Management of Hydrocephalus

Neurosurgery Rotation 3 UiTM MBBS Year 4 (2013/2014) 13th December 2013 1- MUHAMMAD KYIDIR BIN MOHD IDROS 2- NAZURAH NADIA BT. AZIMUDDIN 3- MUHAMAD ASRI B. ABD RAHMAN 4- MOHD RIDZUAN BIN HAMID 5- MOHAMMAD FIKRI B. ROSLI

BASIC ANATOMY AND PHYSIOLOGY

MUHAMMAD KYIDIR BIN MOHD IDROS

3/16/2014

ANATOMY OF VENTRICULAR SYSTEM

2 Lateral Ventricles Largest cavity Occupies large area of cerebral hemisphere Opens through interventricular foramen into the 3rd ventricle

3rd Ventricle Slit-like cavity between the two thalami (each side) Continues posteroinferiorly with Aqueduct of Sylvius 4th Ventricle Pyramidal in shape Posterior to the pons and medulla Extends inferoposteriorly Inferiorly narrow central canal in spinal cord Flows Foramen of Luschka and Foramen of Magendie

CSF PRODUCTION AND FLOW

Physiology of CSF Formation

Humans total volume of CSF is about 120-150 mL and the rate of CSF production is about 0.5 mL/min (400-500 ml/day) and the CSF turnover time of about 5 hours. Secreted by choroid plexus of: Inferior horn of each lateral ventricle Posterior portion of 3rd ventricle Roof of 4th ventricle

It has been estimated that 50-70% of the CSF is formed in the choroid plexuses and the remainder is formed around blood vessels and along ventricular walls.

CSF FLOW
2 Lateral ventricles

2 Foramen of monro
3rd ventricle Aqueduct of Sylvius 4th ventricle 2 lateral foramina of Luschka 1 midline foramen of Magendie Subarachnoid space

With the aids of ciliated ependymal cell, arterial pulsation of choroid plexus

CSF ABSORPTION
Subarachnoid space With the aids of cerebral arteries pulsation
Dead end-relies on pulsations of spinal arteries, movement of vertebral column, respiration, coughing and changing of body positions for further circulations Passive pressure dependent (Oneway valve)

2 ways:
Superiorly-inferior surface of cerebrum and later to lateral aspect of cerebral hemisphere Inferiorly-spinal cord and cauda equina before rises superiorly

Arachnoid granulation (villi)

Venous system (Superior sagittal sinus)

CSF PRESSURE
The pressure is kept remarkably constant In lateral recumbent position
Pressure measured by spinal tap/lumbar puncture is ~60-150 mm water

Pressure may be raised by straining, coughing and compressing internal jugular vein (raised ICP;one-pressure way causing no csf reabsorption, hence increases csf pressure) Production of CSF is not pressure regulated
Continues to be produced even if reabsorption mechanism is obstructed

Normal constituents of CSF

PHYSICAL CHARACTERISTICS OF CSF

Characteristics Appearance Total Volume Rate of production Composition Protein Glucose Chloride Number of cells Clear and colorless 150 ml ~0.5 ml/minute (~500ml/day)

0.15-0.45 g/l 0.5-0.85 g/l (50% blood glucose) 7.2-7.5 g/l

0-3 lymphocytes

Bacterial Meningitis
Appearance - Cloudy & Turbid White Cells - Raised neutrophils Red Cells - Absent Protein - High or Very High Glucose - Very Low

Viral Meningitis
Appearance - Normal White Cells - Raised lymphocytes Red Cells - Absent Protein - Normal or High Glucose - Normal or Low

Tuberculous Meningitis
Appearance - Normal or Slightly Cloudy White Cells - Raised lymphocytes Red Cells - Absent Protein - High or Very High Glucose - Very Low

Subarachnoid Hemorrhage Appearance - Usually blood stained White Cells - Normal Red Cells Present (Very High) Protein - Normal or High Glucose - Normal or Low

Guillan-Barr Syndrome Appearance - Normal White Cells - Normal Red Cells - Absent Protein - High Glucose - Normal or Low

Multiple Sclerosis Appearance - Normal White Cells - Raised lymphocytes Red Cells - Absent Protein - High Glucose - Normal

FUNCTIONS OF CSF
1. Cushions - protects CNS from mechanical trauma 2. Bouyancy

3. Reservoir and assists in the regulation of the contents of the skull - increases brain volume/blood volume will increase CSF volume 4. CNS nourishment 5. Removal of metabolites from CNS

HYDROCEPHALUS
NAZURAH NADIA

Definition
increased volume of cerebrospinal fluid (CSF) within the skull, most frequently in the ventricles.
Textbook of Clinical Neurology, 3rd ed

 The accumulation of excessive CSF within the ventricular system may d/t: 1. Impaired flow (obstructive/noncommunicating) 2. Impaired resorption (communicating) 3. Overproduction (communicating) increase ICP

Classification
1. Non-communicating Hydrocephalus (Obstructive) 2. Communicating Hydrocephalus (Nonobstructive)

1. Non-Communicating Hydrocephalus
Obstructive hydrocephalus CSF flow obstruction in the ventricular system

Ventricular System

Aqueductal stenosis Abscess Chiari malformation Dandy-Walker malformation Hematoma Infectious Klippel-Feil syndrome Mass lesions Tumors & neurocutaneous disorders Vein of Galen malformation Walker-Warburg syndrome X-linked
Clinical pediatric neurology : A sign & symptoms approach, Gerald M. Fenichel

2. Communicating Hydrocephalus
Non-obstructive hydrocephalus Impaired CSF resorption: Sub Arachnoid Hemorrhage(SAH) Functional impairment of the arachnoid granulations (congenital)

Sub Arachnoid Hemorrhage(SAH) Achondroplasia Basilar Impression Benign enlargement of subarachnoid space Choroid plexus papilloma Meningeal malignancy Meningitis Post-hemorrhage

Causes
Infants & children Congenital Acquired Adults Acquired

Examples-Congenital
DANDY WALKER MALFORMATION

Examples-Acquired
SUB ARACHNOID HEMORRHAGE(SAH)

Clinical features

Effects (clinical features)

The clinical features depend on: Age Cause Location of obstruction Duration Rapidity of onset

Setti S.Rengachary, Richard G. Ellenbogen. Principles Of Neurosurgery.Second Edition.2005.Elsevier Mosby

Premature Infants
Apnoea Bradycardia Convex, tense, nonpulsatile fontanelle Distended scalp veins Globoid head shape Rapid head growth

Setti S.Rengachary, Richard G. Ellenbogen. Principles Of Neurosurgery.Second Edition.2005.Elsevier Mosby

Infants
Irritability Vomiting Drowsiness Macrocephaly Distended scalp veins Frontal bossing positive Macewens sign poor head control lateral rectus palsy setting-sun sign
Setti S.Rengachary, Richard G. Ellenbogen. Principles Of Neurosurgery.Second Edition.2005.Elsevier Mosby

Time of development
After suture closed: Increase pressure Expansion of ventricles Progressive head enlargement closed suture cause abnormal head growth

Older children
Headache Vomiting Lethargy Diplopia Papilloedema Lateral rectus palsy Hypereflexia/clonus

Setti S.Rengachary, Richard G. Ellenbogen. Principles Of Neurosurgery.Second Edition.2005.Elsevier Mosby

Acute (high-pressure) hydrocephalus

o High ICP: Headache Nausea Vomiting Papilloedema o Abducens palsy & truncal ataxia, may, incorrectly, suggest a posterior fossa lesion (false localizing sign) o Episodic visual obscurations graying- dangerous pressure waves
Setti S.Rengachary, Richard G. Ellenbogen. Principles Of Neurosurgery.Second Edition.2005.Elsevier Mosby

Chronic or normal pressure hyrocephalus (NPH)

Clinical triad of NPH: Gait disturbance- magnetic, apraxic Urinary incontinence- uninhibited neurogenic bladder Dementia

Setti S.Rengachary, Richard G. Ellenbogen. Principles Of Neurosurgery.Second Edition.2005.Elsevier Mosby

Papilloedema

6th nerve palsy

Imaging studies for hydrocephalus

-MOHD RIDZUAN BIN HAMID-

Imaging modalities
Skull ultrasound Skull radiograph (x-ray) CT Scan MRI

Skull ultrasound
Uses high frequency sound wave Method of choice to diagnose intrauterine cases First investigatory method for infantile cases(6months-2y/o) - open anterior fontanelle, simple procedure, non-invasive Internal cranial structure (ventricles, parenchyma,vessels) are visualized in coronal and saggital planes

Frontal horns of Lateral ventricles

Normal Ventricles

Hydrocephalus

Ventricularhemispheric ratio
Level of Foramen of Monroe in coronal section Distance of the lateral wall of lateral ventricle from midline to the hemispheric width If > 0.35, suggestive of hydrocephalus

Skull radiograph
Simple, inexpensive and non invasive imaging method with great diagnostic value Used in older children
anterior fontanelle closed ultrasound cannot penetrate bony structure

Can detect several diagnostic signs

Enlarged cranium Wide spread / split sutures Disproportionate craniofacial ratio J-shaped sella silver beaten appearance of calvarium

Enlarged cranium

J-shaped sella turcica

Sella extends under anterior clinoid process and looks like J on side.

Silver beaten appearance

-Sign for chronic raised in ICP -Prominent convolutional markings

CT-Scan
Accurately demonstrate ventricular size and shape/presence of blood and calcium deposits Signs of increased ICP - compressed cerebral sulci - obliterated subarachnoid space - transependymal resorption of CSF into white matter Contrast CT -can detect abnormalities- tumor and abscess Limitation: -can only be performed predominantly in axial -require irradiation -less resolution than MRI

Compressed cerebral sulci Enlarged frontal horn

Enlarged 3rd ventricle Enlarged temporal horn

MRI
Can project the brain in axial,coronal,and saggital projections provide better anatomical detail of lesions causing hydrocephalus and is particularly useful in the diagnosis of aqueduct stenosis Can detect transependymal resorption and low grade gliomas more clearly than CT-scan Can detertmine CSF flow across aqueduct

Dilatation of Ventricles

Normal

Dilatation of lateral ventricles Dilatation of fourth ventricles

MRI sagittal image demonstrates dilatation of lateral ventricles with stretching of corpus callosum and dilatation of the fourth ventricle

Stenotic aqueduct

Chiari Malformation

Herniation of part of cerebellar into the foramen magnum

Dandy Walker Syndrome

Everted hypoplastic vermis (long arrow)
Large posterior fossa cyst (short arrow) Hypoplasia of the brainstem and cerebellum (b)

MRI/CT Criteria
Acute Hydrocephalus
Size of both temporal horns (>2mm)/ clearly visible (normally-barely visible) Evans ratio >30%. (Evans ratio-ratio of the largest width of the frontal horns to maximal biparietal diameter. Ballooning of frontal horns of lateral ventricles and third ventricle-might indicate aqueductal obstruction. Upward bowing of corpus callosum on saggital MRI.

Chronic Hydrocephalus
Temporal horns may be less prominent compared to acute hydrocephalus. Third ventricle may herniate into sella turcica Sella turcica may be eroded
Macrocrania (ie, occipitofrontal circumference
>98th percentile) may be present.

Corpus callosum maybe atrophied

Management of Hydrocephalus
Mohammad Fikri Bin Rosli

Management of Hydrocephalus
Acute hydrocephalus: emergency as condition may progress over minutes or hours to coma and death.

Management

Medical

Surgical

Medical management
To delay surgical intervention Not effective in long term treatment of chronic hydrocephalus

Reduce CSF production

Acetazolamide carbonic anhydrase inhibitors Furosemide loop diuretics

Increase CSF reabsorption

Isosorbide

S.Rengachary, G.Ellenbogen, Principle of Neurosurgery 2nd Edition

Surgical management
1) External ventricular drainage (EVD) Temporary measure to relieve hydrocephalus Catheter are inserted to the right of midline, anterior to coronal sutures to enable the tip of catheter rest adjacent to the foramen of monro in lateral ventricle Catheter connected to a drain set which the CSF will drains when the ventricular pressure exceeded 20mmHg

Bailey & Loves Short Practice of Surgery 26th Edition

 Intrathecal antibiotic delivered through EVD

Bailey & Loves Short Practice of Surgery 26th Edition

2) Shunts
To establish a communication between the CSF and a drainage cavity by a tube
Types: Ventriculoperitoneal

Ventriculopleural
Ventriculoatrial Lumbar-peritoneal 3 routes for ventricular catheter insertion: Frontal, Parietal and Occipital

Bailey & Loves Short Practice of Surgery 26th Edition

Ventriculoatrial shunt

Ventriculoperitoneal shunt

Ventriculopleural shunt

Consist of 3 components: Proximal ventricular catheter One way valve: permits CSF flows out of ventricular Opening pressure of the valve can be high, medium or low High pressure may cause inadequate drainage of CSF Low pressure may cause over-drainage of CSF Distal catheter: allow the fluid to flows into the reservoir, allow CSF to be aspirated

Bailey & Loves Short Practice of Surgery 26th Edition

Complications of shunts:
Shunt infection Approximately 1-15% of all shunting procedures Usually due to Staph. Epidermidis and Staph. Aureus Symptoms: Irritability, Anorexia, Low grade fever Treatment Antibiotic Removal of infected shunt Placement of external ventricular drainage. Indication of implanting new shunt: Negative 5-7 days consecutive daily CSF cultures CSF white blood cell <50 CSF protein < 500 mg/dL
S.Rengachary, G.Ellenbogen, Principle of Neurosurgery 2nd Edition

 Shunt blockage due to cellular and proteinaceous debris, choroid plexus adhesion or blood clot Low pressure syndrome due to over-drainage of CSF which consist of headache worsened on standing, neck pain, and nausea Subdural hematoma or subdural hygroma due to collapsed ventricles causing accumulation of fluid in subdural space

S.Rengachary, G.Ellenbogen, Principle of Neurosurgery 2nd Edition

3) Endoscopic third ventriculostomy Useful in obstructive hydrocephalus due to aqueduct stenosis Neuroendoscope inserted into frontal horn of lateral ventricle and then into third ventricle via foramen of monro Complication Reblockage Damage to basillar artery Damage to the fornix

Bailey & Loves Short Practice of Surgery 26th Edition

Other type of hydrocephalus

Hydrocephalus ex vacuo Normal pressure hydrocephalus TBM and hydrocephalus

Hydrocephalus Ex Vacuo
Occur in the presence of brain damage due to stroke, injury or actual shrinkage of brain substance Brain are atrophied and wasted Features are: Increased production of CSF Cerebral atrophy Dilatation of the ventricles ICP usually is normal. HOWEVER Causes: Alzheimer disease, Multiple sclerosis, Multiple strokes, Huntington disease, Leukodystrophies.

http://www.medicinenet.com/hydrocephalus/page2.htm

Mild to moderate cortical atrophy. Large ventricles, particularly the third ventricle and inferior horns of the lateral ventricles.

Normal Pressure Hydrocephalus

Clinical symptoms complex caused by build up of CSF in brain ventricles and cavities Describe as hydrocephalus in absence of papilloedema with normal CSF opening pressure Usually presented in elderly Causes: De novo Following brain insult: subarachnoid hemorrhage, head injury, meningitis, tumour Pathophysiology: Initially, it is due to gradual blockage on the flow of CSF. Then, it may begin as transient high pressure hydrocephalus with subsequent ventricular enlargement With further enlargement of ventricles, CSF pressure return to normal
http://emedicine.medscape.com/article/1150924-overview Bailey & Loves Short Practice of Surgery 26th Edition

 Triad of clinical features: Gait disturbance, urinary incontinence and cognitive decline (dementia) Opening pressure on LP typically normal

Lumbar infusion testing can also be done to measure CSF pressure.

Imaging usually reveals ventriculomegaly Treatment Ventriculoperitoneal shunt

http://emedicine.medscape.com/article/1150924-overview Bailey & Loves Short Practice of Surgery 26th Edition

Tuberculous Basal Meningitis and Hydrocephalus

Caused by Mycobacterium Tuberculosis Usually affect young population Pathophysiology: Occur after initial infection within lung with dessimination of regional lymph node Followed by bacilli seedling into meninges or brain parenchyma Formation of small subpial or subependymal foci of metastatic caseous lesion Rich Foci

Rich Foci may increase in size until it ruptures into subarachnoid space

http://emedicine.medscape.com/article/1166190-overview

 Hydrocephalus is common sequelae of TBM

CT scan intense meningeal enhancement Management:

Medical therapy: steroids eg: prednisolone and anti-tuberculosis drug.

Surgery: Ventriculoperitoneal shunt
Rich Foci

http://emedicine.medscape.com/article/1166190-overview

References
Bailey & Loves Short Practice of Surgery 26th Edition S.Rengachary, G.Ellenbogen, Principle of Neurosurgery 2nd Edition http://emedicine.medscape.com/article/1166190-overview http://emedicine.medscape.com/article/1150924-overview

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