II. Epidemiology

  1. Onset within first two years of life

III. See Also

IV. Definition

  1. Severe Lymphedema onset at birth or as infant
  2. Milroy's Disease is a famial subtype

V. Signs

  1. Lower extremity edema
    1. Right leg more often affected
    2. Bilateral involvement in 25% of cases
  2. Involves foot dorsum up to knee

VI. Associated Conditions

  1. Swelling of external genitalia
  2. Intestinal lymphangiectasia, protein-losing Enteropathy
  3. Cystic Hygroma
  4. Pulmonary lymphangiectasia

VII. References

  1. Sabiston (1997) Surgery, Saunders, p.1574-5
  2. Rockson (2001) Am J Med 110:288-95 [PubMed]

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Ontology: Milroy Disease (C1704423)

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, ミルロイビョウ, 先天性リンパ浮腫, センテンセイリンパフシュ
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