II. Epidemiology
- Onset within first two years of life
III. See Also
IV. Definition
- Severe Lymphedema onset at birth or as infant
- Milroy's Disease is a famial subtype
V. Signs
-
Lower Extremity Edema
- Right leg more often affected
- Bilateral involvement in 25% of cases
- Involves foot dorsum up to knee
VI. Associated Conditions
- Swelling of external genitalia
- Intestinal lymphangiectasia, Protein-losing Enteropathy
- Cystic Hygroma
- Pulmonary lymphangiectasia
VII. References
- Sabiston (1997) Surgery, Saunders, p.1574-5
- Rockson (2001) Am J Med 110:288-95 [PubMed]
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| Definition (NCI) | A congenital condition that results in swelling in the arms or legs, and can occur during adolescence or adulthood. Loss of motion and pain may also accompany the swelling. Protein-rich lymphatic fluid accumulates in tissues, engorging and enlarging vessels and often causing visible swelling, tenderness, and pain. Left untreated, the affected tissues may continue to swell, and can become hardened or fibrotic and susceptible to infection. |
| Concepts | Congenital Abnormality (T019) , Disease or Syndrome (T047) |
| MSH | D008209 |
| ICD10 | Q82.0 |
| SnomedCT | 268355000, 234098006, 75127007, 195489008, 205544008, 157017000, 254199006, 399889006 |
| English | Milroy's Disease, Milroys Disease, Milroy's disease, Congenital lymphoedema, Infantile lymphedema (praecox), Infantile lymphedema(praecox), Infantile lymphoedema (praecox), Infantile lymphoedema(praecox), Primary Lymphedema, Milroy Disease, PRIMARY CONGENITAL LYMPHEDEMA, PCL, NONNE-MILROY LYMPHEDEMA, MILROY DISEASE, LYMPHEDEMA, EARLY-ONSET, MILROYS DIS, MILROY DIS, Primary (congenit.)lymphedema, Primary (congenit.)lymphoedema, Primary (congenital) lymphedema, Primary (congenital) lymphoedema, Milroy's disease (diagnosis), Lymphedema congenital, Lymphoedema congenital, LYMPHEDEMA, HEREDITARY, IA, Nonne Milroy Disease, Nonne Milroy Meige Disease, Nonne-Milroy-Meige Disease, Nonne-Milroy Disease, lymphedema primary, hereditary lymphedema, hereditary lymphoedema, primary lymphedema, congenital lymphedema, milroy disease, milroys disease, primary lymphoedema, milroy's disease, LMPH1A, Hereditary Lymphedema 1, Congenital Hereditary Lymphedemas, Lymphedema, Hereditary, Lymphedemas, Hereditary, Congenital Hereditary Lymphedema, Lymphedema, Early Onset, Hereditary Lymphedema 1s, Hereditary Lymphedemas, Congenital, Early Onset Lymphedemas, Lymphedema, Nonne-Milroy, Primary Congenital Lymphedema, Lymphedema, Primary Congenital, Lymphedemas, Primary Congenital, Hereditary Lymphedema, Congenital, Hereditary Lymphedemas, Lymphedema, Hereditary, Ia, Congenital Lymphedema, Primary, Congenital Lymphedemas, Primary, Lymphedemas, Congenital Hereditary, Early Onset Lymphedema, Lymphedemas, Early-Onset, Nonne-Milroy Lymphedema, Early-Onset Lymphedemas, Primary Congenital Lymphedemas, Lymphedema, Congenital Hereditary, Lymphedemas, Early Onset, Hereditary Lymphedema, Lymphedema, Early-Onset, Early-Onset Lymphedema, Nonne Milroy Lymphedema, Hereditary lymphedema (diagnosis), anomalies of skin hereditary lymphedema, Hereditary Lymphedema Type I, Congenital Familial Lymphedema, Congenital lymphoedema (disorder), Milroy's disease (disorder), Hereditary lymphedema, Hereditary lymphoedema, Primary lymphedema, Primary lymphoedema, Hereditary lymphedema (disorder), Hereditary lymphedema type I (disorder), Hereditary lymphedema type I, Hereditary lymphoedema type I, Milroy lymphedema, Milroy lymphoedema, Nonne-Milroy lymphedema, Nonne-Milroy lymphoedema, Primary congenital lymphedema, Primary congenital lymphoedema, Milroy; edema, Milroy, Nonne-Milroy, edema; Milroy, hereditary; lymphedema, lymphedema; hereditary, Congenital lymphedema |
| Italian | Linfedema congenito, Malattia di Nonne-Milroy, Malattia di Milroy, Linfedema congenito-ereditario, Linfedema ereditario di tipo I, Malattia di Nonne-Milroy-Meige |
| Dutch | congenitaal lymfoedeem, lymfoedeem congenitaal, ziekte van Milroy, Milroy; oedeem, hereditair; lymfoedeem, lymfoedeem; hereditair, oedeem; Milroy, Hereditair lymfoedeem, lymfoedeem, congenitaal, Milroy-ziekte |
| German | Lymphoedem kongenital, Milroykrankheit, Milroy-Krankheit, Hereditaeres Lymphoedem, kongenitales Lymphoedem |
| Japanese | ミルロイ病, ﾐﾙﾛｲﾋﾞｮｳ, 先天性リンパ浮腫, ｾﾝﾃﾝｾｲﾘﾝﾊﾟﾌｼｭ |
| French | Maladie de Milroy, Lymphoedème congénital, Syndrome de Meige-Milroy-Nonne, Trophoedème, Éléphantiasis familial de Milroy |
| Czech | Lymfedém vrozený, Vrozený lymfedém, Nonne-Milroyova nemoc, Nonneův-Milroyův-Meigeův syndrom, Milroyova nemoc, dědičný lymfedém, vrozený lymfatický edém |
| Korean | 유전성 림프 부종 |
| Hungarian | Milroy-betegség, Veleszületett nyirokoedema, Kongenitális lymphoedema, Veleszületett lymphoedema |
| Spanish | enfermedad de Milroy (trastorno), enfermedad de Milroy, linfedema congénito, linfedema hereditario (trastorno), linfedema hereditario tipo I (trastorno), linfedema hereditario tipo I, linfedema hereditario, Linfedema congénito, Enfermedad de Milroy |
| Norwegian | Arvelig lymfødem |
| Portuguese | Linfedema congénito, Doença de Milroy |
