[X chromosome recessively transmitted hydrocephalus with congenital aqueduct stenosis].

Although hydrocephalus is usually a sporadic disorder, there exist some rare but well delineated syndroms, in which recurrence is to be expected. The here presented Bickers-Adams-Syndrome of sex-linked hydrocephalus is caused by congenital aqueductal stenosis. A case of this syndrome is presented in regard to anamnesis, diagnosis, pedigree, autopsy findings and clinical management.