X-linked Hydrocephalus (Bickers-Adams syn drome) in Siblings.

Abstract

Summary: A family with three consecutive male children with prenatal hydrocephalus is reported. The proband had prenatal hydrocephalus, bilateral flexion-adduction deformity of the thumbs and spas ticity. Exclusion of other causes of prenatal hydrocephalus on clinical grounds, implicated X-linked hydrocephalus (Bickers-Adams syndrome) as the most probable diagnosis. Since identification of an X-linked hydrocephalus has important conse quences for genetic counselling, analysis of the family history of any male infant with overt hydrocephalus at birth, is therefore very important.