congenital disorders of CNS

1. HYDROCEPHALUS
PRESENTED BY
JANNET REENA PURANI

5. TERMINOLOGY
• 1) Atrophy – Wasting of any part of the body, due to
degeneration of the cells.
• 2) Hydrocephalus – Enlargement of the skull due to an
abnormal collection of CSF around the brain
• 3) Gliomas – a malignant tumor composed of neuralgial cells
affecting the brain & spinal cord.
• 4) Spina Bifida – a congenital defect of non- union of one or
more. Vertebral arches, allowing protrusion of the meninges
& possibly their contents.

6. • 5) Myeolomeningocele – a protrusion of the spinal cord &
meninges through a defect in the vertebral column.
• 6) Meningocele - a protrusion of the meanings through the
skull or spinal column.
• 7) Dysplasia – abnormal development of tissue

7. CIRCULATION OF CSF
• How does csf (cerebro spinal fluid) circulate through the
brain?
• The brain is like gelatin & floats in csf C.S.F. f lows through
the brain chamber. These chambers are know as ventricles,
& they lie deep inside the brain. The fluid filled ventricles
protect the brain, like a cushion .most of the csf is made in
the choroid plexus, a part of the brain. Surplus csf is
removed from the brain through the Dural venous sinuses a
series of channels

8. • The Dural venous sinuses run down the arachnoids
Ville, a layer of tissue which is like a one way valve.
• the arachnoids Ville allow excess csf to leave the brain
& filter in to the blood stream while at the same times
preventing blood from getting into the brain &
causing damage.

9. • It is important that the production, flow & absorption
of csf occur in such a way that normal pressure is
maintained inside the skull – it is a delicate balance
.csf has three vital functions
• 1) It protects the nervous system ( brain & spinal cord)
from damage
• 2) It removes waste from the brain
• 3) It nourishes the brain with essential hormones.

10. • The Dural venous sinuses run down the arachnoids
Ville, a layer of tissue which is like a one way valve.
• the arachnoids Ville allow excess csf to leave the brain
& filter in to the blood stream while at the same times
preventing blood from getting into the brain &
causing damage.

11. DEFINITION
•A condition marked by an excessive accumulation of
cerebrospinal fluid resulting in dilatation of the cerebral
ventricle & raised intra cranial pressure may all result in
enlargement of the cranium & atrophy of the brain.
There are three types of hydrocephalus

12. TYPES
• CONEGITAL:Congenital hydrocephalus is present in the
infant prior to birth, meaning the fetus developed
hydrocephalus in utero during fetal development.
• AQUIRED:This condition is acquired as a consequence
of CNS infections, meningitis, brain tumors, head
trauma, toxoplasmosis, or intracranial
hemorrhage (subarachnoid or intraparenchymal), and is
usually painful.

13. TYPES
• NORMALPRESSURE:(NPH) is a particular form of chronic
communicating hydrocephalus, characterized by enlarged
cerebral ventricles, with only intermittently elevated
cerebrospinal fluid pressure.
• COMMUNICATING:Communicating hydrocephalus, also
known as nonobstructive hydrocephalus, is caused by
impaired CSF reabsorption in the absence of any obstruction
of CSF flow between the ventricles and subarachnoid space.
• NONCOMMUNICATING:Noncommunicating hydrocephalus,
or obstructive hydrocephalus, is caused by a CSF-flow
obstruction.

14. TYPES
• Hydrocephalus ex vacuo also refers to an enlargement of
cerebral ventricles and subarachnoid spaces, and is usually
due to brain atrophy (as it occurs in dementias), post-
traumatic brain injuries, and even in some psychiatric
disorders, such as schizophrenia.[19] As opposed to
hydrocephalus, this is a compensatory enlargement of the
CSF-spaces in response to brain parenchyma loss; it is not
the result of increased CSF pressure.

15. • 1) Congenital hydrocephalus – This is present at birth.
According to the national health service (UK),
approximately 1 in very 1000bables are boon with
congenital hydrocephalus. It may be caused by an
function in the mother during pregnancy such as
Rubella or mumps, or a birth defect such as spina
bifida.
• It is one of the most common development
disabilities, more common than Down syndrome or
deafness.

16. • 2) Acquired hydrocephalus – This develops after birth,
usually following the conditions like.
• A) Trauma – Birth injury, Head injury or intracranial
hemorrhage
• B) Inflammation- meningitis, encephalitis
• C) Neoplasm – space occupying lesions like Tuberculoma,
subdural hematoma or abscess , glioma, choroid plexuses
papiloedma , psudo tumor cerebri etc.
• D) Chemical – hypervitaminosis A
• E) Connective tissue disorder etc.

17. • 3) Normal pressure hydrocephalus – Only effects
people 50 year or more. It may develop after stroke or
injury. In most cases doctor do not know they it
occurred . 2 In every 100000 people are affected by
normal pressure hydrocephalus in England each year
• A prenatal ultrasound examination can sometimes
detect hydrocephalus in the developing body.

18. ETIOLOGY OF
HYDROCEPHALUS
• 1) Congenital Hydrocephalus ( present at birth)
• The baby is born with a blockage in the cerebral aqueduct
at long passage in the midbrain that connects two large
ventricles.
• This is the most common cause.
• 2) The choroid plexus produces two much csf
• 3) Infections during pregnancy like

19. • A) CMV (cytomegalovirus – The virus is most
commonly transmitted to a child before birth.
• B) Rubella – The virus passes from person to person
via droplets in the air expelled when infected people
cough or sneeze the virus also present in the urine,
feces & on the symptoms of rubella are an elevated
body temperature & a pink rash.

20. • C) Mumps – And acute viral infection in which the
salivary gland particularly parotid gland swell.
• D) Syphilis – STD cause by bacterial treponema
palladium
• E) Toxoplasmosis – An infection cause by a single
celled parasite Toxoplasma Gondi.

21. ACQUIRED
HYDROCEPHALUS
• 1) Brain hemorrhage – Bleeding inside the brain.
• 2) Brain lesions – Due to brain injury, infection,
exposure to certain chemical or problems to the
immune system.
• 3) Brain Tumour – Benign or malignant grouth in the
brain
• 4) Meningitis
• 5) Stroke -

22. PATHOPHYLOSI
OLOGY
• Due to Etiological factors
• Abnormal buildup of csf in the ventricles of the brain.
• It is caused by an obstruction which prevents proper
fluid deranges.
• Increased intracranial pressure inside the skull
• Which compress surrounding brain tissue
• Which leads to progressive enlargement of the head,
convulsions & brain damage.
• Hydrocephalus

23. RISK FACTORS
• Being born prematurely
• Problems during pregnancy
• Problems with fetal development
• Lesions & Tumors
• Infections of the nervous system
• Bleeding in the brain

24. • Having severe head injury
• Hydrocephalus may occurs if
• - To much csf is produced in the choroid plexus
• - One of the ventricles is block narrowed, which stops
or restricts the flow of csf
• - Csf can not filter in to the blood stream.

25. CLINICAL
MANIFESTATIONS
in congenital hydrocephalus
• Breathing difficulties
Muscles in the baby arms & legs may be stiff & prone
contractions
Some of the development stages may be delayed such as sitting
or cradling
Tense Frontanalle – the soft part of the top of the baby's head
bulges outward
Irritable & drowsy
The baby may be unwilling to bend or move his neck or head
the baby may feed poorly

26. The baby's head looks larger than it should be
The baby's scalp is thin & shiny with visible veins on
the scalp
The pupils of the baby's eyes may be right close to
the bottom of the eyelid sometimes known as the
setting sun
High pitched cry
Seizures
Vomiting

27. IN ACQUIRED
HYDROCEPHALUS
• Confusion & Disorientation
• Drowsiness
• Headache
• Irritability which may progressive
• Lack of appetite
• Lethargy

28. • Nausea
• Personality changes
• Problems with eye sight such as blurred or double
vision
• Seizures
• Bowel & urinary incontinence
• Vomiting
• Walking difficulties

29. DIAGNOSTIC EVALUATION IN
ACQUIRED HYDROCEPHALUS
• Medical past & present history about the disease &
History of pregnancy
• Physical Examination & Neurological Assessment
• USG & Xray of skull
• CT Scan & MRI
• Blood investigations
• Persistent widening of squamo –parital suteres
• Typical cracked pots sound

30. COMPLICATIONS
• Seizures
• Herniation of brain
• Persistent increased ICP
• Developmental Delay
• Motor & intellectual handicaps
• Infections

31. • Neurological deficits
• Visual problems
• Aggressive behaviors
• Shunt complications
• Shunt dependency
• V-A Shunt complicated in Endocarditis &
Thromboembolism

32. DIAGNOSTIC
EVALUATION
In congenital hydrocephalus
A Routine Prenatal USG
Physical Examination & Neurological Assessment
Increase in head circumference more than 1cm every 15
days
USG & Xray of skull
CT Scan & MRI
Blood investigations

33. DIFFERENTIAL
DIAGNOSIS
Megalocephaly
Hydrocephaly
Subdural hematoma

34. MEDICAL
MANAGEMENT
• To reduce in increased ICP by carbonic anhydrate
inhibitor
• Acetazolamide ( Diamox ) 50 mgkgday to reduce
CSF production
• Oral glycerol & isosorbide to reduce CSF production
• Definite management in progressive hydrocephalus is
mostly surgical
• Urgent treatment to alleviate ICP

35. SURGICAL MANAGEMENT
• A SHUNT – The surgical procedure insertion of a
drainage system
• This is a catheter ( thin tube with a valve) that is
placed in the brain to drain away excess fluid into
another part of the body such as abdomen or a
chamber in the heart one end is placed in one of the
brains ventricles. It is tunneled under skin to another
part of the body which is better able to absorb the
fluid.

37. Usually this is all that is needed & no further
treatment is required. Some times shunt repair
surgery may be needed if it gets blocked or infected .
Patients with hydrocephalus usually need to have
shunt system in place for the rest of their lives . If the
shunt is placed in a child additional surgeries may be
needed to insert longer tubing as she or he grows.

38. VENTRICULOSTOMY
 T h e surgeon makes a hole in the bottom of a
ventricles so that the excess fluid flows towards the
base of the brain. Normal absorption occurs at the
base of the brain. This producers is sometimes
performed when the flow of fluid between ventricles
is obstructed.

40. PREVENTION
Regular cheekup
Preventions & corrections of infectiousdiseases.
Meningitis vaccine
Preventing head injury

41. THANK YOU