Primary Lymphedema of the Upper Extremities: Clinical and Lymphoscintigraphic Features in 23 Patients.

Abstract:

BACKGROUND:Primary idiopathic lymphedema is an uncommon condition that typically affects the lower extremities. Patients have a malformed lymphatic system that causes subcutaneous fluid and adipose deposition. Rarely, the disease also has been described in the upper extremities. The purpose of this study was to investigate a cohort of patients with primary arm lymphedema to better understand the disease. METHODS:Patients evaluated in our Lymphedema Program between 2008 and 2018 were reviewed for individuals with upper extremity primary lymphedema. Gender, age of onset, morbidity, associated features, and management were identified. Transit of radiolabeled tracer and dermal backflow on lymphoscintigraphy were recorded. RESULTS:Twenty-three patients of 234 individuals with primary lymphedema had upper extremity disease (9.8%). Eleven subjects were male. Age of onset was infancy (n = 15), adolescence (n = 5), or adulthood (n = 3). The disease affected the left arm (n = 11), right arm (n = 9), or both upper extremities (n = 3). Lymphoscintigraphy in 15 patients exhibited delayed transit of tracer and 2 illustrated dermal backflow. One-half of individuals also had primary lower extremity lymphedema (six unilateral and six bilateral). None of the patients in the cohort exhibited a family history of lymphedema. Two individuals had Turner syndrome. Morbidity included infection (n = 5), other lymphatic anomalies (n = 6), and lymphangiosarcoma (n = 1). CONCLUSIONS:The upper extremities are a rare location for primary lymphedema and patients often also have lymphedema of the legs. Compared with that of the lower extremities, primary disease of the arm is more likely to be associated with systemic lymphatic dysfunction and has a lower risk of familial transmission.

journal_name

Lymphat Res Biol

authors

Goss JA,Maclellan RA,Greene AK

doi

10.1089/lrb.2017.0085

subject

Has Abstract

pub_date

2019-02-01 00:00:00

pages

40-44

issue

1

eissn

1539-6851

issn

1557-8585

journal_volume

17

pub_type

杂志文章
  • Does Lymphedema Severity Affect Quality of Life? Simple Question. Challenging Answers.

    abstract:PURPOSE:Lymphedema can have a negative impact on the function and quality of life (QOL) of patients, but most studies have examined lymphedema as a binary variable, rather than a multidimensional disease that ranges in severity. This study explored the potential impact of lymphedema severity on function and overall QOL...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章

    doi:10.1089/lrb.2016.0049

    authors: Lee TS,Morris CM,Czerniec SA,Mangion AJ

    更新日期:2018-02-01 00:00:00

  • Diagnosing Male Unilateral Upper Limb Lymphedema: Determination of Normatively Determined Thresholds Using Bioimpedance Spectroscopy.

    abstract:: Background: The use of bioimpedance spectroscopy (BIS) for the detection of unilateral upper limb lymphedema is growing. Currently, normatively determined diagnostic thresholds using bioimpedance are available for females only. It is unclear if they are suitable for males ...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章

    doi:10.1089/lrb.2018.0051

    authors: Xie AZT,Lee MJ,Dylke E

    更新日期:2020-02-01 00:00:00

  • Lymphatics in human lymphatic filariasis: in vitro models of parasite-induced lymphatic remodeling.

    abstract::Lymphatic filariasis characterized by the dysfunction of the lymphatics can lead to severe (and often) irreversible lymphedema and elephantiasis. Decades of research in the field shows that the establishment of the adult parasites in the lymphatics triggers a cascade of events that ultimately results in tissue scarrin...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章,评审

    doi:10.1089/lrb.2009.0022

    authors: Bennuru S,Nutman TB

    更新日期:2009-12-01 00:00:00

  • Serum Immune Proteins in Limb Lymphedema Reflecting Tissue Processes Caused by Lymph Stasis and Chronic Dermato-lymphangio-adenitis (Cellulitis).

    abstract:BACKGROUND:Lymphedema of limbs affects a large mass of tissues. Pathological changes develop in skin and subcutaneous tissue. Bacterial retention in edema fluid is followed by chronic inflammatory reaction. The question arises whether the chronic processes affecting a large mass of limb tissues are reflected in the ser...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章

    doi:10.1089/lrb.2017.0003

    authors: Zaleska MT,Olszewski WL

    更新日期:2017-09-01 00:00:00

  • Automated measurement of diameter and contraction waves of cannulated lymphatic microvessels.

    abstract:BACKGROUND:Studies of lymphatic function often employ collecting lymphatic vessels that exhibit large-amplitude, spontaneous contractions. Data from such preparations have been analyzed using cardiac pump analogies that require accurate determination of vascular dimensions, including external (OD) and internal (ID) dia...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章

    doi:10.1089/lrb.2006.4.3

    authors: Davis MJ,Zawieja DC,Gashev AA

    更新日期:2006-04-01 00:00:00

  • Extracellular RNA Profile in Mesenteric Lymph from Exemplar Rat Models of Acute and Critical Illness.

    abstract:: Background: Mesenteric lymph (ML) has been implicated in the development of multiple organ dysfunction syndrome in critical illness. Extracellular RNAs play a role in cell-to-cell communication during physiological and disease processes but they are rarely studied in ML. W...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章

    doi:10.1089/lrb.2018.0044

    authors: Hong J,Blenkiron C,Tsai P,Premkumar R,Nachkebia S,Tun SM,Petzer A,Windsor JA,Hickey AJ,Phillips AR

    更新日期:2019-10-01 00:00:00

  • The importance of lymphatics in cerebrospinal fluid transport.

    abstract::Despite the fact that the central nervous system parenchyma does not contain lymphatics, extracranial lymphatic vessels play a very important role in volumetric cerebrospinal fluid (CSF) transport. The most important extracranial location at which lymphatics gain access to CSF is in the nasal submucosa after CSF conve...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章,评审

    doi:10.1089/15396850360495682

    authors: Johnston M

    更新日期:2003-01-01 00:00:00

  • Postoperative evolution of thickness and echogenicity of cutis and subcutis of patients with and without breast cancer-related lymphedema.

    abstract:BACKGROUND:Compared to volume/circumference measurements to diagnose lymphedema, thickening and disturbance of echogenicity of cutis and subcutis associated with the development of lymphedema is measured directly by ultrasonography. The aim of the present study was to investigate the evolution of thickness and echogeni...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章

    doi:10.1089/lrb.2013.0028

    authors: Devoogdt N,Pans S,De Groef A,Geraerts I,Christiaens MR,Neven P,Vergote I,Van Kampen M

    更新日期:2014-03-01 00:00:00

  • Test of Responsiveness and Sensitivity of the Questionnaire "Lymphedema Quality of Life Inventory".

    abstract:BACKGROUND:Lymphedema may affect patients' health-related quality of life (HRQoL) and thus a disease-specific questionnaire is needed. The Lymphedema Quality of life Inventory (LyQLI) has been tested for reliability and validity. The purpose of this study was to test the LyQLI for responsiveness and sensitivity. METHO...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章,多中心研究

    doi:10.1089/lrb.2017.0048

    authors: Klernäs P,Johnsson A,Boyages J,Brorson H,Munnoch A,Johansson K

    更新日期:2018-06-01 00:00:00

  • Lymphatic Stomata in the Adult Human Pulmonary Ligament.

    abstract:BACKGROUND:Lymphatic stomata are small lymphatic openings in the serosal membrane that communicate with the serosal cavity. Although these stomata have primarily been studied in experimental mammals, little is known concerning the presence and properties of lymphatic stomata in the adult human pleura. Thus, adult human...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章

    doi:10.1089/lrb.2014.0009

    authors: Oshiro H,Miura M,Iobe H,Kudo T,Shimazu Y,Aoba T,Okudela K,Nagahama K,Sakamaki K,Yoshida M,Nagao T,Nakaya T,Kurata A,Ohtani O

    更新日期:2015-06-01 00:00:00

  • A Validation Study of Near-Infrared Fluorescence Imaging of Lymphatic Vessels in Humans.

    abstract:BACKGROUND:Near-infrared fluorescence (NIRF) imaging is a new imaging technique that is used to visualize lymphatic vessels in humans. It has a high spatial and temporal resolution, allowing real-time visualization of lymphatic flow. METHODS AND RESULTS:The current study investigated the intra- and inter-individual va...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章

    doi:10.1089/lrb.2016.0061

    authors: Groenlund JH,Telinius N,Skov SN,Hjortdal V

    更新日期:2017-09-01 00:00:00

  • Electrical Stimulation in the Treatment of Lymphedema and Associated Skin Ulcers.

    abstract:: Background: Lymphedema is a disorder in which lymph accumulates in the interstitial spaces due to poor lymphatic flow resulting from hypoplasia or aplasia of the lymphatic vessels, or to morpho-functional alterations that impair lymphatic flow. Lymphedema is a debilitating...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章

    doi:10.1089/lrb.2019.0052

    authors: Baglivo M,Martelli F,Paolacci S,Manara E,Michelini S,Bertelli M

    更新日期:2020-06-01 00:00:00

  • Lymphedema Leads to Fat Deposition in Muscle and Decreased Muscle/Water Volume After Liposuction: A Magnetic Resonance Imaging Study.

    abstract:BACKGROUND:Lymphedema leads to adipose tissue deposition. Water-fat magnetic resonance imaging (MRI) can quantify and localize fat and water. The presence of excess fat and excess water/muscle in the subfascial compartment of the lymphedematous limb has not been investigated before. The aim of this study was to investi...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章

    doi:10.1089/lrb.2017.0042

    authors: Hoffner M,Peterson P,Månsson S,Brorson H

    更新日期:2018-04-01 00:00:00

  • Lymphatic Endothelial Cells under Mechanical Stress: Altered Expression of Inflammatory Cytokines and Fibrosis.

    abstract:BACKGROUND:Secondary lymphedema, resulting from damage to lymphatic vessels, is a common sequela following surgical removal of lymph nodes for cancer. Current therapeutics for treating lymphedema are limited and further research on underlying causes is warranted. Published studies on molecular mechanisms of lymphedema ...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章

    doi:10.1089/lrb.2016.0042

    authors: Wang S,Nie D,Rubin JP,Kokai L

    更新日期:2017-06-01 00:00:00

  • Vascular Alterations in Axillary and Brachial Vessels in Patients with Axillary Web Syndrome After Breast Cancer Surgery.

    abstract:BACKGROUND:Surgical manipulations of the axilla may cause a condition known as Axillary Web Syndrome (AWS). The systems compromised and the sequence of events leading to this syndrome remains unknown. This study evaluated clinical, surgical, and vascular factors associated with onset and duration of AWS after breast ca...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章

    doi:10.1089/lrb.2017.0037

    authors: Furlan C,Matheus CN,Jales RM,Derchain S,Sarian LO

    更新日期:2018-06-01 00:00:00

  • Pathophysiological aspects of lymphedema of human limbs: I. Lymph protein composition.

    abstract::The knowledge of humoral and cellular factors retained in tissues affected by ineffective lymph transport is necessary to comprehend the pathological mechanisms of the evolving changes in the extremity. In this article, we report our observations on changes in the protein composition of lymph in obstructive lymphedema...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章

    doi:10.1089/153968503768330265

    authors: Olszewski WL

    更新日期:2003-01-01 00:00:00

  • Low Lymphatic Pumping Pressure in the Legs Is Associated with Leg Edema and Lower Quality of Life in Healthy Volunteers.

    abstract:BACKGROUND:Lymph is pumped through the collecting lymphatic vessels by both intrinsic and extrinsic forces, propelling it downstream back into circulation. The intrinsic lymph pump relies on the spontaneous contraction of lymphatic muscles to generate the force of pumping lymph (P(lp)). However, the association between...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章

    doi:10.1089/lrb.2014.0015

    authors: Saito T,Unno N,Yamamoto N,Inuzuka K,Tanaka H,Sano M,Sugisawa R,Katahashi K,Konno H

    更新日期:2015-06-01 00:00:00

  • Responsiveness of the Lymphedema Functioning, Disability, and Health Questionnaire for Upper Limb Lymphedema in Patients with Breast Cancer-Related Lymphedema.

    abstract:: Background: The Lymphedema Functioning, Disability, and Health questionnaire for Upper Limb Lymphedema (Lymph-ICF-UL) is a health-related quality-of-life questionnaire for patients with breast cancer-related lymphedema. Previous testing of this questionnaire showed very go...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章

    doi:10.1089/lrb.2019.0073

    authors: De Vrieze T,Gebruers N,Nevelsteen I,Tjalma WAA,Thomis S,De Groef A,Dams L,Devoogdt N

    更新日期:2020-08-01 00:00:00

  • A Study Using Visual Art Methods to Explore the Perceptions and Barriers of Self-Management in Children and Adolescents with Lymphedema.

    abstract:: Background: The aims of this study were to explore, using visual art methodology, how children and adolescents perceive their lymphedema and conceptualize the barriers and enablers in self-management and to explore the role of an educational camp in promoting self-efficacy...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章,多中心研究

    doi:10.1089/lrb.2018.0075

    authors: Moffatt C,Aubeeluck A,Stasi E,Mestre S,Rowan S,Murray S,Quere I

    更新日期:2019-04-01 00:00:00

  • Quality of Life Improvements in Patients with Lymphedema After Surgical or Nonsurgical Interventions with 1-Year Follow-Up.

    abstract:: Background: Lymphedema may impact patients' health-related quality of life (HRQoL). The purpose of this study was to evaluate HRQoL after two different treatments to the 12-month follow-up point. Methods and Results: Study part...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章

    doi:10.1089/lrb.2018.0065

    authors: Klernäs P,Johnsson A,Boyages J,Brorson H,Munnoch A,Johansson K

    更新日期:2020-08-01 00:00:00

  • Outcomes in Vascularized Lymph Node Transplantation in Rabbits: A Reliable Model for Improving the Surgical Approach to Lymphedema.

    abstract:: Background: Conservative nonsurgical treatment has traditionally been applied to lymphedema. Early surgical procedures were invasive and disfiguring and their long-term success was often limited. In contrast, contemporary surgical techniques are much less invasive and have...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章

    doi:10.1089/lrb.2018.0038

    authors: Fernández Peñuela R,Casaní Arazo L,Masiá Ayala J

    更新日期:2019-08-01 00:00:00

  • Clinical Benefits of Supervised Home Care of the Morbidity Management and Disability Prevention Under National Filariasis Elimination Programme.

    abstract:: Introduction: Mass drug administration and morbidity management and disability prevention (MMDP) though considered as two pillars of global program for elimination of filariasis, implementation of MMDP was disregarded in several endemic countries. Limb hygiene is the main ...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章

    doi:10.1089/lrb.2019.0058

    authors: De Britto L,Kamaraj P,Vijayalakshmi G,Das LK,Krishnakumari A,Anbusivam S,Supriya VK,Yuvaraj J

    更新日期:2020-12-01 00:00:00

  • Blood Flow Velocity in Brachial and Subclavian Vessels Immediately After Compressive Procedures for Treatment of Postcancer Therapy Lymphedema in Breast Cancer: A Randomized Blind Clinical Trial.

    abstract:OBJECTIVE:This study sought to evaluate the effect of elastic compression, functional compressive bandaging, and kinesiotherapy on blood flow of the upper limb with lymphedema secondary to the treatment of breast cancer. METHODS:This was a randomized blind crossover clinical trial with a washout period of 7 days betwe...

    journal_title:Lymphatic research and biology

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.1089/lrb.2016.0022

    authors: Rezende MS,Marsengo AL,de Jesus Guirro RR,de Oliveira Guirro EC

    更新日期:2017-03-01 00:00:00

  • Abdominal lymphatic pump treatment increases leukocyte count and flux in thoracic duct lymph.

    abstract:BACKGROUND:Previous studies suggest that rhythmic compression of the abdomen (abdominal lymphatic pump techniques, LPT) enhances immunity and resistance to infectious disease, but direct evidence of this has not been documented. In this study, the thoracic duct of eight anesthetized mongrel dogs was catheterized, so th...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章

    doi:10.1089/lrb.2007.1001

    authors: Hodge LM,King HH,Williams AG Jr,Reder SJ,Belavadi T,Simecka JW,Stoll ST,Downey HF

    更新日期:2007-01-01 00:00:00

  • Assessment of breast cancer-related lymphedema: a comparison of moisture meter and spot bioimpedance measurement.

    abstract:BACKGROUND:Tissue dielectric constant (TDC) and spot bioimpedance measurement (BIA) have a role in the assessment of tissue composition changes in breast cancer-related lymphedema (BCRL). Our aims were to determine whether TDC and spot BIA measures could detect inter-limb differences in BCRL, explore the relationship b...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章

    doi:10.1089/lrb.2014.0032

    authors: Czerniec SA,Ward LC,Kilbreath SL

    更新日期:2015-03-01 00:00:00

  • Developmental angiogenesis of the central nervous system.

    abstract::The vasculature of the central nervous system (CNS) is highly specialized with a blood-brain-barrier, reciprocal neuroepithelial-endothelial cell interactions and extensive pericyte coverage. Developmentally, numerous important signaling pathways participate in CNS angiogenesis to orchestrate the precise timing and sp...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章,评审

    doi:10.1089/lrb.2008.1014

    authors: Mancuso MR,Kuhnert F,Kuo CJ

    更新日期:2008-01-01 00:00:00

  • Determining the precision of dual energy x-ray absorptiometry and bioelectric impedance spectroscopy in the assessment of breast cancer-related lymphedema.

    abstract:BACKGROUND:The composition of breast cancer-related lymphedema (BCRL) has been shown to evolve from the initial accumulation of fluid to the development of fibrotic lesions and abnormal fat deposition. Therefore, precise and reliable assessments of BCRL are required to develop accurate staging and management. Although ...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章

    doi:10.1089/lrb.2012.0020

    authors: Newman AL,Rosenthall L,Towers A,Hodgson P,Shay CA,Tidhar D,Vigano A,Kilgour RD

    更新日期:2013-06-01 00:00:00

  • Passive pressure-diameter relationship and structural composition of rat mesenteric lymphangions.

    abstract:BACKGROUND:Lymph flow depends on both the rate of lymph production by tissues and the extent of passive and active pumping. Here we aim to characterize the passive mechanical properties of a lymphangion in both mid-lymphangion and valve segments to assess regional differences along a lymphangion, as well as evaluating ...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章

    doi:10.1089/lrb.2011.0015

    authors: Rahbar E,Weimer J,Gibbs H,Yeh AT,Bertram CD,Davis MJ,Hill MA,Zawieja DC,Moore JE Jr

    更新日期:2012-12-01 00:00:00

  • Spontaneous electrical activity in sheep mesenteric lymphatics.

    abstract:BACKGROUND:It has recently become apparent that the lymph pump is an electrical entity that rivals the heart in complexity. Many interesting currents have been demonstrated by voltage clamping isolated lymphatic smooth muscle cells, but until now the role of these currents in the intact syncitium has not been studied. ...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章

    doi:10.1089/lrb.2007.5104

    authors: Beckett EA,Hollywood MA,Thornbury KD,McHale NG

    更新日期:2007-01-01 00:00:00

  • HGF and MET mutations in primary and secondary lymphedema.

    abstract:BACKGROUND:Lymphedema is the abnormal accumulation of protein-rich fluid in the interstitial space. Primary lymphedema is a rare genetic condition with both autosomal dominant and autosomal recessive modes of inheritance. Three genes, FLT4 (VEGFR3), FOXC2, and SOX18 cause varying forms of primary lymphedema. In industr...

    journal_title:Lymphatic research and biology

    pub_type: 杂志文章

    doi:10.1089/lrb.2008.1524

    authors: Finegold DN,Schacht V,Kimak MA,Lawrence EC,Foeldi E,Karlsson JM,Baty CJ,Ferrell RE

    更新日期:2008-01-01 00:00:00