Management of the adult with congenital hydrocephalus

Hydrocephalus denotes increased volume of cerebrospinal fluid (CSF) contained within the intracranial ventricular system, with concomitant elevated intracranial pressure (ICP). Increased ventricular volume results from abnormal production, flow, or absorption of CSF. The specification of congenital hydrocephalus indicates diagnosis during prenatal screening, at birth, or the perinatal period. Etiologies of congenital hydrocephalus include structural abnormalities like Dandy–Walker complex, Chiari malformation, or aqueductal stenosis; spinal dysraphism or myelomeningocele; and intraventricular (IVH) or germinal matrix hemorrhage. Hydrocephalus diagnosed early in infancy may also result from neonatal meningitis. The causes of hydrocephalus diagnosed at birth typically differ from hydrocephalus diagnosed in adulthood and require different long-term management strategies. Management of adults with hydrocephalus treated during infancy or childhood represents a challenging process. Several factors account for this complexity, including the multiplicity of shunt revisions, the transition from pediatric to adult-centered care, potential conversion to shunt independence, management of chronic shunting with concomitant headaches, and impact on neuropsychological development. Furthermore, many patients with congenital hydrocephalus experience medical comorbidities requiring coordinated, interdisciplinary care. While pediatric neurosurgeons routinely engage in collaborative care, interdisciplinary models may not represent the routine approach for adult or general neurosurgeons [1]. Optimal neurosurgical management of adult patients with congenital hydrocephalus requires close attention to the complex issues of transitioning care, shunt management, and neuropsychological development.