Lymphangiectasia-lymphedema syndromeI89.0

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Hennekam Syndrome

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DefinitionThis section has been translated automatically.

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.

EtiopathogenesisThis section has been translated automatically.

Autosomal recessive malformations.

LiteratureThis section has been translated automatically.

  1. Kröll A et al (2006) Etiology and epidemiology of primary lymphedema. Phlebology 35: 3-7

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer