Lecture notes for medical students

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2. Introduction & History.

3. Introduction & History.
Term derived from two Greek words-
 “hydro” means water and
 “cephalus” means head.
Hydrocephalus can be defined broadly as a
disturbance of formation, flow, or
absorption of cerebrospinal fluid (CSF) that
leads to an increase in its volume.

4. Etiology

5. Etiology
• Idiopathic
• Congenital
• Traumatic
• Infections /Infestation
• Autoimmune
• Neoplastic (Benign/Malignant)
• Degenerative

6. Etiology
1.CONGENITAL HYDROCEPHALUS
I. Intrauterine infections: Rubella,
Cytomegalovirus, Toxoplasmosis.
II. Trauma: Subarachnoid, Intracranial,
Intraventricular haemorrhages.
III. Congenital malformations:
Dandy-walker syndrome: posterior fossa
cyst continuous with 4th ventricle.

7. Etiology
• Aqueduct stenosis: it accounts for
33% of hydrocephalus cases. Stenosis
of aqueduct of sylvius causes dilation
of lateral and 3rd ventricles. In 2% of
cases this could be familial with X
linked recessive inheritance.
• Arnold-Chiari syndrome- Portions of
cerebellum & brainstem herniating
into cervical spinal canal, blocking
the flow of CSF to the posterior fossa.

8. Etiology
2) ACQUIRED HYDROCEPHALUS: -
• Tuberculosis,chronic & pyogenic
meningitis.
• Post-intraventricular hemorrhage.
• Posterior fossatumors:
medulloblastoma, astrocytoma,
ependymoma.
• Arteriovenous malformation,
intracranial hemorrhage, ruptured
aneurysm.

9. Physiology of csf production &
flow
CSF is secreted at the choroid plexus within the ventricles
by ultra filtration & active secretion.
Lateral ventricles
FORAMEN OF MONRO
3rd ventricle
AQUEDUCT OF SYLVIUS
4th ventricle
CONTD…

10. Contd……
Via lateral foramen of Luschka & foramen
of Magendie
Cistern magna
Cerebral & cerebellar subarachnoid
spaces

11. ……Contd
• A large portion is absorbed through the
arachnoid villi, but the sinuses, veins, brain
substance & dura also participate in
absorption.
• About 20ml of CSF is secreted in an hour.
The total of CSF approximates 50ml in an
infant and 150ml in adults.

12. Pathophysiology & Classification

13. Pathophysiology & Classification
There are two types of hydrocephalus: -
1) Noncommunicating (intraventricular or
obstructive)
2) Communicating hydrocephalus.

14. Noncommunicating
hydrocephalus

15. Noncommunicating
hydrocephalus
• There is blockage between the ventricular &
subarachnoid systems, resulting in an
interference with circulation of CSF & lack
of access to the subarachnoid spaces.
• CSFdistends the ventricles.
• There is a gradual thinning of the brain
substance, which is compressed between the
distended ventricles & the expanding skull.

16. Noncommunicating
hydrocephalus
• Stenosis of the aqueduct of sylivus, either a
congenital defect or acquired.
• postnatally from brain tumors that put
pressure on or extend into the ventricles or
circulation pathways.

17. Communicating
hydrocephalus.

18. Communicating
hydrocephalus.
• There is normal communication between
the ventricles & the subarachnoid space.
• There is an interference with the absorption
of CSF caused by an occlusion of the
subarachnoid cisterns around the brain
stem.
• The fluid that is not absorbed in the
subarachnoid space accumulates,
compressing the brain & distending the
cranial cavity.

19. Communicating
hydrocephalus
• Subarachnoid hemorrhage
• Meningitis
• Toxoplasmosis or cytomegalovirus
infection,
there is obliteration of the subarachnoid
spaces by fibrous tissue reaction, or to
diseases of connective tissue.

20. Clinical Features

21. Clinical Features
• Demography
• Symptoms
• Signs
• Prognosis
• Complications

22. Demography

23. Demography
• Incidence of primary hydrocephalus 1 in
2500 live births,
• one of the most common developmental
disabilities
• Equal male :female.

24. Symptoms & Signs

25. Symptoms & Signs
IN INFANTS-
• Head grows at abnormal rate.
• Anterior fontanel is tense, often bulging, & non pulsatile.
• Scalp veins are dilated & markedly so when infant cries.
• Macewen’s sign- with increase in intracranial volume, the
bones of the skull become thin & the sutures become
palpably separated to produce the cracked pot sound on the
percussion of the skull.
• Frontal bossing with depressed eyes.
• Setting-sun sign- eyes rotated downward, in which sclera
may be visible above iris.

26. Setting sun sign

27. Symptoms & Signs
• Feeds poorly
• Pupils are sluggish, with unequal response to light
• Changes in level of consciousness.
• Opisthotonus position & lower extremity spasticity.
• Cries when picked up & quiets when allowed to lie
still.
• If hydrocephalus is allowed to progress- there will
be disruption in the lower brainstem function as
manifested by difficulty in feeding & a shrill, brief,
high-pitched cry. Eventually the skull becomes
enlarged, & the cortex is destroyed.

28. Symptoms & Signs
• If the condition progress rapidly, the infant
may display emesis, somnolence, seizures &
cardiopulmonary distress.

29. Symptoms & Signs
IN CHILDHOOD-
• Headache on awakening with improvement
following emesis or upright posture.
• Papilledema, strabismus (Squint).
• Irritable & lethargic, Drowsiness
• Apathetic, confused & often incoherent.
• Bulging occiput, nystagmus, ataxia & cranial nerve
palsies.
• Stunted growth and sexual maturation

30. Symptoms & Signs
• IN Adults:
• Cognitive deterioration:
• Headaches: Headache is rarely if ever present in
normal pressure hydrocephalus (NPH).
• Neck pain:
• Nausea Vomiting:
• Blurred vision
• Double vision (horizontal diplopia) from sixth
nerve palsy
• Difficulty in walking
• Drowsiness
• Incontinence (urinary first, fecal later if condition
remains untreated):

31. Investigations

32. Investigations
• Laboratory Studies
– Routine
– Special
• Imaging Studies
• Tissue diagnosis
– Cytology
• FNAC
– Histlogy

33. Diagnostic Studies

34. Diagnostic Studies
Imaging Studies
• X-Ray-erosion of sella turcica, or "beaten
copper cranium" (or "beaten silver
cranium")
• USG
• CT
• Angiography
• MRI
• Endoscopy
• Nuclear scan

35. CT

36. Non Operative Therapy

37. Non Operative Therapy
This can be tried in mild cases of
hydrocephalus.
• Acetozolamide: dose of 50mg/kg/day
dimnishes CSF production.
• Oral glycerol has also been used for the
similar purpose.

38. Operative Therapy

39. Operative Therapy
It may consist of
• The removal of the obstruction (tumor, hemorrhage
or cyst) to the flow of CSF.
• Reduction in the amount of CSF produced through
destruction of a portion of the choroid plexus or a
third or fourth ventriculostomy.
• Shunting of CSF from the ventricle to another site in
the normal circulatory passageway of this fluid.
• Shunting of CSF from the ventricle to an area
outside the CNS, an extracranial body compartment.

40. Operative Therapy
Shunting is the most common procedure to
be done in the surgical management of
hydrocephalus.
Most shunt systems consist of a ventricular
catheter, a flush pump, a unidirectional flow
valve & a distal catheter

41. Types of shunts-
..

42. Types of shunts-
..
1. Ventriculoperitonial(VP) shunt.
2. Ventriculoatrial(VA) shunt.
3. Ventriculopleural shunt.

43. Minimally invasive Therapy

44. Minimally invasive Therapy
• Endoscopic third ventriculostomy- It is a
procedure that has potential for greater
independence from VP or VA shunting in
children with noncommunicating
hydrocephalus. In this procedure a small
opening is made in the floor of the 3rd
ventricle, allowing CSF to flow freely
through previously blocked ventricle, thus
bypassing the aqueduct of sylvius.

45. External drainage

46. External drainage
Rapid-onset hydrocephalus with increased
intracranial pressure (ICP) is an emergency.
• Ventricular tap in infants
• Open ventricular drainage in children and
adults
• LP in posthemorrhagic and postmeningitic
hydrocephalus

47. NPH
• Normal Pressure Hydrocephalus.
• Variant of communicating Hydrocephalus
• Triad of
– Abnormal gait
– Urinary incontinence
– Dementia

48. NPH
• Elderly
• Normal muscle strength; no sensory loss
• Increased reflexes and Babinski response in
one or both feet:
• Variable difficulty in walking:
• Frontal release signs (in late stages):
Appearance of suckling and grasping
reflexes

49. NPH
• Imaging -Ventriculomegaly,
• Diagnostic CSF removal
• Surgical CSF shunting.

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