hydrocephalus
TRANSCRIPT
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Hydrocephalus
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Introduction
Defined as abnormal accumulation of CSF in ventricles and/or subarachnoid space, typically associated with ventricular dilatation and raised ICP
Incidence as isolated congenital disorder 1/1000 live births and with spina bifida in 1/1000 live births
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Normal CSF physiology
Produced by choroid plexus in lateral,third & fourth ventricles by ultrafiltration at rate of 0.3 – 0.35 ml/min i.e. 500ml/day
Average CSF volume is 65 to 140 ml
Normal CSF pressure is 4-5cms of water in infants, 4-10cms in older children & 15cms in adults
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CSF flow
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Classification
On location of block Communicating Non communicating
On cause Physiologic – due to overproduction by CP
papilloma Nonphysiological – due to any other cause
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Pathology
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Signs & Symptoms
Premature infants
Apnea
Bradycardia
Tense AF
Rapid head growth
Globoid head
Older children
Headache
Vomiting
Lethargy
Diplopia, blurred vision
Papilledema ,Lateral rectus palsy
Hyperreflexia, clonus
Infants
Drowsiness, irritability
Vomiting
Macrocephaly, tense fontanelle
Frontal bossing
Distended scalp veins
Poor head control
Lateral rectus palsy, sun set sign
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Signs & Symptoms in adults
progressive headache vomiting progressive dementia epileptic fits urinary incontinence limb weakness papilloedema
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Investigations
Goal of investigations: To confirm diagnosis
Differentiating between communicating and non communicating
To know site of obstruction
To know anatomical detail
For follow up
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Head circumference
35 – 37 cms at birth
Increases at rate of 2cm/ mth for 1st 3 mths 1cm/mth for next 3 mths 0.5cm/mth for the next 6 mths
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CSF examination
Lumbar puncture should be done with care as coning can occur in non communicating hydrocephalus
Pyogenic meningitis, TBM, and intraventricular bleed can be diagnosed
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Radiological investigations
X RAY SKULL Widening of sutures
Silver beaten appearance
Enlargement of pituitary fossa with erosion of dorsal sella
Shallow posterior fossa
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Ultrasonography
Non invasive, no exposure to radiation
Can show lateral & third ventricle but not 4th ventricle or subarachnoid space
Can measure resistive index which is a sensitive indicator
atrial size most useful measurement of ventricular size
Ventriculohemispheral ratio more than 35% indicates ventriculomegaly
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CT scan Provide greater anatomical detail
Can distinguish between communicating and non communicating
With IV contrast tumours / abscess/ bleed/ Ca deposit can be seen
Provides only axial image
Inferior to MRI for visualization of brain stem/posterior fossa
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CT scan
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Magnetic resonance imaging
Provide greatest amount of anatomic detail
Differentiate between subdural effusion & enlarge sub arachnoidal spaces
Visualization of posterior fossa and brain stem
Cine MRI is useful to identify site of obstruction
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Magnetic resonance imaging
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Medical Management
Mannitol decreases ICP
Loop diuretics, Acetazolamide decrease CSF production for a few days
Doesn't resolve ventriculomegaly or affect intellectual outcome
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Surgical treatment
Shunt surgeries
Third Ventriculostomy
Choroid plexectomies/ coagulation
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Shunt surgery
Ventriculoperitoneal shunt – most commonly done
Ventriculoatrial shunt Ventriculopleural shunt Ventriculogallbladder shunt Lumboperitoneal shunt
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VP shunt classification
According to type of valve - spring ball - slit valve - diaphragm
According to pressure of opening - ultra low pressure - low - medium (most commonly used) - high
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VP Shunt - Indications In newborn and children:
Idiopathic hydrocephalus Communicating / obstructive hydrocephalus Myelodysplactic children with healing wound under tension Signs and symptoms of brain stem compression develop in
presence of ventriculomegaly
In adults Signs of elevation of ICP in high pressure hydrocephalus Signs of brain herniation Progressive dementia, gait and urinary disturbance Arachnoid, porencephalic cyst Spontaneous/ iatrogenic CSF leakage Temporary neutralization of elevated ICP in tumours
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VP shunt
ContraindicationsAbsolute Infection specifically ventriculitis Intraventricular hemorrhage Recent peritonitis, Adhesions
Relative Arrested or atrophic hydrocephalus Pending abdominal surgery
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Lumbar Peritoneal Shunt Indications Communicating hydrocephalus with or without small or collapsed
ventricular system
Advantages Extracranial course Avoid complication of IIIrd ventriculostomy
Contraindication Obstructive hydrocephalus
Complication Overdrainage (spinal headache)- most common) Transient root symptom and sign Scoliosis / hyper lordosis / kyphoscoliosis – rare
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Complications of Shunt surgery
Three main groups
1. Mechanical failure – proximal, valve or distal
2. Infection – mainly by staph. Epidermidis & aureus
3. Overdrainage – causing headache
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Endoscopic III Ventriculostomy
Criteria Obstructive hydrocephalus Dilated III ventricle defined as > 1 cm in by coronal plane Floor of the 3rd ventricle suitable for fenestration i.e., attenuated or
bulging downward into interpeduncular cistern.
Indication Posterior fossa tumor
Late onset (over 24 yrs of age) aqueduct block such as tectal tumor
New born with myelomeningocele and associated blockage either at aqueductal or exists of the 4th ventricle
In the patient with the repeated shunt failure
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Endoscopic III Ventriculostomy
Contraindication Chronic meningitis Sub dural haemorrhage / intra ventricular haemorrhage
Complications Infection Bleeding from basilar artery can cause death Hemiparesis, owing to damage to pedicle or its
perforating arteries Hypothalmic damage due to proximity to III ventricle
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Treatment of Hydrocephalus diagnosed in utero
Can cause cephalopelvic disproportion & inhibit labour
USG used for diagnosis
MRI after engagement of head used to visualise cerebral morphology
Severe brain malformation treated by cephalocentesis
Results of ventriculoamniotic shunts discouraging
Babies with normal cerebral morphology delivered by LSCS when maturity documented & treated by shunt surgery
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Fetal USG
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Outcome & Prognosis
Regular follow up essential
Baseline scan post shunt for ventricular size
Prognosis depends on brain morphology & factors like perinatal ischemia, IVH, ventriculitis
Number of shunt revisions / malfunctions not key factors in outcome
Cause of death in these pts is primary disease progression or factors related neither to hydrocephalus nor its treatment