Hydrocephalus in Achondroplasia and Venous Hypertension
The cause of hydrocephalus in achondroplasia is believed to be chronic venous hypertension, which in turn, is due to the anomalous neuroanatomy associated with this disorder. The venous anatomy in achondroplasia is now known to undergo substantial compensation during development, which could partly account for the benign nature of hydrocephalus in this population. The management is controversial, owing to inadequate understanding of the natural history and pathophysiology of this condition. This chapter recapitulates our current understanding of hydrocephalus in achondroplasia, with special emphasis on the role of venous hypertension, its impact on CSF circulation and management of hydrocephalus.