Monday, December 24, 2007

Manipulative therapy of secondary lymphedema in the presence of locoregional tumors

Manipulative therapy of secondary lymphedema in the presence of locoregional tumors

Cancer. 2007 Dec 17

Pinell XA, Kirkpatrick SH, Hawkins K, Mondry TE, Johnstone PA.
Radiation Oncology Department, Emory University School of Medicine, Atlanta, Georgia.

BACKGROUND:
Complete decongestive therapy (CDT), including manual lymphatic drainage (MLD) is a manipulative intervention of documented benefit to patients with lymphedema (LE). Although the role of CDT for LE is well described, to the authors' knowledge there are no data regarding its efficacy for patients with LE due to tumor masses in the draining anatomic bed. Traditionally, LE therapists are wary of providing therapy to such patients with 'malignant' LE for fear of exacerbating the underlying cancer, and that the obstruction will render therapy less effective. In the current study, the authors' experience providing CDT for such patients is discussed.

METHODS:
Cancer survivors with LE were referred to therapists at 2 Atlanta-area clinics. CDT consists of treatment (Phase 1) and maintenance phases (Phase 2). During Phase 1, the patient undergoes manipulative therapy and bandaging daily until the LE reduction plateaus; at that point, Phase 2 (self-care) begins. At the beginning and end of Phase 1, LE is quantified and differences in girth volume calculated. The results for patients completing Phase 1 therapy for LE in the presence of locoregional masses were compared with results for patients with LE in the absence of such disease. Both volume reduction of the affected limb and number of treatments to plateau were analyzed.

RESULTS:
Between January 2004, and March 2007, LE of 82 limbs in 72 patients was treated with CDT and Phase 1 was completed. The median number of treatments to plateau was 12 (range, 4-23 treatments); the median limb volume reduction was 22% (range, -23 to 164%). Nineteen limbs (16 patients) with associated chest wall/axillary or pelvic/inguinal tumors had nonsignificant difference in LE reduction (P = .75) in the presence of significantly more sessions to attain plateau (P = .0016) compared with 63 limbs in 56 patients without such masses.

CONCLUSIONS:
Patients with LE may obtain relief with CDT regardless of whether they have locoregional disease contributing to their symptoms. However, it will likely take longer to achieve that effect. Manipulative therapy of LE should not be withheld because of persistent or recurrent disease in the draining anatomic bed. Cancer 2008. (c) 2007 American Cancer Society.


Wiley InterScience

Thursday, December 20, 2007

Adrenomedullin - hormone for possible treatment of lymphedema?

Hormone Could Ease Painful Lymphedema

12/20/07

THURSDAY, Dec. 20 (HealthDay News) -- A hormone called adrenomedullin may prove an effective drug target for treating lymphedema, a painful swelling of the limbs that can follow breast cancer or other cancer treatment, U.S. researchers say.

It may also help prevent the spread of cancer, according to a team from the University of North Carolina at Chapel Hill School of Medicine.

Adrenomedullin, which is secreted by cells throughout the body, is known to play a role in cardiovascular disease and other cell functions. In a new study, the UNC group found that adrenomedullin also plays an important role in the formation of the lymphatic system in mice.

They said it may be possible to develop drugs that target this hormone in order to help the more than 100 million people worldwide who suffer from lymphedema. The condition occurs when the lymphatic system fails to work properly. In rare cases, it is genetic, but millions suffer lymphedema due to parasitic infections or as the aftermath of cancer therapies.
Currently, the only treatments for lymphedema include massage and the use of low-compression stockings and other garments. But these aren't much help, the UNC researchers said.


"Our research also may lead to therapies to prevent cancer cells from traveling through these lymphatic vessels to infiltrate other parts of the body," senior study author Kathleen M. Caron, assistant professor of cell and molecular physiology and genetics, said in a prepared statement.
The study is published in the Dec. 20 issue of the Journal of Clinical Investigation.


Health News

Hormone May be New Drug Target for Preventing Lymphedema, Tumor Spread

Keywords: HORMAN - LYMPH SYSTEM - ADRENOMEDULLIN - TUMOR SPREAD

Description

A hormone secreted by cells throughout the body and known to play a role in cardiovascular disease and other cell functions is also critical for proper formation of the lymphatic system in mice, according to research from the University of North Carolina at Chapel Hill School of Medicine.

Newswise -- A hormone secreted by cells throughout the body and known to play a role in cardiovascular disease and other cell functions is also critical for proper formation of the lymphatic system in mice, according to research from the University of North Carolina at Chapel Hill School of Medicine.

By targeting this hormone, called adrenomedullin, researchers may be able to treat the more than 100 million people worldwide affected by lymphedema, a condition that causes painful swelling in arms and legs.

"Our research also may lead to therapies to prevent cancer cells from traveling through these lymphatic vessels to infiltrate other parts of the body," said Kathleen M. Caron, senior study author and assistant professor of cell and molecular physiology and genetics at UNC.

Adrenomedullin is a powerful vascular peptide that can widen existing blood vessels and even promote the growth of new ones. But it also has many more functions, such as helping control metabolism, heart rate, thirst and appetite, stress response, antibacterial activity and nerve signal transmission.


The study, published Dec. 20, 2007, in the Journal of Clinical Investigation, demonstrates that this peptide is necessary for yet another function in our bodies: without it, our lymphatic system - an important part of the body's immune system - does not form normally. The lymphatic system includes lymph nodes and a network of thin tubes that transport fluid and immune cells that have leached out of tissues back into the circulatory system. These tubes branch, like blood vessels, into all the tissues of the body. Between two and three liters of the almost colorless fluid called lymph go through the lymphatic system in a day.

If this system fails to function properly, excess fluid collects and swells in tissue, causing lymphedema. In rare instances, the condition is inherited through genetic mutations. For two to three million cancer survivors, it comes as a consequence of early treatment, as the surgical removal of lymph nodes and radiation therapy creates damage to the lymphatic system that lasts a lifetime. But the most common cause, affecting up to 120 million people worldwide, is a parasitic infection.

"Lymphedema is a very serious problem," Caron said. "Not only does it limit your mobility, but it can be quite painful and disfiguring."

The only current treatments for the condition - using low-compression stockings and other garments, and massage - are not much help, Caron says. Before now, only a dozen or so genes had been implicated in the formation of lymphatic vessels, or lymphangiogenesis, and none of them have yet yielded an effective therapy. But through this study, the researchers have described three new targets, adrenomedullin and two of its partners in the cell, which together hold true promise for a pharmaceutical treatment for lymphedema.

Caron and her team of researchers discovered the importance of this hormone in the formation of the lymphatic system after genetically manipulating mice so that they completely lacked either adrenomedullin or its related cell partners. They found that these mice looked a lot like other mice with impaired lymphangiogenesis. Careful examination showed that the lymph sacs that normally take up excess fluid from the tissues were much smaller than they should be, and the sacs without adrenomedullin were made up of fewer cells than normal.

By increasing adrenomedullin within the cells of the lymphatic system, the researchers believe that they can encourage the lymph sacs to proliferate and take up more fluid. Not only could this approach provide a new treatment for lymphedema, but it may also prove useful in preventing the spread of cancer because invasive cancers sometimes penetrate the lymphatic vessels and metastasize to distant sites.

"In cancer treatments of the future, patients suffering from these aggressive cancers could be identified early and could be treated with a drug to inhibit the growth of the lymph vessels that transport the cancerous cells, thus keeping the cancer in check," Caron said.

The research was funded in part by The Burroughs Wellcome Fund, the National Institutes of Health and the American Heart Association.

Study co-authors are Kimberly L. Fritz-Six, William P. Dunworth and Manyu Li, all of the UNC School of Medicine.


Newswise

Saturday, December 15, 2007

Effectiveness of the treatment-phase of two-phase complex decongestive physiotherapy for the treatment of extremity lymphedema.

Effectiveness of the treatment-phase of two-phase complex decongestive physiotherapy for the treatment of extremity lymphedema.

Int J Clin Oncol. 2007 Dec

Yamamoto R, Yamamoto T.
Rhythmic Obstetrics and Gynecology Clinic, 4F-1, No. 26 Keiwa bldg., Kita 24, Nishi 4, Kita-Ku, Sapporo, 001-0024, Japan,
rhythmic@minos.ocn.ne.jp.

BACKGROUND: Complex decongestive physiotherapy (CDP) consists of a two-phase treatment program and is the international standard therapy for lymphedema. However, this therapy is not performed at most hospitals in Japan.

METHODS: The subjects of the present study were 82 Japanese women with lymphedema of an extremity (median age, 64 years; range, 40-86 years). The volume of the affected extremity was compared before and after therapy, and the duration of the CDP treatment phase and rate of edema reduction were ascertained. The associations between the effect of CDP and duration of lymphedema, operative procedure, and radiotherapy were also investigated.

RESULTS: For patients with upper-extremity lymphedema, the median duration of the CDP treatment phase was 6 treatment days (range, 3-26 days), median reduction of edema volume was 328.7 ml (range, 76.6-1258.0 ml; P = 0.0014), and median rate of edema reduction was 58.9% (range, 42.7%-97.1%). For patients with lower-extremity lymphedema, the median duration of the CDP treatment phase was 10 treatment days (range, 2-35 days), median reduction of edema volume was 1573.7 ml (range, 293.9-3471.1 ml; P <>

CONCLUSION: In a study of Japanese women with lymphedema, CDP comprising a two-phase treatment program was clearly effective.

Springer Link

Friday, November 30, 2007

Secondary scrotal lymphedema: A novel microsurgical approach

Secondary scrotal lymphedema: A novel microsurgical approach
Sylvain Mukenge, M.D. 1 *, Carlo Pulitanò, M.D. 1, Renzo Colombo, M.D. 2, Daniela Negrini, B.D. 3, Gianfranco Ferla, M.D. 1
1Department of Surgery, Scientific Institute San Raffaele, Vita-Salute San Raffaele University, Milan, Italy2Department of Urology, Scientific Institute San Raffaele, Vita-Salute San Raffaele University, Milan, Italy3Department of Experimental and Clinical Biomedical Sciences, University of Insubria, Varese, Italy
email: Sylvain Mukenge
(
mukenge.mvunde@hsr.it)

*Correspondence to Sylvain Mukenge, Department of Surgery, Scientific Instistute H San Raffaele, Via Olgettina 60, 20132 Milan, Italy
setDOI("ADOI=10.1002/micr.20426")


Abstract

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.

Wiley InterScience

Thursday, November 22, 2007

Infectious Disease Doctor for Lymphedema

Infectious Disease Doctor

I am a firm believer in the concept of a “team” approach to the management and treatment of lymphedema and its associated complications.

What is a Team Approach?

The team approach is a group of medical providers working together for the well being and health of the lymphedema patient.

In my situation, my team consists of

Primary Care Doctor
Oncologist
Pulmonary Doctor
Infectious Diease Doctor

Why Do I Need an Infectious Disease Doctor?

Until I acquired lymphoma, the most pressing complication that I experienced with my leg lymphedema was recurrent and severe cellulitiis. It was apparent long ago that we desperately needed a way to control it (try to prevent) and if it did occur to promptly and successfully treat the infection.

The result is that I have been under the care of an ID doctor now for over twenty years.
Because of our susceptibility to infections and because our lymphedema
arm or leg is immunocompromised, it is essential that any lymphedema patient with recurrent infections enlist the aide of an “ID” doctor. They are specially trained in infections and are far more qualified to treat them then any other type of doctor.

My ID doc, Dr. Elliott Raizes has been a life saver and a God send in my own battle with lymphedema. Not only does he has that “old fashioned” and rapidly disappearing concern for his patients, but his knowledge of bacterial infections is unbelievable.

How Do I Find an Infectious Disease Doctor?

The easiest way is to simply ask or if need be insist on a referral to one in your area from your PCP. They may already know of a good doctor within your community.

You can also find participating ID doctors in your insurance plan through their “Find a Doctor” service or you can locate one through the internet.

Pat

What is an ID Specialist?

Infectious Disease Specialists are like medical detectives. They examine difficult cases, looking for clues to identify the culprit and solve the problem.

Your ID Physician Has 9-10 Years of Specialized Education & Training

4 years of medical school
3 years training as a doctor of internal medicine
2-3 years specialized training in infectious diseases

Most ID specialists who treat patients also are board certified. They have passed a difficult certification examination by the American Board of Internal Medicine in both internal medicine and infectious diseases.

Reducing Risk of Infectious Disease

One of the best strategies for preventing infectious diseases is immunization. Make sure you and your children receive all recommended vaccinations.

Ask your doctor for advice about other things you and your family can do to prevent infectious diseases.

When You Need an ID Specialist

Many common infections can be treated by your personal physician. Your doctor might refer you to an ID specialist in cases where an infection is difficult to diagnose, is accompanied by a high fever, or does not respond to treatment. ID specialists also see healthy people who plan to travel to foreign countries or locations where infection risk is higher. In these cases, ID specialists can help determine whether special immunizations or other preventive measures are necessary to protect travelers from disease.

Typical Procedures

ID specialists review your medical data, including X-rays and laboratory reports such as blood work and culture data. They also may perform a physical exam to help determine the cause of the problem.

Tests

ID specialists often order laboratory tests to examine samples of blood or other body f luids or cultures from wounds. A blood serum analysis can help the ID specialist detect antibodies that indicate what type of infection you have. These advanced tests can further explain the results of earlier tests, helping to pinpoint the problem.

Treatments

Treatments consist of medicines—usually antibiotics—to help battle the infection and prevent it from returning. These medicines may be given to you orally (in the form of pills or liquids) or administered directly into your veins, via an IV tube. Many ID specialists have IV antibiotic therapy available in their offices, which decreases the likelihood that you will need to be hospitalized.

What Information Should You Give Your ID Specialist?

All medical records related to your condition

X-rays, laboratory reports and immunization records. Often your personal physician will forward this information to the specialist before your scheduled appointment.
A list of all medications you take

This list should include over-the-counter and prescription medications

A list of any allergies you have.

Let the ID specialist know if you are taking birth control pills.

Some antibiotics may interfere with the effectiveness of oral contraceptives.

How Does My ID Specialist Work With Other Medical Professionals?

The ID specialist works with your personal physician to determine which diagnostic tests are appropriate. If treatment is necessary, your doctor and the ID specialist will work together to develop a treatment plan best suited to your needs. Often you will be asked to return to the ID specialist for a follow-up visit. This allows the specialist to check on your progress, confirm that the infection is gone and help prevent it from coming back. If you acquire an infection while in the hospital, the ID specialist will work with other hospital physicians to help direct your care.

The specialist also might provide follow-up care after you go home.

If your ID specialist is also your personal physician, he or she will coordinate your care, referring you to other specialists when necessary.

ID Specialists Are Experts in the Diagnosis and Treatment of Illnesses Caused by Microorganisms.

ID specialists see patients to determine whether their symptoms are due to an infection.

Patients often see ID specialists due to a fever.

Some ID specialists serve as primary care physicians, for example, for people with HIV/AIDS, treating most illnesses and coordinating their patients’ overall care.

In all of these cases, the specialized training and diagnostic tools of the ID specialist can help determine the cause of your infection and the best approach to treatment.

Where Can I Get More Information About the Prevention and Treatment of Infectious Diseases?

Your doctor is your best source of information. In addition, the Infectious Diseases Society of America (IDSA), a professional organization of nearly 8,000 ID physicians, scientists, and other infectious diseases experts, can help point you in the direction of resources and additional information.

Visit IDSA’s website

Infectious Disease

Infectious Disease Internal Medicine Subspecialty

An infectious disease (ID) specialist is a doctor of internal medicine (or, in some cases, pediatrics) who is qualified as an expert in the diagnosis and treatment of infectious diseases. After seven or more years of medical school and postgraduate training, ID specialists complete two to three years of additional training in infectious diseases.

ID specialists have expertise in infections of the sinuses, heart, brain, lungs, urinary tract, bowel, bones and pelvic organs. Their extensive training focuses on all kinds of infections, including those caused by bacteria, viruses, fungi and parasites. Many ID physicians specialize in treating patients with infections due to human immunodeficiency virus (HIV), the cause of AIDS.
Along with their specialized knowledge comes a particular insight into the use of antibiotics and their potential adverse effects. ID specialists also have additional training in immunology (how the body fights infection), epidemiology (how infections spread) and infection control.

What an infectious disease specialist does

The role of an infectious disease specialist is to review a patient's medical data, including records, X-rays and laboratory reports. They may perform a physical examination, depending on the type of problem. They also counsel healthy people who plan to travel to countries where there is an increased risk of infection.

Laboratory studies are often necessary and may include blood studies and cultures of wounds or body fluids. They may order blood serum studies for antibodies indicating the presence of unusual or uncommon diseases. These studies may help explain the results of studies that a general internist may already have done.

Work in the infectious disease specialty is limited to diagnosis and medical treatment. Infectious diseases specialists do not perform surgery.

When you need an infectious disease specialist

Not everyone who has an infectious disease needs an infectious disease specialist. Your general internist can take care of most infections, but sometimes specialized expertise is needed to either diagnose or manage specific infectious diseases.

When a fever raises the suspicion that you may have an infection, when an infection is potentially serious, or when problems occur with treatment, it may be necessary to consult an infectious diseases specialist. ID specialists can provide special insight into tests that will be helpful in diagnosing and understanding the infection and preventing recurrent infections. They can often help determine what treatment you need, if any, and whether you should receive antibiotics. You may not require any treatment, but if you do, they may confer with your personal physician about which diagnostic testing and forms of treatment are best suited to your needs. If you are hospitalized for an infection or acquire an infection while hospitalized, ID specialists will follow and help direct your hospital care. In some cases, they may continue to see you after you go home from the hospital.

How infectious disease specialists work with other physicians

Although infectious diseases specialists sometimes serve as primary care physicians, in most cases you will still need your regular doctor. Usually you will be asked to return to the ID specialist for a follow-up visit to review test results and to be sure that your infection has been eliminated. ID specialists may wish to follow up with you until we feel confident that the infection will not recur. You will resume care with your regular physician when your condition has stabilized or is cured.

Doctors for Adults

See also: Infectious Disease Doctor

Thursday, November 15, 2007

Lymphedema - Medicare - What You Can Do

Lymphedema - Medicare - What You Can Do
I'd like to start everyone off with the following three steps. Take on as much as you all can but don't over-do. I know we all have limited time and energy. The first thing to do is to get your lymphedema under control, then we can start educating and lobbying.

1. If you have recently been denied compression bandages, garments or devices, and you are still within the time period for an appeal, let's get the appeal going. I'll supply the proper forms and help you fill them out. If you have paid for compression materials and have not been formally denied, I'll send you a Medicare claim form. Otherwise I'll help you write a claim letter.

2. I must also know whether you wish to work towards national Medicare legislation or State legislation for the diagnosis and treatment of lymphedema.

Tell me your 9-digit ZIP code and I'll tell you the name and contact info for your legislators with suggestions as to the best ones to contact.

3. When you have some spare time, call your legislators to urge them to support (or introduce) legislation which affects your ability to access quality lymphedema care. Some current bills are as follows:

Stopgap legislation has been introduced in both the Senate and House (S. 543 Nelson/H.R. 1459 Tanner) that would freeze implementation of 75% Rule and prevent additional access problems for individuals requiring lymphedema treatment.

The House recently passed the Children's Health and Medicare Protection (“CHAMP”) Act of 2007, which includes a freeze in the implementation of the 75% Rule at the 60% threshold indefinitely.

The legislation would also allow patients' co-morbidities to continue to be considered for purposes of meeting the 75% Rule quota.

In March 2005 CMS changed their interpretation of the Social Security Act to limit coverage of manual lymph drainage to physical and occupational therapists, thereby eliminating coverage of treatment by specially-qualified nurses, physicians, osteopaths, chiropractors and massage therapists.

This effectively reduced the numberof available trained therapists by some 30 percent. A number of lymphedema treatment clinics around the country have already been forced to close as a result of this policy change. H.R. 1846 Towns attempts to partially rectify this ill-advised policy by restoring physician authority in selecting qualified medical personnel to perform “incident to” services.

Also in March 2006 lymphedema was exempted automatically from therapy caps that were re-imposed on January 1, 2006. But in January 2007 exemption was made dependent on existence of a co-condition. This constitutes another roadblock to access to physician-prescribed treatment for lymphedema patients.

S. 450 Ensign/H.R. 748 Becerra would permanently eliminate therapy caps.

Thanks for helping.

BobRobert Weiss, M.S.Lymphedema Treatment Advocate

Email: LymphActivist@aol.com

Saturday, November 10, 2007

Lymphedema - Medicare News (Not Good)

Lymphedema - Medicare News (Not Good)

My proposal to CMS to add over 150 new and revised codes to the HCPCS Codebook for lymphedema treatment supplies was just rejected again by the CMS HCPCS Workgroup in Baltimore. This was very depressing because they never referred to my legal and medical arguments--only stated that "no insurer (i.e. Medicare, Medicaid, Private Insurance Sector) identified a national program operatingneed to establish unique codes to distinguish all the products listed in this application. Existing codes adequately describe the array of products available."

This reiterates my feeling that I have not gotten the required support from providers, suppliers, patients, manufacturers to convince "Medicare, Medicaid and the Private Insurance Sector" that there could be a "program operating need", and that there is a proposed solution to the problem which has a rational legal and medical basis. My record with Medicare Administrative Law Judges recently, since developing my "prosthetic devices" argument, is 4 favorable, 1unfavorable decisions. Two cases have been appealed to the Medicare Appeals Council in Washington. My argument, in brief, is that compression bandages, garment and devices meet the definition in the Social Security Act of "prosthetic devices" when used in the standard treatment of lymphedema, and are covered by Medicare. They are usually denied because they do not meet the policies on surgical dressings benefit category, which is not their medical use or their benefit category.

I am in the process of preparing a formal request for a national coverage analysis on the coverage of the treatment of lymphedema that would lead to a new national coverage determination and policy, but progress is slow. I am also preparing a formal request to SADMERC for a reclassification of compression sleeves and bandages from uncovered S-codes to covered L-codes, and of compression stockings used in treatment of lymphedema from uncovered A-codes to covered L-codes. The request is also to add codes for other compression garments which are not currently described.

Another important reason for reclassifying compression garments from A-codes (Secondary surgical dressings) to L-codes (prosthetic devices) is that the specifications for elastic compression garments are different for the two functions, and the costs can be different. For example, an off-the-shelf elastic compression stocking used to hold a primary dressing on a venous ulcer (A-code) is reimbursed at $43.27. A custom flat-knit stocking used in the treatment of a lymphatic leg (L-code) has far more stringint technical specifications and costs far more than the circular-knit elastic stocking to manufacture.
Differences in use, medical requirements, manufacturing costs, etc warrant two different code groups.

I hope that I can get more support from patients, providers, suppliers and manufacturers this coming year in my efforts to get CMS to change their lymphedema treatment coverage policies. Only by appealing every denial of medical treatment for lymphedema can we impress on CMS that there IS a program operating need for new coverage policies and codes. Denials are based on non-relevantpolicies, HCPCS codes are being used incorrectly, and there are inadequate codes for the medically necessary coverable items used every day by lymphedema patients.

And if CMS does not chose to understand the issue, and in the absence of a lymphedema lobby, we must rely on legislators' constituents (that means YOU) to create the awareness of a need for legislative change.

Robert Weiss, M.S.

Lymphedema Treatment Advocate

Wednesday, November 07, 2007

Elephantiasis nostras verrucosa complicated with chronic tibial osteomyelitis

Elephantiasis nostras verrucosa complicated with chronic tibial osteomyelitis
Arch Orthop Trauma Surg. 2007 Nov 3

Turhan E, Ege A, Keser S, Bayar A.
School of Medicine, Department of Orthopaedics and Traumatology, Medicine Faculty of Zonguldak Karaelmas University, Kozlu, 67100, Zonguldak, Turkey,
dregementurhan@yahoo.com.

Keywords: Elephantiasis nostras verrucosa - Tibial - Osteomyelitis - Amputation

Elephantiasis nostras verrucosa represents an infrequent clinical entity with cutaneous changes characterized by dermal fibrosis, hyperkeratotic verrucous and papillamotous lesions resulting from chronic non-filarial lymphedema secondary to infections, surgeries, tumor obstruction, radiation, congestive heart failure, and obesity. Although recurrent streptococcal lymphangitis is believed to play a critical role in the origin of elephantiasis nostras verrucosa, the exact pathogenesis of the disorder is not yet clear. Therapeutic efforts should aim to reduce lymph stasis, which will also lead to improvement of the cutaneous changes but unfortunately there is no specific treatment for advanced cases. In this report, we present a patient who was treated by below knee amputation as a result of elephantiasis nostras verrucosa complicated with chronic tibial osteomyelitis.

Springer Link

Tuesday, October 30, 2007

Lymphedema sufferers must cope for a lifetime

Lymphedema sufferers must cope for a lifetime

BY BORDEN BLACK --

Special to the Ledger-Enquirer (Columbus, Georgia)
Tuesday Oct. 30, 2007


------

Lymphedema is one of those little-known conditions that doesn't spark telethons or poster children. It is life-altering, however, and can dictate lifestyle. I should know. I've had lymphedema since the lymph nodes in my legs were removed following cancer surgery.

About lymphedema

The lymph system has been described as the sewage system of the body. Waste products, bacteria, dead cells and large protein molecules are transported in lymph from tissue to the nodes where they are broken down and eliminated. When the system is broken, abnormal accumulation of protein-rich fluid can accumulate, usually in an arm or leg. This results in swelling in the limb and thickening of the skin known as lymphedema. The condition can be painful and disfiguring and can result in infections, depression and decreased mobility.
While primary lymphedema occurs at birth, secondary lymphedema is the result of damage to the lymphatic system. It is the most common form of lymphedema, and frequently occurs in cancer patients. Dr. Kathleen Francis, Medical Director of the Barnabas Lymphedema Treatment Center in Livingston, N.J., points out that if the lymph nodes in the armpit area are removed or radiated as a result of breast cancer treatment, it can lead to lymphedema.


She was among the doctors, therapists and patients who gathered in Atlanta recently for a conference sponsored by the Lighthouse Lymphedema Network. The nonprofit support organization was formed by Joan White, who developed the condition as the result of appendicitis. The idea for the organization grew from a discussion around her kitchen table in 1994. She points out that quality of life is a huge issue for those with the condition, many of whom have survived cancer only to find that they must continue to spend a great deal of time and energy dealing with the resulting lymphedema. The need for support, advice and awareness led to formation of the network.

The problems

Dr. Paula Steward, Medical Director of Lakeshore HealthSouth Rehabilitation Hospital in Birmingham, Ala., explained the problem faced by those suffering from lymphedema by quoting Dr. Robert Lerner: "In the past physicians played down the importance of lymphedema and pointed out that it is rare, that there is no effective treatment and that the patient must learn to live with it... . They have failed to instruct patients on how to avoid lymphedema... and continue to grossly understate the incidence of this serious and lifelong illness." She points out that correct diagnosis is essential in providing effective treatment.

Several other conditions, including inflammatory arthritis and post-phlebitic syndrome, have similar symptoms so accurate tests are needed for proper diagnosis. Currently, ultrasound, magnetic resonance imaging and computed tomography (CT) are used, but a diagnostic tool being employed in Europe is seen as the best option. Dr. Waldemar Olszewski, a world renowned expert in lymphedema, came from the Polish Academy of Science Hospital in Warsaw to discuss with conference attendees isotopic lymphoscintigraphy. The procedure has yet to be approved for use in America, but according to Olszewski: "It is necessary for the establishment of rational therapy."

Once correctly diagnosed, the lymphedema patient faces a lifetime of treatment because there is no cure. Swelling can be managed through a specialized massage technique known as manual lymphatic drainage, by using compression garments and bandages daily, by engaging in regular exercise and maintaining meticulous skin and nail care.

White points out that in many cases physicians are not acquainted with treatment for the condition and patients get most of their information from massage therapists. In fact she says often patients with swelling are diagnosed by therapists. Another difficulty according to White is the failure of insurance companies to pay for therapy or compression garments.

Solutions

Although there is no cure, Dr. David Feingold told those attending the Lighthouse conference that genetics may be the answer. "How do you generate new lymphatics?" That is the question being examined by the genetic researcher. Three genes have been identified, and experiments are under way.

Until an answer is found, awareness and education are the focus of the Lighthouse Lymphedema Network. A bill has been introduced by Georgia state Rep. Debbie Buckner. The house bill provides for a pilot program to test and evaluate a system of coverage for costs associated with the prevention and treatment of lymphedema and to provide for data collection.

The Atlanta organization has also set aside Oct. 22 as Lymphedema Awareness Day in Georgia.

Although awareness is growing, White points out that it is still "an image thing" and many of those with the condition are reluctant to go public and as a result there is still a lack of emphasis.

Ledger Inquirer

Saturday, October 27, 2007

Identification of a novel VEGFR-3 missense mutation in a Chinese family with hereditary lymphedema type I.

Identification of a novel VEGFR-3 missense mutation in a Chinese family with hereditary lymphedema type I.

J Genet Genomics. 2007 Oct

Yu Z, Wang J, Peng S, Dong B, Li Y.

Department of General and Vascular Surgery, Beijing Tongren Hospital, Capital Medical University, Beijing 100730 China.

Keywords: hereditary lymphedema; vascular endothelial growth factor receptor gene (VEGFR-3); missense mutation; autosomal dominant

A novel mutation of vascular endothelial growth factor receptor gene (VEGFR-3), was identified in a four-generation Chinese family with hereditary lymphedema type I (HL-I). Genetic linkage analysis was performed on the known genetic locus for HL-I with a panel of polymorphic markers, and then mutations were screened out by direct sequencing. By genotyping, the family showed the linkage to HL-I locus on 5q35.3. Mutation screening analysis of the exons encoding the intracellular kinase domains of VEGFR-3, revealed a novel missense mutation D1055V. This mutation cosegregated with the disease phenotype in the family and was not found in 100 normal controls.

This finding has expanded the spectrum of the VEGFR-3 gene mutations causing HL-I, and will be useful for further genetic consultation and genetic diagnosis.

Science Direct

Monday, October 08, 2007

Lymphedema: A Comprehensive Review

Lymphedema: A Comprehensive Review
Ann Plast Surg. 2007 Oct
Warren AG, Brorson H, Borud LJ, Slavin SA.
From the *Harvard Medical School, Boston, MA; †Harvard Medical School, Division of Plastic Surgery, Beth Israel Deaconess Medical Center, Boston, MA; and the ‡Department of Plastic and Reconstructive Surgery, Lund University, Malmö University Hospital, Malmö, Sweden.

BACKGROUND:

Lymphedema is a chronic, debilitating condition that has traditionally been seen as refractory or incurable. Recent years have brought new advances in the study of lymphedema pathophysiology, as well as diagnostic and therapeutic tools that are changing this perspective.

OBJECTIVE:

To provide a systematic approach to evaluating and managing patients with lymphedema.

METHODS:

We performed MEDLINE searches of the English-language literature (1966 to March 2006) using the terms lymphedema, breast cancer-associated lymphedema, lymphatic complications, lymphatic imaging, decongestive therapy, and surgical treatment of lymphedema. Relevant bibliographies and International Society of Lymphology guidelines were also reviewed.

RESULTS:

In the United States, the populations primarily affected by lymphedema are patients undergoing treatment of malignancy, particularly women treated for breast cancer. A thorough evaluation of patients presenting with extremity swelling should include identification of prior surgical or radiation therapy for malignancy, as well as documentation of other risk factors for lymphedema, such as prior trauma to or infection of the affected limb. Physical examination should focus on differentiating signs of lymphedema from other causes of systemic or localized swelling. Lymphatic dysfunction can be visualized through lymphoscintigraphy; the diagnosis of lymphedema can also be confirmed through other imaging modalities, including CT or MRI. The mainstay of therapy in diagnosed cases of lymphedema involves compression garment use, as well as intensive bandaging and lymphatic massage. For patients who are unresponsive to conservative therapy, several surgical options with varied proven efficacies have been used in appropriate candidates, including excisional approaches, microsurgical lymphatic anastomoses, and circumferential suction-assisted lipectomy, an approach that has shown promise for long-term relief of symptoms.

CONCLUSIONS:

The diagnosis of lymphedema requires careful attention to patient risk factors and specific findings on physical examination. Noninvasive diagnostic tools and lymphatic imaging can be helpful to confirm the diagnosis of lymphedema or to address a challenging clinical presentation. Initial treatment with decongestive lymphatic therapy can provide significant improvement in patient symptoms and volume reduction of edematous extremities. Selected patients who are unresponsive to conservative therapy can achieve similar outcomes with surgical intervention, most promisingly suction-assisted lipectomy.

PMID: 17901744 [PubMed - as supplied by publisher]

Wednesday, September 26, 2007

Lymphedema, cardiac septal defects, and characteristic facies: Possible new case of Irons-Bianchi syndrome.

Lymphedema, cardiac septal defects, and characteristic facies: Possible new case of Irons-Bianchi syndrome.
Am J Med Genet A. 2007 Sep 12

van Steensel MA, van Geel M, Schrander-Stumpel C, Steijlen PM, Veraart JC.

email: M.A.M. van Steensel (mvst@sder.azm.nl)
*Correspondence to M.A.M. van Steensel, Department of Dermatology, University Hospital Maastricht, P.O. Box 5800, 6202 AZ Maastricht, The Netherlands.

Department of Dermatology, University Hospital Maastricht, Maastricht, The Netherlands.

We describe a Dutch girl with fetal hydrops, congenital lymphedema of the lower legs, complex congenital cardiac malformation, and a typical face with epicanthal folds. This particular combination of symptoms has been previously described by Irons and Bianchi in 1996. Our report confirms their observation and suggests that this particular constellation of symptoms may constitute a new syndrome. Molecular analysis confirms this statement by demonstrating absence of mutations in several genes known to be involved in syndromes with lymphedema.

(c) 2007 Wiley-Liss, Inc.

Wednesday, September 12, 2007

A randomized controlled trial of weight reduction as a treatment for breast cancer-related lymphedema.

A randomized controlled trial of weight reduction as a treatment for breast cancer-related lymphedema.

Cancer. 2007 Sep

Clare Shaw, PhD, RD 1 *, Peter Mortimer, MD 2, Patricia A. Judd, PhD, RD 3
1Department of Nutrition and Dietetics, Royal Marsden National Health Service Foundation Trust, London, United Kingdom2Dermatological Medicine, Royal Marsden National Health Service Foundation Trust, London, United Kingdom3Nutrition and Dietetics, Lancashire School of Health and Postgraduate Medicine, University of Central Lancashire, Preston, Lancashire, United Kingdom

email: Clare Shaw (clare.shaw@rmh.nhs.uk)

Correspondence to Clare Shaw, Royal Marsden National Health Service Foundation Trust, Fulham Road, London SW3 6JJ, United Kingdom

Keywords: diet • weight reduction • breast cancer • lymphedema • intervention

BACKGROUND: Obesity is considered a risk factor for the development of breast cancer-related lymphedema of the arm and as a poor prognostic factor in response to lymphedema treatment. The objective of this study was to examine weight reduction as a treatment for breast cancer-related lymphedema.

METHODS: Twenty-one women with breast cancer-related lymphedema were randomized either to receive dietary advice for weight reduction or to receive a booklet on general healthy eating. They were monitored for 12 weeks.

RESULTS: The primary outcome measure was arm volume at 12 weeks. The results indicated a significant reduction in swollen arm volume at the end of the 12-week period (P = .003) in the intervention weight-reduction group. There was a significant reduction in body weight (P = .02) and body mass index (P = .016) in the weight-reduction group at the end of the 12-week study period.

CONCLUSIONS: Weight loss achieved by dietary advice to reduce energy intake can reduce breast cancer-related lymphedema significantly. Cancer 2007. (c) 2007 American Cancer Society.

Wiley Interscience

Monday, August 20, 2007

The role of immune system in colon metastasis. Lymphanogiogenesis or lymphedema in cancer tissue

The role of immune system in colon metastasis. Lymphanogiogenesis or lymphedema in cancer tissue

Pol Merkur Lekarski. 2007 May

[Article in Polish]
Stańczyk M, Olszewski WL, Gewartowska M, Maruszyński M.
Zakład Chirurgii Transplantacyjnej, IMDiK PAN w Warszawie.
ms@cmdik.pan.pl

The extent of lymph node metastasis is a major determinant for the staging and the prognosis of most human malignancies. Although the clinical significance of lymph node involvement is well documented, molecular mechanisms that promote tumor spread into the lymphatic or blood vascular systems and widespread dissemination are not well understood.

Although there is a large body of evidence that newly visualized lymphatics facilitate formation of metastases, it remains unclear whether these are "new" or simply pre-existing dilated vessels. High level of permeability of tumor blood capillaries brings about high tissue fluid and lymph formation. The physical forces but not the putative cancer-produced VEGF C may be responsible for more lymphatics seen around cancer than in normal tissue.

The main question to be answered is: are there morphologic and functional differences between newly formed and pre-existing intra- or peri-tumoral lymphatics? In our experience specimens of gastric and colon cancer revealed presence of peri-tumoral but not intra-tumoral lymphatics. Tumor tissue contained numerous tissue fluid "lakes" communicating with lymphatics.

We speculate that increased production of lymph in the tumor tissue caused by high blood capillary permeability brings about dilatation of the interstitial space and peri-tumoral lymphatics. Excessive lymph flow may drag tumor cells. This article is reviews current literature on the role of angiogenesis and lymphangiogenesis in cancer metastasis with respect to own research.

PMID: 17679395 [PubMed - in process]

Friday, August 03, 2007

The Thoracic and Abdominal Lymphatics Abstracts

The Thoracic and Abdominal Lymphatics Abstracts

Lymphatic drainage between thorax and abdomen: please take good care of this well-performing machinery...

Acta Clin Belg Suppl. 2007

ZiekenhuisNetwerk Antwerpen, Campus Stuivenberg, Intensive Care Unit, Antwerp, Belgium
.
manu.malbrain@skynet.be

INTRODUCTION: Patients with sepsis often receive large amounts of fluids and the presence of capillary leak, trauma or bleeding results in ongoing fluid resuscitation. This increases interstitial and intestinal edema and finally leads to intra-abdominal hypertension (IAH), which in turn impedes lymphatic drainage. Patients with IAH often develop secondary respiratory failure needing mechanical ventilation with high intrathoracic pressure or PEEP that might further alter lymphatic drainage. This review will try to convince the reader of the importance of the lymphatics in septic patients with IAH.

METHODS: A Medline and PubMed literature search was performed using the terms "abdominal pressure", "lymphatic drainage" and "ascites formation".The references from these studies were searched for relevant articles that may have been missed in the primary search. These articles served as the basis for the recommendations below.

RESULTS: Induction of sepsis with lesion of the capillary alveolar barrier results in an increased water gradient between the capillaries and the interstitium in the lungs. The drainage flow to the thoracic duct is initially increased in order to protect the Lung and maintain the pulmonary interstitium as dry as possible, however this results in increased intrathoracic pressure. Sepsis also increases the permeability of the capillaries in the splanchnic beds. In analogy to the lungs the lymphatic flow in the splanchnic areas increases together with the pressure inside as a physiological response in order to limit the increase in IAP. At a critical IAP level (around 20 cmH2O) the lymph flow starts to decrease and the splanchnic water content progressively increases.The lymph flow from the abdomen to the thorax is progressively decreased resulting in increased splanchnic water content and ascites formation. The presence of mechanical ventilation with high PEEP reduces the lymph drainage further which together with the increase in IAP decreases the lymphatic pressure gradient in the splanchnic regions, with a further increase in water content and IAP triggering a vicious cycle.

CONCLUSION: Although often overlooked the role of lymphatic flow is complex but very important to determine not only the fluid balance in the lung but also in the peripheral organs. Different pathologies and treatments can markedly influence the pathophysiology of the lymphatics with dramatic effects on endorgan function.

PMID: 17469714
[
PubMed - indexed for MEDLINE]

Lymphatic drainage of the peritoneal space: a pattern dependent on bowel lymphatics.


Parungo CP, Soybel DI, Colson YL, Kim SW, Ohnishi S, DeGrand AM, Laurence RG, Soltesz EG, Chen FY, Cohn LH, Bawendi MG, Frangioni JV. Department of Surgery, Brigham & Women's Hospital, 75 Francis Street, Boston, Massachusetts 02115, USA. John V. Frangioni Email: jfrangio@bidmc.harvard.edu Dr. Parungo was the recipient of an award at the SSO meeting.

Keywords: Peritoneal space - Lymph node - Lymphatic drainage - Near-infrared fluorescence - Carcinomatosis - Metastasis

BACKGROUND: Understanding lymph drainage patterns of the peritoneum could assist in staging and treatment of gastrointestinal and ovarian malignancies. Sentinel lymph nodes (SLNs) have been identified for solid organs and the pleural space. Our purpose was to determine whether the peritoneal space has a predictable lymph node drainage pattern.

METHODS: Rats received intraperitoneal injections of near-infrared (NIR) fluorescent tracers: namely, quantum dots (designed for retention in SLNs) or human serum albumin conjugated with IRDye800 (HSA800; designed for lymphatic flow beyond the SLN). A custom imaging system detected NIR fluorescence at 10 and 20 minutes and 1, 4, and 24 hours after injection. To determine the contribution of viscera to peritoneal lymphatic flow, additional cohorts received bowel resection before NIR tracer injection. Associations with appropriate controls were assessed with the chi(2) test.

RESULTS: Quantum dots drained to the celiac, superior mesenteric, and periportal lymph node groups. HSA800 drained to these same groups at early time points but continued flowing to the mediastinal lymph nodes via the thoracic duct. After bowel resection, both tracers were found in the thoracic, not abdominal, lymph node groups. Additionally, HSA800 was no longer found in the thoracic duct but in the anterior chest wall and diaphragmatic lymphatics.

CONCLUSIONS: The peritoneal space drains to the celiac, superior mesenteric, and periportal lymph node groups first. Lymph continues via the thoracic duct to the mediastinal lymph nodes. Bowel lymphatics are a key determinant of peritoneal lymph flow, because bowel resection shifts lymph flow directly to the intrathoracic lymph nodes via chest wall lymphatics.

Springerlink

Lymphatic drainage of the diaphragmatic pleura to the peritracheobronchial lymph nodes.

Surg Radiol Anat. 2003 Apr Okiemy G, Foucault C, Avisse C, Hidden G, Riquet M. Laboratoire d'Anatomie, UFR de Médecine, Reims, France.

Keywords: Diaphragm, Pleural lymphatics, Mediastinum, Lymph node, Lung cancer

Non-small cell lung cancer invading the visceral pleura is characterized by a particular richness of mediastinal lymph node (LN) metastases. This may be due to subpleural lymphatic drainage of tumor cells. The aim of this study was to determine mediastinal LN lymphatic drainage from the diaphragmatic pleura. Subpleural lymphatics of 30 adult cadavers and 12 fetuses were injected with a modified Gerota's medium to permit lymph vessels and nodes to be visualized and then dissected. Each stage of the dissection was described and photographed.

In 32 cadavers mediastinal visceral LN chains were injected, of which 29 originated from the mediolateral portion of the diaphragm. On the right, injections (n=16) demonstrated lymph vessels (n=20) ascending directly along the inferior pulmonary ligaments (n= or after having encircled the inferior vena cava (n=, and lymph vessels passing between the pulmonary veins (n=4); all these lymphatics were connected to the intertracheobronchial nodes and some ascended along the tracheobronchial LN chains in the upper mediastinum.

On the left, injections (n=13) demonstrated lymph vessels (n=16) ascending along the inferior pulmonary ligament (n=5) or along the esophagus (n=11) and connecting to the intertracheobronchial nodes, some of which ascended further in the upper mediastinum (left paratracheobronchial LN chain).

These mediastinal LN chains are the same as those that receive lymph from the pulmonary segments. Lymphatic drainage of the diaphragmatic pleura may add to that of the lung involved in cancer and potentially increases lymphatic spread of tumor cells.

Springerlink

Renal lymphatic drainage and thoracic duct connections: implications for cancer spread.

Lymphology.

2006 Mar Assouad J, Riquet M, Foucault C, Hidden G, Delmas V. Service de Chirurgie Thoracique, Hôpital Européen Georges Pompidou, and Institut d'Anatomie, Biomédicale des Saints-Pères, Paris, France.

Studies on renal lymph drainage have generally described lymph nodes without further investigation of the lymph vessels. Our purpose was to revisit this organ to study the vessel drainage pattern. This investigation was performed on 16 refrigerated adult cadavers. After both kidneys were injected with a blue modified Gerota mass, lymph vessels were dissected until their termination. From the right kidneys (n = 13), lymphatics (n = traveling on the anterior aspect of the inferior vena cava were dissected, reaching interaortocaval and more distant nodes, aorta bifurcation (n = 1) and left lateroaortic (n = 1); posterior lymphatics were observed in all subjects, uniformly connecting to the thoracic duct, either after crossing nodes (n = or directly (n = 5). From the left kidneys (n = 13), anterior efferents (n = 16) were dissected, reaching left lateroaortic and also celiac (n = 4) and iliac (n = 1) nodes; posterior lymphatics were also demonstrated, always connecting to the thoracic duct (3 directly). Renal lymphatics have been found to reach very distant nodes as well as always connecting to the origin of the thoracic duct. This feature suggests an important role in both the formation of the thoracic duct and in the spread of renal cancer.

PMID: 16724507 [PubMed - indexed for MEDLINE]

Regional recruitment of rat diaphragmatic lymphatics in response to increased pleural or peritoneal fluid load

J Physiol. 2007 Mar

Moriondo A, Grimaldi A, Sciacca L, Guidali ML, Marcozzi C, Negrini D. Dipartimento di Scienze Biomediche Sperimentali e Cliniche, Università degli Studi dell'Insubria, Via J.H. Dunant 5, 21100 Varese, Italy. Corresponding author D. Negrini: Dipartimento di Scienze Biomediche Sperimentali e Cliniche, Università degli Studi dell'Insubria, Via J.H. Dunant 5, 21100 Varese, Italy.Email: daniela.negrini@uninsubria.it

The specific role of the diaphragmatic tendinous and muscular tissues in sustaining lymph formation and propulsion in the diaphragm was studied in 24 anaesthetized spontaneously breathing supine rats. Three experimental protocols were used: (a) control; (b) peritoneal ascitis, induced through an intraperitoneal injection of 100 ml kg(-1) of iso-oncotic saline; and (c) pleural effusion, induced through an intrapleural injection of 6.6 ml kg(-1) saline solution. A group of animals (n = 12) was instrumented to measure the hydraulic transdiaphragmatic pressure gradient between the pleural and peritoneal cavities in the three protocols. In the other group (n = 12), the injected iso-oncotic saline was enriched with 2% fluorescent dextrans (molecular mass = 70 kDa); at 30 min from the injections these animals were suppressed and their diaphragm excised and processed for confocal microscopy analysis. In control conditions, in spite of a favourable peritoneal-to-pleural pressure gradient, the majority of the tracer absorbed into the diaphragmatic lymphatic system converges towards the deeper collecting lymphatic ducts. This suggests that diaphragmatic lymph formation mostly depends upon pressure gradients developing between the serosal cavities and the lymphatic vessel lumen. In addition, the tracer distributes to lymph vessels located in the muscular diaphragmatic tissue, suggesting that active muscle contraction, rather than passive tendon stretch, more efficiently enhances local diaphragmatic lymph flow. Vice versa, a prevailing recruitment of the lymphatics of the tendinous diaphragmatic regions was observed in peritoneal ascitis and pleural effusion, suggesting a functional adaptation of the diaphragmatic network to increased draining requirements.

Abstract