{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,2]],"date-time":"2026-11-02T21:57:01Z","timestamp":1793656621075,"version":"4.5.1"},"reference-count":60,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2023,5,6]],"date-time":"2023-05-06T00:00:00Z","timestamp":1683331200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>Primary lymphedema is a heterogeneous group of conditions encompassing all lymphatic anomalies that result in lymphatic swelling. Primary lymphedema can be difficult to diagnose, and diagnosis is often delayed. As opposed to secondary lymphedema, primary lymphedema has an unpredictable disease course, often progressing more slowly. Primary lymphedema can be associated with various genetic syndromes or can be idiopathic. Diagnosis is often clinical, although imaging can be a helpful adjunct. The literature on treating primary lymphedema is limited, and treatment algorithms are largely based on practice patterns for secondary lymphedema. The mainstay of treatment focuses on complete decongestive therapy, including manual lymphatic drainage and compression therapy. For those who fail conservative treatment, surgical treatment can be an option. Microsurgical techniques have shown promise in primary lymphedema, with both lymphovenous bypass and vascularized lymph node transfers demonstrating improved clinical outcomes in a few studies.<\/jats:p>","DOI":"10.3390\/medicina59050894","type":"journal-article","created":{"date-parts":[[2023,5,8]],"date-time":"2023-05-08T04:04:48Z","timestamp":1683518688000},"page":"894","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":51,"title":["Current Concepts in the Management of Primary Lymphedema"],"prefix":"10.3390","volume":"59","author":[{"given":"Jenna-Lynn B.","family":"Senger","sequence":"first","affiliation":[{"name":"Wexner Medical Center, Department of Plastic Surgery, Ohio State University, Columbus, OH 43210, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rohini L.","family":"Kadle","sequence":"additional","affiliation":[{"name":"Wexner Medical Center, Department of Plastic Surgery, Ohio State University, Columbus, OH 43210, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roman J.","family":"Skoracki","sequence":"additional","affiliation":[{"name":"Wexner Medical Center, Department of Plastic Surgery, Ohio State University, Columbus, OH 43210, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,5,6]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"502","DOI":"10.1177\/12034754221112002","article-title":"Pediatric Lymphedema: Study of 180 Patients Referred to a Tertiary Lymphedema Clinic","volume":"26","author":"Colmant","year":"2022","journal-title":"J. Cutan. Med. Surg."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1186\/s13023-020-01652-w","article-title":"Primary lymphedema French National Diagnosis and Care Protocol (PNDS; Protocole National de Diagnostic et de Soins)","volume":"16","author":"Vignes","year":"2021","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1055\/a-1200-1721","article-title":"Chubby Infant\u2014Should One Worry? An Infant with Primary lymphedema\u2014Mini Review and Case Report","volume":"233","author":"Kaczmarek","year":"2021","journal-title":"Klin. Padiatr."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1097\/SAP.0000000000002862","article-title":"Efficacy of Microsurgical Treatment of Primary Lymphedema: A Systematic Review","volume":"88","author":"Fallahian","year":"2022","journal-title":"Ann. Plast. Surg."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1007\/978-3-7091-1646-3_14","article-title":"Clinical disorders of primary malfunctioning of the lymphatic system","volume":"214","author":"Bellini","year":"2014","journal-title":"Adv. Anat. Embryol. Cell Biol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1038\/s41572-021-00309-7","article-title":"Primary lymphoedema","volume":"7","author":"Brouillard","year":"2021","journal-title":"Nat. Rev. Dis. Primers"},{"key":"ref_7","doi-asserted-by":"crossref","unstructured":"Morfoisse, F., Zamora, A., Marchaud, E., Nougue, M., Diallo, L.H., David, F., Roussel, E., Lacazette, E., Prats, A.C., and Tatin, F. (2021). Sex Hormones in Lymphedema. Cancers, 13.","DOI":"10.3390\/cancers13030530"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"766","DOI":"10.1002\/micr.30622","article-title":"Lymphedema microsurgery improved outcomes of pediatric primary extremity lymphedema","volume":"40","author":"Cheng","year":"2020","journal-title":"Microsurgery"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"221e","DOI":"10.1097\/PRS.0b013e3182365c91","article-title":"Primary lymphedema: Definition of onset based on developmental age","volume":"129","author":"Greene","year":"2012","journal-title":"Plast. Reconstr. Surg."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1089\/lrb.2017.0007","article-title":"Surgical Treatment of Primary Lymphedema","volume":"15","author":"Lee","year":"2017","journal-title":"Lymphat. Res. Biol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1111\/cge.12173","article-title":"The classification and diagnostic algorithm for primary lymphatic dysplasia: An update from 2010 to include molecular findings","volume":"84","author":"Connell","year":"2013","journal-title":"Clin. Genet."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"653","DOI":"10.1136\/jmedgenet-2019-106084","article-title":"Update and audit of the St George\u2019s classification algorithm of primary lymphatic anomalies: A clinical and molecular approach to diagnosis","volume":"57","author":"Gordon","year":"2020","journal-title":"J. Med. Genet."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"438","DOI":"10.1111\/j.1399-0004.2010.01394.x","article-title":"A new classification system for primary lymphatic dysplasias based on phenotype","volume":"77","author":"Connell","year":"2010","journal-title":"Clin. Genet."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"620","DOI":"10.1089\/lrb.2018.0032","article-title":"Adult-Onset Primary Lymphedema: A Clinical-Lymphoscintigraphic Study of 26 Patients","volume":"17","author":"Goss","year":"2019","journal-title":"Lymphat. Res. Biol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1089\/lrb.2017.0085","article-title":"Primary Lymphedema of the Upper Extremities: Clinical and Lymphoscintigraphic Features in 23 Patients","volume":"17","author":"Goss","year":"2019","journal-title":"Lymphat. Res. Biol."},{"key":"ref_16","unstructured":"ISSVA (2018). Classification of Vascular Anomalies 2018, International Society for the Study of Vascular Anomalies."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"646","DOI":"10.1016\/j.ejvs.2010.01.018","article-title":"Primary lymphoedema and lymphatic malformation: Are they the two sides of the same coin?","volume":"39","author":"Lee","year":"2010","journal-title":"Eur. J. Vasc. Endovasc. Surg."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"1571","DOI":"10.1097\/PRS.0b013e31820a64f3","article-title":"Differential diagnosis of lower extremity enlargement in pediatric patients referred with a diagnosis of lymphedema","volume":"127","author":"Schook","year":"2011","journal-title":"Plast. Reconstr. Surg."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"2419","DOI":"10.1097\/PRS.0b013e318213a218","article-title":"Primary lymphedema: Clinical features and management in 138 pediatric patients","volume":"127","author":"Schook","year":"2011","journal-title":"Plast. Reconstr. Surg."},{"key":"ref_20","doi-asserted-by":"crossref","unstructured":"Yoshida, S., Koshima, I., Imai, H., Roh, S., Mese, T., Uchiki, T., Sasaki, A., and Nagamatsu, S. (2021). Lymphaticovenous Anastomosis for Age-Related Lymphedema. J. Clin. Med., 10.","DOI":"10.3390\/jcm10215129"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"e29908","DOI":"10.1002\/pbc.29908","article-title":"How we approach lymphedema in the pediatric population","volume":"69","author":"Pateva","year":"2022","journal-title":"Pediatr. Blood Cancer"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"766","DOI":"10.1016\/j.arcped.2017.05.002","article-title":"Les lymphoedemes primaires de l\u2019enfant","volume":"24","author":"Vignes","year":"2017","journal-title":"Arch. Pediatr."},{"key":"ref_23","first-page":"2124","article-title":"Lymph\u0153d\u00e8me primaire","volume":"13","author":"Depairon","year":"2017","journal-title":"Rev. Med. Suisse"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"883","DOI":"10.1097\/PRS.0000000000001631","article-title":"Indication of Lymphaticovenous Anastomosis for Lower Limb Primary Lymphedema","volume":"136","author":"Hara","year":"2015","journal-title":"Plast. Reconstr. Surg."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"138","DOI":"10.1002\/micr.20466","article-title":"Pediatric lymphedema and correlated syndromes: Role of microsurgery","volume":"28","author":"Campisi","year":"2008","journal-title":"Microsurgery"},{"key":"ref_26","doi-asserted-by":"crossref","unstructured":"Bonetti, G., Paolacci, S., Samaja, M., Maltese, P.E., Michelini, S., Michelini, S., Michelini, S., Ricci, M., Cestari, M., and Dautaj, A. (2022). Low Efficacy of Genetic Tests for the Diagnosis of Primary Lymphedema Prompts Novel Insights into the Underlying Molecular Pathways. Int. J. Mol. Sci., 23.","DOI":"10.3390\/ijms23137414"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"140","DOI":"10.1196\/annals.1413.013","article-title":"Phenotypic characterization of primary lymphedema","volume":"1131","author":"Connell","year":"2008","journal-title":"Ann. NY Acad. Sci."},{"key":"ref_28","first-page":"505","article-title":"An undescribed variety of hereditary edema","volume":"56","author":"Milroy","year":"1892","journal-title":"NY Med. J."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"280","DOI":"10.1016\/j.tjog.2011.01.041","article-title":"Prenatal diagnosis of Milroy\u2019s primary congenital lymphedema","volume":"51","author":"Gezginc","year":"2012","journal-title":"Taiwan J. Obstet. Gynecol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1002\/jso.25583","article-title":"Surgical treatment of Milroy disease","volume":"121","author":"Bolletta","year":"2020","journal-title":"J. Surg. Oncol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1111\/j.1549-8719.2010.00030.x","article-title":"Lymphatic dysfunction, not aplasia, underlies Milroy disease","volume":"17","author":"Mellor","year":"2010","journal-title":"Microcirculation"},{"key":"ref_32","first-page":"341","article-title":"Distrophie oedemateuse h\u00e9r\u00e9ditaire","volume":"6","author":"Meige","year":"1898","journal-title":"Presse M\u00e9d."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1111\/j.1749-6632.2002.tb04866.x","article-title":"Research perspectives in inherited lymphatic disease","volume":"979","author":"Ferrell","year":"2002","journal-title":"Ann. NY Acad. Sci."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"1634","DOI":"10.1038\/ejhg.2015.41","article-title":"The lymphatic phenotype in Turner syndrome: An evaluation of nineteen patients and literature review","volume":"23","author":"Atton","year":"2015","journal-title":"Eur. J. Hum. Genet."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"2047","DOI":"10.1056\/NEJMc1802399","article-title":"Incidence of Cellulitis among Children with Primary Lymphedema","volume":"378","author":"Quere","year":"2018","journal-title":"N. Engl. J. Med."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"188","DOI":"10.3348\/kjr.2015.16.1.188","article-title":"Primary lymphedema of the lower limb: The clinical utility of single photon emission computed tomography\/CT","volume":"16","author":"Weiss","year":"2015","journal-title":"Korean J. Radiol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"736242","DOI":"10.3389\/fped.2021.736242","article-title":"The Use of Indocyanine Green Fluorescence Angiography in Pediatric Surgery: A Systematic Review and Narrative Analysis","volume":"9","author":"Dodin","year":"2021","journal-title":"Front. Pediatr."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1016\/j.ejvs.2014.10.023","article-title":"Indocyanine Green Lymphography Findings in Primary Leg Lymphedema","volume":"49","author":"Yamamoto","year":"2015","journal-title":"Eur. J. Vasc. Endovasc. Surg."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1016\/j.cps.2012.08.001","article-title":"Surgical treatment of congenital lymphedema","volume":"39","author":"Becker","year":"2012","journal-title":"Clin. Plast. Surg."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1111\/j.1742-7924.2012.00220.x","article-title":"Health-related quality of life and associated factors in patients with primary lymphedema","volume":"10","author":"Okajima","year":"2013","journal-title":"Jpn. J. Nurs. Sci."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"353","DOI":"10.7150\/ijms.7.353","article-title":"Primary lower limb lymphedema: A focus on its functional, social and emotional impact","volume":"7","author":"Symvoulakis","year":"2010","journal-title":"Int. J. Med. Sci."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"766","DOI":"10.1093\/ptj\/pzaa012","article-title":"Clinical and scintigraphic predictors of primary lower limb lymphedema: Volume reduction during complete decongestive physical therapy","volume":"100","author":"Vignes","year":"2020","journal-title":"Phys. Ther."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"150972","DOI":"10.1016\/j.sempedsurg.2020.150972","article-title":"Lymphedema (Seminars in pediatric surgery)","volume":"29","author":"Greene","year":"2020","journal-title":"Semin. Pediatr. Surg."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1111\/jnu.12117","article-title":"Self-care status, symptom burden, and reported infections in individuals with lower-extremity primary lymphedema","volume":"47","author":"Deng","year":"2015","journal-title":"J. Nurs. Scholarsh."},{"key":"ref_45","first-page":"541","article-title":"Diagnosis and treatment of primary lymphedema. Consensus document of the International Union of Phlebology (IUP)\u20142013","volume":"32","author":"Lee","year":"2013","journal-title":"Int. Angiol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"e2056","DOI":"10.1097\/GOX.0000000000002056","article-title":"Outcomes of Vascularized Lymph Node Transfer and Lymphovenous Anastomosis for Treatment of Primary Lymphedema","volume":"6","author":"Cheng","year":"2018","journal-title":"Plast. Reconstr. Surg. Glob. Open"},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"368","DOI":"10.1016\/j.bjps.2015.10.042","article-title":"Proximal and distal patterns: Different spreading patterns of indocyanine green lymphography in secondary lower extremity lymphedema","volume":"69","author":"Tashiro","year":"2016","journal-title":"J. Plast. Reconstr. Aesthet. Surg."},{"key":"ref_48","first-page":"206","article-title":"Primary lymphedema in children and adolescents: A follow-up study and review","volume":"76","author":"Smeltzer","year":"1985","journal-title":"Pediatrics"},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1002\/micr.30508","article-title":"Comparisons in long-term clinical outcomes among patients with upper or lower extremity lymphedema treated with diverse vascularized lymph node transfer","volume":"40","author":"Ciudad","year":"2020","journal-title":"Microsurgery"},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"958","DOI":"10.1002\/jso.26811","article-title":"Heparin-induced thrombocytopenia and thrombosis in primary lymphedema patients who underwent vascularized lymph node transplantations","volume":"125","author":"Hsu","year":"2022","journal-title":"J. Surg. Oncol."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"736","DOI":"10.1002\/jso.25180","article-title":"Lymph node transplantation for the treatment of lymphedema","volume":"118","author":"Gould","year":"2018","journal-title":"J. Surg. Oncol."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"1227","DOI":"10.1016\/j.bjps.2016.06.028","article-title":"Complete lymph flow reconstruction: A free vascularized lymph node true perforator flap transfer with efferent lymphaticolymphatic anastomosis","volume":"69","author":"Yamamoto","year":"2016","journal-title":"J. Plast. Reconstr. Aesthet. Surg."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1002\/jso.24374","article-title":"Vascularized lymph node transfer from thoracodorsal axis for congenital and post filarial lymphedema of the lower limb","volume":"115","author":"Venkatramani","year":"2017","journal-title":"J. Surg. Oncol."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1002\/jso.25514","article-title":"Intra-abdominal chylovenous bypass treats retroperitoneal lymphangiomatosis","volume":"121","author":"Chen","year":"2020","journal-title":"J. Surg. Oncol."},{"key":"ref_55","first-page":"562","article-title":"Long-term results after microlymphaticovenous anastomoses for the treatment of obstructive lymphedema","volume":"85","author":"Mellow","year":"1989","journal-title":"Plast. Reconstr. Surg."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1055\/s-0029-1243299","article-title":"Comparison of primary and secondary lower-extremity lymphedema treated with supermicrosurgical lymphaticovenous anastomosis and lymphaticovenous implantation","volume":"26","author":"Demirtas","year":"2010","journal-title":"J. Reconstr. Microsurg."},{"key":"ref_57","doi-asserted-by":"crossref","unstructured":"Hayashi, A., Visconti, G., Yang, C.J., Hayashi, N., and Yoshimatsu, H. (2022). Additional Lymphaticovenular Anastomosis on the Posterior Side for Treatment of Primary Lower Extremity Lymphedema. J. Clin. Med., 11.","DOI":"10.3390\/jcm11030867"},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"646","DOI":"10.1016\/j.jvsv.2019.10.013","article-title":"Characteristics and outcomes of lymphaticovenular anastomosis in older patients with bilateral involvement versus younger patients with unilateral involvement in lower extremity lymphedema","volume":"8","author":"Yoshida","year":"2020","journal-title":"J. Vasc. Surg. Venous Lymphat. Disord."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"226","DOI":"10.1016\/j.jvsv.2020.04.025","article-title":"Microsurgery for management of primary and secondary lymphedema","volume":"9","author":"Drobot","year":"2021","journal-title":"J. Vasc. Surg. Venous Lymphat. Disord."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"812","DOI":"10.1016\/j.bjps.2010.10.011","article-title":"Simultaneous multi-site lymphaticovenular anastomoses for primary lower extremity and genital lymphoedema complicated with severe lymphorrhea","volume":"64","author":"Yamamoto","year":"2011","journal-title":"J. Plast. Reconstr. Aesthet. 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