Tuesday, December 30, 2008

Quantitative bioimpedance spectroscopy for the assessment of lymphedema

Quantitative bioimpedance spectroscopy for the assessment of lymphoedema

Breast Cancer Res Treat. 2008 Dec

Ward LC, Czerniec S, Kilbreath SL.
School of Molecular and Microbial Sciences, University of Queensland, St Lucia, Brisbane, QLD, 4072, Australia,
l.ward@uq.edu.au.

The aim was to make bioimpedance spectroscopy (BIS) quantitative for assessment of lymphoedema. Apparent resistivity coefficients were determined for the intra- and extracellular water of arms in a control cohort of women (n = 66). These coefficients were used to predict water volumes in the arms of women with lymphoedema (n = 23) and a separate control group without lymphoedema (n = 13) and to compare these with total arm size measured by perometry. Total arm volume was highly correlated (r = 0.80-0.90) with arm fluid volumes predicted by BIS and the proportional increase in arm size predicted by BIS was not significantly different to that measured by perometry. BIS predicted that the increased volume in the women with lymphoedema was predominantly (60%) due to increase in extracellular fluid. BIS is capable of quantifying the volume increase in limb size seen in lymphoedema.

SpringerLink

Lymphedema in systemic juvenile arthritis: a rare extraarticular feature

Lymphedema in systemic juvenile arthritis: a rare extraarticular feature
Acta Reumatol Port. 2008 Oct-Dec

Ambrósio C, Abreu P, Alexandre M, Malcata A.
Serviço de Reumatologia dos Hospitais da Universidade de Coimbra.


Extraarticular features are usually found in rheumatological diseases sometimes with no correlation with the disease's activity in that moment. Lymphoedema is one of these manifestations and it's defined as a tissue fluid accumulation with gradual onset of swelling of a limb. Because it is an unusual finding, the physiopatological explanation of this feature is still difficult to understand. We describe a case report of lymphoedema on left upper arm, in a patient with systemic juvenile arthritis, with no identified cause and with no associated systemic signs or symptoms.

PMID: 19107091 [PubMed - in process]

Sunday, November 23, 2008

Supplemental surgical treatment to end stage (stage IV-V) of chronic lymphedema.

Supplemental surgical treatment to end stage (stage IV-V) of chronic lymphedema.

*Note - I am posting this for information and it shuld not be taken as an endorsement for the surgical treatment of lymphedema. It is critical that ALL efforts be made to treat and manage lymphedema through manual decongestive therapymanual lymphatic drainage (MLD, CDT) before any attempt at any surgery.

The results from MLD can be remarkable, even with limbs that would otherwise look hopeless.

Pat

Supplemental surgical treatment to end stage (stage IV-V) of chronic lymphedema.

Int Angiol. 2008 Oct

Lee BB, Kim YW, Kim DI, Hwang JH, Laredo J, Neville R.
Department of Surgery, Georgetown University, Washington DC, USA.

AIM: When the lymphedema reaches to its end stages, the complex decongestive therapy (CDT) and/or compression therapy become less effective and increased risk of systemic/general sepsis to become potentially life threatening condition.

METHODS: To improve its clinical management, excisional surgery was performed on 22 patients for their 33 limbs in the end stage of lymphedema as a supplemental therapy, and its efficacy was retrospectively analyzed. Diagnosis was made by radionuclide lymphoscintigraphy and basic laboratory studies (e.g. ultrasonography, magnetic resonance imaging). Twenty-two patients (mean age: 46 years; 3 male and 19 female; 5 primary and 17 secondary) submitted to the excisional surgery on 33 limbs (unilateral: 11; bilateral: 22). Surgery was indicated by further progression of the disease with recurrent sepsis despite adequate antibiotics therapy. A modified Auchincloss-Homan's operation was used to excise grotesquely disfigured soft tissue with advanced dermato-lipo-fibrosclerotic change. The normal limb contour was re-established to allow proper postoperative therapy. Postoperative CDT and compression therapy were mandatorily implemented in all cases.

RESULTS: A postoperative assessment of the treatment results, at 12 months showed an overall improvement in 28 of the 33 limbs: substantial improvement on the condition of limb function and quality of life (QOL), and local and/or systemic sepsis. Eighteen patients with good compliance to maintain the postoperative CDT showed much improved clinical results and QOL through the first interim assessment (24 months).

CONCLUSION: Excisional surgery at the end stage of lymphedema seems to provide substantial improvement of clinical condition and QOL only when mandated postoperative CDT/compression therapy is well kept.

PMID: 18974701 [PubMed - in process]

Wednesday, November 12, 2008

Do Bone Fractures Cause Lymphedema?

Do Bone Fractures Cause Lymphedema?
Over the past few years, there have been several members of my various groups who have reported lymphedema triggered by bone breaks. These cases involve both arm lymphedema and leg lymphedema.

This is highly unusual and even many lymphedema experts are unaware this can occur.

If we start with the premise that some people are born with an already “at risk” lymph system and then understand the exact mechanisms of the lymphatic system response to trauma and the changes within the lymph nodes, then it becomes clear that this is in fact a possibility.


I need to remind readers that I am not a medical professional nor have I ever had formal medical training and/or education. What is presented is a combination of my fifty-five years of living with lymphedema and from the research that I have undertaken.


Pat

Effect of lymphedema on the recovery of fractures

J Orthop Sci. 2007 Nov;
Arslan H, Uludağ A, Kapukaya A, Gezici A, Bekler HI, Ketani A. Department of Orthopedic and Trauma Surgery, University of Dicle, School of Medicine, Diyarbakir, Turkey.

BACKGROUND: Lymphedema delays the healing of any wound by negatively affecting its inflammatory period. Whether it affects bone healing in a similar negative manner is unknown. Therefore, we experimentally investigated the effect of lymphedema on fracture recovery.

METHODS: We used thirty 200- to 250-g Sprague-Dawley rats for the experiment. The rats were randomly divided into two groups of 15 rats each for the experimental lymphedema and control groups. Lymphedema development was confirmed by measuring the circumference and diameter of the extremities together with lymphoscintigraphy. Twenty days after the development of lymphedema, a fracture model was created in both groups in the right tibia with mid-diaphyseal osteotomy and fixing with an intramedullary Kirschner wire. After 6 weeks, all rats were sacrificed and the callus tissue that formed along the osteotomy was compared between groups with respect to radiographic, histological, and biomechanical characteristics.

RESULTS: The three-point bending test yielded an average stiffness value of 1227 N/mm (n = 6) in the control group and 284 N/mm (n = 7) in the experimental lymphedema group (P <>

CONCLUSIONS: Lymphedema negatively affected bone healing in rats. However, the mechanism of this negative effect and its occurrence in humans are still unknown. Further experimental and clinical studies are needed to support and extend our findings.

Springerlink

The healing of tibial fracture and response of the local lymphatic system

Szczesny G, Olszewski WL, Gewartowska M, Zaleska M, Górecki A. Department of Surgical Research and Transplantology, Medical Research Centre, Polish Academy of Sciences, Warsaw, Poland. g.szczesny@cmdik.pan.pl

BACKGROUND: Damage of tissues by mechanical injury and inflammation is followed by reaction of the regional lymphoid tissue, lymphatics, and lymph nodes. In our previous lymphoscintigraphic studies, we showed that closed fractures of a lower limb cause reaction of the local lymphoid tissue. There was dilation of lymphatics draining the site of the fracture and enlargement of inguinal lymph nodes. These changes persisted even after clinical healing of the fracture. In the long-lasting nonhealing fractures, the lymphoscintigraphic pictures were different. The draining lymphatics became obliterated, and the lymph nodes disappeared.


METHODS: In this study, we tried to correlate the lymphoscintigraphic images, reflecting the immune events at the fracture site, with the immunohistochemical observations of the biopsy specimens obtained during corrective operations from the healing and nonhealing fracture gaps. Thirty-eight patients with closed fracture of the tibia without traumatic skin changes were studied.

RESULTS: We confirmed that closed tibial fracture evokes response of the regional lymphatic system. Normal fracture healing with immune cell infiltrates and foci of ossification was accompanied by dilated lymphatics and enlarged lymph nodes. Prolonged nonhealing fracture with lack of cellular reaction in the gap proceeded with decreased mass of lymph nodes.


CONCLUSION: This study provides evidence for existence of a functional axis between wound of bone and surrounding soft tissue and the local lymphatic (immune) system. We hypothesize that the fast healing is regulated by influx into the wound of lymph node regulatory cells, whereas prolonged healing causes gradual exhaustion of the regional lymph node functional elements, and reciprocally impairment in sending regulatory cells to the fracture gap.


Journal of Trauma

Limb lymph node response to bone fracture.

Lymphat Res Biol. 2004;
Szczesny G, Olszewski WL, Zaleska M. Department of Surgical Research and Transplantology, Medical Research Centre, Polish Academy of Sciences, 02-106 Warsaw, Poland.
g.szczesny@cmdik.pan.pl

In previous clinical studies, dilation of afferent lymphatics and enlargement of inguinal lymph nodes (LN) were observed in lymphoscintigrams from patients with persistent posttraumatic edema of lower extremities after fractures and trauma of soft tissues. In this study, changes in rat popliteal and iliac lymph nodes draining lymph from the site of tibial fracture and adjacent soft tissue injury were investigated. The observed parameters were lymph node weight, cell number, phenotype frequency, cell cytokine expression, and reactivity to mitogens. The key observations included: a) increase in the weight and total cell number of the lymph nodes; b) increased autotransformation rate and responsiveness of lymph node cells to mitogen; c) decreased frequency of ED1 macrophages and activated OX8 cytotoxic cells in flow cytometry analysis; d) high expression of OX6 class II-positive, OX7 (stem cells), OX62 (migrating dendritic cells), ED1 (macrophages), and OX12 (B cells) on immunohistochemical sections of LNs with some few HIS48 (granulocytes); e) high expression of NOS3 and TGF beta by lymph node lymphocytes and endothelial cells.


In summary, local lymph nodes reacted to internal wounds, such as bone fracture and injury to adjacent tissues, through mobilization of cells from the blood circulation, along with activation of cellular subsets. The molecular mechanism that provides the signal for this reaction remains unknown. The absence of major changes in the frequency of lymph node cell subpopulations indicates that lymph nodes are constitutively prepared for influx of antigens from damaged tissues and react only with increase in cell number and cell activation. The nature of the reaction, including lack of immunization against autoantigens, remains unclear. Further elucidation will require studies on the mechanism of cross-tolerance to self-antigens during wound healing.


Liebertonline

Lymphedema of the Hand and Forearm Following Fracture of the Distal Radius

By David A. Kasper, DO, MBA; Menachem M. Meller, MD, PhD ORTHOPEDICS 2008; 31:172

February 2008

Lymphedema of the hand following a fracture of the distal radius is a rare complication resulting from abnormal protein-rich fluid accumulation in the affected area. Although lymphedema affects approximately 2.5 million Americans and frequently is associated with breast cancer treatment, its occurrence in the context of a commonplace injury to the wrist is virtually nonexistent.1

The etiology of lymphedema development following fracture care is poorly understood and has been attributed to psychogenic causes. Only one case of lymphedema following a Colles fracture has been reported in the literature.2 In that report, the patient was a 42-year old man who presented with lymphedema after a fall while accidentally being pulled by a chain. After closed reduction of the fracture and immobilization, the patient reported intense pain without swelling. Immediately after removal of the patient’s final cast, his hand began to swell, and he underwent intense physiotherapy, numerous sympathetic nerve blocks, and hospitalization with no improvement. The authors suggested the pathogenesis of the patient’s lymphedema after his fracture was self-induced and psychogenic in nature.


This article presents a case of Colles fracture complicated by nonpitting edema in a 62-year-old woman in whom psychogenic causes were not identified.

Case Report

A 62-year-old right hand-dominant woman fell down a few steps at work onto her outstretched right hand. Evaluation in the emergency room indicated a fracture of the distal radius, and the patient underwent closed reduction (Figure 1) under general anesthesia without a tourniquet.
This resulted in excellent restoration of the skeletal alignment. She was placed in a well-padded short arm cast.

At a routine follow-up visit 10 days later, the patient had complete loss of position, with the fracture reverting to the presurgical misalignment sustained immediately following the injury. She subsequently underwent open reduction and internal fixation using a dorsal plate. Both the surgery and postoperative course were uneventful.


The patient’s history included controlled hypertension, mitral valve prolapse, gastroesophageal reflux disease, rheumatic fever, scarlet fever, and a prior arthroscopic knee procedure. She reported no prior malignancies, and she was compliant with routine general medical care.
Psychological profiling was normal.

Following cast removal, the patient began occupational and physical therapy. Two months postoperatively, the swelling persisted, and she developed increasing asymmetry. She also had progressive nonpitting edema. The patient reported having no pain, hypersensitivity, or other symptoms. She also reported she did not develop any other illnesses or malignancies during this time.


The patient underwent an extensive workup that included electrodiagnostic studies and radiographs of the cervical spine, right shoulder, and right wrist (Figure 2). Computed tomography and magnetic resonance imaging revealed prominent edema adjacent to the capsule (Figure 3). An intravenous Doppler study ruled out deep vein thrombosis of the right upper extremity. A Duplex arterial scan and technetium bone scan revealed no pathological findings other than the fractured wrist.

Her fracture healed satisfactorily without additional loss of position. However, the function of her right hand was limited by the edema (Figure 4). Traditional treatments, such as a Jobst gauntlet (BSN-Jobst, Inc, Charlotte, North Carolina), Kinesio taping (Kinesio, Albuquerque, New Mexico), massage, elevation, and Isotoner gloves (Totes Isotoner Corp, Cincinnati, Ohio) supplemented by home exercises failed to relieve her symptoms.

Treatment subsequently was prescribed with the NormaTec PCD (pneumatic compression device; NormaTec, Newton Center, Massachusetts), and the patient initially used it at home for 4 hours daily. Within 2 weeks, her massive forearm edema dramatically diminished, and her wrist and hand motion normalized. She was able to bring her fingertips down to the proximal palmar crease with good grip, pinch, and opposition.

To inhibit the recurrence of the edema and hand stiffness, the patient has continued to use the device at home approximately 1 hour per week. She requires no compression garments and has not had any episodes of cellulitis (Figure 5).

Discussion

Although lymphedema is a common and severely disabling medical condition, it has not been described following orthopedic injuries such as a Colles fracture. The only previously published case report describing this injury combination attributed the lymphedema to psychogenic causes.2 In our patient, psychogenic causes were not identified.

Lymphedema results when the lymphatic volume in tissue exceeds the lymphatic transport system’s capabilities to clear the fluid. Increased hydrostatic pressure or decreased plasma oncotic pressure creates gradients across the capillary membranes, which causes the excess fluid to spill and accumulate in the interstitial space. Possible causes of this excess fluid production include local inflammation, surgery, infection, cancer, lymphatic obstruction (ie, due to scarring), and trauma.3 Although all body tissues are bathed in interstitial fluid, the lymph circulation still remains a complex, dynamic, and incompletely understood process.4

Lymphedema can be classified into two types: primary and secondary. Primary lymphedema is associated with hypoplastic, hyperplastic, missing, or impaired lymph vessels. Other presentations are classified further by age of onset. However, causes of primary lymphedema are generally unknown and cannot be linked to any specific traumatic event. The most common cause of primary lymphedema is lymphangiodysplasia.

Secondary lymphedema can be attributed to trauma to the lymph nodes or the lymphatic vessels themselves. Secondary lymphedema frequently is seen in surgical patients and is attributed to lymphatic obstruction.3 Speculations suggest secondary lymphedema associated with trauma is a consequence of an infectious or inflammatory process.3

Mechanical injury of the soft tissues and bones of the extremities usually is followed by edema distal to the site and at the site itself but not proximal to it. Patients usually present with a sensation of fullness and pain in the affected area, induration, edema, hyperkeratosis, and xerosis. Functional limitations include decreased range of motion, joint inflexibility, decreased mobility (if the lower limb is affected), and decreased activities of daily living (eg, grooming and dressing).3

For several decades, treatments to relieve lymphedema and traumatic or postoperative edema included manual massage, gradient compression stockings and sleeves, bandaging, taping, and pneumatic compression devices previously referred to as lymphedema pumps. All of these treatments used external compression, but none produced consistently good clinical outcomes.

Additionally, these treatments used static compression strategies, with compression applied and held constant for varying lengths of time. Most of the lymphedema pumps were poorly bioengineered, and their designs lacked understanding of the optimum parameters for noninvasive compression.

Recently, the concept of pneumatic medicine was developed to more clearly characterize and advance the science of external compression strategies. As defined by Avery et al,5 pneumatic medicine is the use of noninvasive, dynamic compression to treat the array of peripheral vascular disorders, including arterial insufficiency, chronic wounds, venous insufficiency, and lymphedema.

The NormaTec PCD uses a multi-cell sleeve or boot that is placed on the affected limb and pneumatically inflated and deflated via a unique Peristalic Pulse dynamic compression strategy. The patented Peristalic Pulse pneumatic waveform consists of a “pulse, gradient hold, release” compression cycle, simulating normal physiology. It incorporates three major physiological concepts: dynamic pulsing compression as seen in the muscle pump of a normal limb, directionality of flow similar to the venous and lymphatic one-way valves, and the effective movement of fluids created by peristalsis. The parameters of the NormaTec PCD are programmed by the physician, and the patient then uses the device independently at home.
A full functional outcome for our patient, who had chronic, clinically significant symptoms, was achieved in a brief period of time after numerous other treatments failed. The Peristalic Pulse compression strategy dynamically decongested the edematous tissues, and her hand and wrist range of motion improved markedly. Our patient has continued to use the device approximately 1 hour per week as maintenance therapy to prevent the return of edema and upper extremity stiffness. No compression garment is required, and compliance with the treatment program has been excellent.


A pathological anomaly that may have been a causative agent in our patient’s proximal edema following reduction of her Colles fracture is complex regional pain syndrome. According to the literature, the incidence of patients with Colles fractures who develop complex regional pain syndrome, albeit controversial, ranges between 2% and 37%.6 Although the pathogenesis is poorly understood, complex regional pain syndrome commonly is triggered by minor injuries such as fractures, crush injuries, peripheral nerve injuries, and other precipitating events that involve abnormal sympathetic nervous system activity.

Complex regional pain syndrome is characterized by pain and tenderness that is described as burning or aching in nature and usually occurring at a distal extremity. Patients with complex regional pain syndrome may develop rapid bony demineralization, trophic skin changes, and vasomotor instability that also are disproportionate to the underlying injury.


Complex regional pain syndrome progresses through three clinical phases. The first phase is characterized by an intense burning pain, edema, warmth, and tenderness of a distal extremity, especially noted around the joints. The joints become stiff, and pain is reproduced on passive and active motion of the joint. During the second phase (3 to 6 months), the patient’s skin becomes thin, cool, and shiny. In the third phase (another 3 to 6 months), the skin becomes atrophic and dry, with progression to flexion contractures and palmar fibromatosis.3

To aid in the diagnosis of complex regional pain syndrome, plain radiographs of patients with fractures may exhibit spotty rarefaction (Sudeck atrophy). Other tests used to substantiate this diagnosis include thermography, bone scan, and sympathetic blockade.

The key component to successful conservative treatment is early diagnosis within 6 to 8 weeks. Conservative treatment modalities include heat, elevation, and desensitization. Chronic disability occurs when the diagnosis and subsequent treatment is delayed. However, some authors have suggested there is no correlation among age, adequacy or number of reductions, or severity of fracture in patients who present with complex regional pain syndrome.3 In our patient, we ruled out complex regional pain syndrome because electromyography, nerve conduction study, radiographs, intravenous Doppler study, duplex arterial scan, and technetium bone scan revealed no pathologic findings other than the fractured wrist.

Some patients present with this syndrome after age 40 years, with the highest incidence in the sixth decade of life. Some patients also present with this anomaly after requiring repeated fracture reductions. Itzchaki et al2 suggested there may be a psychogenic component to this syndrome. Emotional instability was identified in one third of patients with this syndrome.2
Other causes of lymphedema were evaluated extensively in our patient. Local, regional, and metastatic causes such as breast cancer and Pancoast tumor were ruled out as were mechanical dysfunctions such as thoracic outlet syndrome and Milroy disease. Neurological involvement also was ruled out based on normal electroencephalographic readings and nonpathological clinical and physical findings.


The surgical procedure in our patient was uncomplicated and thus lymphedema secondary to any vascular injury was ruled out. Questions that need to be addressed are whether the lymphedema was locally or systemically mediated, or whether the onset of the fracture induced an avascular anastomosis that led to the lymphedema. Our conclusions led us to believe the development of lymphedema of the distal radius following Colles fracture was idiopathic in our patient.

References

Norton S. Managing lymphedema. Advance. 2000; 11(10):1-6. Itzchaki M, Ben-Hur N, Ashur H. Lymphedema of the hand following a fracture of the distal radius. Int Surg. 1978; 63(1):29-30. Patel AT. Lymphedema. In: Frontera WR, Silver JK, eds. Essentials of Physical Medicine and Rehabilitation. 1st ed. Philadelphia, PA: Hanley and Belfus; 2002:575-577. St Louis JD, McCann RL. Lymphatic System. In: Townsend CM, ed. Sabiston Textbook of Surgery. 16th ed. Philadelphia, PA: WB Saunders Co; 2001:1446-1450. Avery KB, Solomon AD, Weber RB, Jacobs LF. Treatment of congenital lymphoedema with sequential intermittent pneumatic compression therapy. The Foot. 2000; 10(4):210-215. Stern PJ, Derr RG. Non-osseous complications following distal radius fractures. Iowa Orthop J. 1993; 13:63-69. Authors Drs Kasper and Meller are from the Department of Orthopedic Surgery, Veterans Hospital, University of Pennsylvania, Philadelphia, Pennsylvania.

Drs Kasper and Meller have no relevant financial relationships to disclose.

Correspondence should be addressed to: Menachem M. Meller, MD, PhD, Department of Orthopedic Surgery, Veterans Hospital, University of Pennsylvania, 424 Stemmler Hall, Philadelphia, PA 19104-6081.

OrthoSuperSite

Tuesday, November 04, 2008

Supplemental surgical treatment to end stage (stage IV-V) of chronic lymphedema.

Normally, I don't post articles suggesting surgeries on lymphedema. Due to the complications that have been experienced by lymphedema patients, I am strongly opposed to what we call a debulking surgery.

However, this study is interesting for a couple reason. The surgery appears to be one I have never heard of - Auchincloss-Homan's procedure and references to stages IV-V which they call "end-Stage" lymphedema.

I research those items to see what I can find and of course will post the info.

In the meantime, here is the abstract.

Supplemental surgical treatment to end stage (stage IV-V) of chronic lymphedema.

Int Angiol. 2008 Oct

Lee BB, Kim YW, Kim DI, Hwang JH, Laredo J, Neville R.
Department of Surgery, Georgetown University, Washington DC, USA.

AIM: When the lymphedema reaches to its end stages, the complex decongestive therapy (CDT) and/or compression therapy become less effective and increased risk of systemic/general sepsis to become potentially life threatening condition.

METHODS: To improve its clinical management, excisional surgery was performed on 22 patients for their 33 limbs in the end stage of lymphedema as a supplemental therapy, and its efficacy was retrospectively analyzed. Diagnosis was made by radionuclide lymphoscintigraphy and basic laboratory studies (e.g. ultrasonography, magnetic resonance imaging). Twenty-two patients (mean age: 46 years; 3 male and 19 female; 5 primary and 17 secondary) submitted to the excisional surgery on 33 limbs (unilateral: 11; bilateral: 22). Surgery was indicated by further progression of the disease with recurrent sepsis despite adequate antibiotics therapy. A modified Auchincloss-Homan's operation was used to excise grotesquely disfigured soft tissue with advanced dermato-lipo-fibrosclerotic change. The normal limb contour was re-established to allow proper postoperative therapy. Postoperative CDT and compression therapy were mandatorily implemented in all cases.

RESULTS: A postoperative assessment of the treatment results, at 12 months showed an overall improvement in 28 of the 33 limbs: substantial improvement on the condition of limb function and quality of life (QOL), and local and/or systemic sepsis. Eighteen patients with good compliance to maintain the postoperative CDT showed much improved clinical results and QOL through the first interim assessment (24 months).

CONCLUSION: Excisional surgery at the end stage of lymphedema seems to provide substantial improvement of clinical condition and QOL only when mandated postoperative CDT/compression therapy is well kept.


PMID: 18974701 [PubMed - in process]

Tuesday, October 28, 2008

Irradiation of lymph nodes areas in breast cancer

Irradiation of lymph nodes areas in breast cancer

Cancer Radiother. 2008 Oct 16

Hennequin C, Romestaing P, Maylin C.
Service de cancérologie-radiothérapie, hôpital Saint-Louis, 1, avenue Claude-Vellefeaux, 75010 Paris, France.

Postoperative radiotherapy after breast surgery increases overall survival by decreasing the local relapse rate. The main site of relapse is the breast or the chest wall. The value of irradiation of the nodal basins is still discussed. It must be emphasized that nodal areas were systematically irradiated in the postmastectomy randomized trials demonstrating a benefit in survival for adjuvant radiotherapy. Axillary relapses are infrequent in case of complete axillary dissection; complementary irradiation could be proposed if the axillary dissection is incomplete or if there is a massive pathologic involvement of the axilla.

Its main complication is lymphoedema, which remains the most frequent sequellae of the treatment of breast cancer. Supraclavicular irradiation is logical in case of axillary involvement. Internal mammary nodes are involved in 20 to 40% of the patients, depending of the axillary involvement and location of the tumor inside the breast. Irradiation of the internal mammary basin is difficult: optimal dosimetry required a CT-based simulation. Its benefit remains to be proved. Internal mammary nodes irradiation probably increased cardiovascular mortality. Sophisticated techniques are needed to spare the heart from irradiation. Two large randomized trials (French group, European Organization for Research and Treatment of Cancer [EORTC]) are currently evaluating the possible benefit of internal mammary irradiation.

PMID: 18951822 [PubMed - as supplied by publisher]



Sunday, October 26, 2008

Treatment of upper limb lymphedema with combination of liposuction, myocutaneous flap transfer, and lymph-fascia grafting

Treatment of upper limb lymphedema with combination of liposuction, myocutaneous flap transfer, and lymph-fascia grafting
Microsurgery. 2008 Oct 22

Qi F, Gu J, Shi Y, Yang Y.
Department of Plastic and Reconstructive Surgery, Zhongshan Hospital, Fudan University, Shanghai, China.

Treatment of obstructive extremity lymphedema remains a challenge in reconstructive surgery, since none of the varieties of procedures have been demonstrated a reliable resolution for the lymphedema. In this report, we present the preliminary results of treatment of severe upper extremity lymphedema with combined liposuction, latissimus myocutaneous flap transfer, and lymph-fascia grafting in 11 patients. All patients had histories of radical mastectomy, irradiation therapy for breast cancer, and frequent onsets of erysipelas. Postoperative measurements in an average of 26 months follow up showed that significant decrease of circumferences of the arms on all levels at surgery side were achieved. The onsets of erysipelas were also reduced. There was no chronic lymphedema found in the donor leg after harvest of the lymph-fascia graft. The results suggest the strategy of liposuction, latissimus myocutaneous flap transfer, and lymph-fascia grafting may provide a useful method for treatment of the chronic upper extremity lymphedema with severe axillary scar contracture.

Wiley InterScience

Tuesday, August 12, 2008

Lymphedema and Pregnancy

Lymphedema and Pregnancy
Introduction

For young women with lymphedema, especially primary lymphedema, the thought of having babies and what might happen is a scary topic with many many questions.

They want to know if their lymphedema will worsen as a result of pregnancy; if they have hereditary lymphedema, can they have a baby and can they pass it on to their children.

While lymphedema can increase your risk of some complications and while you may have to take extra steps to stay healthy, the good news is that you can have a baby, that most women who have lymphedema do not experience a worsening of their lymhpedema.


Will you pass primary lymphedema to your baby?

As you read the following posts, you will find that most have not passed it on. My own grandmother who had primary lymphedema had in children and only one of them had lymphedema. In fact, remember, I am just a layperson, but from my own observations and from the experience of so many many mothers who have lymphedema I absolutely challenge the statistic that if you have primary lymphedema you have a 50% chance of pasing it on. There is no evidence or clinical studies that I know of to substantiate that claim.

See also: Arm and Leg Swelling In a Baby

Here are some tips that I believe will greatly help during your pregnancy.

Tips for Pregnancy and Lymphedema

Diet

A good balance and nutritionl diet is an absolute must, whether or not you have lymphedema. It may be even more important for those with LE.

Internal Links


The Lymphedema Diet
Vitamins
Vitamin Glossary
Minerals, Amino Acids
Healthcastle
Nutritional Tips during Pregnancy

Exercise


It is a well established fact that exercise is necessary to be healthy. The same holds true for keeping in shape during pregnancy.

The idea is not to simply stop exercising because you are pregnant and have lymphedema, but rather to adjust the type of exercises you do. Take a look at our page on exercises for some good ideas and programs on low impact, less strenuous types of exercises.

Exercises for Lymphedema
Exercise in Pregnancy

Weight control

Because excess weight complicate lymphedema, you’ll want to do all you can to avoid gaining weight that would be out of the normal range for your pregnancy. With lymphedema, you can suddently gain weight simply from the fluid accumulation. Should you experience a sudden weight gain, be sure to let your doctor know.

Skin Care

While skin care is critical all the time for lymphedema, it becomes even more important during pregnancy to help avoid possible infections and to help your skin avoid any possible complications.


You’ll want to also continue your program of healthy skin care for lymphedema patients in general.

Lymphedema Skin Care
Safe skin care during pregnancy

Bandaging

Continue your daily bandaging routine. This also can be a great help in control any possible increased swelling.

Compression bandages for lymphedema
Short stretch bandages for lymphedema

Pneumatic Compression Pumps


Because of the documented possibilities of genital lymphedema,I oppose the use of these devices for leg lymphedema and feel even stronger about it during pregnancy.

Why Compression Pumps cause Complications with Lymphedema

Compression garments


There is no documented reason why you should not be able to continue wearing your compression garments, especially during the early phase of your pregnancy.
However, be sure to check with you doctor and your lymphedema therapist.

Obviously, this is going to be difficult, if not impossible during the later stages of your pregnancy,, so you may have to make adaptations.

An alternative to the full waist high garment would be a thigh high. This will help the legs.

Compression garments stockings for lymphedema

Self Massage

Self lymphatic massage can be a great help in continuing to manage your lymphedema and in helping keep the swelling down.

Manual Lymph Drainage

I urge you to continue this if you are already doing so and if you are not to seriously think about seeing a certified lymphedema therapist to designa treatment program in conjuntion with your pregnancy.

Interal Link

Manual Lymphatic Drainage

Rest

As your pregnancy progresses you will find that you tire more quickly Be kind to yourself and allow time for extra rest. This may also be necessary should you have a significant amount of increased swelling.

Possible Complications

To remain as healthy as possible you'll need to have an understanding of the possible complications of pregnancy and of lymphedema. Here are a couple pages of information that should help.

Complications of Lymphedema
Pregnancy Complications

Preeclampsia

Preclampsia, previously known as toxemia seems to be the biggest risk during pregnancy. Infact, it is estimate that as many as 14% of pregnancies involve preeclampsia. It can happen during the second half of pregnancy (20th week) or in the third trimester.

This condition is characterized by a sudden and/or rapid increase in swelling, blood pressure, increased protein in your urine. Other symptoms include upper abdominal pain, usually under the ribs on the right side, dizziness, changes in vision, including temporary loss of vision, blurred vision or light sensitivity, severe headaches, decreased urine output.

This also seems to be the number one problem experienced by those with lymphedema.

Preeclampsia Foundation
Pregnancy, Preeclampsia

Hypertension

Hypertension is one of the most common complications of pregnancy so you will want to familiarize yourself with this possible complication. It is estimate that hypertension complicates aproximately 3 - 5% of pregnancies.

Related high blood pressure disorders during pregnancy include:

Gestational hypertension.

Women with gestational hypertension have high blood pressure, but no excess protein in their urine. Some women with gestational hypertension eventually develop preeclampsia.

Chronic hypertension.

Chronic hypertension is high blood pressure that appears before 20 weeks of pregnancy or lasts more than 12 weeks after delivery. Often, chronic hypertension was present — but not detected — before pregnancy.

Preeclampsia superimposed on chronic hypertension. This term describes women who have chronic high blood pressure before pregnancy and then develop worsening high blood pressure and protein in the urine during pregnancy.

Having lymphedema, whether primary or secondary, does not automatically mean you are more susceptible to this condition.

American Pregnancy Association
Hypertension and Pregnancy

Gestational Diabetes

This is a type of diabetes that some women develop during pregnancy. Between 2 and 7 percent of expectant mothers develop this condition, making it one of the most common health problems of pregnancy.

Gestational Diabetes

Infections

Lymphedema patients are already at a greater risk of infectons due to the immunocompromised condition of the lymphedema leg, with pregnancy and the effects on the immune system you will want to keep an eye on this and familiarize yourself with possible infections, what to look for, how to prevent them and how to treat them.

See also:

Infections Associated with Lymphedema
Immune System and Pregnancy

Experience of Lymphedema People Members

Lazylefty
July 9, 2007

Hi ann. I'm 29 with a 2 yr old and have had secondary LE for 4 yrs now. My drs were concerned with clotting during pregnancy in my affected leg. I'm happy to say that my pregnancy went great with very little issues. I made sure to rest off of my feet as much as possible. I put a stool under my desk at work to keep my feet up. I also did my low impact exercises to help my leg circulation. the hardest part for me was once I was in the hospital I took my stocking off because of the over night stay and with all the fluid they were pumping in me it made the swelling worse in my leg. I couldn't get my stocking on the next day so lucky for me I was in bed all day and not on my feet anyway. I would suggest you wear your stockings, do your massage as often as you can and you'll most likely be fine! good luck!!
Stacy

Ann
July 10, 2007
I have two daughters and did not have any problems with my LE while pregnant. (I have primary in both legs). If you have primary LE you might want to have genetic counseling. My youngest daughter has developed LE i her legs (she's 17) and I had no idea when I was having children that my LE could be inherited. I can't imagine doing anything differently, though I wish that I could have spared her.
Stacy


Silkie
August 1, 2005
Hi ya Nikkid
I am sure if i had known i had lymph when i had my children i would have asked the same question.


I can tell you I did have lymph not diagnosed I gained weight hun my ankles swelled and feet got bigger, had a lot of what they said was fluid retention.

They were worried i had toximia because of my ankles (it was the lymph I didn’t know there was lymph at the time). I had good births and I worked hard with the “fluid as they called it and did reduce took a while couple of years never got back to my original size. I got pregnant again more or less the same type of pregnancy and I had my daughter third and last not a problem except for the weight and swelling, thank God non show signs of lymph but I was terrible conscious of their weight.

If i had known about the lymph I would still have had my children life is about living and loving the lymph and the difficulties we all face are secondary to living our lives


huggggggggg silks xxxxxxxxxxxxxxxxxxxxxx

Tamie
February 20, 2004

During my first pregnancy at 19 years old my hands started to swell. I saw a doctor who said the swelling wasn't that serious, but be sure to take a sit down job. I was working in retail at the time and often as a cashier which standing in one spot all day makes the condition worse!

When I had my 1st child at the age of 20 and was recovering from a c-section the doctors were concerned with my swollen leg that I might get a blood clot in it so I had to wear compression hose during my 2 week ordeal/recovery.
Tamie


Pregnancy and Lymphedema

Several months ago one of our readers asked whether pregnancy caused lymphedema to get worse. I reviewed the published literature and, as is too often the case, found very little published information. In addition, what information was available was based on very limited numbers of patients. To gain additional understanding about pregnancy and lymphedema, I created a pregnancy survey for our readers. 13 women have responded to that survey and I am including a summary of their responses and insights.

12 of the 13 women had primary lymphedema. The average age of onset was 10 years of age and the range was from 1 to 16 years of age. 1 patient had breast cancer and a mastectomy and developed lymphedema during her first pregnancy 9 years later.

Of the 12 patients with primary lymphedema, 9 of 12 (75%) had the onset of lymphedema or developed worse lymphedema during their pregnancy. Most of these women found that the lymphedema got worse in the final months of their pregnancy. 2 women reported having persistent lymphedema after delivery.

8 women reported second pregnancies and 5 of the 8 (63%) had worsening of their lymphedema and all reported that the lymphedema was worse with the second pregnancy. All 3 women who did not report worsening of their lymphedema had miscarriages that occurred between 3 and 5 months of gestation.

4 women reported having third pregnancies and 3 of the 4 had worsening lymphedema with the pregnancy and all reported that the lymphedema became progressively worse with each pregnancy. The remaining woman had a miscarriage.

One woman reported a 4th pregnancy and had worsening lymphedema with the 4th pregnancy but that the lymphedema got better after the delivery of her child.

The one respondent with secondary lymphedema commented that she had been free of lymphedema for 9 years after her mastectomy but developed lymphedema in her hand and forearm during her first pregnancy.

The only women not reporting worsening of their lymphedema during their second and subsequent pregnancies had miscarriages. Miscarriages occur in about 10% of pregnancies so it is notable that so many of the women responding to this survey reported miscarriages. Please keep in mind that a small number of women responded to this survey and any results represent the bias of any small sample. However, it is also possible that the incidence of miscarriages is higher than the expected in women with lymphedema. I will continue to report on additional findings of this survey as we get more information.

Many of the comments made by the women provided interesting insight into the problem of lymphedema during pregnancy so I have included a sample of these comments below.

Comments:

“I am currently in my eighth month of pregnancy and have doubled the size of my left leg. Prior to the pregnancy, I had not swelling in my right leg. Now in my eighth month of pregnancy, my right leg is swelling. I am hoping the swelling in my right leg will go away after the baby is born.”

“By 11-12 weeks of pregnancy, my leg was fuller and growing uncomfortable. I was able to continue working full time as a nurse until the 20th week of pregnancy. At that point my leg was heavy and uncomfortable. I was comfortable, however, if I was lying down. During the pregnancy, I gained over 60 lbs., I was very congested in my entire body. I remember having to put my left leg and foot under cold water to reduce the discomfort. I was unable to wear any shoes other than ballet slippers, and could only do minimal walking around the house. After my daughter was born, one to two weeks after her deliver, my leg returned to essentially a pre-pregnancy baseline. My
leg improved as I took off the weight gain of fat that naturally occurs with pregnancy. ”

“I am currently at the last stage of my third pregnancy, and the swelling is once again more pronounced than in previous months. I tend to be lazier about the stockings this time, so my swelling could probably be better.”

“Thank you for posting this survey, I would have enjoyed having some preview of the effects of childbirth on lymphedema. Overall, pregnancy was a temporary setback, which is an important consideration. However, I was still uncertain enough not to attempt my good luck with a second pregnancy. Who knows what the outcome would be, especially after age 35. My personal experience with this condition has led me to believe that insect bites are far worse for my leg. If I get bites on my left leg, my leg gets worse, and doesn't want to return to baseline. It's as if I “loose ground” whenever this happens. The increase with pregnancy, although very substantial, was reversible. It seemed to me to be in indication of lymphatic system overload, rather than tissue scaring or damage. I did notice that as my weight returned to normal, my leg kept improving.”

Tony Reid MD Ph.D.

Acknowledgment
Peninsula Medical, Dr. Reid's Corner

Pregnancy and Lymphedema: Follow up

Last year I presented data on the relationship between pregnancy and lymphedema. Since that time more women have responded to the survey and I presented the update of that survey at the Lymphedema conference held in Dallas, Texas sponsored by Healthtronix.

This survey was prompted by several questions that were sent to me asking whether pregnancy worsens lymphedema. For example, a woman had primary lymphedema and was considering an abortion because she was very fearful of her lymphedema getting worse. She already had a bad case of lymphedema and felt that if it got much worse she would no be able to function. There was no published data to help answer these questions and so I posted a survey on our web site to help find some answers to this question.


First, I want to say that the results of the survey are limited by a number of factors. The number of women answering this survey, while growing, is still relatively small. In addition, this is not a random sample of all women with primary lymphedema who have had a pregnancy and effective treatment may change the outcome. This survey only documents the experience of the women who have responded. However, I appreciate the fact that these women have taken the time and effort to answer these questions and I hope that this project will continue to develop and provide additional information that is helpful to women facing this problem.

33 women responded to the survey. Of these, 26 had primary lymphedema and this survey will focus on those 26 responses. Most women with secondary lymphedema have it as a result of treatment for breast cancerbreast cancer. As a result, the majority of these women are past their child bearing years. In addition, the treatment, especially chemotherapy, generally causes infertility. So, most of the women who have lymphedema during their child bearing years have primary lymphedema. I will analyze the results of the women with secondary lymphedema separately. Since there are only a few responses, the data is still limited.

The average age of onset of lymphedema in this group of women with primary lymphedema was 10.7 years but the range of responses was very wide. Some women developed lymphedema at birth while others developed lymphedema in their late teens or twenties.

Of the 26 women with primary lymphedema who responded to this survey, 12 of 26 (46%) reported worsening of lymphedema with the first pregnancy. Of the 12 who had worsening of lymphedema during pregnancy, 7 reported that the lymphedema returned to baseline after delivery so that 5 of 26 (19%) reported persistent lymphedema after pregnancy. However, among the women who improved after delivery, 2 of these women subsequently had worsening of lymphedema within a year. As a result, 7 of 26 (27%) reported lymphedema that was worse following their first pregnancy. Here are several comments from these women.

“After delivery my leg went back to it's prior size before becoming pregnant. However, after 7 months my leg again became swollen and progressively got worse.”


“In my second trimester my ankles began to swell and the doctor assumed it was all normal. After the delivery of my child the swelling in my right leg / ankle went away but the swelling in my left leg continued.”

These results suggest that about half of the women with primary lymphedema experienced worsening of lymphedema during their pregnancy. Among the women who reported that their lymphedema worsened with pregnancy, about half of these women reported improvement after delivery of the baby. As a result about 27% (7/26), of the women with primary lymphedema experienced persistent worsening of the lymphedema with pregnancy.

Some of these women had additional pregnancies and I will present the analysis of the results in the subsequent edition of eNews.

Sincerely,
Tony Reid MD, Ph.D

Acknowledgment
Peninsula Medical, Dr. Reid's Corner

Questions on Pregnancy and Lymphedema
National Lymphedema Network

Should I wear a sleeve or bandage while pregnant?

Q. I had a lumpectomy and node dissection 8-1/2 years ago and developed LE within a year of my surgery. I am very diligent in my self-care, i.e., self-MLD, compression sleeve every day, bandaging every night, professional MLD once a year. One question that no one has been able to answer for me is how pregnancy affects LE. One doctor told me that I shouldn't wear a sleeve OR bandage during pregnancy, while MLD therapists tell me I shouldn't alter my routine at all. (FYI: I m not pregnant right now.) I d really like to understand what will happen: can the LE become worse, and how best to continue treatment during pregnancy? Thanks!

A. There is no documented scientific evidence anywhere stating that compression garments should not be worn during pregnancy, or that MLD is contraindicated during pregnancy. Of course, modifications in the abdominal breathing/abdominal clearance would be made during pregnancy. In fact, for lymphedema of the legs, it is essential to maintain compression during pregnancy, to avoid worsening of the swelling from abdominal pressure on the great veins.

There is an increase in total blood volume during pregnancy to support the fetus. This should not have a direct effect on the lymphedema in your upper extremity. My suggestion would be to continue to follow your usual lymphedema management program, including wearing your compression garments and bandaging at night if that has been your routine. I know that you are not pregnant now, but I hope that this information will allay your fears about your lymphedema worsening during pregnancy. You can safely continue to do your self-care program, perhaps with some modifications, if you do become pregnant.

Acknowledgment
National Lymphedema Network

Q: What is the percentage of children born with or who develop lymphedema when the mother has primary lymphedema?

A: Due to the inattention given lymphedema in the U.S., we do not have any concrete data identifying the number of people born with primary lymphedema. Here at the NLN, the number of calls we receive from young parents, concerned that their child will inherit the condition, is increasing. We do see, and talk to, a growing number of people who have multiple generations in their family affected by primary lymphedema, and some who have none at all. However, a number of my patients with primary lymphedema from both backgrounds have had very healthy, lymphedema-free babies who, so far, have not developed the condition. So, at this point, it's very difficult to say what the odds are. Marlys and Charles Witte, M.D.'s at the University of Arizona (Tel: 520-626-6118), are actively working with a number of families, trying to identify some genetic link and/or other correlations. Possible genetic links are also being studied in the Department of Human Genetics at the University of Pittsburgh, PA. For more info about the study, contact Kara Levinson, MS, at: 412-624-4657. Or visit their website at University of Pittsburg - lymphedema. This research data will greatly enhance our ability to forecast a child's susceptibility. If you do have a child with lymphedema: there is a new organization called “PLAN” (Primary Lymphedema Action Network), which focuses on families with young infants born with primary lymphedema.

Q: Are there concerns of permanent deterioration or worsening of a mother's condition if she has primary lymphedema and becomes pregnant (such as spreading to the other leg, additional risk of infection, etc.)?

A: It really depends on the overall condition of the mom. If she is healthy without any other medical problems, there should not be a problem. But it is very important that couples prepare themselves and realize the tremendous re-sponsibility. You'll need to increase your daily care, such as manual lymphatic drainage twice a day, wearing well fitted maternity panty hose (45-55 mm/hg) or, as some women do, wear an additional stocking to add compression. Avoid sodium and drink lots of fluids (water, tea, natural juices, etc). In regard to spreading to another limb - and if you are concerned, I would suggest doing a lymphoscintigraphy (contact the Witte's; see answer to question above) - a very sophisticated diagnostic tool used to visualize the lymphatics - prior to your pregnancy. Also, if you have a history of recurrent onset of lymphangitis in your leg, you will be at greater risk of recurrent infection during pregnancy as a result of increased weight/swelling and protein in the tissue. If severe enough, an infection could cause a miscarriage, so you will want to watch closely for signs and symptoms. The best advice: use common sense and practice meticulous self-care. If you are well, there is no reason that you cannot have a healthy, happy baby.

Q: What are the possible complications from a C-section vs. vaginal delivery and its relation to lymphedema?

A: Both procedures have their concerns. Any time an invasive procedure is performed on a patient with lymphedema, you want to be careful. Especially the woman who has swelling in the pelvic area and lower abdomen needs to make sure to take antibiotics just before, during and after the C-section. Vaginal delivery always has risk factors as well, especially for a woman who is in labor for many hours: usually there is more swelling in the pelvic region and leg(s) from pushing. But once the baby is born, swelling usually subsides in a matter of days.

Q: Is it safe to undergo Manual Lymphatic Drainage during pregnancy?

A: Not only is it safe, but it's extremely important to continue therapy. Your goal is to keep the leg(s) in its optimum condition. Do not forget to use lotion to keep the skin soft and supple. See a podiatrist educated in lymphedema just to make sure that you do not have any possible risk factors such as fungi, Athletes foot, callouses, etc., which could lead into infection. VERY IMPORTANT: Be sure to wear well-fitted high compression maternity stockings.

Additional tips for pregnancy: Educate your GYN and other involved doctors about lymphedema. Get plenty of rest, avoid stress when you can, follow the
18 STEPS TO PREVENTION, and if possible, shoot for winter time for your last trimester, when it's cool. Happy Pregnancy!

National Lymphedema Network

Primary lymphedema and pregnancy

Brunner U, Lachat M.
Departement Chirurgie, Hopital Universitaire, Zurich.


From a retrospective analysis of 15 female patients, it appears that primary lymphedema, reversible at first, tends to become irreversible during successive pregnancies. A remission takes place following the first and second pregnancy, and during a third pregnancy, an irreversible stage is reached.

PMID: 2626470 [PubMed - indexed for MEDLINE]

Suggestions for lymphedema and pregnancy

I was always taught that pregnancy was an indication FOR Manual Lymphatic Drainage and that is was great to have it during pregnancy, provided there were no other contra-indications present (or complications to be considered). As you are having it regularly anyway, it should not be a 'shock to the system'.

Some practitioners might choose to avoid the first tri-mester (but this is more to do with avoiding being associated should anything go amiss in the most vulnerable first three months, I think - although, of course, some people don't actually know they are pregnant until well into the three months anyway).


Hosiery - no reason not to wear it

In fact, anything that you can do (safely) - eg., wearing hosiery and having MLD during pregnancy that will help to keep oedema down has to be a good thing. The body naturally tends to retain fluid during pregnancy - many women experience swollen ankles, carpal tunnel syndrome etc as a natural complication of the extra fluid carried. It will likely make any lymphoedema a little more troublesome, so keeping hosiery on and staying with MLD could help to keep the balance.

If you are able, walking in water would be excellent as it is good for lymphoedema anyway and exercising in water while pregnant is fantastic. The water needs to be about the height of your boobs - don't overdo it, stop before your muscles get fatigued and wear some old, worn out compression hosiery while in the pool for an even greater effect.

The action of walking activates the calf muscles and the lymphatic system of the legs, the induced deeper breathing encourages lymphatic return and the water acts like an all over MLD massage, supporting the skin. You may find that you need to leave the pool to wee quite often!

It is important though, to stop before your muscles get tired. This avoids bringing too much extra circulation to the legs as that could lead to more oedema.


Your regular MLD practitioner would be the person to talk to - would they be happy?

From ULKymph Discussion Board - Author is Anne - who not only has lymphedema but is a Vodder Therapist as well ……..


Another members experience: I have had the L/O symptoms in both my lower legs since I was 12 years old although I think I was born with it.

Five years ago I had my beautiful daughter Ellie and although it was uncomfortable during the last few months as I was carrying extra weight my legs did not really suffer.

I am lucky in that my L/O is pretty mild compared to some sufferers, but I just made sure that at the end of the day (I was commuting to London for work) and whenever they started to ache, I would put my feet up and rest. I also made sure I wore my support stockings (Jobst I find are the best) all the time even during the summer when it was warm.

I dont know whether I have passed the gene onto her and I hope to God that I havent. I just try not to think about it but I would not be without her for the world.

I do notice that my legs do swell up more quickly than say they did 10 years ago but I dont believe that has anything to do with me being a mum and so long as you look after yourself and let your husband/partner spoil you rotten during those 9 months I am sure you will be fine.

Its worth talking to your doctor/specialist though to get a qualified opinion.


Lymphoedema and Pregnancy

By Professor Peter Mortimer, LSN Chief Medical Advisor and Dr Sahar Mansour, Consultant Clinical Geneticist, St. George's Hospital, London

Changes in a Normal Pregnancy

The cardiovascular system undergoes considerable changes during pregnancy with an increase in blood output from the heart by at least 50%. Blood vessels generally enlarge creating a relatively 'under filled' circulation and so to compensate, the kidneys try and conserve salt and water. This leads to fluid retention amounting to some 6-8 litres in the body. The dilution of the plasma proteins encourages fluid to leak from the blood vessels into the tissues. A fall in the threshold of the hormone that encourages a fluid diuresis maintains a fluid retention state. By the end of the pregnancy, 80% of healthy women will have some degree of oedema.
Very little is known about what happens to the lymphatic system during pregnancy. If blood vessels enlarge, i.e. relax, then by implication,
lymphatic vessels are likely to do the same, in which case they will not be as efficient at draining fluid. Normally there is sufficient reserve in lymphatic transport so that any increases in tissue fluid will be compensated for by increases in lymph drainage. If the lymph drainage is already working close to capacity because of a genetic or constitutional weakness in the lymphatic system (but not so severe as to have produced lymphoedema before), then the extra demands of pregnancy may be all that is needed to manifest swelling for the first time.

Other factors that potentially increase the risk of oedema during pregnancy are weight gain and a reduction in exercise levels.While fluid retention will increase weight, so will obesity. Lean women who eat to appetite gain as much as 1kg in the first 10 weeks and women with a tendency to obesity will gain much more. Such weight gain will probably have an adverse effect on lymph drainage, particularly in the legs. It is difficult to maintain exercise levels during pregnancy because of tiredness and the awkwardness the pregnancy brings to walking. Exercise is, of course, crucial for good lymph drainage in the legs.

Like other blood vessels, the veins in the leg tend to enlarge during pregnancy. Varicose veins often develop, which will result in a further filtration of fluid from the blood into the tissues of the leg and so make oedema worse.

Pre-eclampsia

Pre-eclampsia (used to be called toxaemia of pregnancy) is specific to pregnancy and manifests with hypertension (raised blood pressure), a leak of protein by the kidney, and oedema. The cause is not known, but the syndrome of pre-eclampsia usually develops from the mid-point in the pregnancy (20 weeks onwards), and resolves completely after delivery. Generalised oedema is an inconsistent feature. It may develop suddenly and is associated with accelerated weight gain (due to fluid retention). Although the ankles and feet will be the commonest site for the swelling due to the effects of gravity, oedema can occur anywhere in the body including the chest and the abdomen (ascites is free fluid in the abdominal cavity). The generalised nature of the oedema would suggest that the fault lies with the blood vessels leaking more fluid into the tissues rather than any failure of the lymphatic system, but nobody knows. As mentioned earlier, any such increase in tissue fluid will inevitably demand more of the lymphatic vessels to drain the fluid and any failure to do so will increase the oedema further.

Diuretics are best avoided in pregnancy because they result in an even greater 'under fill' of the blood circulation. Drugs called 'calcium channel blocking agents' are recommended for the raised blood pressure, but do tend to interfere with the working of lymphatic vessels and may increase ankle oedema.

Lymphoedema in Pregnancy

A major concern of any young female patient with lymphoedema is “What will happen to my lymphoedema if I become pregnant?” The answer is that it is likely to get worse because of the fluid retention, but it should be manageable and fully recover once the baby is born. The extra bodily fluid retained during the pregnancy will include the part of the body affected by the lymphoedema; so extra effort will be required to ensure that this extra fluid is drained by the local lymphatic system that is already failing. So if a leg is affected by lymphoedema, for example, then extra measures to control the swelling may be necessary. These measures may include longer periods of rest with the leg elevated, manual lymphatic drainage, or an additional compression garment. Not every woman with lymphoedema suffers any exacerbation of swelling during pregnancy. In many, the lymphoedema remains unaffected, and so what is described here is the worst case scenario.

There is no reason to believe pregnancy harms the lymphatic system, and so a full recovery would be expected following delivery. Nevertheless, as with returning to one's original weight and bodily shape, recovery of the lymphoedematous limb may take a bit of time and effort. Increasing levels of exercise and dieting may be necessary.

Genetics, Lymphoedema and Pregnancy

Primary lymphoedema is due to an underlying abnormality in the lymphatics. Although the swelling may not be present until later in life, the abnormality is probably present at birth. It is now recognised that there are some causes of primary lymphoedema that are inherited.

Therefore a woman (or man) with primary lymphoedema may have a child with the same condition.

Family History

The best indicator that there is a genetic cause of lymphoedema is the presence of other affected individuals in the family. The commonest way that primary lymphoedema is inherited is from parent to child. This mode of inheritance is called autosomal dominant inheritance. There are two copies of most genes. An autosomal dominant condition is due to an alteration, or 'spelling mistake', in one of the copies. The baby can inherit either the affected gene or the unaffected gene, so the risk to the offspring of inheriting an autosomal dominant condition is 1 in 2, or 50%. Some of the genetic causes of primary lymphoedema are well recognised and are described in more detail below.

Milroy's Disease

Milroy first described a large family with lymphoedema presenting at birth in 1892. It was clear from the family history that this condition was autosomal dominant, and therefore being transmitted from parent to child.Milroy's disease presents predominantly at birth with swelling of the lower limbs, usually the feet. The swelling can increase, or improve, or remain stable. Boys sometimes have extra fluid in the scrotum, but this rarely causes any problems. Milroy's disease is not usually associated with any other abnormalities.Most of the carriers of this condition have some swelling of the lower limbs, but it is recognised that some carriers of the condition are not affected, but may have affected offspring. The lymphoedema in Milroy's disease is due to a lack of lymphatic channels in the lower limbs (hypoplasia or aplasia). The gene for this condition, Vascular Endothelial Growth Factor Receptor 3 (VEGFR3) was identified only recently. This gene is important in the development of the lymphatics of the baby.

Lymphoedema-Distichiasis Syndrome

This condition is another autosomal dominant cause of primary lymphoedema. However, the lymphoedema usually presents in late childhood or puberty. The age of onset and severity of the swelling varies even within families. The swelling is usually associated with the presence of extra eyelashes on the inner side of the eyelids.

Although the swelling presents later, it is still due to an underlying abnormality of the lymphatic channels. Lymph scans in affected individuals have shown that there are a normal or excess number of lymphatic channels with delayed uptake of lymph in the inguinal lymph nodes, suggesting an abnormality in the function of the lymphatic channels. The mechanism is still unknown. This condition is sometimes associated with other congenital abnormalities. About one third of affected individuals have drooping of the eyelid (ptosis) which occasionally requires surgical correction. There is a slightly increased risk of heart disease at birth (8%). This is not usually severe, but may require surgical repair. A few affected individuals also have a cleft palate (3%).

The gene for this condition has been identified; it is a very small gene called FOXC2. It clearly has a role in the development of the lymphatics and eye, but very little is understood about its function.

Risk of Inheriting Lymphoedema

The risk of inheriting lymphoedema for those types where the gene is known and in which a family history exists, is approximately 50%, i.e. 1 in every 2 births. There are, of course, many other causes of primary lymphoedema.Many of these may be genetic but not inherited. Often the underlying cause is not known. The baby is at an increased risk of inheriting the lymphoedema if any of the following are present:

1. If one parent is affected and has a family history of lymphoedema
2. If the affected parent has distichiasis (extra eyelashes)
3. If the lymphoedema is symmetrical and bilateral.


The baby is at low risk of inheriting lymphoedema if:

1. The affected parent has no family history of lymphoedema
2. There is no distichiasis
3. The swelling is unilateral (including lower limbs)
4. The swelling is not in the lower limbs.


Even if a baby inherits the gene for lymphoedema, it does not mean to say he or she will be as severely affected as the parent. Indeed, the lymphoedema may be very mild despite a severely affected parent.

How Can You Tell If the Baby is Affected

Ultrasound examinations performed during the pregnancy may pick up oedema in a foot or around the back of the neck, both signs that the child may be affected. In the majority of cases, no abnormalities will be observed, and it may only be after birth or sometime later in life that the lymphoedema becomes obvious. In the future it may be possible to test the baby for the offending gene during the pregnancy, but this is not possible at present.

Prevention of Lymphoedema

In the years to come, we hope it will be possible to correct the faulty gene before the baby is born so that the lymphoedema can be reversed. This has been achieved in animals, but not yet in humans. Insertion of the normal gene instead of the faulty one is called gene therapy. It may be possible to do this in adults already affected by lymphoedema. There is hope!

Lipoedema and Pregnancy

Pregnancy may trigger or exacerbate lipedema and worsen the lymphoedema component of lipoedema.

Lipoedema is a condition that results in swelling of the hips, thighs or legs in females. Fluid does contribute to the swelling, but the main component is fat, but in a way different from obesity. In addition to swelling, which gives rise to a 'bottom heavy' or 'chunky, shapeless legs' appearance, symptoms of tissue tenderness and easy bruising are commonplace. Lipoedema tends to develop or deteriorate at times of hormonal change, e.g. puberty, pregnancy and menopause. The condition may not be apparent during the pregnancy because of all the other changes that take place. Following the pregnancy, however, weight loss may prove difficult from the lower half of the body (bottom, thighs and legs). Dieting tends to result in fat loss from face, neck and chest, but not the legs. Treatment is difficult, but a vigorous exercise regimen and healthy eating are recommended. The fluid component of lipoedema appears to be related to poor lymph drainage from the areas of fat deposition. As the fluid increases, so more noticeable oedema develops, particularly in the feet. This is called lipoedemalymphoedema syndrome (lipolymphoedema). Pregnancy may therefore trigger or exacerbate lipoedema.

Conclusions


In summary, in female patients with lymphoedema, pregnancy may create additional concerns with regard to adverse effects on the swelling and the fear of passing on the condition to any offspring. In most cases these concerns are unfounded. Any increase in swelling can usually be managed satisfactorily with the help of a lymphoedema therapist, with a full return to normal once the baby is born. In many individuals the lymphoedema will not change. In the event of a child inheriting lymphoedema, it does not follow that their condition will be the same or worse than that of the parent. The recent upsurge in our knowledge of the genes and proteins involved in lymphatic growth

Lymphedema Association of Australia

Be well - Be Safe - Enjoy that Baby