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==Diagnosis== Diagnosis is generally based on signs and symptoms, with testing used to rule out other potential causes.<ref name="Lymphedema Diagnostic workup" /> An accurate diagnosis and staging may help with management.<ref name="Lymphedema Diagnostic workup">{{cite journal | vauthors = Grada AA, Phillips TJ | title = Lymphedema: Diagnostic workup and management | journal = Journal of the American Academy of Dermatology | volume = 77 | issue = 6 | pages = 995โ1006 | date = December 2017 | pmid = 29132859 | doi = 10.1016/j.jaad.2017.03.021}}</ref> A swollen limb can result from different conditions that require different treatments. Diagnosis of lymphedema is currently based on history, physical exam, and limb measurements. Imaging studies such as lymphoscintigraphy and indocyanine green lymphography are only required when surgery is being considered.<ref name="Lymphedema Diagnostic workup" /> However, the ideal method of staging to guide treatment is controversial because of several different proposed protocols.<ref>{{cite journal | vauthors = Burnand KM, Glass DM, Mortimer PS, Peters AM | title = Lymphatic dysfunction in the apparently clinically normal contralateral limbs of patients with unilateral lower limb swelling | journal = Clinical Nuclear Medicine | volume = 37 | issue = 1 | pages = 9โ13 | date = January 2012 | pmid = 22157021 | doi = 10.1097/RLU.0b013e31823931f5 | s2cid = 34921176}}</ref><ref>{{cite journal | vauthors = Tiwari A, Cheng KS, Button M, Myint F, Hamilton G | title = Differential diagnosis, investigation, and current treatment of lower limb lymphedema | journal = Archives of Surgery | volume = 138 | issue = 2 | pages = 152โ161 | date = February 2003 | pmid = 12578410 | doi = 10.1001/archsurg.138.2.152 | doi-access = free}}</ref> Lymphedema can occur in both the upper and lower extremities, and in some cases, the head and neck. Assessment of the extremities first begins with a visual inspection; color, presence of hair, visible veins, size and any sores or ulcerations are noted. Lack of hair may indicate an arterial circulation problem.<ref name="Jarvis2004">{{Cite book|url=https://books.google.com/books?id=aDiJoAEACAAJ&pg=PA530|title=Physical Examination and Health Assessment|author=Jarvis, C.|publisher=Saunders Elsevier|year=2004|isbn=978-1-4160-5188-6|edition=5th|pages=530โ553|access-date=2018-03-14|archive-date=2023-01-13|archive-url=https://web.archive.org/web/20230113000219/https://books.google.com/books?id=aDiJoAEACAAJ&pg=PA530|url-status=live}}</ref> In cases of swelling, the extremities' circumference is measured over time for reference. In early stages of lymphedema, elevating the limb may reduce or eliminate the swelling. Palpation of the wrist or ankle can determine the degree of swelling; assessment includes a check of the pulses. The axillary or inguinal lymph nodes may be enlarged due to the swelling. Enlargement of the nodes lasting more than three weeks may indicate infection or other illnesses (such as sequela from breast cancer surgery) requiring further medical attention.<ref name="Jarvis2004" /> Diagnosis or early detection of lymphedema is difficult. The first signs may be subjective observations such as a feeling of heaviness in the affected extremity. These may be symptomatic of early-stage lymphedema where accumulation of lymph is mild and not detectable by changes in volume or circumference. As lymphedema progresses, definitive diagnosis is commonly based upon an objective measurement of differences between the affected or at-risk limb and the opposite unaffected limb, e.g. in volume or circumference. No generally accepted criterion is definitively diagnostic, although a volume difference of 200 ml between limbs or a {{Convert|4|cm|abbr=on}} difference (at a single measurement site or set intervals along the limb) is often used. [[Bioelectrical impedance analysis|Bioimpedance]] measurement (which measures the amount of fluid in a limb) offers greater sensitivity than other methods.<ref>{{cite journal | vauthors = Ward LC | title = Bioelectrical impedance analysis: proven utility in lymphedema risk assessment and therapeutic monitoring | journal = Lymphatic Research and Biology | volume = 4 | issue = 1 | pages = 51โ56 | year = 2006 | pmid = 16569209 | doi = 10.1089/lrb.2006.4.51}}</ref> Devices like SOZO <ref>{{Cite web |date=2019-06-05 |title=SOZOยฎ Digital Health Platform {{!}} ImpediMed |url=https://www.impedimed.com/products/sozo/ |access-date=2024-03-20 |website=www.impedimed.com |language=en-US |archive-date=2024-03-20 |archive-url=https://web.archive.org/web/20240320163547/https://www.impedimed.com/products/sozo/ |url-status=live }}</ref> utilize Bioimpedence Analysis (BIA) by sending a current through the body and measuring the resultant impedance. Another approach involves Tissue Dielectric Constant (TDC) measurement, used by devices such as Delfin Technology's MoistureMeterD and LymphScanner,<ref>{{Cite web |title=Delfin Technologies - Skin research instruments |url=https://delfintech.com/ |access-date=2024-03-20 |website=Delfin Technologies |language=en-US |archive-date=2024-03-20 |archive-url=https://web.archive.org/web/20240320163549/https://delfintech.com/ |url-status=live }}</ref> which employ microwaves to detect changes in the dielectric properties of tissue. These innovative techniques have become integral to official protocols for lymphedema detection.<ref>{{Cite journal |last1=Shah |first1=Chirag |last2=Whitworth |first2=Pat |last3=Valente |first3=Stephanie |last4=Schwarz |first4=Graham S. |last5=Kruse |first5=Megan |last6=Kohli |first6=Manpreet |last7=Brownson |first7=Kirstyn |last8=Lawson |first8=Laura |last9=Dupree |first9=Beth |last10=Vicini |first10=Frank A. |date=2023 |title=Bioimpedance spectroscopy for breast cancer-related lymphedema assessment: clinical practice guidelines |journal=Breast Cancer Research and Treatment |volume=198 |issue=1 |pages=1โ9 |doi=10.1007/s10549-022-06850-7 |issn=0167-6806 |pmc=9883343 |pmid=36566297}}</ref> Chronic venous stasis changes can mimic early lymphedema, but are more often bilateral and symmetric. [[Lipedema]] can also mimic lymphedema, however lipedema characteristically spares the feet beginning abruptly at the [[malleolus]] (ankle).<ref name="Lymphedema Diagnostic workup" /> As a part of the initial work-up before diagnosing lymphedema, it may be necessary to exclude other potential causes of lower extremity swelling such as [[kidney failure]], [[hypoalbuminemia]], [[Heart failure|congestive heart-failure]], protein-losing [[kidney disease]], [[pulmonary hypertension]], obesity, pregnancy and drug-induced [[edema]].{{citation needed|date=November 2021}} ===Classification=== [[File:Cheng's Lymphedema Grading.png|thumb|upright=1.6|Cheng's Lymphedema Grading]] The International Society of Lymphology (ISL) Staging System is based solely on subjective symptoms, making it prone to substantial observer bias. Imaging modalities have been suggested as useful adjuncts to the ISL staging to clarify the diagnosis, such as Cheng's Lymphedema Grading tool, which assesses the severity of extremity lymphedema based on objective limb measurements and provides appropriate options for management.<ref>{{cite journal | title = The diagnosis and treatment of peripheral lymphedema: 2013 Consensus Document of the International Society of Lymphology | journal = Lymphology | volume = 46 | issue = 1 | pages = 1โ11 | date = March 2013 | pmid = 23930436 | author1 = International Society of Lymphology}}</ref><ref>{{cite journal | vauthors = Patel KM, Lin CY, Cheng MH | title = A Prospective Evaluation of Lymphedema-Specific Quality-of-Life Outcomes Following Vascularized Lymph Node Transfer | journal = Annals of Surgical Oncology | volume = 22 | issue = 7 | pages = 2424โ2430 | date = July 2015 | pmid = 25515196 | doi = 10.1245/s10434-014-4276-3 | s2cid = 13376881}}</ref><ref>{{cite book|url=https://books.google.com/books?id=0_JwCgAAQBAJ|title=Principles and Practice of Lymphedema Surgery|last1=Cheng|first1=Ming-Huei|last2=Chang|first2=David W.|last3=Patel|first3=Ketan M.|date=13 July 2015|publisher=Elsevier Health Sciences|isbn=978-0-323-29897-1|access-date=14 March 2018|archive-date=13 January 2023|archive-url=https://web.archive.org/web/20230113000234/https://books.google.com/books?id=0_JwCgAAQBAJ|url-status=live}}</ref> ==== I. Grading ==== {{multiple image|perrow = 1|total_width=600 <!-- Layout parameters --> | align = right | direction = vertical | background color = <!-- box background as a 'hex triplet' web color (include the initial #) --> | width = <!-- displayed width of each image in pixels (an integer, omit "px" suffix); overrides "width[n]"s below --> | caption_align = <!-- left (default), center, right --> | image_style = <!-- border:1; (default) --> | image_gap = <!-- 5 (default)--> <!-- Header --> | header_background = <!-- header background as a 'hex triplet' web color (include the initial #) --> | header_align = <!-- center (default), left, right --> | header = <!-- header text --> <!--image 1--> | image1 = Upper limb lymphedema.jpg | width1 = <!-- displayed width of image; overridden by "width" above --> | alt1 = | link1 = | thumbtime1 = | caption1 = Severity of upper extremity lymphedema in different stages<ref name="Cheng2015" /> <!--image 2--> | image2 = Lower Limb Lymphedema.png | width2 = <!-- displayed width of image; overridden by "width" above --> | alt2 = | link2 = | thumbtime2 = | caption2 = Severity of lower extremity lymphedema in different stages<ref name="Cheng2015">Principles and Practice of Lymphedema Surgery. Cheng MH, Chang DW, Patel KM (Editors). Elsevier Inc, Oxford, United Kingdom. {{ISBN|978-0-323-29897-1}}. July 2015.</ref> <!-- and so on, to a maximum of 10 images (image10) --> <!-- Footer --> | footer_background = <!-- footer background as a 'hex triplet' web color (include the initial #) --> | footer_align = <!-- left (default), center, right --> | footer = <!-- footer text --> }} * '''Grade 1''': Spontaneously reversible on elevation. Mostly pitting edema. * '''Grade 2''': Non-spontaneously reversible on elevation. Mostly non-pitting edema. * '''Grade 3''': Gross increase in volume and circumference of Grade 2 lymphedema, with eight stages of severity given below based on clinical assessments. ===== II. Staging ===== As described by the Fifth [[World Health Organization|WHO]] Expert Committee on [[Filariasis]],<ref>{{cite web|url=http://whqlibdoc.who.int/hq/2001/WHO_CDS_CPE_CEE_2001.26a.pdf|title=Treatment and Prevention of Problems Associated with Lymphatic Filariasis|publisher=World Health Organization|access-date=2014-05-16|url-status=live|archive-url=https://web.archive.org/web/20120418030537/http://whqlibdoc.who.int/hq/2001/WHO_CDS_CPE_CEE_2001.26a.pdf|archive-date=2012-04-18}}</ref><ref>{{cite journal | vauthors = | title = Lymphatic filariasis: the disease and its control. Fifth report of the WHO Expert Committee on Filariasis | journal = World Health Organization Technical Report Series | volume = 821 | pages = 1โ71 | year = 1992 | pmid = 1441569}}</ref> and endorsed by the [[American Society of Lymphology]],<ref>{{cite book|url={{google books |plainurl=y |id=a4ZtTzIWBjUC}}|title=Lymphedema: Diagnosis and Treatment|last1=Tretbar|first1=Lawrence L|last2=Morgan|first2=Cheryl L.|last3=Lee|first3=Byung-Boong |date=6 May 2010|publisher=Springer Science & Business Media|isbn=978-1-84628-793-0|first4=Simon J. |last4=Simonian |first5=Benoit |last5=Blondeau}}{{Page needed|date=September 2011}}</ref>{{Citation needed|date=September 2011}} the staging system helps to identify the severity of lymphedema. With the assistance of medical imaging, such as [[Magnetic resonance imaging|MRI]] or [[CT scan|CT]], staging can be established by the physician, and therapeutic or medical interventions may be applied:{{citation needed|date=February 2021}} * '''Stage 0''': The lymphatic vessels have sustained some damage that is not yet apparent. Transport capacity is sufficient for the amount of lymph being removed. Lymphedema is not present. * '''Stage 1''' : Swelling increases during the day and disappears overnight as the patient lies flat in bed. Tissue is still at the pitting stage: when pressed by the fingertips, the affected area indents and reverses with elevation. Usually, upon waking in the morning, the limb or affected area is normal or almost normal in size. Treatment is not necessarily required at this point. * '''Stage 2''': Swelling is not reversible overnight, and does not disappear without proper management. The tissue now has a spongy consistency and is considered non-pitting: when pressed by the fingertips, the affected area bounces back without indentation. [[Fibrosis]] found in Stage 2 lymphedema marks the beginning of the hardening of the limbs and increasing size. * '''Stage 3''': Swelling is irreversible and usually the limb(s) or affected area becomes increasingly large. The tissue is hard (fibrotic) and unresponsive; some patients consider undergoing reconstructive surgery, called "debulking". This remains controversial, however, since the risks may outweigh the benefits and further damage done to the lymphatic system may make the lymphedema worse. * '''Stage 4''': The size and circumference of the affected limb(s) become noticeably larger. Bumps, lumps, or protrusions (also called knobs) on the skin begin to appear. * '''Stage 5''': The affected limb(s) become grossly large; one or more deep skin folds is present. * '''Stage 6''': Knobs of small elongated or rounded sizes cluster together, giving mossy-like shapes on the limb. Mobility of the patient becomes increasingly impaired. * '''Stage 7''': The person becomes "handicapped", and is unable to independently perform daily routine activities such as walking, bathing and cooking. Assistance from the family and health care system is needed. ====Grades==== Lymphedema can also be categorized by its severity (usually compared to a healthy extremity):<ref>{{cite journal | vauthors = Lee TS, Morris CM, Czerniec SA, Mangion AJ | title = Does Lymphedema Severity Affect Quality of Life? Simple Question. Challenging Answers | journal = Lymphatic Research and Biology | volume = 16 | issue = 1 | pages = 85โ91 | date = February 2018 | pmid = 28453410 | doi = 10.1089/lrb.2016.0049}}</ref> * '''Grade 1''' (mild edema): Involves the distal parts such as a forearm and hand or a lower leg and foot. The difference in circumference is less than {{Convert|4|cm|abbr=on}} and no other tissue changes are present. * '''Grade 2''' (moderate edema): Involves an entire limb or corresponding quadrant of the trunk. Difference in circumference is {{Convert|4|-|6|cm|abbr=on}}. Tissue changes, such as pitting, are apparent. The patient may experience [[erysipelas]]. * '''Grade 3a''' (severe edema): Lymphedema is present in one limb and its associated trunk quadrant. Circumferential difference is greater than {{Convert|6|cm|abbr=on}}. Significant skin alterations, such as [[Keratin#Cornification|cornification]], [[keratosis]], [[cysts]] or [[fistulae]], are present. Additionally, the patient may experience repeated attacks of [[erysipelas]]. * '''Grade 3b''' (massive edema): The same symptoms as grade 3a, except that two or more extremities are affected. * '''Grade 4''' (gigantic edema): In this stage of lymphedema, the affected extremities are huge, due to almost complete blockage of the lymph channels. ===Differential=== Lymphedema should not be confused with edema arising from [[chronic venous insufficiency]], which is caused by compromise of venous drainage rather than lymphatic drainage. However, untreated venous insufficiency can progress into a combined venous/lymphatic disorder known as [[Edema#Veins|phlebetic lymphedema]] (or phlebolymphedema).<ref>{{Cite journal |title=Sequelae of Untreated Venous Insufficiency |year=2005 |pmc=3036289 |last1=Nicholls |first1=S. C. |journal=Seminars in Interventional Radiology |volume=22 |issue=3 |pages=162โ168 |doi=10.1055/s-2005-921960 |pmid=21326689}}</ref><ref>{{Cite journal |last=Farrow |first=Wade |date=2010 |title=Phlebolymphedema-a common underdiagnosed and undertreated problem in the wound care clinic |journal=The Journal of the American College of Certified Wound Specialists |volume=2 |issue=1 |pages=14โ23 |doi=10.1016/j.jcws.2010.04.004 |issn=1876-4983 |pmc=3601853 |pmid=24527138}}</ref><ref>{{Cite web |last1=Publishing |first1=Licorn |last2=Guiboles |date=2009-11-24 |title=The causes of edema in chronic venous insufficiency |url=https://www.phlebolymphology.org/the-causes-of-edema-in-chronic-venous/ |access-date=2023-09-01 |website=Servier โ Phlebolymphology |language=en-US |archive-date=2023-09-01 |archive-url=https://web.archive.org/web/20230901182645/https://www.phlebolymphology.org/the-causes-of-edema-in-chronic-venous/ |url-status=live }}</ref>
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