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Primair lymfoedeem
Prenatal onset
Segment
Syndromic
Systemic involvement
Vascular anomalies
Detection of lymphatic abnormality in the prenatal period. Isolated pedal oedema is
excluded from this difinition as this may be a presentation of Milroy disease.
A region of the body affected by lymphoedema (i.e. face, conjunctiva, genitale, upper
limbs, lower limbs - each constitute one body part). Multisegmental refers to more
than one segment affected by lymphoedema. Bilateral lower limb swelling is not
considered to be multisegmental lymphoedema.
A constellation of abnormalities, one of which is lymphoedema.
Systemic lymphatic problems persisting beyond the newborn period or
manifesting at any age thereafter. This includes hydrops fetalis, chylous ascites,
intestinal lymphangiectasia, pleural and pericardial effusions, and pulmonary
lymphangiectasia.
Include congenital vascular abnormalities.
Definition of terminology used in the classification pathway
(vervolg)
Definition
Meaning
Bron: F.C. Connell et al. The classification and diagnostic algorithm for primary lymphatic dysplasia: an update from 2010 to
include molecular findings.
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