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===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 = 1β11 | 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 = 2424β2430 | 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 = 1β71 | 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 = 85β91 | 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.
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