Lymphangiectasia-lymphedema syndrome I89.0
Synonym(s)
Hennekam Syndrome
Definition
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Rare syndrome with mental retardation, severe congenital and progressive lymphedema of the extremities and face, genitals and lymphangiectatic malformations in various organs (e.g. gastrointestinal) with consecutive hypoproteinemia. Furthermore, anomalies of the face (flat face, epicanthus, hypertelorism) of the extremities (syndactyly, pes equinovarus) and epileptic seizures are described. Multiple hemangiomas may be associated.
Etiopathogenesis
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Autosomal recessive malformations.
Literature
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- Kröll A et al (2006) Etiology and epidemiology of primary lymphedema. Phlebology 35: 3-7
Recommended articles
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Lymphatic malformations;Disclaimer
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Prof. Dr. med. Peter Altmeyer