Aagenaes syndrome is a condition which is characterized by inborn hypoplasia of the lymph vessels. This eventually causes recurring cholestasis on the formative years lymphedema on the legs. This could develop into hepatic cirrhosis and giant cell hepatitis (which could occur with portal tract fibrosis). The name of Aagenaes syndrome was derived from a pediatrician in Norway named Oystein Aagenaes. The name was finalized in 1968 when the condition was first described by Aagenaes. It is sometimes called cholestasis-lymphedema syndrome or simply CLS.
The genetic foundation is still unknown but it is an autosomal recessive condition which is inherited. The most common trait of the syndrome is a widespread lymphatic defect. It is believed that people who marry among their own tend to have a bigger chance of producing children that have the syndrome.
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