- Active distension of the ventricular system resulting from inadequate passage of cerebral spinal fluid (CSF) from its point of production to point of absorption
- CSF compartment progressively enlarged at the expense of brain and/or spinal tissue
Pediatric prevalence estimated at 1:1,000 children
- Intraventricular hemorrhage (IVH)
- Maternal medications (isotretinoin)
- VACTERL association
X-linked mutation in L1CAM gene most common genetic cause, accounting for 10% males with idiopathic isolated hydrocephalus, may also be associated with congenital muscular dystrophies/neuronal migrational defects (Walker-Warburg and muscle-eye-brain phenotypes)
- CSF originates from choroid plexus and interstitial fluid of brain/spinal cord and travels via both unidirectional bulk flow and pulsatile flow from cardiac cycle; starts in lateral ventricles → foramina of Monro → 3rd ventricle → aqueduct of Sylvius → 4th ventricle → foramina of Luschka and Magendie → subarachnoid space → into either arachnoid villi to venous system or to lymphatic sinuses
- Hydrocephalus results from obstruction to flow, impaired reabsorption, or rarely overproduction of CSF.
- Obstructive hydrocephalus: Blockage of flow or impaired absorption can necessitate urgent evaluation.
- Acute (hours) (i.e., direct ventricular obstruction) → surgical emergency
- Subacute/progressive (weeks to months) → increased ventricle size/interstitial edema = if not treated can cause brain herniation/arrest
- Chronic (years) (i.e., late-onset aqueductal stenosis) → ventricles and skull slowly increase in size = slow loss of brain volume
- Communicating hydrocephalus—no morphologic obstruction to CSF, less time sensitive
- May be secondary to arachnoid pathology or impaired elasticity of spinal dura
Hydrocephalus is a heterogeneous condition with varying classification systems, organized here as “congenital,” present at birth, or “acquired,” occurring after birth, although some conditions may fit into both groups.
- Congenital hydrocephalus
- Aqueductal stenosis—most common cause of congenital hydrocephalus (~10% cases); stenosis of aqueduct of Sylvius (passageway between 3rd and 4th ventricles)
- Neural tube defect–associated—that is, myelomeningoceles and Chiari II malformation (cerebellum and brainstem extend into foramen magnum)
- Arachnoid cysts—most often located in posterior fossa, 3rd ventricle/suprasellar
- Dandy-Walker malformation—4th ventricle enlarged/cystic as either outlet obstruction or failure of cerebellar development
- Craniosynostosis/skeletal dysplasias—that is, achondroplasia, FGF mutations
- Other associated syndromes—that is, trisomies 13, 18, 21, mucopolysaccharidoses (type II [Hunter], type VI [Maroteaux-Lamy]), Apert and Crouzon syndromes, NF1
- IVH—most in setting of prematurity. Posthemorrhagic hydrocephalus develops secondary to meningeal adhesions, clots, and granular ependymitis in 35% of all neonates surviving IVH; worse prognosis when associated with moderate/severe ventriculomegaly, echolucencies, or white matter injury
- Infection (meningitis) can lead to leptomeningeal adhesions and granulations that block reabsorption of CSF (high-risk infections include enterovirus, CMV, lymphocytic choriomeningitis virus [LCMV], toxoplasmosis; may also occur intrauterine).
- Brain tumors—in children, most often posterior fossa tumors compressing 4th ventricle
- Head injury—blood from ruptured vessels may cause inflammation, scarring of meninges, or clot.
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