Hydrocephalus is an abnormal buildup of cerebrospinal fluid in the brain that increases pressure inside the skull. It can be congenital, developing before birth due to infections, genetic factors, or issues with brain development. It can also be acquired after birth due to head injuries, tumors, or infections. The excess fluid causes brain tissue to be compressed and brain damage over time. Spina bifida is a birth defect where the spinal column does not fully close, leaving part of the spinal cord and meninges exposed. Both conditions require lifelong management, often involving surgical insertion of shunts to drain fluid and prevent further brain damage.

1. HYDROCEPHALUS
AND
SPINA BIFIDA
MR.SACHIN T.GADADE
M.SC(N) PEDIATRICS

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. Hydrocephalus
• 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. • 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.

13. • 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.

14. • 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.

15. 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

16. • 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.

17. • 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.

18. 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 -

19. Pathophylosiology
• 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

20. Risk factors
• Being born prematurely
• Problems during pregnancy
• Problems with fetal development
• Lesions & Tumors
• Infections of the nervous system
• Bleeding in the brain

21. • 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.

22. 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

23. 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

24. In Acquired Hydrocephalus
• Confusion & Disorientation
• Drowsiness
• Headache
• Irritability which may progressive
• Lack of appetite
• Lethargy

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

26. 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

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

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

29. 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

30. Differential diagnosis
Megalocephaly
Hydrocephaly
Subdural hematoma

31. 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

32. SURGICAL MANAGEMENT e
• 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.

34. 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.

35. Ventriculostomy
The 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.

37. Preventions
Regular cheek up
Preventions & corrections of infectious diseases.
Meningitis vaccine
Preventing head injury

38. Spina bifida ( Neural Tube defects)

39. Spina bifida ( Neural Tube defects)
• Introductions – Neural tube defects are the congenital
malformations of the CNS resulting from a defective
closure of the neural tube. During early
embryogenesis between 3rd
& 4th
• Week of intrauterine life. It involves the defects in the
skull, vertebral column the spinal cord & other
portion of CNS.

42. Some vertebra overlying the spinal cord are not fully formed
& remain unfused & open if the opening is enough this
allows a portion of spinal cord to protrude through the
opening in the bones their may or not be a fluid filled sac
surrounding the spinal cord.
Other neural tube defects includes anencephaly a condition
in which the portion of the neural tube which will become
the cerebrum does not flows & encephalocle which result
when other parts of the brain remain unfused.

43. Definition of spina bifida –( latin – split spine )
It is a developmental birth defect caused by the
incomplete closure of the embryonic neural tube
Incidence- 1-5/1000 live births
Risk is 2nd
sibling is more
The defect is usually obvious at birth & varies in
severity.

44. Etiology
Idiopathic
Maternal radiation exposure
Drugs like valprolic acid
Exposure to chemicals
Malnutrition especially folic acid deficiency
Genetic dominant

45. Classification
Spina bifida occulta
Spina bifida cystica ( myelomeningocele )
Meningocele
Lipomeningocele
The most common sites of the malformation in the
lumbar & sacral region

46. Spina bifida occulta
Occulta is Latin for hidden
This is one of the mildest form of spina bifida . It is
most frequent & most benign neural tube defects.
there is defective closure of the posterior arch &
lamina of the vertebrae,
Usually L5-S1.there is no protrusion of the meninges.

47. Clinical manifestations
• Asymptomatic
• Dysplasia of the spinal cord
• Cutaneous lesions over the defect as tuft of hears lipoma
hemangioma, dermal sinus or as dimple in the skin
• Intra spinal lesions like desmoids cyst
• Intramedullary Lipoma which produces neurological
deficit.
• Progressive deformity of the foot.
• Changing in maturation pattern.
• Alterations in the gait.

48. Diagnostic evaluations
History of the child
Myelo CT Scan & MRI help to confirm the diagnosis
Blood investigations
SURGICAL MANAGEMENT
Laminectomy is done & intra spinal lesions is excised.

49. Meningocele
• Definition – it is a hernial protrusion of the meninges
through a midline defect in the posterior vertebral arch.
• A fluctuating cystic swelling filled with CSF & covered by a
thin transparent membrane or with a skin . It
transillumiantes easily. It is generally found in the lower
back i.e. lumbosacral region. It may also be found in the
thoracic region & in the skull(cranial meningocele). The
spinal cord & nerve roots are usually normal.

50. Clinical manifestations
Asymptomatic
Neurological deficits are present below the level of
the lesion.
Lower limb paralysis
Musculoskeletal deformities
Incompetence of anal & bladder sphincters.
CSF leakage may present & there is chances of
infections.

51. Diagnostic evaluation
History of the child
Physical examination
X-ray spine & skull and CT Scan
Blood investigations
Neurological examinations

52. Medical Management
• Head circumference of the child should be measured dally
• Anterior fontanels to be checked for bulging or widening
• The meningocele sac should be protected from infections &
injury
• The infant should be positioned on the abdomen to avoid
pressure on the sac.
• Sac is protected with sterile moist dressing

53. Surgical management
Surgical closure of the sac should be done as early as
possible to prevent infections.
PROGNOSIS
Prognosis is generally good unless hydrocephalus &
neurological deficits are developed.

54. Meningomyelocele ( spina bifida
cystica )
• In spina bifida cystic , a cyst protrudes through the defect I
the vertebral arch. these conditions can be diagnosed in
uteri on the basis of elevated levels of alpha-fetoprotein
after amniocentesis & by USG. Spina bifida cystic may result
in hydrocephalus & neurological deficits.
• Meningomyelocele in the lumbar area.

55. • External sac with cerebrospinal fluid
• Spinal cord wedged between the vertebral.
• This is a serious & common form , the unfused
portion of the spinal column allows the spinal cord to
protrude through an opening.

56. Clinical manifestations
• Some degree of paralysis
• Loss of sensation below the level of the spinal cord
defect.
• Signs of hydrocephalus can be seen
• Postural abnormalities like clump foot, dislocation of
hips .
• Spasticity & hyperactive reflexes may present

57. • Ambulatory problems, loss of sensations, deformities of hip ,
feet & loss of muscle tone.
• Pain in the lower back radiating towards the leg& back of
the knee.
• The back portion of the brain is displaced from the back of
skull down in to the upper neck.
• Risk of infections of CNS & rupture of the sac .

58. Diagnostic evaluation
• Through history of the child.
• Clinical manifestations.
• Physical & Neurological assessment
• X- ray, CT Scan & MRI is helpful to make a diagnosis.
• Routine blood & urine examination.
• Prenatal diagnosis by amniocentesis & estimation of
Alpha feto-protein.

59. Medical & surgical Management
• Management of this condition can be done by surgical
correction of the defect & essential care of the infant.
• correction of musculoskeletal deformities & regulation of
bladder & bowels functions should be performed.
• Prevention of injury & infection of the sac by positions with
protective covering with sterile dressing & prevention of
skin break down.
• Monitoring of signs of hydrocephalus e.g. head
circumference, fontanalles & feeding behavior.

60. • Provision of adequate nutrition.
• Promotion of urinary elimination & bowel regularity.
• Prevention of leg or hip deformities & often
complications.
• Peri- operative care, promotion of growth
development.
• Emotional & psychological support
• Health teaching regarding care of the child is also
important aspect of management.

61. THANK YOU