Transcript Slide 1 - muhadharaty.com

‫‪Congenital CNS‬‬
‫‪abnormality‬‬
‫إعــــداد‬
‫م‪.‬د‪.‬علي طارق عبد الواحد‬
‫إختصاص جراحة الجملة العصبية‬
‫كلية الطب ‪ /‬جامعة بغداد‬
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 defect
2. Hydrocephalus
3. Craniocynostosis
4. Tethered cord syndrome
5. Encephalocele
6. Arnold-chiari malformation
7. Dandy-walker malformation
Definition:
Neural tube defect
. 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.
Hydrocephalus
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
Treatment
Medical : 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, VPleural shunt , four complications of shunt
(infection, hemorrhage, malfunction, chronic
subdural hematoma) , the advance treatment is
ETV( endoscopic third ventriculostomy ) .
Hydrocephalus
CRANIOSYNOSTOSIS
Definition:
Premature closure of cranial sutures .
Types :
1. Scaphocephaly (most common) due to
closure of sagittal suture.
.
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.
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