Your browser doesn't support javascript.
loading
Show: 20 | 50 | 100
Results 1 - 20 de 847
Filter
2.
J Vasc Surg Venous Lymphat Disord ; 10(2): 436-444.e1, 2022 03.
Article in English | MEDLINE | ID: mdl-34352420

ABSTRACT

OBJECTIVE: Chronic primary or secondary lymphedema has huge effects on patients' quality of life (QOL) because of the associated swelling and pain, decreased range of motion, and depression and anxiety and generally requires numerous adaptations. Many studies have shown a positive objective effect of lymphovenous anastomoses (LVAs) on chronic lymphedema. In the present study, we assessed the effect of LVAs on QOL in patients with primary or secondary lymphedema of the lower extremity at 6 months after surgery and examined the correlation between changes in the QOL and volumetric measurements. METHODS: Only patients with either primary or secondary lymphedema of the lower extremity who had undergone LVAs were included in the present study. To assess QOL, a specially designed questionnaire based on the Lymphedema Quality of Life Inventory was used to evaluate the subjective therapeutic results from the patients' perspective. Objective therapy success was assessed using three-dimensional volumetric measurements of the lower leg. The measuring points, for both the subjective and the objective measurements, were the day before and 6 months after therapy. RESULTS: The mean change in volume at 6 months after LVA was -6.5% ± 5.6% (P < .001). Significantly better QOL in terms of physical (37.6% ± 25.2%) and psychosocial (27.0% ± 43.0%) domains and practical restrictions (22.3% ± 24.8%) was found (P < .001 for all). No correlation was found between QOL improvement and volume decrease (P > .05). CONCLUSIONS: For patients with lymphedema of the lower extremity, LVAs can lead to a significant volumetric decrease and QOL improvement at 6 months after treatment with no demonstrable relationship between QOL improvement and volume reduction.


Subject(s)
Lower Extremity/surgery , Lymphedema/surgery , Quality of Life , Surveys and Questionnaires , Adult , Aged , Anastomosis, Surgical , Chronic Disease , Cost of Illness , Female , Humans , Lower Extremity/pathology , Lower Extremity/physiopathology , Lymphedema/pathology , Lymphedema/physiopathology , Male , Middle Aged , Predictive Value of Tests , Prospective Studies , Time Factors , Treatment Outcome
3.
Ann Vasc Surg ; 78: 263-271, 2022 Jan.
Article in English | MEDLINE | ID: mdl-34480978

ABSTRACT

BACKGROUND: Pediatric lymphedema can result in irreversible, debilitating limb swelling, tissue fibrosis, skin ulcers, infection, and impaired limb function in children at an early age. Manual lymphatic drainage (MLD) is a noninvasive technique, which is a part of intensive decongestive therapy to reroute lymphatic flow to healthy channels used to manage lymphedema. Outcomes of this treatment option in children have not been studied. We evaluated the effect of decongestive therapy involving MLD in pediatric patients with complex lymphatic anomalies by measuring treatment progress and functional outcomes via changes in limb circumference, limb functionality, dexterity, skin quality, and pain. METHODS: A single-institution retrospective study on a cohort of 8 pediatric patients with lymphatic anomalies who completed a course of MLD was conducted from 2015 to 2017 to investigate the role MLD plays in their lymphedema reduction. Pain scores were measured on a scale of 0-10, with 0 being no pain and 10 being the worst pain imaginable. The functional performance was measured by the Canadian Occupational Performance Measurement questionnaire. RESULTS: Among all patients, there were 4 cases affecting the upper extremities, 4 affecting the lower extremities, and 3 affecting the truncal region. Five of 8 patients demonstrated a reduction in lymphedema with an average girth reduction of 8.2% in the lower extremities, 3.0% in the upper extremities, and 7.4% in the truncal regions. In unilateral cases, the difference in limb circumference between the affected and normal extremity decreased by an average of 25.6%. Four patients completed the Canadian Occupational Performance Measurement questionnaire with an average improvement of 30% in daily task performance. Three patients reported complete resolution of pain. CONCLUSIONS: MLD can be used as a reliable noninvasive method for decongestion and analgesia to delay the onset of lymphedema-associated fibrosis and long-term disability in children with complex lymphatic malformations.


Subject(s)
Drainage , Lymphedema/therapy , Adolescent , Age Factors , Child , Female , Humans , Infant , Lymphedema/diagnostic imaging , Lymphedema/physiopathology , Male , Retrospective Studies , Treatment Outcome
4.
Phys Ther ; 101(12)2021 12 01.
Article in English | MEDLINE | ID: mdl-34636891

ABSTRACT

OBJECTIVE: The main aims of this study were to (1) create a patient-reported outcome measure (PROM) item bank for measuring the impact of upper quadrant edema (UQE) on physical function by calibrating responses to newly developed items; and (2) assess reliability, validity, and administration efficiency of scores based on computerized adaptive test (CAT) and 10-item short-form (SF) administration modes. METHODS: This was a retrospective study including data from patients treated in outpatient rehabilitation clinics for UQE that responded to all 27 candidate items at intake. Item response theory model assumptions of unidimensionality, local item independence, item fit, and presence of differential item functioning were evaluated. UQE-CAT- and UQE-SF-generated scores were assessed for reliability, validity, and administration efficiency. RESULTS: The total cohort included 3486 patients (mean [SD] age = 61 [13] years; range = 14-89 years). After removing 2 items, a 25-item solution was supported for its unidimensionality and fit to the item response theory model with reliability estimates of more than 0.93 for scores based on both CAT and SF administration modes. No items demonstrated differential item functioning. Scores discriminated among multiple patient groups in clinically logical ways and were moderately responsive to change with negligible floor and acceptable ceiling effects. CAT scores were generated using an average of 5.6 items (median = 5). CONCLUSION: Scores on the UQE PROM were reliable, valid, and efficient for assessing perceived physical function of patients with upper quadrant edema; thus, the measure is suitable for research and routine clinical administration. IMPACT: The newly developed UQE PROM is reliable and valid and offers efficient administration modes for assessing perceived physical function of patients with UQE caused by lymphatic and venous disorders, both for research and routine clinical care in busy outpatient rehabilitation settings. As an item response theory-based measure, the UQE PROM allows administration of condition-specific functional questions with low response burden for patients. This study supports a transition to PROMs that are based on modern measurement approaches to achieve high accuracy and efficiency.


Subject(s)
Edema/physiopathology , Edema/therapy , Lymphedema/physiopathology , Lymphedema/therapy , Patient Reported Outcome Measures , Surveys and Questionnaires/standards , Venous Insufficiency/physiopathology , Venous Insufficiency/therapy , Adolescent , Adult , Aged , Aged, 80 and over , Humans , Middle Aged , Reproducibility of Results , Retrospective Studies , Young Adult
6.
Cancer Treat Res Commun ; 29: 100459, 2021.
Article in English | MEDLINE | ID: mdl-34563789

ABSTRACT

BACKGROUND: Breast cancer-related lymphedema (BCRL) is a lifelong condition. Millions who develop breast cancer are younger than retirement age and at a lifetime risk for developing BCRL. Rural and small-town survivors may face unique challenges in terms of access to health care and BCRL/survivorship resources. This multiple-case study describes how BCRL influences the work experiences and quality of life (QoL) of survivors living in rural and small towns in Missouri. METHODS AND MATERIALS: Thirteen survivors from rural and small towns in Missouri completed semi-structured interviews and a standardized QoL instrument. Cases were analyzed using in-vivo and open-coding techniques and constant cross-case comparative methods. Twelve of the 13 participants' data are synthesized into themes to represent an illustrative case. The 13th case is presented as a contradictory (rival) case. RESULTS: Four themes are represented within the illustrative case - multiple medical encounters; the development of self-care routines; the reciprocity of work/live activities, triggers, and adjustments; and rural/small-town cultural impact. Upon BCRL diagnosis, survivors received intensive treatments, eventually establishing self-care routines. Survivors identified strategies for working around their BCRL when completing work and home responsibilities. The contradictory (rival) case was more recently diagnosed and, as such, had not established self-care and coping mechanisms in the same way. CONCLUSIONS AND IMPLICATIONS: Survivors alleviate BCRL symptoms and improve their QoL by establishing self-care strategies. This provides guidance for client-centered survivorship care-planning and occupational rehabilitation of rural survivors with BCRL. This study provides the foundation for developing information for rural survivors that supports mental preparation and coping skills for BCRL self-management.


Subject(s)
Breast Neoplasms/complications , Lymphedema/physiopathology , Quality of Life/psychology , Return to Work/statistics & numerical data , Aged , Breast Neoplasms/mortality , Cancer Survivors , Female , Humans , Lymphedema/mortality , Middle Aged , Rural Population
7.
Rev. cir. (Impr.) ; 73(3): 370-377, jun. 2021. tab
Article in Spanish | LILACS | ID: biblio-1388834

ABSTRACT

Resumen El lipedema es una enfermedad común, frecuentemente subdiagnosticada, crónica y progresiva, que genera un gran deterioro en la calidad de vida. Consiste en el depósito anormal de tejido adiposo subcutáneo principalmente en las extremidades inferiores, afectando casi exclusivamente a mujeres. Sus síntomas principales son el dolor, la sensibilidad y la facilidad para producir equimosis. Desde el punto de vista fisiopatológico, existiría una susceptibilidad poligénica combinada con trastornos hormonales, microvasculares y linfáticos que pueden ser en parte responsables del desarrollo del lipedema. Se clasifica, según la distribución de la grasa en cinco tipos y, según la gravedad de la enfermedad, en cuatro etapas. El diagnóstico es eminentemente clínico y se debe diferenciar de otras patologías que producen aumento de volumen de las extremidades, especialmente el linfedema y obesidad. Es importante realizar un estudio funcional del sistema linfático cuando el diagnóstico es dudoso o para la etapificación del lipedema, por lo que la correcta interpretación de estos resultados es fundamental. El tratamiento está enfocado en disminuir la discapacidad y evitar la progresión, con el fin de mejorar la calidad de vida. Actualmente, la liposucción es un tratamiento efectivo para el lipedema, sin embargo, las técnicas empleadas para la lipectomía en el lipedema son diferentes a las técnicas utilizadas para la liposucción con fines estéticos. Las técnicas selectivas que respetan los vasos linfáticos tienen mejor rendimiento para reducir el volumen de grasa, retrasar la progresión, reducir el dolor, reducir la alteración marcha y mejorar la calidad de vida en estos pacientes.


Lipedema is a common, frequently under-diagnosed, chronic and progressive disease that generates an important detriment in quality of life. It consists in an abnormal deposit of subcutaneous adipose tissue mainly in the lower extremities, almost exclusively affecting women. Its main symptoms are pain, sensitivity and the ease of causing bruising. From the pathophysiological point of view, there would be a polygenic susceptibility combined with hormonal, microvascular and lymphatic disorders that may be partly responsible for the development of lipedema. It is classified according to the distribution of fat into five types and, according to the severity of the disease, in four stages. The diagnosis is eminently clinical and must be differentiated from other diseases that cause an increase in the volume of the extremities, especially lymphedema and obesity. It is important to carry out a study of the lymphatic system functionality when the diagnosis is not clear or for lipedema staging, so the correct interpretation of these results is essential. Treatment is focused on reducing disability and preventing progression, in order to improve quality of life. Liposuction is currently an effective treatment for lipedema, however, the techniques used for lipectomy in lipedema are different from the techniques used for liposuction for cosmetic purposes. Selective techniques that spare the lymphatic vessels have better results reducing fat volume, delaying progression, reducing pain, reducing gait disturbance, and improving quality of life of these patients.


Subject(s)
Humans , Lipedema/surgery , Lipedema/diagnosis , Lipedema/physiopathology , Quality of Life , Lymphedema/physiopathology
8.
Am J Med Genet A ; 185(7): 2153-2159, 2021 07.
Article in English | MEDLINE | ID: mdl-33851505

ABSTRACT

Hypotrichosis-lymphedema-telangiectasia syndrome (HLTS) is a rare condition caused by pathogenic variants in the SOX18 gene. SOX18 plays a key role in angio- and lymphangiogenesis due to its expression in venous endothelial cells from which the lymphatic system develops. It is also expressed in embryonic hair follicles, heart, and vascular smooth muscle cells. The main clinical symptoms of HLTS include sparse hair, alopecia totalis, lymphedema, most often affecting lower limbs, and telangiectatic lesions. Only 10 patients with a SOX18 pathogenic variant have been described that presented with additional features such as hydrocele, renal failure, arterial or pulmonary hypertension, aortic dilatation, and facial dysmorphism. Here, we summarize these phenotypic variations and report an additional HLTS patient, with a 14-nucleotide de novo duplication in SOX18 and congenital ileal atresia, a feature not previously associated with HLTS.


Subject(s)
Genetic Predisposition to Disease , Hypotrichosis/genetics , Lymphangiogenesis/genetics , Lymphedema/genetics , SOXF Transcription Factors/genetics , Telangiectasis/genetics , Adolescent , Child , Child, Preschool , Endothelial Cells/metabolism , Endothelial Cells/pathology , Female , Gene Duplication/genetics , Humans , Hypotrichosis/physiopathology , Infant , Infant, Newborn , Lymphedema/physiopathology , Male , Telangiectasis/physiopathology
9.
Physiol Rev ; 101(4): 1809-1871, 2021 10 01.
Article in English | MEDLINE | ID: mdl-33507128

ABSTRACT

Primary lymphedema is a long-term (chronic) condition characterized by tissue lymph retention and swelling that can affect any part of the body, although it usually develops in the arms or legs. Due to the relevant contribution of the lymphatic system to human physiology, while this review mainly focuses on the clinical and physiological aspects related to the regulation of fluid homeostasis and edema, clinicians need to know that the impact of lymphatic dysfunction with a genetic origin can be wide ranging. Lymphatic dysfunction can affect immune function so leading to infection; it can influence cancer development and spread, and it can determine fat transport so impacting on nutrition and obesity. Genetic studies and the development of imaging techniques for the assessment of lymphatic function have enabled the recognition of primary lymphedema as a heterogenic condition in terms of genetic causes and disease mechanisms. In this review, the known biological functions of several genes crucial to the development and function of the lymphatic system are used as a basis for understanding normal lymphatic biology. The disease conditions originating from mutations in these genes are discussed together with a detailed clinical description of the phenotype and the up-to-date knowledge in terms of disease mechanisms acquired from in vitro and in vivo research models.


Subject(s)
Lymphatic System/growth & development , Lymphatic System/physiology , Lymphedema/genetics , Animals , Humans , Lymphangiogenesis/genetics , Lymphangiogenesis/physiology , Lymphatic Vessels/physiopathology , Lymphedema/physiopathology
11.
J Vasc Surg Venous Lymphat Disord ; 9(3): 811-819.e2, 2021 05.
Article in English | MEDLINE | ID: mdl-33309902

ABSTRACT

OBJECTIVE: Lymphedema is a chronic, progressive and burdensome disease that is known to have a substantial impact on quality of life (QOL). Hence, the assessment of QOL is an important aspect of any study which seeks to evaluate outcomes after lymph reconstructive surgery. We therefore aimed to analyze currently available patient-reported outcome measurements (PROMS) for patients with lower limb lymphedema (LLL) with regard to their psychometric properties. Furthermore, we intended to evaluate the change in QOL in patient undergoing lymphovenous anastomosis (LVA) or vascularized lymph node transfer (VLNT). METHODS: A literature research of four databases on studies that included PROMS for LLL was conducted. All selected studies were assessed for validity according Consensus-based Standards for the selection of Health Measurement Instruments. Studies that used the Lymph Quality of Life Measure for Limb Lymphedema (LYMQOL) after LVA and/or VLNT were included for quantitative analysis. RESULTS: In total, 988 studies were screened, of which 30 studies were included in this review. We identified six validated PROMS to assess LLL. Of those, the LYMQOL was the most commonly used questionnaire. Based on the LYMQOL, a significant improvement of QOL in LLL was noted in all studies after reconstructive lymph surgery (LVA/VLNT) regardless of lymphedema etiology, stage, or time since diagnosis. On the basis of the Consensus-based Standards for the selection of Health Measurement Instruments criteria, the Lymphoedema Functioning, Disability and Health Questionnaire for Lower Limb Lymphoedema seems to be superior in terms of its psychometric properties. CONCLUSIONS: A significant improvement in the QOL in patients with LLL after reconstructive lymph surgery can be observed. Future studies on reconstructive lymph surgery need to include both objective and congruent volume measurements, as well as data on QOL based a well-validated PROM such as the Lymphoedema Functioning, Disability and Health Questionnaire for Lower Limb Lymphoedema.


Subject(s)
Lower Extremity/surgery , Lymph Nodes/transplantation , Lymphatic Vessels/surgery , Lymphedema/surgery , Patient Reported Outcome Measures , Plastic Surgery Procedures , Quality of Life , Adult , Anastomosis, Surgical , Disability Evaluation , Female , Functional Status , Humans , Lower Extremity/diagnostic imaging , Lower Extremity/physiopathology , Lymph Nodes/diagnostic imaging , Lymph Nodes/physiopathology , Lymphatic Vessels/diagnostic imaging , Lymphatic Vessels/physiopathology , Lymphedema/diagnosis , Lymphedema/physiopathology , Male , Middle Aged , Predictive Value of Tests , Psychometrics , Plastic Surgery Procedures/adverse effects , Reproducibility of Results , Time Factors , Treatment Outcome
12.
Gynecol Oncol ; 160(1): 244-251, 2021 01.
Article in English | MEDLINE | ID: mdl-33109392

ABSTRACT

OBJECTIVE: To assess quality of life (QOL) in patients who developed lower-extremity lymphedema (LLE) after radical gynecologic cancer surgery on prospective clinical trial GOG 244. METHODS: The prospective, national, cooperative group trial GOG-0244 determined the incidence of LLE and risk factors for LLE development, as well as associated impacts on QOL, in newly diagnosed patients undergoing surgery for endometrial, cervical, or vulvar cancer from 6/4/2012-11/17/2014. Patient-reported outcome (PRO) measures of QOL (by the Functional Assessment of Cancer Therapy [FACT]), body image, sexual and vaginal function, limb function, and cancer distress were recorded at baseline (within 14 days before surgery), and at 6, 12, 18, and 24 months after surgery. Assessments of LLE symptoms and disability were completed at the time of lower limb volume measurement. A linear mixed model was applied to examine the association of PROs/QOL with a Gynecologic Cancer Lymphedema Questionnaire (GCLQ) total score incremental change ≥4 (indicative of increased LLE symptoms) from baseline, a formal diagnosis of LLE (per the GCLQ), and limb volume change (LVC) ≥10%. RESULTS: In 768 evaluable patients, those with a GCLQ score change ≥4 from baseline had significantly worse QOL (p < 0.001), body image (p < 0.001), sexual and vaginal function (p < 0.001), limb function (p < 0.001), and cancer distress (p < 0.001). There were no significant differences in sexual activity rates between those with and without LLE symptoms. CONCLUSIONS: LLE is significantly detrimental to QOL, daily function, and body image. Clinical intervention trials to prevent and manage this chronic condition after gynecologic cancer surgery are needed.


Subject(s)
Genital Neoplasms, Female/surgery , Lymphedema/physiopathology , Lymphedema/psychology , Adult , Aged , Aged, 80 and over , Female , Humans , Leg/pathology , Middle Aged , Postoperative Complications/etiology , Postoperative Complications/physiopathology , Postoperative Complications/psychology , Prospective Studies , Quality of Life
13.
Cardiovasc Res ; 117(8): 1877-1890, 2021 07 07.
Article in English | MEDLINE | ID: mdl-33067626

ABSTRACT

Vascular endothelial growth factor receptors (VEGFRs) are part of the evolutionarily conserved VEGF signalling pathways that regulate the development and maintenance of the body's cardiovascular and lymphovascular systems. VEGFR3, encoded by the FLT4 gene, has an indispensable and well-characterized function in development and establishment of the lymphatic system. Autosomal dominant VEGFR3 mutations, that prevent the receptor functioning as a homodimer, cause one of the major forms of hereditary primary lymphoedema; Milroy disease. Recently, we and others have shown that FLT4 variants, distinct to those observed in Milroy disease cases, predispose individuals to Tetralogy of Fallot, the most common cyanotic congenital heart disease, demonstrating a novel function for VEGFR3 in early cardiac development. Here, we examine the familiar and emerging roles of VEGFR3 in the development of both lymphovascular and cardiovascular systems, respectively, compare how distinct genetic variants in FLT4 lead to two disparate human conditions, and highlight the research still required to fully understand this multifaceted receptor.


Subject(s)
Cardiovascular System/metabolism , Heart Defects, Congenital/metabolism , Lymphatic System/metabolism , Lymphedema/metabolism , Vascular Endothelial Growth Factor Receptor-3/metabolism , Animals , Cardiovascular System/pathology , Cardiovascular System/physiopathology , Disease Models, Animal , Gene Expression Regulation, Developmental , Heart Defects, Congenital/genetics , Heart Defects, Congenital/pathology , Heart Defects, Congenital/physiopathology , Humans , Lymphatic System/pathology , Lymphatic System/physiopathology , Lymphedema/genetics , Lymphedema/pathology , Lymphedema/physiopathology , Mice, Transgenic , Morphogenesis , Mutation , Signal Transduction , Vascular Endothelial Growth Factor Receptor-3/genetics
14.
Asian J Surg ; 44(1): 169-173, 2021 Jan.
Article in English | MEDLINE | ID: mdl-32513633

ABSTRACT

OBJECTIVE: In the conventional Charles' procedure for lower-limb lymphedema, full-thickness skin grafts (FTSGs) or flaps are the preferred treatment for areas around the knee and ankle because of the belief that FTSGs or flaps result in slighter contracture relative to split-thickness skin grafts (STSGs). However, the use of FTSGs or flaps prolongs operation and increases the risk of partial graft loss; should partial graft loss occur, additional grafting is required for remnant defects to avoid significant scarring after secondary healing. The senior author (HCC) thus modified the Charles' procedure and used STSGs around the knee and ankle. The aim of this study was to elucidate the long-term outcomes of STSGs in HCC-modified Charles' procedure, including its attendant complications, such as joint contracture, range-of-motion limitations, and the presence of hypertrophic scars. METHODS: Participants were patients (n = 142) who underwent HCC-modified Charles' procedure and STSGs between 1990 and 2016 for advanced lymphedema; the follow-up was at least 3 years. We detail our modification for improving the take of STSGs in the first operation and the rehabilitation protocol. RESULTS: The active flexion of knee was >90° in 89.4% and 70°-90° in 10.6% of patients. The active plantar flexion of ankle was 30° in 90.8% and 20°-30° in 9.2% of patients. In Stiefel Grading System, 85.9% were "Excellent," 12.0% were "Good," 2.1% were "Fair," and 0 were "Poor." CONCLUSION: STSGs in HCC-modified Charles' procedure yield satisfactory outcomes without joint contracture. Early physiotherapy and the primary take of STSGs are crucial to good functional outcomes.


Subject(s)
Ankle Joint/surgery , Knee Joint/surgery , Lymphedema/surgery , Skin Transplantation/methods , Adolescent , Adult , Aged , Cicatrix, Hypertrophic/epidemiology , Cicatrix, Hypertrophic/etiology , Female , Humans , Knee Joint/physiopathology , Lymphedema/physiopathology , Lymphedema/rehabilitation , Male , Middle Aged , Physical Therapy Modalities , Range of Motion, Articular , Recovery of Function , Skin Transplantation/adverse effects , Surgical Flaps , Time Factors , Treatment Outcome , Young Adult
15.
Braz J Phys Ther ; 25(2): 203-213, 2021.
Article in English | MEDLINE | ID: mdl-32518025

ABSTRACT

BACKGROUND: Lymphoedema is a chronic condition that has significant detrimental impact on patients' quality of life. Secondary lymphoedema often results from anti-tumour treatment, in contrast to primary lymphoedema which is the result of genetic abnormalities that leads to an abnormal development of the lymphatic system. OBJECTIVE: To describe and compare the experience of individuals with primary and secondary lymphoedema. METHODS: A total of 19 patients (mean ±â€¯standard deviation age: 56.7 ±â€¯16.2 years), 8 with primary and 11 with secondary lymphoedema, participated in this qualitative phenomenological study. Purposeful sampling method was applied. We recruited participants from specialised lymphoedema units of two physical therapy clinics and the Lymphoedema Patient Association in Spain. Data collection methods included unstructured and semi-structured interviews. An inductive thematic analysis was used. RESULTS: The findings suggest differences between the experience of living with primary or secondary lymphoedema. Also, those with lower extremity lymphoedema have more pain, fatigue, and functional limitations. Three primary themes emerged: "Emotional challenge", "Adapting your life to a new situation," and "lymphoedema management". CONCLUSIONS: The experience of living with lymphoedema involves aspects of fear, suffering, coping, and management of the disease and differs between secondary and primary causes. However, it seems to be more dependent on its location than its cause.


Subject(s)
Lymphedema/physiopathology , Chronic Disease , Fatigue , Humans , Lymphedema/genetics , Middle Aged , Qualitative Research , Quality of Life/psychology , Spain
16.
J Vasc Surg Venous Lymphat Disord ; 9(2): 482-488, 2021 03.
Article in English | MEDLINE | ID: mdl-32599305

ABSTRACT

OBJECTIVE: The objective of this study was to explore the pathologic process underlying primary lymphedema. METHODS: Twenty-seven patients with unilateral congenital arm lymphedema who visited our clinic from January 1, 2014, to May 30, 2019, were enrolled. The patients' clinical signs and the findings of indocyanine green (ICG) lymphography, skin tissue immunohistochemical staining, and whole exome sequencing of tissue and blood were evaluated. RESULTS: Among the 27 patients, 11 were diagnosed with stage II and 16 were diagnosed with stage III lymphedema. No lymphatic vessels were visualized in the affected arm in 25 of 27 (93%) patients who underwent ICG lymphography; likewise, no lymphatics were found in the territories of axillary lymph node drainage in the trunk, irrespective of any anomalies of the axillary lymph nodes. In only two (7%) patients, an unclear lymphatic trunk gradually appeared in the dorsum of the affected hand. The number of initial lymphatics was increased in the skin specimens of all nine patients in whom lymphatics were not demonstrated by ICG lymphography. Among 14 tested patients, we found compound heterozygote variants in the PIEZO1 gene in only one (7%) patient. Two missense variants, c.4072C>T; p.Arg1358Cys and c.5033C>T; p. Ala1678Val, were identified and found to have been inherited from the father and mother, respectively. No other pathogenic or likely pathogenic variants of currently known lymphedema-related genes were identified in the remaining 13 patients. No genetic difference was found between the lymphedematous and nonedematous healthy skin tissue of the same person. CONCLUSIONS: Segmental or regional dysfunction of the dermal initial lymphatics causes congenital arm lymphedema and may have implications for clinical treatment.


Subject(s)
Lymphatic System/physiopathology , Lymphedema/physiopathology , Skin Abnormalities/physiopathology , Adolescent , Adult , Child , Child, Preschool , Female , Fluorescent Dyes , Genetic Predisposition to Disease , Heredity , Humans , Immunohistochemistry , Indocyanine Green , Infant , Ion Channels/genetics , Lymphatic System/abnormalities , Lymphatic System/diagnostic imaging , Lymphedema/congenital , Lymphedema/diagnosis , Lymphography , Male , Middle Aged , Mutation, Missense , Pedigree , Phenotype , Retrospective Studies , Skin Abnormalities/diagnosis , Skin Abnormalities/genetics , Upper Extremity , Exome Sequencing , Young Adult
17.
J Vasc Surg Venous Lymphat Disord ; 9(2): 471-481.e1, 2021 03.
Article in English | MEDLINE | ID: mdl-32470617

ABSTRACT

OBJECTIVE: Although the development of lymphatic collaterals is expected following lymphedema, little is known about the anatomic details of such compensatory pathways or their association with symptoms. Magnetic resonance lymphangiography (MRL) has been shown to be superior to lymphoscintigraphy and indocyanine green lymphography in visualizing lymphatics. This study aimed to analyze MRL images of lower limbs to elucidate the patterns of lymphatic collateral formation and their association with the clinical stages of lymphedema. METHODS: We enrolled 56 consecutive patients (112 lower limbs) with lymphedema who underwent MRL. Two radiologists performed a consensus reading of MRL images for the presence or absence of collateral lymphatic pathways, and the results were compared with the clinical stages. Furthermore, the frequency of abnormal MRL findings in 43 asymptomatic lower limbs of patients with unilateral lymphedema was analyzed and compared with that in the 69 symptomatic lower limbs of the patients. The imaging findings were also compared with the cause of lymphedema. RESULTS: All three collateral pathways (anterolateral, deep, and posteromedial lymphatics) were visualized at a higher (P < .05) frequency in stage II than in stage 0 lower limbs. The frequency of visualization of the three collaterals was significantly higher in symptomatic (stages I-III) lower limbs than in asymptomatic (stage 0) lower limbs. Most (76.8%) of the symptomatic limbs exhibited at least one of these collaterals, and the frequency was significantly higher than in the asymptomatic limbs (P < .001). Most (81.4%) of the asymptomatic (stage 0) lower limbs had at least one abnormal finding in terms of lymphatic circulation, although this proportion was significantly lower compared with the symptomatic limbs (98.6%). The collaterals tended to appear less frequently in primary lymphedema than in secondary lymphedema, reaching statistical significance in the posteromedial lymphatics. CONCLUSIONS: These results suggested that the two superficial lymphatic groups and the deep lymphatic system act as major collaterals of the lower limbs in patients with lymphedema. Furthermore, MRL of most patients with unilateral lymphedema demonstrated abnormal findings, including collateral formation, not only in the affected lower limb but also in the asymptomatic lower limb. In primary lymphedema, the collaterals may appear less frequently than in secondary lymphedema. Collaterals should be taken into consideration in planning the site of lymphaticovenous anastomosis and assessing disease progression. MRL can visualize preclinical alterations in lymphatic flow and compensatory pathways; therefore, we expect that it will be useful for the early diagnosis of lymphedema.


Subject(s)
Lymphatic Vessels/diagnostic imaging , Lymphedema/diagnostic imaging , Lymphography , Magnetic Resonance Imaging , Adolescent , Adult , Aged , Aged, 80 and over , Cross-Sectional Studies , Female , Humans , Lower Extremity , Lymphatic Vessels/physiopathology , Lymphedema/etiology , Lymphedema/physiopathology , Male , Middle Aged , Predictive Value of Tests , Retrospective Studies , Young Adult
18.
J Vasc Surg Venous Lymphat Disord ; 9(3): 745-752, 2021 05.
Article in English | MEDLINE | ID: mdl-33137494

ABSTRACT

OBJECTIVE: Lymphedema is associated with significant morbidity and healthcare resource usage. Conventional therapy efficacy has been limited, with poor surgical salvage options. Preliminary studies have demonstrated that the use of advanced pneumatic compression devices (APCDs) improves clinical outcomes. However, limited evidence regarding their role in healthcare cost mitigation or health-related quality of life (QOL) is available. METHODS: The present postmarket, multicenter, single-arm, observational clinical trial conducted in the Veterans Affairs Healthcare System evaluated patients with a diagnosis of primary or secondary edema of unilateral or bilateral lower extremities treated with the Flexitouch APCD (Tactile Medical, Minneapolis, Minn) from February 2016 to March 2019. The patients were assessed at baseline and 12, 24, and 52 weeks from enrollment by limb circumference, QOL assessments (short form-36 and Lymphedema Quality of Life), device compliance, cellulitis episodes, and lymphedema-related healthcare use since the previous visit. The primary endpoints of interest were the QOL at baseline compared with at 12 weeks, unscheduled lymphedema-related clinic visits, and hospital admissions at 52 weeks. The secondary endpoints included the change in limb girth and QOL at 52 weeks compared with baseline. RESULTS: A total of 178 patients with lower extremity lymphedema were prospectively enrolled. The present study reports the interim data for the first 74 subjects to complete 52 weeks of APCD treatment. The cohort was predominately male (94.6%), elderly (mean age, 67 years), obese (median body mass index, 32 kg/m2), and most commonly enrolled for the treatment of phlebolymphedema (71.6%) with largely bilateral lower extremity involvement (91.9%). No significant difference was seen in QOL at 12 weeks. However, at 52 weeks, the Lymphedema Quality of Life scores had significantly improved from baseline (6.3 vs 7.4; P < .0001) and the short form-36 had demonstrated significant improvement from baseline in the physical component (38.6 vs 40.8; P = .035), with an effect toward overall improvement in the mental component (49.9 vs 51.3; P = .549). The limb circumference had decreased significantly at 12 weeks compared with baseline (28.5 cm vs 27.7 cm; P = .0005) in the most affected lower extremity, and this reduction had remained stable for the study duration. APCD treatment was associated with a significant reduction in cellulitis episodes (24.3% vs 8.1%; P = .005), lymphedema-related clinic visits (2.2 vs 0.7; P = .02), urgent care visits (1.2 vs 0.3; P = .004), and hospital admissions (0.5 vs 0.1; P = .047) per patient. CONCLUSIONS: The Flexitouch APCD resulted in initial significant limb girth reduction as early as 12 weeks and a steady and sustained improvement in health-related QOL for ≤1 year. The latter was likely reflective of a decrease in cellulitis episodes and fewer associated lymphedema-related clinic and urgent care visits and hospital admissions.


Subject(s)
Cellulitis/therapy , Intermittent Pneumatic Compression Devices , Lower Extremity/physiopathology , Lymphedema/therapy , Quality of Life , Aged , Cellulitis/pathology , Cellulitis/physiopathology , Female , Home Care Services , Humans , Lower Extremity/pathology , Lower Extremity/physiology , Lymphedema/pathology , Lymphedema/physiopathology , Male , Middle Aged , Product Surveillance, Postmarketing , Prospective Studies , Remission Induction , Surveys and Questionnaires , Time Factors , Treatment Outcome , United States , Veterans Health Services
19.
Lymphology ; 53(3): 109-117, 2020.
Article in English | MEDLINE | ID: mdl-33350285

ABSTRACT

Lymphedema arises due to a malfunction of the lymphatic system and can lead to massive tissue swelling. Complete decongestive therapy (CDT), consisting of manual lymphatic drainage (MLD) and compression bandaging, is aimed at mobilizing fluid and reducing volume in affected extremities. Lymphatic dysfunction has previously been associated with chronic inflammation processes. We investigated plasma ADMA as an indicator of endothelial function/inflammation before-, during- and after-CDT. Also assessed were vascular function parameters such as carotid-femoral pulse wave velocity (PWVcf), flow-mediated dilata-tion (FMD) and retinal microvasculature analysis. 13 patients (3 males and 10 females, 57 ± 8 years old (mean ± SD), 167.2 ± 8.3 cm height, 91.0 ± 23.5 kg weight), with lower limb lymphedema were included. Vascular function parameters were assessed on day 1, 2, 7, 14 and 21 of CDT, pre- and post-MLD. ADMA was significantly lower post-MLD (p=0.0064) and tended to reduce over three weeks of therapy (p=0.0506). PWVcf weakly correlated with FMD (r=0.361, p=0.010). PWVcf, FMD and retinal microvasculature analysis did not show changes due to physical therapy. The novel results from this study indicate that lymphedema does not affect endothelial func-tion and lymphedema patients may therefore not have a higher risk of cardiovas-cular diseases. Our results further suggest that manual lymphatic drainage with or without full CDT could have potentially beneficial effects on endothelial function in lymphedema patients (by reducing ADMA levels), which has not been reported previously.


Subject(s)
Endothelium/metabolism , Lymphedema/metabolism , Lymphedema/therapy , Physical Therapy Modalities , Aged , Compression Bandages , Endothelium/physiopathology , Female , Humans , Lymphedema/etiology , Lymphedema/physiopathology , Male , Manual Lymphatic Drainage , Middle Aged , Pulse Wave Analysis , Treatment Outcome
20.
Adv Skin Wound Care ; 33(11): 570-580, 2020 Nov.
Article in English | MEDLINE | ID: mdl-33065678

ABSTRACT

GENERAL PURPOSE: To present a comprehensive review of lymphedema, including its pathophysiology, assessment, diagnosis, and treatment. TARGET AUDIENCE: This continuing education activity is intended for physicians, physician assistants, nurse practitioners, and nurses with an interest in skin and wound care. LEARNING OBJECTIVES/OUTCOMES: After participating in this educational activity, the participant will:1. Summarize the etiology, pathophysiology, and clinical manifestations of lymphedema.2. Describe the diagnostic and treatment approaches for patients who have lymphedema. ABSTRACT: Lymphedema is a condition characterized by localized protein-rich swelling caused by damaged or malfunctioning lymphatics. Patients with lymphedema have an increased risk of infection because of the lymphostatic nature of the disease. Chronic ulceration of the skin can make individuals vulnerable to infection leading to serious, sometimes fatal, complications. Proper diagnosis and treatment modalities can aid in the prevention of these complications and ensure better outcomes for the patient.


Lymphedema is a condition characterized by localized protein-rich swelling caused by damaged or malfunctioning lymphatics. Patients with lymphedema have an increased risk of infection because of the lymphostatic nature of the disease. Chronic ulceration of the skin can make individuals vulnerable to infection leading to serious, sometimes fatal, complications. Proper diagnosis and treatment modalities can aid in the prevention of these complications and ensure better outcomes for the patient.


Subject(s)
Clinical Competence , Inservice Training , Lymphedema/diagnostic imaging , Lymphedema/therapy , Attitude of Health Personnel , Humans , Lymphedema/physiopathology , Physical Examination
SELECTION OF CITATIONS
SEARCH DETAIL
...