MODERATOR; DR.M.D.RAVI PRESENTER; DR.

CHARANRAJ

Vascular Supply
About 18% of the total blood volume in the body

circulates in the brain, which accounts for about 2% of the body weight. The blood transports oxygen, nutrients, and other substances necessary for proper functioning of the brain tissues and carries away metabolites. Loss of consciousness occurs in less than 15 seconds after blood flow to the brain has stopped, and irreparable damage to the brain tissue occurs within 5 minutes.

Arterial supply
The circle of Willis (named after the English

neuroanatomist Sir Thomas Willis) is a confluence of vessels that gives rise to all of the major cerebral arteries. It is fed by the paired internal carotid arteries and the basilar artery. When the circle is complete, it contains a posterior communicating artery on each side and an anterior communicating artery. The circle of Willis shows many variations among individuals. Despite these variations, occlusion of each of the major cerebral arteries usually produces a characteristic clinical picture.

CIRCLE OF WILLIS
Located at base of brain
Anterior part lies in front Formed by:
Terminal part of the ICA

of optic chiasm Posterior part situated just below the mamillary bodies Allows for excellent collateral circulation Occlusion of an individual artery Often completely compensated by collateral circulation through the CoW

Proximal parts of the

anterior, middle and posterior cerebral arteries Anterior and posterior communicating arteries

1 Vertebral artery 2 AICA 3 Basilar artery 4 Superior cerebellar artery 5 Posterior cerebral artery 6 Posterior communicating artery 7 Middle cerebral artery 8 ICA 9 Opthalmic artery 10 Anterior cerebral artery 11 Anterior communicating artery 12 Hypothalamic artery 13 Anterior choroidal artery

INTERNAL CAROTID ARTERY

Arises from the Passes anteromedially

COMMON CAROTID ARTERY Enters skull through carotid canal in the temporal bones (petrous)

through the cavernous sinus Divides at medial end of the lateral sulcus Anterior and Middle cerebral arteries Anterior circulation of the brain

Branches of ICA
Anterior cerebral arteries Smaller terminal branch of the ICA
Enters longitudinal fissure Connected to the opposite anterior cerebral

artery by anterior communicating artery (part of the CoW)

Curves over corpus callosum

Central branch supply deep masses of gray matter w/in the cerebral hemisphere Supply:
Cortical branches supply all medial surface of cerebral cortex up to parieto-occipital sulcus Corpus callosum Approximately 1 inch of the frontal and parietal cortex on the superior aspect of their lateral surface (this include the leg area of the precentral gyrus) Anterior portions of the basal ganglia and internal capsule

 Middle Cerebral Arteries

Largest
Runs laterally in the lateral sulcus Cortical branches supply entire lateral surface of

the hemisphere EXCEPT Area supplied by anterior cerebral artery Inferolateral surface supplied by posterior cerebral artery Occipital pole

Supply all motor area except leg area. Supply parts of the internal capsule and basal ganglia. Central branches supply deep masses of gray matter within the cerebral hemisphere

 Ophthalmic artery Enters orbit through optic canal, below and lateral to optic nerve Supplies the eye, including retina and optic nerve Posterior communicating

Choroidal artery
Enter inferior horn of

artery
Runs backward to join

lateral ventricle to supply choroid plexus Branches may help supply the optic tract, LGB, internal capsule and crus cerebri

posterior cerebral artery at interpeduncular fossa Deep depression on inferior of midbrain between cerebral peduncles Part of the circle of willis

VERTEBRAL ARTERY
Branch of the 1st part of the subclavian

artery
Acends the neck through the transverse

foramina of upper 6 cervical vertebrae

Enters skull through foramen magnum

Cranial branches
Meningeal arteries Anterior and posterior spinal arteries Posteroinferior cerebellar artery Largest branch of the vertebral artery and supplies parts of the cerebellum and the dorsolateral portion of the rostral medulla Occlusion: lateral medullary syndrome Medullary arteries Along with posteroinferior cerebellar artery, supply most of the medulla

 At lower border of pons, vertebral arteries unite to form the BASILAR ARTERY
Ascends along the ventral midline of the pons and

terminates near the rostral border of pons by dividing into 2 posterior cerebral arteries
Vertebrobasilar arterial system

posterior circulation of brain

Branches:

Branches to pons, cerebellum, internal ear Labyrinthine artery Follows the course of the CN VIII and supplies inner ear Anterior inferior cerebellar artery Supplies part of the pons and the anterior and inferior regions of the cerebellum Superior cerebellar artery supplies part of the rostral pons and superior region of the cerebellum Pontine branches supply most of pons

Posterior cerebral arteries

Formed by the terminal bifurcation of the basilar artery Anastomosis with the posterior communicating artery in the CoW Supply: Lateral surface of the hemisphere occipital pole and inferior temporal lobe Medial surface of the hemisphere occipital lobe and posterior 2/3 of temporal lobe

VENOUS DRAINAGE
The venous drainage of the brain and coverings

includes:
the veins of the brain itself, the dural venous sinuses, the dura's meningeal veins, and the diploic veins between the tables of the skull.

VENOUS DRAINAGE OF BRAIN

Veins of the brain have no muscular tissue

in the wall and no valves

Veins Located in the SAS Venous sinuses located between 2 layes of

the dura

Superficial veins Drain the cortex and the more superficial hemispheric white mater mainly into the superior sagittal and cavernous sinuses Deep or internal veins Drain the deep hemispheric white mater and basal ganglia into the 2 internal cerebral veins w/c unite to form the great cerebral vein Great cerebral vein Formed by the union of 2 internal cerebral veins and drains into the straight sinus

Superficial and deep veins of the brain drain into the dural venous sinuses (which in turn drain into the internal jugular veins)

NEURAL TUBE DEFECTS

A major birth defect caused by abnormal development

of the neural tube, the structure present during embryonic life which gives rise to the central nervous system -- the brain and spinal cord.

 Neural tube defects (NTDs) are among the most

common birth defects that cause infant mortality (death) and serious disability.

Neurolation occurs in 3 steps:

1. Formation of the Neural Plate

2. Bending of the Neural Folds With Formation of the Neural Groove and Neural Folds

Elevation of the Neural Folds

3. Convergence of the Neural Folds

Fusion of the Neural Folds

Neural Tube Development

Normal embryological development
Neural plate

development -18th day Cranial closure 24th day (upper spine) Caudal closure 26th day (lower spine)

Embryology

Incidence and Prevalence

Incidence 1/1000 Prevalence Increased incidence in families of Celtic and Irish heritage (genetic or environmental?) Increased incidence in minorities (genetic or environmental?) Increased incidence in families

Etiology
Neural Tube defects may result from: Largely unknown
Combination of environmental and

genetic causes
Teratogens

Nutritional deficiencies - notably, folic acid deficiency A high fever during pregnancy or epileptic women who have taken the drug valproic acid to control seizures may have an increased risk of having a baby with spina bifida.

 All pregnancies are at risk for an NTD.

However, women with a history of a previous pregnancy resulting in a fetus with an NTD are at higher risk.

 So are women with a close relative (brother,

sister, niece, or nephew) who has an NTD, women with type 1 diabetes mellitus, women with seizure disorders being treated with valproic acid or carbamazepine, and women or their partners who themselves have an NTD.

 There are two types of NTDs. The most

common type are called the open NTDs. Open NTDs occur when the brain and/or spinal cord are exposed at birth through a defect in the skull or vertebrae. Examples of open NTDs are spina bifida (myelomeningocele), anencephaly, and encephalocele.

 Rarer types of NTDs are called closed NTDs.

Closed NTDs occur when the spinal defect is

covered by skin. Common examples of closed NTDs are lipomyelomeningocele, lipomeningocele, and tethered cord.

Neural Tube Defects

What are the common Neural Tube Defects (NTDs)

Spina Bifida - 60% Anencephaly - 30%

Encephalocele - 10%

What is Spina Bifida?

(Latin: "split spine")
Occurs when the two sides of the spine fail to close

and protect the spinal cord

There are two forms of spina bifida: 1) Spina bifida occulta (mildest form)

2) Spina bifida manifesta (or cystica) which includes two types of spina bifida: meningocele and myelomeningocele
3) Lipomeningocele (rare)

Spina bifida occulta

In this group of neural tube defects, the

meninges do not herniate through the bony defect. This lesion is covered by skin (ie, closed), therefore rendering the underlying neurologic involvement occult or hidden.

 These patients do not have associated

hydrocephalus or Chiari II malformations. Often, a skin lesion such as a hairy patch, dermal sinus tract, dimple, hemangioma, or lipoma points to the underlying spina bifida and neurologic abnormality present in the thoracic, lumbar, or sacral region.

 Presence of these cutaneous stigmata

above the gluteal fold signifies the presence of an occult spinal lesion.

SPINA BIFIDA
Sacral dimple: dermal

sinus track with spina bifida

 Dimples below the gluteal fold signify a benign, non neurologic finding such as a pilonidal sinus. This is an important point for differentiating the lesions that have neurologic involvement from those that do not

Neurologic pathology
Spina bifida occulta (occulta = closed) A condition involving nonfusion of the halves of the vertebral arches without disturbance of the underlying neural tissue

Signs and symptoms of occult spinal disorders in children include the following
: Radiologic signs

Lamina defects Hemi vertebrae Scoliosis Widening of interpedicular distance Butterfly vertebrae

Cutaneous stigmata

Capillary hemangioma Caudal appendage Dermal sinus Hypertrichosis

ORTHOPAEDIC PROBLEMS
Extremity asymmetry Foot deformities
Neurological problems Weakness of leg or legs Leg atrophy or asymmetry Loss of sensation, painless sores Hyperreflexia Unusual back pain Abnormal gait Radiculopathy Urologic problems Neurogenic bladder Incontinence Vesicouretral reflux

Spina bifida manifesta

The 2 major types of defects seen here are

myelomeningoceles and meningoceles. Cervical and thoracic regions are the least common sites, and lumbar and lumbosacral regions are the most common sites for these lesions.

 Myelomeningocele is a condition in which the spinal

cord and nerve roots herniate into a sac comprising the meninges. This sac protrudes through the bone and musculocutaneous defect.

 The spinal cord often ends in this sac, in which it is

splayed open, exposing the central canal. The splayedopen neural structure is called the neural placode. Certain neurologic anomalies, such as hydrocephalus and Chiari II malformation, accompany myelomeningocele.

 In addition, myelomeningoceles have a higher

incidence of associated intestinal, cardiac, and esophageal malformations, as well as renal and urogenital anomalies.

 Most neonates with myelomeningocele have

orthopedic anomalies of their lower extremities and urogenital anomalies due to involvement of the sacral nerve roots.

 A meningocele is simply herniation of the meninges

through the bony defect (spina bifida). The spinal cord and nerve roots do not herniate into this dorsal dural sac. These lesions are important to differentiate from myelomeningocele because their treatment and prognosis are so different from myelomeningocele.

 Neonates with a meningocele usually have normal

findings upon physical examination and a covered (closed) dural sac. Neonates with meningocele do not have associated neurologic malformations such as hydrocephalus or Chiari II.

Neurologic pathology

Meningocele (cele = sac) Fluid-filled sac with meninges involved but neural tissue unaffected

Myelomeningocele or spina bifida: meninges and spinal tissue protruding through a dorsal defect in the vertebrae

Neurologic pathology
Lipomeningocele (lipo = fat) lipoma or fatty tumor located over the lumbosacral spine. Associated with bowel & bladder dysfunction Lipomeningocele

Lipomyelomeningocele
this is a skin-covered neural tube defect. The neonate often presents with a skin-covered mass above the buttocks

Source: http://www.surgical-tutor.org.uk/default-home.htm?system/hnep/neural_tube.htm~right

Prognosis
Spina bifida is a: static non-progressive defect with worsening from secondary problems.
The prognosis for a normal life span is generally good for a child with good health habits and a supportive family/caregiver.

 Treatment Surgical
Prenatal screening

Ultrasound Amniocentesis

complex and life long

Spine Xrays and/or spinal ultrasound

Impairments associated with Spina Bifida

Physiological changes below the level of the lesion generally include:

abnormal nerve conduction, resulting in: somatosensory losses motor paralysis, including loss of bowel and bladder control

Impairments associated with Spina Bifida

Anatomical changes below the level of lesion:
musculoskeletal deformities (scoliosis)
joint and extremity deformities (joint contractures, club foot, hip

subluxations, diminished growth of non-weight bearing limbs) osteoporosis abnormal or damaged nerve tissue

Impairments associated with Spina Bifida

Anatomical changes associated with a cervical lesion: An enlarged head caused by hydrocephalus

Hydrocephalus

 Cerebellar tonsillar herniation Small posterior fossa

Chiari II (Arnold Chiari)

foramen magnum Kinking of medulla (Zformation) Beaking of the quadrigeminal plate Hydrocephalus Myelomeningocele

Extension of medulla below

Chiari II (Arnold-Chiari)
Cerebellar tonsillar herniation Small posterior fossa

Extension of medulla below

foramen magnum Kinking of medulla (Zformation) Beaking of the quadrigeminal plate Hydrocephalus Myelomeningocele

Arnold-Chiari (Chiari II)

Arnold-Chiari (Chiari II)

Arnold-Chiari (Chiari II)

Arnold-Chiari malformation
Signs/symptoms secondary to brainstem and lower

cranial nerve dysfunction. Findings (best seen on MRI):

Caudal displacement of posterior fossa structures,

including cervicomedullary junction, pons, medulla, 4th ventricle, and cerebellar tonsils. Classically, the cervicomedullary junction is described as having a kink-like deformity.

Operative Results
The most commonly-performed surgery is

suboccipital craniectomy (essentially opens up the foramen magnum), with or without C1 laminectomy and dural graft patch.

Anencephaly
Anencephaly is the most severe form of neural tube

defect. Rachischisis and craniorachischisis, often used as synonyms, refer to a severe deformity in which an extensive defect in the craniovertebral bone causes the brain to be exposed to amniotic fluid. Neonates with anencephaly rarely survive more than a few hours or days.

 Historically, these children have been the subject of

myths, folklore, and superstitions, and have been referred to as monsters based on their unusual and frightening appearance.

 The fetus has a partially destroyed brain, deformed

forehead, and large ears and eyes with often relatively normal lower facial structures. Both genetic and environmental insults appear to be responsible for this outcome. The defect normally occurs after neural fold development at day 16 of gestation but before closure of the anterior neuropore at 24-26 days' gestation.

 Anencephaly is the most common major CNS

malformation in the Western world, and no neonates survive. It is seen 37 times more frequently in females than in males. The recurrence rate in families can be as high as 35%. The incidence is highest in Ireland, Scotland, Wales, Egypt, and New Zealand and lowest in Japan.

 Symptoms
Mom- Polyhydramnios Baby- absence of brain/skull

Diagnosis
Ultrasound

Treatment
None, incompatible with life

Management
Comfort Measures Support Parents

Encephalocele

Defined: Brain and membranes outside skull Causes: Genetic, unknown Treatment: Surgical Management: complex

Encephalocele
Encephaloceles are occur when the anterior neuropore fails to close during days 26-28 of gestation. Incidence of this anomaly is 10% of the incidence of spina bifida cystica. In the United States, approximately 80% of lesions are found on the dorsal surface of the skull, with most near the occipital bone. In contradiction, most encephaloceles in Asia are ventral and involve the frontal bone.

 In the Philippines and other Pacific Rim countries,

incidence of anterior encephaloceles that present as hypertelorism, obstructed nares, anterior skull masses, and cleft palate, among other presentations, is high. In most lesions, the sac that has herniated through a midline skull defect is covered with epithelium.

Diagnosis and Detection

Amniocentesis
AFP - indication of abnormal leakage

Blood test

Maternal blood samples of AFP Ultrasonography

Amniocentesis
Needle inserted through abdominal wall
Sample of fluid from amniotic sac removed Fetal cells can be tested to determine

abnormalities Ultrasound is used to guide needle placement Employed when mother is at high risk for giving birth to child with abnormalities

Chorionic Villus Sampling (CVS)

Can detect abnormalities earlier than amniocentesis Between 10-12 weeks of gestation Instead of amniotic fluid, a sample of the villi of the

chorion are collected and tested

Carries a greater risk than amniocentesis

Chorionic Villus Sampling (CVS)

A plastic catheter is inserted through the cervix and guided by ultrasound

Method 1: Chorionic Villus Sampling

Chorionic Villus Sampling (CVS)

A biopsy needle is inserted through the abdominal wall and guided by ultrasound

Method 2: Chorionic Villus Sampling

Alpha-fetoprotein (AFP) Test

Blood test performed at 15-20 weeks into

pregnancy Measures the amount of AFP to detect neuraltube defects (high levels). Because of the number of false positives, it is used mainly as a screening test

 Prevention
folic acid 0.4 mg daily pre, 1 mg daily preg

Identify
Prenatal At birth

Protect pre-op and post-op

Skin integrity to prevent infection Special handling to reduce nerve damage

Support
Parental coping Pictures of similar defects corrected

Genetic Counseling
For future pregnancy In early pregnancy, therapeutic abortion

Complications
Permanent disability

Education
Symptoms of hydrocephalus Symptoms of meningitis Follow up for monitoring to assess neurologic damage

How Can NTDs be Prevented?

All women of childbearing age should receive 0.4 mg (400 micrograms) of folic acid daily prior to conception of planned or unplanned pregnancies and continue thru 1st trimester
Women with a history of NTD and should receive daily supplementation of 0.4 mg (4000 micrograms)

of folic acid starting three months prior to conception and continuing thru the 1st trimester

Questions?
Which of the following is the most common

congenital anomaly? A congenital Neural tube defects B congenital Limb defects C congenital heart diseases D congenital Renal agenesis

C congenital heart diseases

 Which of the following is the most common

congenital CNS Malformation ? A Spina bifida BAnencephaly CHydrancephaly

A spina bifida

A MRI