Congenital CNS abnormalityإعــــداد

. عبد. طارق علي د مالواحد

إختصاصجراحة العصبية الجملة

جامعة / الطب كليةبغداد2015

Introduction

. The development of the (CNS) is as complicated as that of any other system in human body.

. Not all CNS malformations can be explained on the basis of known embryologic processes.

. Human embryo has 23 stages of development, the stage 8 to 23 related to

CNS development

Types of congenital CNS abnormality

There are many types of CNS abnormality :

1 .Neural tube defect2 .Hydrocephalus

3 .Craniocynostosis4 .Tethered cord syndrome

5 .Encephalocele6 .Arnold-chiari malformation

7 .Dandy-walker malformation

Neural tube defect Definition:

. The most common problem to affect the spinal column is failure of neural tube to close fully ,producing one of many patterns of neurospinal dysraphism or spina bifida.

. Incidence of spina bifida 1 to 2 per 1000 live births. Mostly affected dorsolumbar spine .

.Hydrocephalus occurs in 80% of cases of spina bifida .

Neural tube defect

.Types of spina bifida:1 .Spina bifida occulta : the posterior

vertebral arch has a defect with in it but there is no herniation of the neural tube , spina bifida occulta is found in 10% of the population.

There may be a hairy patch ,fatty lump ,skin pigmentations ,dermal sinus .

Neural tube defect

Neural tube defect

Neural tube defect 2 .Spina bifida cystica: there is skin

cover the defect (meningocele) may be contain CSF only and patient neurologically intact.

Neural tube defect 3 .Spina bifida aperta: the neural tube is open

with out skin cover ,mostly associated with neurological manifestations & CSF leakage

with meningitis (myelomeningocele) .

Neural tube defect

Managements:.Prenatal screening to detect alpha- fetoprotein

in maternal blood or in amniotic fluid and used of the ultrasound to detected up to

80%of neural tube defect ..Complete neurological examination .

.OFC measure to check of hydrocephalus..MRI of brain and whole spine.

.Plain X-ray of spine. .Neural tube defect associated with five other

systemic congenital abnormality (CNS,GIT,GUT,CPS,and skeleton system)

Neural tube defect

.Treatment of majority of cases of dysraphism needs surgical repair to prevent of meningitis and cosmetic factor.. Advance neurosurgical procedures repair of neural tube defect intrauterine by endoscopic surgery..Urgent surgical repair of rupture spina bifida (CSF leak) to prevent of meningitis.

Neural tube defect

Hydrocephalus Definition : a disproportionate increase in

the amount of CSF within cranium, usually in association with a rise in ICP.

Basic and physiological sciences of CSF:.Volume is around 140 ml and normal ICP (5-15 mmHg).

.Daily production of CSF( 480 ml ).

.CSF volume is replaced three time daily.

.Produced by choroid plexus, absorbed by arachnoids villi.

.CSF protect and support the brain and spinal cord.

Hydrocephalus

Three main causes of hydrocephalus :

1.Obstructions : most common cause of

hydrocephalus (congenital aqueduct stenosis ),

tumors ,hemorrhage, infection.

2. Overproduction : uncommon cause , mostly due to

choroid plexus papilloma.

3.Impaired resorption : rare cause , due to

congenital absence of arachnoids villi.

Hydrocephalus

Types of the hydrocephalus:

1. Non communicating

hydrocephalus(obstructive):Occlusion of normal pathways of CSF flow with in

ventricular system by tumors or aqueduct stenosis.2. Communicating hydrocephalus(non obstructive):Occlusion of pathway of CSF flow with in basal cistern,

subarachnoid space , arachnoids villi. All ventricles was dilated .

Hydrocephalus

Clinical features of hydrocephalus:

In neonate period: increasing OFC, tense fontanelles ,feeding problem and sunsetting eyes, and dilated scalp veins.

In children and adults: symptoms of increase ICP ( headache, vomiting ,nausea and decreasing of consciousness ) .

In the older age: dementia, ataxia, visual disturbance, urinary incontinence .

Hydrocephalus

Investigations 1.Skull x-ray shows : separation of sutures, erosion

of pituitary fossa, copper beating appearance of

skull , fontanel tapping.

2.Ultrasonography : when the anterior fontanelles

is patent, to visualize ventricular system .

3.Native brain CT-scan .

4.Brain MRI to avoid ionizing radiation in CT-scan

Hydrocephalus

TreatmentMedical : reduced CSF production by

acetazolamide (Diamox Tab) , fruzemide (Lasix) may be used in short term. In long term the medications un affected therefore surgical intervention is required.

Surgical : drainage of CSF in the another absorptive viscous, V-P shunt ,V-A shunt, V-Pleural shunt , four complications of shunt (infection, hemorrhage, malfunction, chronic subdural hematoma) , the advance treatment is ETV( endoscopic third ventriculostomy ) .

Hydrocephalus

Hydrocephalus

Definition:Premature closure of cranial sutures .Types :1.Scaphocephaly (most common) due to

closure of sagittal suture.

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CRANIOSYNOSTOSIS

Types:2. Trigocephaly is caused by premature

closure of metopic suture.

CRANIOSYNOSTOSIS

Types:3. Plagiocephaly is caused by premature

closure of unilateral coronal or lambdoid sutures.

CRANIOSYNOSTOSIS

Types:4. Brachycephaly is caused by premature

closure of bilateral coronal sutures.

CRANIOSYNOSTOSIS

Treatment: Surgical intervention most do with in first year .

Aims of surgery 1.Cosmetic .2.Improve airway.3.Increase the volume of the skull.

CRANIOSYNOSTOSIS

Thanks