[Therapy of lymphedema]. / Therapie des Lymphödems.

Lymphedema is a chronic and progressive disease when untreated. It can be assumed that more than 5% of the population suffer from a form of lymphedema. The therapy options depend on the individual symptoms and the wishes of the patient and include conservative measures as well as surgery or a combination of both.

The effects of reduction operation with genital lymphedema on the frequency of erysipelas and the quality of life.

Genital lymphedema represents a severe disability for patients particularly when complicated by erysipelas, the most frequent complication. The objectives of this study were: to investigate the frequency of erysipelas in patients with genital lymphedema and genital lymphatic cysts who underwent evaluation for surgical treatment, to observe the influence of resection operations on the frequency of erysipelas, and to measure changes in the quality of life due to the resection. A total of 93 patients with genital lymphedema were studied. All patients underwent integrated care treatment in the Földi Clinic, Hinterzarten and the Department of Plastic and Hand Surgery of the University Hospital Freiburg during the period between 1997 and 2007. 44 of these patients underwent surgical treatment of genital lymphedema. The results indicate that lymphatic cysts were the most important risk-aggravating factor for recurrent erysipelas with lymphorrhea in the genital region (p < 0.001). Following the resection operation, however, the number of erysipelas incidents significantly decreased (p < 0.001). In addition, the antibiotic dose could be reduced after surgery (p = 0.039) and an improved quality of life was achieved (p < 0.001).

MR imaging of the lymphatic system: distribution and contrast enhancement of gadodiamide after intradermal injection.

We examined 24 lower extremities in 12 patients with lymphedema to evaluate the distribution and enhancement of gadodiamide after intradermal injection for MR imaging of lymphatic pathways in lymphedematous patients. The lymphedema was bilateral in 8, unilateral in 4, and 3 patients suffered from lymphedema in the genital region. 9 mL of gadodiamide and 1 mL of mepivacainhydrochloride 1% were divided into 5 portions and injected intradermally into the dorsal aspect of each foot. For MR imaging, a 3D spoiled gradient-echo sequence (Volumetric Interpolated Breathold Examination, VIBE) was performed. We detected the beaded appearance of lymphatic vessels extending from the injection site in 22 lower extremities (92%). In 13 lower extremities (54%), lymphatic vessels of the upper leg could be visualized. A contrast enhancement was observed in 16 out of 24 inguinal lymph node groups (67%). After 15 minutes of contrast material application, concomitant venous enhancement was detected in all lower extremities (100%). In 15 lower extremities (63%), collateral vessels with dermal backflow areas between lymphatic vessels were seen. Thus, intradermal injection of gadodiamide allows the visualization of lymphatic pathways in patients with lymphedema. In comparison to the venous system, lymphatic vessels show a tendency to have the highest contrast material uptake in the later acquisitions of 35, 45, and 55 minutes after intradermal injection of gadodiamide. Furthermore, 3D MIP reconstructions supported the identification of the lymphatic vessels and differentiation from veins due to the different angles of view.

Development, validation and clinical use of the FLQA-I, a disease-specific quality of life questionnaire for patients with lymphedema.

BACKGROUND: It is known from clinical practice that lymphatic diseases can be associated with reductions of quality of life (QoL). Due to the lack of validated methods, only few studies however have systematically investigated the QoL in lymphedema. The aim of the study was 1) to develop a standardized QoL questionnaire specific for lymphedema and 2) to assess the QoL in these patients. PATIENTS AND METHODS: We developed and tested the FLQA-l, a novel QoL questionnaire developed specific for use in lymphedema on the basis of the previously validated FLQA vein questionnaire. The questionnaire consists of 92 items that refer to the following scales: Physical status, everyday life, social life, emotional well-being, treatment, satisfaction and profession/household. 392 patients with primary (n = 246) and secondary (n = 146) lymphedema were included in the validation study. RESULTS: The FLQA-l showed good internal consistency; Cronbach's alpha was higher than 0.75 in all scales. There were no floor and ceiling effects and satisfactory item selectivity. The test-retest reliability, sensitivity to change and convergent validity with other psychometric instruments were satisfactory. Clinically, patients with lymphedema showed markedly impaired QoL in all fields, compared to persons with early stage venous insufficiency, and comparable reductions of QoL, compared to patients with venous leg ulcer. CONCLUSION: These data indicate that the FLQA-l is a reliable and valid questionnaire for the assessment of QoL in lymphedema. Since the QoL is impaired in many patients with lymphedema, QoL evaluation may be helpful for clinical diagnostics as well as for outcome measurement of specific edema therapy.

Use of a myocutaneous flap after resection of a large lymphangioma.

UNLABELLED: We report on an 11-year-old girl with a large lymphangioma involving the lower third of the abdominal wall, the mons pubis, both labia majora, the perianal region and extending into the pelvic bones. Because of an increasing lymphorrhea from the cutaneous lesions resulting in considerable discomfort and skin infections, the patient sought medical advice. After lymphological check-up excluded the existence of a chylous reflux, the patient was presented at a multidisciplinary medical council. A palliative surgical treatment was recommended consisting of the resection of the most affected suprapubic region and the coverage of the resulting tissue defect with a gracilis myocutaneous flap. Postoperatively, a venous stasis at the tip of the skin paddle developed, which was relieved by the use of leeches and required secondary closure. Despite these complications, the surgical intervention yielded an acceptable cosmetic result, a diminution of lymphorrhea and hence subjectively some relief. CONCLUSION: Due to the variability of lymphangiomas, an assessment by a multidisciplinary consultation is proposed. With respect to therapy, the use of a myocutaneous flap represents one of the therapeutic options for large cutaneous lymphangiomas.

Effect of complex decongestive physiotherapy on gene expression for the inflammatory response in peripheral lymphedema.

Complex decongestive physiotherapy (CDP), consisting of manual lymph drainage, compression bandaging, remedial exercises and skin care, mobilizes accumulated edema fluid and increases lymph flow. On the other hand, it also has a beneficial therapeutic effect on fibrosclerosis. Because little is known of its possible mode of action on a molecular level, this preliminary study evaluated CDP in patients with peripheral leg lymphedema as to the potential role of gene expression in the inflammatory response. The quantitative expression of genes for CD14, interferon-gamma receptor (IFN gamma R), tumor necrosis factor-alpha (TNF alpha), integrin alpha 4 beta 1 (VLA-4), tumor necrosis factor receptor p55 (TNFR1) and CD44 (standard form) was examined in 9 patients with primary or secondary leg lymphedema before and after phase 1 of CDP. Overall, there was a decrease of expression of these pro-inflammatory genes after CDP, suggesting that biologic mechanisms implicated in the inflammatory cascades in other disorders are also involved in the fibrosclerotic reactivity in lymphedema. However, whereas each patient acted as his or her own control before and after CDP, gene expression in normal patients and normal limbs before and after CDP needs to be examined before the full meaning of these observations can be understood.

[Revolving door patient in lymphology--possibilities and limits of therapy and patient motivation during inpatient and ambulatory conditions]. / Drehtürpatient in der Lymphologie--Möglichkeiten und Grenzen der Therapie und Patientenmotivierung unter stationären und poststationären Bedingungen.

Patients suffering from chronic diseases necessitate a continuous treatment and self-care. Independent from its etiology the method of choice of the treatment of chronic lymphedemas of the limbs is "Complex Decongestive Physiotherapy" (CDP). CDP is carried out according to the requirements either as an outpatient- or an inpatient-treatment. In some cases CDP has to be applied not in its usual, regular form but in a modified manner. The lecture deals with problematic cases and with the limits of our therapeutic possibilities.

Treatment of lymphedema and patient rehabilitation.

Even without clinically manifest lymphedema, in the overwhelming majority of patients after breast cancer treatment the function of the lymphatics is impaired. As a consequence, appropriate preventive measures have to be carried out. The method of choice of the treatment of lymphedema is combined physiotherapy.

Analysis of lymphatic drainage in various forms of leg edema using two compartment lymphoscintigraphy.

The anatomical and functional status of the epifascial and subfascial lymphatic compartments was analyzed using two compartment lymphoscintigraphy in five groups of patients (total 55) with various forms of edema of the lower extremities. Digital whole body scintigraphy enabled semiquantitative estimation of radiotracer transport with comparison of lymphatic drainage between those individuals without (normal) and those with leg edema by calculating the uptake of the radiopharmaceutical transported to regional lymph nodes. A visual assessment of the lymphatic drainage pathways of the legs was also performed. In patients with cyclic idiopathic edema, an accelerated rate of lymphatic transport was detected (high lymph volume overload or dynamic insufficiency). In those with venous (phlebo) edemas, high volume lymphatic overload (dynamic insufficiency) of the epifascial compartment was scintigraphically detected by increased tracer uptake in regional nodes. In patients with deep femoral venous occlusion (post-thrombotic syndrome). subfascial lymphatic transport was uniformly markedly reduced (safety valve lymphatic insufficiency). On the other hand, in the epifascial compartment, lymph transport was accelerated. In those patients with recurrent or extensive skin ulceration, lymph transport was reduced. Patients with lipedema (obesity) scintigraphically showed no alteration in lymphatic transport. This study demonstrates that lymphatic drainage is notably affected (except in obesity termed lipedema) in various edemas of the leg. Lymphatic drainage varied depending on the specific compartment and the pathophysiologic mechanism accounting for the edema. Two compartment lymphoscintigraphy is a valuable diagnostic tool for accurate assessment of leg edema of known and unknown origin.

The treatment of lymphedema.

BACKGROUND: Before the treatment of arm lymphedema after breast carcinoma treatment with complex decongestive physiotherapy can be initiated, it is mandatory to differentiate between benign and malignant forms (due to relapse) and to establish the diagnosis of accompanying diseases, if present. METHODS: In benign lymphedemas, the aim of complex decongestive physiotherapy is to restore the symptom free "Stage 0 of latency" and to maintain fitness for work. The palliative treatment of malignant lymphedemas results in the amelioration of the quality of life. RESULTS: The results of treatment depend on the experience of the physician in clinical lymphology, on the training and dedication of the lymphedema therapist, and on the compliance of the patient. CONCLUSIONS: A study concerning gene expression has shown that complex decongestive physiotherapy influences the pathological alterations of the interstitium in lymphedema patients.

[Involvement of the lymphatic system in primary non-lymphogenic edema of the leg. Studies with 2-compartment lymphoscintigraphy]. / Die Beteiligung des Lymphgefässsystems beim primär nicht-lymphogenen Odem des Beines. Untersuchungen mit der Zwei-Kompartiment-Lymphszintigraphie.

Two-compartment lymphoscintigraphy was developed to examine the sub- and epifascial lymphatics of the leg. Digital images were evaluated visually and semiquantitatively by calculating the uptake of activity within the lymph nodes. The data from patient groups with four different types of leg edema were compared with those of the control group to prove the involvement of the lymphatics in the non-lymphatic edema. The cyclic idiopathic edema demonstrated an accelerated transport of the lymph consistent with a high volume insufficiency. In phlebedema the high volume insufficiency was epifascially so distinct, that it could be detected scintigraphically. In post thrombotic syndrome the transport of the lymph was reduced dramatically corresponding to a safety valve insufficiency. Epifascially however, an accelerated lymph flow was observed due to compensatory mechanisms. The lipedema did not show any scintigraphic abnormalities. These results show that two-compartment lymphoscintigraphy can detect alterations in lymphatic function secondary to non-lymphogenic leg edema. The lymphatic function is changed according to the underlying pathophysiology which may be facilitate the differential diagnosis of such a leg edema.

Benign supraclavicular tumorous lymphangiectasia--a new disease?

We describe an isolated recurrent non-inflammatory tumorous swelling of the supraclavicular fossa in four premenopausal women. Ultrasonography, magnetic resonance imaging and computer tomography of the neck each suggested an inhomogeneous mass consistent with "lymphangioma." In each patient the clinical course and histopathologic findings suggested that the swellings were due to chronic localized lymph stasis with subsequent lymphangiectasia, possibly initiated by intermittent obstruction of the juncture of the thoracic or right lymph duct with the internal jugular vein. Enlargement may have been hormonally triggered by estrogens as each woman was taking oral contraceptive pills at the onset of the disease. To characterize this unique entity, we have termed the disorder benign supraclavicular tumorous lymphangiectasia.

The importance of the subfascial lymphatics in the diagnosis of lower limb edema: investigations with semiquantitative lymphoscintigraphy.

A new method of semiquantitative lymphoscintigraphy for the evaluation of lower limb edema is characterized by (1) the evaluation of both the epifascial and subfascial system in order to assess type and stage of the edema, (2) the use of high-resolution digital whole-body imaging to facilitate the calculation of functional parameters, and (3) the use of active, standardized ergometry for reproducibility. The appearance time of 99mTc-labeled human albumin nanocolloid in inguinal lymph nodes after injection and the percent uptake of colloid into lymph nodes at 40 and 120 min after injection served as functional parameters. Patients with edema of the lower limb were compared with normal subjects. In patients with primary lymphedema the two lymphatic compartments are functionally compromised. Early and advanced stages of postthrombotic syndromes can be distinguished by characteristic lymphoscintigraphic patterns in epifascial and subfascial lymphatic compartments. These results indicate that only the separate evaluation of both the epifascial and subfascial compartments allows an accurate functional assessment of the lymphatics in lower limb edema.