Catalog

Hennekam lymphangiectasia-lymphoedema syndrome

No MMS code

Link to WHO Definition

Hennekam lymphangiectasia-lymphoedema syndrome – http://id.who.int/icd/entity/162216708

Hennekam lymphangiectasia-lymphoedema syndrome (Hennekam syndrome) is characterised by the association of lymphoedema, intestinal lymphangiectasia, intellectual deficit and facial dysmorphism. Lymphoedema affects the face, lower limbs and genitalia; if not present at birth, it tends to appear by early infancy. Intestinal lymphangiectasia may result in protein-losing enteropathy, growth retardation, peripheral oedema and ascites. Facial signs include a flat face, a broad and depressed nasal bridge, hypertelorism, epicanthal folds, a small mouth, and low-set ears.