatic collectors which have cell walls consisting of three layers including smooth muscle. They are thinner and more fragile than veins and have many valves. These thin walls are easily distended when over-filled which can prevent valves from closing and result in back flow.

These then flow into the lymphatic ducts - the thoracic duct is the major collector and receives lymph from the entire body except the right upper quadrant, right arm and right head and neck. It originates as a large sac-like structure called the cisterna chyli in the lumbar region then flows into the vascular system at the anastomosis of the left subclavian vein and left internal jugular vein. Lymph from the upper right quadrant drains in to the right lymphatic duct at the junction of the right subclavian vein and right internal jugular vein.

Lymph Nodes

These are small bean-shaped structures located along the lymphatic vessels in chains or clusters. The node receives lymph from valved afferent vessels. The lymph is then filtered through the node and passes out through a single efferent vessel which also contains valves to prevent backflow. The nodes are covered by a fibrous capsule and produce and contain lymphocytes and macrophages to phagocytose irregular cells.

They lymph nodes associated with the female gynaecological system are:

bulletexternal iliac nodes - drain vulva, cervix and lower portion of the uterus;
bulletinternal iliac nodes - received drainage from all internal pelvic organs as well as vulva, clitoris and urethra;
bulletcommon iliac nodes - receives lymph drainage from cervix and upper portion of vagina;
bulletinferior gluteal nodes - receives lymph drainage from cervix, the lower portion of the vagina and Bartholin's glands;
bulletsuperior gluteal nodes - receives drainage from cervix and vagina;
bulletsacral nodes - drain cervix and vagina;
bulletsubaortic nodes - drain cervix and vagina;
bulletaortic nodes - drain cervix, uterus, oviducts and ovaries;
bulletrectal nodes - drain cervix and vagina;
bulletparauterine nodes - drain vagina, cervix and uterus;
bulletsuperficial femoral nodes - drain external genitalia of the vulvar region, gluteal region, leg and foot;
bulletdeep femoral nodes - drains the leg.

(Droegemeuller, M., Herbst, A.L., Mishel, D.R. and Stenchever, M.A. "Comprehensive gynaecology, Mosby, St Louis (1987).

Lymph Circulation

The lymphatic system is a one-way system of transporting fluids and proteins from the tissue spaces back to the blood circulation. When the blood flows to this tissues only 95% returns to the heart via the venous system - the remaining 5% is carried by the lymphatic system (Sherwood 1993). The fluid exchange from blood to cells to the lymphatics occurs at the capillary bed. Pressure gradients in normal circumstances - high capillary plasma hydrostatic pressure and low plasma colloid osmotic pressure force water and small proteins out of the arterial capillaries. Larger proteins are transported out of the capillaries by vesicles because they are too large to fit through the gaps in capillary protein. High blood colloidal osmotic pressure and high tissue hydrostatic pressure work so fluid is reabsorbed into the venous capillaries (Sherwood, 1993). The lymphatic system drains away excess filtered fluid and larger molecules such as proteins and bacteria.

Lymph fluid moves through the lymphatics as a result of:

bulletinspiration - as the diaphragm descends, the intra-abdominal pressure increases as the intra-thoracic pressure decreases. This creates gradient in the thoracic duct and encourages the lymph flow upwards;
bulletskeletal muscle contractions - this creates pressure on the walls of collector vessels and during exercise lymph flow can increase 10 to 15 times (Anthony and Thibodeau, 1993);
bulletarterial pulsations;
bulletpostural changes - elevation of a limb can encourage lymph flow; 
bulletintermittent rhythmic contraction of the walls of the lymph vessels 4;
bulletpassive compression of body tissues - bandaging and compression garments;
bulletone-way valves prevent back flow.

The lymph then flows back into the main circulation via the left and right subclavian veins.

Pathophysiology.

Where damage to the structure or function of the lymphatic system occurs - such as surgical removal or blockage by scarring due to radiotherapy - the lymphatic transport system is impaired. This results in a state called lymphostasis which does not automatically cause lymphoedema 5. This is because of the transport that the lymphatics are providing may be adequate for the fluid load it needs to carry. However, if the transport capacity remains impaired and the fluid load increases then there is no safety valve to channel away excess fluid - resulting in lymphoedema.

At the microcirculation level this occurs by an increase in hydrostatic pressure within the lymph vessel causing distension of the vessel and preventing valve closure. The protein rich fluid can then flow backwards pushing open the inlet valves of the initial lymphatics into the interstitial space. The increase protein concentration in the tissues draws more fluid from the capillaries in to the interstitial space creating an even greater fluid load. In severe cases, the oedema is so great that the anchoring filaments which join the lymphatics to the surrounding cells can be torn causing the initial lymphatics to collapse 5.

Accumulation of protein rich fluid and impairment of the macrophages can result in fibrosis of the tissues and an increased risk for cellulitis 2. Infection resulting in an inflammatory response of increased blood supply and increased capillary permeability can potentially worsen the lymphoedema.

Diagnosis.

Not all swollen limbs are lymphoedema. A diagnosis can only be made once a thorough history and examination has occurred and other conditions have been ruled out. The diagnosis must be made by a medical practitioner.

Diagnosis tests available include:
bulletlymphoscintigraphy - involves the injection of a radio-isotope into the web space between the great toe and second toe. A gamma camera then records the flow speed and spread of the lymph fluid;
bulletMagnetic Resonance Imaging (MRI).

Differential Diagnosis:
bulletmetastatic or recurrent disease;
bulletCCF - presents with generalised oedema;
bulletNephrotic syndrome;
bulletLiver disease
bulletVaricose veins
bulletDeep venous thrombosis and post-thrombotic syndrome - exclude this by duplex scanning;
bulletChronic venous insufficiency - including development of ulceration;
bulletLipodema
bulletCellulitis.

Treatment.

bulletcomplex lymphoedema therapy 
bulletmanual lymph drainage;
bulletskin care;
bulletexercises;
bulletcompression;
bulletbandaging
bulletgarments
bulletpneumatic compression
bulletelevation
bulletlaser treatment
bulletpharmacological management
bulletpsychosocial support
bulletsurgical treatment.

Complex Lymphoedema Therapy.

This approach involves a combination of manual lymphatic drainage, skin care, exercises and compression bandaging followed by the use of low stretch compression garments and maintenance of good skin condition and self-massage.

Manual Lymphatic Drainage.

This specialised form of massage relies on use of the superficial lymphatics to redirect lymph from oedematous areas to areas with apparently functional lymphatics. Because it uses the superficial lymphatics as a channel, the massage is very light. Massage is performed by ensuring the most proximal region is treated first then the distal to avoid congestion in the tissues. There are a number of different massage techniques.

Skin Care. 

Maintenance of good skin integrity is important in avoiding infection which could cause further swelling due to the inflammatory response. Education to patients to avoid trauma to the limb is essential as well as recommending use of low allergenic soaps and moisturisers.

Exercises. 

Contraction of skeletal muscles and deep breathing enhance the movement of fluid into the initial lymphatics and up through the thoracic duct. As the initial lymphatics take approximately five seconds to fill and one second to empty into the collecting vessels, the exercise pace needs to allow for this (Piller, 1994). Exercises should be conducted when bandages or compression garments are applied for extra support for compliant tissues.

Compression. 

Encourages the return of fluid into the venous capillaries and helps fill the initial lymphatic capillaries by raising the interstitial hydrostatic pressure. Compression also helps to reduce dilated lymphatic vessels allowing the valves to meet and thereby enhances valve function. In addition, compression enhances the action of the skeletal muscle pump by providing resistance to skeletal muscle contraction.
bulletBandaging - Low stretch bandages are applied in combination with special padding to distribute the pressure over body prominences. Low stretch bandages are chosen in preference to high stretch because they maintain a low resting pressure for comfort and a high working pressure to improve flow in deeper vessels. Low stretch bandaging when expertly applied is an important component of treatment, however it can be ineffective or dangerous if it is not carried out by trained therapists.
bulletGarments - Low stretch garments with graduated compression maintain the reduction achieved in treatment. Patients should be measured for garments when their limb size has plateaued during treatment. Practitioners should decide between ready-made and custom-made garments dependent upon the patient's condition, degree of compression required, age, independence and lifestyle. There are several suppliers of both ready-made and custom garments.

Pneumatic Compression

Another method of compression therapy is use of pneumatic compression devices "sleeves". Whilst effective in reducing the oedema volume, this form of therapy has limited effect on protein reabsorption 6. Accordingly, manual lymphatic stimulation and drainage must be used prior to pneumatic compression.

Elevation.

In the early stages of lymphoedema, elevation of the affected limb is useful in reducing swelling. However, it has limited usage for more advanced cases and must be used in combination with other therapeutic techniques 7.

Laser Treatment

Experimental treatment using Helium Neon and Gallium Arsonide lasers has been shown to stimulate lymph contractility, lymph vessel regrowth and macrophage activity. Further research into this form of therapy is necessary.

Pharmacological Management.

bulletDiuretics. Although marginally useful during the early stages of lymphoedema treatment, long term use of diuretics is discouraged because of the potential for depletion of intravascular fluid and electrolyte imbalance 8.
bulletBenzopyrones - operate by stimulating macrophage to break down excess protein. Although effective in conjunction with other therapies, there are a number of serious potential side-effects. These side-effects include severe liver injury, mild nausea and diarrhoea. Accordingly, benzopyrones should be used cautiously 9.
bulletAntibiotics - can be administered for the treatment of cellulitis and other complications of lymphoedema.

Psychosocial Support

Issues relating to disease progression, body image, self-esteem and depression often arise in cases of lymphoedema. Reference to an appropriate counsellor is recommended to help the patient manage these issues. This site has information on the psychological/psychiatric and chaplaincy issues relating to gynaecological cancer and its side effects and treatments.

Surgical Management.

bulletResection. There are several operative techniques designed to improve lymphoedema, however, surgery should only be considered if complex lymphatic therapy (CLT) fails to achieve satisfactory results. In addition, surgery damages the superficial lymphatic vessels which reduces the efficacy of later CLT. Examples of operative techniques include:
bulletDebulking surgery involves the removal of excess skin and tissue
bulletLiposuction is generally discouraged as not only does it damage the superficial lymphatic vessels but must be periodically repeated as results are typically temporary.
bulletPhysiologic procedures. These promote the return of lymph to the blood circulation by a variety of microsurgical technique 10.

References

1. (Rotmensch J., Rubin, S.J., Sutton, H.G.., Javaeri, G., Halpem, H.J., Schwarz, L.J., Stewart, M., Weichselbaum, R.R., and Herbst, A.L.(1990) "Preoperative radiotherapy followed by radical vulvectomy with inguinal lymphadenectomy for advanced vulval cancer" Gynaecologic Oncology, 36, 181-84).

2. Pappas, C.J and O'Donnell, T.F. (1992) "Long term results of compression treatment for lymphoedema" Journal of Vascular Surgery, 16, 555-564; Piller, N.B (1990) "Macrophage and tissue changes in the developmental phases of secondary lymphoedema and during conservative treatment with benzopyrones" Arch Histol 53 (supp) 209-218

3. Anthony, C.P. and Thibidou, G.A "Anatomy and physiology" Mosby, St Louis, 1993

4. Ganong, W.F. "Review of Medical Physiology", Appleton and Lange, Norwalk, 1993

5. Casely-Smith and Casely-Smith, Modern Treatment of Lymphoedema, Lymphoedema Association of Australia, Adelaide, 1994

6. Leduc, A. and Leduc (1990) "Physical Treatment of Oedema", European Journal Lymphology and related problems 1: 8-10

7. Swedborg, I., Norrefolk, J.R., Piller, N.B. and Asard, C. (1993) "Is elevation an effective means for the resolution of post mastectomy lymphoedema?" Scandinavian Journal of Rehabilitation Medicine 25: 75-82; and Consensus Document of the International Society of Lymphology - Executive Committee (1995) "The Diagnosis and Treatment of Peripheral Lymphoedema" Lymphology 28: 113-117

8. Consensus Document of the International Society of Lymphology - Executive Committee (1995) "The Diagnosis and Treatment of Peripheral Lymphoedema" Lymphology 28: 113-117

9. Australian Adverse Drug Reactions Bulletin (1995) vol 14(3): 11; AND Consensus Document of the International Society of Lymphology - Executive Committee (1995) "The Diagnosis and Treatment of Peripheral Lymphoedema" Lymphology 28: 113-117

10. Consensus Document of the International Society of Lymphology - Executive Committee (1995) "The Diagnosis and Treatment of Peripheral Lymphoedema" Lymphology 28: 113-117

 

Anna Linning
B.Occ.Thy. (UQ)
Bayside District Health Service Convenor,
Oedema Management Special Interest Group, OT Australia (Qld)

 Sandra King
 Occupational Therapist
Jobst Education Co-ordinator, Biersdorf Australia Ltd

 

 

 

 

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