Caroli syndrome in childhood. Review and report of a patient with an unbalanced karyotype

: This paper describes the clinical symptomatology, the pathogenesis, diagnosis and therapy of the Caroli'Syndrome with a special reference to differences to other congenital hepatic diseases with similar manifestations. We report on a male infant with a Caroli'Syndrome, which is associated with a chromosomal imbalance and other malformations and discuss the genetical background.