Lymphedema Microsurgery

When this page was first presented, the field of microsurgery for lymphedema was limited to the placement of shunts in locations of missing nodes or of extreme scar caused blockages.

In updating this particular page, we will keep it as a general information source, but we have done individual pages on specific microsurgical techniques and will continue to add individual pages as newer techniques are tried and verified.

The key to success in this treatment modality would appear to be surgical intervention very early in the development of the condition.

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LYMPHATIC MICROSURGERY: A MODERN WEAPON IN THE FIGHT AGAINST PERIPHERAL LYMPHEDEMA

C. Campisi, F. Boccardo

Peripheral lymphedema can be distinguished in primary and secondary from the etiopathogenetic point of view. Primary lymphedemas have no clearly identifiable cause (idiopathic) although one or more triggering aetiologic factors can often be identified. Congenital lymphedemas, namely those with onset at birth, are also included in this category. Sometimes, congenital lymphedemas are hereditary-familial (Nonne-Milroy’s disease) and often, but not always, associated with chromosomal abnormalities1. Primary lymphedemas generally have their onset after birth. Depending on the time of onset, lymphedemas may have early or late onset, which can be triggered by even trivial traumas, such as infection or surgery. Especially in female individuals, predisposing factors are to be found in some specific periods of their sexual life (i. e. puberty, pregnancies, menopause) or in alterations in their neuro-hormonal conditions (so called neuro-endocrine lymphedemas).

Primary lymphedemas can be found within more general pictures of lymphatic-lymph node hyperplasia, dysplasia or impaired lymph production. Lymph nodes, lymphatic vessels or both structures can be involved in abnormal lymph flow2,3. However, in most cases of hypoplasia, lymph node involvement, which leads to the obstruction of lymph vessels, can almost constantly be demonstrated. From a physiopathologic and diagnostic point of view, this picture is totally overlapping that of standard secondary lymphedemas resulting from lymphadenectomy and/or radiation therapy. Approximately 90% of all primary lymphedemas are characterized by hypo- and hyperplasia involving the lymph nodes and/or lymphatic collectors in the affected region as well the their walls and/or valves. The number of lymphatic collectors in the area involved is significantly increased in 8-10% of primary lymphedemas. This hyperplasia is generally associated with structural dysplasia of the lymphatic collectors and lymph nodes. Hypo-dysplasia in many so called primary lymphedemas can also be confirmed by a diminished ability to form and activate an adequate collateral circulation, whenever such an ancillary structure may become essential (i. e. after traumas, infections, surgery etc.). Defective lymphogenesis is as important as lymphodynamic impairment. A condition of “hyper-lymphogenesis” may be the result of pre-existing regional arterial-venous hyperstomies, arterial-venous fistulas (for example in Klippel-Trénaunay’s disease) or related angiodysplasia4,5,6. Reduced or absent production of lymph as a result of agenesis, hypoplasia or impaired permeability of so called initial lymphatics (or lymphatic capillaries) is a very rare, if not exceptional condition. Finally, apart from insufficient lymph drainage along anatomically pre-established pathways, as already mentioned above, also top-to-bottom lymph and/or chylous reflux should be mentioned among lymphodynamic abnormalities. This reflux is caused by impaired or insufficient anti-gravitational structures (Tosatti7, 1974) which normally feature valves, the reticular myo-elastic structure of lymphatic collector walls, and the structural architecture of lymph nodes (gravitational reflux lymphedemas and chyloedemas).

Unlike primary ones, the etiology of secondary lymphedemas can be clearly identified with the patient’s physical and clinical examination (definable also as acquired). Therefore, it is possible to distinguish post-traumatic, post-infection and post-inflammation lymphedemas (post-lymphangitis, post-phlebitic, etc.) caused by radiotherapy, surgery, paralysis, (primary or secondary) neoplasm, parasites (Filaria bancrofti). This latter type is endemic in some tropical or sub-tropical areas in Asia, Africa and Latin America. However, it should be mentioned that even the so called secondary or acquired lymphedemas have fairly often some congenital predisposition. For example, post-mastectomy lymphedemas, with a 5% to 35% incidence of cases also depending on whether surgery is associated with radiation therapy, is known to be more likely to occur when there is no deltoid pathway. In this event, which may be due to anatomic abnormality or obstruction secondary to surgical trauma, radiation therapy or acute lymphangitis, the lymph is drained directly in the supraclavicular lymph nodes, skipping the axillary stations. With preventive lymphoscintigraphic studies comparing the arm ipsilateral to the breast cancer site, patients with a higher risk of developing secondary lymphedemas could be identified, who should therefore receive preventive therapeutic treatment. Predisposing factors for congenital wall-valve dysplasia of the lymphatics are always indicated by some A. even in Filaria related lymphedemas (Olszewski, Jamal et al.8,9, 1994). Therefore, based on these data, the classification proposed by Tosatti7 more than thirty years ago seems to be still valid.

Materials and Methods

A) Diagnostic Assessment

The diagnosis of lymphedemas is first of all based on medical history and objective examination. In this way, the time and conditions of onset, location, evolution and, consequently, extent, volume and physical-semiological features of lymphedema can be assessed and a differential diagnosis from phlebo-edema can be made. Lymphedema is hard to the touch, while venous edema is soft (the latter one has the typical fovea sign under finger compression). This difference substantially depends on the stagnant lymph being an excellent pabulum for fibroblasts in the subcutaneous connective tissue, which mature more rapidly into fibrocytes thus forming fibro-sclerotic connective tissue. Lymphatic edema has a typically rhizomelic or total (“columnar”) location, whereas the venous edema has an acromelic arrangement, except for flegmasia alba coerulea dolens, caused by acute deep thrombophlebitis of the femoral-iliac region. Unlike phleboedema, lymphedema does not usually evolve into dystrophic-dyschromic skin lesions and ulcers; it is more likely to be complicated by acute, reticular, diffuse and erysipeloid lymphangitis, caused by gram-positive cocci infections promoted by lymph stasis. Phleboedema is often associated with varices and varicophlebitis. Unlike lymphedema, especially after the night, it is subject to rapid postural changes and is characterized by abnormal Doppler venous flow rates with significant venous pressure increase when the patient is in clinostatic and orthostatic position (Bartolo10, 1983). However, mixed forms of lympho-phleboedema or phlebolymphedema also exist, with prevalence of either venous (like in stage III post-phlebitic syndrome) or lymphatic component. The much more complex picture of angiodysplasia characterized by congenital, arterial-venous macro and micro-fistulas (like in the above mentioned Klippel-Trénaunay’s disease) is also to be included in these mixed forms. Gigantism with elongation of the affected limb and more or less severe foot dysmorphism (upper limb localization is extremely rare), flat, map-like angioma, “Port wine” colour with hyperhidrosis of the plant surface are all typical signs of this disease. There are also some spurious forms, masked by prevailing lymphedema and therefore more difficult to recognize. In these cases, arterial-venous circulation investigations and, in particular, Doppler venous pressure measuring may not be helpful, and further instrumental investigations may be required (i.e. phleboscintigraphy, phlebography, and even digital arteriography when angiodysplasia is suspected).

For the time being, lymphangioscintigraphy and direct lymphangiography are the most suitable investigations for lymphedemas. Lymphangioscintigraphy11,12,13 is the most popular method employed for the screening of lymphedemas. Since it is not really invasive, it can be easily repeated in the patient follow-up, especially after microsurgery. A small tracer dose (technetium-Tc99m) adsorbed in colloid spherules (colloid sulphide, rhenium, dextran) is injected in the dermis-hypodermis, in the interdigital spaces. The lymphotropic nature of these substances permits to display the “preferential” lymphatic pathways with a gamma-camera, and to measure the flow rate and lymph node uptake. Tracer clearance measurement is a very useful parameter from a lymphodynamic point of view. Direct lymphangiography14,15 is better indicated in the study of gravitational reflux lymphedema or chyloedema of the lower limbs and/or external genitalia, when requiring a surgical treatment (Kinmonth, 1982). In this examination, ultrafluid Lipiodol® is injected into a lymphatic collector, preferably previously isolated with microsurgery, of the foot or hand dorsum. This type of investigation is slightly invasive and not without some, although rare, complications of general (i. e. pulmonary microembolism, in case of peripheral lympho-venous fistulas, anaphylactic reaction to Lipiodol) or local nature (i.e. infection on the site of skin incision, acute lymphangitis, lymphorrhea etc.). However, if performed according to well established standards, direct lymphangiography has no statistically significant sequelae. This examination can also be performed in children. It enables a morpho-functional study of the surface circulation and, with the use of proper technical artifices, also of deep circulation. Lymphangioscintigraphy is the examination of choice, while lymphangiography should be resorted to only in cases of doubtful interpretation and more likely to be treated surgically.

More recently, also CT (Computerized Tomography), Ultrasonography and, according to our original preliminary studies, Lymphangio Magnetic Resonance offer pre-operatively important and, sometimes, determinant data upon lymphatic disfunctions.

Indirect lymphangiography performed with dermo-hypodermic injection of a water-soluble contrast medium (Iotasul®) although proved to be useful to clarify some etiopathologic aspects of primary lymphedemas, has so far failed to enter current clinical practice. The same can be said for fluorescent microlymphography (Bollinger16, 1981), even though recent studies by Allegra and Co-workers17 proved that this investigation can give important parameters in the clinical assessment of lymphedema and of its evolution. The conventional Houdack-McMaster lymphochromic test with the injection of an intradermal-subepidermal injection of a modest amount of highly lymphotropic vital stain (Bleu Patent V) is used today as a preliminary investigation in direct lymphangiography and microsurgery for a better and faster assessment of lymphatics. Recent studies by Olszewski and Bryla18 (1994) and Campisi19 (1996) have developed a system to measure endolymphatic pressure and lymphatic flow rate. These parameters, together with venous pressure assessment, help measure the lymph-venous pressure gradient which is essential for a correct approach to microsurgical treatment of lymphedemas. With this method, a lymphatic vessel is isolated and cannulated at the lower third of the leg medial surface. With this method, even during microsurgery, any changes in the flow-pressure rate can be recorded in clino- and orthostatic position, at rest and under dynamic conditions. Following experimental works on dogs (Chang20, 1985), deviations in lymphatic flow and pressure were recorded in patients with lymphedema (Olszewski and Bryla18, 1994) and, in particular in candidates for microsurgery (Campisi et al.19, 1994). In agreement with the predictions of Yamada20 (1969), these studies have shown that a valuable lymphatic-venous gradient exists and, above all, that its stable values, especially when no body activity is taking place (like at night), are an important element to assess before microsurgery in order to assess long and medium term results.

B) Clinical Considerations

Apart from some exceptional cases of acute post-lymphangitis and/or post-traumatic lymphedemas, it is normally a chronic, progressing, ingravescent and disabling condition characterized by the progressive volume increase of the limb/s involved, up to elephantiasis, with severe functional impairment. This disease, which evolves by phases, is characterized by frequent acute lymphangitic, erysipeloid, recurrent complications with subsequent severe septic conditions, dermato-liposclerotic indurated cellulitis, chronic fibro-sclerotic lymphoadenitis (in primary lymphedemas) and lymphostatic verrucosis22,23. The degeneration into lymphangiosarcoma (see Steward-Treves syndrome) is a rare sequela, more likely to occur in post-mastectomy lymphedemas, not to be confused with local cutaneous recurrence of breast cancer. Sometimes, lymphedema can be associated, especially when involving the lower extremities, with Kaposi’s sarcoma, the latter one not necessarily caused by HIV-related acquired immunodeficiency-syndrome24.

C) Therapeutic Options

At the end of the ‘60s, there were very few therapeutic solutions to the treatment of lymphedema. Only the most severe and advanced cases of elephantiasis proper were surgically treated, mainly in order to reduce the volume of lymphedematous limbs. The most popular surgical methods were those according to Charles25 (1912) or total resection of skin-lipid layers, Thompson26 (1967) or drainage with scarred sub-fascial skin flap, and Servelle27 (1947) or total surface lymphangectomy. Being highly demolishing and invasive operations, they could not be recommended in less advanced or initial stages and even less so in children. However, owing to the physiopathologic and clinical investigations particularly of Földi et al.28 (1973) the foundations were laid for a conservative medical-physical treatment, with the development of the manual lymphatic drainage, clinically codified by Vodder29 (1969), Földi himself and Leduc30 (1980). Even compression therapy with the use of special machines available in different models (air, mercury compression) has been improved and gradually propagated. The specific medical treatment envisages the use of antibiotics, penicillin in particular, according to long-term protocols recommended by Olszewski22 (1994). In cases of lymphedemas with acute erysipeloid lymphangitis, anti-inflammation drugs, mild diuretics administered only if necessary and in any case associated with the essential hygienic measures and the constant use of suitable bandages and elastic compression means are also recommended. The positive effect of benzopyrones has recently been discovered, mainly as a result of the research conducted by Casley-Smith31 (1986). However, the correct use of this category of drugs for the treatment of lymphedemas has not yet been codified. Even thermotherapy used in ancient China (ovens) and in the Latin-Mediterranean world (hot-wet compress), recently proposed again by Chang32 (1985) in a more modern version (dry hot air produced by microwaves) and by Campisi, Boccardo et al.33 (1994) (hot-wet air in a closed circuit), for the treatment of post-lymphangitis lymphedemas, despite some encouraging results, has so far failed to obtain general consensus. Conservative-treatment resistant cases, the need to shorten the duration of the disease, spare the patients frequent and long hospitalization, and allow them to go back to their family and work, have spurred the research for new, more suitable, no longer resective and symptomatic surgical solutions, aiming at correcting the mechanisms of lymphedemas34. As early as in the ‘70s, Tosatti7 (1974) proposed a method of antigravity ligation of dilated and insufficient lymphatics for the treatment of the lower extremities and/or external genitalia due to gravitational reflux35-36. This method is one of the first models of functional, direct surgical approach to lymphatics and/or lymph nodes. The advent of Microsurgery has given an outstanding contribution to this new approach. Lymphnodal-venous and multiple lymphatic-venous anastomosis (Degni37, 1974; Olszewski38, 1984) came to the fore. At the same time39, based on more clinical experience (Campisi et al.40, 1994) and improved surgical equipment and techniques fig1, greater knowledge was gained of lymphangiology and the results of lymphatic microsurgery. These methods are beneficial not only to secondary fig2, fig3, but also primary lymphedemas fig4, fig5, since early intervention is possible even in young children fig6, fig7) with some adequate modifications of techniques such as lymphatic-capsule-venous anastomosis (Campisi41, 1994). In the great majority of so called primary lymphedemas, especially of the lower extremities, hystopathologic lymph node alterations exist (pulpal fibrosclerosis, with dilatation of afferent lymphatics and leiomuscular wall hyperplasia: fig8) which characterize the obstructive nature of this type of lymphedema, similarly to secondary ones (Campisi et al.42, 1994). Therefore, ‘derivative’ lymphatic microsurgery (Campisi et al.43, 1991) is typically expressed in multiple lymphatic-venous anastomosis (fig9, fig10). For cases where lymphostatic disease is associated also with venous impairment, (from venous hypertension to varices, from acute surface and/or deep thrombophlebitis to post-phlebitic sequelae), which are a contraindication to derivative lymphatic-venous surgery, ‘reconstructive’ lymphatic microsurgical techniques have more recently been developed (Campisi et al.44, 1994). It is thus possible to obtain long-term satisfactory results with the use of segmental auto-transplantation of lymphatic collectors, which can be performed only for the treatment of monolateral lymphedema (Campisi45, 1984; Baumeister46, 1988). Also, interposition autologous venous grafting or lymphatic-venous-lymphatic plasty (fig11, fig12 can be employed (Campisi et al.47, 1991). With this technique, which is easier and faster to be performed than the previous one, also bilateral lymphedemas can be treated. A direct end-to-end anastomosis between the lymphatic collectors upstream and downstream the obstacle, as an alternative solution to the venous or lymphatic graft, can be performed only very rarely, specially in secondary lymphedemas. The use of free microvascular lymphatic or lymph nodal flaps (Becker48, 1991; Trévidic et al.49, 1994) is still under clinical testing. However, it opens up very interesting prospects to the treatment of lymphedemas which fail to respond to a correct conservative medical therapy and which, for congenital (aplasia or hypoplasia) or acquired (elephantiasis with diffuse obstructive lymphangitis) reasons, cannot benefit from the above mentioned derivative or reconstructive microsurgical techniques.

Results

With a follow-up to be planned at 1, 3, 6 and 12 months and once a year at least for the first 5 years after surgery, positive results from Lymphatic Microsurgery can be achieved in all patients, with greater evidence among patients who have undergone operations at stage II or III. Comparative measurements of the circumferences of the various segments of the lymphedematous limb, volumetric studies fig13 and lymphangioscintigraphy18 fig14, fig15 are essential to demonstrate the efficacy of derivative and reconstructive microsurgery.

Discussion and Conclusions

In the general therapeutic scenario for lymphedemas, the role to be played by surgery versus medical-physical conservative treatment can be easily defined. The so called Combined Physiotherapy (Földi51, 1994) is the treatment of choice for most lymphedemas In non-responsive cases (up to 30%-40%), the drainage function of the lymphatic circulation, can, at least partially, be recovered by means of Lymphatic Microsurgery52 to be performed as early as possible. The rather constant outcome can further be improved with a subsequent conservative treatment. Major resective surgery has no longer reason to exist. Only in rare cases, as soon as the results of microsurgical and/or medical conservative treatment have become stable, does minor resective surgery still find some indications for aesthetic-reductive purposes.

With regard to prevention of secondary lymphedemas, finally, early diagnosis plays an important role as well as the selection of high-risk patients for the onset of lymphostatic disease after oncological lymphadenec-tomies, especially if associated with radiotherapy (Campisi53, 1994; Pissas54, 1995). In these cases, early microsurgery is a reasonable suggestion in order to fight, from their very onset, lymphedemas which, based on a reasonable statistical probability, are expected to show unrelenting progression.

References

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Correspondance to

Corradino Campisi, MD
Professor of Surgery and Microsurgery

Address:

Via Assarotti, 46/9
16122 Genoa, ITALY
Telephone number:
39 10 8393755
Fax number:
39 10 811465

http://www.rjhrm.ro/dump_articol.php?id_numar=5&id_articol=30&limba=EN

.................

Department of Surgery, Unit of Lymphatic Surgery and Microsurgery, San Martino Hospital, University of Genoa, Italy. campisicorradino@tin.it

Authors report modern diagnostic and therapeutic procedures used in the correct assessment and treatment of congenital lymphatic and chylous disorders. Lymphatic dysplasias can be clinically represented only by peripheral lymphedema or be associated with more complex dysfunctions of chyliferous vessels and the thoracic duct (chylous ascitis, chylothorax, etc.) It is, therefore, useful to perform a complete diagnostic evaluation of each patient before carrying out any therapeutical approach. Lymphoscintigraphy, lymphangio-MR, oil contrast lymphography, and lymphangio-CT are the common diagnostic tools used in these cases, variable associated depending above all on the complexity of the pathology. From the therapeutical point of view, microsurgical methods proved to bring successful and long lasting results, both with derivative lymphatic-venous anastomoses and reconstructive lymphatic-venous-lymphatic anastomoses. Better long-term results are obtained in earlier stages.

Department of Surgery, Scientific Institute San Raffaele, Vita-Salute San Raffaele University, Milan, Italy. mukenge.mvunde@hsr.it

Secondary scrotal lymphedema is an infrequent complication of radical cystectomy assiociated with pelvic lymphadenectomy. We report a case of secondary lymphedema of male genitalia presenting more than 4 years after a radical cystectomy with extended pelvic lymphadenectomy for adenocarcinoma of the bladder. Microsurgical lymphovenous anastomoses are usually performed using only the scrotal lymphatics excluding the testicular lymphatics drainage. We have experimented a new microsurgical technique based on lymphovenous anastomosis between the collectors of the spermatic funiculus and the veins of the pampiniform plexus, allowing the testicular lymphatic drainage.

Microsurgery for treatment of peripheral lymphedema: long-term outcome and future perspectives.

Department of Surgery, Unit of Lymphatic Surgery and Microsurgery, San Martino Hospital, University of Genoa, Genoa, Italy. campisicorradino@tin.it

Authors report over 30 years of their own clinical experience in the treatment of chronic peripheral lymphedemas by microsurgical techniques performed at the Center of Lymphatic Surgery of the University of Genoa, Italy. Over 1,500 lymphedema patients were treated with microsurgical techniques. Derivative lymphatic-venous techniques were most often used. For those cases where a venous disease was associated to lymphedema, reconstructive lymphatic microsurgery techniques were performed (lymphatic-venous-lymphatic-plasty). Objective assessment was undertaken by water volumetry and lymphoscintigraphy. Volume changes showed a significant improvement in over 83%, with an average follow-up of more than 10 years. There was an 87% reduction in the incidence of cellulitic attacks after microsurgery. Microsurgical lymphatic-venous anastomoses have a place in the treatment of peripheral lymphedema and should be the therapy of choice in patients who are not sufficiently responsive to nonoperative treatment. Improved results can be expected with operations performed at earlier lymphedema stages.

.................

Demonstration protocol for the anatomopathological study of
lymphatic vessels in lymphedema

Claudia Stein Gomes1, Fernando Silveira Picheth1, Ezio Fulcheri2, Corradino Campisi3, Francesco Boccardo3

1. Division of Angiology and Vascular Surgery , Hospital Santa Casa de Misericórdia de
Curitiba - PUCPR, Brazil
2. Institute of Pathological Anatomy, San Martino Hospital, University of Genoa, Italy
3. Surgery Division (DISCAT), Section of Emergency Clinical Surgery, Center of Lymphology and Microsurgery San Martino Hospital, University of Genoa, Italy

Correspondence:
Dr. Claudia Stein Gomes

Rua Padre Anchieta, 2004/1302
CEP 80730-000 - Curitiba - PR
Brazil
Tel.: +55 (41) 335.2135
Fax: +55 (41) 322.9892
E-mail: steingomes@sulbbs.com

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Lymphedema is a pathology characterized by an increase in the volume of soft tissues in the affected region that can evolve into large deformities such as in cases of elephantiasis. Lymphedema is caused by a lack of lymph transport by the lymphatic vessels (congenital or idiopathic), or is secondary to inflammatory, infectious, irradiative or surgical processes. The disease affects a large number of patients, mainly after oncological interventions or after inflammatory and/or infection processes.

Treatment of this pathology includes manual lymphatic drainage methods, pressotherapy and elastic compression. Surgical treatment varies from excision of skin and subcutaneous tissue to more thorough treatment, with microsurgical techniques for lymphatic venous anastomosis between lymphatic collectors and a competent vein with the aid of an operation microscope 1.

Through microsurgery, performed at inguinal level for lower limbs and at the brachial level for upper limbs, where there are pre and post lymphatic collectors lymph nodes with diameters ranging from 0.5 to 1 mm, it becomes possible to perform anatomopathological studies of the perilymphatic and lymphoid tissues. Normally, some histopathological lesions of the lymphatic vessels are identified and described 2, which are the basis for the different types of lymphedema. These are certain constrictive and dystrophic modifications of the vessels, generically classified as vascular wall fibrosis and increased periadventitial matrix, which are interpreted as indirect signals and sequelae of acute or chronic inflammatory reaction. Nevertheless, these lesions do not justify the pathological basis of the different conditions, nor can they explain the polymorphous or undetermined clinical stages.

The lymphatic vessels are essentially composed of an endothelium with its valvular apparatus, of a generally fine wall and of the adventitia. The vessel wall is composed of a deep layer of the intima and a medium layer that consist of a cellular part (fibroblasts and smooth-muscle cells) and a non-cellular part (elastic fibers, collagen and proteoglycans). The vasa vasorum is found in the adventitial layer. All components of lymphatic vessels are covered by a perivascular sheath and are primarily responsible for lymph transport.

The anatomopathological study of lymphatic vessels is not easily performed, once the lymphatic vessels are small-caliber structures. With common histological stainings, such as hematoxylin-eosin, one can only observe the fibrosclerotic alterations on the vessel walls, quantify the component of the periadventitial matrix and look for the elements of inflammatory reaction. From this viewpoint, the study is purely morphological. In addition, the use of specific stains is necessary to allow the lymphatic vessels to be identified and differentiated from blood vessels. The endothelium of the lymphatic vessels does not produce a sufficient quantity of coagulation factor VIII to be evaluated as a histological section with immunohistochemical methods 3. Only after an adequate lysis with proteolytic enzymes (collagenase or trypsin) is it possible to uncover the antigenic sites and provide evidence for factor VIII in the endothelium of the lymphatic vessels.

The objective of this study is to suggest a protocol for the study of lymphatic vessels in order to obtain the data necessary for a wider understanding of the morphology and the paraphysiological (compensating) or frankly pathological (degenerative) alterations of the lymphatic circulation in primary and secondary lymphedema.

Therefore, in order to understand the pathology of the lymphatic vascular system, it is important that specialists shift from a morphological study to a morphofunctional study, which provides evidence for the functional characteristics of the vessel walls in terms of residual contractile capacity or hypertrophic and hyperplastic reaction of the smooth-muscle components.

METHOD

Sampling

The material obtained from the surgical intervention can be of two types: an isolated segment from the lymphatic collector or some fibrous-fatty tissue which surround the lymphatic vessels. The material should be fresh and should arrive as quickly as possible to the anatomic pathology laboratory. If possible, this material should also be marked with surgical thread in one of its extremities to serve as an orientation for the study.

The pathologist should maintain the material in a closed container with neutral formalin 4 and avoid the cooptation and distortion of lymphatic vessels

Fixation

The fixation should be brief, taking no more than 12 hours, to avoid lesion to the antigenic sites.

Embedding

When dealing with a piece of fibrous-fatty tissue and lymphatic vessels, paraffin embedding should be made after the material is cut into macroscopic sections.

When dealing with a segment from the lymphatic collector, it should be maintained in an erect position and embedded in agar 5 before being embedded in paraffin.

Slide preparation

Routinely, 11 glass slides are prepared. These slides are stained in the following manner:

- First slide: hematoxylin-eosin stain;

- Second slide: Masson's trichrome stain;

- Third slide: silver impregnation method for reticular fibers;

- Fourth slide: Weigert's elastic stain

- Fifth slide: Van Gieson stain;

- Sixth slide: immunohistochemical stain with smooth-muscle (antiactin) antibodies;

- Seventh slide: immunohistochemical stain with antivimentin antibodies;

- Eight slide: immunohistochemical stain with antidesmin antibodies;

- Ninth slide: CD 31 stain;

- Tenth slide: CD 34 stain;

- Eleventh slide: hematoxylin-eosin stain. antibodies;

- Seventh slide: immunohistochemical stain with antivimentin antibodies;

- Eight slide: immunohistochemical stain with antidesmin antibodies;

- Ninth slide: CD 31 stain;

- Tenth slide: CD 34 stain;

- Eleventh slide: hematoxylin-eosin stain.

Slide Reading Analysis

The slide analysis is performed in order to make a quantitative and distributive evaluation of the lymphatic vessel cells and the perilymphatic tissue cells.

The first stains that are studied to identify the lymphatic vessel are the CD 31 and CD 34 stains.

The endothelium of the blood vessels is selectively stained by the immunohistochemical staining with the anti-CD 34 antibody, whereas the endothelium of the lymphatic vessel is usually negative for this staining. However, the endothelium of lymphatic and blood vessels is stained with the anti-CD 31 antibody 3,6.

Afterward, the morphological structure of the vessel is evaluated using the hematoxilin-eosin stain: the diameter and thickness of the wall, valves and periadventitial matrix (Figure 1) are all assessed. As for the vessel lumen, it may be with a reduced, normal or increased caliber. The wall may be fine, normal, thickened or fibrotic. The valvular apparatus may be absent, normal or prominent and weakened. Finally, the periadventitial matrix may be slightly, partially or fully evident (Figure 2). This algorithm should serve as a preliminary guide in the microscopic observation of the serial section, allowing for the observation of basic morphological parameters. In such a way, the identification of signs of phlogosis, when present in the lymphatic vessels, suggest an inflammatory lymphedema 2,7.

Using the Masson, Weigert and Van Gieson trichrome processes, it is possible to obtain a more refined structural evaluation of the vessels, including an observation of the components of the intercellular matrix such as collagen, elastic fibers and reticular fibers 2. Using immunohistochemical techniques such as the Avidin Biotin Peroxidase Complex (ABC) method, it is also possible to study the cellular part of the lymphatic wall 2,3,6. With the antivimentin antibody, it is possible to evidence the presence of fibroblasts, fibrocytes and also smooth-muscle cells. Desmin only stains the myofibroblasts, and smooth-muscle actin stains the myofibroblasts and also the smooth-muscle cells of tunica intima and media. For the study of the smooth-muscle cells present on the lymphatic vessel wall, observations are made in terms of: quantity, whether it be average, scarce or increased; distribution of fine bundles, whether they be large or fragmented; and typology, whether it is be fragile, hypertrophic or dysplastic. Therefore, depending on the predominant cell group in the vessel wall and in the periadventitial matrix, a regression of contractile fibers may be suspected, as, for example, in cases in which there is a high degree of fibrosis or chronic postsurgical lymphedema resulting from the predominance of a degenerative process of the lymphatic wall 2.

DISCUSSION

In medical literature, there are few published works that refer to the study of lymphatic vessels in peripheral lymphedema 2,7-12. This results from the fact that there are no major surgical treatment centers for this pathology where surgeons work in connection with anatomic pathology laboratories.

The findings of the Center of Lymphology and Microsurgery of the University of Genoa, Italy, which has a vast experience in the microsurgical treatment of limb lymphedema through lymphatic venous anastomoses, allowed the development of major anatomofunctional studies on lymphatic vessels, which resulted from biopsies performed during surgical interventions (as demonstrated in the numerous reports published by Campisi et al. 13-18).

Currently, there is even a classification proposed by these pathologists from the research group of Genoa for the lymphatic and lymph node alterations found in patients with secondary lymphedema who were submitted to microsurgical treatment for lymphatic venous anastomosis 2. This classification was proposed thanks to a adequate technique which allowed the presentation of the diagnosis based on both the simple morphology (diameter of the lymphatic vessels, presence of fibrosis or inflammatory signals) and the functional morphology (evaluation of the contractility and activity of the wall) of the lymphatic vessels.

In the near future, there is a possibility for the expansion of research with a larger number of cases and with samples taken from other segments of the affected limb. With such information, a detailed study of the distinct features of this disease can be developed.

We believe that, in order to perform an ample study of the lymphatic vessels in diverse anatomic pathology laboratories, a protocol must first be created for a better understanding of this complex pathology.

REFERENCES

1. Campisi C, Boccardo F. Linfedemas - Tratamento por técnicas microcirúrgicas. In: Brito CJ, Duque A, Merlo I, Murilo R, Lauria F Fº, editores. Cirurgia Vascular. Rio de Janeiro: Revinter; 2002. p. 1246-77.
2. Dellachà A, Fulcheri E, Boccardo F, Campisi C. Patologie latenti dei vasi linfatici come possibili substrati del linfedema cronico secondario. Linfologia 1998;2:20-4.
3. Culling CFA, Allison RT, Barr WT. Cellular Pathology Technique. 4th ed. Woburn (MA): Butterworth-Heinemann; 1985.
4. Carson F, Martin JK, Lynn JA. Formalin fixation for electron microscopy: a re-evaluation. Am J Clin Pathol 1973;49:365-73.
5. Ventura L, Bologna M, Ventura T, Colimberti P, Leocata P. Agar specimen orientation technique revisited: a simple and effective method in histopathology. Ann Diagn Pathol 2001;5(2):107-9.
6. Lapertosa G, Baracchini P, Fulcheri E, Tanzi R. Small blood vessels or lymphatic channels with neoplastic microemboli: a comparative immunohistochemical study. Verh Dtsch Ges Path
7. Badini A, Fulcheri E, Campisi C, Boccardo F. A new approach in histopathological diagnosis of lymphedema: pathophysiological and therapeutic implications. Lymphology 1996;29 Suppl :190-8.
8. Campisi C, Badini A, Boccardo F. Anatomo-pathological bases in the management of primary lymphedema and microsurgical implications. Lymphology 1994;27 Suppl :546-9.
9. Badini A, Fulcheri E. Vantaggi dell'immunoistochimica nella diagnostica istopatologica del linfedema. Minerva Cardioangiol 1997;45:17-24.
10. Pfister G, Saesseli B, Hoffmann U, Geiger M, Bollinger A. Diameters of lymphatic capillaries in patients with different forms of primary lymphedema. Lymphology 1990;23(3):140-4.
11. Rada IO, Tudose N, Fedorac R. Fibrosclerosis of tunica media in the prenodal lymphatic vessels of patient with lymphedema. Morphol Embryol (Bucur) 1986;32(2):93-7.
12. Kinmonth JB, Wolfe JH. Fibrosis in the lymph nodes in primary lymphoedema. Histological and clinical studies in 74 patients with lower-limb oedema. Ann R Coll Surg Engl 1980;62:344-54.
13. Campisi C, Zattoni J, Siani C, et al. Twenty year clinical experience in the microsurgery management of lymphedema. Lymphology 1994;27 Suppl :651-7.
14. Campisi C. Lymphatic microsurgery: legend or reality? Phlebolymphology 1994;7:11-15.
15. Campisi C. The modern surgery of lymphedema. Lymphology 1996;29 Suppl :210-21.
16. Campisi C, Boccardo F. Frontiers in lymphatic microsurgery. Microsurgery 1998;18:462-71.
17. Campisi C, Boccardo F. Role of microsurgery in the management of lymphoedema. Int Angiol 1999;18(1):47-51.
18. Degni M. New techniques of lymphatic-venous anastomosis for the treatment of lymphedema. J Cardiovasc Surg (Torino) 1978;19(6):577-80.

J Vasc Br - Official Publication of the Brazilian Society of Angiology and Vascular Surgery

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Lymphatic microsurgery for the treatment of lymphedema.

Abstract

Jan. 26, 2006

Campisi C, Davini D, Bellini C, Taddei G, Villa G, Fulcheri E, Zilli A, Da Rin E, Eretta C, Boccardo F.

Section of Lymphatic Surgery and Microsurgery, Department of Surgery, S. Martino Hospital, University of Genoa, Genoa, Italy.

One of the main problems of microsurgery for lymphedema consists of the discrepancy between the excellent technical possibilities and the subsequently insufficient reduction of the lymphoedematous tissue fibrosis and sclerosis. Appropriate treatment based on pathologic study and surgical outcome have not been adequately documented. Over the past 25 years, more than 1000 patients with peripheral lymphedema have been treated with microsurgical techniques.

Derivative lymphatic micro-vascular procedures has today its most exemplary application in multiple lymphatic-venous anastomoses (LVA). For those cases where a venous disease is associated to more or less latent or manifest lymphostatic pathology of such severity to contraindicate a lymphatic-venous shunt, reconstructive lymphatic microsurgery techniques have been developed (autologous venous grafts or lymphatic-venous-Iymphatic-plasty - LVLA).

Objective assessment was undertaken by water volumetry and lymphoscintigraphy. Subjective improvement was noted in 87% of patients. Objectively, volume changes showed a significant improvement in 83%, with an average reduction of 67% of the excess volume. Of those patients followed-up, 85% have been able to discontinue the use of conservative measures, with an average follow-up of more than 7 years and average reduction in excess volume of 69%. There was a 87% reduction in the incidence of cellulitis after microsurgery. Microsurgical lymphatic-venous anastomoses have a place in the treatment of peripheral lymphedema and should be the therapy of choice in patients who are not sufficiently responsive to nonsurgical treatment.

Improved results can be expected with operations performed earlier at the very first stages of lymphedema.

Microsurgery 26: 65-69, 2006.PMID: 16444753

[PubMed - as supplied by publisher]

Is there a role for microsurgery in the prevention of arm lymphedema

Abstract

January 26, 2006

Campisi C, Davini D, Bellini C, Taddei G, Villa G, Fulcheri E, Zilli A, Da Rin E, Eretta C, Boccardo F.

Section of Lymphatic Surgery and Microsurgery, Department of Surgery, S. Martino Hospital, University of Genoa, Genoa, Italy.

The secondary lymphedema of the upper limb (post-mastectomy lymphedema) has an incidence, in patients who underwent axillary lymphadenectomy for breast cancer, between 5 to 25%, up to 40% after radiotherapic treatment.

We studied 50 patients treated for breast cancer. The patients were divided in two groups of 25 each, comparable for age, sex, pathology and treatment and followed up to 5 years after operation for breast.

One group of 25 patients was controlled only clinically (physical examination, water volumetry) at 1-3-6 months and 1-3-5 years from breast cancer treatment.

The other group of 25 patients was followed also by lymphatic scintigraphy performed pre-operatively and after 1-3-6 months and 1- 3-5 years from operation. In the first group, followed only clinically, lymphedema appeared in 9 patients after a period variable from 1 week to 2 years, with highest incidence between 3 and 6 months. In the second group of 25 patients, the preventive therapeutic protocol allowed to have a clinically evident arm lymphedema only in 2 patients.

The comparison of the two groups of 25 patients proved a statistically significant difference in the appearance of arm secondary lymphedema (p = 0.01, using Fisher's exact test). The diagnostic and therapeutic preventive procedures allow to reduce the incidence rate of lymphedema significantly, in comparison with patients who did not undergo this protocol of prevention.

October 10, 2005

Although lymphedema in the extremities is a troublesome adverse effect following radical resection of various cancers, conventional therapies for lymphedema are not always satisfactory, and new breakthroughs are anticipated. With the introduction of supermicrosurgical techniques for the anastomosis of blood or lymphatic vessels less than 0.8 mm in diameter, we have developed a novel method of lymphaticovenular anastomosis for the treatment of primary as well as secondary lymphedema in the extremities. Here, we review the pathophysiological aspects of lymphedema, emphasizing the importance of smooth-muscle cell function in the affected lymphatic walls. We then describe the theoretical basis and detailed operative techniques of our lymphaticovenular anastomoses. Although technically demanding, especially for beginners, we believe that this method will become a new clinical standard for the treatment of lymphedema in the near future.

Publication Types:

The use of vein grafts in the treatment of peripheral lymphedemas: long-term results.

Campisi C, Boccardo F, Zilli A, Maccio A, Napoli F.

Department of Specialistic Surgical Sciences, Anaesthesiology and Organ Transplants (DISCAT), Emergency Surgical Clinic Section, Lymphology and Microsurgery Center, S. Martino Hospital, University of Genoa, 16122 Genoa, Italy. campisi@unige.it

This study evaluates long-term results of the treatment of peripheral lymphedemas by the microsurgical reconstructive technique of interposed vein grafts. The technique consists of the use of autologous vein grafts to reconstruct lymphatic pathways where there is a block to the lymphatic circulation of the limb, whether of congenital or acquired etiology. The venous segment represents a sort of "bridge" between afferent and efferent lymphatic collectors (lymphatic-venous-lymphatic plasty [LVLA]). The results also proved to have positive long-term effects after microsurgical operation. Follow-up evaluation was performed clinically by water volumetry and instrumentally by lymphangioscintigraphy. With this LVLA technique, peripheral lymphedemas can be treated when derivative lymphovenous shunts cannot be used because of impaired venous circulation in the same lymphedematous limb. The new aspect of the study is that we report long-term clinical and instrumental results.

PMID: 11494381 [PubMed - indexed for MEDLINE]

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Microsurgical Techniques for Lymphedema Treatment: Derivative Lymphatic-Venous Microsurgery.

Campisi C, Boccardo F.

Department of Specialistic Surgical Sciences, Anesthesiology, and Organ Transplants (DI.S.C.A.T.), Section of General and Emergency Surgery, Lymphology and Microsurgery Center, S. Martino Hospital, University of Genoa, Largo R. Benzi 8, 16132, Genoa, Italy.

We analyzed clinicopathologic and imaging features of chronic peripheral lymphedema to identify imaging findings indicative of its exact etiopathogenesis and to establish the optimal treatment strategy. One of the main problems of microsurgery for lymphedema is the discrepancy between the excellent technical possibilities and the subsequently insufficient reduction of the lymphedematous tissue fibrosis and sclerosis. Appropriate treatment based on pathologic studies and surgical outcome have not been adequately documented. Over the past 25 years, 676 patients with peripheral lymphedema have been treated with microsurgical lymphatic-venous anastomoses. Of these patients, 447 (66%) were available for long-term follow-up study. Objective assessment was undertaken by water volumetry and lymphoscintigraphy. Objectively, volume changes showed a significant improvement in 561 patients (83%), with an average reduction of 67% of the excess volume. Of the 447 patients followed, 380 (85%) have been able to discontinue the use of conservative measures, with an average follow-up of more than 7 years and average reduction in excess volume of 69%. There was an 87% reduction in the incidence of cellulitis after microsurgery. Microsurgical lymphatic-venous anastomoses have a place in the treatment of peripheral lymphedema and should be the therapy of choice in patients who are not sufficiently responsive to nonsurgical treatment. Improved results can be expected with operations performed early, during the first stages of lymphedema.

PMID: 15129351 [PubMed - as supplied by publisher]

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Supermicrosurgical lymphaticovenular anastomosis for the treatment of lymphedema in the upper extremities.

Koshima I, Inagawa K, Urushibara K, Moriguchi T.

Department of Plastic and Reconstructive Surgery, Okayama University Medical School and Kawasaki Medical School, Japan.

Over the last eight years, the authors analyzed obstructive lymphedema of a unilateral upper extremity in a total of 27 females, comparing the use of supramicrosurgical lymphaticovenule anastomoses and/or conservative treatment. The most common cause of edema was mastectomy, with or without subsequent radiation therapy for breast cancer. As an objective assessment of the extent of edema, the circumferences of the affected and opposite normal forearms were measured at 10 cm below the olecranon of the arm. Twelve of these patients received continual bandaging. In these patients, the average excess circumference of the affected arm was 6.4 cm over that of the normal forearm; the average duration of edema before treatment was 3.5 years; the average period for conservative treatment was 10.6 months; and the average decrease in circumference was 0.8 cm (11.7 percent of the preoperative excess). Twelve patients underwent surgery and postoperative continual bandaging. In these patients, the average excess circumference was 8.9 cm; the average duration of edema before surgery was 8.2 years; the average follow-up after surgery was 2.2 years; and the average decrease in circumference was 4.1 cm (47.3 percent of the preoperative excess). These results indicated that supermicrolymphaticovenular anastomoses with postoperative bandaging have a valuable place in the treatment of obstructive lymphedema.

Publication Types:
Case Reports

PMID: 10993089 [PubMed - indexed for MEDLINE]

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Planning and monitoring of autologous lymph vessel transplantation by means of nuclear medicine lymphoscintigraphy

[Article in German to English]

Weiss M, Baumeister RG, Hahn K.

Klinik und Poliklinik fur Nuklearmedizin, Ludwig-Maximilians-Universitat Munchen, Germany. mayo.(email)weiss@nuk.med.uni-muenchen.de

Autologous lymph vessel transplantation significantly improves the lymph drainage in patients with primary and secondary lymphedema. The aim of the present study was to prove whether scintigraphic long-term follow-up could demonstrate the function of autologous lymph vessels and the persisting success of this microsurgical technique respectively. In this study, visual and semiquantitative lymphoscintigraphy was used to prove the function of lymphatic vessel grafts in 20 patients comparing a preoperative baseline study with postoperative follow-up investigations once a year for a period of seven years. The reason for microsurgical lymph vessel transplantation was a primary (n = 4) or a secondary (n = 16) lymphedema. In 12 cases the transplantation site was at the upper extremity, in eight cases at the lower limb. In 17/20 patients lymphatic function significantly improved after autologous lymph vessel transplantation compared to the preoperative findings, as verified by visual improvement of lymph drainage and decrease of a numeric transport index. In 5/20 cases the vessel graft could be visualized directly. In these patients with scintigraphic visualization of the vessel graft, the transport index decreased to a significantly greater extent compared to the preoperative baseline study. 3/20 patients did not benefit from microsurgical treatment. Lymphoscintigraphy has shown to be an easy, reliable and readily available technique to assess lymphatic function on the long run. Scintigraphic visualization of the vessel graft showed a significantly better postoperative outcome than those without. The scintigraphic visualization of the vessel graft therefore seems to indicate a favourable prognosis regarding lymph drainage.

PMID: 12968217 [PubMed - indexed for MEDLINE]

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Long-term follow-up after lymphaticovenular anastomosis for lymphedema in the leg.

Koshima I, Nanba Y, Tsutsui T, Takahashi Y, Itoh S.

Department of Plastic and Reconstructive Surgery, Graduate School of Medicine and Dentistry, Okayama University, Japan.

Over the last 9 years, the authors analyzed lymphedema of the lower extremity in a total of 25 patients, comparing the use of supermicrosurgical lymphaticovenular anastomosis and/or conservative treatment. The most common cause of edema was hysterectomy, with or without subsequent radiation therapy for uterine cancer. Among 12 cases that underwent only conservative treatment, only one case showed a decrease of over 4 cm in the circumference of the lower leg. The average period for conservative treatment was 1.5 years, and the average decreased circumference was 0.6 cm (8 percent of the preoperative excess). Thirteen patients were followed after lymphaticovenular anastomoses, as well as pre- and postoperative conservative treatment. The average follow-up after surgery was 3.3 years, and eight patients showed a reduction of over 4 cm in the circumference of the lower leg. The average decrease in the circumference, excluding edema in the bilateral leg, was 4.7 cm (55.6 percent of the preoperative excess). These results indicate that supermicrosurgical lymphaticovenular anastomosis has a valuable place in the treatment of lymphedema.

Publication Types:
Case Reports

PMID: 12858242 [PubMed - indexed for MEDLINE]

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Vein graft interposition in treating peripheral lymphoedemas.

Campisi C, Boccardo F.

Department of Specialistic Surgical Sciences, Anaesthesiology and Organ Transplants, Emergency Surgical Clinic Section, Lymphology and Microsurgery Centre, S. Martino Hospital, University of Genoa, Italy. campisi@unige.it

The technique of interposed vein grafts (Lymphatic-Venous-Lymphatic Plasty: LVLA) consists in using autologous vein grafts to reconstruct lymphatic pathways where there is a block to the lymphatic circulation of the limb due to a congenital or acquired reason. The venous segment represents a sort of "bridge" between afferent and efferent lymphatic collectors. The study aims at evaluating long-term results of the treatment of peripheral lymphoedemas by the microsurgical reconstructive technique of LVLA. The results proved to be positive also in the long term after microsurgical operation. The follow-up was performed by water volumetry and isotopic lymphography. This technique of interposed vein grafts allows peripheral lymphoedemas to be treated when derivative lympho-venous shunts can not be used due to an impaired venous circulation in the same lymphoedematous limb.

PMID: 12968219 [PubMed - indexed for MEDLINE]

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Supramicrosurgical lymphaticovenular anastomosis for the treatment of lymphedema in the extremities

[Article in Japanese]

Koshima I, Inagawa K, Etoh K, Moriguchi T.

Department of Plastic and Reconstructive Surgery, Kawasaki Medical School, Kurashiki, Japan.

During the past eight years, we treated obstructive lymphedema of a unilateral upper extremity in 27 females and of a unilateral or bilateral lower extremity in 35 males and females with supramicrosurgical lymphaticovenular anastomoses and/or conservative treatment. The most common cause of upper limb edema was mastectomy with or without subsequent radiation therapy for breast cancer, and that of lower limb edema was hysterectomy with radiation. As an objective assessment of edema, the circumferences of the affected and opposite normal forearms or lower legs were measured 10 cm below the olecranon of the arm or the lower border of the patella. In patients who received conservative treatment (12 arms and 12 legs), the average excess circumferential length of the affected arm and leg was 6.4 and 7.1 cm over that of normal extremities, average duration of edema before treatment was 3.5 and 5.2 years, average period for conservative treatment was 10.6 months and 1.5 years, and average decreased circumferential length was 0.8 and 0.6 cm, respectively. The rate of circumferential decrease over 4 cm was none in arm and 16.7% in leg edema. In patients who underwent surgery (12 arms and 16 legs), the average excess circumferencial length was 8.9 and 9.8 cm, average duration of edema before surgery was 8.2 and 8.9 years, average follow-up after surgery was 2.2 and 3.3 years, and average decrease in excess circumference was 4.1 and 2.7 cm, respectively. The rate of circumferential decrease over 4 cm was 58.3% in arms and 50% in legs. These results indicate that supramicrolymphaticovenular anastomoses have a valuable place in the treatment of obstructive lymphedema.

PMID: 10516971 [PubMed - indexed for MEDLINE]

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DIFFERENTIAL DIAGNOSIS, INVESTIGATION, AND CURRENT TREATMENT OF LOWER LIMB LYMPHEDEMA

07/12/04

Tiwari A, Cheng KS, Button M, Myint F, Hamilton G. Arch Surg. 2003;138:152-161.

This is a very useful cumulative review. Its objective was to look at the differential diagnosis, investigation methods, and treatments for lower-limb edema available in the West. The article needs some comments and updating.

The classic classification of lymphedema proposed by Kinmonth is largely outdated. It applies mostly to so-called “primary” lymphedema. Lymphoscintigraphy and histological studies of lymphatics and nodes in man have shown that both lymph vessels and nodes are normally developed and have a normal structure. There are, however, major degenerative changes, such as subintimal hyalinosis and depletion of node lymphocytes. This is why vessels and nodes appear as thin and small structures on lymphography. Generally, we only talk today about acquired or obstructive lymphedema unless there are obvious malformations (Milroy’s disease and others). Addressing problems of secondary lymphedema, the authors state that lymphatics have “excellent regenerative capabilities.”

This is the case in rodents, but not so much in humans. In man, after lymph node dissection, there is an immediate growth of minute lymphatics bridging the gap but never of lymphatic collectors. These vessels undergo rapid occlusion by a scar. In addition, skin infections, which are very common in lymphedema, totally destroy the lymphatics over the course of time.

Secondary lymphedema may not necessarily develop first in the foot and calf. It can develop primarily in the thigh or arm after lymph node removal.

In mixed venolymphatic limb edema lymphoscintigraphy is extremely useful in differentiation from pure lymphedema. In addition, this diagnostic technique has recently become very useful in diagnosis of lymphedema after bone fractures. We found that long-lasting posttraumatic edema is asssociated with dilatation of lymphatics, slow lymph flow, and enlargement of nodes, which lasts for months.

With respect to massage therapy, the tissue fluid is pressed through the tissue and perivascular spaces and not through obstructed lymphatics. This was nicely shown on our lymphoscintigrams.

Unfortunately, this review does not mention the generally accepted method of treatment of acute episodes of dermatolymphangioadenitis (DLA). This is seen in more than 50% of patients with obstructive lymphedema. Treatment is with antibiotics and these may have to be given intravenously in severe cases. Also, there is no mention of prophylaxis of DLA recurrences by administration of long-acting penicillin.

Finally, as a designer of lymphovenous shunts (1966), I should correct the authors’ comments on “shunts occluded owing to venous thrombosis.” This was not true. Surprisingly, no thrombosis was seen in our first five clinical cases of inguinal node-femoral vein shunt on phlebography. There was a total endothelialization of the cut surface of the implanted node and a nice union of venous and lymphatic endothelium.

"Next issue (N°29) of the International Venous Digest by Fax will be sent to you on 07 September 04. We wish you an enjoyable summer break."

Prof Waldemar Olszewski, Warsaw, Poland

Servier

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Lymphedema Shunts

Do lymph node vein anastomoses work?

Answer:

(From Grabb and Smith CD-ROM:)

“…Lymphovenous and lymph node–venous shunts have been performed since the 1960's. In these procedures, a neighboring vein is anastomosed to lymphatic vessel or node. Lympholymphatic shunts were developed in the 1970's and are utilized to bypass regions of lymphatic obstruction. In these procedures, autologous lymphatic vessels are harvested from a nondiseased extremity and transposed or transplanted to bridge-occluded lymphatics. These shunts have been used in selected patients with hyperplastic lymphedema, but are ineffective in hypoplastic types, which represents most cases of primary lymphedema. Microlymphaticovenous anastomosis has also been used for lymphedema of male and female genitalia…”

Yale Surgery

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Post-mastectomy lymphedema: surgical therapy

Campisi C, Boccardo F, Casaccia M.

Dipartimento di Scienze Chirurgiche Specialistiche, Anestesiologia e Trapianti d'Organo (DISCAT), Sezione di Clinica Chirurgica d'Urgenza, Centro di Linfologia e Microchirurgia, Ospedale S. Martino, Universita degli Studi di Genova. campisi@unige.it

After some preliminary remarks concerning epidemiological data about post-mastectomy lymphedema, on the basis of specific etiologic and pathophysiologic aspects, authors report a modern clinical and instrumental staging of lymphedema and an accurate diagnostic protocol, which allows not only to study lymphedema at late stages, but also to individuate the disease at earliest stages. Protocols of medical, physical and rehabilitative treatment mostly used today are schematically described, and they include proper igienic measures for the prevention bacterial and micotic infections, manual lymph drainage, sequential compression therapy, exercises, thermotherapy, bandages and elastic garments. Authors underline above all the importance of Microsurgery in treating post-mastectomy lymphedema, by means of modern methods of lymphatic microsurgery, derivative or reconstructive (multiple lymphatic-venous anastomoses, lymphatic-venous-lymphatic plasty). The operation of multiple lymphatic-venous anastomoses represent the mostly used technique. The registry consists of 194 microsurgical operations, performed in patients treated and followed-up statistically in the last 15 years, with positive result in over 80% of cases.

MeSH Terms:

Arm*/surgery
Comparative Study
English Abstract
Female
Follow-Up Studies
Human
Lymphatic System/surgery
Lymphedema/diagnosis
Lymphedema/etiology
Lymphedema/rehabilitation
Lymphedema/surgery*
Mastectomy/adverse effects*
Microsurgery
Physical Therapy Techniques
Time Factors

PMID: 12704985 [PubMed - indexed for MEDLINE]

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Lymphoscintigraphy for non-invasive long term follow-up of functional outcome in patients with autologous lymph vessel transplantation

[Article in German]

Weiss M, Baumeister RG, Tatsch K, Hahn K.

Klinik und Poliklinik fur Nuklearmedizin, Ludwig-Maximilians-Universitat Munchen, Deutschland.

AIM: Autologous lymph vessel transplantation significantly improves the lymphdrainage in patients with primary and secondary lymphedema. The aim of the present study was to answer the question, whether scintigraphic long-term follow up and semiquantitative evaluation of lymphatic flow could prove the persisting success of this sophisticated microsurgical technique.

METHODS: In this study visual and semiquantitative lymphoscintigraphy was used to prove the function of lymphatic vessel grafts in 20 patients (17 females, 3 males) comparing a preoperative baseline study with postoperative follow up investigations for a period of 7 years. The reason for microsurgical lymph vessel transplantation was in 4 patients a primary and in 16 patients a secondary lymphedema. In 12 cases the transplantation site was at the upper extremity, in 8 cases at the lower limb. RESULTS: In 17/20 patients lymphatic function significantly improved after autologous lymph vessel transplantation compared to the preoperative findings, as verified by visual improvement of lymph drainage and decrease of a numeric transportindex. In 5 cases the vessel graft could be directly visualized. In these patients with scintigraphic visualization of the vessel graft the transportindex decreases to a significantly greater extent compared to the preoperative baseline study. Only 3 patients did not benefit from microsurgical treatment

CONCLUSION: Lymphoscintigraphy combined with semiquantitative estimation of lymphatic transport kinetics has shown to be an easy, reliable and readily available technique to assess lymphatic function before and after autologous lymph vessel transplantation. Thus, the method is not only helpful in planning microsurgical treatment but also in monitoring the postoperative improvement of lymph drainage. Patients with scintigraphic visualization of the vessel graft showed a significant better postoperative outcome than those without. The scintigraphic visualization of the vessel graft therefore seems to indicate a favourable prognosis regarding to lymph drainage.

PMID: 8999422 [PubMed - indexed for MEDLINE]

The purpose of this study was to monitor the functional outcome of microsurgical intervention on lymph drainage by means of non-invasive, readily available lymphoscintigraphy. Eight patients with primary or secondary lymphoedema of the lower limb were investigated before and for 8 years after autologous lymph vessel transplantation. For scintigraphy, technetium-99m labelled nanocolloid was subcutaneously injected into the first interdigital space of the affected limb. Sequential images were acquired up to 6 h p.i.; for semiquantitative evaluation a numerical transport index was established by assigning scores of up to 9 on each of five criteria: lymphatic transport kinetics, distribution pattern of the radiopharmaceutical, time to appearance of lymph nodes, visualisation of lymph nodes and visualisation of lymph vessels/grafts. Ti values <10 were considered normal. In all eight patients, lymphatic function significantly (P</=0.01) improved after microsurgical treatment. Permanent function of vessel grafts was indicated by persistently low Ti values during the entire observation period, impressively demonstrating the success of this complex microsurgical technique. Patients with scintigraphic visualisation of the vessel graft (n=2/8) showed a substantially better postoperative outcome than those without visualisation of the vessel graft. The findings indicate that lymph vessel transplantation significantly improves lymph drainage in patients with primary or secondary lymphoedema of the lower limb. Thus, lymphoscintigraphy is helpful not only in planning microsurgical treatment but also in monitoring the postoperative outcome.

Publication Types:

Microsurgical lymphovenous anastomosis for treatment of lymphedema: a critical review.

Gloviczki P, Fisher J, Hollier LH, Pairolero PC, Schirger A, Wahner HW.

Section of Vascular Surgery, Mayo Clinic, Rochester, MN 55905.

Lymphovenous anastomoses (LVA) offer ideal physiologic treatment for lymphedema, and our experimental data support late patency. Between Jan. 1, 1982, and April 1, 1986, 18 patients underwent operation for chronic lymphedema; LVA could be performed in 14 patients (10 women and four men). Six patients had secondary lymphedema of the upper extremity. One of eight patients with lymphedema of the lower extremity had filariasis, and seven had primary lymphedema. Mean follow-up was 36.6 months (range: 5 to 57 months). Limb circumference and volume, number of postoperative episodes of cellulitis, and lymphoscintigraphy were used to assess results. Improvement occurred in three upper extremities and two lower extremities. There was no change in five extremities, and in four patients the edema progressed. One patient with primary lymphedema and four of seven patients with secondary lymphedema improved. Only one of five patients benefited from one anastomosis; however, all patients with more than two anastomoses improved. Lymphoscintigraphy was performed in 10 patients. No lymphatic channel was visualized before operation in three patients, and at operation none was found. In four other patients lymph channels localized by lymphoscintigraphy were identified during operation. Significant improvement was documented by lymphoscintigraphy in one patient after operation, and this patient had permanent improvement 30 months later. Patients with primary lymphedema had disappointing results, but four of seven patients with secondary lymphedema benefited from LVA, especially if several anastomoses could be performed. Lymphoscintigraphy appears to be a suitable method of both identifying patent lymph channels before surgery and determining function of LVA after operation. However, presently objective data to prove the clinical efficacy of this operation are lacking.

PMID: 3367429 [PubMed - indexed for MEDLINE]

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While we have a number of support groups for lymphedema... there is nothing out there for other lymphatic disorders. Because we have one of the most comprehensive information sites on all lymphatic disorders, I thought perhaps, it is time that one be offered.

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