Lymphedematous HIV-associated Kaposi's sarcoma.
July 2006 - Journal of Cutaneous Pathology
Ramdial PK, Chetty R, Singh B, Singh R, Aboobaker J.
Department of Anatomical Pathology, Nelson R Mandela School of Medicine, Durban, South Africa.
Advanced Kaposi's sarcoma is frequently associated with chronic lymphedema (cLO). The histopathological features of lymphedematous HIV-associated KS (KS) are poorly documented and the co-existence of fibroma-like nodules in lymphedematous KS is under-recognized. The aims of this study were to assess the clinicopathological spectrum and diagnostic difficulties associated with lymphedematous KS and to highlight the clinicopathological profile of fibroma-like nodules. In addition, the pathogenesis of fibroma-like nodules and cLO is revisited.
Prospective 17-month clinicopathological study of all biopsies from patients with lymphedematous KS. Seventy-four biopsies, the majority from the lower limbs, from 41 patients were evaluated. Nineteen, 14, five and three patients had one, two, three or four biopsies each, respectively. In 14 biopsies, there was poor clinicopathological correlation of KS stage. Exclusive lesional KS (patch, plaque, nodule or lymphangioma-like) was identified in 29 biopsies; 23 and eight biopsies demonstrated KS or fibroma-like morphology and the adjacent dermis demonstrated cLO. There was variable intratumoral and peritumoral venous compression and lymphatic dilatation. Fourteen biopsies demonstrated cLO exclusively.
Smaller fibroma-like nodules lacked KS spindle cells, whereas >5 mm nodules demonstrated focal KS spindle cell proliferation and aggregation on extensive sectioning. The subcutis of 42 biopsies demonstrated variable fibrosis, hemosiderin deposits, lymphocytes, plasma cells, KS, interstitial granular material and pools of lymph fluid. Subcutaneous abscesses were identified in six biopsies.
All biopsies had variable epidermal features of cLO. cLO influences clinicopathological correlation of KS stage and may also mask the presence of KS and the co-existence of subcutaneous abscesses. Smaller fibroma-like nodules are hypothesized to be a manifestation of cLO that have the potential to acquire the characteristics of KS. Lymphatic and venous obstruction, protein-rich interstitial fluid, tissue hemosiderin and subcutaneous infection are hypothesized to play a combined role in the evolution and perpetuation of cLO.
PMID: 16872469 [PubMed - in process]
Monday, July 31, 2006
Sunday, July 23, 2006
A New Mouse Model to Study Acquired Lymphedema
Martin Schneider, Annelii Ny, Carmen Ruiz de Almodovar, and Peter Carmeliet*
Competing Interests: The authors have declared that no competing interests exist.
Martin Schneider, Annelii Ny, Carmen Ruiz de Almodovar, and Peter Carmeliet are at the Center for Transgene Technology and Gene Therapy, Flanders Interuniversity Institute for Biotechnology, University of Leuven, Leuven, Belgium.
* To whom correspondence should be addressed. E-mail: peter.carmeliet@med.kuleuven.be
PLoS Med. 2006 July; 3(7): e264. Published online 2006 July 18. doi: 10.1371/journal.pmed.0030264.
Copyright : © 2006 Schneider et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
More than 1 million women worldwide develop breast cancer every year [ 1]. For many of them, surgical removal of the breast remains the first line of treatment for this potentially deadly disease. Because breast cancer cells spread via lymph vessels, at the time that the breast is resected, locally involved lymph nodes in the armpit and part of the axillary lymphatic network are usually also removed. Consequently, normal drainage of lymph is often interrupted, causing swelling of the affected arm due to lymph accumulation—a condition termed lymphedema. Acquired lymphedema in humans may also result from irradiation, trauma, or (parasitic) infection.
While the swelling causes discomfort and disability, an even greater health threat lies hidden in the structural and functional changes inside the chronically lymphedemic tissue. Adipocytes, keratinocytes, and fibroblasts accumulate, transforming the initially soft swollen tissue into a hard fibrotic mass with fatty degeneration and a stiff, thickened skin [ 2–4]. In addition, there is reduced trafficking of antigen-presenting and other immune cells to the lymph nodes, and so these cells are less likely to be able to perform their immune surveillance function to defend the host against foreign antigens. As a result, the tissues affected by lymphedema are prone to persistent inflammation and infection [ 2, 4]. The increased interstitial tissue pressure may collapse the veins, further aggravating the condition and in severe cases even necessitating amputation.
Almost unimaginably, close to 400 years after the discovery of lymph vessels, there is still no cure for lymphedema, and current medical practice still relies on ancient procedures, such as manual lymph drainage via massage. A better knowledge of the molecular cues underlying the abnormalities that characterize the inflammatory tissue response to lymph stagnation is thus urgently needed to provide novel perspectives for lymphedema treatment. However, research progress in the field has been hampered by the lack of suitable experimental animal models. The new mouse model of acquired lymphatic insufficiency reported by Tabibiazar et al. in PLoS Medicine [ 5] may help to overcome some of these obstacles.
There is still no cure for lymphedema, and current medical practice relies on ancient procedures.
The New Mouse Model
How did the authors develop and characterize the model? Microsurgical ablation of the lymph vessels in the tail of the mouse resulted in lymph stagnation, lymph vessel dilation (with a marked increase in tail volume), accumulation of fibroblasts, fat, and skin cells, impaired clearance of immune cells from the tail, and profound accumulation of inflammatory cells. This novel model thus closely recapitulates several hallmark features of acquired lymphedema in humans.
The model also differs from the human disease in certain aspects, however. For instance, a mouse tail lacks lymph nodes, which may play a critical role in fostering the immune response and inflammation in the edematous tissue in human lymphedema [ 6]. It remains to be assessed whether the absence of lymph nodes influences the tissue response to lymph stasis in the mouse tail. The hydrodynamic and cellular mechanisms and the rate of lymph drainage in a horizontally positioned mouse tail also differ from those in a supine human limb, in which positional changes and muscle contractions determine lymph drainage [ 7]. It is unclear to what extent these differences influence the disease course and severity. Moreover, it will be interesting to characterize the long-term chronic structural changes in the lymphedemic mouse tail.
Interestingly, not only were lymph vessels dilated in the mouse tail model, but there were also 10-fold more lymphatic vessels in the lymphedemic tail than in control tails. It remains to be determined whether this increase in the number of lymphatic vessels in the lymphedemic tissue is a peculiarity of the mouse tail model, as very little information about lymphatic hyperplasia in humans with lymphedema is available. While the precise reason for this increased lymphatic vessel density remains unclear, it is conceivable that the large accumulation of inflammatory cells and the release of inflammatory cytokines may have stimulated lymphangiogenesis [ 8, 9]. Another unresolved issue is to what extent inflammatory cells are merely trapped in the edematous tissue as silent bystanders or, instead, actively determine disease progression. The role of inflammation in the tissue response to lymphedema in this mouse model could be readily tested by using immunocompromised mice or knockout mice lacking specific inflammatory mediators, or by treating mice with anti-inflammatory agents.
Comparison with Previous Models
To what extent does this new model differ from existing ones? Is the new model any better?
Several models of lymphedema have been previously developed. Some were developed via surgical ablation or irradiation of the lymphatics in the rabbit ear or mouse tail [ 10–13], others via inactivation or mutation of lymphangiogenic genes in mice, such as the Chy mice carrying a heterozygous Vegfr3 mutation [ 14]. These models have been generally used to study the physiological regulation of lymph flow and to assess the therapeutic potential of VEGF-C to stimulate lymphatic revascularization.
However, apart from the Prox1 +/− mouse, which accumulates fat as a consequence of lymphatic vascular leakage [ 15], none of these models has been used to study the chronic secondary tissue changes in response to lymphedema. Thus, unlike the previous models, Tabibiazar and colleagues' new mouse model of acquired lymphedema, in combination with powerful mouse genetics, offers exciting opportunities to dissect the molecular basis of the response to lymphedema. In addition, this new model should be valuable to evaluate the therapeutic potential of novel anti-lymphedema drugs.
Clinical Implications
One of the reasons why treatment of lymphedema is still in its infancy relates to our lack of understanding of the changes induced by lymph stagnation. Previous studies primarily focused on identifying the gene programs switched on in lymphatic endothelial cells in response to lymphangiogenic stimuli [ 16, 17]. However, more cell types participate in the complex response to lymph stasis than lymphatic endothelial cells alone. For instance, chyle itself stimulates adipogenesis [ 15], and it is conceivable that yet unidentified chemokines and growth factors in lymph affect other cell types in edematous tissues as well.
As Tabibiazar et al. show, their new model provides opportunities to identify sets of genes previously unsuspected to be involved in acquired lymphedema. Their current analysis has already identified genes regulating inflammation, immunity, complement activation, fibrosis, oxidative stress, and angiogenesis. Obviously, the functional significance of the current gene profile will need to be further validated. Other small animal models, such as the recently generated Xenopus laevis tadpole model of lymphangiogenesis, may accelerate the functional screening of such candidates [ 18]. Overall, the new mouse model of acquired lymphedema promises to increase our understanding of this largely unexplained disease, and hopefully accelerate the development and testing of new treatments for this devastating disorder.
Footnotes
Funding: MS is supported by the Deutsche Forchungsgemeinschaft (Germany), AN is supported by the European Union Sixth Framework Programme via a Marie Curie Intra European Fellowship, and CRA is a recipient of a fellowship from the Federation of European Biochemical Societies.
Citation: Schneider M, Ny A, Ruiz de Almodovar C, Carmeliet P (2006) A new mouse model to study acquired lymphedema. PLoS Med 3: e264. DOI: 10.1371/journal.pmed.0030264
References
Stewart BW, Kleihues P. , editors. World cancer report. Geneva: World Health Organization; 2003. 352 pp.
Alitalo K, Tammela T, Petrova TV. Lymphangiogenesis in development and human disease. Nature. 2005;438:946–953. [PubMed]
Rockson SG. Lymphedema. Curr Treat Options Cardiovasc Med. 2006;8:129–136. [PubMed]
Szuba A, Rockson SG. Lymphedema: Anatomy, physiology and pathogenesis. Vasc Med. 1997;2:321–326. [PubMed]
Tabibiazar R, Cheung L, Han J, Swanson J, Beilhack A, et al. Inflammatory manifestations of experimental lymphatic insufficiency. PLoS Med. 2006;3:e254. doi: 10.1371/journal.pmed.0030254. [PubMed]
Angeli V, Ginhoux F, Llodra J, Quemeneur L, Frenette PS, et al. B cell-driven lymphangiogenesis in inflamed lymph nodes enhances dendritic cell mobilization. Immunity. 2006;24:203–215. [PubMed]
Swartz MA, Boardman KC. The role of interstitial stress in lymphatic function and lymphangiogenesis. Ann N Y Acad Sci. 2002;979:197–210. [PubMed]
Mouta C, Heroult M. Inflammatory triggers of lymphangiogenesis. Lymphat Res Biol. 2003;1:201–218. [PubMed]
Ristimaki A, Narko K, Enholm B, Joukov V, Alitalo K. Proinflammatory cytokines regulate expression of the lymphatic endothelial mitogen vascular endothelial growth factor-C. J Biol Chem. 1998;273:8413–8418. [PubMed]
Hagendoorn J, Padera TP, Kashiwagi S, Isaka N, Noda F, et al. Endothelial nitric oxide synthase regulates microlymphatic flow via collecting lymphatics. Circ Res. 2004;95:204–209. [PubMed]
Lee-Donaldson L, Witte MH, Bernas M, Witte CL, Way D, et al. Refinement of a rodent model of peripheral lymphedema. Lymphology. 1999;32:111–117. [PubMed]
Szuba A, Skobe M, Karkkainen MJ, Shin WS, Beynet DP, et al. The
rapeutic lymphangiogenesis with human recombinant VEGF-C. FASEB J. 2002;16:1985–1987. [PubMed]
Yoon YS, Murayama T, Gravereaux E, Tkebuchava T, Silver M, et al. VEGF-C gene therapy augments postnatal lymphangiogenesis and ameliorates secondary lymphedema. J Clin Invest. 2003;111:717–725. [PubMed]
Karkkainen MJ, Saaristo A, Jussila L, Karila KA, Lawrence EC, et al. A model for gene therapy of human hereditary lymphedema. Proc Natl Acad Sci U S A. 2001;98:12677–12682. [PubMed]
Harvey NL, Srinivasan RS, Dillard ME, Johnson NC, Witte MH, et al. Lymphatic vascular defects promoted by Prox1 haploinsufficiency cause adult-onset obesity. Nat Genet. 2005;37:1072–1081. [PubMed]
Hong YK, Shin JW, Detmar M. Development of the lymphatic vascular system: A mystery unravels. Dev Dyn. 2004;231:462–473. [PubMed]
Tammela T, Petrova TV, Alitalo K. Molecular lymphangiogenesis: New players. Trends Cell Biol. 2005;15:434–441. [PubMed]
Ny A, Koch M, Schneider M, Neven E, Tong RT, et al. A genetic Xenopus laevis tadpole model to study lymphangiogenesis . Nat Med. 2005;11:998–1004. [PubMed]
PLOS MEDICINE
Competing Interests: The authors have declared that no competing interests exist.
Martin Schneider, Annelii Ny, Carmen Ruiz de Almodovar, and Peter Carmeliet are at the Center for Transgene Technology and Gene Therapy, Flanders Interuniversity Institute for Biotechnology, University of Leuven, Leuven, Belgium.
* To whom correspondence should be addressed. E-mail: peter.carmeliet@med.kuleuven.be
PLoS Med. 2006 July; 3(7): e264. Published online 2006 July 18. doi: 10.1371/journal.pmed.0030264.
Copyright : © 2006 Schneider et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
More than 1 million women worldwide develop breast cancer every year [ 1]. For many of them, surgical removal of the breast remains the first line of treatment for this potentially deadly disease. Because breast cancer cells spread via lymph vessels, at the time that the breast is resected, locally involved lymph nodes in the armpit and part of the axillary lymphatic network are usually also removed. Consequently, normal drainage of lymph is often interrupted, causing swelling of the affected arm due to lymph accumulation—a condition termed lymphedema. Acquired lymphedema in humans may also result from irradiation, trauma, or (parasitic) infection.
While the swelling causes discomfort and disability, an even greater health threat lies hidden in the structural and functional changes inside the chronically lymphedemic tissue. Adipocytes, keratinocytes, and fibroblasts accumulate, transforming the initially soft swollen tissue into a hard fibrotic mass with fatty degeneration and a stiff, thickened skin [ 2–4]. In addition, there is reduced trafficking of antigen-presenting and other immune cells to the lymph nodes, and so these cells are less likely to be able to perform their immune surveillance function to defend the host against foreign antigens. As a result, the tissues affected by lymphedema are prone to persistent inflammation and infection [ 2, 4]. The increased interstitial tissue pressure may collapse the veins, further aggravating the condition and in severe cases even necessitating amputation.
Almost unimaginably, close to 400 years after the discovery of lymph vessels, there is still no cure for lymphedema, and current medical practice still relies on ancient procedures, such as manual lymph drainage via massage. A better knowledge of the molecular cues underlying the abnormalities that characterize the inflammatory tissue response to lymph stagnation is thus urgently needed to provide novel perspectives for lymphedema treatment. However, research progress in the field has been hampered by the lack of suitable experimental animal models. The new mouse model of acquired lymphatic insufficiency reported by Tabibiazar et al. in PLoS Medicine [ 5] may help to overcome some of these obstacles.
There is still no cure for lymphedema, and current medical practice relies on ancient procedures.
The New Mouse Model
How did the authors develop and characterize the model? Microsurgical ablation of the lymph vessels in the tail of the mouse resulted in lymph stagnation, lymph vessel dilation (with a marked increase in tail volume), accumulation of fibroblasts, fat, and skin cells, impaired clearance of immune cells from the tail, and profound accumulation of inflammatory cells. This novel model thus closely recapitulates several hallmark features of acquired lymphedema in humans.
The model also differs from the human disease in certain aspects, however. For instance, a mouse tail lacks lymph nodes, which may play a critical role in fostering the immune response and inflammation in the edematous tissue in human lymphedema [ 6]. It remains to be assessed whether the absence of lymph nodes influences the tissue response to lymph stasis in the mouse tail. The hydrodynamic and cellular mechanisms and the rate of lymph drainage in a horizontally positioned mouse tail also differ from those in a supine human limb, in which positional changes and muscle contractions determine lymph drainage [ 7]. It is unclear to what extent these differences influence the disease course and severity. Moreover, it will be interesting to characterize the long-term chronic structural changes in the lymphedemic mouse tail.
Interestingly, not only were lymph vessels dilated in the mouse tail model, but there were also 10-fold more lymphatic vessels in the lymphedemic tail than in control tails. It remains to be determined whether this increase in the number of lymphatic vessels in the lymphedemic tissue is a peculiarity of the mouse tail model, as very little information about lymphatic hyperplasia in humans with lymphedema is available. While the precise reason for this increased lymphatic vessel density remains unclear, it is conceivable that the large accumulation of inflammatory cells and the release of inflammatory cytokines may have stimulated lymphangiogenesis [ 8, 9]. Another unresolved issue is to what extent inflammatory cells are merely trapped in the edematous tissue as silent bystanders or, instead, actively determine disease progression. The role of inflammation in the tissue response to lymphedema in this mouse model could be readily tested by using immunocompromised mice or knockout mice lacking specific inflammatory mediators, or by treating mice with anti-inflammatory agents.
Comparison with Previous Models
To what extent does this new model differ from existing ones? Is the new model any better?
Several models of lymphedema have been previously developed. Some were developed via surgical ablation or irradiation of the lymphatics in the rabbit ear or mouse tail [ 10–13], others via inactivation or mutation of lymphangiogenic genes in mice, such as the Chy mice carrying a heterozygous Vegfr3 mutation [ 14]. These models have been generally used to study the physiological regulation of lymph flow and to assess the therapeutic potential of VEGF-C to stimulate lymphatic revascularization.
However, apart from the Prox1 +/− mouse, which accumulates fat as a consequence of lymphatic vascular leakage [ 15], none of these models has been used to study the chronic secondary tissue changes in response to lymphedema. Thus, unlike the previous models, Tabibiazar and colleagues' new mouse model of acquired lymphedema, in combination with powerful mouse genetics, offers exciting opportunities to dissect the molecular basis of the response to lymphedema. In addition, this new model should be valuable to evaluate the therapeutic potential of novel anti-lymphedema drugs.
Clinical Implications
One of the reasons why treatment of lymphedema is still in its infancy relates to our lack of understanding of the changes induced by lymph stagnation. Previous studies primarily focused on identifying the gene programs switched on in lymphatic endothelial cells in response to lymphangiogenic stimuli [ 16, 17]. However, more cell types participate in the complex response to lymph stasis than lymphatic endothelial cells alone. For instance, chyle itself stimulates adipogenesis [ 15], and it is conceivable that yet unidentified chemokines and growth factors in lymph affect other cell types in edematous tissues as well.
As Tabibiazar et al. show, their new model provides opportunities to identify sets of genes previously unsuspected to be involved in acquired lymphedema. Their current analysis has already identified genes regulating inflammation, immunity, complement activation, fibrosis, oxidative stress, and angiogenesis. Obviously, the functional significance of the current gene profile will need to be further validated. Other small animal models, such as the recently generated Xenopus laevis tadpole model of lymphangiogenesis, may accelerate the functional screening of such candidates [ 18]. Overall, the new mouse model of acquired lymphedema promises to increase our understanding of this largely unexplained disease, and hopefully accelerate the development and testing of new treatments for this devastating disorder.
Footnotes
Funding: MS is supported by the Deutsche Forchungsgemeinschaft (Germany), AN is supported by the European Union Sixth Framework Programme via a Marie Curie Intra European Fellowship, and CRA is a recipient of a fellowship from the Federation of European Biochemical Societies.
Citation: Schneider M, Ny A, Ruiz de Almodovar C, Carmeliet P (2006) A new mouse model to study acquired lymphedema. PLoS Med 3: e264. DOI: 10.1371/journal.pmed.0030264
References
Stewart BW, Kleihues P. , editors. World cancer report. Geneva: World Health Organization; 2003. 352 pp.
Alitalo K, Tammela T, Petrova TV. Lymphangiogenesis in development and human disease. Nature. 2005;438:946–953. [PubMed]
Rockson SG. Lymphedema. Curr Treat Options Cardiovasc Med. 2006;8:129–136. [PubMed]
Szuba A, Rockson SG. Lymphedema: Anatomy, physiology and pathogenesis. Vasc Med. 1997;2:321–326. [PubMed]
Tabibiazar R, Cheung L, Han J, Swanson J, Beilhack A, et al. Inflammatory manifestations of experimental lymphatic insufficiency. PLoS Med. 2006;3:e254. doi: 10.1371/journal.pmed.0030254. [PubMed]
Angeli V, Ginhoux F, Llodra J, Quemeneur L, Frenette PS, et al. B cell-driven lymphangiogenesis in inflamed lymph nodes enhances dendritic cell mobilization. Immunity. 2006;24:203–215. [PubMed]
Swartz MA, Boardman KC. The role of interstitial stress in lymphatic function and lymphangiogenesis. Ann N Y Acad Sci. 2002;979:197–210. [PubMed]
Mouta C, Heroult M. Inflammatory triggers of lymphangiogenesis. Lymphat Res Biol. 2003;1:201–218. [PubMed]
Ristimaki A, Narko K, Enholm B, Joukov V, Alitalo K. Proinflammatory cytokines regulate expression of the lymphatic endothelial mitogen vascular endothelial growth factor-C. J Biol Chem. 1998;273:8413–8418. [PubMed]
Hagendoorn J, Padera TP, Kashiwagi S, Isaka N, Noda F, et al. Endothelial nitric oxide synthase regulates microlymphatic flow via collecting lymphatics. Circ Res. 2004;95:204–209. [PubMed]
Lee-Donaldson L, Witte MH, Bernas M, Witte CL, Way D, et al. Refinement of a rodent model of peripheral lymphedema. Lymphology. 1999;32:111–117. [PubMed]
Szuba A, Skobe M, Karkkainen MJ, Shin WS, Beynet DP, et al. The
rapeutic lymphangiogenesis with human recombinant VEGF-C. FASEB J. 2002;16:1985–1987. [PubMed]
Yoon YS, Murayama T, Gravereaux E, Tkebuchava T, Silver M, et al. VEGF-C gene therapy augments postnatal lymphangiogenesis and ameliorates secondary lymphedema. J Clin Invest. 2003;111:717–725. [PubMed]
Karkkainen MJ, Saaristo A, Jussila L, Karila KA, Lawrence EC, et al. A model for gene therapy of human hereditary lymphedema. Proc Natl Acad Sci U S A. 2001;98:12677–12682. [PubMed]
Harvey NL, Srinivasan RS, Dillard ME, Johnson NC, Witte MH, et al. Lymphatic vascular defects promoted by Prox1 haploinsufficiency cause adult-onset obesity. Nat Genet. 2005;37:1072–1081. [PubMed]
Hong YK, Shin JW, Detmar M. Development of the lymphatic vascular system: A mystery unravels. Dev Dyn. 2004;231:462–473. [PubMed]
Tammela T, Petrova TV, Alitalo K. Molecular lymphangiogenesis: New players. Trends Cell Biol. 2005;15:434–441. [PubMed]
Ny A, Koch M, Schneider M, Neven E, Tong RT, et al. A genetic Xenopus laevis tadpole model to study lymphangiogenesis . Nat Med. 2005;11:998–1004. [PubMed]
PLOS MEDICINE
Sunday, July 16, 2006
Lymphedema Patient Education - Editorial
We who have lymphedema are usually met with either indifference or ignorance of our condition by the medical community. From the discussions in so many of the online lymphedema support groups, this would outwardly appear as one of the biggest obstacles to achieving both a correct diagnosis and treatment of the condition.
I want to share a post I made in our Advocates for Lymphedema group, this past week in reference to this:
Comment from Anne, one of our members:
Maybe we need to go back to the very basic education and left people know that LE is treated in the same way whether it's primary or secondary. For instance, lower leg LE requires MLD and bandaging or compression garb whetherit'sprimary or secondary. Anne
My response:
And that the result is going to be the same if untreated...same end....same complications. There are also so many challenges too. Not only is education for the medical community critical, but the larger community as well...including those who make the laws.
But, I am beginning to think patient education may well be the greatest challenge of them all. There are so many many sites on the internet with credible, valuable information.
Organizations like the NLN, LRF, Lymph Canada, Lymph Assoc of Australia on and on that work as hard as they can to bring this information to the patients.Yet, I have noticed that the amount of misinformation has absolutely exploded...and what has happened????
Patients are drawn to that instead of to the solid information. At some point we as patients have to have enough self value and discernment to demand proof and evidence for what we read....and stop devouring info that in the long run is going to be damaging for us.
I know that sometimes it seems I go overboard about this. Maybe I do...but lymphedema is something that I have had to experience every single, solitaryday of my life. And when the curtains falls, it probably won't be from lymphoma...it will be from what is happening with the hereditary lymphedema.
So for me it is serious serious business. A quickie example and then I'll get off my soap box :-)
The very best document available any where on travel precuations with lymphedema is from the National Lymphedema Network....and to their credit, they have updated it a well based on new information.
Yet...circulating now among the online support groups is a document ontravel precautions specifically for chronic venous insufficiency. The sad thing is that patients are gobbling this up and thinking it will help them with lymphedema. What's up with things like that????????
Those precautions for CVI will spell serious trouble for lymphedema patients...so why are we reading and believing that document while ignoring the NLN's document....I honestly truely don't understand this.
So how do we crack this nut??
How do we get patients to care enough about themselves to want peer reviewed, credible information?
OK....I'm done....time for another round of morning coffee.
Pat
There are other glaring examples from just the last couple weeks.
In one group a woman posts that she uses the "ancient Mayan Abdominal massage." Why? because she claims it helps circulation and helps "organ alignment."
In yet another group, someone says we need to have a vegetarian diet because there are toxins and bacteria that immediately go to the lymphatics of the neck when we eat certain foods and meats...even before we swallow the food.
Then there was a post on the 7 stages of lymphedema....ironically this list is an old staging system for lymphatic filiarsis and has nothing to do with either primary lymphedema or secondary lymphedem as we experience it in the industrialized nations.
And in the same group that brough us the air travel misinformation and the staging info another one that states in stage zero, the lymphatics are dilated. Wrong, it depends on the lymphedema involved as to whether or not the lymphatics are dilated or constricted.
I could go on and on, but I am sure you get the jest. There simply is no substitution for evidence based medicine and scientific information.
This kind of proaganda is put out through the internet by patients who act as self-appointed "gurus." and seldom is it questioned.
Then we wonder why the medical community looks down on and frowns upon their patients participating in these groups.
If we do not, as lymphedema patients, do not care enough abou at ourselves to seek out, lay hold of and use verified, clinically tested information how do we expect the medical community to care about us, to respect us, or to take us seriously?
I want to share a post I made in our Advocates for Lymphedema group, this past week in reference to this:
Comment from Anne, one of our members:
Maybe we need to go back to the very basic education and left people know that LE is treated in the same way whether it's primary or secondary. For instance, lower leg LE requires MLD and bandaging or compression garb whetherit'sprimary or secondary. Anne
My response:
And that the result is going to be the same if untreated...same end....same complications. There are also so many challenges too. Not only is education for the medical community critical, but the larger community as well...including those who make the laws.
But, I am beginning to think patient education may well be the greatest challenge of them all. There are so many many sites on the internet with credible, valuable information.
Organizations like the NLN, LRF, Lymph Canada, Lymph Assoc of Australia on and on that work as hard as they can to bring this information to the patients.Yet, I have noticed that the amount of misinformation has absolutely exploded...and what has happened????
Patients are drawn to that instead of to the solid information. At some point we as patients have to have enough self value and discernment to demand proof and evidence for what we read....and stop devouring info that in the long run is going to be damaging for us.
I know that sometimes it seems I go overboard about this. Maybe I do...but lymphedema is something that I have had to experience every single, solitaryday of my life. And when the curtains falls, it probably won't be from lymphoma...it will be from what is happening with the hereditary lymphedema.
So for me it is serious serious business. A quickie example and then I'll get off my soap box :-)
The very best document available any where on travel precuations with lymphedema is from the National Lymphedema Network....and to their credit, they have updated it a well based on new information.
Yet...circulating now among the online support groups is a document ontravel precautions specifically for chronic venous insufficiency. The sad thing is that patients are gobbling this up and thinking it will help them with lymphedema. What's up with things like that????????
Those precautions for CVI will spell serious trouble for lymphedema patients...so why are we reading and believing that document while ignoring the NLN's document....I honestly truely don't understand this.
So how do we crack this nut??
How do we get patients to care enough about themselves to want peer reviewed, credible information?
OK....I'm done....time for another round of morning coffee.
Pat
There are other glaring examples from just the last couple weeks.
In one group a woman posts that she uses the "ancient Mayan Abdominal massage." Why? because she claims it helps circulation and helps "organ alignment."
In yet another group, someone says we need to have a vegetarian diet because there are toxins and bacteria that immediately go to the lymphatics of the neck when we eat certain foods and meats...even before we swallow the food.
Then there was a post on the 7 stages of lymphedema....ironically this list is an old staging system for lymphatic filiarsis and has nothing to do with either primary lymphedema or secondary lymphedem as we experience it in the industrialized nations.
And in the same group that brough us the air travel misinformation and the staging info another one that states in stage zero, the lymphatics are dilated. Wrong, it depends on the lymphedema involved as to whether or not the lymphatics are dilated or constricted.
I could go on and on, but I am sure you get the jest. There simply is no substitution for evidence based medicine and scientific information.
This kind of proaganda is put out through the internet by patients who act as self-appointed "gurus." and seldom is it questioned.
Then we wonder why the medical community looks down on and frowns upon their patients participating in these groups.
If we do not, as lymphedema patients, do not care enough abou at ourselves to seek out, lay hold of and use verified, clinically tested information how do we expect the medical community to care about us, to respect us, or to take us seriously?
Sunday, July 09, 2006
CLINICAL VIGNETTE - Lymphedema
UCLA Department of Medicine
Bruce Landres, M.D.
Introduction
Lymphedema represents the failure of the lymphatic system to adequately drain lymphatic fluid from the interstitial tissue. Although lymphedema can occur in any part of the body, the term is generally used to represent an accumulation of fluid and subsequent distortion of a limb. In the earlier medical literature, this generally referred to a congenital defect in the lymphatic system. In a more contemporary review, lymphedema is significantly more common as a consequence of surgery or radiation leading to the destruction of lymphatic channels. There is also a third cause: the parasitic infestation, filariasis. Since it is rarely seen in the U.S., this etiology will not be discussed. This case report presents a congenital albeit late onset (praecox) form of lymphedema.
Case Report
A 28-year-old male presented for the first time for follow-up after a bout of cellulitis of the right lower extremity. The patient had had an uneventful childhood and was well until the age of 17 years when he presented with cellulitis of the right calf. At that time there was no prior swelling of the limb and no apparent wound or site of entry for the infection. However, the severity and persistence of the infection prompted hospitalization and treatment with intravenous antibiotics. In the aftermath of that infection, the patient had chronic swelling of the affected limb and recurrent minor bouts of cellulitis in that leg. Because of this, he underwent a lymphangiogram in at age 20, which demonstrated right lower extremity soft tissue irregular swelling, with absent lymphatic contrast consistent with lymphedema. Over the subsequent eight years the patient had a total of four additional infections, requiring antibiotics but no further hospitalizations.
The patient's family history was unremarkable for similar disease.
The patient's physical examination was unremarkable, except for a brawny pitting edema, judged to be 2-3/4+ in severity involving the right lower extremity from the knee down, with hyperpigmentation and a right mid-calf diameter of 30 cm compared to left mid-calf diameter of 26 cm. There was no inguinal or other adenopathy.
A lymphoscintigraphic study of the right lower extremities showed a reduced pattern of flow consistent with obstructive changes in that leg. Reduced numbers of nodes and nodal uptake was felt to represent congenital absence of lymphatic tissue.
Discussion
The causes of lymphedema, and the subsequent development of a swollen limb, are commonly classified as either congenital absence of lymphatics, hypo-or hyperplasia of lymphatics, or obstruction/destruc-tion of lymphatics. The incidence of congenital lymphedema in the population younger than 20 years of age is 1.15 per 100,000 with female predominance in 87% of the cases.1 In contrast the overall incidence of secondary lymphedema in the general population is reported to be more than four hundred times more common than congenital cases, and is seen in 5 persons per 1,000. Lymphedema has been reported to occur in 2%-60% of breast cancer patients in long-term follow-up. Lymphedema reportedly occurs in as high as 56% of patients following groin or pelvic surgery depending upon the treatment used.2,3
Congenital absence of lymphatics, or Milroy's Disease, leads to the onset of progressive swelling of a limb at birth with a familial occurrences of the condition, but no other anomalies. The etiology has been traced to the long arm of chromosome 5q. Interestingly the gene for vascular endothelial growth factor has been mapped to this same region of chromosome 5q, and there are data to suggest that a missense mutation in this gene may also be linked to lymphedema.4
Another type of primary lymphedema is lymphedema praecox or Meige's lymphedema. Generally this occurs prior to the age of 35 years most commonly at or around the time of puberty.5 This is reported to account for 94% of primary lymphedema.6 Some forms of this lymphedema are associated with a linkage to chromosome 16q.4
A third form of primary lymphedema, tarda, occurs after age 35 and represents approximately one in sixteen cases of primary lymphedema.
Surgeries for cancer of the breast, prostate, bladder, uterus or skin can remove or disrupt lymphatics through lymph node dissection, and represent a much more common cause of lymphedema. Radiation therapy causes fibrosis of involved areas, compromising normal lymphatic drainage and collateral pathways. Additionally, radiation to nodes can compromise an individual's later response to infection. Tumor growth, either primary or metastatic, can serve as a physical obstruction to lymph drainage causing secondary edema.
Also, infection, such as lymphangitis or cellulitis increases local blood flow and capillary permeability, thereby increasing the fluid load in a compromised lymphatic system. This leads to fibrosis, increasing inability of the lymphatic vessels to drain the limb and more edema.5 This process creates a protein rich environment in the affected edematous limb which promotes infection and worsening of the edema.
Notwithstanding the varieties of lymphedema, the consequences can be profound. The progression of the edematous limb to disfigurement can have severe psychological impact on the patient. There can also be an associated numbness and tingling, discomfort and even pain. The patient may have a sensation of tightness of the skin and heaviness of the limb. Loss of mobility of the limb, hyperkeratinosis, compromised wound healing, and recurrent skin infection are also possible.5
Rockson reports an incidence of acute cellulitis in 6% of patients followed over a 42-month period.6 In these cases the most common organism is non-group A beta-hemolytic streptococci. Beta-lactam agents, in particular penicillin G, have been the mainstay of treatment in these cases. However, this treatment is largely empirically based due to a lack of controlled trials.7
The diagnosis of lymphedema is made clinically and confirmed radiographically. Lymphedema should be considered in newborns and young adults with an unexplained edematous limb, the presentation of a brawny edema, or recurrent cellulitis in a chronically edematous limb. It may be necessary to use venous doppler studies and venography in the clinical setting to rule out venous obstructive disease as the cause of edema. Myxedema with pretibial edema can also be ruled out by a simple blood test.
The diagnosis of lymphedema is easily confirmed using isotopic lymphography studies, such as lymphoscintigraphy or lymphangioscintigraphy which have supplanted oil contrast lymphography and are considered to be the gold standard.6 These studies provide superior imaging of the lymphatics, as well as reduce the risk of anaphylactic reaction which can be seen when contrast dyes are used.
Magnetic resonance imaging and computerized tomography may also help by demonstrating the absence of edema within the muscular compartment and the honeycomb distribution of edema within the epifascial plane, along with thickening of the skin.6 There is also an anatomic delineation of lymphatic and nodal architecture.
When there is a question of malignancy, lymph node biopsy is a consideration. However, this may exacerbate a lymphedematous state by further compromising lymphatic drainage. In such a presentation, fine needle aspiration of a suspicious lymph node may provide a reasonable alternative.8
Once the diagnosis has been established, there are a variety of approaches to the treatment of lymphedema which have been shown to be effective. Generally, however, the purpose of therapy is palliation, not cure.
Bernas described the need for a combination of physical therapies involving skin care, exercises, multi-layered bandage wrapping and manual lymphedema treatment, with the later addition of compression by elastic stockings.8 He stressed that manual lymphedema treatment is directed towards massage of areas proximal to the lymphedematous area, and then directing the treatment more distally. Exercises have been shown to reduce the volume of a limb swollen with lymphedema.
Other potential therapies include intermittent pneumatic compression after manual lymphedema treatment, using a sequential pump and tight fitting elastic stockings, elevation of the affected limb which seems to be effective in the early stages of the disease, and the aggressive use of antibiotics both prophylactically and to treat infections. Diuretics are felt to be useful in the short-term, but long-term administration is felt to be of marginal value and even deleterious.5,8
Several therapeutic modes are felt to be either experimental or of questionable value. Undirected deep massage may actually be harmful to the remaining lymphatics if done too forcibly. The application of heat to the affected limb in addition to compression is felt to be experimental but possibly helpful. The medication class, benzopyrones (coumarin) are thought to activate extra-lymphatic absorption of tissue proteins and stimulate the remaining lymphatic collectors but the medications effects have yet to be proven. Local injection of hyaluronidase to help loosen the extra-cellular matrix is of doubtful value.8
It is important to note that no special diet is helpful for lymphedema. However, obesity has long been reported to be both a precipitant and an exacerbating agent for lymphedema, so weight reduction to ideal body weight is a mainstay of treatment.4,6
It is important to remember that lymphedema is a lifelong disease with potential disfiguring consequences. Psychosocial support and quality of life assessment should be an integral part of any treatment program.5,8
The use of surgery for correction appears to be controversial. The simplest operation is the debulking of the excess skin and lymphedematous tissue to improve both the disfigurement and weight of the limb. However, this may interfere with later CPT. Microsurgery techniques hold promise but require an experienced surgeon. Also lymph-venous and lymph nodal venous shunt show promise especially when done early and prior to irreversible fibrosclerosis.8
Campisi argued that microsurgical creation of lymphatic venous anastomoses is more effective than CPT.9 In 665 cases, 87% noted subjective improvement, and 83% showed a 67% reduction in volume with observable results after the first day of surgery. 85% of the patients were able to discontinue more conservative methods of treatment (e.g. CPT). There was also an 87% reduction in the incidence of cellulitis following surgery over an average follow up of seven years. Lymphoscintigraphy visualized flow through the lymphatic venous grafts even 15 years after the operation. However, Campisi did not discuss any complications noted from the surgery. He also did not mention the fact that his conclusions were based on outcomes of surgeries performed by surgeons with extensive experience in this type of surgery. Additionally, he did not report the basis for selection for surgery and whether his numbers represented all patients or just a small subset of individuals with lymphedema.9
In fact Araujo reported that only 30% of individuals with lymphedema may be suitable for lymphatic venous anastomoses and only half of these operations are successful at 5 years.10 Hafez reported that surgery is indicated in only 10% of patients with primary or secondary lymphedema. A large variation exists in the efficacy of surgery to ameliorate lymphedema. The success rate ranged from 30%-74% with a failure rate as high as 25%.2
Conclusion
Although primary lymphedema is uncommon, secondary lymphedema has become more prevalent as we become more effective in treating various cancers. Studies have shown that physicians seldom discuss the potential risk of lymphedema with patients prior to a surgical procedure. In one study, seven of ten physicians did not feel adequate to provide treatment for lymphedema.3 Since lymphedema is a condition with significant psychological and physical morbidity, physicians should be aware of it as a potential unfortunate consequence of surgical and radiation therapies and move aggressively to minimize its consequences to the patient. Medical management of this condition seems to be the treatment of choice with a smaller subset of individuals benefiting from surgical intervention.
REFERENCES
Harel L, Amir J, Nussinovitch M, Straussberg R, Varsano I. Lymphedema praecox seen as isolated unilateral arm involvement: case report and review of the literature. J Pediatr. 1997 Mar;130(3):492-494.
Hafez HM, Wolfe JH. Lymphedema. Ann Vasc Surg. 1996 Jan;10(1):88-95.
Petrek JA, Senie RT, Peters M, Rosen PP. Lymphedema in a cohort of breast carcinoma survivors 20 years after diagnosis. Cancer. 2001 Sep 15;92(6):1368-1377.
Brice G. The genetics of primary lymphoedema. British Lymphology Society Annual Conference Proceedings 2000. Accessed 9/1/2002. Kelly D. A Primer on Lymphedema. 2002. Pearson Education; New Jersey. p. 165.
Rockson SG. Lymphedema. Am J Med. 2001 Mar;110(4):288-295.
Woo PC, Lum PN, Wong SS, Cheng VC, Yuen KY. Cellulitis complicating lymphoedema. Eur J Clin Microbiol Infect Dis. 2000 Apr;19(4):294-297.
Bernas MJ, Witte CL, Witte MH. The diagnosis and treatment of peripheral lymphedema: draft revision of the 1995 Consensus Document of the International Society of Lymphology Executive Committee for discussion at the September 3-7, 2001, XVIII International Congress of Lymphology in Genoa, Italy. Lymphology. 2001 Jun;34(2):84-91.
Campisi C, Boccardo F. Lymphedema and microsurgery. Microsurgery. 2002;22(2):74-80.
Araujo JA, Curbelo JG, Mayol AL, Pascal GG, Vignale RA, Fleurquin F. Effective management of marked lymphedema of the leg. Int J Dermatol. 1997 May;36(5):389-392.
Article
Bruce Landres, M.D.
Introduction
Lymphedema represents the failure of the lymphatic system to adequately drain lymphatic fluid from the interstitial tissue. Although lymphedema can occur in any part of the body, the term is generally used to represent an accumulation of fluid and subsequent distortion of a limb. In the earlier medical literature, this generally referred to a congenital defect in the lymphatic system. In a more contemporary review, lymphedema is significantly more common as a consequence of surgery or radiation leading to the destruction of lymphatic channels. There is also a third cause: the parasitic infestation, filariasis. Since it is rarely seen in the U.S., this etiology will not be discussed. This case report presents a congenital albeit late onset (praecox) form of lymphedema.
Case Report
A 28-year-old male presented for the first time for follow-up after a bout of cellulitis of the right lower extremity. The patient had had an uneventful childhood and was well until the age of 17 years when he presented with cellulitis of the right calf. At that time there was no prior swelling of the limb and no apparent wound or site of entry for the infection. However, the severity and persistence of the infection prompted hospitalization and treatment with intravenous antibiotics. In the aftermath of that infection, the patient had chronic swelling of the affected limb and recurrent minor bouts of cellulitis in that leg. Because of this, he underwent a lymphangiogram in at age 20, which demonstrated right lower extremity soft tissue irregular swelling, with absent lymphatic contrast consistent with lymphedema. Over the subsequent eight years the patient had a total of four additional infections, requiring antibiotics but no further hospitalizations.
The patient's family history was unremarkable for similar disease.
The patient's physical examination was unremarkable, except for a brawny pitting edema, judged to be 2-3/4+ in severity involving the right lower extremity from the knee down, with hyperpigmentation and a right mid-calf diameter of 30 cm compared to left mid-calf diameter of 26 cm. There was no inguinal or other adenopathy.
A lymphoscintigraphic study of the right lower extremities showed a reduced pattern of flow consistent with obstructive changes in that leg. Reduced numbers of nodes and nodal uptake was felt to represent congenital absence of lymphatic tissue.
Discussion
The causes of lymphedema, and the subsequent development of a swollen limb, are commonly classified as either congenital absence of lymphatics, hypo-or hyperplasia of lymphatics, or obstruction/destruc-tion of lymphatics. The incidence of congenital lymphedema in the population younger than 20 years of age is 1.15 per 100,000 with female predominance in 87% of the cases.1 In contrast the overall incidence of secondary lymphedema in the general population is reported to be more than four hundred times more common than congenital cases, and is seen in 5 persons per 1,000. Lymphedema has been reported to occur in 2%-60% of breast cancer patients in long-term follow-up. Lymphedema reportedly occurs in as high as 56% of patients following groin or pelvic surgery depending upon the treatment used.2,3
Congenital absence of lymphatics, or Milroy's Disease, leads to the onset of progressive swelling of a limb at birth with a familial occurrences of the condition, but no other anomalies. The etiology has been traced to the long arm of chromosome 5q. Interestingly the gene for vascular endothelial growth factor has been mapped to this same region of chromosome 5q, and there are data to suggest that a missense mutation in this gene may also be linked to lymphedema.4
Another type of primary lymphedema is lymphedema praecox or Meige's lymphedema. Generally this occurs prior to the age of 35 years most commonly at or around the time of puberty.5 This is reported to account for 94% of primary lymphedema.6 Some forms of this lymphedema are associated with a linkage to chromosome 16q.4
A third form of primary lymphedema, tarda, occurs after age 35 and represents approximately one in sixteen cases of primary lymphedema.
Surgeries for cancer of the breast, prostate, bladder, uterus or skin can remove or disrupt lymphatics through lymph node dissection, and represent a much more common cause of lymphedema. Radiation therapy causes fibrosis of involved areas, compromising normal lymphatic drainage and collateral pathways. Additionally, radiation to nodes can compromise an individual's later response to infection. Tumor growth, either primary or metastatic, can serve as a physical obstruction to lymph drainage causing secondary edema.
Also, infection, such as lymphangitis or cellulitis increases local blood flow and capillary permeability, thereby increasing the fluid load in a compromised lymphatic system. This leads to fibrosis, increasing inability of the lymphatic vessels to drain the limb and more edema.5 This process creates a protein rich environment in the affected edematous limb which promotes infection and worsening of the edema.
Notwithstanding the varieties of lymphedema, the consequences can be profound. The progression of the edematous limb to disfigurement can have severe psychological impact on the patient. There can also be an associated numbness and tingling, discomfort and even pain. The patient may have a sensation of tightness of the skin and heaviness of the limb. Loss of mobility of the limb, hyperkeratinosis, compromised wound healing, and recurrent skin infection are also possible.5
Rockson reports an incidence of acute cellulitis in 6% of patients followed over a 42-month period.6 In these cases the most common organism is non-group A beta-hemolytic streptococci. Beta-lactam agents, in particular penicillin G, have been the mainstay of treatment in these cases. However, this treatment is largely empirically based due to a lack of controlled trials.7
The diagnosis of lymphedema is made clinically and confirmed radiographically. Lymphedema should be considered in newborns and young adults with an unexplained edematous limb, the presentation of a brawny edema, or recurrent cellulitis in a chronically edematous limb. It may be necessary to use venous doppler studies and venography in the clinical setting to rule out venous obstructive disease as the cause of edema. Myxedema with pretibial edema can also be ruled out by a simple blood test.
The diagnosis of lymphedema is easily confirmed using isotopic lymphography studies, such as lymphoscintigraphy or lymphangioscintigraphy which have supplanted oil contrast lymphography and are considered to be the gold standard.6 These studies provide superior imaging of the lymphatics, as well as reduce the risk of anaphylactic reaction which can be seen when contrast dyes are used.
Magnetic resonance imaging and computerized tomography may also help by demonstrating the absence of edema within the muscular compartment and the honeycomb distribution of edema within the epifascial plane, along with thickening of the skin.6 There is also an anatomic delineation of lymphatic and nodal architecture.
When there is a question of malignancy, lymph node biopsy is a consideration. However, this may exacerbate a lymphedematous state by further compromising lymphatic drainage. In such a presentation, fine needle aspiration of a suspicious lymph node may provide a reasonable alternative.8
Once the diagnosis has been established, there are a variety of approaches to the treatment of lymphedema which have been shown to be effective. Generally, however, the purpose of therapy is palliation, not cure.
Bernas described the need for a combination of physical therapies involving skin care, exercises, multi-layered bandage wrapping and manual lymphedema treatment, with the later addition of compression by elastic stockings.8 He stressed that manual lymphedema treatment is directed towards massage of areas proximal to the lymphedematous area, and then directing the treatment more distally. Exercises have been shown to reduce the volume of a limb swollen with lymphedema.
Other potential therapies include intermittent pneumatic compression after manual lymphedema treatment, using a sequential pump and tight fitting elastic stockings, elevation of the affected limb which seems to be effective in the early stages of the disease, and the aggressive use of antibiotics both prophylactically and to treat infections. Diuretics are felt to be useful in the short-term, but long-term administration is felt to be of marginal value and even deleterious.5,8
Several therapeutic modes are felt to be either experimental or of questionable value. Undirected deep massage may actually be harmful to the remaining lymphatics if done too forcibly. The application of heat to the affected limb in addition to compression is felt to be experimental but possibly helpful. The medication class, benzopyrones (coumarin) are thought to activate extra-lymphatic absorption of tissue proteins and stimulate the remaining lymphatic collectors but the medications effects have yet to be proven. Local injection of hyaluronidase to help loosen the extra-cellular matrix is of doubtful value.8
It is important to note that no special diet is helpful for lymphedema. However, obesity has long been reported to be both a precipitant and an exacerbating agent for lymphedema, so weight reduction to ideal body weight is a mainstay of treatment.4,6
It is important to remember that lymphedema is a lifelong disease with potential disfiguring consequences. Psychosocial support and quality of life assessment should be an integral part of any treatment program.5,8
The use of surgery for correction appears to be controversial. The simplest operation is the debulking of the excess skin and lymphedematous tissue to improve both the disfigurement and weight of the limb. However, this may interfere with later CPT. Microsurgery techniques hold promise but require an experienced surgeon. Also lymph-venous and lymph nodal venous shunt show promise especially when done early and prior to irreversible fibrosclerosis.8
Campisi argued that microsurgical creation of lymphatic venous anastomoses is more effective than CPT.9 In 665 cases, 87% noted subjective improvement, and 83% showed a 67% reduction in volume with observable results after the first day of surgery. 85% of the patients were able to discontinue more conservative methods of treatment (e.g. CPT). There was also an 87% reduction in the incidence of cellulitis following surgery over an average follow up of seven years. Lymphoscintigraphy visualized flow through the lymphatic venous grafts even 15 years after the operation. However, Campisi did not discuss any complications noted from the surgery. He also did not mention the fact that his conclusions were based on outcomes of surgeries performed by surgeons with extensive experience in this type of surgery. Additionally, he did not report the basis for selection for surgery and whether his numbers represented all patients or just a small subset of individuals with lymphedema.9
In fact Araujo reported that only 30% of individuals with lymphedema may be suitable for lymphatic venous anastomoses and only half of these operations are successful at 5 years.10 Hafez reported that surgery is indicated in only 10% of patients with primary or secondary lymphedema. A large variation exists in the efficacy of surgery to ameliorate lymphedema. The success rate ranged from 30%-74% with a failure rate as high as 25%.2
Conclusion
Although primary lymphedema is uncommon, secondary lymphedema has become more prevalent as we become more effective in treating various cancers. Studies have shown that physicians seldom discuss the potential risk of lymphedema with patients prior to a surgical procedure. In one study, seven of ten physicians did not feel adequate to provide treatment for lymphedema.3 Since lymphedema is a condition with significant psychological and physical morbidity, physicians should be aware of it as a potential unfortunate consequence of surgical and radiation therapies and move aggressively to minimize its consequences to the patient. Medical management of this condition seems to be the treatment of choice with a smaller subset of individuals benefiting from surgical intervention.
REFERENCES
Harel L, Amir J, Nussinovitch M, Straussberg R, Varsano I. Lymphedema praecox seen as isolated unilateral arm involvement: case report and review of the literature. J Pediatr. 1997 Mar;130(3):492-494.
Hafez HM, Wolfe JH. Lymphedema. Ann Vasc Surg. 1996 Jan;10(1):88-95.
Petrek JA, Senie RT, Peters M, Rosen PP. Lymphedema in a cohort of breast carcinoma survivors 20 years after diagnosis. Cancer. 2001 Sep 15;92(6):1368-1377.
Brice G. The genetics of primary lymphoedema. British Lymphology Society Annual Conference Proceedings 2000. Accessed 9/1/2002. Kelly D. A Primer on Lymphedema. 2002. Pearson Education; New Jersey. p. 165.
Rockson SG. Lymphedema. Am J Med. 2001 Mar;110(4):288-295.
Woo PC, Lum PN, Wong SS, Cheng VC, Yuen KY. Cellulitis complicating lymphoedema. Eur J Clin Microbiol Infect Dis. 2000 Apr;19(4):294-297.
Bernas MJ, Witte CL, Witte MH. The diagnosis and treatment of peripheral lymphedema: draft revision of the 1995 Consensus Document of the International Society of Lymphology Executive Committee for discussion at the September 3-7, 2001, XVIII International Congress of Lymphology in Genoa, Italy. Lymphology. 2001 Jun;34(2):84-91.
Campisi C, Boccardo F. Lymphedema and microsurgery. Microsurgery. 2002;22(2):74-80.
Araujo JA, Curbelo JG, Mayol AL, Pascal GG, Vignale RA, Fleurquin F. Effective management of marked lymphedema of the leg. Int J Dermatol. 1997 May;36(5):389-392.
Article
Teens with Lymphedema Conference - Agenda and Registration
REGISTRATION INFORMATION:
INTRODUCTION
The Adelprise Lymphedema Association (Association of patients affected by Primary and Secondary Lymphedemas) believes that due to the lack of existing information it is time to celebrate the “I Encounter of Youngsters and Teenagers affected by Lymphedema.”
The knowledge and insights gained from these workshops are important because from the moment an individual is diagnosed with lymphedema they need to understand this condition, know how it affects their body, and learn how to successfully manage the treatment of this condition. We also believe it is important to understand the steps to protect against making the condition worse and to know how to use the self-help resources that can improve the quality of life. Having lymphedema creates emotional and social difficulties especially for young patients and their families. Separate sessions for youth and their parents will provide insights that increase the potential of successful treatment and an improved quality of life.
OUR OBJECTIVES ARE TO:
Provide participants with useful information about current therapies and opportunities to practice these skills.
Encourage particpants to share fun with supportive new friends while gaining insights that can improve their quality of life.
Organizing Committee:
Raúl Calleja de Busts
Merche Sánchez González
Maite Alonso Duarte
Pablo Montero Garzo
Teresa Merino Clemente
Zuriñe Blasco Etxeguren
Scientific Committee:
Dra. Adora Fernández Domene
Ana Belen Pardo Porto
Luís Miguel Amurrio
Amaia San Miguel López
Mª Jesus Gómez Lago
SUGGESTED LODGING
International Dialing Code and Telephone #
Hotel Barcelo * * * 34 945130000
Hotel Chancellor Ayala * * * * 34 945228100
Hotel Wellinton * * 34 945175707
Hotel Atxuri * * 34 945255800
Hotel Fact * * 34 945147230
Note: Lodging and meals are at the participant’s expense
WHO SHOULD ATTEND
* Youngsters from all countries between the ages of 12 to 18 years who are affected by Lymphedema.
* Presentations will be in Spanish and English.
REGISTRATION
There is no charge for registration; however it is necessary to complete and return the registration form before October 1, 2006. Attendance is limited and early registration is recommended.
Mail this to: THE ASSOCIATION ADELPRISE C/VICENTE ABREU N UNDER 01008 VITORIA-GASTEIZ (SPAIN)
Youth must include a completed “Parent’s Permission and Authorization Form,” signed by either parent or their tutor, authorizing the attendance and participation in the Youth Encounter. Participating youths must be accompanied by an adult, parent, or properly credited tutor.
PRACTICAL INFORMATION
* Participants should wear comfortable clothes to the workshops.
* Those attending the self-bandaging workshop must bring their own bandages.
PROGRAM
FRIDAY, OCTOBER 27, 2006 4:00 - 4:30 pm
Greeting and Documentation Delivery
4:30 - 7:30 SELF HELP WORKSHOP
D. Luís Miguel Amurrio (Psychologist of AACC of Álava)
THEORETICAL – PRACTICAL CLASS
PowerPoint presentations on important topics Group discussions of these concerns Sharing experiences and insights Addressing problems and sharing solutions Stress reduction techniques to improve the quality of life.
19.30 h. - PAST, CURRENT SITUATION AND PERSPECTIVE OF FUTURE OF THE LYMPHEDEMA
Mrs. Zuriñe Blasco Etxeguren (President of Adelprise) Addressing problems and sharing solutions Stress reduction techniques to improve the quality of life.
SATURDAY—OCTOBER, 28
9:30 to 1:30 (with a half hour break).
WORKSHOPS:
Self-Manual Lymph Drainage, Prevention
Techniques, and Self-Care Of Lymphedema
Mª Jesus Gómez Lake - Physiotherapist and Mª Ángel Santamaría Aspiazu-Nurse Graduate
PRACTICAL THEORY:
The lymphatic system
Location of the lymph glands
Functions of the lymphatic system
Rules for self-manual lymph drainage
Movements to stimulate lymph glands
Learning about the directions for drainage of the lymphatic system.
Guidelines on the importance of preventing the complications of lymphedema. Recommendations to keep in mind regarding self- care, infections, hygiene, and rules for daily life.
2.00 noon Lunch Break
4:00 FUNCTIONAL RECOVERY OF LYMPHEDEMA
Pedro Gómez de Segura (Graduated in Physical Education)
PRACTICAL CONTENT
Breathing Exercises
Exercises for the neck, shoulders, elbows, hands, and fingers Stimulation of the trunk and abdomen areas
Mobilization of the hips, legs, knees, ankles, feet, and fingers
6:00 pm ROUND TABLE DISCUSSION
"Medical Aspects of the Lymphedema, and Support Treatment Therapies"
8:30 JOINT DINNER FOR PARTICIPANTS (Optional)
SUNDAY, OCTOBER 29, 2006
9.30 h. SELF-BANDAGING WORKSHOP
Dra. Fernández adores Domene (Teacher of scientific and practical Manual Lymphatic Drainage)
THEORETICAL – PRACTICAL CLASS
Lymphatic anatomy of the affected areas Demonstration of materials used for bandaging Caution and care to take with the bandages Active and participative class Self-bandaging demonstration Learning of the technique individually Help exercises
1:30 pm - Participants joint Conclusions
2:00 - End and farewell of the Youth Encounter
*********************************
REGISTRATION FORM I
Joint Meeting of Youngsters With Lymphedema Registration
Form Registration is FREE but must be completed by October 1, 2006
Full Name _________________________________________
Age _________________
Street Address ___________________________________________________________
City ________________________________________
State __________________________________
Zip/Postal Code______________________
Country _________________________
Telephone ___________________________
E-Mail _____________________________
Optional Joint Dinner: 20 Euros (Please check yes if you will be attending) [ ] YES, I plan to attend this dinner. [ ] NO, I do not plan to attend the dinner.
SIGNATURE____________________________________
*For the optional Joint Dinner, please make payment to the following
CAJA LABORAL
Authorization from Parent or Tutor
I do hereby give permission for this youth to attend I JOINT MEETING OF YOUNGSTERS WITH LYMPHEDEMA Name of Attendee
_________________________________________
Age ___________
Street Address ____________________________________________
City ________________________________________
State __________________________________
Zip/Postal Code__________________________
Country _________________________
Telephone ___________________________
E-Mail _____________________________
Signature of Parent or Tutor _____________________________________
Please circle title to indicate of Parent or Tutor:
Please return the Registration Form and a copy of Bank Receipt to confirm Attendance to the Joint Dinner to:
ASSOCIATION ADELPRISE
C/VICENTE ABREU Nº UNDER 01008
VITORIA-GASTEIZ (SPAIN)
Registration date limit: October, 1, 2006
INTRODUCTION
The Adelprise Lymphedema Association (Association of patients affected by Primary and Secondary Lymphedemas) believes that due to the lack of existing information it is time to celebrate the “I Encounter of Youngsters and Teenagers affected by Lymphedema.”
The knowledge and insights gained from these workshops are important because from the moment an individual is diagnosed with lymphedema they need to understand this condition, know how it affects their body, and learn how to successfully manage the treatment of this condition. We also believe it is important to understand the steps to protect against making the condition worse and to know how to use the self-help resources that can improve the quality of life. Having lymphedema creates emotional and social difficulties especially for young patients and their families. Separate sessions for youth and their parents will provide insights that increase the potential of successful treatment and an improved quality of life.
OUR OBJECTIVES ARE TO:
Provide participants with useful information about current therapies and opportunities to practice these skills.
Encourage particpants to share fun with supportive new friends while gaining insights that can improve their quality of life.
Organizing Committee:
Raúl Calleja de Busts
Merche Sánchez González
Maite Alonso Duarte
Pablo Montero Garzo
Teresa Merino Clemente
Zuriñe Blasco Etxeguren
Scientific Committee:
Dra. Adora Fernández Domene
Ana Belen Pardo Porto
Luís Miguel Amurrio
Amaia San Miguel López
Mª Jesus Gómez Lago
SUGGESTED LODGING
International Dialing Code and Telephone #
Hotel Barcelo * * * 34 945130000
Hotel Chancellor Ayala * * * * 34 945228100
Hotel Wellinton * * 34 945175707
Hotel Atxuri * * 34 945255800
Hotel Fact * * 34 945147230
Note: Lodging and meals are at the participant’s expense
WHO SHOULD ATTEND
* Youngsters from all countries between the ages of 12 to 18 years who are affected by Lymphedema.
* Presentations will be in Spanish and English.
REGISTRATION
There is no charge for registration; however it is necessary to complete and return the registration form before October 1, 2006. Attendance is limited and early registration is recommended.
Mail this to: THE ASSOCIATION ADELPRISE C/VICENTE ABREU N UNDER 01008 VITORIA-GASTEIZ (SPAIN)
Youth must include a completed “Parent’s Permission and Authorization Form,” signed by either parent or their tutor, authorizing the attendance and participation in the Youth Encounter. Participating youths must be accompanied by an adult, parent, or properly credited tutor.
PRACTICAL INFORMATION
* Participants should wear comfortable clothes to the workshops.
* Those attending the self-bandaging workshop must bring their own bandages.
PROGRAM
FRIDAY, OCTOBER 27, 2006 4:00 - 4:30 pm
Greeting and Documentation Delivery
4:30 - 7:30 SELF HELP WORKSHOP
D. Luís Miguel Amurrio (Psychologist of AACC of Álava)
THEORETICAL – PRACTICAL CLASS
PowerPoint presentations on important topics Group discussions of these concerns Sharing experiences and insights Addressing problems and sharing solutions Stress reduction techniques to improve the quality of life.
19.30 h. - PAST, CURRENT SITUATION AND PERSPECTIVE OF FUTURE OF THE LYMPHEDEMA
Mrs. Zuriñe Blasco Etxeguren (President of Adelprise) Addressing problems and sharing solutions Stress reduction techniques to improve the quality of life.
SATURDAY—OCTOBER, 28
9:30 to 1:30 (with a half hour break).
WORKSHOPS:
Self-Manual Lymph Drainage, Prevention
Techniques, and Self-Care Of Lymphedema
Mª Jesus Gómez Lake - Physiotherapist and Mª Ángel Santamaría Aspiazu-Nurse Graduate
PRACTICAL THEORY:
The lymphatic system
Location of the lymph glands
Functions of the lymphatic system
Rules for self-manual lymph drainage
Movements to stimulate lymph glands
Learning about the directions for drainage of the lymphatic system.
Guidelines on the importance of preventing the complications of lymphedema. Recommendations to keep in mind regarding self- care, infections, hygiene, and rules for daily life.
2.00 noon Lunch Break
4:00 FUNCTIONAL RECOVERY OF LYMPHEDEMA
Pedro Gómez de Segura (Graduated in Physical Education)
PRACTICAL CONTENT
Breathing Exercises
Exercises for the neck, shoulders, elbows, hands, and fingers Stimulation of the trunk and abdomen areas
Mobilization of the hips, legs, knees, ankles, feet, and fingers
6:00 pm ROUND TABLE DISCUSSION
"Medical Aspects of the Lymphedema, and Support Treatment Therapies"
8:30 JOINT DINNER FOR PARTICIPANTS (Optional)
SUNDAY, OCTOBER 29, 2006
9.30 h. SELF-BANDAGING WORKSHOP
Dra. Fernández adores Domene (Teacher of scientific and practical Manual Lymphatic Drainage)
THEORETICAL – PRACTICAL CLASS
Lymphatic anatomy of the affected areas Demonstration of materials used for bandaging Caution and care to take with the bandages Active and participative class Self-bandaging demonstration Learning of the technique individually Help exercises
1:30 pm - Participants joint Conclusions
2:00 - End and farewell of the Youth Encounter
*********************************
REGISTRATION FORM I
Joint Meeting of Youngsters With Lymphedema Registration
Form Registration is FREE but must be completed by October 1, 2006
Full Name _________________________________________
Age _________________
Street Address ___________________________________________________________
City ________________________________________
State __________________________________
Zip/Postal Code______________________
Country _________________________
Telephone ___________________________
E-Mail _____________________________
Optional Joint Dinner: 20 Euros (Please check yes if you will be attending) [ ] YES, I plan to attend this dinner. [ ] NO, I do not plan to attend the dinner.
SIGNATURE____________________________________
*For the optional Joint Dinner, please make payment to the following
CAJA LABORAL
Authorization from Parent or Tutor
I do hereby give permission for this youth to attend I JOINT MEETING OF YOUNGSTERS WITH LYMPHEDEMA Name of Attendee
_________________________________________
Age ___________
Street Address ____________________________________________
City ________________________________________
State __________________________________
Zip/Postal Code__________________________
Country _________________________
Telephone ___________________________
E-Mail _____________________________
Signature of Parent or Tutor _____________________________________
Please circle title to indicate of Parent or Tutor:
Please return the Registration Form and a copy of Bank Receipt to confirm Attendance to the Joint Dinner to:
ASSOCIATION ADELPRISE
C/VICENTE ABREU Nº UNDER 01008
VITORIA-GASTEIZ (SPAIN)
Registration date limit: October, 1, 2006
Wednesday, July 05, 2006
First European Congress for Teens with Lymphedema
First European Congress for Teens with Lymphedema
On 27, 28 and 29 of October 2006
The first European Congress for youngsters with lymphedemawill be held in Vitoria, Spain.
The congress is meant for youngsters in the age range of 13-18.Purpose is to share information and experiences.
No admission fees.
Last date for registration: October 1, 2006.Contact for further information:
AdelpriseFederation Vicente AbreuC/Painting Vicente Abreu 701008 Vitoria SPAIN
Tel. 945 226 061 or 630 618 103
adelprise@yahoo.es
*********************************
Primer Congreso-Encuentro Europeo de Jóvenes con Linfedema
“ TERAPIAS DE APOYO EN EL TRATAMIENTO Y ADAPTACIÓN SOCIAL”
La Asociación de Linfedema ADELPRISE pone en conocimiento la próxima celebración del Primer Congreso-Encuentro Europeo de Jóvenes con Linfedema, enfocado principalmente para que éstos chicos puedan compartir experiencias, y se apoyen mutuamente en su condición.
Este Congreso-Encuentro estará dirigido por distintos profesionales especializados, y es para jóvenes de entre 13 a 18 años.
La inscripción y participación son gratuitas.
El viaje y la acomodación son por cuenta de los participantes. Se organizarán grupos de edades distintos, si la demanda lo requiere.
Simultáneamente se celebrará el X Aniversario de Adelprise, con eventos abiertos y dirigidos a todos/as los/as demás afectados y público en general, y también recomendada para los padres o cuidadores de los jóvenes.
Las personas interesadas en asistir y participar deben solicitar y devolver los formularios debidamente rellenados antes de la fecha indicada.
El interés primordial de los organizadores de éste encuentro es proporcionar información, evitar aislamientos, dar esperanza, y transmitir optimismo a los jóvenes, para que en el futuro puedan tener y disfrutar de una buena Calidad de Vida.
PROGRAMA
VITORIA - Días: 27, 28 y 29 de Octubre - 2006 / Resumen de Actividades
•Recepción a los asistentes y entrega de Documentación
•Charla sobre Linfedema, su pasado, la situación actual, y las perspectivas de futuro
•Aspectos Médicos del Linfedema
•Terapias de apoyo en el tratamiento de Linfedema
•Recuperación Funcional •Taller de Auto-Ayuda •
Taller de Auto-Drenaje
Dirección para solicitar Información y formularios del I Congreso Encuentro de Jóvenes con Linfedema:
ADELPRISE
Asociación de Enfermos de Linfedemas Primarios y Secundarios
FEDERACIÓN VICENTE ABREU C/ Pintor Vicente Abreu, 7, 01008 Vitoria- Gastéis
Tel. 945 226 061 ( Zuriñe ) 630 618 103
e-mail: adelprise@yahoo.es
Fecha límite de entrega de formularios para inscripción: DIA 1 DE OCTUBRE 2006
**************************
Lymphedema People
On 27, 28 and 29 of October 2006
The first European Congress for youngsters with lymphedemawill be held in Vitoria, Spain.
The congress is meant for youngsters in the age range of 13-18.Purpose is to share information and experiences.
No admission fees.
Last date for registration: October 1, 2006.Contact for further information:
AdelpriseFederation Vicente AbreuC/Painting Vicente Abreu 701008 Vitoria SPAIN
Tel. 945 226 061 or 630 618 103
adelprise@yahoo.es
*********************************
Primer Congreso-Encuentro Europeo de Jóvenes con Linfedema
“ TERAPIAS DE APOYO EN EL TRATAMIENTO Y ADAPTACIÓN SOCIAL”
La Asociación de Linfedema ADELPRISE pone en conocimiento la próxima celebración del Primer Congreso-Encuentro Europeo de Jóvenes con Linfedema, enfocado principalmente para que éstos chicos puedan compartir experiencias, y se apoyen mutuamente en su condición.
Este Congreso-Encuentro estará dirigido por distintos profesionales especializados, y es para jóvenes de entre 13 a 18 años.
La inscripción y participación son gratuitas.
El viaje y la acomodación son por cuenta de los participantes. Se organizarán grupos de edades distintos, si la demanda lo requiere.
Simultáneamente se celebrará el X Aniversario de Adelprise, con eventos abiertos y dirigidos a todos/as los/as demás afectados y público en general, y también recomendada para los padres o cuidadores de los jóvenes.
Las personas interesadas en asistir y participar deben solicitar y devolver los formularios debidamente rellenados antes de la fecha indicada.
El interés primordial de los organizadores de éste encuentro es proporcionar información, evitar aislamientos, dar esperanza, y transmitir optimismo a los jóvenes, para que en el futuro puedan tener y disfrutar de una buena Calidad de Vida.
PROGRAMA
VITORIA - Días: 27, 28 y 29 de Octubre - 2006 / Resumen de Actividades
•Recepción a los asistentes y entrega de Documentación
•Charla sobre Linfedema, su pasado, la situación actual, y las perspectivas de futuro
•Aspectos Médicos del Linfedema
•Terapias de apoyo en el tratamiento de Linfedema
•Recuperación Funcional •Taller de Auto-Ayuda •
Taller de Auto-Drenaje
Dirección para solicitar Información y formularios del I Congreso Encuentro de Jóvenes con Linfedema:
ADELPRISE
Asociación de Enfermos de Linfedemas Primarios y Secundarios
FEDERACIÓN VICENTE ABREU C/ Pintor Vicente Abreu, 7, 01008 Vitoria- Gastéis
Tel. 945 226 061 ( Zuriñe ) 630 618 103
e-mail: adelprise@yahoo.es
Fecha límite de entrega de formularios para inscripción: DIA 1 DE OCTUBRE 2006
**************************
Lymphedema People
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