Lecture Notes 4.
2: Hydrocephaly
Definition
a disturbance of cerebrospinal fluid (CSF) formation, flow, or absorption, leading to an
increase in volume occupied by this fluid in the central nervous system (CNS).
A lot of fluid in your kopf :^)
Pathophysiology
most CSF is produced by the choroid plexus
CSF flows to the lateral ventricle, then to the interventricular foramen of Monro, the third
ventricle, the cerebral aqueduct of Sylvius, the fourth ventricle, the 2 lateral foramina of
Luschka and 1 medial foramen of Magendie, the subarachnoid space, the arachnoid
granulations, the dural sinus, and finally into the venous drainage
Total volume of CSF in an adult is 120 mL.
Etiology
Brainstem malformation causing stenosis of the aqueduct of Sylvius
Dandy-Walker malformation.
Arnold-Chiari malformation type 1 and type 2
Congenital toxoplasmosis (Cats )
Bickers-Adams syndrome
Tumors
Hemmorrhage
Iatrogenic
Idiopathic
Clinical Picture
Slowing of mental capacity dilatation: This can lead
to obesity and to precocious
Headaches (initially in the morning)
puberty or delayed onset of puberty.
that are more significant than in
infants because of skull rigidity Difficulty in walking secondary to
spasticity: This affects the lower limbs
Neck pain suggesting tonsillar preferentially because the
herniation periventricular pyramidal tract is
Vomiting, more significant in the stretched by the hydrocephalus.
morning Drowsiness
Blurred vision: This is a consequence Head enlargement: Head
of papilledema and later of optic circumference is at or above the
atrophy 98th percentile for age.
Double vision: This is related to
Dysjunction of sutures: This can be
unilateral or bilateral sixth nerve palsy seen or palpated.
Stunted growth and sexual Dilated scalp veins: The scalp is thin
maturation from third ventricle and shiny with easily visible veins.
Tense fontanelle: The anterior are retracted, and the white sclerae
fontanelle in infants who are held may be visible above the iris.
erect and are not crying may be
excessively tense. Increased limb tone: Spasticity
preferentially affects the lower limbs.
Setting-sun sign: In infants, it is The cause is stretching of the
characteristic of increased intracranial periventricular pyramidal tract fibers
pressure (ICP). Ocular globes are by hydrocephalus
deviated downward, the upper lids
Diagnosis
History
Clinical Examination
CBC, Genetic testing, Biochemical profiles, LP
CT. MRI
Treatment
Decreasing CSF secretion by the choroid plexus - Acetazolamide and furosemide
Increasing CSF reabsorption - Isosorbide (effectiveness is questionable)
Surgical-Shunting, Choroid plexectomy,