Dear Dr. Marga:
My 32-year old daughter was just diagnosed with congenital lymphedema of her left leg. She started having leg swelling about 6 months ago. She has seen many doctors for this problem and has completed 10 or more tests in radiology. She does not have cancer and has never had surgery or radiation. She is not overweight and her doctors have not found any circulation problems.
I read about the lymph node transplant surgeries you offer for cancer patients and was wondering if my daughter might be a candidate. I am so worried about her condition and we would be willing to travel to any of your offices to have you see her.
Thank you for your response,
Thank you so much for contacting us about your daughter’s situation. I am sorry to hear of her leg swelling and hope we can help.
First and foremost, it appears that your daughter has been doing the right thing by working with her home physicians to ensure that she has the correct diagnosis.
Unilateral leg swelling in an adult female requires an extensive clinical investigation. It is important that such individuals be evaluated for abnormal blood clots in the deep veins of the legs (DVT) in addition to a possible new cancer in the pelvis or inguinal region. In women, this would include gynecologic cancers (uterine, cervical, and ovarian), lymphoma, melanoma, and even colon or rectal cancer. In men, we would need to include prostate cancer. This is likely why your daughter’s primary care physician ordered so many radiographic studies when she first presented.
Congenital lymphedema in an adult is what we call a “diagnosis of exclusion” – all possible known physiologic etiologies of secondary lymphedema must be excluded not just DVT or cancer, but venous insufficiency, HIV with associated Karposi sarcoma, obesity, soft tissue bacterial or parasitic infection and progressive soft tissue changes associated with prior irradiation.
Once a diagnosis of congenital lymphedema is made, it is important for patients and family members to seek appropriate education regarding risk reduction and a referral to a Certified Lymphedema Therapist (CLT) for Complex Decongestive Therapy (CDT) or “conservative management“. This would include skin care, Manual Lymphatic Drainage (MLD), grey foam-based short stretch compression bandaging and supervised exercise in addition to a physician monitored weight loss program if indicated. Phase 1 CDT programs typically extend over 4-8 weeks with the therapist preforming MLD and end with a transition to compression garments for daytime use and nighttime compression alternatives. Phase 2 of CDT is patient driven and focuses on self -MLD and compression management with intermittent supervision with their therapist.
In my practice, more sophisticated surgical interventions as lymphovenous bypass, lymphaticolymphatic bypass and lymph node transfers for lymphedema is restricted to patients who have failed conservative management. What this means is that a patient has been evaluated and treated by a CLT with minimal reduction in symptoms in the setting of adequate patient compliance. Furthermore, we are very interested in patients who experience repeated infections or progressive pain in the setting of prior irradiation even if they have had good size reduction with CDT.
You did not mention if your daughter had been seen by a CLT. If not, that should be your next step in her care. You should also refer the Position Papers of the National Lymphedema Network relating national standards of diagnosis, treatment, risk reduction, exercise, travel, CLT training requirements and screening for breast cancer patients. More information about lymph node transfers is available on National Institute of Lymphology.
I did want to share with you an example of a primary lymphedema patient that we treated with CDT at our office in New Orleans… I will call her “Miss Pamela” to protect her identity.
Miss Pamela presented to us with Stage 3 leg lymphedema (elephantiasis) of more than 10–years duration. She had been evaluated by her primary care physician and was found not to have cancer or a DVT. She denied any prior pelvic surgeries other than a C-Section and had never had pelvic irradiation. She denied a family history of lymphedema and had not traveled to areas endemic for filarial infection.
On interview, Miss Pamela did relate a past medical history significant for bilateral, symmetric leg swelling in the setting of her 3 pregnancies, the first being in her twenties. She related that with her third pregnancy, with twins, her left leg never returned to its original size after the babies were born. Furthermore, she related that the leg had become larger and larger over the last decade. Miss Pamela denied any form of cellulitis or soft tissue infections of the leg or pelvis at the time of her delivery or anytime thereafter.
At the time of her clinic evaluation, Miss Pamela related that she was having problems with ambulation and finding appropriate fitting clothing and shoes. She noted that she was experiencing progressive leg and right hip pain. She had suffered several falls but had never sustained a fracture. Careful discussion revealed that Ms. Pamela suffered significant social isolation although she continued to work 5 days weekly and was an active member of her church. When Miss Pamela presented to us, she was interested in surgical options to address her lymphedema as a means specifically to address her decreased mobility and pain.
After her evaluation that included an extensive history and physical examination, we concluded that Miss Pamela likely suffered congenital lymphedema but with a secondary component. We felt that she may have sustained a mechanical injury to her lymphatic system during her third pregnancy with twins. Our hypothesis was that added weight gain and a twin gestation resulted in an injury to what may have been a less than normal lymphatic system, an injury not so different from that which morbidly obese patients may suffer.
During Miss Pamela’s evaluation, we noted that she had never been referred to a CLT for CDT. So, that is where we started. We elected to treat Miss Pamela with with Vodder-based MLD twice daily and full-length compression bandaging 23-hours daily for 4-months. She was transitioned to a single leg daytime compression garment and tennis shoes and she continues to bandage at night. Her husband was educated to preform MLD in the home setting once daily. Ms. Pamela now takes walks for pleasure and she remains dedicated to her school and church. Way to go Ms. Pamela!
Danna, I hope this might help your daughter.
I have included Miss Pamela’s pre and post-treatment photos below. See what you think! CDT does work and ALL patients should start there prior to moving forward with any surgical intervention.