A new severity classification of lower limb secondary lymphedema based on lymphatic pathway defects in an indocyanine green fluorescent lymphography study.

Most protocols for lymphatic imaging of the lower limb conventionally inject tracer materials only into the interdigital space; however, recent studies indicate that there are four independent lymphatic vessel groups (anteromedial, anterolateral, posteromedial, and posterolateral) in the lower limb. Thus, three additional injection sites are needed for lymphatic imaging of the entire lower limb. We aimed to validate a multiple injection designed protocol and demonstrate its clinical benefits. Overall, 206 lower limbs undergoing indocyanine green fluorescent lymphography with the new injection protocol were registered retrospectively. To assess the influence of predictor variables on the degree of severity, multivariable logistic regression models were used with individual known risk factors. Using a generalized linear model, the area under the curve (AUC) of the conventional clinical model, comprising known severity risk factors, was compared with that of the modified model that included defects in the posterolateral and posteromedial groups. Multivariable logistic regression models showed a significant difference for the posteromedial and posterolateral groups. The AUC of the modified model was significantly improved compared to that of the conventional clinical model. Finding defects in the posteromedial and posterolateral groups is a significant criterion for judging lymphedema severity and introducing a new lymphedema severity classification.

Surgical Approach to Lymphedema Reduction.

PURPOSE OF REVIEW: For patients who have or may develop lymphedema due to oncologic resection, surgical options are available to prevent and treat this chronic disease. Here, we review the current pathophysiology, classification systems, surgical preventive techniques, and treatment options for lymphedema reduction. RECENT FINDINGS: Preventive surgical techniques, including de-escalation of axillary surgery, sentinel lymph node biopsy (SLNB), axillary reverse mapping (ARM), and lymphedema microsurgical preventive healing approach (LYMPHA), have been shown to reduce the incidence of lymphedema. Water displacement remains the gold standard for measuring limb volume and classification of lymphedema; however, lymphoscintigraphy and ICG lymphography are two novel imaging techniques that are now utilized to characterize lymphedema and guide management. Complete decongestive therapy (CDT) remains the mainstay of treatment. Vascularized lymph node transfer (VLNT) and lymphovenous bypass have shown promising results, particularly in advanced lymphedema stages. Combination therapy, incorporating both surgical and non-surgical approaches to lymphedema, yields best patient outcomes. Lymphedema is a chronic disease wherein management requires a combination of surgical and conservative treatments. Standardization in lymphedema staging, key outcome indicators, and quantitative data will be critical to establish the absolute best practices in lymphedema diagnosis and treatment.

Relationship between perimetric increase and fluoroscopic pattern type in secondary upper limb lymphedema observed by Indocyanine green lymphography.

To ascertain the relationship between the perimetric differences obtained between the limbs and the type of fluoroscopic pattern observed by Indocyanine green (ICG) lymphography in patients with upper limb lymphedema.A correlational descriptive study was carried out in 19 patients with upper limb lymphedema secondary to breast cancer. The perimetric increase was recorded in 11 anatomical regions after ICG injection, fluoroscopic patterns were identified using an infrared camera. The ICG patterns were categorized into worse (stardust, diffuse) or better (linear, splash) patterns.The pattern coincidence between the anterior and posterior regions of the edematous extremities was 45%. At the wrist level, a difference of 2 cm was associated with the presence of a worse fluoroscopic pattern, whereas perimeter differences of 4.25 cm in the elbow and 2.25 cm in the arm (12 cm from the epicondyle) were associated with the presence of a better fluoroscopic pattern.The perimetric differences observed between the healthy and affected upper limbs in 4 specific anatomical areas allowed us to predict the type of fluoroscopic pattern. ICG lymphography has facilitated the study of the posterior regions of edema, which are difficult to visualize using other imaging techniques.

Update and audit of the St George's classification algorithm of primary lymphatic anomalies: a clinical and molecular approach to diagnosis.

Primary lymphatic anomalies may present in a myriad of ways and are highly heterogenous. Careful consideration of the presentation can lead to an accurate clinical and/or molecular diagnosis which will assist with management. The most common presentation is lymphoedema, swelling resulting from failure of the peripheral lymphatic system. However, there may be internal lymphatic dysfunction, for example, chylous reflux, or lymphatic malformations, including the thorax or abdomen. A number of causal germline or postzygotic gene mutations have been discovered. Some through careful phenotyping and categorisation of the patients based on the St George's classification pathway/algorithm. The St George's classification algorithm is aimed at providing an accurate diagnosis for patients with lymphoedema based on age of onset, areas affected by swelling and associated clinical features. This has enabled the identification of new causative genes. This update brings the classification of primary lymphatic disorders in line with the International Society for the Study of Vascular Anomalies 2018 classification for vascular anomalies. The St George's algorithm considers combined vascular malformations and primary lymphatic anomalies. It divides the types of primary lymphatic anomalies into lymphatic malformations and primary lymphoedema. It further divides the primary lymphoedema into syndromic, generalised lymphatic dysplasia with internal/systemic involvement, congenital-onset lymphoedema and late-onset lymphoedema. An audit and update of the algorithm has revealed where new genes have been discovered and that a molecular diagnosis was possible in 26% of all patients overall and 41% of those tested.

Indocyanine green lymphography findings in older patients with lower limb lymphedema.

OBJECTIVE: Lymphedema is classified as primary or secondary according to the underlying cause. Primary lymphedema is hereditary and is considered a consequence of an inherited abnormality of the lymphatic system. Secondary lymphedema, however, is a consequence of lymphatic failure resulting from trauma, parasitic infection, or iatrogenic obstruction. Primary lymphedema is divided into three broad groups, namely, lymphedema congenita, lymphedema praecox, and lymphedema tarda. With the exception of lymphedema tarda, it is thought that age-related deterioration in lymphatic pump function is caused by oxidative stress. The aim of this study was to evaluate and to classify indocyanine green (ICG) lymphography findings in patients with lower limb lymphedema to ascertain whether there is a pattern to age-related deterioration. METHODS: There were 56 patients (104 edematous lower limbs) who had undergone ICG lymphography and for whom the lower extremity lymphedema (LEL) index had been calculated enrolled in this study. Specific inclusion criteria were used to exclude other causes of edema. ICG lymphography images were recorded in the plateau phase (12-18 hours after injection), when no further changes of images would be expected. The LEL index was calculated by summation of the squares of the circumference for five areas in each lower extremity divided by the body mass index. RESULTS: The clinical lymphedema pattern was determined as bilateral in 48 patients and unilateral in 8 patients. Patients with bilateral lymphedema were significantly older than those with unilateral lymphedema (76.40 ± 8.03 years vs 53.13 ± 14.12 years; P < .01). The ICG lymphography pattern was categorized as linear, low enhancement (LE), distal dermal backflow (DB), or extended DB in bilateral lymphedema. ICG lymphography showed the DB pattern on both the thigh and lower leg regions in all eight legs with unilateral lymphedema. There were also significant between-group differences in the LEL index (linear vs distal DB, P < .05; linear vs extended DB, P < .01; linear vs unilateral, P < .01; LE vs extended DB, P < .01; LE vs unilateral, P < .01; distal DB vs extended DB, P < .05; and distal DB vs unilateral, P < .01). CONCLUSIONS: In this study, unilateral lymphedema, with its younger age at onset, severity, and unilateral dominance, corresponded to lymphedema tarda. In contrast, bilateral lymphedema corresponded to senile lymphedema, which is distinct from primary lymphedema in general and lymphedema tarda in particular. Age-related deterioration in lymphatic pump function rather than iatrogenic obstruction or genetic abnormality is likely to account for the characteristic older age at onset of lymphedema and its progression from the distal region.

Terapia descongestiva compleja en grados iniciales de linfedema secundario a cáncer de mama / Complex decongestive therapy in the early stages of breast cancer-related lymphoedema

Objetivos: Analizar los resultados del tratamiento con terapia descongestiva compleja (TDC) aplicada por primera vez en sujetos con linfedema secundario a cáncer de mama (LSCM) grados 0-I. Material y métodos: Estudio descriptivo retrospectivo con sujetos diagnosticados de LSCM de grado 0 y I en una Unidad de Linfedema, a los que se les realiza TDC por primera vez entre los años 2013 y 2016. Se compara el porcentaje de exceso de volumen (PEV) del linfedema en 3 intervalos (inicial, postratamiento y en la revisión médica) y se calcula la reducción del mismo (RPEV) después del tratamiento. Se registran datos demográficos, clínicos y las características del linfedema. Resultados: Fueron incluidas 27 mujeres, con una media de edad de 55 años (49-62). El 88,90% de ellas (n = 24) intervenidas de linfadenectomía y el 85,20% (n = 23) con radioterapia coadyuvante. Se realizaron de media de 23 sesiones. Previo a la TDC 7 participantes tenían LSCM de grado 0 y 20 de grado I; después del tratamiento 16 participantes tenían linfedema de grado 0 y 11 de grado I. La media inicial de PEV fue de 11,76%, 8,77% después del tratamiento y 7,57% en la revisión médica. El PEV se redujo en 22 participantes después del tratamiento (81,50%). Exceptuando un caso, la reducción de volumen se mantuvo estable en la revisión médica. La RPEV después del tratamiento fue 28,15% y 44,79% en la revisión médica. Conclusión: Este estudio muestra unos resultados favorables al empleo de TDC para el tratamiento de linfedema secundario a cáncer de mama de grado 0 y/o I

Objectives: To analyse the outcomes of Complex Decongestive Therapy (CDT) applied for the first time in patients with Breast Cancer-Related Lymphoedema (BCRL) stages 0-I. Material and methods: A descriptive and retrospective study performed on subjects with BCRL severity stages 0 and I recruited in the Lymphology Unit, and who had CDT for the first time between 2013 and 2016. A comparison was made of the percent excess volume (PEV) of lymphoedema at 3 intervals (initial, post-treatment, and medical check-up) and we calculated the percent excess volume reduction (PEVR) after treatment. Demographic data, clinical and lymphoedema characteristics were recorded. Results: The study included a total of 27 women, with mean age of 55 years (49-62). A lymphadenectomy had been performed on 88.90% (n = 24), and 85.20% (n = 23) had radiation therapy. There was a mean of 23 sessions. Before CDT, 7 participants had stage 0 BCRL and 20 had stage I. After treatment, 16 participants had lymphoedema stage 0 and 11 had stage I. The mean initial PEV was 11.76%, 8.77% after treatment and 7.57% in the medical check-up. PEV was reduced in 22 participants after treatment (81.50%). There was only one case where the reduction of volume remained stable in the medical check-up. The mean PEVR after treatment was 28.15% and 44.79% in the medical check-up. Conclusion: This study shows favourable results of Complex Decongestive Therapy in Breast Cancer-Related Lymphoedema stages 0 and I

Validity of the Novel Taiwan Lymphoscintigraphy Staging and Correlation of Cheng Lymphedema Grading for Unilateral Extremity Lymphedema.

OBJECTIVE: The aim was to validate the new Taiwan Lymphoscintigraphy Staging, correlate it with Cheng Lymphedema Grading (CLG) and evaluate the treatment outcomes of unilateral extremity lymphedema. BACKGROUND: No consensus has been reached for diagnosis and staging for patients with lymphedema among medical specialties. METHODS: We included 285 patients with unilateral extremity lymphedema using lymphoscintigraphy. Lymphoscintigraphy was correlated to clinical symptoms and signs, and classified into normal lymphatic drainage, partial obstruction, and total obstruction. Inter- and intraobserver reliability of Taiwan Lymphoscintigraphy Staging, correlation between Taiwan Lymphoscintigraphy Staging and clinical findings were conducted. Patients were categorized in "surgical" (n = 154) or "nonsurgical" (n = 131) groups for outcome evaluation. RESULTS: Lymphoscintigraphy found 11 patients (3.9%) with normal lymphatic drainage, 128 (44.9%) with partial obstruction, and 146 (51.2%) with total obstruction. Taiwan Lymphoscintigraphy Staging showed high interobserver agreement [intraclass correlation coefficient: 0.89 (95% confidence interval, 0.82-0.94)], and significantly correlated to computed tomography volumetric difference (r = 0.66, P < 0.001) and CLG [intraclass correlation coefficient: 0.79 (95% confidence interval 0.72-0.84)]. At a mean follow-up of 31.2 ±â€Š2.9 months, significant improvement in the circumferential difference (from 23.9% ±â€Š17.6% to 14.6% ±â€Š11.1%; P = 0.03) with a mean circumferential reduction rate of 40.4% ±â€Š4.5% was found in surgical group. At a mean follow-up of 26.6 ±â€Š8.7 months, the nonsurgical group had increase of mean circumferential difference from 24.0% ±â€Š17.2% to 25.3% ±â€Š19.0% (P = 0.09), with a mean circumferential reduction rate was -1.9% ±â€Š13.0%. CONCLUSIONS: The Taiwan Lymphoscintigraphy Staging is a reliable diagnostic tool, correlated with clinical findings and CLG, aiding in the selection of the appropriate treatment to achieve favorable long-term outcomes in unilateral extremity lymphedema.

Lipoedema is not lymphoedema: A review of current literature.

Lipoedema is a rare painful disorder of the adipose tissue. It essentially affects females and is often misdiagnosed as lymphoedema or obesity. It is globally misdiagnosed or underdiagnosed, and the literature is lacking appropriate guidance to assist clinicians towards this diagnosis. However, the need to recognise this disorder as a unique entity has important implications to establish proper treatment and, therefore, its tremendous effect on patients. Early diagnosis and treatment can turn these patients' lives upside down. The aim of this review is to focus on the clinical guidance, differential diagnosis, and management strategies. In addition, other aspects of lipoedema, including epidemiology and pathogenesis, are also being discussed here. Lipoedema is distinct from obesity and distinct from lymphoedema, although it might progress to involve the venous and lymphatic system (venolipedema or lympholipedema or both). Late diagnosis can leave the patient debilitated. Management of lipoedema includes weight loss, control of oedema, complex decongestive physiotherapy, liposuction, and laser-assisted lipolysis. However; there are increasing reports on tumescent liposuction as the preferred surgical option with long-lasting results. The role of more randomised controlled studies to further explore the management of this clinical entity remains promising.

A Review of Lymphedema for the Hand and Upper-Extremity Surgeon.

Secondary lymphedema of the upper limb is frequently seen in Western countries after cancer treatment (most often breast in women). It is a chronic disease that affects quality of life and functioning. In its extreme form, it may be debilitating. A review is given of the pathology, nonsurgical treatment, and surgical treatment with a protocol.

Diagnostic accuracy of bioimpedance spectroscopy in patients with lymphedema: A retrospective cohort analysis.

BACKGROUND: Bioimpedance spectroscopy (BIS) is used by healthcare specialists to diagnose lymphedema. BIS measures limb fluid content by assessing tissue resistance to the flow of electric current. However, there is debate regarding the validity of BIS in diagnosing early lymphedema. Indocyanine green (ICG) lymphography has been established as the most accurate diagnostic modality to date for lymphedema diagnosis. In this retrospective study, we test the sensitivity, specificity, and diagnostic accuracy of BIS in diagnosing lymphedema by referencing its results with ICG lymphography. METHODS: Patients presented to the University of Iowa Lymphedema Center from 2015 to 2017 were evaluated with a standardized protocol that included history and physical examination, a validated lymphedema-specific quality-of-life assessment (LYMQOL), circumference -measurement-based index, BIS, and ICG lymphography. Diagnostic accuracy of BIS was assessed using ICG lymphography as a reference test. RESULTS: Fifty-eight patients had positive ICG lymphography results, which confirmed the diagnosis of lymphedema. ICG lymphographic findings consistently correlated with clinical examination, LYMQOL evaluation, and lymphedema indices. By contrast, BIS demonstrated a false-negative rate of 36% - 21 out of 58 patients had normal BIS readings, but a positive ICG lymphography result. The 21 false-negative results occurred in patients with early-stage disease. Sensitivity and specificity for BIS were 0.64 and 1, respectively. CONCLUSION: BIS carries an excessively high rate of false-negative results to be dependably used as a diagnostic modality for lymphedema. ICG lymphography highly correlates with other tracking modalities, and it remains the most reliable tool for diagnosing lymphedema.

Alternativa quirúrgica para el linfedema obstructivo de los miembros mediante el implante de drenajes de silicona hidrófobos / Alternative surgical treatment for chronic lower limb lymphoedema by implantation of hydrophobic silicone drainage tubes

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An approach to familial lymphoedema.

Lymphoedema is the build-up of lymphatic fluid leading to swelling in the tissues. Most commonly it affects the peripheries. Diagnosis is based on clinical assessment and imaging with lymphoscintigraphy. Treatment is supportive with compression garments, massage, good skin hygiene and prompt use of antibiotics to avoid the complication of cellulitis. Most commonly, lymphoedema occurs as a result of damage to the lymphatic system following surgery, trauma, radiation or infection. However, it can be primary, often associated with a genetic defect that causes disruption to the development of the lymphatic system. Common genetic conditions associated with lymphoedema include Turner syndrome and Noonan syndrome; however, there are numerous others that can be classified based on their clinical presentation and associated features. Herein we discuss how to diagnose and classify the known primary lymphoedema conditions and how best to investigate and manage this group of patients.

Diagnosis of Upper Quadrant Lymphedema Secondary to Cancer: Clinical Practice Guideline From the Oncology Section of the American Physical Therapy Association.

The Oncology Section of the American Physical Therapy Association (APTA) developed a clinical practice guideline to aid the clinician in diagnosing secondary upper quadrant cancer-related lymphedema. Following a systematic review of published studies and a structured appraisal process, recommendations were written to guide the physical therapist and other health care clinicians in the diagnostic process. Overall clinical practice recommendations were formulated based on the evidence for each diagnostic method and were assigned a grade based on the strength of the evidence for different patient presentations and clinical utility. In an effort to maximize clinical applicability, recommendations were based on the characteristics as to the location and stage of a patient's upper quadrant lymphedema.

The Dutch lymphedema guidelines based on the International Classification of Functioning, Disability, and Health and the chronic care model.

Lymphedema is a chronic and progressive condition due to an imbalance between lymphatic filtration and transport capacity. A relative overload of fluid is caused by lymphatic impairment (afterload impairment); an absolute overload is caused by increased filtration (preload raised). Lymphedema can result in considerable disability and loss of quality of life. The Dutch Society of Dermatology organized a task force to create guidelines using the International Classification of Functioning, Disability, and Health. As lymphedema is a chronic condition, a new approach was chosen according to the chronic care model. In the development of the guidelines, the following topics of lymphedema care were proposed: (1) lymphedema detection; (2) clinimetric instruments; and (3) rational treatment modalities. A systematic review of the literature published up to June 2013 was conducted. Statements were based on scientific evidence and experience. The guidelines propose recommendations based on an interdisciplinary approach to lymphedema using a continuum of care from prevention to initial treatment phase, maintenance phase, and follow-up. An integrated treatment program can be conservative or surgical. A therapeutic program depends on stage and origin of lymphedema; International Classification of Functioning, Disability, and Health-based limitations; needs of the patient; ability to perform self-management; and ability to reduce patient-related risk factors, such as overweight and lack of exercise.

Lower Body Lymphedema in Patients with Gynecologic Cancer.

Lower body lymphedema is a chronic condition and a significant cause of morbidity following treatment of gynecologic cancer that strongly impacts patients' quality of life (QoL). Most studies on secondary lymphedema have been performed on the upper limb after breast cancer treatment and much less is known about lower body lymphedema after gynecologic malignancies. This review focuses on secondary lymphedema due to gynecologic cancer treatment, analyzing its incidence in the different types of gynecologic cancer, diagnosis, risk factors, impact on QoL and treatment. A systematic search of Medline has been performed to track the studies evaluating lower body lymphedema after treatment for endometrial, ovarian, cervical and vulvar cancer. Unfortunately, there is no consensus about a uniform evaluation and, as a consequence, the reported incidence is broadly different among the studies. Standardization in lymphedema evaluation is required to better compare the outcome of different types of treatment.

[Lipedema: up-to-date of a long forgotten disease]. / Das Lipödem: Aktuelle Aspekte einer lange vergessenen Erkrankung.

Lipedema is a chronic disorder of subcutaneous adipose tissue of unknown etiology not uncommon among post-puberty women. The disease has a negative impact on self-esteem, mobility, and quality of life. Lipedema is characterized by symmetrical, disfiguring hyperplastic adipose tissue combined with bruising and pain. Untreated lipedema fosters osteoarthritis, secondary lymphedema, limited mobility, and psychosocial stigmatization. Treatment consists of conservative complex decongestive therapy and surgery by microcannular tumescent liposuction. Liposuction is the only available treatment capable to reduce the pathological adipose tissue durable and to prevent complications.

Use of compression therapy in patients with lymphoedema.

Compression therapy is an effective and common approach to the management of lymphoedema, a chronic condition requiring long-term treatment. Lymphoedema is caused by the accumulation of fluid in soft tissue, resulting in swelling of the affected limb or area. Lymphoedema may be primary, as a result of a genetic abnormality, or secondary, as a result of injury, including non-accidental injury, or following treatments such as radiotherapy and/or surgery. The condition is characterised by a high protein oedema and secondary skin changes. This article outlines features of the various types of compression therapy used to treat patients with lymphoedema, including details of their application, effectiveness, indications and contraindications.

Combined Surgical Treatment in Breast Cancer-Related Lymphedema.

BACKGROUND: Lymphedema is a well-known sequela of breast but no consensus has been reached about the ideal treatment. Surgical approaches, however, are receiving increased attention. Various microsurgical reconstructive techniques aim to restore anatomy and function of the lymphatic system in upper limb breast cancer-related lymphedema (BCRL). We combined two techniques, lymphaticovenous anastomosis (LVA) and autologous lymph node transplantation (ALNT) after carefully selecting those who may benefit from the surgery. We called this the "combined surgical treatment (CST)" approach. METHODS: From June 2007 to December 2011, we performed CST in 106 patients with upper limb BCRL. Clinical evaluation and diagnostic imaging studies were performed preoperatively in all the patients. CST was offered to patients with stage I/II lymphedema, according to the criteria of the International Society of Lymphology (ISL). RESULTS: Overall 59 of the 106 patients underwent LVA, 7 underwent ALNT, and 40 underwent both the techniques. All 47 lymph node (LN)-flaps survived but 11 (22%) required surgical revision within 3 days. A total of 21 LN-flaps (45%) showed no radiotracer uptake at 1 year. Around 1 to 7 LVAs for each patient (average 3.4) were performed. Preoperative versus postoperative excess circumference decreased between 12 and 86.7% (average 39.72%). Arm circumference decreased between 0.9 and 6.1 cm (average 2.75 cm). The number of episodes of lymphangitis per year decreased from 1.8 to 0.2. CONCLUSION: Preoperative assessment is essential to select patients who can benefit from surgery for lymphedema and to choose the best surgical approach in each case. Our satisfactory results in well-selected cases encourage further research into surgical treatment for BCRL.

Development of consensus International Classification of Functioning, Disability and Health (ICF) core sets for lymphedema.

To understand the challenges of patients with lymphedema it is important to describe functioning and to measure the effectiveness of treatment in changing functioning. The International Classification of Functioning, Disability and Health (ICF) offers an international framework to classify functioning of persons in their personal environment. ICF Core Sets are lists of selected ICF categories concerning those important aspects of functioning that are most likely to be affected by a specific health problem or disease. These Core Sets make it easier and faster to describe and communicate the patient's problems and to define treatment goals. Furthermore, they are available to health care providers of all professions, researchers, health insurance companies and policy-makers. The objective of this document is to present the outcomes of a consensus conference held to determine the first versions of the ICF Core Sets for lymphedema. Frequency rankings were made of the ICF categories derived from four preparatory studies, being: a) a systematic review; b) a qualitative study; c) an expert survey; and d) a cross-sectional study. By means of working group discussions and plenary sessions, a final consensus on ICF categories was achieved and Comprehensive and Brief Core Sets for lymphedema for the upper limb, lower limb, and midline lymphedema were defined. These ICF Core Sets contain different items in each region. Future validation of these Core Sets for health professions and for countries is needed.