hydrocephalus: disorder of excessive amount of cerebrospinal fluid which accumulates in the ventricles and subarachnoid space
- CSF is produced by the choroid plexus -- epithelium and connective tissue in cerebral ventricles
- epithelial cells produce CSF using active transport which is dependent upon carbonic anhydrase
- CSF production is generally constant unless intracranial pressure is increased (absorption matches the production)
- production rate ~20mL/hour, complete turnover 3-4 time a day
- hydrocephalus results from imbalance between inflow and outflow
- obstruction of circulation
- inadequate absorption
- overproduction
- leads to increased intracranial pressure and ventricular dilation
- etiology: congenital, neural tube defects, CNS malformations, intrauterine infections, choroid plexus carcinoma, infections, tumors, post-hemorrhagic
- symptoms: headache, N/V, personality/behavior changes, lethargy
- bradycardia, hypertension, altered respiratory rate
- macrocephaly
- compression of cranial nerves
- Progressive (need to manage or will get worse)
- most effective treatment: surgical drainage using a ventriculostomy shunt (prevents excess accumulation of CSF, shunts CSF to either systemic circulation or peritoneum)
- Medical therapy:
- diuretics: furosemide and acetazolamide decrease CSF production
- used for short periods in slowly progressive hydrocephalus that is too unstable for surgery or until surgery can be done
- diuretics generally not effective for infants
- acetazolamide can be used for: acute mountain sickness, edema, glaucoma, retinal edema,
- need to dose adjust for renal impairment, caution in liver disease (can induce coma
- Mechanism of action: inhibits carbonic anhydrase from catalyzing the reversible hydration of carbon dioxide and dehydration of carbonic acid
- delays abnormal paroxysmal excessive discharge from CNS neurons and affects promotion of diuresis and urinary alkalinization
- adverse effects: metabolic acidosis, stevens Johnson syndrome, hepatic necrosis, agranulocytosis, thrombocytopenia, angle-closure glaucoma
- monitoring: CBC and electrolytes
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