SEMINAR“MANAGEMENT OF THE

HYDROCEPHALUS AND OUTCOME”

PRESENTED BY : DR. SURAJ JAIN

MODERATOR : PROF. Dr. V.K. RAINA

INTRODUCTIONHydrocephalus is common neurological problem. Attention to different hydrocephalic conditions come in light mainly by innovative development in imaging and operative methods. The strategy of treating hydrocephalus has changed, especially because of scientific approach to CSF circulation, by possibility of measuring CSF flow resistance and by MRI based flow studies of the CSF circulation. Treatment of hydrocephalus have changed from shunting which give rise to many complication and re operations to minimally invasive endoscopic procedures.

Definition - hydrocephalus is defined as pathological increase in intracranial CSF volume with increased intracranial pressure (ICP).CSF Dynamic

Secretion-Most CSF (70%) secreted in choroids plexus within ventricle

-Extra choroid CSF production occur in subarachnoid space-Transapendymally

Absorption-Mainly in arachnoid villi in superior sagital sinus

-Also occur in leptomeningis -Ventricular system-Lymphatics

SIGNS AND SYMPTOMS1) IN INFANTS

• rapid skull growth• macrocrania• bulging fontanelle• splitting of cranial sutures• shiny skin with distended scalp vein• sunset appearance• opthalmoplegia• papilloedema• frontal bossing• transcranial illumination

2. IN CHILDREN•headache•vomiting•alteration of consciousness•double vision•behavioral change•memory loss•papilloedema•convulsions

3. IN ADULTS•progressive headache•vomiting•progressive dementia•epileptic fits•urinary incontinence•limb weakness•papilloedema

INVESTIGATIONGoal of investigations:

1) To confirm diagnosis2) Differentiating between communicating and non communicating3) To know site of obstruction4) To know anatomical detail5) For follow up

1. CSF EXAMINATION2. HEAD CIRCUMFERENCE3. FUNDUS EXAMINATION4. RADIOLOGICAL INVESTIGATIONS A. X RAY SKULL•widening of sutures•silver beaten appearance•enlargement of pituitary fossa with erosion if dorsal sella•shallow posterior fossa B. ULTRASONOGRAPHY•atrial size is the most useful measurement of ventricular size•normal size in newborn is 7.6mm. more than 10mm indicates ventriculomegaly•Ventriculohemispheral ratio more than 35% indicates ventriculomegaly•Hanging choroid sign, nomally less than 25% more than 75 % indicates ventriculomegaly

Advantages of USG are• no exposure to radiation• can be performed bedside• axial, sagittal, coronal image can be obtained• can demonstrate lateral & 3rd ventricular

morphology, interventricular mass and periventricular leukomalakia

Disadvantages of USG are• operator dependant• not reproducible• cant demonstrate the exact site and cause of

obstruction• cant evaluate 4th ventricle and sub arachnoid space

pathology

CT SCANAdvantages of CT Scan are as follows• can be performed quickly• greater anatomical detail• preferential dilatation of occipital horn may be seen• can distinguish between communicating and non

communicating varietiesDisadvantages of CT Scan are as follows• provides only axial image• exposure to radiation• inferior to MRI for visualization of brain

stem/posterior fossa

MAGNETIC RESONANCE IMAGING Advantages are as follows

• provide greatest amount of anatomic detail• provide clues in etiology• differentiate between subdural effusion & enlarge sub

arachnoidal spaces• visualization of posterior fossa and brain stem• Cine MRI is useful to identify site of obstruction• no radiation exposure

RADIOGRAPHIC FEATURES• concomitant & proportional dilatation of temporal horn• enlargement of anterior & posterior recess of 3rd ventricle• shortening of mammilopontine distance• narrow ventricular angle• widening of radius of frontal horn• cortical effacement• attenuation of flow voids in 3rd ventricle, aqueduct of sylvius,

4th ventricle & transependymal oedema

MANAGEMENTTreat Cause of hydrocephalus:Medical Management :

1. Mannintol : 2. Hyperventilation :3. Loop diuretics :4. Steroid :5. Acetazolamide :6. Barbiturate coma :

SURGICAL TREATMENT :Goal of surgery:

To decrease ICPTo maximize the patient’s neurological

outcome and cognitive functionTo maximize the size of cortical mantle

while minimally complicationLP and Ventricular tap:These are the simple and quick methods

VP SHUNTIndication:In newborn and children:In patient without a treatable cause of hydrocephalus

Idiopathic hydrocephalusFailed III ventriculostomyCommunicating / obstructive hydrocephalusMyelodysplactic children with healing wound under tensionSing and symptom of brain stem compression develop in presence of ventriculomegaly

Indication of shunt operation in adult:Symptoms and signs of elevation of ICP in high pressure hydrocephalusProgressive signs of brain herniationProgressive dementia gait and urinary disturbance : Other indication in which endo III ventricular can

be appliedMultiple operated hydrocephalusArachnoid and porencephalic cystSpontaneous/ iatrogenic CSF leakageTemporary neutralization of elevated ICPContraindication :Absolute :Infection specific ventriculitisIntraventricular hemorrhageRelative :Arresteo or atropic hydrocephalusHydran encephaly

COMPLICATIONS

Common Uncommon

Cranial Subcutaneous Peritoneal

Infection Subdural hygromas

Shunt irrigation Peritonitis

Obstruction Subdural hematoma

Hemiparesis Hematoma

Shunt disconnection

Shunt fracture

PseudocystPerforation

hernias

Over drainage

1) Infection : 5-10% Risk of shunt infection is :Organism:Gram positive staphylococcus epidermis 40% cases, staphy aureuns 20%, propionbacterium,

streptococcus corneobacteriaGram negative organism 15%, pseudomonas enterobacteriumMultiple strain in 20% shunt infectionSign and symptoms:Shunt failureMeningismusHeadacheNausea / vomitingIrritabilityFever, lethargy acute abdomenInvestigations:CBCBlood cultureUrine cultureRadiographic shunt scanUSG abdomen/ CT scan abdomenCSF tapTreatments:IV antibiotics – broad spectrum, IV vancomycin + rifampicinIV cetraxone + gentamycinIntrathecal aminoglycoside-Cure rate of the shunt infection in situ with intravenous antibiotic along have reported in the

range of 30%.-Removal of the shunt.

Mechanical failure:Occur in the 17% all shunt cases.Is most common complication of shunt.Proximal occlusion is most common Shunt catheter can be occluded from variety of cause such as :Debris/ blood/ proteinIntraparenchymal placementChoroid plexusCoaptation of ventricular wallGliosisInfectionCatheter breakage of discontinuity is second most common cause

of the shunt failure.Shunt obstruction occur more than a year after insertion usually

because choroids plexus had occluded the ventricular catheter.

Symptom:HeadacheNausea/ vomitingDrowsinessIrritabilitySix nerve palsyBehaviour disturbanceSubcutaneous fluid collectionDiagnosis :

Pumping the shuntX-ray head, chest and abdomenCT scanShunt tap

Treatment :Replace either malfunction component or entire system.

Over drainage:When patient is sitting or standing the differential hydrostatic pressure of shunt system is proportional to alpha height of vertical column of CSF in peritoneal tube distal to the valve.This pressure lead to gravitational induced siphoning and negative ICP.

Symptom:Postural headacheNauseaLassitudeDiplopiaElevated ICP syndromeSlit ventricular syndromeOcclusion of aquaduct of sylviusSubdural haematomaOrthostatic hypotension

Treatment:Upgrading the valve to a higher pressureBy placing an antisiphan device By performing an endoscopic IIIrd ventriculostomy

Lumbar Peritoneal ShuntIndications:Communicating hydrocephalus with or without small or collapsed ventricular systemAdvantages:Extracranial courseAvoid complication of IIIrd ventriculostomy Contraindication:Obstructive hydrocephalusComplication:Overdrainage (spinal headache)- most common)Transient root symptom and signScoliosis / hyper lordosis / kyphoscoliosis – rare

Endoscopic IIIrd VentriculostomyCriteria:Candidate should have obstructive hydrocephalusCandidate should have dilated IIIrd ventricle which arbitary

define as > 1 cm in by coronal planeFloor of the 3rd ventricle should be suitable for fenestration i.e.,

attenuated or balging downward into inter pendicular cistern. Area of attenuation should be atleast 5 mm in diameter and should be in front of basilar artery.

Indication:Posterior fossa tumorLate 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 ventrical. In the patient with the repeated shunt failure

Contraindication:Chronic meningitisSub dural haemorrhage / intra ventricular

haemorrhageComplications:Infection rate 2-15% Bleeding for the basilar artery or its branches Hemiparesis, owing to damage to pedicle or its

perforating arteriesHypothalmic damage due to the proximity to

third ventricle

Endoscopic Choroid Plexus Coagulation:Indication:Infant with slowly progressive communicating hydrocephalus Success rate is 64% at 10 yrs follow upHydrocephalus as a result of CSF over production eg. Choroids plexus

papilloma and hyperplasiaCommunicating hydrocephalus where shunt insertion is not feasibleIntractable shunt failureContraindications:Obstructive hydrocephalus Rapidly progressive communicating hydrocephalus with acutely raised ICPComplications:Mortality

In 1 series on 125 patients mortality is 1.Post operative fit 1.9%Severe low pressure state (0.8%) Infection 0.5%Overall 35% patient remained shunt independent after 10 year follow up

CONCLUSIONIn new millennium shunt placement and

revision are low risk. Operation but long term risk of infection obstruction and antecedent neurological morbidity is quite high as no shunt system is ideal. The rapid development in the technology and enormous amount of clinically experience with CSF shunting point to next generation of shunts which could be ventriculosinus (superior sagital sinus) shunt or computerized electronic shunt, till that shunting should be avoid whenever possible and endoscopic III ventriculostomy should be attempted in appropriated selected patient, CSF diversion have lead to decrease mortality and have improved overall outcome in the patient with hydrocephalus.