hydrocephalus diagnosis and management
TRANSCRIPT
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HYDROCEPHALUS
EVALUATION & MANAGEMENT
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Anatomy and PhysiologyVentricular System & CSF
• 80% from the choroid plexus• Interstitial spaces Production• Ependymal lining• Dura of nerve root sheaths
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CHOROID PLEXUS
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Anatomy and PhysiologyCSF
• Absorbtion: - Primarily by the Arachnoid villi
• Rate of production- 0.3ml/min or approx 450ml/24 hrs
• Turnover: 3 times/day
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CSF CIRCULATION
• Lateral ventricles – Foramen of Monro
• 3rd Ventricle – Cerebral Acqueduct
• 4th Ventricle – F. of Magendie & Luschka
• Perimedullary and Perispinal subarachnoid spaces – upward to
the basal cistern
• Superior and lateral surfaces of the cerebral hemispheres
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CSF Flow path
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CSF PRESSURE
• The CSF volume and pressure are
maintained every minute by the
systemic circulation
• CSF pressure is in equilibrium
with capillary pressure (arteriolar
tone)
• Hypoventilation – ↑ in blood PCO2 – ↓ pH & ↓ arteriolar resistance – ↑ cerebral blood flow – ↑ CSF pressure
• Hyperventilation has the
opposite effect
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CSF PRESSURE
• Normal adult intracranial pressure 2-
8 mmHg
• Up to 16 mmHg are considered
normal
• ICP higher than 40 mmHg or lower BP
may combine to cause ischemic
damage to the brain
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Definition
• An increase in CSF volume in an enlarged ventricular system resulting
- primarily from decreased absorbtion - rarely b’coz of increased production• Prevalance: 1-1.5%• Incidence: 0.3-3.5%- Upto 20% after SAH- 1% after meningitis
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Definition
• Results in ventricular enlargement• Lat ventricles - frontal and occipital horns• Volumes decrease in cerebral sulci, fissures
and cisterns
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Classification• Functional
• Clinical
• Age wise
• Pathological
• ICP/ R-out
• Special Types
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Functional
• Communicating:- Block at the level of the arachnoid
granulations
• Non-communicating:- Block proximal to the arachnoid granulations
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Clinical
• High pressure hydrocephalus - Acute - Chronic• Normal pressure hydrocephalus
• Arrested hydrocephalus
• Hydrocephalus ex vacuo
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Age wise
• Paediatric• Juvenile/Adult
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Pathological
• Congenital1. Chiari type 1 malformation2. Chiari type 2 malformation and/or
Meningimyelocele3. Primary aqueductal stenosis4. Secondary aqueductal gliosis ( germinal matrix
hge)5. Dandy Walker malformation6. Rare X- linked disorder
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Pathological• Acquired1. Infectious - Post meningitic - Granuloma - Cysticercosis - Abscess
2. Post haemorrhagic - SAH - IVH - Trauma
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Pathological
• Acquired 3. Secondary to mass effect - Non neoplastic - Neoplastic - Choroid plexus papilloma - Post operative - Neurosarcoidosis - Assoc with spinal tumours - Constitutional ventriculomegaly
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ICP
• High Pressure - Monitored ICP > 15mmhg - B waves - R out increased
• Normal Pressure - Monitored ICP < 15mmHg - R out increased
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Special Types
HYYDROCEPHALUS EX VACUO• enlargement of the ventricles due to loss of
cerebral tissue (cerebral atrophy)• usually as a function of normal ageing• Accelerated by Alzheimer's disease,
Creutzfeldt-Jakob, Alcoholism
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Special TypesEXTERNAL HYDROCEPHALUS• enlarged subarachnoid spaces over the frontal poles in the first year of life • ventricles are normal or minimally enlarged • may be distinguished from subdural hematoma by the "cortical vein sign" • usually resolves spontaneously by 2 years of age
• Etiology :• Unclear • Defect in CSF resorption is postulated• External hydrocephalus (EH) may be a variant of communicating
hydrocephalus
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Special Types
ARRESTED HYDROCEPHALUS• Compensated hydrocephalus interchangeably• There is no progression or deleterious
sequelae requiring CSF shunting • Criteriae in the absence of a CSF shunt: - Near normal ventricular size - Normal head growth curve - Continued psychomotor development
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Special Types
OTITIC HYDROCEPHALUS• Obsolete term• Describes the increased ICP in patients with
otitis media
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Special Types
HYDRANENCEPHAL Y • A post-neurulation defect• Total or near-total absence ofthe cerebrum • Intact cranial vault and meninges• Intracranial cavity being filled with CSF• There is usually progressive macrocrania• Most commonly cited cause : B/L ICA infarcts• Infection - Congenital or neonatal herpes - Toxoplasmosis - Equine virus
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Special Types
ENTRAPPED FOURTH VENTRICLE • AKA isolated fourth ventricle, • 3rd Ventricle X 4th ventricle X Foramina of
Luschka or Magendie- Post-infectious hydrocephalus( fungal) - Repeated shunt infections• Choroid plexus of the 4th ventricle : produces
CSF which enlarges the ventricle
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Special Types
NPH• Classic triad: - Dementia - Gait disturbance - Urinary incontinence • Communicating hydrocephalus on CT or MRI • Normal pressure on random LP • Symptoms remediable with CSF shunting
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NPH
• Etiology- Post SAH - Post-traumatic - Post-meningitic- Following posterior fossa surgery - Tumors including carcinomatous meningitis - Also seen in -15% of patients with Alzheimer's disease - Deficiency of the arachnoid granulations- Aqueductal stenosis
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CLINICAL FEATURES
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INFANCY• Head grows at alarming rate with hydrocephalus.
– First sign: Bulging pulsatile fontanelles
– Tense, non-pulsatile anterior fontanelle
– Dilated scalp veins
– Thin skull bones with separated sutures
• Cracked pot sounds on percussion : Mc Ewans
sign
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INFANCY
• Depressed eyes or SUN SET sign
– Eyes downward with sclera visible
above
• Pupils sluggish with unequal response to
light
• Irritability, lethargy, feeds poorly,
• Changes in Level of Consciousness
• Arching of back (Opisthotonus)
• Lower extremity spasticity
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INFANCY
• Brain Stem Compression
– Swallowing difficulties, Stridor, Apnea, Aspiration,
Respiratory difficulties
• Lower Brainstem Dysfunction
– Difficulty in sucking and feeding
– High-pitched shrill cry
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INFANCY
• Emesis, Somnolence, Seizures, and Cardio Pulmonary Distress
• Severely affected infants may not survive neonatal period
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CHILDHOOD
• Headache on awakening, improvement following emesis or sitting
• Papilledema, strabismus, and Extrapyramidal signs, ataxia
• Irritability, Lethargy, Apathy, Confusion, and often incoherent
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SYMPTOMS AND SIGNS
• Irritability
• Poor feeding
• Headache
• Nausea, vomiting
• Diplopia
• Visual impairment
• Dementia
• Incontinence
• Gait disturbances
• Accelerated head growth
• Bulging fontanelles
• Forced down gaze
• Developmental delay
• Exotropia
• Papilledema
• Posturing
• Bradycardia
• Apnea / Death
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Evaluation
• Clinical• CT• MRI• ICP• R (out)• Isotope cisternography
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Clinical
• Occipito Frontal Circumference- OFC of a normal infant = Distance from Crown to
Rump• Indicators:- Crossing curves- Head growth > 1.25cm/wk- OFC approaching 2 SD above normal- Out of proportion with body length or weight, even
if normal for age
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CT CRITERIAE
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CT CRITERIAE
<40% - Normal
FH/ID 40-50% - Borderline
> 50% - Hydrocephalus
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Evan’s Index
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CT/ MRI FindingsAcute Hydrocephalus
• Preferential AP dilatation of the Temporal Horns > 2mm
• Ballooning of the Frontal Horns and 3rd Ventricles (Mickey Mouse sign)
• Periventricular interstitial edema• Flattening of the Inter-hemispheric and Sylvian
fissures• Upward bowing of corpus callosum on sagittal MRI• 4th Ventricle normal in size
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CT/ MRI FindingsChronic Hydrocephalus
• Temporal horns may be less prominent• 3rd ventricle may herniate into Sella Turcica• Erosion of Sella• Corpus callosum atrophy• Irreversible white matter demyelination
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R (Out)
• Assesses the degree of blockage to CSF absorbtion back into the blood stream
• Simultaneous infusion of artificial CSF and measurement of ICP
• Spinal subarachnoid space cannulated• ICP monitor inserted• Calculated resistance value high
Better response to surgery
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Isotope Cisternography
• Radioisotope injected into Lumbar Sub-arachnoid space
• Absorbtion of CSF monitored periodically over 96 hrs
• Positive cisternogram does not predict response to shunt surgery
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TREATMENT OF HYDROCEPHALUS
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THERAPEUTIC MANAGEMENT
• Goals:
– Relieve hydrocephaly
– Treat complications
– Manage psychomotor problems
– Usually surgical
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Drug Therapy
• The choroid plexus shares many ion pumps and enzyme
systems with renal tubular epithelium
– Acetazolamide:
Start @ 25mg/kg/day PO TID
Increase @ 25mg/kg/day to 100mg/kg/day
Simultaneously start Frusemide @1mg/kg/day
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Drug Therapy
To counteract acidosis: • tricitrate (Polycitra®) 4 ml/kg/day divided QID (each ml is
equivalent to 2 mEq of bicarbonate, and contains 1 mEq K+ and 1 mEq Na+)
• measure serial electrolytes, and adjust dosage to maintain serum HC03 > 18 mEqIL .
• change to Polycitra-K® (2 mEq K+ per ml, no Na+) ifserum potassium becomes low
• or to sodium bicarbonate if serum sodium becomes low
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Drug Therapy
• Watch for electrolyte imbalance and acetazolamide side effects:
- Lethargy - tachypnea- diarrhea - paresthesias • Perform weekly CT scan and insert ventricular shunt
if progressive ventriculomegaly occurs. • Otherwise, maintain therapy for a 6 month trial, then
taper dosage over 2-4 weeks
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Spinal Taps
• HCP after IVH may be transient• Serial taps (ventricular or LP) may temporize until
resorption resumes • LPs only for Communicating HCP• No reabsorption when the protein content of the CSF
is < 100 mg/dl
Spontaneous resorption unlikely
SHUNTING
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Surgical Modalities
1. Choroid Plexectomy2. 3rd Ventriculostomy3. Shunts
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Choroid Plexectomy
• Described by Dandy in 1918 for communicating hydrocephalus
• May reduce the rate but does not totally halt CSF production
• Open surgery associated with a high mortality rate
• Endoscopic choroid plexus coagulation - 1910
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3rd Ventriculostomy
• Resurgence of interest in third ventriculostomy (TV) with the recent increased use ofventriculoscopic surgery
• Indications: - Obstructive HCP. - Mgt of shunt infection - Subdural hematomas after shunting - Slit ventricle syndrome
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3rd Ventriculostomy
• Contraindications: - Communicating Hydrocepalus - Tumor - Previous shunt - Previous SAH - Previous whole brain radiation - Significant adhesions visible when perforating
through the floor of the 3rd ventricle at the time of performance of TV
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3rd Ventriculostomy
• Complications- Hypothalamic injury - Transient 3rd and 6th nerve palsies - Uncontrollable bleeding - Cardiac arrest - Traumatic basilar artery aneurysm
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Shunts
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Types of Shunt
Shunt Types By Categorya. VP Shunt»Most commonly used shunt in modern era» Lateral ventricle is the usual proximal location» Intraperitoneal pressure
b. Ventriculo-atrial shunt (Vascular shunt)» Through jugular veins to sup. Vena cava» Treatment of choice in abdominal abnormalities
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c. Torkildsen shunt: »Shunting ventricle to cisternal space»Rarely used»Effective only in acquired obstructive
hydrocephalus
d. Miscellaneous:»Pleural space»Gall bladder»Ureter/Urinary Bladder
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e. Lumbo-peritoneal shunt:
»Only for communicating hydrocephalous
f. Cyst/Subdural-Peritoneal shunt:
»Draining arachnoid cyst/subdural
hygroma cavity
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SHUNT MATERIALS
• Shunts are composed of Silastic material made from silicone.
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VP SHUNT
• Shunt systems include three components: – Ventricular catheter
– One way valve
– Distal catheter
• The ventricular catheter – Straight piece of tube
– Closed on the proximal end
– With multiple holes upto 2cm for the entry of CSF
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VA Shunt
• The VA shunt
– Must be accurately located
– Requires frequent revisions
– Distal end position to be maintained
– Infection may be more serious
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VP SHUNT
• If both the VPS & VAS do not function to absorb CSF the shunt have to
placed in the pleural space
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POST-OP CARE
• Observe for signs of Increased ICP
– Assessment pupil size
– Cushing’s Reflex
– Abdominal distention
• due to CSF peritonitis or post-op ileus due to catheter placement.
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Complicationsi. General:
a. Obstructionb. Disconnectionc. Infectiond. Erosion through Skine. Seizuresf. Metastatic routeg. Silicone allergy
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• VP Shunt- Inguinal hernia- Hydrocele- Peritonitis- Intestinal Obstruction- Volvulus- Migration of tip to scrotum/ bowel/ stomach- Malposition of tip- Over-shunting- Needs frequent length adjustment
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VA shunt:
– Requires repeated lengthening:
– High risk of infection/septicaemia:
– Risk of retrograde flow of blood: in case of valve
malfunction (rare)
– Shunt embolus
– Vascular complications: perforation,
thrombophlebitis, pulmonary micro-emboli
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LP Shunt:– Laminectomy incurs 15% chance of scoliosis
– Progressive cerebellar tonsillar herniation (up to 70%)
– Slit ventricle syndrome
– Overshunting is harder to control
– Difficult proximal end revision (if required:
– Lumber radiculopathy
– CSF leak
– Difficult pressure regulation
– Bilateral 6th, 7th, nerve dysfunction due to overshunting
– High incidence of arachnoiditis & adhesions