Håkan Brorson

Lymphedema - A comprehensive review

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.

liposuction Combined with Controlled Compression Therapy Reduces Arm Lymphedema More Effectively than Controlled Compression Therapy Alone

&NA; Arm lymphedema after breast cancer therapy has been treated with various forms of conservative and surgical treatment during recent years. The clinical results usually have been modest or, in some instances, even disappointing. In a previous series of patients treated with the new liposuction technique combined with controlled compression therapy, we found, however, an overall edema reduction of 106 percent after 1 year. The purpose of this study was both to investigate how much the surgical procedure contributes to the outcome and to clarify the importance of controlled compression therapy. Twentyeight patients were, therefore, prospectively matched into two groups. One group received liposuction combined with controlled compression therapy, and one group received the therapy alone. Additionally, the therapy group was compared with our complete group of patients treated thus far with liposuction combined with therapy (n = 30). The prospective study using matched pairs (n = 14) showed that liposuction combined with controlled compression therapy is significantly more effective than the therapy alone (p < 0.0001), with a mean difference of about 1000 ml during the entire 1‐year observation period. The beneficial effect of liposuction was confirmed by the comparison between the controlled compression therapy group and our complete group of patients treated with liposuction combined with the therapy, as the edema reduction figures after 1 year were 47 percent and 104 percent, respectively (p < 0.0001). In six patients who had surgery and a complete reduction of the edema, the compression garments were removed for 1 week, 1 year postoperatively. A marked increase in the arm volume was observed, which was immediately remedied by reapplying the garments. We conclude that liposuction combined with controlled compression therapy reduces arm lymphedema more efficiently than the therapy alone. Continued use of compression garments is, however, important to maintain the primary surgical outcome. (Plast. Reconstr. Surg. 102: 1058, 1998.)

Liposuction in arm lymphedema treatment.

Breast cancer is the most common disease in women, and up to 38% develop lymphedema of the arm following mastectomy, standard axillary node dissection and postoperative irradiation. Limb reductions have been reported utilizing various conservative therapies such as manual lymph and pressure therapy. Some patients with long-standing pronounced lymphedema do not respond to these conservative treatments because slow or absent lymph flow causes the formation of excess subcutaneous adipose tissue. Previous surgical regimes utilizing bridging procedures, total excision with skin grafting or reduction plasty seldom achieved acceptable cosmetic and functional results. Microsurgical reconstruction involving lympho-venous shunts or transplantation of lymph vessels has also been investigated. Although attractive in concept, the common failure of microsurgery to provide complete reduction is due to the persistence of newly formed subcutaneous adipose tissue, which is not removed in patients with chronic non-pitting lymphedema. Liposuction removes the hypertrophied adipose tissue and is a prerequisite to achieve complete reduction. The new equilibrium is maintained through constant (24-hour) use of compression garments postoperatively. Long-term follow up (7 years) does not show any recurrence of the edema.

Breast cancer-related chronic arm lymphedema is associated with excess adipose and muscle tissue.

BACKGROUND Arm lymphedema is a common complication after breast cancer treatment. Although conservative treatment can be used to reduce swelling, treatment often fails, possibly due to chronic edema being transformed from lymph fluid to subcutaneous fat, a condition called nonpitting lymphedema. It is currently unknown if the excess volume is solely due to excess in fat. This study evaluated whether dual energy X-ray absorptiometry (DXA) could be used to estimate the excess fat, muscle, and bone tissue in patients with arm lymphedema. METHODS AND RESULTS Eighteen women with arm lymphedema were investigated. Measurements were converted to volume values and compared with values obtained using plethysmography (PG). Linear regression equations and correlation equations were used to compare the DXA and the PG techniques in regard to total volume and excess volume in the lymphedematous arm. DXA was used to estimate excess fat, muscle, and bone volume in the lymphedematous arm. Both DXA and PG provided similar total arm volume and excess volume measurements for the lymphedematous arm. The lymphedematous arm showed 73% more fat, 47% more muscle, and 7% more bone by volume in the lymphedematous arm. CONCLUSIONS Both excess fat and muscle volume contributed to the total excess volume in nonpitting arm lymphedema; excess soft tissue developed the first few years after breast cancer surgery. DXA can be used to identify patients with excess fat in their arms and thus unsuitable for conservative treatment and may be useful in estimating the amount of fat to remove in patients scheduled for liposuction.

Complete reduction of lymphoedema of the arm by liposuction after breast cancer

The incidence of lymphoedema of the arm after mastectomy ranges between 8% and 38%, and it is an appreciable problem from both functional and social aspects. Conservative and previous surgical regimens have not been completely successful. In the light of these experiences, liposuction clearly constitutes an interesting new surgical approach, which is potentially capable of effecting predictable and reliable improvements in patients with lymphoedema. Twenty eight women with lymphoedema of the arm after breast cancer were consecutively treated by liposuction. Limb compression with a compression garment was instituted immediately after operation. All patients had been given radiotherapy after the operation for breast cancer. Mean preoperative volume of oedema was 1845 ml (range 570-3915), and mean volume of aspirate was 2250 ml (range 1000-3850); volume of aspirate correlated linearly with the volume of preoperative oedema. There were no major surgical complications, but blood transfusion was necessary in eight patients whose volume of aspirate exceeded 2000 ml. After 12 months (n = 24), an average reduction in volume of oedema of 106% was found. Such a normalisation can be expected in patients with oedema that amounts to about 2500 ml. Although the oedema cannot be completely removed in more severe cases, substantial reduction is beneficial from both functional and cosmetic aspects. We conclude that liposuction is safe and effective for reducing lymphoedema of the arm after operations for breast cancer. In a one-stage procedure, oedematous and hypertrophic fat tissue can be removed with an excellent clinical outcome.

Circumferential suction‐assisted lipectomy for lymphoedema after surgery for breast cancer

The incidence of arm lymphoedema after treatment for breast cancer ranges from 1 to 49 per cent. Although most women can be treated by non‐operative means with satisfying results, end‐stage lymphoedema is often non‐responsive to compression, where hypertrophy of adipose tissue limits the outcome value of compression or massage.

From lymph to fat: liposuction as a treatment for complete reduction of lymphedema.

There is some controversy regarding liposuction for late-stage lymphedemas. Although it is clear that conservative therapies such as complex decongestive therapy and controlled compression therapy should be tried in the first instance, options for the treatment of late-stage lymphedema that is not responding to treatment is not so clear. Liposuction has been used for many years to treat lipodystrophy. Some results have been far from optimal; however, improvements in technique, patient preparation, and patient follow-up have led to a greater and a wider acceptance of liposuction as a treatment for lymphedema. This article outlines the benefits of using liposuction and presents the evidence to support its use.

Liposuction in Dercum's disease: impact on haemostatic factors associated with cardiovascular disease and insulin sensitivity.

Berntorp E, Berntorp K, Brorson H, Frick K (University of Lund, Malmö, Sweden). Liposuction in Dercums disease: impact on haemostatic factors associated with cardiovascular disease and insulin sensitivity. J Intern Med 1998; 243: 197–201.

Standardised measurements used to order compression garments can be used to calculate arm volumes to evaluate lymphoedema treatment

Abstract Lymphoedema treatment outcome can be evaluated by calculating estimated limb volumes directly by water displacement (plethysmography; PG), or indirectly by circumference measurements (CM) and using the formula for a truncated cone. This study assessed the correlation between PG and circumference volume measurements to assess whether the correlation is acceptable, and if circumference measurements can be used to accurately assess arm volume. Ten women with unilateral lymphoedema after breast cancer treatment with a mean age of 66 (range 50–83) years volunteered for arm volume estimates by PG and circumference measurements. The coefficient of variation (CV%) for all methods was calculated. Two Excel-based formulae of the truncated cone were developed; one for fixed 4-cm intervals leading to 10 volume segments (CM-10-VS), and one for varying intervals leading to four volume segments (CM-4-VS). The CV% was 0.609% for PG, 0.628% for CM-10-VS, and 0.632% for CM-4-VS. As expected, PG generated a significantly larger volume of both arms because it includes the hand. The difference between CM-10-VS and CM-4-VS measurements was not significant. All three measurement methods showed a high coefficient of correlation (0.813–0.915), and a high coefficient of regression (0.863–1.089). The excess volume, which is used to determine treatment outcome, showed the respective values of 0.932–0.978 and 0.963–1.020, respectively. Using circumference measurements identical to those used when ordering made-to-measure compression garments speeds up volume measurements and can be used safely to evaluate lymphoedema treatment outcome.

Review of Dercum's disease and proposal of diagnostic criteria, diagnostic methods, classification and management

Definition and clinical pictureWe propose the minimal definition of Dercum’s disease to be generalised overweight or obesity in combination with painful adipose tissue. The associated symptoms in Dercum’s disease include fatty deposits, easy bruisability, sleep disturbances, impaired memory, depression, difficulty concentrating, anxiety, rapid heartbeat, shortness of breath, diabetes, bloating, constipation, fatigue, weakness and joint aches.ClassificationWe suggest that Dercum’s disease is classified into: I. Generalised diffuse form A form with diffusely widespread painful adipose tissue without clear lipomas, II. Generalised nodular form - a form with general pain in adipose tissue and intense pain in and around multiple lipomas, and III. Localised nodular form - a form with pain in and around multiple lipomas IV. Juxtaarticular form - a form with solitary deposits of excess fat for example at the medial aspect of the knee.EpidemiologyDercum’s disease most commonly appears between the ages of 35 and 50 years and is five to thirty times more common in women than in men. The prevalence of Dercum’s disease has not yet been exactly established.AetiologyProposed, but unconfirmed aetiologies include: nervous system dysfunction, mechanical pressure on nerves, adipose tissue dysfunction and trauma.Diagnosis and diagnostic methodsDiagnosis is based on clinical criteria and should be made by systematic physical examination and thorough exclusion of differential diagnoses. Advisably, the diagnosis should be made by a physician with a broad experience of patients with painful conditions and knowledge of family medicine, internal medicine or pain management. The diagnosis should only be made when the differential diagnoses have been excluded.Differential diagnosisDifferential diagnoses include: fibromyalgia, lipoedema, panniculitis, endocrine disorders, primary psychiatric disorders, multiple symmetric lipomatosis, familial multiple lipomatosis, and adipose tissue tumours.Genetic counsellingThe majority of the cases of Dercum’s disease occur sporadically. A to G mutation at position A8344 of mitochondrial DNA cannot be detected in patients with Dercum’s disease. HLA (human leukocyte antigen) typing has not revealed any correlation between typical antigens and the presence of the condition.Management and treatmentThe following treatments have lead to some pain reduction in patients with Dercum’s disease: Liposuction, analgesics, lidocaine, methotrexate and infliximab, interferon α-2b, corticosteroids, calcium-channel modulators and rapid cycling hypobaric pressure. As none of the treatments have led to long lasting complete pain reduction and revolutionary results, we propose that Dercum’s disease should be treated in multidisciplinary teams specialised in chronic pain.PrognosisThe pain in Dercum’s disease seems to be relatively constant over time.