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Congenital LymphedemaAka: Lymphedema Congenita, Milroy's Disease

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  1. Epidemiology
    1. Onset within first two years of life
  2. See Also
    1. Lymphedema
  3. Definition
    1. Severe Lymphedema onset at birth or as infant
    2. Milroy's Disease is a famial subtype
  4. 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
  5. Associated Conditions
    1. Swelling of external genitalia
    2. Intestinal lymphangiectasia, protein-losing enteropathy
    3. Cystic Hygroma
    4. Pulmonary lymphangiectasia
  6. References
    1. Sabiston (1997) Surgery, Saunders, p.1574-5
    2. Rockson (2001) Am J Med 110:288

Milroy Disease (C1704423)

ConceptsCongenital Abnormality (T019) , Disease or Syndrome (T047)
ICD9757.0
MSHD008209
EnglishCongenital lymphedema, Congenital lymphoedema, Hereditary lymphedema type I, Hereditary lymphoedema type I, MILROY DIS, Milroy Disease, Milroy lymphedema, Milroy lymphoedema, Milroy's disease, MILROYS DIS, Milroys Disease, NONNE-MILROY LYMPHEDEMA, Nonne-Milroy lymphoedema, NONNE-MILROY-MEIGE SYNDROME, PCL, Primary congenital lymphedema, Primary congenital lymphoedema
Spanishenfermedad de Milroy, linfedema congenito, linfedema hereditario tipo I
Parent ConceptsHereditary lymphedema (C0026147), Ambiguous concept (C1274012)
SourcesCOSTAR, DXP, MSH, MTH, MTHICD9, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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