hydrocephalus

1. HYDROCEPHALUS
Dr.N.Vinay kumar
Under the guidance of Dr.JM
Pawar sir
1

2. INTRODUCTION
 Hydrocephalus is a common clinical
problem seen in pediatric neurosurgical
practice.
 Hydrocephalus involves dilatation of the
cerebral ventricular system with
corresponding, compressive effects on the
parenchyma.
 It can be communicative or obstructive
types. Congenital, acquired, infective, and
secondary hydrocephalus have different 2

3. HISTORY
 Hydrocephalus has been recognized for centuries.
Accumulation of fluid in various intracranial
compartments was recognized by Hippocrates (BC
460–377) and Claudies Galen (130–200 AD).
 The studies of Thomas Willis (1621–1675) facilitated
the understanding of ventricular system and CSF
pathways.
 Franciscus Sylvius (1614–1672), Alexander Monroe
(1733–1817) and Francois Magendie (1783–1855)
have made important anatomical contributions for the
CSF pathway
3

4.  Finally, Key and Retzeus (1876) established the
modern concept of CSF circulation. At that stage, the
diagnosis and management was not clear, resulting
in high mortality.
 Dandy and Blackfan (1913) further contributed by
creating experimental models of hydrocephalus
which led to the classification and differentiation
between the non-communicating (obstructive) and
communicating forms with distinct possible treatment
strategies.
 The history of the treatment of hydrocephalus has
been described in detail by John Scarff in 1963.
Walter Dandy proposed 3 ventriculostomy in 1922 for 4

5.  CSF isaclearfluid producedby dialysisof blood in
the choroid plexus. (70-80%)
 A small proportion of CSF may be produced from
ventricular ependyma and brain parenchyma
 Once produced, CSF is then circulated, due to
hydrostatic pressure.
 CSF is produced at a rate of 0.33 ml/min, which is
approximately 500 ml/day. The total volume of
CSF varies with age and in the adults is 100–150
ml of which 15–25 ml is contained within the
ventricles.
5
WARM UP

6. 6

7.  The incidence of congenital
hydrocephalus is about 0.2–0.5/1000
live births.
 A higher incidence has been reported
in elderly primiparous mothers. It can
be associated with a variety of
physiological and pathological
conditions.
7

8.  Sex
Males = females.
EXCEPT in Bickers-Adams syndrome ,an X-linked
hydrocephalus transmitted by females and manifested in
males.
NPH has a slight male preponderance.
 Age
bimodal age curve. One peak occurs in infancy and is related
to the various forms of congenital malformations. Another
peak occurs in adulthood, mostly resulting from NPH. (40%)
8

9. DEFINITION
 Hydrocephalus is
defined as abnormal
enlargerment of head as
result from impaired
circulation and/or
absorption of CSF or in
rare increased
production by chroid
plexus papilloma.
 Also known as
Hydrodynamic
disorder of CSF 9

10.  The obstruction may be within the
ventricular system resulting in Non-
communicating hydrocephalus or the
impairment of circulation is throught
the subarachnoid space or defective
absorption in venous system resulting
in communicating hydrocephalus.
10

11.  The CSF is formed primarily in the ventricular
system by the choroid plexus, which is situated in
the lateral, third, and fourth ventricles.
 Although most CSF is produced in the lateral
ventricles, approximately 25% originates from
extrachoroidal sources, including the capillary
endothelium within the brain parenchyma.
 There is active neurogenic control of CSF
formation because adrenergic and cholinergic
nerves innervate the choroid plexus.
 Stimulation of the adrenergic system diminishes
CSF production, whereas excitation of the
cholinergic nerves may double the normal CSF
production rate.
11

12.  In a normal child, approximately 20 mL/hr of CSF
is produced. The total volume of CSF
approximates 50 mL in an infant and 150 mL in
an adult.
 Most of the CSF is extraventricular. The choroid
plexus forms CSF in several stages; through a
series of intricate steps, a plasma ultrafiltrate is
ultimately processed into a secretion, the CSF.
 CSF flow results from the pressure gradient that
exists between the ventricular system and
venous channels. Intraventricular pressure may
be as high as 180 mm H2O in the normal state.
12

13.  Hydrocephalus resulting from obstruction within
the ventricular system is called obstructive or
non-communicating hydrocephalus.
 Hydrocephalus resulting from obliteration of the
subarachnoid cisterns or malfunction of the
arachnoid villi is called nonobstructive or
communicating hydrocephalus.
13

14. COMMUNICATING NON- COMMUNICATING
Achondroplasia Aqueductal stenosis
Choroid plexus of papilloma Infectious
Benign enlargement of
subarachnoid space
X linked
Meningitis Chiari malformation
Meningeal malignancy Dandy walker malformation
Post haemorrhagic Klippel feil syndrome
Abscess , haematoma
Tumors , Neurocutaneous
disorders
Vein of Gallen malformation
14
HYDRANCEPHALY
Holoprosencephaly
Massive hydrocephalus
POrencephaly

15. PATHOGENESIS
The hydrocephalus may be due to any of given
mechanism either alone or in combination.
15
Increased Production Chroid plexus papilloma
Obstruction to the flow -Intraventricular formen by
inflammatory exudates,
tumors or blood clots.
- Congenital stenosis of
cerebral Aqueduct
Decreased absorption Obstruction to outflow

16. ETIOLOGY
1. CONGENITAL
 Intra uterine infections
- Toxoplasmosis, Rubella , Cytomegalovirus
 Congenital Malformations
- Arnold Chiari malformations
- Agenesis of foramina Monro
- Arteriovenous malformation
- Brainstem malformation
- Cranial defects
- Dandy walker malformations
16

17. 2. ACQUIRED
 Infections
- Meningitis
- TORCH
- Cysticercosis
- Brain abscess
 Post haemorrhagic
- Head injury
- Subarachnoid haemorrhage
17

18.  Mass lesions
- Posterior fossa tumors
- Astrocytoma
- Ependymoma
 Sagittal sinus thrombosis
 Hypervitaminosis A
18

19. CLASSIFICATION
COMMUNICATING HYDROCEPHALUS
 External or non obstructive hydrocephalus
 There is no obstruction to Csf flow from chroid
plexus to arachnoid villi
 Rather Obstruction lies outside ventricular
system, usually in basal cisterns or arachnoid
villi.
 Resorption is impaired due to scarring, fibrosis
following infection, inflamation or haemorrhage.
19

20. CAUSES
 Meiningitis
 Haemorrhage
 Arnold Chiari malformation
 Congenital absence of arachnoid
granulations called Pacchioni’s
granulations
20

21. NON-COMMUNICATING
HYDROCEPHALUS
 Internal or obstructive
 There is block in the circulation within the
ventricular system
CAUSES
 Tumors (medulloblastoma,astrocytoma)
 Cysts, abscess and haematoma
 Cerebellar abscess
 Cerebellar or brain stem haematoma
21

22. SYMPTOMS
 Headache and vomiting – more in morning, better
on sitting and it becomes continuous as
hydrocephalus progresses
 Urinary incontinence presenting as urgency,
frequency which follows diminished awarness to
urinate as it progresses
 Blurred vision due to papilloedema
 Drowsiness
 Horizontal diploplia – 6th nerve palsy
 Spasticity in lower limbs – diffulity in walking
22

23. SIGNS
 Macrocephaly – increase in
occipito frontal circumference
more than 2cm/month in first
6months
 Sutural seperation
 Fontanels – delayed closure,
AF bulging and tense
 Dilated scalp veins
 Skin over scalp is thin and
shiny 23

24.  Sun set sign
 Macewen sign (crack pot sign)
 Transillumination - > 2-2.5cm in frontal and
over 2cm in occipital in abnormal
 Bradycardia
 Hypertension
 Altered respiration and sometimes apnoea
 Papilloedema 24

25.  Pyramidal signs in lower limbs
- Pyramidal fibres are stretched giving
plantar extensor reflex and
exaggerated DTR s with increased
tone
 Lateral gaze palsy
 High pitch cry
25

26. VARIATIONS IN
PRESENTATION
1. NEW BORNS
- OFC increasing rapidly more than 97th
centile
- Bulging AF with association of scalp signs
- Poor feeding
- Frequent vomiting
- Reduced activity
- Association spina bifida with or without
meningocele or meningomyelocele
26

27. 2. INFANTS
- Large head size
- Poor feeding
- Irritability
- Decreased acitivity
- Lack of interest in surroundings
- Sunset sign
- Vision problem
27

28. 3. Toddlers and children
- Headache and vomiting
- Neck pain (cerebellar tonsil herniation)
- Horizontal diplopia (6th nerve)
- Lethargy and drowsiness
- Delayed milestone
- Urinary incontinence
- Memory loss
- Progressive macrocephaly
- Gait disturbances
28

29. By Aetiology factors
 Tumors – headache more significant and
vomiting
 Site of obstruction
- Foramen of Munro -> Frontal prominence
- Fourth vetricle -> Prominent occiput
29

30. INVESTIGATION
1. Finding the etiology
- TORCH screening
- CSF analysis for protein levels and opening
pressure
2. Confirmation of diagnosis
 Xray
- Enlargement of skull
- Widening of sutures
- Silver bitten appearance
- Ballooning of sella turcica
- Calcification in toxoplasmosis and cytomegalo 30

31. Silver beaten appearance
 These are rounded zones of bony
Attentuation of the cranial bones Caused by
pressure from the cerebral cortical gyri,
resulting from premature closure of cranial
sutures in infants and by extension,
increased intracranial pressure.
31

32. 32

33. 33

34.  CT scan
- In obstructive
variant both
lateral and third
ventricles are
dilated
- In communicating
there is
generalised
enlargement of
ventricles
- Size of temporal
horns more than
2mm 34

35. - Presence of
periventricular
lucencies and
absent sulci are
indicator of raised
ICT
(warning signs)
35

36. - There can be unilateral dilation of ventricle
in Obstruction of foramen of Munroe
IMAGE
36

37. - Dilation of lateral ventricle and third
vetricle in aqueductal stenosis
(triventriculomegaly), dilation of lateral,
third and fourth due to outlet obstruction
37

38. - Ballooning of frontal and third
ventricle, Mickey mouse ventricles
38

39. 39

40.  MRI
- Shows the extent of dilation
and ventricle, site and
nature of block
- Midsagital MRI will show
reveals type of aqueductal
stenosis and and length of
obstruction.
 USG
- Through AF can
demonstrate ventricular
enlargement
40

41. MANAGEMENT
1. Medical management
- It provides temporary relief and uses drug which
act either by decreasing CSF secretion by
choroid plexus or increasing CSF resorption
 Acetazolamide – it inhibits the enzyme carbonic
anhydrase and reduces CSF production
 Loop diuretics – Furosemide increases water
excretion and inhibits resorption of sodium and
chloride
 Steroids (dexmethasone) can be recommended
for short time in post infective hydrocephalus to
dissolve exudates
41

42. 2. SURGICAL
(a) Shunt Procedures
- These are done to divert CSF
- Into peritoneum, pleural or right atrium
- Catheter placement, bypassing the obstruction and
draining CSF
42

43.  Ventriculoperitoneal shunt
- Most common type used
- From lateral ventricle to
peritoneum
Eg. Upathyaya shunt
Chopra shunt
Pudenz shunt
Sri Chitra Shunt
Splitz holter shunt
43

44.  Ventriculoatrial shunt
- Connect the cerebral
ventricle to the right
atrium of heart through
jugular vein and superior
vena cava
- Indicated in Obese
patient, peritonitis or any
abdominal disease
- Needs to be lengthened
as child grows, causes
glomerulonephritis and
endocarditis 44

45.  Lumboperitoneal shunt
- From lumbar space to peritoneal cavity
- For communicating hydrocephalus,CSF
fistula and pseudocerebri tumor
45

46.  Shunt between lateral ventricle and
cisterna magna called Torkildsen shunt
done in obstructive hydrocephalus.
 Ventriculopleural shunt
46

47.  Endoscopic third Ventriculostomy
- An osteomy is made along the floor of third
ventircle which drains CSF in prepontine cistern,
bypassing aquesuct of sylvius obstruction
 VENTRICULAR TAPPING
 An Ommaya reservoir is an
intraventricular catheter system
that can be used for the aspiration
of CSF or for the delivery of drug
47

48.  Other surgical procedures
- Choroid plexectomy
- Choroid plexus coagulation
- Cerebral aqueductoplasty
- Repeate lumbar puncture
48

49. COMPLICATIONS OF SHUNT
 It could be classified as mechanical like
blockage, disconnection, migration and
relative shortening of length.
 Flow rate complications are CSF over
drainage leading to subdural haematoma,
headaches, cranial deformities and
asymmetrical drainage leading to trapping or
isolation of part of ventricular system.
 Besides, ascites, loculations, hydrocele,
perforation of stomach, bowel, gall bladder
and vagina are seen.
49

50.  Shunt infection is common complication
accounting for mortality and morbidity for which
contamination of shunt system at the time of
surgery is the primary cause.
 70% present within 2months of the procedure
and remaining by 6 months.
 Characterised by fever, irritability, meningitis and
even a general lack of well being.
 Can be confirmed by CSF examination
 Most common strategy is removal of shunt with
placement of external drain and antibiotics.
50

51. SPECIAL TYPES OF HYDROCEPHALUS
1) Post hemorrhagic
- Hydrocephalus in babies with germinal matrix
hemorrhage in preterms account for 20%
- Ventricular catheter with subcutaneous reservoir
is safer option
- Recently , intraventricular fibrinolytic therapy is
instituted soon after the haemorrhage, has
shown to reduce shunt dependency.
51

52. 2) Normal pressure hyhdrocephalus
- Usually seen in adulthood, type of communicating
hydrocephalus
- Associated with head injury or meningitis
52

53. 3) Hydrocephalus with venous hypertension
- the deformed base skull results in narrowing of
jugular foramen leading to impaired intracranial
venous drainage
- The raised pressure with the cranial venous
sinuses reduces the pressure gradient results in
impaired absorption
- Similar clinical situations have been described in
achondroplasia
53

54. 4)Multiloculated hydrocephalus
- Occurs after initial episodes of neonatal
meningitis or a germinal matrix haemorrhage
- Multiple shunt or single shunt with multiple
fenestrations or craniotomy with lysis of
intraventricular septations, cyst aspirations or
cyst fenestrations with shunt insertion.
- The intellectual outcome has been poor, only
20% are expected to reach normal level
54

55. 5) Hydrocephalus ex vacuo
-Indicated enlargement of ventricles secondary to
parenchymal damage
- Associated with aging, head trauma, severe
infection, hypoxia or ischemic insults.
- Also following chemotherapy and radiotherapy
has been noted
- Structural abnormalities of brain like
calpocephely, holoprocencephaly and agenesis
of corpus callosum are also associated.
55

56. INTELLECTUAL OUTCOME
The prognostic factors reported are
(a) Clinical – etiology, degree of sensory and
motor deficits, level of meningocele, severity of
hydrocephalus, degree of ventricular dilation,
seizures and ventriculitis
(b) also – age of surgery, degree of ventricular
dilation, continuing of shunt function and
complication, type of hydrocephalus, associated
anomalies
56

57.  In addition, genetic , social, educational and
economic background also influence the issue.
 IQ more than 70 has been considered as normal
 The extent of improvement of mental age was
significantly higher when csf drainge was done
prior to 6 months.
 The children with OFC more than 50cm and shunt
intervention done beyond age of 18 months had
developed higher incidence of subdural
haematoma and intellectual outcomes were poor.
 Suggesting cortical mantle losses its ability to
reconstitute, beyond a stage.
57

58. PREVENTION
 Early diagnosis and treatment of CNS
infection
 Neural tube defects should be
prevented by giving folic acid to
mother
 Mother should be screened for
intrauterine infection before
conception and treated
 Genetic counselling for genetic
disorders 58

59. FOLLOW UP
- Head circumference should be monitored
- parents should be educated regarding
signs of shunt infections or blockage
- Periodic re-evaluation should be done
59

60. Chiari malformation
 Type I typically produces symptoms during
adolescence or adult life and is usually not associated
with hydrocephalus.
 deformity consists of displacement of the cerebellar
tonsils into the cervical canal.
 Syrinx of the spinal cord,especially the cervical region
should be looked for on MRI imaging.
 obstruction of the caudal portion of the fourth ventricle
during fetal development is responsible
60

61.  Patients complain of recurrent headache,
neck pain, urinary frequency, and
progressive lower-extremity spasticity.
61

62.  type II Chiari malformation is characterized by
progressive hydrocephalus with a
myelomeningocele.
 This lesion represents an anomaly of the
hindbrain, probably owing to a failure of
pontine flexure development during
embryogenesis, and results in elongation of
the fourth ventricle and kinking of the
brainstem, with displacement of the inferior
vermis, pons, and medulla into the cervical
canal
62

63.  symptoms during infancy, consisting of stridor,
weak cry, and apnea, which may be relieved by
shunting or by decompression of the posterior
fossa.
 abnormalities of gait,spasticity, and increasing
incoordination (including the arms and hands)
during childhood.
 Plain skull radiographs show a small posterior
fossa and a widenedcervical canal. CT scanning
with contrast and MRI display the cerebellar tonsils
protruding downward into the cervical canal and 63

64. Dandy-Walker malformation
 cystic expansion ofthe fourth ventricle in the
posterior fossa and midline cerebellar
hypoplasia,which results from a developmental
failure of the roof of thefourth ventricle during
embryogenesis .
 Infants present with a rapid increase in head size
and a prominent occiput.
 Transillumination of the skull may be positive. Most
children have evidence of long-tract signs,
cerebellar ataxia, and delayed motor and cognitive64

65.  The Dandy-Walker malformation is
managed by shunting the cystic cavity
(and on occasion the ventricles as well)
in the presence of hydrocephalus.
65

66. Thank You
66