Lymphedema Therapy in the Vascular Anomaly Patient

Dec 2005, Vol. 3, No. 4: 253-255
Stanley G. Rockson, M.D.

Stanford Center for Lymphatic and Venous Disorders, Division of Cardiovascular Medicine, Stanford University School of Medicine, Falk Cardiovascular Research Center, Stanford, California.

Lymphedema Therapy in the Vascular Anomaly Patient:
Therapeutics for the Forgotten Circulation

STANLEY G. ROCKSON, M.D.

Any discussion of lymphatic dysfunction and therapeutics must concern itself initially with the role of the lymphatic circulation in health and disease.1 In normal physiology this system is concerned with the flux of lymph from the extracellular space which, in turn, contributes to the maintenance of total body homeostasis.

A second, vital function is tied to the transport of leukocyte and antigen-presenting cells to lymphoid organs.2 Finally, the gastrointestinal lymphatics play an integral role in the nutritive absorption of lipids from the intestinal lumen.

In disease, the lymphatic system can be afflicted by either congenital (heritable) or acquired pathology. In most instances, including those that prevail in the vascular anomalies, disease is accompanied by the presence of either regional or generalized lymphedema, a condition that supervenes when there in an inherent imbalance between the formation of interstitial fluid and its absorption, as lymph, into the lymphatic vessels.

In the presence or absence of associated vascular anomalies, structural abnormalities in the lymphatic conduits will have predictable effects upon functional derangements and disease manifestations.3 Lymphatic aplasia, hypoplasia, and primary valvular insufficiency produce lymphatic hypertension, decreased contractility, and secondary valvular insufficiency. Obliteration or disruption of lymph vessels, as might occur in proliferative lesions of this vasculature, will lead to lymph stasis, with accumulation of lymph, retained
interstitial proteins, and glycosaminoglycans in the skin and subcutaneous structures.

With chronicity, these changes are accompanied by disruption of elastic fibers and activation of keratinocytes, fibroblasts, and adipocytes. The resultant stimulation of collagen production leads to thickening of the skin, subcutaneous tissue overgrowth, and fibrosis.

In addition to the chronic edema and fibrosis of lymphedema, lymph stasis has numerous additional longterm attributes: lymphostasis in the arterial wall, in concert with other vascular responses, leads to local arteriovenous flow disturbances; similar effects are seen in the ligaments and tendons; and disturbed immune traffic, in concert with the physical derangements in the tissues, predisposes to frequent, recurrent episodes of bacterial
soft tissue infection (cellulitis).

A consideration of lymphatic dysfunction in the context of vascular anomalies raises the question of the heritable nature of certain lymphatic disorders. In addition to the variety of chromosomal aneuploidy that has been described (trisomy 13, 18, 21, triploidy, Klinefelter syndrome, Turner syndrome), various dysmorphogenic genetic abnormalities have been associated with lymphedema and complex vascular anomalies: Noonan Syndrome; Noone-Milroy Hereditary Lymphedema; Meige Lymphedema (Lymphedema Tarda); Lymphedema Distichiasis; Cholestasis-lymphedema syndrome (Aagenaes syndrome);4 Lymphedema- microcephaly-chorioretinopathy;5 Neurofibromatosis type I (von Recklinghausen); Lymphedema-Hypoparathyroidism Syndrome; and Klippel-Trenaunay-Weber Syndrome.2,6

Lymphedema, even when superimposed upon a more complex vascular presentation, is most often readily identified by its physical characteristics, including pitting edema, peau d’orange, cutaneous fibrosis, and positive ‘Stemmer’ sign;7,8 where doubt exists, the presence of lymphatic vascular insufficiency can be verified by indirect radionuclide lymphoscintigraphy.3,9

Once identified, the lymphatic component of a vascular anomaly presentation mandates an aggressive physiotherapeutic approach that will require life-long emphasis. It has been proposed that meticulous measures designed to reduce edema may reduce the likelihood of disease progression and reduce the frequency of soft-tissue infection.10

Decongestive lymphatic therapy is an empirically derived, effective, multi-component technique for the reduction of volume of the involved limb and for the maintenance of integumentary health. It has been suggested that this method accelerates the transport of lymph and, thereby, facilitates the dispersal of retained interstitial protein.11

Decongestive lymphatic therapy integrates meticulous skin care, exercise, and the use of compressive elastic garments with daily application of a specific massage technique known as manual lymphatic drainage.10

The manual technique emphasizes the recruitment of watershed pathways for lymphatic flow through stimulation of edema-free zones of the trunk and uninvolved extremities; the mild tissue compression that is accomplished during manual lymphatic drainage enhances filling of the initial lymphatics and augments transport capacity through the cutaneous lymphatic dilatation and the development of accessory lymph collectors.12

Manual lymphatic drainage (MLD) should not be confused with other forms of therapeutic massage that have no effect on lymphatic contractility. Adjunctive to external compressive techniques in the limb, MLD will represent the mainstay of therapy for involvement of the head, neck, breast, and genitalia, where the external application of ancillary compressive devices and garments is difficult or impossible.

Where feasible, however, it is essential to maximize compression for edema volume to be effectively reduced. In the acute treatment phase of limb lymphedema, this compression therapy will take the form of multi-layer short stretch bandage application, which has been demonstrated to augment the effect of joint/muscle pump upon lymph transit through the limb;13 furthermore, as tissue pressure is increased, there is a reduction in the abnormally increased ultrafiltration which, in turn, leads to improved fluid reabsorption.

Once edema volume reaches a nadir, the maintenance approach to the limb will require the use of fitted elastic garments for use during non-recumbency; nocturnal compression may also be required. Relatively inelastic sleeves and underwear that transmit high-grade compression (40 to 80 mm Hg) will prevent reaccumulation of fluid after successful decongestive treatments.

Garments must be fitted properly and replaced every 3 to 6 months. Complete decongestive physiotherapy, including manual lymphatic drainage, compression bandaging, garments and skin care, is an effective treatment modality for many patients for chronic lymphatic dysfunction.

When examined in a series of patients with either upper or lower extremity lymphedema, with an average follow-up of 9 months, average volume reductions of 59% and 68% were observed in upper and lower limbs, respectively;14 maintenance self-management technique are effective in sustaining the majority of this benefit in compliant patients.14,15

Ancillary therapeutic measures can be considered as an adjunct to the elements of standard decongestive lymphatic therapy.10 Notably, intermittent pneumatic compression has been shown to add benefit to the decompressive effects of standard therapies, albeit in the context of cancer-associated, rather than primary, lymphedema.16 Other modalities, including low-level laser application17 and hyperthermia18 continue to be investigated.

Insight into the fundamental pathophysiology of lymphedema remains incomplete. It is clear, however, that the limb swelling that results from the increased volume of interstitial fluid and progressive fibrotic changes causes the patients to experience impairment in functions of daily living and engenders psychological dysfunction that stems from the chronic disease state and the associated impairment in body image and self-esteem.19–21

While a cure for lymphedema does not exist, intensive and sustained decongestive lymphatic physiotherapy should be advocated as the treatment choice to reduce edema, symptoms and functional impairment in patients with both simple and complex forms of lymphedema.

LYMPHEDEMA IN VASCULAR ANOMALY